A father and daughter had sensorineural deafness in conjunction with a saddle-nose defect, severe myopia, and juvenile cataracts. The saddle-nose defect can be traced vertically in three preceding generations without the presence of detected auditory or visual defects. It is postulated that this syndrome is a genetic disease transmitted as an autosomal dominant with variable expressivity. Future reporting of other families will help to clarify the precise development of the auditory disorder and the genetic transmission.