Congenital Aural Atresia

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”
 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
 Facial Nerve
• Bony dehiscence of fallopian canal
frequently occurs
• Anomalous course
• Abnormal position of mastoid segment of
facial nerve places it at jeopardy when
drilling.
 Classification of Auricle
(Bailey)
• Grade I: Microtia, minor malformation, with
auricle being smaller but with all parts
discernible
• Grade II: Microtia, the auricle is
prepresented by a curving or vertical ridge
of tissue
• Grade III: Microtia, auricle is lost, only a
small rudimentary soft tissue structure
 Classification of Auricle
(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
 Classification of Ombredanne
• Major Malformation
– EAC and TM are abscent
– Canal stenosis
– Reduced size of middle ear, ossicles
deformed, fused, or fixed.
– Dehiscence or displacement of faical nerve
– Inner ear function is usually normal
 Classification of Ombredanne
• Minor Malformation
– Involving the middle ear
– Conductive hearing loss
– Abnormalities of the stapes may be more
severe than major group
– EAC may be mild stenotic
 Classifications of External/Middle
Ear
• 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
 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
• Bone conduction ABR testing
• If ear canal patent, ECochG can be used.
 Evaluation
• Temporal bone CT is necessary for surgery.
• For evaluation of possible cholesteatoma
• 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
 CT
• Coronal
Axial Scans
Scans
– The
Stapes
body of malleus and incus
– Oval
Incudostapeidal
window joint
– Vestibule
Round windows
 CT
• 5~6 years
– Performed near the time of operation
– Rarely applicable to immediate rehabilitative
plans for very young children
– Poor cooperation in very young children
– Cholesteatoma formation are older than 3
years
 Medical Management
• Unilateral Atresia
– No immediate medical intervention is
necessary
• Bilateral Atresia
– Early amplification is essential in infants.
– Initial audiologic evaluations can be
completed in first few months.
 Surgical Repair
• Candidacy
– ABSOLUTE REQUIREMENTS
• 1. Normal inner ear
• 2. Normal cochlear function
 Surgical Repair
• Unilateral:
– An improvement in the hearing threshold to
25dB or better eliminates the handicap of
unilateral hearing loss.
• Bilateral:
– To restore sufficient hearing so that
amplification is no longer needed.
– The best ear is selected for the initial surgical
procedure.
 Surgical Repair
• Timing
– 6-7 years of age
– Pneumatization of temporal bone is well
advanced
– Much cooperation
 Classification
• Jahrsdoerfer,
– HRCT Score 1992
– • </= 5/10: poor temporal bone findings
Based on HRCT
• 6/10: marginal
– Score correlates to likelihood of successful surgery
• 7/10: fair
• 8/10: good
• 9/10: very good
• 10/10: excellent
 Jahrsdoefer Grading System for
Congenital Atresia
Parament Points
Stapes present 2
Oval windows patent 1
Middle ear space 1
Facial nerve 1
Malleus/incus complex 1
Mastoid pneumatization 1
Incus-stapes connection 1
Round windows 1
External ear appearance 1
 Surgical Techniques
• Postauricular Approach
– Infrequently used
– Advantages:
• More familiar approach
• Identification of sinodural angle and lateral SCC
as landmarks
• Better visibility
– Disadvantages:
• Creation of mastoid cavity
– Larger defect to be skin grafted
– Prolonged healing
 Surgical Techniques
• Anterior Approach
– Popularized by Jahrsdoerfer, most frequently
utilized approach
– Advantage:
• Avoidance of mastoid cavity
– Disadvantages:
• Unfamiliar approach
• Lack of landmarks
 Postauricular Incision
• TMJ anteriorly
• Middle cranial
fossa dura
superiorly
• Mastoid air
cells
posteriorly
 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
 Conclusion
• Complex and Challenging Problem
• Goals:
– Restore functional hearing
– Construct patent and infection-free EAC