Congenital Anomalies of the Inner Ear

Histopathological findings of temporal bones from 3 cases of congenital anomalies were reported. These included 2 cases of Mondini type inner ear hypoplasia/dysplasia and a case of hypophosphatasia.Case 1: A two months old girl with hydrocephalus died of pneumonia on October 9, 1968. The temporal bones showed Mondini type hypoplasia of the inner ear. The cochleae were of a single coil. (the right cochlea: 8.5mm long, the left: 9.5mm long.) The organ of Corti was normal. There was a PAS positive deposit in the basal cell layer of the stria vascularis and the spiral ligament adjacent to the stria. No marked change was noted in the vestibular system.Case 2: A 17 months old boy with the cleft lip and the cleft palate, showing Mondini type inner ear dysplasia. This case was already reported in December issue of Annals of Otology, Rhinology and Laryngology, 1970.Case 3: A full-term girl of hypophosphatasia. This was documented and discussed in detail in December issue of Journal of Laryngology and Otology, 1968.