The Mondini Dysplasia—from Early Diagnosis to Cochlear Implant

Abstract

The early diagnosis of the Mondini dysplasia by means of electrocochleography and temporal bone polyto-mography. and the prevention of further hearing loss through the endolymphatic subarachnoid shunt operation. brought new data on the follow-up of patients with this ma1formation: They were found to present specific medi cal and educational problems. Children with concomitant serous otitis media were found to develop profound hear ing loss following insertion of ventilation tubes due to fistula formation in the oval window. One cochlear im plant. employing the technique developed by William House, was performed in a 22-year-old patient in whom the dysplasia had not been previously recognized. The importance of early diagnosis and treatment will be em phasized.