CONGENITAL AURAL

ATRESIA

Dennis R. Maceri, M.D., F.A.C.S.

Associate Professor
Otolaryngology- Head and Neck Surgery
Keck School of Medicine
 “ The better developed the
auricle the better the middle
ear”
Kountakis, 1995
 Atresia
Epidemiology
 1 in 10,000 to 15,000 births
 Up to 50% of the time associated with some
craniofacial syndrome
 Unilateral : Bilateral, about 3:1
 30% are bilateral
 Atresia : Microtia, 7:1
 Slightly more common on the right
 Male : Female, 2:1
 Atresia
Aural atresia associated with 22/71 known
craniofacial syndromes
– Treacher Collins (Mandibulofacial Dysostosis)
– Nager Syndrome (Acrofacial Dysostosis)
– Cruzoun’s Craniofacial Dysostosis
– Goldenhar’s Syndrome
– Hemifacial Microsomia
 Aresia Surgery
 First attempt to surgically correct aural
atresia was by Thomson in 1843
 Shambaugh, 1967, recommended
unilateral surgery only if the cochlear
reserve allowed hearing to improve by
25dB
 Jahrsdoerfer, 1978, first large series using
the anterior approach
Embryology
8 Week Stage
Embryology
8 Week Stage
 Embryology
 The external ear forms earlier than the
middle ear
 Microtia and Atresia imply an arrest in
development at any stage
 The earlier the arrest, the less developed
the middle ear
 Embryology
 Inner ear and labyrinth develop at 3
weeks from an invagination of ectoderm
called the ottic placode
 Middle ear space, mastoid, eustachian
tube and part of the drum develop from
the first pharyngeal pouch at 30 weeks
 Mastoid air cells continue to develop up to
age 5
 Embryology
 First Branchial Arch-(Meckel’s)
– Malleus head, body, tensor tympani (V3)
– Short process of the Incus
 Second Arch (Hyoid) (Reicherts)
– Manubrium of the malleus
– Stapes superstructure
– VIIth nerve
 Foot plate from the otic capsule and 2nd
arch
 Embryology
 External ear (ectoderm) is derived from the 1st
branchial cleft. A solid core of epithelium
migrates inward toward the 1st branchial pouch
(endoderm)
 Embryology
Solid core of
tissue migrates
inward

External canal
re-canalizes by
the 6th month
 Embryology
 The external canal starts to hollow out
(recanalize) during the 6th month and
progresses from medial to lateral
 Arrest of recanalization process leads to
the various deformities seen in atresia
 Formed tympanic membrane and bony
canal with a stenotic membranous canal
leads to canal cholesteatoma
 Embryology
 Arrested growth of the ossicles leaves
them in various stages of formation
– Stapes often malformed but mobile
– The incus and malleus are fused
– Altered course of facial nerve
 Atresia
Embryology – 7th Month
 Canalization complete
 Mastoid separation from mandible

Normal No mastoid
posterior-inferior growth
growth

Normal Atresia
 Atresia
Embryology
 Mastoid growth affects the facial nerve
position

Normal 120o Acute Curve in

Curve Atresia
Atresia
Classification of Deformities
After Colman-3 types
 Minor Aplasia-incomplete recanalization

 Moderate Aplasia- the tympanic bone has

developed but has failed to recanalize

 Severe Aplasia-complete absence of the

external canal
 Atresia
Classification-Severe Aplasia, no tympanic
bone
 Atresia
Complete Atresia
 Atresia
Complete Atresia
 Atresia
Moderate Aplasia
 The most common, solid mass of compact
bone that has failed to recanalize
 Atresia
Minor Aplasia-partial recanalization
 Middle space constricted, often with severe
ossicular abnormalities
 Microtia

Types of Microtia (Based on Marx 1926)

Class I Mild deformity, auricle smaller, all
parts of ear identifiable
Class II ½ to 2/3 normal size with partially
retained structures
Class III Severely malformed, peanut
shaped
 Radiological Evaluation
 High resolution CT in coronal and axial
planes
 Axial to delineate malleus, incus and I-S
joint and round window
 Coronal to delineate stapes, oval window
and vestibule
 3-D CT of little help
 Timing indicated by timing of surgery
 Grading System
Variable Points
 Based on high
Stapes Present 2
resolution CT scan
Oval Window Open 1
 A score of 5 or less Facial Nerve 1
denotes a poor Middle Ear Space 1
candidate Mastoid Pneumatization 1
 Microtia indicates an Malleus/Incus Complex 1
arrest in development Incudostapedial Joint 1
and abnormalities of Round Window 1
the middle ear External Ear Appearance 1
Total Points 10

Kountakis, Helidonis and Jahrsdoerfer Arch. Oto 1995

 Atresia
Grade of Microtia as an Indicator of ME
Development
Microtia Grade Radiological Score
I 8.5
II 7.2
III 5.9

Kountakis Arch Otolaryngol 1995

 Radiological Evaluation
 Good middle ear
and mastoid
aeration
 Prominent
ossicular mass
with incus and
malleus fused
 Open oval
window
 Score of 8
 Radiological Evaluation
 Score of 8
2-stapes & OW
2-I-S joint, Incus
1-Aerated ME space
1-Bony cover on VII
1-Aerated mastoid
1-Pinna
 Radiological Evaluation
 Poor middle ear and
mastoid development
 No definable ossicular
mass
 Closed oval window
 Not a good candidate
 A score of 3
Microtia
Microtia Repair
 Audiological Evaluation
 50-60 dB conductive loss
 Usually normal sensorineural function
 Unilateral- behavioral audiometry
 ABR for infants
 Bilateral atresia presents a masking dilemma
 Bone conduction ABR can help resolve the
masking problem (Wave I on stimulated ear)
 Surgical Considerations
 Most consider repair in bilateral atresia
 Many are reluctant to operate on unilateral
cases
– Not simply the hearing loss
– Expectations of hearing recovery
– Lifetime care of mastoid cavity
– Potential risks to facial nerve and labyrinth
– 55-65% achieve 25 dB speech-hearing level
 Surgical Considerations
 Most surgeons choose the anterior
approach to avoid the mastoid cavity
 40% of patients with unilateral atresia are
not surgical candidates such as those with
severe aplasia as in Treacher Collins
syndrome
 Bilateral atresia- best ear by CT done as
child approaches school age
 Surgical Considerations
Timing of surgery
 Usually performed after age 6 or 7 years
 This allows for microtia repair to be done
first (If Medpor used,canalplasty done
first)
 Canal cholesteatoma in the stenotic ear
usually develops in canals less than 2mm
in diameter. If ear unfavorable, canalplasty
alone is offered
 Canal Cholesteatoma
 Usually in ears with minor aplasia
 Canal <4mm, 50% incidence
 Surgical Technique
 Minor aplasia- canal widening and middle
ear ossicular work with tympanoplasty
 Moderate Aplasia
– Mastoid or posterior approach
– Anterior approach
 Surgical Technique
Anterior Approach
 Middle ear approached through the atretic
bone with a limited mastoid opening
 Surgical Technique

 The posterior wall of the

glenoid fossa becomes
the anterior wall of the
new ear canal
 The epitympanum is the
first part of the middle
ear encountered
 Fused ossicles identified
 Surgical Technique
 Atretic bone removed at times with a curette
 Globular mass separated from the stapes to
avoid cochlear trauma
 Course of facial nerve determined
 Ossiculoplasty performed
 Tympanic membrane grafted
 Meatoplasty
 Split thickness skin graft (.008-.010 inches)
lines the canal
 Surgical Technique
 The Meatoplasty must be aligned with the
newly created bony canal
 Surgical Technique
 4cm X 6cm split
thickness skin graft
 The graft is positioned
in the canal and sewn
to the meatal margin
 Graft stabilized with
Merocel wicks and
hydrated with ear
drops
 Hearing Results
 Post-op hearing level of 30 dB or better In
50-75% of patients with moderate or
severe aplasia
 20 dB or better in 15-20%
– Bellucci 20 dB in 50% @ 2 years
– Schuknect similar results at 1.3 years
– De La Cruz 56 patients 53% @ 20 dB at 6 mo.
– Lambert early 60% @25 dB, 46% >1 yr.
 Alternatives to Surgery
 Bone anchored hearing aid (BAHA)
 BAHA
Hearing results better than with BC aids
 Surgical Complications
 Persistent or recurrent conductive hearing loss
– Lateralization of graft
– Scar tissue
 SNHL
 VIIth Nerve injury
 30 % revision rate
– Re-stenosis
– Graft migration
– Inadequate hearing
 Chronic cavity infection
 Summary
 Choose your patients carefully
 Realize your potential v. patient
expectations
 Appropriate radiographic studies
 Accurate audiological analysis
 Facial nerve monitoring
 Know when NOT to operate