Audiologic evaluation in Charcot-Marie-Tooth disease

Scand Audiol Suppl. 1988;30:211-3.


We have carried out a hearing study with pure tone audiometry and ABR of 39 patients affected by hereditary motor and sensory neuropathy or Charcot-Marie Tooth disease. We have noticed a hearing deficiency in a significant percentage of cases (28%). We attribute the abnormalities to disorders in the area of the VIIIth nerve and of the brainstem. No significant differences appear in the results obtained between patients belonging to type I and patients belonging to type II of this disease.

MeSH terms

  • Adolescent
  • Adult
  • Audiometry, Evoked Response
  • Audiometry, Pure-Tone
  • Charcot-Marie-Tooth Disease / complications*
  • Child
  • Female
  • Hearing Disorders / diagnosis
  • Hearing Disorders / etiology*
  • Humans
  • Male
  • Middle Aged
  • Muscular Atrophy, Spinal / complications*