Académique Documents
Professionnel Documents
Culture Documents
By
Dr. T. Balasubramanian M.S. D.L.O.
Histology:
Histologically, cholesteatoma appears as a benign keratinizing squamous cell cyst
made up of three components:
1. Cystic content
2. Matrix
3. Perimatrix
Classification:
Cholesteatoma can be classified according to their presumed etiology into:
Congenital cholesteatoma and Acquired cholesteatoma.
Acquired cholesteatoma: As the name itself suggests this condition is acquired in
nature. It can be further sub classified into:
1. Primary acquired cholesteatoma
2. Secondary acquired cholesteatoma
Primary acquired cholesteatoma: In this condition there is no history of preexisting
or previous episodes of otitis media or perforation. Lesions just arise from the attic
region of the middle ear.
Pathophysiology:
5 theories have been proposed to explain the pathophysiology of acquired
cholesteatoma.
1. Cawthrone theory: This theory suggested by Cawthrone in 1963 suggested
that cholesteatoma always originated from congenital embryonic cell rests
present in various areas of the temporal bone.
2. Tumarkins theory: Tumarkin was of the view that cholesteatoma was
derived by immigration of squamous epithelium from the deep portion of the
external auditory canal into the middle ear cleft through a marginal
perforation or a total perforation of the ear drum as seen in acute necrotizing
otitis media (secondary acquired cholesteatoma).
3. Toss theory of invagination: Toss theorized that persistent negative pressure
in the attic region causes invagination of pars flaccida causing a retraction
pocket. This retraction pocket becomes later filled with desquamated
epithelial debris which forms a nidus for the infection to occur later.
Common organisms known to infect this keratin debris are Psuedomonas, E.
coli, B. Proteus etc.
4. Metaplasia: This theory was first suggested by Wendt in 1873. He took into
consideration the histological changes seen in various portions of the middle
ear cavity. The attic area of the middle ear cavity is lined by pavement type
of epithelium. This epithelium undergoes metaplastic changes in response to
subclinical infection. This metaplastic mucosa is squamous in nature there by
forming a nidus for cholesteatoma formation in the attic region.
5. Habermanns epithelial invasion theory: This theory suggests that following
perforation of the Sharpnells membrane, epithelium invades into the attic
area.
Secondary acquired cholesteatoma: This type always follows active middle ear
infection which manages to destroy the ear drum along with the annulus. The
destruction of annulus predisposes to epithelial migration from the external
auditory canal into the attic region. This type of destruction is common in acute
necrotizing otitis media following exanthematous fevers like measles etc.
Etiology: Acute necrotizing otitis media occurs commonly in infants and young
children who are seriously ill due to scarlet fever, measles, pneumonia and
influenza.
Pathology: Essential pathology of acute necrotizing otitis media is true necrosis of
ear drum tissue along with its annulus. Usually these lesions are commonly caused
due to the virulence of the organisms involved i.e. beta-hemolytic streptococci.
Necrosis starts to occur in those areas of ear drum which have the poorest blood
supply. The first area to suffer is the central kidney shaped area of pars tensa. The
necrotic tissue sloughs out and is not replaced by normal tissue.
Symptoms:
Symptoms of acute necrotizing otitis media include:
Evaluation:
History: Careful otological history should be elicited.
Common presenting complaints are:
a.
b.
c.
d.
e.
f.
g.
h.
Hearing loss
Otorrhoea (foul smelling)
Otalgia
Nasal obstruction
Tinnitus
Vertigo
Previous h/o middle ear disease
Progressive unilateral deafness with foul smelling discharge should always
evoke suspicion of the disorder
Physical examination:
a.
b.
c.
d.
e.
Audiological evaluation:
Pure tone audiometry will commonly manifest with conductive deafness. If
conductive deafness is more than 60 dB levels, then ossicular disruption / incus
necrosis should be suspected.
Role of imaging:
CT scan of temporal bone will not only help in identification of the extent of
lesion, but also will help in diagnosing intracranial extension.
Congenital cholesteatoma:
Congenital cholesteatoma is defined as pearly white mass behind an intact ear
drum, in the absence of history of otitis, Otorrhoea, ear drum perforation or other
otological procedures.
Derlakis criteria for diagnosis of congenital cholesteatoma:
1. No history of middle ear infections
2. No history of ear discharge
3. Presence of cholesteatomatous mass behind a intact ear drum
Levenson in 1986 excluded history of middle ear infections from the above criteria
as congenital cholesteatoma can commonly involve and perforate the ear drum.
Incidence of congenital cholesteatoma ranges from 0.2 0.25 per 100,000
population.
Pathogenesis: Various theories have been proposed to explain the pathogenesis of
congenital cholesteatoma.
Teeds epithelial cell rest theory: Teed in 1936 explained that the persistence of
squamous epithelial cell rests in the temporal bone lead to the formation of
congenital cholesteatoma.
Implantation theory: Friedberg observed viable squamous epithelial cells in the
amniotic fluid present in the middle ears of neonates and hypothesized that this was
a possible source of congenital cholesteatoma.
Ruedi's invagination theory: This theory suggests that in utero infection of
tympanic membrane causes invagination of ear drum into the middle ear cavity
causing congenital cholesteatoma.
Epithelial migration theory: This theory was proposed by Aimi. He suggested that
ectoderm from the embryonic external auditory canal overcomes a hypothetical
restrictive mechanism of the tympanic ring and migrates into the middle ear.
Epidermoid formation theory of Michael: Michael observed nests of squamous
epithelium which could occasionally keratinize in the lateral wall of the embryonic
tympanic cavity below the level of pars flaccida. These nests of squamous
epithelium usually involute. Failure of this involution process could be a cause for
congenital cholesteatoma.
Clinical features:
1. Painless Otorrhoea (foul smelling). Pain may be present when associated
with otitis externa.
2. Hearing loss
3. Giddiness may be present when labyrinth is eroded. Commonly it is the
lateral semicircular canal that is involved.
4. Attic perforation may be seen / Attic retraction pocket may be seen.
5. Granulation tissue may be present in the attic region.
6. Complications of cholesteatoma may cause intense headache / spiking fever
7. Fistula test may be positive if labyrinth is involved.
Evaluation:
Tuning fork tests will demonstrate commonly conductive deafness. In rare cases
the hearing thresholds may even be normal due to bridging effect of cholesteatoma.
Mixed loss / sensori neural hearing loss may be present if labyrinth is involved.
Pure tone audiometric findings will more or less coincide with that of tuning fork
tests.
CT scan of temporal bone should be performed in the presence of intra cranial
complications.
Gradual deepening of the skin incision exposes the periosteum. This is stripped
away from the mastoid cortex using a sharp periosteal elevator. A post auricular
skin flap is raised, and is pushed anteriorly to be held in place by a roller gauze tied
through it. Now the external auditory canal, ear drum and the mastoid cortex
becomes visible in the same view.
Cutting burrs are used to drill out the cortical bone from the mastoid cortex. Two
incisions are made. One horizontal and one vertical. The horizontal cut is made just
below the supra mastoid crest. This starts from the anterior portion of the
Maceven's triangle extending posteriorly up to the sino dural angle. This line
approximately indicates the level of dura and hence dissection should not go above
this line. The second vertical cut is made along the external auditory canal starting
from the Maceven's triangle up to the mastoid tip.
MacEven's triangle: is the surface marking for mastoid antrum in adults. The
antrum lies about 1.5 cm below this triangle.
Antrum is entered by drilling the mastoid cortex. The antral and periantral air cells
are exenterated. The aditus is identified. It is widened in the anterosuperior
direction. It should not be widened in an inferior direction because the incus could
become dislodged. After the aditus is widened, the posterior meatal wall is thinned
out.
The middle ear is cleared off the infective material and oedematous mucosa after
elevation of tympanomeatal flap. Ossicular chain is checked for functional
continuity. If the incus is necrosed, ossicular prosthesis is introduced.
Wound is closed in layers.
Complications of cholesteatoma:
1.
2.
3.
4.
5.