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Case Reports in Neurological Medicine
Volume 2012, Article ID 581920, 4 pages
Case Report

An Unusual Case of Bilateral Agenesis of the Cochlear Nerves

1Department of Otorhinolaryngology-Head and Neck Surgery, University of Brasilia, DF, Brazil
2Department of Radiology, University of Brasilia, DF, Brazil

Received 16 April 2012; Accepted 26 June 2012

Academic Editors: J. E. Cohen, R. Hashimoto, and J. Lazareff

Copyright © 2012 Vítor Yamashiro Rocha Soares et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Imaging of the cochlea and internal auditory canals are increasingly important nowadays because of the growing number of cochlear implants being performed throughout the world. We report a case of a 4-year-old boy who was born deaf and was being evaluated in our service for possible cochlear implantation. Audiometry disclosed profound bilateral deafness. The magnetic resonance imaging revealed only two nerves in each inner auditory canal: one in the anterior superior quadrant, identified as the facial nerve, and one on the posterior quadrants, representing both the superior and inferior vestibular nerves. The semicircular canals were not seen and the vestibule had dysplastic morphology. The diagnosis was bilateral agenesis of the cochlear nerves and semicircular canals.