Presentation
Conductive hearing loss, congenital microtia and external auditory canal atresia. Planning bone-anchored hearing aid placement.
Patient Data
Age: 8 years
Gender: Male
From the case:
Congenital aural atresia
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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).