AUDIOLOGICAL FINDINGS IN A PATIENT WITH CHARCOT MARIE TOOTH DISEASE: A CASE STUDY.

Charcot Marie Tooth (CMT) disease is the most common inherited disorder of the peripheral nervous system affecting approximately 1 in 2500 individuals. CMT disease could be viewed as a specific type of auditory neuropathy. Weaknesses of hands and feet are often noticed with some cases clearly having auditory complaints. A 23 year old woman with CMT disease was evaluated audiologically. She had no auditory complaints. Pure tone and speech audiometry findings were normal. Abnormal auditory brainstem responses were found bilaterally. Latencies of waves I, III and V were extremely prolonged and, wave morphology was poor at the leftside. Cervical vestibular evoked myogenic potentials were recorded. There was no response at the leftside. Our results provide some evidence that in Charcot MarieTooth disease the involvement oftheVIII cranial nerve is more frequent than clinically expected and is probably related to a demyelinated process. [ABSTRACT FROM AUTHOR]