Congenital Aural Atresia

Hwa J Son, M.D.

Faculty Advisor: Tomoko Makishima, MD, PhD
October 17, 2007
UTMB Otolaryngology
Grand Rounds Presentation

Epidemiology
1 in 10,000 to 20,000 live births
Male > Female
Right side > Left side
Unilateral > Bilateral (3:1)
Bony atresia > Membranous

Embryology
1st branchial groove forms meatal plate at 6
- 8 wks resorbs/forms EAC at 21st wk
Auricle
1,2 BA
8 -12 wk

Middle Ear
1,2 BA
5 -12 wk

Inner ear
otic capsule
3 - 20th wk

Associated Ear Deformity

Common
Middle Ear
Malleus/Incus fusion

External Ear
Microtia
Severity of microtia correlates with
malformation of middle ear

Uncommon
Stapes footplate
Inner Ear problems

Etiology
Sporadic
Syndromic (11-47%) Goldenhars,
Treacher-Collins, branchio-oto-renal
syndrome, de Grouchy syndrome (18q-) and
Crouzon syndrome.

Familial within immediate family

4.9%, extended family 10.3%

Non-Syndromic Associations
Facial asymmetry (36.5%)
FN weakness (15.2%)
Cleft lip/palate (4.3%)
Urogenital defects (4%)
CV malformation (2.5%)
Macrostomia (2.5%)
Congenital cholesteatoma (4-7%)

History
Hx of teratogens
microtia- thalidomide, isoretinoin,
vincristine, cholchicine, cadmium

Family hx
Hearing status, speech development,
past surgical procedures
Frequency of otitis media

Physical Exam
Location of condyle: posterior position
poor prognosis atretic plate and ME
are poorly developed
Size of mastoid (palpate mastoid tip,
spine of Henle, condyle, zygomatic
arch)
Craniofacial anomaly
Severity of microtia

Grades of Microtia

Schucknecht Classification

Audiological Assessment

When: ABR within 2-3 months of birth

Results

Bone conduction usually normal

Air conduction maximal at 60dB

Intervention
-

Normal or u/l confirm with behavioral

audiogram at 6 M age, no need for aid
B/L: Need bone conduction hearing aid, early
speech therapy

Imaging
When? At several months
Look for

Status of the inner ear

Extent of temporal bone pneumatization;
Course of the facial nerve
Presence of the oval window/stapes footplate.

Also important

Size of bony atretic plate

Soft tissue contribution to the atresia
Size/status of the middle ear cavity
Presence/absence of congenital cholesteatoma.

Example 1

Example 2

Jahrsdoerfer Grading System

Parameter
Stapes
Oval window open
Middle ear space
Facial nerve normal
Malleus-incus complex present
Mastoid well pneumatized
Incus-stapes connection
Round window normal
Appearance of external ear

Points
2
1
1
1
1
1
1
1
1

Treatment Options
Non-reconstructive option
Bone conduction hearing aids
BAHA

Atresiaplasty +/- auriculoplasty

Non-Reconstructive Option
Bone conduction hearing aid (BCHA)
Ideally before age 6M for b/l CAA.

BAHA
Surgically-implanted, percutaneous
titanium screw fixture
Osseointegrates into the temporal
bone.

Advantages of BAHA
Sound energy is not attenuated by the
skin and intervening soft tissues
More comfortable at contact site
Does not preclude
reconstruction later
on because of
surgical scars

Surgery for BAHA

Needs 2-stage surgery in young
children (age 2-10)
1st: titanium screw
2nd: 3M later, skin-penetrating abutment

Complications limited to:

local infection / inflammation (7.5%)
failure to osseointegrate (2.5%)

BAHA Results
Grandstrom(1993, L4)
100% subjective satisfaction and speech
thresholds <30 dB in 39 pt with Js Score <6,

Hakansson et al.(1995, L4)

147 pt. PTA <45 dB 89% with subjective
improvement.
PTA 46-60 with 61% and PTA >60 with 22%

Lustig et al.(2004, L4)

40 pt (9 w/ CAA) - ABG closure of <10 in 80%, <5
in 60%.

2004 BAHA Consensus

Statement (L5)
BAHA best option when:
ABG>30 with external canal occlusion
tx resistant OM

B/l case, give b/l

conductive aid before
age 6M
Awaiting reconstruction:
BAHA can be used in
children <2yo with
steel or elastic band

Reconstructive Surgery

Goal of Reconstructive Surgery

Create a patent, skin-lined EAC
Postoperative air-bone gap within 20
to 30 dB.
Long term hearing result that is
adequate for speech/language
development in children
Cosmetically appealing auricle

Difficulty of Surgery
Altered anatomy, absence of
anatomical landmarks
Fear of injuring FN
SNHL from drilling
Difficulty of placing skin graft
Chance of meatal stenosis
Concern of post-op infection

Staging of Surgery
Stage 1: Cartilage graft harvest,
sculpturing, implantation
Stage 2: Lobule repositioning (z-plasty
to more posterior/inferior)
Stage 3: Elevation of auricle
Stage 4: Tragus formation,
atresiaplasty

Contraindications
Significant SNHL or inner ear malformation.
Limited middle earmastoid pneumatization
or a significantly hypoplastic middle-ear
cleft.
Anomalous facial nerve or aberrant major
vascular structure.
Middle cranial fossa tegmen sagging
Would restrict anatomic access to the middle-ear
cleft.

Timing of Surgery
Start at age 6Y or later.
Limitations for earliest date
Rib cartilage maturation
Post-op cooperation

Reconstruction of auricle first

Chance of success/healing best without
vascular compromise

Operate on better ear first

Canaloplasty

Tympanoplasty, Skin grafting

Meatoplasty

Pitfalls
Second genu makes acute angle turn
instead of 90
Vertical course crosses the middle ear

Follow-up Care
Meatal suture out in 7 days
Packing removed at 2 and 3 wks
Antibiotic drops for 8-12 wks
Audiogram at 8 wks, 6 M and yearly
New ear canal must be debrided under
microscope q 6-12M

Final Cosmetic Result

Hearing Results
Jahrsdoerfer (1992, L4)
90 operated on, 37 with grade 8-9.
SRT <10 in 11% and SRT 10-25 in 78%

Chandrasekhar (1995, L4)

92 pt, ABG <30 in 60% of primary and in 54% of
revision sx

Murphy (1997, L4)

33% of partial atresia with SRT <20 dB and 15%
of total atresia

De La Cruz (2003, L4)

116 ears, 58% ABG <30 for primary, 56% for
revision

Long Term Hearing

De La ruz (2003, L4)
90 ears with >6M f/u.
PTA: 59.9dB 45.3 dB 52.5 dB
ABG: 45.1 dB 28.1 dB 32 dB

Hearing gets worse long term, but not

statistically significant
Ossicular chain refixation the most
common cause of HL

Results Summary
Surgeon dependent
Whats termed success varies between
studies
May not be appropriate for pre-school
and school aged children (SRT <15 per
audiology literature)

Complication
Lateralization of TM (22-28%)
up to 12 M post-op

Meatal stenosis (cartilagenous >

bony): 8-12%
SNHL inner ear damage (2%)
Facial nerve injury (<1%)
Fixation/discontinuity of ossicles
(11.5%)

Measures to Avoid Complication

Lateralization of TM
Cut off nitrous oxide 30 min before grafting.
The graft anchored medially to the malleus and
the tab placed into the protympanum.
Use of silastic button.

Stenosis:
Use of large STSG
Merocel wicks

SNHL:
Caution with drilling,
Use laser

FN injury
Intra-op monitor

Surgery or implantable hearing

devices?

Surgery vs. BAHA

Evans (2006, L4)
Compare hearing results, complications, cost
Reconstructed
Hearing
gain
Cost

17.3 dB per ear

BAHA
2-stage
31.8

BAHA
1-stage
31.8

$51506

$42449

$28341

$1238

$826

Cost/dB $2909

Results
Cost
BAHA covered by Medicare
Cost about 1/4 of sx per dB gain

Hearing
93% of reconstructive sx pt still needed some
form of sound amplification
BAHA achieves HL <15dB with normal bone
curves

Complications
Reconstruction : Canal stenosis (22.2%), ROE
(19%), canal prolapse(5%), cholesteatoma (3%)
BAHA with fewer serious complications: 1/6 with
hypertrophic scar

BAHA for Binaural Hearing

Use of BAHA in Unilateral CAA

Wazen et al.
Prospective case series with 9 pt.

Pt benefited in tonal, spondee

threshold
Significant improvement in handicap
score (from questionnaire)
Gain binaural benefit in
localization/speech perception in
noise

Case Presentation #1

53 yo M with no other signif PMHx presents to B-clinic as a referral from

audiology. He has Left microtia s/p microtia repair? at age 9 in Mexico,
wearing BCHA on right side. He wishes to be re-fitted for another BCHA.

Case Presentation #1

Case Presentation #2
RP is a 10 M old boy presenting to
pedi ENT clinic for f/u after failing OAE
as a newborn on Right side.
PE showed left microtia with narrow
EAC at bony-cartilaginous junction.
Right side stenotic, TM not visualized
ABR showed repeatable V-wave
Left: <30dB HL air stimulation
Right: <30dB bone stimulation

Conclusion
Early identification of problem important for
hearing amplification and special education
Patient classification with audio/CT
important for predicting results of sx
Severity of microtia predict ME deformity
BCHA should be fitted before age 6M for b/l
BAHA with better audio results and
satisfaction than BCHA for non-sx candidate
Sx needs careful planning with
multidisciplinary effort, careful timing

Conclusion
Surgery
Questionably adequate audio result for
children

BAHA
Good hearing results and more cost
effective for hearing gain

Binaural BAHA
Recommended for further gain in speech
in noise and localization

Bibliography

Nuijten I. Admiraal R. Van Buggenhout G. Cremers C. Frijns JP. Smeets D. van Ravenswaaij-Arts C. Congenital aural atresia in 18q deletion
or de Grouchy syndrome. [Case Reports. Journal Article]Otology & Neurotology. 24(6):900-6, 2003 Nov. UI: 14600472

Dostal A. Nemeckova J. Gaillyova R. Vranova V. Zezulkova D. Lejska M. Slapak I. Dostalova Z. Kuglik P. Identification of 2.3-Mb gene locus
for congenital aural atresia in 18q22.3 deletion: a case report analyzed by comparative genomic hybridization. [Review] [46 refs] [Case
Reports. Journal Article. Research Support, Non-U.S. Gov't. Review] Otology & Neurotology. 27(3):427-32, 2006 Apr.

Wazen JJ. Spitzer J. Ghossaini SN. Kacker A. Zschommler A. Results of the bone-anchored hearing aid in unilateral hearing loss. [Journal
Article] Laryngoscope. 111(6):955-8, 2001 Jun.

Granstrom G. Bergstrom K. Tjellstrom A. The bone-anchored hearing aid and bone-anchored epithesis for congenital ear malformations.
[Comparative Study. Journal Article. Research Support, Non-U.S. Gov't] Otolaryngology - Head & Neck Surgery. 109(1):46-53, 1993 Jul.

Jahrsdoerfer RA. Yeakley JW. Aguilar EA. Cole RR. Gray LC. Grading system for the selection of patients with congenital aural atresia. [Case
Reports. Journal Article] American Journal of Otology. 13(1):6-12, 1992 Jan.

Murphy TP. Burstein F. Cohen S. Management of congenital atresia of the external auditory canal. [Journal Article] Otolaryngology - Head
& Neck Surgery. 116(6 Pt 1):580-4, 1997 Jun.

De la Cruz A. Teufert KB. Congenital aural atresia surgery: long-term results. [Journal Article] Otolaryngology - Head & Neck Surgery.
129(1):121-7, 2003 Jul.

Jahrsdoerfer RA. Yeakley JW. Aguilar EA. Cole RR. Gray LC. Grading system for the selection of patients with congenital aural atresia.
[Case Reports. Journal Article] American Journal of Otology. 13(1):6-12, 1992 Jan.

Murphy TP. Burstein F. Cohen S. Management of congenital atresia of the external auditory canal. [Journal Article] Otolaryngology Head & Neck Surgery. 116(6 Pt 1):580-4, 1997 Jun.

Bibliography, pt. 2

Ear and Temporal b one Surgery. Wiet. 2006. Thieme

Pediatric Otolaryngology. Wetmore et al. 2000 Thieme

Evans AK. Kazahaya K. Canal atresia: "surgery or implantable hearing devices? The expert's question is
revisited". [Journal Article] International Journal of Pediatric Otorhinolaryngology. 71(3):367-74, 2007
Mar.

Chandrasekhar SS. De la Cruz A. Garrido E. Surgery of congenital aural atresia. [Journal Article]
American Journal of Otology. 16(6):713-7, 1995 Nov.

Snik AF. Mylanus EA. Proops DW. Wolfaardt JF. Hodgetts WE. Somers T. Niparko JK. Wazen JJ. Sterkers O.
Cremers CW. Tjellstrom A. Consensus statements on the BAHA system: where do we stand at present?.
[63 refs] [Consensus Development Conference. Journal Article] Annals of Otology, Rhinology, &
Laryngology - Supplement. 195:2-12, 2005 Dec.

Lustig LR. Arts HA. Brackmann DE. Francis HF. Molony T. Megerian CA. Moore GF. Moore KM. Morrow T.
Potsic W. Rubenstein JT. Srireddy S. Syms CA 3rd. Takahashi G. Vernick D. Wackym PA. Niparko JK.
Hearing rehabilitation using the BAHA bone-anchored hearing aid: results in 40 patients. [Clinical Trial.
Journal Article. Multicenter Study] Otology & Neurotology. 22(3):328-34, 2001 May.

Hakansson B. Liden G. Tjellstrom A. Ringdahl A. Jacobsson M. Carlsson P. Erlandson BE. Ten years of
experience with the Swedish bone-anchored hearing system. [Comparative Study. Journal Article] Annals
of Otology, Rhinology, & Laryngology - Supplement. 151:1-16, 1990 Oct.

Jahrsdoerder, RA., Mason, JC. Congenital aural atresia. Operative Techniques in Otolaryngology-Head
and Neck Surgery, Vol 14, No 4 (Dec), 2003: PP247-151