Auditory function in hereditary motor and sensory neuropathy (Charcot-Marie-Tooth disease).
Fourteen patients with hereditary motor and sensory neuropathy (HMSN), 12 of Type I and 2 of Type II, were assessed for auditory dysfunction. Five patients complained of hearing loss and all had pure-tone audiograms outside the normal range, while one patient who did not complain of hearing impairment also had an abnormal pure-tone audiogram. Assessment of loudness function, speech audiometry and brainstem auditory evoked potentials (BAEP) suggested that the hearing loss was the result of VIII nerve dysfunction, a conclusion supported by the abnormality of the electrocochleogram (ECochG) in one patient.
Raglan
E
E
Prasher
D K
DK
Trinder
E
E
Rudge
P
P
D000293:Adolescent; D000328:Adult; D005072:Evoked Potentials, Auditory; D005260:Female; D005360:Fibula; D006309:Hearing; D034381:Hearing Loss / Q000209:etiology; D009477:Hereditary Sensory and Autonomic Neuropathies / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009133:Muscular Atrophy / Q000150:complications / Q000503:physiopathology*