Vous êtes sur la page 1sur 34

A-PDF Split DEMO : Purchase from www.A-PDF.

com to remove the watermark

I lheIar
)()(I)(
Part
Eight prominent signs and symptoms
CLINICALMANIFESTATI0NS.
primarily with diseasesof the ear and temporal
fl:errassociated
Otalgia usually is associatedwith inflammation of the external
or middle ear, but it may represent pain referred from involvement of the teeth, temporomandibular joint, or pharynx. In
young infants, pulling or rubbing the ear along with general irritability or poor sleep, especiallywhen associatedwith fever, may
be the only signs of ear pain. Ear pulling alone is not diagnostic
of ear pathology.
Purulent otorrhea is a sign of otitis externa, otitis media with
perforation of the tympanic membrane (TM), drainage from the
middle ear through a patent tympanostomy tube, or, rarely,
drainage from a branchial cleft sinus. Bloody drainage may be
associatedwith acute or chronic inflammation (often with granulation tissue),trauma, neoplasm, foreign bodS or blood dyscrasia. Clear drainage suggestsa perforation of the TM with a serous
middle-ear effusion or, rarely, a cerebrospinal fluid leak draining
through defects (congenital or traumatic) in the external auditory
canal or from the middle ear.
Hearing loss results either from disease of the external or
middle ear (conductive hearing loss) or from pathology in the
inner ear, retrocochlear structures, or central auditory pathways
(sensorineuralhearing loss). The most common cause of hearing
loss in children is otitis media (OM).
Swelling around the ear most commonly is a result of inflammation (e.g., external otitis, perichondritis, mastoiditis), trauma
(e.g., hematoma), benign cystic masses,or neoplasm.
Vertigo is a specific type of dizzinessthat is defined as any illusion or sensation of motion. Dizziness is less specificthan vertigo
and refers to an altered orientation in space.Vertigo is an uncommon complaint in children; the child or parent may not volunteer information about balance unless asked specifically. The
most common cause of dizzinessin young children is eustachian
tube-middle-ear disease,but true vertigo also may be caused by
labyrinthitis, perilymphatic fistula between the inner and middle
ear due to trauma or a congenital inner ear defect, cholesteatoma
in the mastoid or middle ear, vestibular neuronitis, benign paroxysmal vertigo, Meniere disease,or diseaseof the central nervous
system. Older children may describe a feeling of room-spinning
or turning; younger children may expressthe dysequilibrium only
by falling, stumbling, or clumsiness.
Nystagmus may be unidirectional, horizontal, or ierk nystagmus. It is vestibular in origin and usually is associated with
vertlgo.
Tinnitus rarely is described spontaneously by children, but it
is common, especially in patients with eustachian tube-middleear diseaseor sensorineural hearing loss (SNHL). Children may
describetinnitus if asked directly about it, including laterality and
the quality of the sound.
The facial nerve may be dehiscentin its course
FACIALPARALYSIS.
through the middle ear in as many as 50"/" ol patients. Infection
with local inflammation, most commonly in acute OM, may lead
to a temporary paralysis of the facial nerve. It also may be due

atresia choanae, retarded growth, genital hypoplasia, and ear


anomalies), or it may be associated with other cranial nerve
abnormalities and craniofacial anomalies.

EXAMINATION
PHYSICAT
Complete examination with special attention to the head and
neck may reveal a condition that may predispose to or be associated with ear diseasein children. The facial appearanceand the

PaoooseBoard

Figure 63.5-1.Methods of restrainingan infant for examination and for prosr.h as tympanocentesisor myringotomy. (From Bluestone CD' Klein
..irr.,
Media in Infants and Children,2nd ed. Philadelphia' \UB Saunders,
Otitis
lO:
1 , 9 9 5p, 9 r . l

2518I PART)filX I TheEar

NORMAL
Position-neutral
Color-normal
Translucency-translucent
Mobility-moves brisklywith slight
positiveand negativepressure

NEGATIVEMIDDLEEAR
PRESSURE
Position-retracted
Color-normal
Translucency-translucent
Mobility-moves only with applied
negativepressure

ACUTEOTITISMEDIA
Position-full to bulging
Color-red (can be pink,white,or
yellow)
Translucency-opaque
Mobility-poor when both positive
and negative pressuresare
applied

FLUIDLEVEL
Position-retracted
Color-yellow or amber
Translucency-translucent
Mobility-same as with high
negativepressure,but fluid level
and bubbleschangewith applied
pressure

OTITISMEDIAWITH EFFUSION
Position-usuallyretracted
Color-white (or yellowor blue)
Translucency---opaque(may be
translucent)
Mobility-poor when both positive
and negativepressuresare
applied

PERFORATTON
(ORPATENT
ryMPANOSTOMY
TUBE)
Position-neutralor retracted
Color-white,pink,red,or normal
Translucency-translucent
or
opaque
Mobility-none
Figure 635-2' Common conditions of the middle ear, as assessedwith the otoscope. (From Bluestone CD,
Klein JO: Otitis Media in Infants and Children,
e d . P h i l a d e l p h i aS, T BS a u n d e r s2, 0 0 f , p 1 3 1 . )

andEvaluationr 2619
6it5I GeneralConsiderations
Chapter
with one hand and holding the child's head against the parent's
chest with the other hand. If necessary,the child's legs can be held
between the parent's knees.To avoid ear trauma with movement,
the examiner should hold the otoscope with the hand placed
firmly against the child's head or face, so that the otoscope moves
with the head. Pulling up and out on the pinna straightens the
ear canal and allows better exposure of the TM.
'When
examining the ear, inspection of the auricle and external
auditory meatus for infection may aid in the evaluation of complications of OM. External otitis mav result from acute OM with
iischa.ge, or inflammation of the posterior auricular area may
indicate a periosteitis or subperiostealabscessextending from the
mastoid air cells. The presenceof preauricular pits or skin tags
also should be noted because affected children have a slightly
higher incidence of SNHL.
Cerumen is a protective, waxy, water-repellent coating in the
ear canal that may interfere with examination. Cerumen usually
is removed using the surgical head of the otoscope, which allows
passageof a wire loop or a blunt curette under direct visualization. Other methods include gentle irrigation of the ear canal with
warm water, which should be performed only if the TM is intact,
or instillation of a solution such as diluted hydrogen peroxide in
the ear canal (with intact TM only) for a few minutes to soften
the wax for suction removal or irrigation. Some commercial
preparations such as trolamine polypeptide oleate-condensate
(Cerumenex) may cause dermatitis of the external canal with
chronic use and should be used only under a physician's
suDervrslon.
lnflammation of the ear canal with associated pain often
indicates external otitis. Abnormalities of the external auditory
canal include stenosis (common in children with trisomy 21),
bony exostoses, otorrhea, and the presence of foreign bodies.
Cholesteatoma of the middle ear may manifest in the canal as
intermittent foul-smelling drainage, sometimes associated with
white debris; cholesteatoma of the external canal may appear as
a white, pearl-like mass in the canal skin. White or gray debris
of the canal suggestsfungal external otitis. Newborn ear canals
are filled with vernix caseosa,which is soft and pale yellow and
should disappear shortly after birth.
The TM and its mobility are best assessedwith a pneumatic
otoscope. The normal TM is in a neutral position; a bulging TM
may be caused by increased middle-ear air pressure, with or
without pus or effusion in the middle ear; a bulging drum may
obscure visualization of the malleus and annulus. Retraction of
the TM usually indicates negative middle-ear pressure,but it also
may result from previous middle-ear diseasewith fixation of the
ossicles,ossicular ligaments, or TM. When retraction is present,
the bony malleus appears more prominent, and the incus may be
more visible posterior to the malleus.
The norrnal TM has a silvery-gray, "waxed paper" aPpearance
(Fig. 535-2). A white or yellow TM may indicate a middle-ear
effusion. A red TM alone may not indicate pathology, because
the blood vesselsof the membrane may be engorged as a result
of crying, sneezing, or nose blowing. A normal TM is translucent, allowing the observer to visualize the middle-ear landmarks: incus, promontory, round window niche, and, often, the
chorda tympani nerve. If a middle-ear effusion is present, an airfluid level or bubbles may be visible (seeFig.635-2).Inability to
visualize the middle-ear structures indicates opacification of the
drum, usually causedby thickening of the TM, a middle-ear effusion, or both. Assessmentof the light reflex usually is not helpful,
because a middle ear with effusion reflects light as well as a
normal ear.
TM mobility is helpful in assessingmiddle ear pressuresand

TM position and brisk TM movement to both positive and


negailve pressures.
Eardrum retraction is most common when negative middle-ear
pressureis present; with even moderate negative middle-ear presiure there is no visible inward movement with applied positive

is mixed with air, the TM may still have some mobility. Outward
eardrum movement is less likely in the presenceof severenegative middle-ear pressure or middle-ear effusion.
The TM that exhibits fullness (bulging) moves to applied pos-

inflammatory processesor neoplasms. Specializedassessmentof


labyrinthine'function should be considered in the evaluation of a
chiid with a suspectedvestibular disorder (see Chapter 540).

Figure 635-3. Tympanocentesiscan be performed with a needle attached to a


tuberculin syringe (left) or by using an Alden-Senturiacollection trap (Storz
Instrument Co, St. Louis). (From BluestoneCD, Klein lO: Otitis Media in
Infants and Children,2nd ed. Philadelphia,I7B Saunders,1995' p 127'l

2520r PAHTXXIXr TheEar

young children is considered,the number of affected chjldren


increasessubstantially.

rncludeatresiaor stenosis,impacted cerumen,or foreign bodies.


In the middle ear, perforation of the tympanic membrine (TM),
discontinuitl'or fixation of the ossicularchain, otitis media (OM j
with effusion, orosclerosis,and cholesteatomacan causeCHL.
Damage to or maldevelopmentof structuresin the inner ear
can causesensorineuralhearingloss (SNHL). Causesinclude hair
cell desrructionfrom noise, disease,or ototoxic agents;cochlear
malformation; perilvmphaticfistula of the round oi oval window
rnembrane;and lesionsof the acousticdivision of the Sth nerve.
A combination of CHL and SNHL is considereda mixed hearing
loss.

two ears properln ro processspeechwhen it is slightly degraded,


and to integrateauditory information when it is delivereJfastei
alrhough rhey can processit when deliveredat a slow rate. These
deficrtsmay manifesr as poor artention or academicor behavior
problems rn school. Srrategiesfor coping with such disordersare
available for older children, and identification and documenration of the cenrral audirory processingdisorder often is valuable
becauseonce parentsand teachersare aware of a valid reasonfor
the child's poor artention or behavior,adjusrmentscan be made.
. The etiology of a hearing impairment depends on
he hearing loss is conductive oi sensorineuial.Most
CHL is acquired,with middle ear fluid the most common cause.
Congenital causes include anomalies of the pinna, external ear
canal, TM, and ossicles. Rareln congenital cholesteatoma or
other masses in the middl. ."r -"y present as CHL. TM

perforation (e.g., trauma, OM), ossicular discontinuity (e.g.,


infection, cholesteatoma, trauma), tympanosclerosis, acquired
cholesteatoma, or masses in the ear canal or middle ear (e.g.,
Langerhans' cell histiocytosis, salivary gland tumors, glomus
tumors, rhabdomyosarcoma) also may present as CHL. Uncommon diseasesthat affect the middle ear and remporal bone and
may present with CHL include orosclerosis,osteopetrosis,fibrous
dysplasia, and osteogenesisimperfecta.
SNHL may be congenitalor acquired.Acquired SNHL may be
causedby genetic,infectious,autoimmune, anatomic, traumatic,
ototoxic, and idiopathic factors (Tables636-1.,636-2,636-3, and
636-41. The recognized risk factors accounr for about 50% of
casesof moderate to profound SNHL. The most common infectious cause of congenital SNHL is cytomegalovirus (CMV),
which infects 1/100 newborns in the USA (seeChapter 252\. Of
these, 6,000-8,000 infants each year will have clinical manifestations, including approximately 75% with SNHL. Congenital
CMV warranrs special arrention because it is associated with
hearing loss in its sympromaric and asymptomatic forms, and the
hearing loss may be progressive. Some children with congenital
CMV have suddenlylost residualhearing at 4-5 yr of age.Much
less common congenital infectious causesof SNHL include toxoplasmosis and syphilis. Congenital CMV, toxoplasmosis, and
syphilis also may presenr with delayed onset of SNHL, months
to years after birth. Rubella, once the most common viral cause
of congenital SNHL, is very uncommon becauseof effective vaccination programs. In utero infection with herpes simplex virus
i s r a r e . a n d h e a r i n gl o s si s n o t a n i s o l a r e dm a n l f e s t a r i o n .

(BIRTH-AGE
FoRUsE
W|TH
Ntot{ATts
28D)Wt{EilUiltVERsAr
SCREEIilNG
tSiloTAvAttABtE
(hildhood
Family
history
ofhereditary
sensorineural
hearing
los
Inutero
infection,surh
ascytomegalovirus,
rubella,
syphilis,
herpes
simplex,or
toxoplasmosis
(raniofacial
anomalies,
induding
those
withrnorphologic
pinna
abnormalities
ofthe
anoearcanat
weighr
q (33 lb)
Birth
<1500
Hyperbilirubinemia
ataserum
level
requiring
exchange
trandusion
ftotoxic
medrcationt
including
butnotlimited
totheaminoglyc0sides,
used
inmultiple
cou6es
0rin
combination
withloop
diuretic5
Eacerial
meningitis
Apgar
scores
of0-4at1min0r0-6at5 mjn
Mechanical
ventilation
lasting
>5d
Stigmata
orother
findings
asso(iated
withasyndrome
known
toinclude
asensorineural
and/or
conductive
hearing
loss
(AGT
IORUsE
WITH
INFAIITS
29D-2YR}WHEN
CEflTAIN
HEATTI{
C()NDITIONS
DEVTTOP
THAT
REOUIRE
RESCREET'llt{G
Parent/rareglver
concern
regarding
hearing,speech,
language,and/or
developmental
delay
Bactenal
meningitis
andother
infections
aso(iated
withsensorineural
hearing
loss
Head
trauma
associated
withlosofconsciousnes
orskull
fracture
stigmata
0rother
findings
associated
withasyndrome
known
toindude
asensorineural
and/or
conductive
hearing
los
ftotoxic
medicationtincluding
butnotlimited
tochemotherapeutic
agen6
oraminoglycosides
used
in
(ou6es
multiple
0rincombination
withl00p
diuretics
Rerunent
orpersistent
otitis
media
witheffr.lsion
foratIeast
3 mo
(AGE
TOR
UsE
WITH
INIA}ITS
29D-3YB)WH()
REQUIRT
PERIODIC
MONITORING
OTHEARING
(Some
newborns
andinfants
maypass
initial
hearing
periodi(
screening
butrequire
monitoring
ofhearing
todetect
delayed-onset
sensorineural
and/or
conductive
hearing
loss.lnfants
wilhthese
indkat06
require
hearing
evaluation
atleast
every
6 m0untilage3yr,and
atappropriate
intervals
thereafter.)
I1{DICAT()RS
ASSOCIATED
WITH
DTI.AVED{NsET
SENSORIIIEUf,AI
HEARIIIG
LOSS
Family
history
ofhereditary
childhood
hearing
los
infectron,
lnulero
such
ascytomgalovirus,
rubella,syphilis,
hupes
simplex,
ortoxoplasmosis
Neurofrbromato'sis
type
2andneurodegenerative
disorden
IiIDICATOR5
ASSOCIATED
W]IHCOI{DUOIVI
HHRIIIG
TOSS
Re(urrent
orperistent
otitis
media
whheffusi0n
Anatomic
deformities
andother
dis0rde6
thataffed
eushrhian
tube
fun(tion
Neurodegene{ative
disorde6
Adapted
(ommitEe0nlnfafltHeainqi9g4
fromAmerican
Acadeoy
0fPediitrics,J0int
tommittee
onlnfantHearingi]oint
Porition
slatemenl.Pedlrlri(
1995;95:
l5?.

636 r HearingLossr 2621


Chapter

mcus

GENE

AUDIO
PHENOTYPE

Conductive
hearing
loss
fixati0n
mimicklng
otoselerosis;
dueto5tapes
progressive
superimposed
5NHL
DIAPH1 Low{requency
loss
beginning
inthefirstdecade
andprogressing
t0all
DTNA1
withprofound
loses
frequencies
topmdure
aflataudioprofile
throughout
theauditory
range
K(NO4 5ymmetrical
high-frequency
sensorineural
loss
beginning
inthefint
DFNA2
decade
andprogresing
over
allfrequenries
losbeqinning
inthethird
6l83
Symmetriral
hiqh-frequency
sensorineural
decade
W.SI
Early-onset
low{requency
sensorinerual
loss;about
75%offamilies
DFNA6i14/]8
(arrymissense
mutalions
in
dominantly
segregating
thisaudioprofile
thefterminal
domain
ofwolframin
Progresive
sloplng
DFNAl
O
TYA4
loss
beginning
inthesecond
decade
asaflattogently
audioprofile
thatbecomes
sloping
withage
steeply
(0111
A2 (ongeniul
mid.ftequency
loss
thatshows
DFNAl3
sensorineural
age-related
progression
arross
theauditory
range
progressive
inthesecond
decade
POU4I3 Bilateral
sensorineural
loss
beginning
DINA15
progresive
loss
beginning
inthesecond
decade;
DFNA20/26 A(rG|
Bilateral
sensorineural
withage,the
loss
In(rea5es
withthrsh0ld
shifts
inallfreguenries,
asloping
ismaintained
lnmost
cases
although
confgurati0n
(0mmon
genotype,
loss
varies
frommildtoprofound
Themost
DFNB]
6J82,
Hearing
inabout
isassociated
withsevere
toprofound
5NHL
G]86
35del6/35delG,
in
topmfound
deafness
Bobserved
90%ofaffected
children;severe
carrying
one
only60%ofchildren
whoarecompound
heterozygotes
in
6182
SNHL-causing
allele
variant;
35delG
allele
and
any
other
mutati0ns,
severe
children
canying
two6J82
SNHL<ausing
missense
toprofound
deahess
isnotobserved
(see
Table
DFN84
5LQ6A4 DFN84
andPendredt
syndrome
636J)areallelic
DFNB4
withdilatation
0fthe
ve$ibular
aqueduct
hearing
losisassociated
Inthehigh
frequencies,the
losis
and
can
beunilateral
orbilatetal
varies
degree
ofloss
severe
toprofound;
inthelowftequencies,the
(prelingual),but
progressive
widely
becongenital
0nset
can
postlingual
losalso
rscommon
is
I2SrRNA Degree
ofhearing
losvaries
frommildtoprofound
butusually
mtDNA
pre(ipitous
affe(ted;
>G
symmetlGl;
highfrequencies
arepreferentially
1555A
therapy
losinhearing
can
occur
after
aminoglycoside
DFN]

P)u3f4

loss
5NHL,sensorineufal
hearing

RlH,
Bale
lFJlWhite
KR:
Sens0nBeural
hearing
losinchildren
/ancet2ffi5365:879
From
5mith

Other oostnatal infectious causesof SNHL include neonatal


group B sireptococcalsepsisand bacterialmeningitisat any age.
Streptocctccuspneumoniae is the most common cause of bacterial meningitis that results in SNHL after the neonatal period and
has become lessfrequent with the routine administration of pneumococcal conjugate vaccine.Haemophilus influenzaetype b, once
the most common causeof meningitisresulting in SNHL, is rare

INTECTIONS
CONGENITAT
[ytomegalovrrus
virus
choriomeninqitis
Lymphocync
avttus
Rubel
qondii
[oxoplosno
pollidun
Treponeno
INFEOIONs
ACQUIRED
Borrelio
burgdarferi
virus
Barr
Epsteif
tnfluenzoe
Hoenophtlus
V US
LASSA
virus
Measles
vtrus
Mumps
neningittdh
Neisseria
entero\/tru5eS
Non-oolio
folciparum
Plosnodium
5trcatacacut
Dneun1niae
virus
zoster
Var(ella
2005J65:879-890
lan(Pf
hearing
losinchildten
KR:Sensorineural
lFlr.,While
Bifl,Bale
From
5mith

owing to the Hib conjugate vaccine. Uncommon infectious causes


of SNHL include l,yme disease, parvovirus 819, and varicella.
Mumps, rubella, and rubeola, all once common causes of SNHIin children, are rare owing to vaccination programs.
(ienetic causes of SNHL probablv are responsible for as many

as -50% of SNHL cases(seeTables636-2 and 636-3). Thesedisorders may be associatedwith other abnormalities'may be part
of a named syndrome, or may exist in isolatlon' SNH[. often
occurs with abnormalitiesof the ear and eye and with disorders
of the metabolic, musculoskeletal,integumentarn renal, and
nervous systems.Autosomal dominant hearing lossesaccount for
about 107u of all casesof childhood SNHL. Waardenburg(types
I and II) and branchio-otorenal syndromes represent2 of the
most common autosomal dominant syndromic types of SNHL.
Tvoes of SNHL are coded with a 4-letter code and a number, as
fc,ilows, DFN = deafness, A = dominant, B = recessive, and
number = order of discovery,e.g. DFNA 13. Autosomal dominant conditionsin addition to thosejust discussedinclude:DFNA
I t o 1 1 , 1 3 , 1 5 , 1 7 , 2 0 , 2 2 , 2 8 , 3 6 , 4 8 a n d m u t a t i o n si n t h e c r y s tallin gene (CRYM). Autosomal recessivegenetic SNHL, both
syndromic and nonsyndromic, accounts for about 80% of all
clildhood casesof SNHI-. Usher syndrome (types 1,2, and 3),
Pendredsyndrome, and the Jervell and Lange-Nielsensyndrome
(one form of the long Q-T syndrome)are 3 of rhe most common

GENE

PHENOTYPE

DOMINAI'IT
(W51)
Waardenberg

PAX]

relattve
frst-degree
forelock;
andanaffeoed
irses;
white
los;heterochromic
hearing
congenital
acanthorum;
aiteria
indude
dystop
Major
diagnostic

(W52)
Waardenberq

M/lF,others

Branchio-otorenal

EYAl

RECESSIVE
syndrome
Pendred's

5LC26A4

biateral
The
(40%),
lities
abnorma
ear(3070)
andexternal
renal
ial(7070),
pits{85%),
andbranch
prea
uricular
a incl
udehealngoss(9870),
Diagngstic
iriter
indegree,
andmildtoprofound
otmixed,
sensonneural,
loscanbeconductive,
hearing

and
butcanbelae-onset
cases,
inmany
toprofound
andsevere
n0npr0gressive,
loss
thatrscongenital,
hearing
include
sensorineural
Dlagnostir
cfitetia
perchlorate
test0rg0iter'
dlscharge
andanabnormai
hypop
asia;
cochlear
withorwithout
progresive;
aqueduct
oIthevestlbular
bilaeral
dllation
(ommonly
until
notdiagnosed
pigmentosa
(0nqenital,
areflexia,
andretlnitis
vestibular
loss,
hearing
indude
biatera
Diagnoit
c criteria
1(USH1) USHIA,
MY07A,
USHIC,(DH23,
, andprofound
Ushersyndrometype

USHIE,
P(DHI
5,UsHlG
(U5H2) USH2A,USH28,USH2(,othe$
Ushersyndrometype2
type
3(USH3) USH]
Usher
syndrome
functron
ldnref
2005;365:879-8m
Bale
KR:Sensoaneural
hearinq
loss
inchildren
From
Smirh
RJH,
lFlrWhite

I 169 6r,
2622I PART11;1IX
syndromic recessivetypes of SNHL. Other autosomal recessive

autosomal recessive(DNFB 1) and autosomal dominant (DNFA


3) and in sporadic nonsyndromic patienrs with SNHL; up to 50%
of nonsyndromic SNHL may be related to a mutarion of
connexin-25. Mutations of the GJ82 geneco-localize with DFNA
3 and DFNB 1 loci on chromosome 13, are associatedwith auto-

hearing loss. Congenital anomalies causing CHL include malformations of the ossiclesand middle-ear srructures and atresia of
the external auditory canal.
Many genetically determined causes of hearing impairment,
both syndromic and nonsyndromic, do not express themselves
until some time afrer birth. Alport, Alstrom, and Down syndromes, von Recklinghausen disease, and Hunter-Hurler syndrome are genetic diseases that may have SNHL as a late
manifestation.
SNHL also may occur secondary to exposure to toxins, chemicals, and antimicrobials. Early in pregnancy, the embryo is particularly vulnerable to the effects of toxic substances.Ototoxic
drugs, including aminoglycosides,loop diuretics, and chemotherapeutic agents (cisplatin) also may cause SNHL. Congenital
SNHL may occur secondary to exposure to these drugs as well
as to thalidomide and retinoids. Certain chemicals, such as
quinine, lead, and arsenic, may cause hearing loss both pre- and
postnatally.
Trauma, including temporal bone fractures, inner ear concussion, head trauma, iatrogenic trauma (e.g., surgery, extracorporeal membrane oxygenation [ECMO]), radiation exposure, ind
noise, also may cause SNHL. Other uncommon causesof SNHL
in children include immune disease (systemic or limited to the
inner ear), metabolic abnormalities, and neoplasms of the temporal bone.
Sudden hearing loss in a previously healthy child is uncommon
but may be due to otitis media or other middle ear pathologies.
Usually these causes are obvious from the history and physical
examination. Sudden loss of hearing in the absence of obvious
causesoften is due to a vascular event affecting the cochlear apparatus or nerve, such as embolism or thrombosis (secondary to
prothrombotic conditions). Additional causesinclude perilymph
fistula, drugs, trauma, and the first episode of Meniere Jyndrome.

abnormalities are quite common; in as many as 20To of children


with SNHL, obvious or subtle temporal bone abnormalities are
seen on high-resolution CT scanning or MRI.

progressive, or fluctuating; and affecting a pafi, or all, of the


audible spectrum. Other factors, such as intelligence, medical or
physical condition (including accompanying syndromes), family
support, age at onset, age at time of identification, and prompt-

vestibular aqueduct and cochlear dysplasiaare present in this section.


In the larger of the two inset images of a normal temporal bone, the
vestibular aqueduct is visible but much smaller (arrota). The cochlea
appears normal, and in the smaller inset image of a more inferior
axial section, the expected number of cochlear turns can be clearly
counted (*in internal auditory canal). (From Smith RJH, Bale JF Jr,
White KR: Sensorineural hearing loss in children. Lancet 2005:
365:879-890.1

636 I HearingLossr 2623


Chapter

THBTSHOTO
AVERAGI
TEVET
(dB)AT500-2,000
Hz(ANSI) DES$lPTlotl
Normal
0-15
mnge
16-25
Slight
hearing
los

(OMMON
CAU5TS

(IFNOT
DEGRTE
OFHAIJOIffP
ON BEHEARD
WHAT
P(OBABIE
I'if[05
rN15TYRot iltf)
AMPLITICATION TRIATID
WITHOUT

hearinq
Conductive
oss
perforation,
0tltismedia,Tl\4
tympanosclerosis;
Eustachian
tubedysfunctron;
some
SNHt

Allspeech
sounds
may
Vowel
headdearly,
sounds
(0n50nanI
mt55
unvOrceo
so|]nds

N0ne
1n
dy(unoion
Mildauditory
rearn
ng
language
some
inperceiving
Difficulty
speecn
s0!n05

25 30

t\4itd

perforation,
0titis
media,TM
ero5i5,severe
tympanos(
dysfunctron,
SN|1L
eustachian

learnrng
dysfunction
sounds, Auditory
ofspeech
Hears
onlysome
language
retardation
iVild
sounds
voiced
thelouder
ptoblems
Mildspeech
lnalteftr0n

l0 50

Moderate
hearing
los

Chronic
otitis,
earcanaJ/mlddle
ear
anomaly,5Nl-1L

at
so|]nds
|\4isses
mostspeech
level
normal
conversationa

5070

hearlng
los
Severe

sound
ofnormal
Hears
nospeech
SNHL
ormixed
loss
duetoa
ronversation5
combinalon
ofmddle-ear
dsease
andsensorineural
invol\/ement

70+

los
Profound
hearing

ormixed
5NHL

orother
sounds
Hean
nospeech

problems
Speerh
retar0al0n
Language
[eaming
dysfunction
hattentr0n
probLems
speech
Severe
rebrdation
Languaqe
Learning
dysfunction
tnaIIent]0n
problems
Severe
speech
retardation
Language
on
Learninq
dystunct
l0attentr0n

None
aid;
ofneed
forhearing
Consideration
ttaining
speech
reading;
auditoty
5peech
therapy
5urgery
Appr0pnale
Preterentral
seating
Hear
ngaid
Lipreading
Auditory
training
Speech
therapy
surqery
Appr0pflate
plus
corsiderati0n
0t
Alloftheabove,
(lassfoom
situation
special

asstgnment
A| 0fth..above;pr0bable
tospecial
dases

probable
asignment
Alloftheabove;
orschools
classes
tosperial

los;Tlvl,
AN5l,
Aflrerican
Nati0nal
Standards
lnstitut;5NHL,sensorineural
hearing
tympanrr
membrane
fromNonhern
ll,DownsMP:He1in|
in(h/drc4
&Wilkins,1991
It/0dified
4thedBaltimore,Williams

ness of intervention, also affect the impact of hearing loss on a


child.
Most hearing-impaired children have some usable hearing.
Only 6% of those in the hearing-impairedpopulation have bilateral profound hearing loss. Hearing loss very early in life can
affect the developmentof speechand language,social and emotional development,behavior,attention, and academicachievement. Some cases of hearing impairment are misdiagnosed
because affected chrldren have sufficient hearing to respond to
environmenlal sounds and can learn some speechand language
but when challenged in the classroom cannot perform to full
potential.
Even mild or unilareral hearing loss may have a detrimental
effect on the development of a young child and on school performance. Children with such hearing impairments have greater
difficulty when listening conditions are unfavorable (e.g., background noise and poor acoustics),as may occur in a classroom.
The fact that schools are auditory-verbal environments is unappreciatedby those who minimize the impact of hearing impairment on learning.Hearing loss should be consideredin any child
with speechand languagedifficultiesor below-par performance,
poor behavior,or inattention in school (Table 636-5).
Children with moderate, severe,or profound hearing impairment and those with other handicapping conditions often are
educated in classesor schools for children with special needs.The
auditory managementand choicesregarding modes of communication and educationfor children with hearing handicapsmust
be individualized,becausethesechildren are not a homogeneous
group. A team approach to individual casemanagementis esserrtial, becauseeach child and family unit has unique needs and
abilities.
Hearing impairment can have a maior
HEARINGSCREEI{ING"
impact on a child's development, and becauseearly identification
improves prognosis, screening programs have been widely and
strongly advocated. Data from the Colorado newborn screening
program suggest that if hearing-impaired infants are identified
and treated by age 6 mo, these children (with the exception of

those with bilateral profound impairment) should develop the


same level of languageas their age-matchedpeers who are not
hearing impaired. This is compelling support for the establishment of mindated newborn hearing screening programs for all
children. The American Academy of Pediatricsendorsesthe goal
of universal detection of hearing loss in infants before 3 mo of
age, with appropriate intervention no later than 6 mo of age. Currentlv, heaiing screening has been mandated in 32 states in the
USA.
Until mandatedscreeningprograms are establisheduniversally,
manv hosoitals will continue to use other criteria to screen for
hearing lois. Some use the high-risk criteria (seeTable 636-1'l to
decide-which infants to screen; some screen all infants who
require intensive care; and some do both. The problem with using
hieh-risk criteria to screenis that 50% of casesof hearingimpairmint will be missed, either because the infants are hearing
impaired but do not meet any of the high-risk criteria, or because
they develop hearing loss after the neonatal period.
The recommended hearing screening techniques are either
otoacoustic emissions (OAE) testing or auditory brainstem
evoked responses(ABR). The ABR test' an auditory evoked electrophvsiologic responsethat correlates highly with hearing, has
been used Juccessfully and cost-effectively to screen newborns

2624I PARTXXIX r TheEar


garten that further hearing screening takes place. Primary care
physicians and pediatricians should be alert to the signs and
symptoms of childhood hearing impairment, so that children with
hearing impairment who have not been screenedformally can be
identified as early as possible.
ldentificationol Hearing lmpairment.The impact of hearing
impairment is greatest on an infant who has yet to develop language; therefore, identification, diagnosis, description, and treatment should begin as soon as possible.In general,infants with a
prenatal or perinatal history that puts them at risk (see Table
636-1,1 or those who have failed a formal hearing screening
should be monitored closely by an experienced clinical audiologist until a reliable assessmentof auditory function has been
obtained. Pediatriciansshould encouragefamilies ro cooDerare
with the follow-up plan. lnfants who are born at risk bui who
were not screenedas neonates(often becauseof transfer from one
hospital to anorher)should have a hearingscreeningby age 3 mo.
Hearing-impaired infants who are born at risk or are screened
for hearing loss in a neonatal hearrng screenlngprogram account
for only a porrion of hearing-impaired chitdien. Children who
are congenitallydeaf becauseof autosomal recessiveinheritance
or subclinical congeniral infection often are not identified until
1-3 yr of age. Usually, those with more severe hearing loss are
identified at an earlier age, but identification often ociurs larer
than the age at which intervention can provide an oprimal
o u r c o m e .C h i l d r e nw i r h n o r m a l h e a r i n gd e v e l o pa n e x r e n s i v e
langu_ageby 34yr of age (Table 636-6) and ixhibit behaviors
reflecting normal audirory function (Table G36-7). Failure to
fulfill these criteria should be reason for an audiolosic evaluation. Parental concern about hearing and any delayed development of speech and language should alert the pediatrician,
becauseparenral concern usually precedesformal identification
and diagnosisof hearing impairment by 6 mo to 1.yr of age.
CLINICAL
AUDI0L0GIC
EVALUATI0N.
Even the younsesrinfants can
be evaluatedfor audirory funcrion. tJ/hen hearing impairmenr is
suspectedin a young child, reliable and valid estimaresof auditory function can be obtained. Successful treatment strategies
for hearing-impairedchildren rely on prompr identification ind
ongoing assessmentto define rhe dimensionsof auditory function. Cooperation among the pediatricianand specialistsin areas
sych ag audiology,speechand languagepathology,educarion,and
child development is necessaryro oprimize audiiory-verbal development. Therapy for hearing-impairedchildren includes considering and often fitting an amplification device, monitoring
hearing and auditory skills, counseling parents and families,
advising teachers,and dealing wirh public agencies.
Audiometry.The technique of rhe audiologic evaluation varies
as a function of rhe age or developmental level of the child, the
reason for the evaluation, and the child's otolosic condition or
h i s t o r y .A n a u d i o g r a mp r o v i d e sr h e f u n d a m e n r a d
l e s c r i p t i o no f
hearing sensitivity(Fig.536-2). Hearing thresholdsare assessed
as a function of frequencyusing pure rones(sinewaves)at octave
intervals from 250-8,000 Hz. Earphonestypically are used, and

AGT
DIVETOPMENT
IMO) NORMA|'
0-4
Should
startle
toloud
sounds,quiet
tomother!
voi(e,
momentarily
cease
activity
when
sound
ispresented
ataconversational
level
5-6
presented
Should
conectly
plane,
localize
tosound
inahorizontal
begin
tolmrtate
sounds
in
repert0ire
ownspee(h
0ratleast
recjprocally
vocalize
withanadult
7-12
presented
5hould
conectly
localize
tosound
inanyplane
quietly
5hould
respond
toname,
even
when
spoken
1l-15
point
Should
toward
anunexpected
sound
ortofamihar
objects
orpersons
when
asked
16-18
Should
follow
gestural
simple
directions
without
visual
orother
cues;(an
betrained
t0rea(h
toward
aninteresting
toyatmidline
when
asound
ispresented
point
19-)4
parts
Should
play
t0body
when
asked;
by21-24mo,
canbetrained
toperform
audiometry
Frcm
Matkin
ND:Eady
ncognttion
andreferral
0fhearing-impalred
children
PediltRevi984;6:i5jReprodured
bypermis\ionofPediottits

hearing is assessedindependently for each ear. Airconducted


signals are presentedthrough earphones(or loudspeakers)and
are used to provide information about the sensitivity of the auditory system.These same rest sounds can be deliveredto the ear
through an osciliator thar is placed on the head, usually on the
mastoid. Such signalsare consideredbone-conductedbecausethe
bones of the skull transmit vibrations as sound energy directly to
the inner ear, essentially bypassing the outer and middle ears. In
a normal ear, and also in children with SNHL, the air and bone
conduction thresholdsare the same. In those with CHL. the air
and bone conduction thresholds differ. This is called the air-bone
gap, which indicatesthe amounr of hearing loss attributable to

PURE-TONE
AUDIOGRAM
Frequency(cycles/sec)
125

250

500

CI

1000

2000

4000

8000

10
0
10
20
JU

40
50
60

\j

70
80
90
100

AGE
{M0)
12
18
24
l0
36
48

REFTRRAT
GUIDETINTs
F()R(HITDREN
WITH'sPEEfi"DETAY
Nodifferentiated
babbling
0rvocal
imitation
Nouseofsingle
words
Single-word
vocabulary
of<10words
<100words;
noevidence
of2-word
combinatiofls;
unintellioible
<200words;
nouseoftelegraphk
sentenceJ;
clarity
<5t)%
<600words;
nouseofsimple
se$tences
dariry<80%

FromlvatkinND:[arlyrecognitionandrfe(al0fhearing-impairedchildren
pediltRev19g4;6151
Reprodwedbypermrssionol Pediottks

110
AUDIOGRAMKEY
Air

Bone

Right
Left

Figure 636-2. Audiogram showing bilateral conductivehearing loss.

636 r Hearingloss r 2625


Ghapter
'lfith
mixed hearing
dysfunction in the outer and/or middle ear.
loss, both the bone and air conduction thresholds are abnormal,
and there is an air-bone gap.
Speech BecognitionThreshold. Another measure useful for
describing auditory function is the speechrecognition threshold
(SRT), which is the lowest intensity level at which a score of
approximately 50% correct is obtained on a task of recognizing
spondee words. Spondee words are 2-syllable words or phrases
that have equal stresson each syllable,such as baseball,hotdog,
and pancake. Listeners must be familiar with all the words for a
valid test result to be obtained. The SRT should correspond to
the averageof pure-tonethresholdsat 500, 1,000, and 2,000 Hz,
the pure-tone average (PTA). The SRT is relevant as an indicator
of a child's potential for development and use of speechand language; it also serves as a check of the validity of a test because
children with nonorganic hearing loss (malingerers)may show a
discrepancy between the PTA and SRT.
The basic battery of hearing tests concludeswith an assessment
of a child's ability to understand monosyllabic words when presentedat a comfortable listening level. Performance on such word
intelligibility tests assistsin the differential diagnosis of hearing
impairment and provides a measure of how well a child performs
when speech is presented at loudness levels similar to those
encountered in the environment.
For children
PlayAudiometty.Hearing testingis age-dependent.
at or above the developmental level of a 5-6 yr old, conventional
test methods can be used.For children 30 mo to 5 yr of age,play
audiometry can be used. Responsesin play audiometry usually
are conditioned motor activities associatedwith a game, such as
dropping blocks in a bucket, placing rings on a peg, or completing a puzzle.The techniquecan be usedto obtain a reliableaudiogram for a preschool child. For those who will not or cannot
repeat words clearly for the SRT and word intelligibility tasks,
pictures can be used with a pointing response.
Audiometry.For children betweenthe ages
VisualBeinforcemenl
of about 6-30 mo, visual reinforcementaudiometry (VRA) commonly is used.In this technique,the child is observedfor a headturning response on activation of an animated (mechanical) toy
reinforcer. If infants are properly conditioned, by giving sounds
associatedwith the visual toy cue, VRA can provide reliableestimates of hearing sensitivity for tones and speechsounds. In most
applicationsof VRA, sounds are presentedby loudspeakersin a
sound field, so no ear-specificinformation is obtained. Assessment of an infant often is designed to rule out hearing loss that
would affect the developmentof speechand language.Normal
sound field response levels of infants indicate sufficient hearing
for this purpose despite the possibility of different hearing levels
in the 2 ears.
Audiometty,Used as a screeningdevice
BehavioralObservation
for infants <5 mo of age, behavioral observation audiometry
(BOA) is limited to unconditioned, reflexive responsesto complex
(not frequency-specific) test sounds such as noise, speech, or
music presentedusing calibrated signalsfrom a loudspeakeror
uncalibratednoisemakers.Responselevelscan vary widely within
and among infants and usually do not provide a reliableestimate
of sensitivity.
Assessmentof a child with suspectedhearing loss is not complete until pure-tone hearing thresholds and SRTs (a reliable
audiogram) have been obtained in each ear. BOA and VRA in
sound-field testing give estimates of hearing responsivity in the
better hearing ear.
Acoustic lmmittanceTesting.Acoustic immittance testing is a
standard part of the clinical audiologic test battery and includes
tympanometry. It is a useful objective assessmenttechnique that
provides information about the status of the middle ear. Tympanometry can be performed in a physician's office and is helpful
in the diagnosis and management of OM with effusion, a
common cause of mild to moderate hearing loss in young
children.

5WTTP
OTAIRPRTS'URI
sFTED
<50daPa/sec*
200daPa/seC
(hildren
(l-5 yr)

Lower
limit
Median
limit
Upper

Adults

limit
Lower
Median

Upper
limit

030
055
090
056
0,85
136

036
061
106
027
072
138

*Earcanal
tail0llympanoglam
atLowett
based
0nadminance
volume
measutement

tEar
fu adultsi
for(hikkoandat+200daPa
tail0ftympanqram
atlowe$
0nadmifian(e
canal
measurementbased
daPa,detaPastah
(edimr]:fleul|r4
W5
prindples
InRintelnan
applirrmn
0fclini(al
J[Tympanometry:8asi(
RH,5hanl6
fiomMargolis
Adapted
l991,pp
179-245
/$erirnenl2nd
edAuslin,TX,PR0D[D,

Tympanometry provides a graph of the middle


nsmit sound energy (admittance' or compliance)

*Hfl"::i:,:ii::T::
;l'#i"!'T;1il:'n:?

ments measure acoustic admittance, the term admittance is used


tn. principles apply to whatever units of measurement are
f;;::..

is used to estimate the acoustic admittance of the ear canal and


middle-ear system. Admittance can be expressedin a unit called
a millimho (mmho) or as a volume of air (mL) with equivalent
acoustic admittance. The cest is performed so that an estimate
can be made of the volume of air enclosed between the probe tip
and TM. The acoustic admittance of this volume of air is
deducted from the overall admittance measure to obtain a
measure of the admittance of the middle-ear system alone. Estimating ear canal volume also has a diagnostic benefit, becausean
abnorLally large value is consistent with the presence of an
in the TM (perforation or tube).
opening
^
OncJ the admittance of the air mass in the external auditory
canal has been eliminated, it is assumedthat the remaining admittance measufe accurately reflects the admittance of the entire
middle-ear system. Its value is controlled largely by the dynam-,
ics of the TM. Abnormalities of the TM can dictate the shape of

though it is estimated from a dynamic measure (seeFig. 639-4P').


Children with
lN 0TlTlSMEDIAWITH EFFUSI0N.
TYMPAN0METRY
OM with effusion often have reduced peak admittance or high

2626r PABTXXIX I The Ear

instruments are designedto present reflex activating srgnals(pure


tones of various frequencies or noise), either to thi same oi the
contralateral ear, while monitoring admittance. Very small admittance changes rhar are time-locked ro presentations of the signal
are consideredto be a result of middle-ear muscle reflexes.Ad;ittance changes may be absent when rhe hearing loss is sufficient
to prevent the_signal from reaching the loudness level necessary
to elicit the reflex or when a middle-ear condition affects the ear;s
ability to monitor a small admittance change. Reflexes usually
are.absentin patients with CHL due to the presenceof an abnormal transfer sysrem; thus, the ART is useiul in the differential
diagnosis of hearing impairment. The ART also is used in the
assessmentof SNHL and the integrity of the neurologic components of the reflex arc, including cranial nerves VILnd VItf.AuditoryBrainstemBesponse.The ABR tesr is used ro screen
newborn hearing, confirm hearing loss in young children, obtain
ear-specificinformation in young children, and test children who

the low-frequency region of the cochlea in all cases, and this


may affect interpretation.
The ABR test does not assess"hearing." It reflects auditory
neuronal electric responsesthat can be correlated to behavioral
hearing thresholds, but a normal ABR result only suggeststhat
the auditory system, up to rhe level of the midbrain, is responsive to the stimulus used. Conversely, a failure to elicit an ABR
indicates an impairment of the system'ssynchronous responsebut
does not necessarily mean that there is no "hearing." The behavioral response to sound sometimes is normal when no ABR
can be elicited, such as in neurologic demyelinating disease.The
ABR test may be used to infer whether and at what level of the
auditory system impairment exists.
Hearing lossesthat are sudden, progressive, or unilateral are
indications for ABR testing. Although it is believed that the different waves of the ABR reflect activity in increasingly rostral
levels of the auditory system, the neural generators of the
response have not been precisely determined. Each ABR wave

in respect to age-appropriate forms. Delayed or missing waves in


the ABR result often have diagnostic significance.

that.unreliable or erroneous conclusions will affect a parient,s

response.

The ABR rest has two major uses in a pediatric setting. As an


audiometric rest, it provides information on rhe ability of the
peripheral auditory system to transmir information to t'he audi-

on infants or parients who are difficulr to test. ABR thresholds


using click stimuli correlate best with behavioral hearing thresholds.in the higher frequencies(1,000-4,000 Hz); responsivityin
the low frequencies requires different stimuli (toni bursts or
filtered clicks) or rhe use of masking, neither of which isolares

(TEOAEs) may be used to check the integrity of the cochlea. In


the neonatal period, detection of OAEs can be accomplished
during natural sleep, and TEOAEs can be used as screeningrests
in infants and children for hearing at the 30 dB level of hiaring
loss. They are less time-consuming and elaborate than ABR and

and may incorrectly indicate a cochlear hearing disorder. If a


hearing loss is suspectedbased on the absenceof OAE, the ears
should be examined for evidence of pathology, and then ABR
testing should be used for confirmation and identification of the
type, degree, and laterality of hearing loss.
Acoustic Reflectometry.In acoustic reflectometry, a hand-held
instrument is placed next to the opening of a child's ear canal
and 80-dB sound is delivered rhar varies in frequency from
2,000-4,500 Hz in a 100-msec period. The instruminr -L"rur.,
the total level of reflected and transmitted sound. Some ohvsicians have found this device useful to help gauge the pr.r.n.. o.
absenceof middle-ear fluid, and a commercial version is marketed
to parents as a way to monitor ear fluid. The instrument does not
provide any information about hearing; if the presenceof chronic
fluid is suggested,audiometric evaluation should be obtained.
T
a

lfith the use of universal hearing screeningin many


United States, the early diagnosis and treatment of

636 E Hearingls55 r 2627


Chapter

(MO)PCVT
AGE
ATFIRST
DOSE
2-6
1-11
12-)3
24,59
>60

PCVT
STRIIS
PRIMARY
moapart'
3doses,2
1 r^.^- 1 -^.-.,rI
I U U ) r ) , 1 i l U d p d Tl

I 00se5,
I m0apart
I 00ses,
I m0apan
Notrndi(atedll

D05t
P(v7AD0tTr0r'lAt
1dose
a 12-15moofage'
1d o saet1 2 - 1 5m oo f a g e t
Notindiiated
Notindl(ated
Notindi(ated

a >24moofage!
Indicated
at>24moofageq
hdicated
at>24 moofageq
Indicated
lndicaeds
lndiGted

conjugale
varrine
shofiage
resolved
/i4MWR
2043,52.M-447
Pneumococral
)
'For
chrldren
vaccinated
atage<1 yt minimum
interval
between
doses
is4 wks
+The
(omDleled
afterthe
series
hasbeen
additional
dose
should
beadministered
28 weeks
0fimarv
(NoRfl-9)
MMI&R
2000;49
infants
0ftheAdvisory
[0mmittee
0nlmmunization
Pratti(s
andyoung
children:
Recommendations
tMinimum
intervdl
between
doses
rs8 weeks
:PtV/rsnotrecommended
generally
forchildren
aged
25 yrs
FromPneumocotcalvaccinati0nf0fc0ch|ealimplant(andidate5andrcipien6'Updatedrec0mmendati0n50ftheAdv0[y[0mm|fee0

children with hearing loss is common. Testingfor hearing loss is


possible even in ver,v young children, and should be done if
parentsare suspiciousof a problem. Any child with a known risk
factor for hearing lossshould be evaluatedin the 1st 6 mo of Life.
Once a hearing loss is identified, a full developmental and
speechand languageevaluation is needed.Parental counseling
and involvernentare required in all stagesof the evaluation and
treatment or rehabilrtation.A conductive hearing loss often can
be correctedthrough treatment of a middle-eareffusion (i.e.,ear
tube placen'rent)or surgical correction of the abnormal soundcondu*ing mechanism.Children with SNHI, should be evaluated
for possiblehearing aid use bv a pediatric audiologist. Hearing
aids mav be firted for children as young as 2 mo of age. Compelling evidencefrom rhe hearingscreeningprogram in Colorado
shou's that identification and amplification before age 6 mo
makes a verv significanrdifferencein the speechand language
abiliries of affected children. comoared with those casesidentified and amplified after the age of 6 mo. In thesechrldren,repeat
audiologic testing is needed to reliablv identify the degree iif
hearing loss and to fine-tunethe use of hearing aids.
Infants and your.rgchildren with profound congenitalor prelrnsual onset of deafnesshave benefitedfrom multichannelcochlear
implants (Fig. 636-3). These implants bypassinjury to the organ
of Cortr and provide neural stimulation by way of an external
microphone and a signal processorthat digitizesauditory stimuli
into digital radiofrequencv impulses. Cochlear implantation
before age 2 vr (and even 1 yr) improves hearing and speech,
e n a b l i n eo v e r 9 0 ' % o f c h i l d r e n t o b e i n m a i n s t r e a me d u c a t i o n .
Most develop age-appropriateauditory perceptionand oral lang u a g es k i l l s ,
A seriouscomplication of cochlear implants is an excessively
high incidenceof pneumococcalmeningitis.All children receiv
rng :r cochlearimplant must be vaccinatedwith the PCV-7 vaccine
( T a b l e6 3 6 - 9 ) .
The best approach to the educationof children u'ith signifrcant
hearing loss is a subjectof ongoing controversy.Becausewe live
in a predorninantl,vspeakingworld, some have advocateda pure
auditorv and oral approachto hearingtherapy.However, because
affectedchrldrenoften are slow to developcommunication skills,
many advocatea total communication approach; dependingon
the individual child's needs,this techniqueusesa mixture of sign
language,lip-reading,hearing aids, and speech.The appropriate
program for each child dependson rhe patjent, familr',and available resources.
G e n e t i cC o u n s e l i n gF.a m i l i e so f c h i l d r e nw i t h t h e d i a g n o s i so f
SNHI-, or a svndrome associatedwith SNHL and/or CHL,
should consider genetic counseling,which will allow for a drscussionof the likelihood of similar diagnosesin future pregnancies. The geneticrstalso mav help in the evaluation and further
testing of the patient with hearing loss to establisha diagnosis.

i , ,,r. :' 'r' ; All cochlear implants share ke,v components! rncluding a
rnrcrophonc, specch proccssor, and transmitter coil' shown ln a behind-the-ear
posrtion in thrs diagram. The mlcrophone/speech processor picks up
environmental sounds and cligitises them into coded signals. The signals are
sent to thc transmitter coil and rela,ved through the skin to the internal device
imbeclclcd in the skull The internal devtce converts the code to electronic
signals, n'hrch are transmitted to the electrode arra,v wrapping arouncl the
-I'he
inset shows the radiographic appearance of the stimulating eleccochlea
trode array. Reprocluced with permission from MED-EL Corporation, Innsb r u c k , A u s t r t a . ( F r o m S m r t h R J H , B a l e J F ' J r , ! i l h r t e K R : S e n s o r i n e u r a lh e a r i n g
loss rn children. I'ancet 2005 365:879-890.)

Berskv-FirkserL, Sun S: Llntversalnewborn hearing screenings:A three-year


cxperience.Pediatrics1997;99: l
B e n - Y o u s eTf , N e s s S L , M a d e o A C , e t a l : A m u t a t i o n o f P C D H I 5 a m o n g
AshkenaziJews with the type 1 Usher s,vndrome N Engl I Med 2003;
348:1664-1'670
BiernathKR, ReefhuisJ, Vhitney CG, et al: Bacterialmeningitrsamong chrl
dren with cochlear implants beyond 24 months after implantation' Pedr
dtrics 2006:LL7:284-289

2623rPARIXXXrTheEar
Capaccio P, Ottaviani F, Cuccarini V, et al: Sudden hearing loss and
MTHFR 677C>T/'1,298A>Cgene polymorphisms. Genet Med 2005;7:206208.
Centers for Disease Control and Prevention: pneumococcal vaccination for
cochlear implant candidates and recipients: Updated recommendations of
the Advisory Committee on Immunization practices. MMWR 2003t
52:739-740.
Cox LC: Otoacoustic emissions as a screening tool for sensorineural hearing
loss. J Pediatr 1,997;130:685-686.
Del Castillo I, Villamar M, Moreno-Pelayo MA, et al: A deletion involving the
connexin 30 gene in nonsyndromic hearing impairments. N Engl J Med
2002t345: 243-249.
Eilers RE, Oller DK: Infant vocalizations and early diagnosis of severehearing
impairment. J Pediatr 1994;124:799-203.
Ftgazzola L, Cerutti N, Mannavola D, er al: Differential diagnoses berween
Pendred and pseudo-Pendredsyndromes: Clinical, radiologic, and molecular studies. Pediatr Res 2002:51,:479484.
Gates GA, Miyamoto RT: Cochlear implants. N Engt ! Med 2003;349:
421,423.
Grote JJ: Neonatal screening for hearing impairment. Lancet 2000;355:
5 13-5 14.
Hinson Jl Fantin VR, Schcinberger
J, et al: Missensemurations in the BCSlL
gene as a cause of Bjiirnstad syndrome. N Engl I Med 2007;356:809-81,9.
Kemper AR, Downs SM: A cost-effectivenessanalysis of newborn hearing
screeningstrategies.Arch Pediatr Adolesc Med 2000;154:484.
Kennedy C, McCann D, Campbell MJ, et al: Universal newborn screeningfor
permanent childhood hearing impairmenr: An 8 year follow up of a controlled trial. Lancet 20051366:660-662.
Mason JA, Herrmann KR: Universal infant hearing screening by automated
auditory brainstem response measurement. pediatrics 1998:1,01:221228.
Moeller MP: Early intervention and language development in children who
are deaf and hard of hearing. Pediatrics 2000;106:E43.
Morell RJ, Kim HJ, Hood LJ, et al: Mutations in the connexin 26 gene(GJB2I
among Ashkenazi Jews with nonsyndromic recessivedeafness.N Engt Med
J
19 9 8 ; 3 3 9 : 1
5 0 0 - 15 0 5 .
Monon CC, Nance WE: Newborn hearing screening-a silent revolution.
N Engl J Med 2006;354:2151-2164.
Nikolopoulos TP, Archbold SM, O'Donoghue GM: Does cause of deafness
influence outcome after cochlear implantation in children? pediatrics
2 0 0 6 ; 11 8 : 1 3 5 0 - 1 3 5 5 .
Nikolopoulos TP, Dyar D, Archbold S, et al: Development of spoken language
grammar following cochlear implantation in perlingually deaf children.
Arch Otolaryngol Head Neck Surg 2004;1.30:629-633.
Niskar AS, Kieszak SM, Holmes A, et al: prevalence of hearing loss among
children 5 to 19 yearsof age.JAMA 7998;279:1.077-1075.
Pirozzo S, Papinczak I Glasziou P: Vhispered voice test for screening for
hearing impairmenr in adults and children: systematic rcview. BMJ i003;
327:967-970.
Ramsden RT: Prognosis after cochlear implantation. BMJ 2004;32g:
419420.
Reefhuis J, Honein MA, Whitney CG, et al: Risk of bacterial meningitis
in children with cochlear implants. N Engl J Med 2003;349:435445.
Schultz JM, Yang I Caride AJ, et al: Modification of human hearing loss by
plasma-membrane calcium pump PMCA2. N Engl
1 Ued ZOOS;SSi:
1 55 7 - 1 . 5 5 4 .
Smith RJH: Deafness: from bedside to bench and back. Lancet
20021360:656-657.
Smith RJH, Bale JF Jr, White KR: Sensorineural hearing loss in children.
Lancet 2005; 365:879-890.
Thomas MA, Der Kaloustian VM, Tewfik TL: Connexin mutation testing of
children wirh nonsyndromic, autosomal recessivesensorineuralhearing llss.
J O to laryngo I 2004 ;33 :189-792.
Thompson DC, McPhillips H, Davis RL, et al: Universal newborn hearing
screening:Summary of evidence.JAMA 200I;296:2000-2010.
Waltzman SB, Roland T Jr: Cochlear implantation in children younger than
12 months. Pediatrics 2005J,6:4g7493.
Willems PJ: Genetic causesof hearing loss. N Engt J Med 2000;342:7101.
Wilson C, Roberts A, StephensD: Aetiological investigation of sensorineural
hearing loss in children. Arch Dis Chitd 205:90:307-309.
Wolf B, SpencerR, Gleason T: Hearing loss is a common feature of symptomatrc children with
profound
biotinidase deficiency. pediairics
2002;'J,40:242-246.
Yoshinaga-Itano C, SedeyAL, Coulter DK, et al: Language of early- and lateridentified children with hearing loss. pediatrics 1.999;1,02:1151-7J,7
l.

The external and middle ears, derived from the 1st and 2nd
branchial arches and grooves, grow throughout puberty, but the
inner ear, which develops from the otocyst, reaches adult size and
shape by mid-fetal development. The ossicles are derived from
the 1st and 2nd arches (malleus and incus), and the stapesarises
from the 2nd arch and the otic capsule. The malleus ind incus
achieve adult size and shape by the 15th wk ofgestation, and the
stapes achieves adult size and shape by the 18th wk of gestation.
Although the pinna, ear canal, and tympanic membrane (TM)
continue to grow after birth, congenital abnormalities of these
structures develop during the 1st half of gestation. Malformed
external and middle ears may be associated with serious renal
anomalies, mandibulofacial dysostosis, hemifacial microsomia,
and other craniofacial malformations. Facial nerve abnormalities
may be associared with any of the congenital abnormalities of the
ear and temporal bone. Malformations of the external and middle
ears also may be associatedwith abnormalities of the inner ear
and both conductive (CHL) and sensorineural hearing loss
(SNHL).

ties a team approach with other specialists can assist in guiding


therapy.
PINNA MAIFOBMATIOI{S. Severe malformations of the external
ear are rare, but minor deformities are common. Isolated abnormalities of the external ear occur in approximately l%o of children. A pitJike depression just in front of the helix and above
the tragus may represent a cyst or an epidermislined fistulous
tract. These are common, with an incidence of approximately
8/1,000 children, and may be unilateral or bilateral and familial.
The pits require surgical removal only if there is recurrent infection. Accessory skin tags, with an incidence of t-211,,000, canbe
removed for cosmetic reasons by simple ligation if they are
attached by a narrow pedicle. If the pedicle is broad-basedor con-

children >5 yr of age, when the pinna has reached about 80% of
its adult size.

inferior in placement than normal auricles, and the location and


function of the facial nerve may be abnormal. Surgery ro correct
microtia is considered for both cosmetic and functional reasons;
children who have some pinna can wear regular glasses,a hearing
aid, and earrings and fiel more normal-in aplearance. If thi
microtia is severe, some patients may opt for creation and attachment of a prosthetic ear, which cosmetically closely resemblesa
real ear. Surgery to correct severe microtia may involve a multistage procedure including carving and transplantation of auto-

Chaptel{i:18r External0titis (otitis Externalr 2629


genous cartilage rib grafts, and local soft tissue flaps. Cosmetic
reconstruction of the auricle usually is performed between 5-7 yr
of age, and is performed before canal atresia repair in children
deemed appropriate for this surgery.
AUDITORY
CONGENITAT
STENOSISOR ATRESIAOFTHE EXTERNAT
CANAI. Stenosisor atresia of the ear canal often occurs in association with malformation of the auricle and middle ear; minor
stenosesmay occur in isolation. In some genetic syndromes, e.g.,
trisomy 21, ear canals are narrow. Audiometric evaluation of
these children should be undertaken as early in life as possible.
Most children with significant CHL secondary to bilateral atresia
wear bone conduction hearing aids for the first severalyr of life.
Diagnosis, evaluation, and surgical planning often are aided by
Cl and sometimes MRI, of the temporal bone. Mild casesof ear
canal stenosis do not require surgical enlargement unless the
patient develops chronic external otitis or severe cerumen
impaction that affects hearing.
Reconstructive ear canal and middle-ear surgery for atresia
usually is considered for children >5 yr of age who have bilateral
deformities resulting in a significant CHL. The aim of reconstructive surgery is to improve hearing to a point where the child
may not need a hearing aid or to provide an ear canal and pinna
so that the child can derive improved benefit from an airconduction hearing aid. CT evidence of an adequate middle-ear
cleft, ossicles,and mastoid is required to perform the surgery; the
position of the facial nerve, which often is in an abnormal location in these children, also must be considered. The use of boneanchored hearing aids (BAHA) may provide an option for some
of these children when they are older.

C0NGENITAI CH0LESTEAT0MA.A congenital cholesteatoma


usually appears as a white, round, cystlike structure medial to
an intact TM. Cysts are seen most commonly in the anteriorsuperior portion of the middle ear, although they can present in
other locations and within the TM or in the skin of the ear canal'
Affected children often have no prior history of otitis media

the dura, with consequent meningitis or a brain abscess.


Cholesteatoma should be removed surgically after CT scan and
hearing evaluation, and appropriate antibiotic therapy. Congenital ch-olesteatoma is an aggressive disease, and early surgical
removal and close monitoring will help prevent Permanent
damage to the middle and inner ear.

C0NGENITAIMIDDIE-EABMAIF0RMATI0NS. Children may have


congenital abnormalities of the middle ear as an isolated defect
or in association with other abnormalities of the temporal bone,
especially the ear canal and pinna, or as part of a syndrome,
Affected children usually have CHL but may have mixed CHL
and SNHL. Most malformations involve the ossicles,with the
incus most commonly affected. Other less common abnormalities of the middle ear include persistent stapedial arterg highriding jugular bulb, and abnormalities of the shape and volume
of the aerated portion of the middle ear and mastoid; all present
problems for a surgeon. Depending on the type of abnormality
and the presenceof other anomalies, surgery may be considered
to improve hearing.
C0NGENITALINNER EAR MA[F0RMAT|0NS. Congenital inner ear
malformations have been identified and classified as a result of
improvements in imaging modalities, especiallyCT and MRI. As
many as 20% of children with SNHL may have anatomic abnormalities identified on CT or MRI. Congenital malformations of
the inner ear usually are associated with SNHL of various
degrees,from mild to profound. These malformations may occur
as isolated anomalies or in association with other syndromes,
genetic abnormalities, or structural abnormalities of the head and
neck. Enlarged vestibular aqueducts have been identified on
imaging studies in association with SNHL; although no theraPy
exists for this condition, it may be associated with progressive
SNHL in some children, and, therefore, diagnosis may have some
prognostic value.
Congenital perilymphatic fistula (PLF) of the oval or round
window membrane may present as a rapid-onset, fluctuating, or
progressive SNHL with or without vertigo and often is associated with congenital inner ear abnormalities. Middle ear exploration may be required to confirm this diagnosis, because no
reliable nonoperative diagnostic test exists. It may be necessary
to repair a PLF to prevent possible spread of infection from the
middle ear to the labyrinth, to stabilize hearing loss, and to
improve vertigo when present.

a protective, waxy, water-repellent coating.


The normal flora of the external canal consists mainly of

nous bacteria.
ET|OI0GY. External otitis (swimmer's ear, although it can occur
without swimming) is caused most commonly by P. aerugin-osa,
but S. aureus, Enterobacter lerogenes' Proteus mirabilis,
Klebsiella pneumoniae, streptococci' coagulase-negative staphylococci,-diphtheroids' and fungi such as Candida and
Aspergitlui alsb may be isolated. External otitis results from

2630r PABTXXIX r thg p3t


chronic irritation and maceration from excessivemoisture in the
canal. The loss of protective cerumen may play a role, but
cerumen impaction with trapping of water also can cause infection. Inflammation of the ear canal due to herpesvirus, varicellazoster,other skin exanthems, and eczema also may predisposeto
external otitis.

mation of the canal or resolving acute otitis exrerna. Conductive


hearing loss may result from edema of the skin and tympanic
membrane (TM), serous or purulent secretions,or the canal skin
thickening associatedwith chronic external otitis.
Edema of the ear canal, erythema, and thick, clumpy otorrhea
are prominent signs of the acute disease.The cerumen usually is
white and soft in consistency,as opposed to its usual yellow color
and firmer consistency.The canal often is so tender and swollen

pressure.

Surgical intervenrion to obtain cultures or debride devitalized


tissue may be necessary. P. aeruginosa is the most common
c_ausative
organism of necrotizing otitis externa. Fortunateln this
disease is rare in children and is seen only in association with
immunocompromise or severe malnourishment. In adults it is
associatedwith diabetesmellitus.
DIAGN0
culosis,

. Diffuse exrernal otitis may be confused with furuntis media (OM), and mastoiditis. Furuncles occur in

polymyxin (active against gram-negative bacilli, notably


Pseudomonas spp.) and corricosteroids are highly effective in
treating most forms of acute external otitis. Newer preparations
of eardrops (e.g., ofloxacin, ciprofloxacin) are available that do
not contain potenrially ototoxic antibiotics. If canal edema is
marked, the patient may need referral to a specialist for cleaning
and possible wick placemenr. Some recommend otic corticosteroids in addition to otic antibodies. A wick can be inserted into
the ear canal and topical antibiotics applied to the wick 3 times

externa. An approach to management is noted in Figure 538-1.


As the inflammatory process subsides,cleaning the canal with
a suction or cotton-tipped applicator to remove the debris
enhancesthe effectivenessof the ropical medications. In subacute
and chronic infections, periodic cliansing of the canal is essential. In severe,acute external otitis associatedwith fever and lymphadenitis, oral or parenteral antibiotics may be indicated; arrear
canal culture should be done, and empiric antibioric rrearment
can then be modified if necessary,based on susceptibilify of rhe
organism cultured. A fungal infection of the external audirory
canal, or otomycosis, is characterized by fluffy white debris,
sometimes with black spores seen; rreatment includes cleaning
and application of antifungal solutions such as clotrimazole oi
nystatin; other antifungal agents include m-cresyl acetate 25"/o,
gentian vrolet 2To, and thimerosal 1 : 1,000.

The most effective prophylaxis is instillation of dilute alcohol or


acetic acid (27') immedrately after swimming or bathing. During
an acute episode of otitis externa, patients should not swim
and the ears should be protected from excessivewater during
bathing.

OTHER
DISEASES
OFTHEEXTERNAT
EAR
FURUNCUL0SIS.
Furunculosis is caused by S. aureus and affects
only the hair-containing outer third of the ear canal. Mild forms
are treated with oral antibiotics active against S. aureus. If an
abscessdevelops, incision and drainage may be necessary.
ACUTECEttULlTlS, Acute cellulitis of the auricle and external
auditory canal usually is caused by group A streptococcus ano
occasionally by S. aureus. The skin is red, hot, and indurated,
without a sharply defined border. Fever may be present with
Iittle or no exudate in the canal. Parenteral administration of
penicillin G or a penicillinase-resistantpenicillin is the therapy of
cnorce.

Topical otic preparations containing neomycin


st gram-positive organisms and some gram-negarive
notably Proteus spp.) with either colistin or

PEBICH0NDRITIS
AND CH0NDRITIS.
Perichondritisis an infection
involving the skin and perichondrium of the auricular cartilage;
extension of infection to the cartilage is termed chondritis. The
ear canal, especiallythe lateral aspect,also may be involved. Early
perichondritis may be difficult to differentiate from cellulitii
because both are characterized by skin that is red, edematous,
and tender. The main cause of perichondritis/chondritis and cellulitis is trauma (accidental or iarrogenic, laceratron or contusion), including ear piercing, especially when done through the
cartilage. The most commonly isolated organism in perichondritis and chondritis is P. aeruginosa, although other gram-negauve
and, occasionally,gram-positive organisms may be found. Treat-

Ghapter638 r Externalotitis (otitis ExternalI 26i11

I Patientage 2 W or older
with diffuseAOE
I

Prescribe
basedon painseverity
analgesics

Prescribesystemicantimicrobialactive
againsl Pseudomonas aeruginosa and
Staphylococcusaureus,with or without
topicaltherapy,plus other management
basedon underlyingcondition

Extension
outside
earcanalor host
factors*requiring
therapy?
systemic

|ruo
I

.Factorsrequiringsystemictherapyinclude
diabetes,immunedeficiency,or inabilityto
effectivelydelivertopicaltherapydespiteauraltoilet

Perforatedtympanic
membrane(knownor
suspected)or
tympanostomytube?

PrescribetopicaltheraPYwith a
non-ototoxicpreparation

Prescribetopicaltherapybasedon
benefits,cost,compliance,preference

obstructedearcanal

Yes

auraltoiletto remove
Perform
> obstructing
debris;Placewickif
drugdelivery
edemaprevents

Educatepatientor caregiveron how


to administertopicaldrops

I v""

Assessdrugdelivery
to therapy,
adherence
needto changetheraPy

Figure 63g-1. Flowchart for managing acute otitis externa.(From RosenfeldRM, Brown L, Cannon CR, et al: Clinical practiceguideline:acute otitis externa.
Ololaryngol Head Neck Surg 2006;154t54-S23. O 2005 American Academy of Otolaryngology-Head and Neck Surgery Foundation, Inc )

ment involves systemic, often parenteral, antibiotics; surgery to


drain an abscessor remove nonviable skin or cartilage may also
be needed. Removal of all ear iewelrv is mandatorv in the presence of infection.
Various dermatoses(seborrheic,contact, infectious
DERMAT0SES.
eczematoid, or neurodermatoid) are common causes of inflammation of the external canal; scratching and the introduction of
infecting organisms causeacute external otitis in theseconditions.
Seborrheic dermatitis is characterized by greasy scales that
flake and crumble as they are detached from the epidermis; associated changes in the scalp, forehead, cheeks, brow, postauricular areas, and concha are usual.

Contact dermatitis of the auricle or canal may be caused


by earrings, or by topical otic medications such as
neomycin, which may produce erythema' vesiculation, edema,
and weeping. Poison ivn oak, and sumac also may- prod.ce
contact dermatitis. Hair care products have been implicated in
sensitive individuals.
Infectious eczematoid dermatitis is caused by a purulent infection of the external canal, middle ear' or mastoid; the purulent

2632r PABTXXIX r TheEar


Neurodermatitis is recognized,by intense itching and erythematous, thickened epidermis localized to the concha and orifice
of the meatus.

GENERAI
CONSIDEBATIONS
matitis is suspected.

are more common in males; exostoses are more common


than osteomas. Surgical treatment is recommended when large
masses cause cerumen impaction, ear canal obstruction, or
hearing loss.

Haddad J Jr: Care of the draining ear in children. Emerg peds 1,995;8:75.
Nussinovitch M, Rimon A, Volovitz B, er al: Cotton-tip applicators as
a leading causeof oritis externa. Int J pediatr Otolaryngol 2004;6g:433435
Roland PS,Eaton DA, Gross RD, et al: Randomized,placebo-controlledevaluation of Cerumenex and Murine ."r*"* ...ou"l products. Arch Otol a r y n g o lH e a d N e c h S u r g 2 0 0 4 ; 1 3 0 : t l 7 S - 1 1 7 7 .
Roland PS, StromanDW: Microbiology of acuteotitis exrerna.Laryngoscope
2002;712:1166-1177 .
RosenfeldRM, Brown L, Cannon CR, er al: Clinical practiceguideline:acute
otitis externa. Otolaryngol Head Neck Surg 2006;134:54-523.
Van Balen FAM, Smit WM, Zuithoff pA, et al: Clinical efficacy of three
common treatmentsin acuteotitis exrernain primary care:randomizedcon_
t r o f f e dr r i a l . B M l 2 0 0 3 ; 3 2 1 : 1 2l0- l 2 0 3 .

Otitis media (OM) is second only to rhe common cold among illnessesthat bring a child to the physician's office in the United
States. The peak incidence and prevalence is from 6-20 mo of
age. Otitis media figures importantly in the differential diagnosis
of fever, is the most common reason for prescribing antimicrobial drugs to children, and often is the sole or at least primary
basis for undertaking the operations mosr commonly performed
in infants and young children: myringotomy with insertion of
tympanostomy tubes and adenoidectomy. An important characteristic of OM is its propensiry to become chronic and recur. The
earlier in life a child experiencesthe 1st episode, the greater the
degree of subsequent difficulty he or she is likely ro experience,
in terms of frequency of recurrence, severity, and persistenceof
middle-ear effusion.
Accurate definition and diagnosis in infants and young children
often is difficult (Table 639-l). Symptoms may be absent or not

well-being and the optimal method of management remain open


to question and are the subjects of controversy. There is no con,
sensus among authorities concerning the risk: benefit ratios of
available medical and surgical trearments and rhe likelihood of
long-term consequencesof OM. Although OM can be responsible for serious infectious complications, middle- and innir-ear
damage, hearing impairment, and indirect impairments of speech,
language,cognitive, and psychosocialdevelopment, most cises of
OM are not severeand are self-limitine.
The term otitis media has 2 main .oipon.ntr, acute infection,
which is termed suppurative or acute otitis media (AOM); and
inflammation accompanied by effusion, termed nonsuppurative
or secretory otitis media, or otitis media with effusion (OME).
These 2 main types of OM are interrelated: acute infection
usually is followed by residual inflammation and effusion that, in
turn, predisposechildren to recurrent infection. Middle-ear effusion (MEE) is a feature of both AOM and OME; in both conditions, is an expression of the underlying middle-ear mucosal
inflammation. In children with OM. mucosal inflammation also
is present in the mastoid air cells, which are in continuity with
the middle-ear cavity. MEE results in the conductive hearing loss
associatedwith OM. The hearing loss is variable, ranging from
none to as much as 50 decibels hearing level (dB HL). Lossesof
21-30 dB HL are usual. Although most individual episodes of
OM subside within several wk, MEE persists for >3 mo in
approximately t0-25% of cases.
EP|DEM|OIOGY.
Factors believed to affect rhe occurrence of OM
include age, gende! race, genetic background, socioeconomic

*Adapted from the chaprer in the 17'h


edition by Jack L. Paradise,MD.

Chapter
639I otitis Media I 2633
to the mechanics of breastfeedingin a study of infants with cleft
formula) was deliv(2)thepresence
(1) a history
oflvlEE,
ofacute
onset
ofsrgns
andsymptoms,
Adiagnosis
ofA0Mrequires
andsymptoms
ofmiddle-ear
inflammation
and(3)signs
ofAOM
indudes:
Thedefinrtion
earinflammation
andMEE
abrupt,
onset
ofsigns
andsymptoms
ofmiddle
Recent,
usually
indicated
Thepresence
ofMEE,
byanyofthefollowing:
B u l g ionfgt h e T M
Lrmited
orabsent
mobility
0ftheTlil
Air-fluid
level
behind
theIM
Otorrhea
rnflammation,
indicated
byeither
orsymptoms
ofmiddle-ear
5igns
Distinct
erythema
oftheTM,or
(discomfon
wnhorpredudes
ininterference
clearly
referable
totheear[s]
thattesults
Dlstinct
otalgia
normal
artivity
orsleep)

relation between the


o tobacco smoke has
been found in some studies but not others. Becausehousehold
smoking correlates inversely with socioeconomic status, possible
confounding by socioeconomic factors further complicates valid
assessmentof risk. Studies that have used objective measuresto
derermine infant exposure to second-hand tobacco smoke, such
as cotinine levels, hive more consistently identified a significant
link between tobacco smoke and OM. This evidencesuggeststhat
exDosure to tobacco smoke should be considered an important
risk factor in the development of OM.

lv1Et,
middle-ear
effrsion;]l\.4,tympanic
membrane
A0[1,
acute
otilrs
media;
PPdirl(J
Otitis
medra
Diagn0srs
and]'ranagment
0facule
0nl\4anagement
0fAcute
Otitrs
[4edia:
From
5ub(ommittee
2004;111.1451-1465

Age. The odds of developingat least 1 episodeof OM has been


reported to be 63-85% by 12 mo of age and 66-99% by 24 mo
of age. The percentage of days with MEE has been reported to
be 5-27% during the lst year of life and 6-78% during the 2nd
year oI life. Rates were highest during the ages6-20 mo. After
2yr of age, the incidence and prevalenceof OM decline progressively,although the diseaseremains relatively common into
the early school-ageyears.The most likely reasonsfor the higher
rates in infants and younger children include less well-developed
immunologic defensesand lessfavorable eustachiantubal factors
involving both the structure and function of the tube. The age of
onset of OM is an important predictor of the development of
recurrent and chronic OM, with earlier age of onset having an
increased risk for exhibiting these difficulties later in life.
Gender.The incidenceof OM is greater in boys than in girls.
In a large child development/OM study in Pittsburgh that
involved close monitoring in a sociodemographicallydiverse population, infant boys consistently had higher mean cumulative proportions of days with MEE than infant girls. Boys have
predominated in most reported studies of the treatment of OM,
and, compared with girls, consistently had higher rates of operations aimed at relieving the effects or reducing the occurrence
of OM, i.e., tympanostomy tube insertion, tympanoplasty,and
adenoidectomy, facts that support a greater predilection for the
diseasein boys, and also suggestgreater severity in boys.
Race. Otitis media is especiallyprevalent and severeamong
Native American, Inuit, and Indigenous Australian children.
Studies comparing the occurrence of OM in white children and
black children have given conflicting results, but most of the
studies have reported higher rates in white children.
GeneticBackground.Middle-ear diseaseis commonly observed
to tend to "run in families," and a number of studies have suggested that OM has a heritable component. There is a higher
degree of concordance for OM among monozygotic than among
dizygotic twins.
SocioeconomicStatus. Poverty has long been considered an
important contributing factor to both the development and the
severity of OM. Various component elements contributing to
this relationship can be surmised to include crowding, limited
hygienic facilities, suboptimal nutritional status, limited accessto
medical care, and limited resourcesfor complying with prescribed
medical regimens.
BreastMilk Comparedto FormulaFeeding.Most studiesexamining the question of breast-milk feeding versus formula feeding
have found that breast-milk feeding provides a protective effect
against OM. The effect is probably limited but may be greater in
socioeconomically disadvantaged children. The protective effect
has been shown to be attributable to the milk itself rather than

Otitis media is universal among infants


clefts, and also is highly prevalent among
us cleft palate, other craniofacial anomalies, and Down syndrome' The common feature in these coneeniial anomalies is a deficiency in the functioning of the
!ustachian tube, which predisposeschildren with theseconditions
to middle ear disease.
PneumococcalVaccination. Streptococcus pneumoniae, or
pneumococcus,is the pathogen most,commonly-identified in
oatients with acute OM. Vaccination of infants with a coniugate
vaccine is standard practice in the USA. Overall,
on.u-o.o..ul
ihe reduction in incidence of OM with vaccination appears to be
modest, lowering visits to physicians and antibiotic prescriptions
for OM by only 6-SY".Yaccrnation does appear to have a more
protective effect, however, in limiting.frequent OM episodesand
ih. n..d for surgical intervention with tympanostomy tubes.
ETIOLOGY
d
6

a. Pathogenic bacteria can be isolated by staniques from middle-ear fluid in approximately


of well-documented AOM; in the remain-

is similar to that in older infants.


Evidence of respiratory viruses also may be found in middleear exudates of children with AOM, either alone or' more com-

reaction assays,a much higher rate of bacterial pathogens can be

2634I PARTXXIX I ThEEAI


demonstrated. It remains uncertain whether viruses alone can
cause AOM under any circumstances, or whether their role is

wall. Another possible factor is defective velopharyngeal valving,


which may result in disturbed aerodynamic and hydrodynamic
relationships in the nasopharynx and proximal portions of the
eustachian tubes. In children with other craniofacial anomalies
or with Down syndrome, the high prevalence of OM is attributed to structural and/or functional eustachian tubal abnormalities; histologic evaluation of the eustachian tube in these parienr
populations demonstrates such abnormalities.

Ji;,T:i:,filiJ'"'iiH.l,'#*Jil

O
techniques. Using polymerase chain
reaction assays,middle-ear fluids have been found to contain evi-

Viral infection of the upper respiratory tract results in releaseof


cytokines and inflammatory mediators, some of which may cause
eustachian tube dysfunction. Respiratory viruses also may
enhance nasopharyngeal bacterial colonization and adherence
and impair host immune defensesagainst bacterial infection.

Under usual circumstances,the eustachiantube is passively


closed and is. opened by contraction of the tensor veli palatini
muscle. In relation to the middle ear, the eustachiantube has 3
main functions: ventilation, protection, and clearance.The most

develop in the absence of pre-existing eustachian tube obstruction.is uncertain; if so, the infection undoubtedly would lead
quickly to tubal obstruction as a consequenceof both inflammatory edema and the accumulation of middle-ear secretions.

of the tube in infants and young children may increase the likelihood of reflux from the nasopharynx and impair passive gravitational drainagethrough the eustachiantube.-Anotherpo.ribl.
explanation for improved eustachiantube function and decreased
OM incidence as infants mature is that the luminal diameter
increases, reducing the opportunity for tubal obstruction and
dysfunction.
In children with cleft palate, where OM is a nearly universal
finding, the main factor underlying the chronic middle-ear inflammation is impairment of the opening mechanism of the eustachian
tube, due perhaps ro greater-rhan-nbrmal compliance of the tubal

rent sinopulmonary infection; these deficienciesprobably underlie the susceptibility to infection. Children with recurrent OM
that is not associatedwith recurrent infection at other sites rarely
have a readily identifiable immunologic deficiency.Nonetheless,
evidencethar subtle immune deficits play a role in the pathogenesis of recurrent AOM is provided by studies involving antibody
responses to various types of infection and immunization; by
the observation that breast-milk feeding, as opposed to formula
feeding, confers limited protecrion against the occurrence of OM
in infants with cleft palate; and by studies in which young children with recurrent AOM achieved a measure of protection from
intramuscularly administered bacterial polysaccharide immune
globulin or intravenously administered polyclonal immunoglobulin. This evidence, along with the documented decreaseinlncidence of upper respirarory tract infections and OM as children,s
immune systems mature is indicative of the importance of a
child's innate immune system in the pathogenesisof OV.
Evidence that respiratory allergy is a primary etiologic agent in
OM is not convincing; however, in children who have both allergies and OM, it seemspossible rhat the otitis may be aggravated
by the allergy.

upper respiratory tract infections also occur, and occasionally


there may be no symptoms, with AOM discovered at a routine

chapter 6il9 I otitis 14sii6 r 2635

health examination. OME often is not accompanied by overt


complaints of the child but usually is accompanied by hearing
loss. This hearing loss may manifest as changesin speechpatterns
but often goes undetected if it is unilateral or mild, especially in
younger children. Balance difficulties or dysequilibrium also can
be associated with OME, and older children may complain of
mild discomfort or a senseof fullness in the ear (seeChapter 535).
pactlng lt.

EXAMINATION
OFTHEEARDRUM
0T0SC0PY.For clinicians other than otolaryngologists, who may
use an operating microscope, the eardrum ordinarily is viewed by
means of an otoscope. Two types of otoscope heads are available:
surgical or operating, and diagnostic or pneumatic. The surgical
head embodies a lens that can swivel over a wide arc and an unenclosed light source, providing ready access of the examiner's
instruments to the external auditory canal and tympanic membrane. Use of the surgical head is optimal for removing cerumen
or debris from the canal under direct observation, and is necessary for satisfactorily performing tympanocentesis or myringotomy. The diagnostic head incorporates a larger lens, an enclosed
light source, and a nipple for the attachment of a rubber bulb
'When
an attached speculum is fitted snugly into the
and tubing.
external auditory canal, an airtight chamber is created, consisting of the vault of the otoscope head, the bulb and tubing,
the speculum, and the proximal portion of the external canal.
Although examination of the ear in young children is a relatively
invasive procedure that often is met with lack of cooperation by
the patient, this task can be enhanced if done with as little pain
as possible. The outer portion of the ear canal contains hairbearing skin and subcutaneous fat and cartilage that allow a
speculum to be placed with relatively little discomfort. Closer to
the tympanic membrane the ear canal is made of bone and is lined
only with skin and no adnexal structures or subcutaneous fat; a
speculum pushed too far forward and placed in this area often
causes skin abrasion and pain. Using a rubber-tipped speculum
or adding a small sleeveof rubber tubing to the tip of the plastic
speculum may serve to minimize patient discomfort and enhance
the ability to achieve a proper fit and an airtight seal.
Learning to perform pneumatic otoscopy is a critical skill in
being able to assessa child's ear and in making an accurate diagnosis of OM (seeFig. 635-1). By observing as the bulb is alternately squeezed gently and released, the degree of tympanic
membrane mobility in response to both positive and negative
pressure can be estimated, providing a critical assessmentof
middle-ear fluid, which is a hallmark sign of both AOM and
OME. lfith both types of otoscope heads, bright illumination
also is critical to adequate visualization of the tympanic membrane.
AUDIT0RYCANAI. If the tympanic memCIEARINGTHEEXTERNAT
brane is obscured by cerumen, the cerumen may be removed
under direct observation through the surgical head of the otoscope, using a Buck curette (N-400-0, Storz Instrument Co).
Remaining bits can then be wiped away using a Farrell applicator (N-2001A, Storz Instrument Co), with its tip (triangular in
cross section) wrapped with a bit of dry or alcohol-moistened
cotton to create a dry or wet "mop." Alternatively, gentle suction
may be applied, using a No. 5 or 7 French ear suction tube.
During this procedure it may be most advantageous to restrain
the infant or young child in the prone position, turning the child's
head to the left or right as each ear is cleared. One adult, usually
a parent, can place one hand on each of the child's buttocks and
brace the child's hips against the examining table, using his or
her own weight for additional bracing if necessary.Another adult
can restrain the child's head with one hand and the child's free
arm with the other, changing hands for the opposite ear. In children old enough to cooperate, usually beginning at about 5 yr of

TYMPANICMEMBRANEFINDINGS.Important characteristicsof the

alone may result from crying or vascular flushing. Abnormal


whiteness of the membrane may result from either scarring or the
presenceof liquid in the middle-ear cauity;,liquid also may impart
an amber, paie yellow, or (rarely) bluish color. NormallS the
membrane is translucent, although some degree of opacity may
be normal in the first few mo of life; later, opacification denotes
either scarring, or more commonly, underlying effusion. Structural changeslnclude scars, perforations, retraction,pockets, anda more sevire complication of OM, cholesteatomaformation. Of
all the visible characteristicsof the tympanic membrane, mobil-

common finding.
DIAGN0SIS.Correct diagnosis of AOM is important to guide clin-

membrane or otalgia (seeTable 639-7). A simplified differentiating schema establiihes a diagnosis of AOM when, in a.ddition to
having MEE, a child gives evidence of recent, clinically import".rt .-ut pain or the tympanic membrane shows marked redness

2636I PARTXXIX r ths Ert

At least two of:


1. AbnormalTM color:
white,yellow,amber,or blue
2. Opacificationnot due to scarring
3. Decreasedor absentmobility

Or

Bubbles
or air-fluid
interfaces
behindtheTM

Acutepurulentotorrhea
notdueto otitisexterna

No Acute
Inflammation

At leastoneof:
1. Substantial
earpain,including
unaccustomed
tuggingor
rubbingof theear
2. Markedrednessof theTM
3. Distinct
fullnessor bulgingof theTM

Otitis media with effusion

(oME)

Figure 639-1. Algorithm for distinguishingbetweenacute otitis media (AOM) and otitis media with effusion (OME). TM,
tympanic membrane.

CommonlS both before and after episodesof OM and also in


the absence of OM, the rympanic membrane may be retracted as
a consequenceof negative middle-ear air pressure.The presumed

Figure 639-2. Tympanic membrane in acute otitis media (AOM).

Figure 639-3. Tympanic membrane in otitis media with effusion (OME).

Ghapterliil9 r OtitisMedia I 2tiil7


cause is diffusion of air from the middle-ear cavity more rapidly
than it is replaced via the eustachiantube. Mild retraction cannot
be considered pathologic, although in some children it is accompanied by mild conductive hearing loss. More extreme retraction,
howeveq is of concern, as discussedlater in the section on sequelae of OM.
TYMPAN0METBY.
Tympanometry, or acoustic immittance testing,
is a simple, rapid, atraumatic test that, when performed correctly,
offers obiective evidenceof the oresenceor absenceof MEE. The
tympanogram provides information about tympanic membrane
compliance in electroacoustic terms that can be thought of as
roughly equivalent to tympanic membrane mobility as perceived
visually during pneumatic otoscopy. The test dependson the facts
that the absorption of sound by the tympanic membrane varies
inversely with its stiffness and that the stiffness of the membrane
is least, and accordingly its compliance is greatest, when the air
pressuresimpinging on each of its surfaces-middle-ear air pressure and external canal air pressure-are equal. In simple terms,
anything tending to stiffen the tympanic membrane, such as tympanic membrane scarring or middle-ear fluid, reduces the tympanic membrane compliance, which is recorded as a flattening of
the curve of the tympanogram. An ear filled with middle-ear fluid
usually has a very noncompliant tympanic membrane and, therefore, a flattened rympanogram tracing.
Tympanograms may be grouped into 1 of 3 categories (Fig.
539-4\. Tracings characterized by a relatively steep gradient,
sharp-angled peak, and middle-ear air pressure (location of the
peak in terms of air pressure)that approximate atmospheric pressure (Fig. 639-4A) (type A curve) are assumedto indicate normal
middle-ear status. Tracings characterizedby a shallow peak or no
peak and by negative or indeterminate middle-ear air pressure,
and often termed flat or type B curve (Fig. 539-aB), usually are
assumedto indicate the presenceof a middle-ear abnormaliry that
is causing decreasedtympanic membrane compliance. The most
common such abnormality by far in infants and children is MEE.
somewhat
Tracings characterized by intermediate findings-a
shallow peak, often in association with a gradual gradient
(obtuse-angledpeak) or negative middle-ear air pressure,or combinations of thesefeatures (Fig. 539-aC), may or may not be associated with MEE, and must be considered nondiagnostic or
equivocal. In general, the more shallow the peak, the more
gradual the gradient, and the more negative the middle-ear air
pressure, the greater the likelihood of MEE.
'When
reading a tympanogram it is important to look at the
volume measurement also provided. A patient with a tympanic
membrane perforation or patent tympanostomy tube will have a
flat, type B tympanogram and a "high volume." The tympa-

nometer measures and records the volume of the external auditory canal, and if a tympanic membrane perforation or a patent
tympanostomy tube is present, the volume of the middle ear and
masioid air cells as well. A volume reading of >1.0mL should

noses of MEE. Importantly, even though rympanometry can


predict the probabiliry of MEE, it cannot distinguish the effusion
of OME from that of AOM.

(see below) effective against non-typable H' influenzae'

TREATMENT
of AOM
ofAcuteOtitisMedia.Individualepisodes
Management
customarily have been treated with antimicrobial drugs. Concern
about increasesin bacterial resistancehas prompted some authors
to recommend withholding antimicrobial treatment in some cases
unless symptoms persist for 2-3 days, or worsen (Table 639-2).
Three faitors argue in favor of routinely treating children who
have bona fide AOM (seeTable 539-1 andFig. 639-2) with an
antimicrobial drug. First, pathogenic bacteria cause a large
majority of cases.Second' symPtomatic improvement and resolution of infection occur more promptly and more consistently
with antimicrobial treatment than without, even though most
untreated caseseventually also resolve. Finally, prompt and adequate antimicrobial treatment may prevent the development of

rt
r.5

mI
1.5

ml

.5

.5

.5

- 606 -300

+3OO

daPa

-600

-300

r300

-609

deP.

-300

+300

drPr

Figure 639-4. Tympanogramsobtained with a Grason-StadlerGSI 33 Middle Ear Analyzer,exhibiting (A) high admittance,steepgradient (i.e., sharp-angled
p."t;, a"d midd'le-iar ^L pr"..r.r. approximating atmosphericpressure(0 decaPascals
[daPa]);(B) low admittanceand indeterminatemiddle-earair pressurel
and (C) somewhat low admittance,gradual gradient,and markedly negativemiddle-earair pressure.

2638r PARTXXIX r TheEar


to assessfor lack of spontaneous resolution or worsening of
symptoms and that patients should be provided with adequate
analgesicmedications-acetaminophen or ibuprofen-during the
AGE
CERTAIN
DIAGNOSIs
UTICERTAIN
DIAGIIOSIS
period of observation.Topical otic agents (e.g., Auralgen) also
<6 mo
Antibacterial
therapy
Antibacterial
therapy
may be effective for pain relief. Vhen pursuing the practice of
6 mo-2y
Antibacterial
therapy
Antibacterial
therapy
ifsevere
illness;
watchful waiting in patients with AOM, the certainry of the diag0bservation
option*
if nonsevere
illness
nosis, the patient's age, and the severity of the diseaseshould be
>2y
Antibacterial
therapy
ifsevere
illness,
0bservation
option*
considered.For younger patients, <2 yr of age, it is recommended
0bservati0n
0ption*
ifnonsevere
illness
to treat all confirmed diagnoseso{ AOM. In very young parienrs,
Thistablewas
modified
withpelm6si0n
[rOm
theNewYork
State
DepartmentofHealrh
aodtheNewYork
Region
0tit6Projed
<6 mo of age, even presumed episodesof AOM should be treated
[ommittee.
*0bservation
isanappropriateoption
onlywhen
follow-upcan
beensuredand
antibacterial
agenrstarted
ifsymptomspe6ist due to the increasedpotential of significant morbidity from infec0Iw06en
Nonsevere
rllneJstr
mildotalgia
andftver<l9tinthepast
24h0u6severc
illness
ismoderatet0severeotalgia tious complications. In children 6-24 mo of age who have a ques0rfever
>39T.A(ertdin
diagnos8
0fA0[,]meers
all] (rireria:{j)
rapid
onse!(2)signs
ofMtqand(j) signs
andsymptionable diagnosis of OM but are severely ill, defined as
lomsofmiddle-ear
infiammaion
temperature of >102'F (>39"C), significant otalgia, or toxrc
Fromsub(ommine
onManagement
of Acute
OtitisMedia.
Diagnosis
andma0agement
of a(uteotatis
mediaPediirrnc
-1 465
2004;'l
11.1451
appearance, antibiotic therapy is also recommended. However,
children rn this age group in whom the diagnosis is questionable
and the diseaseis not severecan be observed for a period of 2-3
days with close follow-up. In children >2yr of age, observation
might be consideredin all episodesof non-severeOM or episodes
of questionable diagnosis, with antibiotic therapy reserved for
suppurativecomplications.ft seemslikely rhat the sharp decline
confirmed, severeepisodesof AOM.
in such complicarionsduring the last half-centurymay be attribured, at least in part, ro the widespread routine use of antimicrobials for AONI. In the Netherlands, where initial antibiotic
BACTEBIAIBESISTANCE.
Bacteria have a remarkable ability to
treatment routrnelv is rvithheld from mosr children older than 6
develop resistanceto antimicrobial drugs, and bacterial resistance
mo of age, and where onlv approximately 30o/oof children with
has becomean increasingproblem in the past decade.Personsat
AOM receive anribiotics, the incidence of acute mastoiditis,
greatest risk of harboring resistant bacteria include children who
a l t h o u g h l o w ( i n c h i l d r e n < 1 4 y r o f a g e , 3 . 8 / 1 0 0 , 0 0 0p e r s o n
are <2 yr of age; are in regular contact with large groups of other
years), appears slightlv higher than rares in orher countries
children, especially in daycare setings; or who recently have
with hrgher anribioric prescription rates bv about 1-2
received antimicrobial treatment. Bacterial resistanceis a oarticepisodes/100,000person years. Follow-up of children in the
ular problem in relation to OM. The development of reiistant
N e t h e r l : r n d sm l v h e g e n e r a l l vm o r e a : s r d u o u st h a n i s c u s t o m a r v
bacterial strains and their rapid spread have been fostered and
in other counrries,including the United Srates,where failure to
facilitated by selective pressure resulting from extensive use of
improve or \\rorsening symptoms might not be detected as
antimicrobial drugs, the mosr common target of which, in chilpromptlv.
dren, is OM. In addition, many strains of each of the pathogenic
These considerarions in treating AOM with antimicrobial
bacteria that commonly causeAOM are resistant to certain comt h _ e r a pm
r u s r h e h a l a n c e da g a i n s tt t e c o n t i n u e di n c r e a s i n gr a r e s
monly used antimicrobial drugs.
of bacterial antin-ricrobialresistance.In countries such ls the
Although antimicrobial resistancerates vary from country to
Netherlands, where use of antibiotics for OM is much less
country, in the USA approximately 40'/' of strains of nontypable
common, the antimicrobial resistance rates for the maior
H. inlluenzae and almost all strains of M. catarrhalis currently
pathogensin OM are substantiallylower than those rn countries
are resistantto aminopenicillins(e.g.,ampicillin, amoxicillin). In
that routinelv rrear AOM with anribiotics. Given rnar mosr
most cases the resistance is attributable to production of Bepisodesof OM resolvesponraneouslv,
consensusguidelineshave
lactamase, and can be overcome by combining amoxicillin with
been published br. the American Academy of Pediatricsto assist
a B-lactamase inhibitor, namely, clavulanate, or by using a
clinicianswho wish ro consrdera period of "watchful waiting"
antibiotic. However, occasionalstrainsof nonB-lactamase-stable
or observation prior to trearing AOM with anribiorics (Tables
typable H. inlluenzae that do not produce B-lactamaseare resrs639-2 and 639-31Fig. 5.19-5).The most important aspectof these
tant to aminopenicillins and other B-lactam anribiotics by virtue
guidelinesis that close follow-up of the patient -usi be ensured
of alteratronsin their penicillin-bindingproteins.

TEMPTRATURT
>39TAND/OR
SEVERE
ATDIAG}IOSIS
TOR
PATIENTS
BEING
TRTATEO
OIATGIA
Iil|rIATI,Y
WITH
ANTIEACTTRIAI.
AGTTITS

No

Yes

CtII{IGITY
DTTIIITD
TREATMENT
FAITURE
AT
48-72HRAITER
IIIIIIAI.MANAGTMENT
I/I'ITH
OBSERVATIOI{
OPTION

O.IiII(AITY
DETITIED
TREATMTNT
TAII,URE
AT
48-72HRATTTR
II{ITIAT
MAI'IAGTMINT
WITH
AiITIBACTERNI
AGEI'IT5

AI.TTR}IATIVE
TOR
ATTERNATIVT
FOR
ATTERl{ATIVE
FOR
PENICITTINAI.TERGY
RE(OMMEI{DED
PENIflTtIN
ATTERGY
REC()MMENDED
PENICILLINATI,ERGY
Non-typel.cefdrnir,
Amoxicillin,80-90mq/kg
Non-type
1:cefdinir;
Amoxicillin-clavulanate,90mg/kg
Non-type
l:(eftriaxong3
(efuroxime,
perday
cefuroxime,
cefpodoxime; per
day
ofamoxirillin
days;
type
1:clindamyrin
cefoodoxlme;type
(omponent,with
typeliazithromycin,
64 mg/kg
1:azithromy(in,
perday
darithromycin
ofdavulanate
clarjthromycin
Amoxiciliin-clavulanate,feftriaxone,
1or3days Amoxicillin,
davulanate, Ceftriaxone,l
or3days
[eftriaxone,3
days
Tympanocentais,
dtnda
mycin
90mg/kgpu dayof
perday
90mg/kg
of
amoxkillin,
wirh64 mgikg
amoxl(itlin,with
6_4
mg/kg
perdayof(lavulanate
perday
ofclavulanate
RT(OMMEI{DTD
Amoxkillin,8O-90
mg/kg
perday

From
sJb(ommittee0n
Management
0fArute
0titisl\,4edia:Diagno5is
andmanagment
0facute
otitismediapediltti,J20Q4;113:145j-1M5

639 I otitis Media r 2639


Chapter

1
A diagnosisot acuteotitismedia
requrres:
1) Historyof acuteonset of signs
and symptoms

Theclinician i
assessespain. i

2) The presenceof middle-ear


effusion
3) Signsand symptomsof middleear inflammation

Yes
Clinicianrecommends
treatmentto reduce
parn.

Amoxicillinat a dose of
80-90 mg/kg/dayis the
initialantibacterialof
choicefor most children

.Criteriafor antibacterial
treatmentor observationin
childrenwith nonsevereillness:t
'1)<6 mo: antibacterial
treatment
treatmentwith certain
2) 6 mo to 2 years:antibacterial
diagnosisor severeillnessor observationwith uncertain
diagnosisand nonsevereillness
3) 2 years and older:antibacterialtreatmentif severe
illnessor observewith nonsevereillnesswith certain
diagnosis;observationfor uncertaindiagnosis.
fOaregiveris informedand agreesto the optionof
observation
Caregiveris able to monitorchild and returnshould
conditionworsen
Systemsare in placefor readycommunicationwith
and obtainingmedication
reevaluation,
the clinician,
if necessary.
Figure 639-5. Management of AOM. (From Subcommittee on Management of Acute Otitis Media: Diagnosis and management of acute otitis media. Pediatrics
Continued
204 :11.3:145 l-1. 4 6 5.1

2640r PABTXXIXr TheEar

16

Did patientrespond
to initialtreatment
intervention(either
antibacterial
treatmentor
observation)?

Clinicianreassesses
and confirms
diagnosisof AOM.

18
Assessfor othercauses
of illnessandmanage
appropriately.
Patientfollow-up
as appropriate.
19
Clinicianshouldinitiate
antibacterialtreatmentfor children
initiallymanagedwith observaton
or changeanitbacterialtreatment
for patientsinitiallymanagedwith
antibacterialtherapy

Antibacterialchoice
shouldbe basedon the
likelypathogen(s)
presentand on clinical
expenence.

Figure619-i. s6n1'd

Approximarely 50% of strains of S. pneumonlae are penicillinnonsusceptible,divided approximately equally berweenpenicillinintermediate and, even more difficult to treat. penicillin-resistant
strains. A much higher incidence of resistanci ir r..tt in children

Many penicillin-resistant strains of S. pneumoniae also are resis-

in resistanceto clindamycin, which otherwise is generally effective against resisrant strains-of S. pneumoniae. Uilike ,.rirtr.,..
to BJactam antibiotics, macrolide resisrancecannot be overcome
by.increasing the dose. Although vancomycin previously was a
fail-safe antimicrobial in trearing S. pneum'oniae,clinical casesof
vancomycin-tolerant S. pneumoniae have been identified, further
raising the importance and hazard of antimicrobial resistance.
FIRST-tlNEANTIMICROBIAL
TREATMENT,
Amoxicillin remains the
preferred drug for uncomplicated AOM under mosr circum-

a0 m{kg/2a hr to 80-100 mg/k{24 hr usually is effective against

penicillin-intermediate and some penicillin-resistant strains. This


higher dose should be used particularly for children <2yr of age
who recently have receivedtreatment with BJactam drugs or been
exposed to large numbers of other children, becauseit is in these
children that the prevalenceof nonsusceptiblestrains of S. pneumoniae is highest. A limitation of amoxicillin is that it may be
inactivated by the B-lactamasesproduced by many strains of
nontypable H, influenzae and most strains of M. catarrhalis.
Fortunately, episodesof AOM caused by these pathogens often,
although by no means always, resolve spontaneously. Allergies
to penicillin antibiotics should be caregorized as rype t hypersensitivity, consisting of urticaria or anaphylaxis, and those
that fall short of type 1 reactions, such as rash formation. For
children with a non-rype 1 reaction in which cross-reacriviry
with cephalosporins is less of a concern, first-line therapy with
cefdinir would be an appropriate choice (see Table 639-3). ln
children with a type 1 reaction or known sensitivity to
cephalosporin antibiotics, or in whom palatability or convenience
of administration are of overriding importance, azithromycin is
an appropriate alternative first-line drug. Resistanceto trimerhoprim-sulfamethoxazole by many strains of both H. influenzae and
S. pneumoniae and a reported high clinical failure rate in children with AOM treated initially with this antimicrobial argue
against its use as first-line treatment.
DURATION0F TREATMENT.The duration of trearment of AOM
often has been set at 10 days, apparently extrapolated from the
optimal duration of treatment of streptococcal pharyngitis with
penicillin. However, 10 days may be unduly long for some children while not long enough for others. Studiescomparing shorter
with longer durations of treatment suggest that short-course
treatment often is inadequate in children <6 yr of age, and particularly in children <2 yr of age. Thus, for most episodesin most
children, treatment that provides tissue concentrations of an
antimicrobial for at least 10 days seemsadvisable. Treatment for
shorter periods, of 3-5 days, may be appropriate for older children with mild episodeswho improve quickly; however, in these
cases, one could reasonably argue that simple observation
without antimicrobial therapy would be the preferred intervention. Treatment for >10 days may be required for children who

Ghapterfil9I otitis Media I 264t


are very young or are having severeepisodesor whose previous
experiencewith OM has been problematic.
F0tt0W.UP. The principal goals of follow-up are to assessthe
outcome of treatment and to differentiate between inadequate
response to treatment and early recurrence. Accordingly, the
appropriate interval for follow-up should be individualized.
Follow-up within days is advisable in the young infant with a
severe episode or in a child of any age with continuing pain.
Follow-up within 2 wk is appropriate for the infant or young
child who apparently has been having frequent recurrences. At
that point, the tympanic membrane is not likely to have returned
to normal, but substantial improvement in its appearanceshould
be evident. In the child with only a sporadic episode of AOM and
prompt symptomatic improvement, follow-up 1mo after initial
examination is early enough; in older children, no follow-up may
be necessary.Importantlg the continuing presence of MEE alone
following an episode of AOM is not an indication for additional
or second-line antimicrobial treatment.

T0 FIRST-LINE
TREATMENT,
UNSATISFACTORY
RE$P0NSE
AoM is
essentiallya closed-spaceinfection, and its resolution depends on
both eradication of the offending organism and restoration of
middle-ear ventilation. Factors contributing to unsatisfactory
response to first-line treatment, in addition to inadequate antimicrobial efficacy, include poor compliance with treatment
regimens, concurrent or intercurrent viral infection, persistent
eustachian tube dysfunction and middle-ear under-aeration, reinfection from other sites or from incompletely eradicated middle
ear pathogens, and immature or impaired host defenses.Despite
these many potential factors, switching to an alternative or
secondline drug is reasonable when there has been inadequate
improvement in symptoms or in middle-ear status as reflected in
the appearance of the tympanic membrane, or when the persistence of purulent nasal discharge suggeststhat the antimicrobial
drug being used has less than optimal efficacy. SecondJine drugs
also may be used appropriately when AOM develops in a child
already receiving antimicrobial therapy or in an rmmunocompromised child, or in a child with severesymptoms whose previous experience with OM has been problematic.
'When
TREATMENT.
SECOND-LINE
treatment of AOM with a firstline antimicrobial drug has proven inadequate, a number of
second-line alternatives are available (see Table 639-3). Drugs
chosen for second-line treatment should be effective against Blactamase-producing strains of H. influenzae and M. catarrhalis
and against susceptible and most nonsusceptible strains of S.
pneumoniae. Only 3 drugs have been shown clearly to meet
that requirement: amoxicillin-clavulanate, cefuroxime axetil, and
intramuscular ceftriaxone. Because high-dose amoxicillin
(80-100 mg/kglza\ is effective against most strains of S. pneumoniae and because the addition of clavulanate extends the
effective antibacterial sDectrum of amoxicillin to include
p-lactamase-producing
high-dose
amoxicillinbacteria,
clavulanate is particularly well suited as a second-line drug for
treating AOM. The 14 : 1 amoxicillin-clavulanate formulation
contains twice as much amoxicillin as the previously available
7 : 1 formulation. Diarrhea, especially in infants and young children, is a common adverse effect, but may be ameliorated in some
cases by feeding yogurt, and usually is not severe enough to
require cessationof treatment. The 2 other drugs have important
limitations for use in young children. The currently available suspension of cefuroxime axetil is not palatable, and its acceptance
is low. Ceftriaxone treatment entails both the pain of intramuscular iniection and substantial cost; the inlection may need to be
repeated once or twice at 2- or 3-day intervals to achieve the
desired degree of effectiveness.Nonetheless, use of ceftriaxone is
appropriate in severecasesof AOM when oral treatment is not

feasible, or in highly selected cases after treatment failure using


orally administered second-line antimicrobials (e.g., amoxicillinclavulanate, cefuroxime axetil), or when highly resistant S.
pneumoniae is found in aspirates obtained from, diagnostic tympanocentesis. Cefdinir was not available when the above recommendations were made. However, clinical Practice and efficacy
with cefdinir suggest that it deserves consideration as a secondline agent as well, with antibacterial spectrums similar to those
of cefuroxime axetil, and good rates of compliance' as the medication is quite palatable.
The newir macrolides, clarithromycin and azithromycin, have
only limited activity against nonsusceptible strains of S. p-neumoniae and against p-lactamase-producing strains of H. influenzae. Macrolidl use also appears to be a major factor in causing
increases in rates of resistance to macrolides by group A streptococcus and S. pneumoniae. Of the cephalosporins other than
cefuroxime axetil and cefdinir, cefpodoxime seems the most
promising. However, compliance may be affected by the lack
bf palata6ility of this medication. Cefprozil possessessomewhat
lesi B-lactamase stability than either cefuroxime or cefpodoxime. Cefaclor and loracarbef have limited activity against B-

penicillin-nonsusceptibleS. pneumoniae.
Myringotomy is a timeMYRING0T0MYAND TYMPAN0CENTESIS.
honored treatment for AOM but is not commonly needed in chil-

central nervous system infection; and immunologic compromise.


Myringotomy should be considered as third-line theraPy in
o"ii.ttir who have falled 2 courses of antibiotics for an episode
^of
eOV. In children with AOM in whom clinical response to

a clinician with this capacity." Performance of tympanocentesis


can be facilitated by use of a specially designed tympanocentesis
asplrator.
Recurrenceof AOM after
AFTERTREATMENT,
EARTYRECURRENCE
apparent resolution may be due to either incomplete eradication

2842I PARTX|(IX r The Ear


of infection in the middle ear or upper respiratory rracr or ro reinfection by the same or a different bacterium. Recurrencewithin

MANAGEMENT
OFOTITIS
MEDIA
WITHEFFUSION
To determine the course of an episode of OME, and to distinguish between persistenceand recurrence,examination should be
conducted monthly until resolution, and hearing should be
assessedif effusion has been present for >3 mo (see Fig. 639-1,1.
Rational management of OME depends to a considerable degree
on understanding its natural history and its possible complications and sequelae.Most cases of OME resolve without treatment within 3 mo; limited data are available concerning the
subsequentlong-term course of children in whom OME does not

MYBINGOTOMYAND INSERTIONOT TYMPANOSTOMYTUBES.

made to consider referral for this procedure. However, when a


patient requires 3-4 courses of antibiotics for episodesof AOM
in a 5-mo period or 5-5 episodes in a 12-mo period, potential
surgical managemenr of the child's AOM should be discussed
with the parenrs.

of other difficulties in children that also should be considered.


These include predisposition to recurring AOM, pain, disturbance of balance,and tinnitus. Long-term sequelaethat have been
demonstrated to be associated with OME include pathologic
middle-ear changes; atelectasisof the tympanic membrane and
retraction pocket formation; adhesive OM; ossicular discontinuiry; cholesteatoma; and conductive and sensorineural hearing
loss. Long-term adverse effects on speech, language, and cognil
tive and psychosocial development also are noted. Nonetheless,
some studies have demonstrated that the long-term adverse
impact of OME on development may be small. In considering the
impact of OME on developmenr, it is especially important ro take
into consideration the overall presentation of the child. Although
it is unlikely that OME causing unilateral hearing loss in the mild

VARIABTESINFTUENCING
MANAGEMENTDECISIONS
FOROTITIS
MEDIA WITH EFFUSION,
Patient-related variables that affect decisions on how to manage OME include the child's age; the frequency and severity of previous episodesof AOM and the interval
since the last episode;the child's language development; the presence or absence of a history of adverse drug reactions, concrrrrent medical problems, or risk factors such as daycare attendance

that must be consideredin the treatment of OME include whether

lmportant. In some cases with very thick, tenacious discharge,


topical therapy may be inhibited becausethe medicatiott ."rrrioi
be delivered to rhe sire of infection, and suctioning and removal
of the secretions,often done with referral to an ot;laryngologist,
may be quite helpful. Ifhen children with tube otorrhe-a fai'i to
improve satisfactorily with conventional outpatient management,
they may require tube removal or hospitalization to recetveparenteral antibiotic treatment, or both.

MEDICAI TREATMENT,Antimicrobials have definite but limited


efficacy in resolving OME, presumably because they help eradicate nasopharyngealinfection or inapparent middle-ear infection,
or both. However, mainly because of the short-term nature of
their benefit and because of the contribution of antimicrobial
usage to the development of bacterial resistance,rourine anrimicrobial treatment of OME, previously recommended by some

639 I 0titis Media r 264i1


Ghapter
authorities, no longer seems wise. Instead, treatment should be
limited to casesin which there is evidenceof associatedbacterial
upper respiratory tract infection or untreated middle-ear infection. For this purpose, the most broadly effective drug available
should be used, as recommended for AOM.
The efficacy of oral corticosteroids in the treatment of OME is
probably short-term at best, and guidelines for the treatment of
OME have determined that the risk : benefit ratio for these medications would argue against their use. Antihistamine-decongestant combinations are not effective in treating children with
OME, but their efficacy has not been tested in children with environmental allergies. Antihistamines alone, decongestantsalone,
and mucolytic agents are unlikely to be effective. Allergic management might prove helpful in children with problematic OME
who also have evidence of upper respiratory allergn but supporting data are lacking. In children without such evidence,
allergy tesring is not indicated. Inflation of the eustachian tubes
by the Valsalva maneuver or other means has no proven longterm efficacy.

COMPIICATI0NS0F ACUTE0TlTlS MEDIA. Most complications of


AOM consist of the spread of infection to adjoining or nearby
srructures or the development of chronicity, or both' Suppurative
complications are relatively uncommon in children in developed
countries, but occur not infrequently in disadvantaged children
whose medical care is limited or nonexistent. Complications of

MYBINGOTOMYAND INSERTIONOF TYMPANOSTOMYTUBES.


When OME persistsdespite an ample period of watchful waiting,
usually 3-5 mo or perhaps longer in children with unilateral effusion, the question arisesas to surgical intervention. Myringotomy
alone, without tympanostomy tube insertion, permits evacuation
of middle-ear effusion and sometimes is effective; often, because
the incision heals before the middle ear mucosa returns to normal
the effusion soon re-accumulates.Inserting tubes offers the likelihood that middle-ear ventilation will be sustained for at least as
long as the tube remains in place and functional, about 12 mo on
average, and nearly uniformly reverses the conductive hearing
loss associatedwith OME. Occasional episodesof obstruction of
the tube lumen and premature tube extrusion may limit the effectivenessof tympanoitomy tubes, and tubes also ca.r be associated
with otorrhea. However, placement of tympanostomy tubes
usually is effective in providing resolution of OME in children.
Sequelaefollowing tubal extrusion include residual perforation
of the eardrum, tympanosclerosis, localized or diffuse atrophic
scarring of the eardrum that may predisposeto the development
of atelectasisor a retraction pocket or both, residual conductive
hearing loss, and cholesteatoma.Fortunately, the more serious of
these sequelaeoccur infrequently. Recurrence of middle-ear effusion following the extrusion of tubes does develop, especially in
younger children. However, most children without underlying
craniofacial abnormalities require only 1 set of tympanostomy
tubes, with developmental changes providing improved middleear health and resolution of their chronic OME by the time of
tube extrusion. Because even previously persistent OME often
clears spontaneously during the summer months, watchful
waiting through the summer seasonalso is advisable in most children with OME who are otherwise well. Finally, in considering
surgical management of OME in children, primarily in those with
bilateral diseaseand hearing loss, it has been demonstrated that
placement of tympanostomy tubes provides an improvement in
their quality of life.

TYMPANICMEMBRANEPERF0RAT|0N.Rupture of the tympanic


membrane can occur with episodes of either AOM or OME.
Although damage to the tympanic membrane from theseepisodes
usually heals spontaneouslS chronic perforations can develop in
a small number of casesand require further surgical intervention'

CREA5T
Maybeabsent
Ac|rte
mastoiditis
withperiosteitis
withsubpenostea
absces
Absent
Arute
mastoidrtis
Periosteitis
0fplnna
wrthponauri(ular
extensi0n lnta(t
Inta(t
withp0staurirular
extension
txternai
otitls
ymphadenitis
Inuct
Postauricular
*Postduflcular
(fold)
pinna
area
cfease
between
andposlauticular

the skin of the external auditory canal resulting from contamination by purulent discharge from the middle ear. The skin often
is erythematous, edematous, and tender' Management consists
of proper hygiene combined with systemic antimicrobials and
otoiopical drops as appropriate for treating AOM and tube
otorrnea.

0TlTlS MEDIA' Chronic suppurative OM


CHR0NICSUPPURATIVE

required.
ACUTEMAST0|D|T|S.Technically, all cases of AOM are accompanied by mastoiditis by virtue of the associatedinflammation of
ihe mastoid air cells. However, early in the course of the disease

provides satisfactory resolution.


In acute mastoid osteitis, or coalescent mastoiditis, infection
has progressedfurther, causing destruction of the bony trabecu-lae of tLe mastoid (Fig. 639-6). Frank signs and symptoms of

EXTI
RNAI.(ANAI-INFECTION
ANDSYMPTOM5
POSTAURI(UUA
SIGI{s
TENDERNESS
ERYTHEMA MASS
NO
Usually
NO
Ie5
No
Yes
Yes
Maybe
NO
Usually
No
Ies
Yes
Usually
No
Ie5
No
([ircumscrrbed) Maybe
Yes
NO

p l3l
WB5aunde6,
2001,
edPhiladeLphta,
Media
inlnfanrs
antl(hildren,3i
From
Blrestone
[D Klein
] a kditots).)riils

ly
Usua
Usually
NO
t\0
NO

2644r PART
XXIXr TheEar

centmastoiditis;
righrmasroidis normal.Mastoidsurgerywasperformed
in
thiscase.(FromBluestone
CD, KleinJO [edirors]:
OtitisMediain Infantsand
Children,3rded.Philadelphia,
WB Saunders,
2001.,
p 337.1

suppurativ_eOM, paralysis of the external rectus muscle, and pain


in the ipsilateral orbir. Rarely, mastoid infection spreads ro rhe
neck musclesthat attach ro the mastoid tip, resulting in an abscess
in the neck, termed a Bezold abscess.

osteitis require intravenous antimicrobial treatment and mastoidectomy, with the extent of the surgery depending on the
extent of the disease process. As far as possibl-, choice of the
antimicrobial regimen should be guided by the findings of microbiologic examination.
Each of the variants of mastoiditis also may occur in subacute
o_rchronic form. Symptoms are correspondingly less prominent.
Chronic masroidiris always is accompanied by chronii suppurative OM, and occasionally will respond to the conseivative
regimen recommended for that condition. In most cases.however.
mastoidectomy also is required.
FACIALPABALYSIS.
The facial nerve, as ir traversesthe middle ear

temporal bone, lined by keratinized, stratified squamous epithelium and containing desquamated epithelium and/or keratin
(see Chapter 537). Acquired, as distinct from congenital,
cholesteatoma most often develops as a complication of longstanding chronic OM. However, the condition also may develop
from a deep retraction pocket of the tympanic membrane or
as a consequence of epithelial implantation in the middle-ear
cavity from traumatic perforation of the tympanic membrane or
insertion of a tympanosromy tube. Cholesteatomas tend to
expand progressivelS causing bony resorption, and may extend
intracraniallS with potentially life-threatening consequences.
Cholesteatoma should be suspectedif otoscopy shows a discrete,
whitish opacity of the eardrum or a polyp protruding through a
defect in the eardrum; or white caseousdebris persistently overlies the eardrum, especiallyits superior portion; or persistentmalodorous aural discharge is present. When cholesteatoma is
suspected, consultation with an otolaryngologist should be
sought immediately. Delayed recognition and treatment can have
significant long-term consequences,including the need for more
extensive surgical treatment, permanent hearing loss, facial nerve
injury, labyrinthine damage with loss of balance function, and
intracranial extension. Tympanomastoid surgery is required for
treatment of cholesteatoma.

inner ear. Cholesteatoma or chronic suppurative OM is the usual


source. Symptoms and signs include vertigo, tinnitus, nausea,
vomiting, hearing loss, nystagmus, and clumsiness.Treatment is
directed at the underlying condition and must be undertaken
promptly to preserve inner ear function and prevent the spread
of infection.

INTRACRANIAT
COMPTICATIONS
Meningitis, epidural abscess,subdural abscess,focal encephalitis,
brain abscess,lateral sinus thrombosis (also called sigmoid sinus
thrombosis), and otitic hydrocephalus each may develop as a
complication of acute or chronic middle-ear or mastoid infection, through direct extension, hematogenous spread, or thrombophlebitis. Bony destruction adjacent to the dura often is
involved, and a cholesteatoma may be present, In a child with
middle-ear or mastoid infection, the presence of any systemic
symp-tom, such as fever, headache, or lethargS of extreme degree,
or a finding of meningismus or of any central nervous systemiign
on physical examination should prompt suspicion of an intracranial complication.
'When
an intracranial complication is suspected,lumbar puncture should be performed only after imaging studies establishthat
there is no evidenceof mass effect or hydrocephalus. In addition
to examination of the cerebrospinal fluid, culture of middle ear
exudate obtained via tympanocentesismay identify the causative
organism, thereby helping guide the choice of antimicrobial
drugs, and myringotomy should be performed to permit middleear drainage.

possible presenceof a perilymphatic fistula.


Lateral sinus thrombosis may be complicated by dissemination
of infected thrombi with resultant develbpment of septic infarcts
in various organs. Diagnosis is facilitated through MRI. M"rtoidectomy may be required even in the absenceof osteitis or coalescent mastoiditis, especially in the case of propagation or
embolization of infected thrombi. However, mastoiditis sometimes can be treated with tympanostomy tube placement and

d|9 I otitisMediaI 2645


Ghaprer
intravenous antibiotics. Anticoagulation therapy also may be
considered in the treatment of lateral sinus thrombosis; however,
an otolaryngologist should be consulted before initiating this
therapy to coordinate the possible need for surgical intervention
prior to anticoagulation.
Otitic hydrocephalus, also termed benign intracranial hypertension (pseudotumor cerebri), is an uncommon condition that
consists of increased intracranial pressure without dilatation of
the cerebral ventricles, occurring in association with acute or
chronic OM or mastoiditis (see Chapter 604). The pathogenesis
is uncertain, but the condition commonly is associated with
lateral sinus thrombosis, and the pathophysiology may involve
obstruction by thrombus of intracranial venous drainage into the
neck, producing a rise in cerebral venous pressure and a consequent increase in cerebrospinal fluid pressure. Symptoms are
those related to increased intracranial pressure. Signs may
include, in addition to evidence of OM, paralysis of 1 or both
lateral rectus muscles and papilledema. MRI can confirm the
diagnosis. Treatment measuresinclude the use of antimicrobials
and drugs such as acetazolamide or furosemide to reduce
intracranial pressure,mastoidectomy, repeated lumbar puncture,
lumboperitoneal shunt, and ventriculoperitoneal shunt. If left
untreated, otitic hydrocephalus may result in loss of vision secondary to optic atrophy.

of chronic suppurativeOM, from trauma, or as a result of failure


of closure of the tympanic membranefollowing extrusion of a
tympanostomy tube, A chronic perforation almost always is
amenableto surgicalrepair, usually after the child has beenfree
of OM for an extendedperiod.
Permanentconductivehearingloss may result from any of the
conditions just described. Rarely, permanent sensorineural
hearinglossmay occur in associationwith acuteor chronic OM,
presumablyfrom the spreadof infection or products of inflammation through the round window membrane,or as a consequenceof suppurativelabyrinthitis.

PHYSICAL
SEOUETAE

PREVENTION

The physical sequelae of OM consist of structural middle-ear


abnormalities resulting from long-standing middle-ear inflammation. In most instances,these sequelaeare consequencesof severe
and/or chronic infection, but some also may result from the presumably noninfective inflammation of long-standing OME. The
various sequelae may occur singly, or interrelatedly in various
combinations.
Tympanosclerosis consists of whitish plaques in the tympanic
membrane and nodular deposits in the submucosal layers of the
middle ear. The changes involve hyalinization with deposition of
calcium and phosphate crystals. There may be associated conductive hearing loss, but this is uncommon. In developed countries, probably the most common cause of tympanosclerosis is
rympanostomy tube insertion.
Atelectasis of the tympanic membrane is a descriptive term
applied to either severe retraction of the tympanic membrane
caused by high negative middle-ear pressure or loss of stiffness
and medial prolapse of the membrane, presumably as a consequence of long-standing retraction or severe or chronic inflammation. A retraction oocket is a localized area of atelectasis.
Atelectasis often is transient and usually is not accompanied by
symptoms, but a deep retraction pocket may lead to erosion of
the ossiclesand adhesive otitis, and may serve as the nidus of a
cholesteatoma.For a deep retraction pocket, and for the unusual
instance in which atelectasisis accompanied by symptoms such
as otalgia, tinnitus, or conductive hearing loss, tympanostomy
tube insertion is necessary.
Adhesive OM consists of oroliferation of fibrous tissue in the
middle-ear mucosa, which may. in turn. result in impaired movement of the ossicles, rarefuing osteitis and ossicular discontinuity, conductive hearing loss, and cholesteatoma.The hearing loss
may be amenable to surgical correction.
Cholesterol granuloma is an uncommon condition in which the
tympanic membrane appears to be dark blue, reflecting the presence of thick, granulomatous material in the middle-ear cavity.
The condition appears to result more often from long-standing
OME than from frank middle-ear infection. Tympanostomy tube
insertion alone does not provide satisfactory relief, and the
required treatment is middle ear and mastoid surgery.
Chronic perforation may develop after spontaneous rupture of
the tympanic membrane during an episode of AOM, as sequelae

Generalmeasuresto prevent OM consistof breast-milkfeeding;


avoidance,insofar as possible,of exposureto individualswith
respiratoryinfection;avoidanceof environmentaltobaccosmoke;
vaccination.
and pneumococcal

DEVE[OPMENTAI
SEOUETAE
POSSIBIE
Permanenthearing loss in children has a significant negative
impact on development,particularly delays in speechand languige. The degreeto which OM impactslong-term development
in children is difficult to assess,and there have been conflicting
studies examining this question. However, the developmental
impact is most likely to be significant in children with greater
levils of hearingloss, hearingloss that is sustainedfor longer
periodsof time, and hearingloss that is bilateral,and in those
children who haveother developmentaldifficultiesor risk factors
for developmentaldelay.

IMMUNOPROPHYTAXIS
Heptavalentpneumococcalconiugatevaccinereducesthe overall
number of episodesof AOM by only 6-87", but with a 57%o
reductionin ierotype-specificepisodes.Reductionsof 9:2.3o/oare
seenin children with historiesof frequent episodes,and a 20"/"
reduction is seenin the number of children undergoing rympa-

PROPHYTAXIS
ANTIMICROBIAT

OF
ANDINSERTION
MYRINGOTOMY
TUBES
WMPANOSTOMY
In children with persistent OME, several studies have shown tympanostomy tube insertion to be effective in: reducing their subiequent proportion of time with MEE, improving their hearing

2646I PARTXXIX r The Ear


levels, and also reducing their recurrence rate of AOM. In children with AOM, studies have demonstrated that tympanostomy
tubes are quite effective in reducing the rate of recurrent AOM
in patients. In both OME and AOM, quality-of-life studies have
demonstrated a significant improvement in quality of life in children who have undergone surgical management with tympanostomy tube placement.
How best to manage the individual child who is severely
affected with recurrent AOM musr remain a matrer of individual
judgment and depends on a number of factors, including the
severity of the episodes, risk factor profile, tolerance of antimicrobial therapS hearing assessment,parental preferences,and the
child's overall health and development. Reasonableinterventions
include continued reliance on episodic treatmenr with antimicrobial therapy, watchful wairing in episodesof AOM to attempt to
reduce the overall use of antibiotics, or referral for tympanostomy tube placement.

ADENOIOECTOMY
Adenoidectomy is efficacious ro some exrenr in reducing the risk
of subsequent recurrences of both AOM and OME in children
who have undergone tube insertion and in whom, after extrusion
of tubes, OM continues to be a problem. Efficacy appears to be
independent of adenoid size, and probably derives from removal
of a focus of infection. In younger children with recurrent AOM
who have not previously undergone tube insertion, however, adenoidectomy usually is not recommended along with the tube
insertion, unless significant nasal airway obstruction or recurrent
rhinosinusitis is associated, in which case, performing adenoidectomy might be considered.

Acuin J: Chronic suppurative otitis media. BMJ 2002;325:1159-1160.


American Academy of Family Physicians;American Academy of Otolaryngology-Head and Neck Surgery; American Academy of Pediatrics Subcommittee on Otitis Media With Effusion: Otitis media with effusion. Pediatrics
2004;113:1412-1429.
American Academy of Pediatrics Subcommicee on Management of Acute
Otitis Media: Diagnosis and management of acute otitis media. pediatrics
2004;1,13:1451-1465
.
Arguedas A, Dagan R, PichicheroM, et al: An open-label,double rympanocentesisstudy of levofloxacin therapy in children with, or at high risk for,
recurrent or persistent acute otitis media. Pediatr lnfect Dis J 2005;25:
11 0 2 - 11 0 8 .
Bauchner H, Marchant CD, Bisbee A, et al: Effectiveness of Centers for
Disease Control and Prevention recommendations for outcomes of acute
otitis media. Pediatrics2O06;1.77
:70O9-1077.
Bluestone CD: Otitis media and congenital perilymphatic fistula as a cause of
sensorineuralhearing loss in children. Pediatr lnfect Dis J 1,988;7:5741.
Bodor FF: Systemicantibiotics for treatment of the coniunctivitis-otitis media
syndrome.Pediatr Infect Dis J 1989;8:287-290.
Bodor FF, Marchant CD, Shurin PA, et al: Bacterial etiology of conjunctivitis-otitis media syndrome. Pediatrics 1,985;75:26-28.
Fireman B, Black SB, ShinefieldHR, et al: Impact of the pneumococcalconjugate vaccine on otitis media. Pediatr lnfect Dis 2003;22:1,0-16.
J
Garbutt J, Rosenbloom I, Wu J, et al: Empiric first-line antibiotic rreatment
of acute otitis in the era of the heptavalent pneumococcal conjugate vaccine.
Pediatrics 2006;'1.1.7:e1087-1,094.
Hall-StoodleyL,HuFZ, GieskeA, et al: Direct detectionof bacterialbiofilms
on the middle-ear mucosa of children with chronic otitis media. /AMA
2006;296:202-21L.
Hammaren-Malmi S, Saxen H, Tarkkanen J, et al: Adenoidectomy does not
significantly reduce rhe incidence of otitis media in conjunction with the
insertion of tympanostomy tubes in children who are younger than 4 years:
A randomizedtrial. Pediatrics2005;1 16: 185-1 89.
Heikkinen I Thint M, Chonmaitree T: Prevalence of various respiratory
viruses in the middle ear during acute oritis media. N Engl J Med
'1.999:340:260-264.
Herbert RL, King GE, Bent JP: Tympanostomy tubes and water exposure.
Arch Otolaryngol Head Neck Surg 1998;1,24:1718.

Isaacson G, Rosenfeld RM: Care of the child with tympanostomy tubes: A


visual guide for the pediatrician. Pediatrics 1.994;93:924.
JoseJ, Coatesworth AP, Anthony R, et al: Life-threatening complications after
partially treated mastoids.BMJ 2003;327:4142.
KerschnerJE, Lindstrom DR, PomeranzA, et al: Comparison of caregiver
otitis media risk factor knowledgein suburbanand urban primary careenvironments. lnt J Pediatr Otorbinolaryngol 2005;69:49-56.
Koivunen P, Uhari M, Luotonen J, et al: Adenoidectomy versus chemoprophylaxis and placebo for recurrent acute otitis media in children aged under
2 years: Randomized controlled trial. BMJ 2004;328:487490.
Marchetti F, Ronfani L, Nibali SC, et al: Delayed prescription may reduce the
use of antibiotics for acute otitis media. Arch Pediatr Adolesc Med
2005:1.59:679-684.
McCormick DP, Chonmaitree T, Pittman C, et al: Nonsevere acute otitis
media: A clinical trial comparing outcomes of watchful waiting versus
immediateantibiotic trearmenr.Pediatrics 2005;115 : 1455-1455.
Palmu AAI, Herva E, Savolainen H, et al: Association of clinical signs and
symptoms with bacterial findings in acute otitis media. Clin lnfect Dis
2004138:234-242.
ParadiseJL, Elster BA, Tan L: Evidence in infants with cleft palate that breast
milk protects against otitis media. Pediatrics 1994;94:853-860.
Paradise JL, Feldman HM, Campbell Tl et al: Tympanostomy tubes and
'J.l
developmental outcomes ^t 9 to
years of age.N Engl J Med 2007i356:
248-261.
Rovers MM, Glasziou P, Appelman CL, er al: Antibiotics for acute otitis
media: a meta-analysis with individual patient data. Lancet 2006;368:
1429-1434.
Rovers MM, Schilder AGM, Zielhuis GA, et al: Otitis media. Lancet
2004;363:465473.
SamuelJ, FernandesCMC, SteinbergJL: Intracranial otogenic complications:
A persisting problem. Laryngoscope L986;96:272.
Spiro DM, Tay Kl Arnold DH, et al: Wait-and-seeprescription for the treatment of acute otitis media. JAMA 2006;296:1235-1241Stenstrom R, PlessIB, Bernard P: Hearing thresholds and tympanic membrane
sequelaein children managed medically or surgically for otitis media with
effusion.Arch Pediat AdolescMed 2005;159:1151-1156.
Subcommittee on Management of Acute Otitis Media: Diagnosis and managemenr of acute otitis media. Pediatrics 2004;113:1451-1465.
Tonnaer ELGM, Graamans K, SandersEAM, Curfs JHAJ: Advances in understanding the pathogenesis of pneumococcal otitis media. Pediatr Infect
Dis J 2006;25:546-552.
Valtonen H, Tuomilehto H, Qvarnberg Y, et al: A l4-year prospective followup study of children treated early in life with rympanostomy tubes: Part l:
Clinical outcomes. Arch Otolaryngol Head Neck Surg 2005;137:293-298.
Valtonen H, Tuomilehto H, Qvarnberg I et al: A 14-year prospective followup study of children treated early in life with tympanostomy tubes: Part 2:
Hearing outcomes. Arch Otolaryngol Head Neck Surg 2005;131.:299-303.
Van Heerbeek N, Straetemans M, Wiertsema SB et al: Effect of combined
pneumococcal conjugate and polysaccharidevaccination on recurrent otitis
media with effusion. Pediatrics 2006t 1.17:603-608.
Zapalac JS, Billings KR, Schwade ND, et al: Suppurative complications of
acute otitis media in the era of antibiotic resistance.Arch Otolaryngol Head
N eck Surg 2002;1,28:660-6 6 3.

Genetic factors may affect the anatomy and function of the inner

types of bony dysplasia. All of these can cause both conductive


and sensorineural hearing loss as well as vestibular dysfunction.

Ghapter64ll I TheInnerEarand Diseasesof the BonyLabytinthI 2647


Use of currently available vaccines reduces the risk for bacterial
meningitis and the associatedsensorineuralhearing loss.
VIRUSES,The most common cause of childhood sensorineural
hearing loss (SNHL) is congenital cytomegalovirus (CMV) infection (see Chapter 252). Stabihzation or possibly reversal of the
hearing loss may be possible by using ganciclovir in very young
infants with congenital CMV infection. Other viral causes of
SNHL include congenital rubella as well as acquired mumps,
rubella, rubeola (measles),and fifth disease,caused by parvovirus
B19. Many other viruses also occasionally are associated with
SNHL. In as many as 50% of cases,hearing loss, which usually
is bilateral, although it often is asymmetric, progresses and
worsens over weeks to years.
Before an effective vaccine was introduced, rubella was responsible for as many as 60o/" of casesof childhood SNHL. Vaccination in developed countries has reduced the rate of rubella by
>97%. SimilarlS measlesand mumps are now uncommon causes
of SNHL in the United States becauseof successfulvaccination
programs.
Herpes simplex encephalitis may also be associated with
SNHL, which is more common in children with congenital herpesvirus infection. Acyclovir and other antiviral agents may help
the hearing loss and other central nervous system manifestations
(seeChapter 242).
T0X0P[ASM0SIS. Toxoplasma gondii is a protozoan that
may cause congenital SNHL. In the United States, about 3,000
children are born each yr with congenital toxoplasmosis,
and approximately 25o/" of untreated patients have SNHL.
If maternal infection is documented during the fetal period,
medical therapy may be able to prevent some of the clinical
manifestations, including SNHL oJ the offspring (see Chapter
287).
BACTERIAL
MENfNGITIS.Since the Haemophilus influenzae type b
vaccine was introduced, Streptococcuspneumoniae and Nelsseria meningitidis have become the leading causes of bacterial
meningitis in children in the United States. Hearing loss occurs
more commonly with S. pneumoniae, with an estimated incidence
of 15-207". Approximately 60% of the associatedhearing loss
is bilateral, although it often is asymmetric. If hearing loss is
present at the time of presentation with meningitis, and especially
if it is severeto profound, the likelihood of significant improvement is low. However, if the hearing loss develops after admission for treatment and is not severe,stabilizatton or improvement
is possible. Late progression of SNHL also has been noted in
some children years after meningitis. In the United States and
many other developed countries, bacterial meningitis is one of the
major causesof profound deafnessleading to cochlear implantation in children. The introduction of pneumococcal conjugate
vaccine is expected to lead to a reduction in SNHL due to pneumococcal meningitis.
Studieshave shown favorable trends in the course and outcome
after administration of dexamethasonefor hearing loss and other
neurologic deficits associated with bacterial meningitis (see
Chapter 602.I), although its effectiveness,especially lor S. pnewmoniae and N. meningitidis meningitis, generally has not reached
statistical significance because of the small number of cases in
the trials. A meta-analysis of 1.1 studies conducted from 1988
to L996 showed that dexamethasone reduced severe hearing
loss associated with H. influenzae type b meningitis regardless
of the timing of administration of dexamethasone (before or
with antibiotics vs. later) or of the antibiotic used. For Dneumo-

coccal meningitis, the meta-analysis showed a benefit of dexamethasone only when given early and only for protection against
severe hearing loss, Too few cases of meningococcal meningitis
were included to assessthe effect of dexamethasone on this
entlty.
SYPHftlS. Congenital syphilis, caused by Treponema pallidwm,
may cause SNHL in 3-38% of affected children (see Chapter
215). The exact incidence is difficult to ascertain, because the
hearing loss may not develop until adolescenceor even adulthood. When the condition is identified, treatment with antibiotics
and corticosteroids may improve the hearing loss.
0F THE lt{NEREAR.Labyrinthitis may be a com0THERDISEASES
plication of direct spread of infection from acute or chronic otitis
media (OM) or mastoiditis and also may complicate bacterial
meningitis as a result of organisms entering the labyrinth through
the internal auditory meatus, endolymphatic duct' perilymphatic
duct, vascular channels, or hematogenous spread. Clinical manifestations of labyrinthitis may include vertigo, dysequilibrium,
deep-seatedear pain, nausea, vomiting, nystagmus, and SNHL.
Acute suppurative labyrinthitis, charactetized by abrupt, severe
onset of these symptoms, requires intensive antimicrobial
therapy. If it is secondary to OM, otologic surBery may be
requiied to remove underlying cholesteatomaor drain the middle
ear and mastoid, in addition to antibiotics. Acute serous
labyrinthitis, with milder symptoms of vertigo and hearing loss,
may develop secondary to middle-ear infection as well. It usually
responds well to antibiotics and corticosteroids, with improvement in both vertigo and hearing. Chronic labyrinthitis, most
commonly associatedwith cholesteatoma, presents with SNHL
and vestibular dysfunction that develops over time; surgery is
required to remove the cholesteatoma.Chronic labyrinthitis may
alsb occur uncommonly secondary to long-standing OM' with
the slow development of SNHL, usually starting in the higher frequencies,and possibly with vestibular dysfunction' AdditionallS
jnd more commonly, children with chronic middle-ear fluid often
are unsteady or off balance, a situation that improves immediately when the fluid resolves.
Otosclerosis. an autosomal dominant diseasethat affects only

stapeswith a mobile prosthesis often is successful.


Osteogenesisimperfecta (OI) is a systemic disease that may
involve both the middle and inner ears (see Chapter 699).
Hearing loss occurs in about 207" of yotngchildren and as many
as 90"/" of adults with this disease.The hearing loss most com-

progresslve.
Osteopetrosis, a very uncommon skeletal dysplasia' may
involve the temporal bone, including the middle ear and ossicles'
resulting in a moderate to severe,usually conductive hearing loss.
Recurrent facial nerve paralysis also may occur as a result of
excessbone deposition; with each recurrence' less facial function
may return (see ChaPter 597).

2648

PART XXlX m The Ear

---dim
Joseph Maddad Jr;

I
I I

!&L&iL.

"'I-"

AURICLE AND EXTERNAL AUDITORY CANAL. Auricle trauma is


common in certain sports, and quick drainage of a hematoma can
prevent irreversible damage. Hematoma, with accumulation of
blood 17rt\\rrn the p c r ~ ~ h t r n c i n u,ind
n ~ r1-1~s,~rrll,lpe,m,l\ ~ollon
trdurn.1 rr, the plnna .ir~dIr r\prclall\ cclrnlnorl In teenagers r e l ~ t c d
r o rvrrctlrn~o r l>o\~rig.Imrnce11,irc nccdle d\plr,itlon 01. t 4 hcn t h r
r\~
r or r c ~ u r r c n t .I I I L I ~ I O I I~ n e di r c ~ ~ r ~.lnJ
. i ~ GIc
l l c ~ r ~ ~ ~I \ r c\ri
o r ~ni\ ~
prr5wrc J r c c \ ~ n g.lrr I i r ~ c s s a r \t i , prr\cclt prr~chondrrtrs,\vhrih

can result in cartilage loss and a cauliflower ear deformity. Sports


helmets should be warn when appropriate during activities when
head trauma is possible.
Frostbite of the auricle should be managed
rewarm- by. rapidly
ing the exposed pinna with warm irrigation or warm compresses.
Foreign bodies in the external canal are common in childhood.
These &n often be removed in the office setting without general
anesthesia if the child is mature enough to understand and coopcr.lr~*. ~ r ~ cI \l yrrrpcrlr r c \ r r . i ~ n r ~llt; .ln aclrcl~l,ltrI~raJlrghr.\ur
~ng
g~L.ilti~"1cio r o \ ~ o p c .t)r {rr(lmicro\copc I \ u\cd tor \ ~ \ u . ~ l ~ rthe
rrlycct: drlJ ~t .lpproprrdte in\trurnrnr\. \uch '19 .~llrgdtort o r ~ e p .
W I ~ CI r r ~ p , o r .I I ~ I U I I I icrurn'n
iurcirc, o r \ u ~ t i o nare I I ~ J ,
JcperrJlng tin thc \h.lpe c > t thc ohlcct. Grnrlr IrrlKatlon o t the car
i . l t ~ a l w ~ r ht x d r ti~niprrcrturctr..lrcr ,)r s.lllrlr 111,1\. 1 ) ~11srd to
rernovr ver! small Ohlci~s.I l ~ r to r ~ l v~t r h ~r>lnpanr; rnernhranc
(T.fli I, I I I ~ A L ;-lttcni~itr~f
~ .
r c ~ ~ ~ o \ot
. i l ol31cct tro111 rrr~lggling
and ~n.iilcqi~dtc
r11ols result< 111.I
ihrlci o r tv~tllpoor vi.;u,~lizar~on
t c r r ~ t i cc~ljl ~ l ~\ vl ~ t h.I ~\r.(~~Ileri
JIIJhlcecl~ngw r c;111;11J I ~nlLI\J then
rn.lndarr ~ ~ t l r .111c\tlic-,i~
r ~ l
tor rerntw.11 ot tlir ot>rrit. I~rtlicult
t o r c ~ g nI?crd~r\,e\prLr.lll\ rl~cr\rth.lt .Ire I~rgt,,derpl\ crnl-reclrlc~i

vertigo, nystagmus, severe tinnitus, moderate to severe hearing


loss, or cerebrospinal fluid (CSF) otorrhea. At the time of exploration, it is necessary to inspect the ossicles, especially the stapes,
because they may have been dislocated or fractured; examination
also is made for sharp objects that may have penetrated the oval
or round windows. Sensorineural hearing loss (SNHL) results if
the stapes subluxates or dislocates into the oval window, or if
either the oval or round window is penetrated. Children should
not be given access to cotton-tipped applicators, as they commonly cause ear trauma. Contact with small objects should be
limited to times of parental supervision.
Perilymphatic fistula (PLF) may occur after sudden barotrauma
or an increase in CSF pressure. It should be suspected in a child
who develops a sudden SNHL or vertigo after physical exertion,
deep water diving, air travel, playing a wind instrument, or significant head trauma. The leak characteristically is at the oval or
the round window and may be associated with congenital abnormalities of these structures or an anatomic abnormality of the
cochlea or semicircular canals. PLFs occasionally close spontaneously, but immediate surgical repair of the fistula is recommended to control vertigo and to stop any progression of the
SNHL; even timely surgery does not usually restore the SNHL.
No reliable test is known for PLF, so middle-ear exploration is
required for diagnosis and treatment.

TEMPORAL BONE FRACTURES. Children are particularly prone to


basilar skull fractures, which usually involve the temporal bone.
Temporal bone trauma should be considered in head injuries, and
the status of the ear and hearing should be evaluated. From
70-80% of temporal bone fractures are longitudinal and are
commonly manifested by bleeding from a laceration of the external canal or tympanic membrane; postauricular ecchymosis
(Battle sign); hemotympanum (blood behind an intact TM); CHL
resulting from TM perforation, hemotympanum, or ossicular
injury; delayed onset of facial paralysis (which usually improves
spontaneously); and temporary CSF otorrhea or rhinorrhea (from
or associated with canal swelling, are best removed under general
CSF running down the eustachian tube). Transverse fractures of
anesthesia. Disk batteries are removed emergently because they
the temporal bone have a graver prognosis than longitudinal fracleach a bas~cfluid that can cause severe tissue destruction. Insects
tures and are often associated with immediate facial paralysis.
O I I o r I I C ~ O L J I I ~ ~ . '111d J r e
111 ~ 1 1 ~ 21n.11 i r r tlr\r L~llcci13 IIII r11111cr~l
Facial paralysis may improve if caused by edema, but surgical
the11 rcrncncrl u n ~ l e rotornlcros~rrlxcc\Amln.ltion
decompression of the nerve is often recommended, if there is no
.After ,i trrrClcrl h(rJ\ I,rcr1lcnc.J trom thc r\rcrn.ll i,tn.~l. the
evidence of clinical recovery and facial nerve studies are unfa7 1 1 \lioulil 11~.r.lrr.ti~llr ~ n c ~ c . ~tor
t r dP ~ ) \ \ I I > I L rr,turn,ltl~nrrtoravorable. If the facial nerve has been transected, surgical decomtion rlr tor ,I prc-rk\lrilng n~rJ~tiz-r.irctturron. I t .I toretgrl I r ~ ) d \
pression and anastomosis offer the possibility of some functional
IIJ\ rr\ulterl 111 ,\cute ~r~tl.imrn,itloriof t h r i ~ n a l .rrc,lrlncrlt ,IS
recovery. Transverse fractures are also associated with severe
described for acute external otitis should be instituted (see
SNHL, vertigo, nystagmus, tinnitus, nausea, and vomiting assoChapter 638).
ciated with loss of cochlear and vestibular function; hemotympanum; rarely, external canal bleeding; and CSF otorrhea, either
M A f l l C -AhlD
m.T r ~ u r n a t l cpzrtoratlon in the external auditory canal or behind the TM, which may exit
the nose via the eustachian tube.
o f the TJ.1 I I S L I . ~ ~ occur.;
~!
nr J rr.,~111ot .I c~~clilrri
c s t e r n ~ li o m If temporal bone fracture is suspected or seen on radiographs,
o r ~.I t o r e l g ~ohleit 5uih
presslon, <uch ns .I >lLiP,o r p c ~ i i . r r . ~ t ~hv
gentle examination of the pinna and ear canal is indicated;
as n \ r ~ c ko r ccrtrtrri-r~ppeci .~pplli,lr~rr.
Ttir pcrforarion 111;1y he
lacerations or avulsion of soft tissue is common with temporal
lincar o r s r c l i ~ r r .It 1 % rilo.;t t ~ - e q ~ l c ~ lItIII y[he dntZrIOr portroll ot
bone fractures. Vigorous removal of external auditory canal
thr.
tellhri wherl 11 1 5 c.1~1srr1h\. ir)lnpre\>lon. . I I I ~ ma!- hc In
blood clots or tympanocentesis is not indicated, because clot
any q t ~ ~ i l r o
a t~ rhr
~ r T1.I wht'r~i . l i ~ \ < d17)
iorcign ohiecr. S y s removal may further dislodge the ossicles or reopen CSF leaks.
rcniic ,rnrrh~otli\ a n d toplc31 orli I I ~ ~ I C . ~ I I ~.Ire
I > \iior requlrrd
The effectiveness of prophylactic antibiotics to prevent meningitis
I \ p r c i e ~ ~7~r.1urn.irli
t.
T h l pcrtor,lutilcrs < i l p p ~ ~ r a r i votcrrr11c.l
c
in patients with basilar skull fractures and CSF otorrhea or rhi\ , 11 I \ 1tl1porta1it t o rt-.aI~r,~tc
:~rl~f
rions o t v e ~IICJI
~ ~ ~ I ~ I I ~ J I I ~ O L I \ I Iiilr
rnonitnr rhc p.ltrrnt'\ h c . ~ r ~ r ito
g cn.;urC r l i n r sponr,irlrous he;il~rig norrhea cannot be determined because studies to date are flawed
occur\. li rhc 1-hl J o e \ riclt Iie.11 \ z ~ r h i n\e\.rr,ll rilorlrhs. \ ~ ~ r c r c a l by biases. If a patient is afebrile and the drainage is not cloudy,
watchful waiting without antibiotics is indicated. Surgical interis
graft rcp;irr s h o t ~ l dhe i o n i ~ d i - r e d .A s long ,I\ thi* pcrior.~t~trn
vention is reserved for children who require repair of a nonhealpre>rnt, orc~rrlrr~l
rn;i\ occur trom \v.lrcr C I I I C ~ tht'
I ~ ~mrcldlc e,lr
ing T M perforation, who have suffered dislocation of the
lr
' ~ L V I I I I I ~o~r II7,lrhing:
I~~
trom the car ;arl.~l, tvh~clii L i I l c ~ i c ~~111r11ig
re\t~ltrng ossicular chain, or who need decompression of the facial nerve.
.~pproprlLitrprecdL1tlons , I I < I L I I ~ Iw tLi!ic~i.I'eric)r~t1011<
SNHL can also follow a blow to the head without an obvious
n
frtrln p . , ~ ~ c r r a t ~f~oirgr l q l h o ~ l l c \,lrr less 11kel\ r r j hcnl t h ~ ~thaw
fracture of the temporal bone (labyrinthine concussion).
c a i ~ s e dby cclrnprcssioti. , A i ~ J ~ o r n c ter r~ac~ u i n a t i c ~re{-c~ls
n
J i011ductive hearing loss (CHL), with larger air-bone gaps seen in
At!tfmG''tffAtllWA. This results from exposure to high-intensity
larger perforations. Immediate surgical exploration is indicated if
5ounJ (tirexvnrLs, gunfire, rock music, heavy machinery) and is
the injury is accompanied by one or more of the following:
LI

L~

Chapter642I Tumorsof the EarandTemporalBone I 2609


initially manifested by a temporary decreasein hearing threshold,
most commonly at 4,000 Hz on an audiometric examination, and
tinnitus. If the sound is between 85-140 dB. the loss is usually
temporary (after a rock concert), but both the hearing loss and
the tinnitus may become permanent with chronic noise exposure;
the frequencies from 3,000 to 5,000 Hz are most often involved.
Sudden, extremely loud (>140 dB), short-duration noises with
loud peak components (gunfire, bombs) may cause permanenr
hearing loss after a single exposure. Ear protection and avoidance of chronic exposure to loud noise are preventive measures.
Hearing loss due to chronic noise exposure should be entirely
preventable. Parents should be made aware of the dangers of
acoustic trauma, from the environment and from the use of headphones, and should take measuresto minimize exposure.

Hough JVD, Stuart WD: Middle ear iniuries in skull trauma. Laryngoscope
1968; 78:899-937.

Suspicion for tumors of the ear and temporal bone may be raised
by the appearanceof a mass in the middle ear or mastoid, as well
as signs of bleeding or local destruction. Imaging with CT or MRI
allows for biopsy guidance to confirm the diagnosis and establish a treatment regimen.
Benign tumors of the external canal include osteomas and
monostotic and polyostotic fibrous dysplasia. Osteomas present

as bony masses in the canal and require removal only if hearing


is impaired or external otitis results; osteomas may be confused
clinically with exostoses(see Chapter 501'.2).
Eosinophilic granuloma, which may occur in isolation or as
part of the systemic Langerhans cell histiocytosis (see Chapter
507), should be suspected in patients with otalgia, otorrhea
(sometimes bloody), hearing loss, abnormal tissue within the
middle ear or ear canal, and roentgenographic findings of a
sharply delineated destructive lesion of the temporal bone. Definitive diagnosis is made by biopsy. Treatment depends on the site
of the lesion and histology. Depending on the site, it may be
treated by surgical excision, curettage, or local radiation. If the
lesion is part of a systemic presentation of Langerhans' cell histiocytosis, chemotherapy in addition to local therapy (surgery
with or without radiation) is indicated. Long-term follow-up is
necessary whether the temporal bone lesion is a single isolated
lesion or part of a multisystem disease.
Symptoms and signs of rhabdomyosarcoma originating in the
middle ear or ear canal include a mass or polyp in the middle ear
or ear canal, bleeding from the ear, otorrhea, otalgia, facial paralysis, and hearing loss. Other cranial nerves also may be
involved. Diagnosis is based on biopsS but the extent of disease
is determined by both CT and MRI of the temporal bone, skull
base, and brain. Management usually involves a combination of
chemotherapS radiation, and surgery (see Chapter 501).
Non-Hodgkin lymphoma and leukemia also may Present rarely
in the temporal bone. Although primary neoplasms of the middle
e^r ^re very uncommon in children, they include adenoid cystic
carcinoma, adenocarcinoma, and squamous cell carcinoma.

is needed for diagnosing these tumors early.