[Deafness as a symptom of neural muscular atrophy (Charcot-Marie-Tooth disease)]

Peroneal muscular atrophy (Charcot-Marie-Tooth-disease) is a hereditary disease of the peripheral motor and sensory neurons. In most patients the distal extremities are affected but involvement of the cranial nerves has also been described. We describe a 58 year old patient suffering from this disease accompanied by bilateral hearing loss. Audiometric examinations over a period of 10 years showed no progression and indicate a neural hearing loss. This was finally verified by auditory potentials (EAEP); J V and J III were prolonged, indicating a neural origin of hearing loss. Thus the lesion must be localised to the cranial nerves or brainstem and represent a symptom of the peroneal muscular atrophy.