Abstract
High resolution computed tomography (HRCT) of the temporal bone has enabled us to diagnose inner ear anomalies easily. Otorhinolaryngologists will be able to diagnose Mondini type malformation more frequently. A 55-year-old female visited us because of hearing loss and vertigo. Pure tone audiograms revealed bilateral deafness. Bilateral Mondini type inner ear malformations were observed in the HRCT of the temporal bone. Her family history revealed that there were five women with severe hearing impairment. We perform temporal bone HRCT and audiograms on three women with impaired hearing and a woman with normal hearing in her family. Pure tone audiograms of three women revealed nearly bilateral deafness and temporal bone HRCT showed bilateral Mondini type inner malformations similar to one another. The CT of the woman with normal hearing showed completely normal findings. Autosomal dominant inheritance was suspected because of the family history.
