[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.
D001301:Audiometry, Pure-Tone; D001309:Auditory Threshold; D002607:Charcot-Marie-Tooth Disease / Q000175:diagnosis*; D003638:Deafness / Q000175:diagnosis*; D005072:Evoked Potentials, Auditory; D006801:Humans; D008297:Male; D008875:Middle Aged; D009133:Muscular Atrophy / Q000175:diagnosis*; D004856:Postural Balance; D014012:Tinnitus / Q000175:diagnosis
Schwerhörigkeit als Symptom der neuralen Muskelatrophie (Charcot-Marie-Tooth-Krankheit).