Abstract
Inner ear malformations contributing to bilateral profound hearing loss in adulthood are extremely rare. High-resolution computed tomography of the temporal bones and magnetic resonance imaging revealed unilateral duplicated internal auditory canal (IAC) and bilateral semicircular canal aplasia in such an adult. This is only the fourth reported case in literature known to us of duplicated IAC in adults and first in which there is a description of isolated bilateral lateral semicircular canal aplasia as the sole labyrinthine abnormality. Dolichoectasia of the vertebrobasilar artery was also present in the contralateral ear causing compression of the cochlear nerve and profound deafness in the other ear for which cochlear implantation was done.
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