We performed light and electron microscopic studies on the temporal bones of a patient with genetic aplastic deafness, in which the right ear had a Mondini-type defect and the left ear a Michel-type anomaly. The round window of each ear was absent. The stapedial foot plate of the right ear was depressed at its central part and was covered by thin membrane. The course of the internal auditory meatus of the left ear was deviated ventrally. There was total aplasia of the cochlea in the left ear, while that of the right ear showed only three-quarters of a turn. In this ear, three sites of spherical bulging were found and were believed to represent outer hair cells. Other structures of the normal cochlear duct were not present. The modiolus of the left ear was round and dome-shaped, contained sparse nerve fibers and a primitive spiral ganglion. The saccule, utricle and semicircular canals of each ear were missing, so that a common cavity of the vestibular system was present. Several otoliths could be seen under the surface of the membrane covering the common cavity. No sensory cells or their related tissues could be found in either ear.