Canal cholesteatoma: an alarming sequela of congenital aural atresia
DOI:
https://doi.org/10.18203/issn.2454-5929.ijohns20241331Keywords:
Atresia, External auditory canal, Congenital, Congenital microtia, CholesteatomaAbstract
Congenital aural atresia is an external ear malformation that may have diverse clinical presentations ranging from conductive hearing loss to the formation of canal cholesteatoma. Canal cholesteatoma, once developed can aggressively erode the bony boundaries of the middle ear cleft and lead to dangerous complications. Here we present the clinical outlook and surgical management of a child with congenital aural atresia with extensive canal cholesteatoma and complications. A twelve-year-old girl with congenital aural atresia and microtia of the right ear presented with right sided infra-auricular abscess with overlying skin ulceration. She also had right sided grade 4 facial paralysis. Pure tone audiometry revealed severe conductive hearing loss on the right side. In contrast, a high-resolution computed tomography scan showed extensive soft tissue on the right side completely destroying the posterior bony canal wall, with erosion of the sigmoid plate and the mastoid tip. We performed a right sided modified radical mastoidectomy and canal atresia repair along with the removal of the necrotic and infected tissues. The little girl recovered uneventfully with improved facial nerve function and without any sign of cholesteatoma recidivism. In congenital aural atresia, early surgical intervention is the key to prevent the formation of canal cholesteatoma and subsequent complications.
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