CT of severe inner ear anomalies, including aplasia, in a case of Wildervanck syndrome.

The Wildervanck (or cervico-oculo-acoustic) syndrome consists of the characteristic triad of Klippel-Feil anomaly, abducens nerve palsy, and congenital deafness. It is a rare "disorder of polygenic heredity with sex limitation predominant to the female" (female-male ratio is 10: 1) ( 1 ). Hearing loss is most commonly neurosensory due to developmental inner ear anomalies. Only a single case of associated congenital facial nerve paralysis has been described (2). We report this case of Wildervanck syndrome to display computed tomography (CT) images of inner ear aplasia (a rare anomaly) and to cite some unusual features, namely male sex and facial nerve paralysis.