Three Cases of Bilateral Semicircular Canal Aplasia with Normal Cochlear Development in the Same Family Member / 대한이비인후과학회지

Semicircular canal aplasia is rare congenital inner ear anomaly. During embryogenesis, congenital malformation of vestibular labyrinth usually associates with cochlear anomalies. Two cases of semicircular canal aplasia with normal or near-normal cochlear development was reported in the English literature. We present three patients with computed tomographic findings of bilateral total semicircular canal aplasia with normal cochlear development in the same family member. Two patients had significant conductive hearing loss due to congenital stapedial anomalies and the other was congenital deafmute. Temporal bone CT scan revealed total absence of the semicircular canals bilaterally and both cochleas were normal in three cases.