Inner ear histopathology in genetically determined congenital deafness

Histopathology in seven cases with conegenital anomalies of the inner ear of varying degree are described. Five of these had bilateral and one unilateral deafness, with function undetermined in one. Five exhibited wide variation in degree of bony aplasia. All seven had some degree of membranous aplasia. One case presented the Wildervanck syndrome. The known anomalies were limited to the ears in six. Family history of deafness was negative in six, unknown in one. Diagnosis of probable genetic origin is based mainly on the type of pathology.