Auditory function in hereditary motor and sensory neuropathy (Charcot-Marie-Tooth disease)

Acta Otolaryngol. Jan-Feb 1987;103(1-2):50-5. doi: 10.3109/00016488709134697.


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.

MeSH terms

  • Adolescent
  • Adult
  • Evoked Potentials, Auditory
  • Female
  • Fibula
  • Hearing Loss / etiology
  • Hearing*
  • Hereditary Sensory and Autonomic Neuropathies / physiopathology
  • Humans
  • Male
  • Middle Aged
  • Muscular Atrophy / complications
  • Muscular Atrophy / physiopathology*