aural-atresia-slides-071017

aural-atresia-slides-071017 - Congenital Aural Atresia Hwa...

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Unformatted text preview: Congenital Aural Atresia Hwa J Son, M.D. Tomoko Makishima, M.D. 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 21 st wk Auricle 1,2 BA 8 -12 wk Middle Ear 1,2 BA 5 -12 wk Inner ear otic capsule 3 - 20 th 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 Points Stapes 2 Oval window open 1 Middle ear space 1 Facial nerve normal 1 Malleus-incus complex present 1 Mastoid well pneumatized 1 Incus-stapes connection 1 Round window normal 1 Appearance of external ear 1 Treatment Options...
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This note was uploaded on 12/28/2011 for the course STEP 1 taught by Professor Dr.aslam during the Fall '11 term at Montgomery College.

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aural-atresia-slides-071017 - Congenital Aural Atresia Hwa...

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