Scheibe Dysgenesis of the inner ear

: A Scheibe-type dysgenesis of the inner ear was found in the temporal bones of an 85-year-old deaf-mute woman. Both temporal bones showed similar histopathological findings, which were confined mainly to the cochlea and saccule. The stria vascularis was atrophic and contained cysts with eosinophilic content throughout the cochlea. The scala media had disappeared owing to marked collapse of Reissner's membrane. The saccule showed collapse. Many eosinophilic spherical masses were observed in the area of the macula sacculi and attached to the saccular wall.