Congenital aural atresia

Case contributed by Francis Deng
Diagnosis certain

Presentation

Conductive hearing loss, congenital microtia and external auditory canal atresia. Planning bone-anchored hearing aid placement.

Patient Data

Age: 8 years
Gender: Male
ct

Right ear:

  • microtia: abnormally low and malformed pinna
  • external auditory canal atresia: complete bony and soft tissue obliteration of the expected course of the external auditory canal
  • middle ear cavity underdevelopment but no opacification to suggest cholesteatoma
  • ossicular malformation: absent or hypoplastic malleus, malformed incus with short long/lenticular process, shallow angulation of the incudostapedial articulation Normal stapes
  • normal inner ear structures

Left ear: normal

Case Discussion

This patient has known microtia and external auditory canal atresia noticed at birth. A CT evaluation performed as an infant ruled out congenital cholesteatoma. The current study was obtained for surgical planning for bone-anchored hearing aid placement. It demonstrates the common associations of pinna, external auditory canal, middle ear cavity, and ossicular abnormalities, together termed congenital external and middle ear malformations (CEMEM).

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