MONDINI DYSPLASIA AND CONGENITAL CYTOMEGALOVIRUS-INFECTION

We report a case of bilateral temporal bone anomalies in a child with symptomatic congenital cytomegalovirus infection and severe, bilateral sensorineural hearing loss identified at 3 months of age. High-resolu tion temporal bone computed tomography (HRCT) revealed bilateral findi ngs of a short, malformed cochlea lacking an interscalar septum, a sho rt and wide internal auditory canal, and an enlarged vestibular aquedu ct, features diagnostic of bilateral Mondini dysplasia. To determine t he importance of this observation, we completed HRCT in five additiona l children between 7 months and 9 years of age who had evidence of sym ptomatic congenital cytomegalovirus infection. One child with profound sensorineural hearing loss had severe bilateral temporal bone dysplas ia with a small cochlea lacking an interscalar septum, an abnormal ves tibule, and a large cochlear aqueduct. Of the remaining four children, hearing thresholds ranged from normal to profoundly decreased, but th eir HRCT scans were normal to visual inspection. When inner ear dimens ions of these temporal bones were compared with norms established by P appas and coworkers, however, seven of the eight ears had short cochle as and narrow lateral semicircular canals, and three ears had short or narrow vestibules. These results indicate that congenital cytomegalov irus infection may cause anomalies or growth disturbances of the tempo ral bone.