Abnormal auditory evoked potentials in Déjérine-Sottas disease. Report of two cases with central acoustic and vestibular impairment.

Two cases of hereditary motor sensory neuropathy type III (Déjérine-Sottas disease) examined by audiological, vestibular and electrophysiological methods are reported. In both cases there were signs of vestibular and acoustic central pathway involvement, shown by vestibular examination and by the study of auditory evoked potentials. The presence of central involvement in this hereditary neuropathy suggests central as well as peripheral myelin alteration.