Cochlear implantation in the congenitally malformed ear.

A child with a congenital hearing loss and auricular malformations was evaluated for cochlear implantation. Preoperative radiologic studies demonstrated a common cavity cochlear malformation and a contralateral narrow internal auditory canal. Intraoperative findings included an aberrantly coursing facial nerve, preventing routine placement of the electrode array. The surgical approach was consequently modified and the cochlea was accessed through a malformed semicircular canal. Cochlear implantation can be performed safely and effectively in children with congenitally malformed ears.