Otologic manifestations of the hydantoin syndrome

The temporal bones of a newborn infant with hydantoin syndrome showed multiple middle ear and inner ear anomalies. There was a constellation of bony and membranous defects involving the oval and round windows, cochlear ducts, cochlear aqueducts, endolymphatic ducts and sacs, and vestibular labyrinths. To the authors' knowledge, supernumerary vestibular sensory epithelial structures and an inner ear epidermoid cyst have not been previously reported. Wide communications between the subarachnoid space and inner ear were of surgical relevance.