The Mondini dysplasia - a clinical study.

The authors describe a clinical approach to the diagnosis and treatment of sensory-neural hearing loss associated to the Mondini dysplasia. The first element of diagnostic suspicion was represented either by a history of fluctuant and progressive hearing loss or by electrocochleography patterns showing multiple-peaked acoustic nerve action potentials similar to those observed in Ménière's disease. Confirmation of the diagnosis was obtained by temporal bone polytomography. An endolymphatic subarachnoid shunt was performed in 22 ears of 18 patients. The results, although preliminary, suggest that the shunt effectively contributes to stabilize the hearing and arrest the further progression of the hearing loss.