Classify aural atresia. Discuss its evaluation and treatment 2013

Question – Classify congenital aural atresia. Discuss in brief its evaluation and treatment options. (4+3+3) December 2013

Aural Atresia

1. Aural Atresia:
   1. 90% nonsyndromic.
   2. 10% syndromic.
   3. 20–30% bilateral
   4. When it occurs alone, it is due to failure of canalisation of the ectodermal core that fills the dorsal part of first branchial cleft.
   5. The outer meatus, in these cases, is obliterated with fibrous tissue or bone while the deep meatus and the tympanic membrane are normal.
   6. Associations:
      1. Microtia (55–93%)
      2. Malformed SCCs (10%).
      3. Malformed cochlea (5%).
      4. Cholesteatoma (4–7%).
      5. Stapes fixation (4%).
   7. Fusion of malleus/incus is most common middle ear anomaly.
      1. Footplate is usually normal.
   8. Otitis media should be treated aggressively to preserve hearing in the normal contralateral ear.

External Auditory Canal –

• -EAC derived from 1st branchial groove and initially represented by a solid core of epithelial cells that extends down to area of tympanic ring and first pharyngeal pouch
• -in middle trimester, absorption of epithelial cells begins, progressing in medial to lateral direction
• -malformation of tympanic bone produces atretic bone at level of tympanic membrane and results in atresia of ear canal

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