Congenital Aural Atresia

Elizabeth J. Rosen, MD
Arun K. Gadre, MD
1/8/03
 Congenital Aural Atresia
 Embryology
 Classification
 Evaluation
 Surgical Repair
 Results
 Complications
 Controversies
 Congenital Aural Atresia
 Definition: “a birth defect that is
characterized by hypoplasia of the external
auditory canal, often in association with
dysmorphic features of the auricle, middle
ear and, occasionally, the inner ear
structures”
--Harold F.
Schuknecht, 1989
 Congenital Aural Atresia
 Incidence: 1 in 10,000-20,000
 Unilateral 3-5x more common than
Bilateral
 Males > Females
 Right > Left
 Inheritance—sporadic
 Autosomal recessive or dominant
 Congenital Aural Atresia
 Associations  Syndromes
– Hydrocephalus – Treacher-Collins
– Goldenhar’s
– Posterior cranial
– Crouzon’s
hypoplasia
– Mobius’
– Hemifacial microsomia
– Klippel-Feil
– Cleft palate – Fanconi’s
– GU anomalies – DiGeorge
– Pierre Robin
– VATER
– CHARGE
 Embryology
 Auricle
– 4th week of gestation
– 1st and 2nd branchial
arches
– Hillocks of His
 1—tragus
 2—helical crus
 3—helix
 4—antihelix
 5—antitragus
 6—lobule
 Embryology
 External Auditory
Canal
– 8th week of gestation
– 1st branchial groove
– Medial migration of a
solid core of epithelial
cells
– Recanalization during
6-7th months of
gestation
 Embryology
 Ossicles
– 4th week of gestation
– Meckel’s cartilage
 Malleus head and neck
 Incus body and short
process
– Reichert’s cartilage
 Malleus handle
 Incus long process
 Stapes suprastructure
 Embryology
 Labyrinth
– 3rd week of gestation
– Invagination of otic
placode to form otic
vesicle
– Semicircular canals—
6th week
– Utricle and Saccule—
8th week
– Cochlea—7-12th weeks
 Embryology
 Facial Nerve
– 4-5 weeks of gestation
 Classification
 Altmann’s
– Grade I
 Hypoplastic EAC,
temporal bone, TM;
normal or slightly
hypoplastic middle ear
cleft; normal or slightly
deformed ossicles
– Grade II
 Absent EAC; small middle
ear cleft; osseous atresia
plate; fixed and
malformed ossicles
– Grade III
 Absent EAC; markedly
hypoplastic or absent
middle ear cleft; absent or
severely deformed ossicles
 De la Cruz
– Minor
 Normal mastoid
pneumatization
 Normal oval window
 Reasonable oval window-
facial nerve relationship
 Normal inner ear
– Major
 Poor pneumatization
 Absent or abnormal oval
window
 Abnormal horizontal facial
nerve
 Anomalous inner ear
 Classification
 Schuknecht
 Type A  Type C

 Type B  Type D
 Classification
 Jahrsdoerfer, 1992
– Based on HRCT
temporal bone findings
– Score correlates to
likelihood of
successful surgery
 Evaluation
 History
– Details of pregnancy
– Family history
 Physical Examination
– Microtia
– Severity of EAC stenosis
– Craniofacial development
 Evaluation
 Audiologic Evaluation—ABR before
leaving the hospital
– Unilateral atresia
 Auditory function of the “normal” ear

– Bilateral atresia
 Establish presence of cochlear function
 FIT WITH BONE CONDUCTION AID
 Evaluation
 High Resolution CT Temporal Bone
– Age 5-6 years
– Axial and Coronal
– Evaluate
 Middle ear and mastoid pneumatization

 Anatomy of ossicles

 Inner ear morphology

 Course of facial nerve

 Surgical Repair
 Candidacy
– ABSOLUTE REQUIREMENTS
 1. Normal inner ear

 2. Normal cochlear function

– HRCT Score
 </= 5/10: poor

 6/10: marginal

 7/10: fair

 8/10: good

 9/10: very good

 10/10: excellent
 Surgical Repair
 Timing
– Microtia repair should be performed prior to
undertaking atresia repair
– 5-6 years of age
– Controversy:
 Between Stages 2 and 3 of microtia repair
 2 months after completion of microtia repair
 Surgical Repair
 Transmastoid Approach
– Infrequently used
– Advantages:
 More familiar approach
 Identification of sinodural angle and lateral SCC as

landmarks
– Disadvantages:
 Creation of mastoid cavity

– Larger defect to be skin grafted

– Prolonged healing
– Lifelong maintenance
 Surgical Repair
 Anterior Approach
– Popularized by Jahrsdoerfer, most frequently
utilized approach
– Advantage:
 Avoidance of mastoid cavity
– Disadvantages:
 Unfamiliar approach

 Lack of landmarks
 Surgical Repair
 Video
– American Academy of Otolaryngology—Head
and Neck Surgery Foundation
 Congenital Disorders—Volume #4
 Harold F. Schuknecht, MD
 Results
 Difficult to interpret
– Different classification
of atresia
– Different criteria for
surgical candidacy
– Different definition of
“successful” outcome
– Different periods of
follow-up
 Results
 Stability of Hearing Levels
– Lambert, 1998
– Early postoperative period (<1yr)
 60% 25dB or better
 70% 30dB or better

– Prolonged follow-up (1-7.5yrs)

 46% 25dB or better

 50% 30dB or better

 Complications
 EAC restenosis
– Highly variable: 8-50%
– Correlation to severity of atresia
 TM lateralization
– 5-26% of cases
– Easier to prevent than to correct
 Chronic infection
– Reconstructed EAC lacks normal keratin
migration and cerumen production
– Create wide meatus, fix restenosis, frequent
follow-up with canal debridement
 Complications
 Facial Nerve Injury
– 1.0-1.5%
– Vulnerability
 Skin incision

 Dissecting in the glenoid fossa

 During canalplasty

 Transposing the nerve

 Dissecting preauricular soft tissue

– Prevention
 Preoperative evaluation of HRCT

 Intraoperative facial nerve monitoring

 Complications
 Sensorineural Hearing Loss
– Up to 15% of cases
– 4,000-8,000 Hz
– Acoustic trauma to the inner ear
 Transmission of drill energy

 Drill injury to ossicles

 Manipulation of ossicular chain

– Avoidance—Meticulous technique
 Controversies
 Surgical Repair of Unilateral Atresia
– Historically
 One hearing ear = normal speech and language

development
 No indication for surgery

– Recently
 Unilateral hearing loss = auditory, linguistic and

cognitive deficits
 Improved preop evaluation, patient selection,

surgical techniques, predictable results

 Surgery indicated
 Controversies
 Timing of Unilateral Repair
 Conclusion
 Complex and Challenging Problem
 Goals:
– Restore functional hearing
– Construct patent and infection-free EAC
 Rewarding Surgery
Bibliography

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Philadelphia; 1994.
•Kamerer, DB. Congenital and Acquired Atresia of the External Auditory Canal. In, Operative Otolaryngology, Head and Neck Surgery,
E.N.Meyers, Ed. W.B. Saunders, Philadelphia; 1997.
•Schuknecht, HF. Congenital Aural Atresia. Laryngoscope, 99; Sept 1989: 908-917.
•Lambert, PR. Congenital Aural Atresia. In, Head & Neck Surgery—Otolaryngology, 2nd Ed, B.J.Bailey, Ed. Lippincott-Raven, Philadelphia;
1998.
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