150 The Auditory and Vestibular Manifestations of Charcot Marie-Tooth Disease

Objective: To present the auditory-vestibular manifestations of Charcot Marie-Tooth Disease (CMT). Case Report: A 53-year-old woman complained of progressive imbalance over 1 year. Imbalance was described as the sense that she was about to fall to one side, was exacerbated by rapid head acceleration, and had no relation to postural changes. Symptoms were continuous, and she fell multiple times. She used a cane for 1 year, but for the last month required a walker. Bilateral tinnitus and hearing loss developed over the last year. CMT had been diagnosed about 20 years earlier, with progressive distal limb weakness and wasting and peripheral sensory neuropathy. The neurotologic examination was normal except for the following: There was mild tremor in the left hand. Unsupported stance was unsteady, and she fell with eye closure. Vibrotactile sensation was reduced at the hands and ankles. Audiometry demonstrated a mild, somewhat asymmetric sensorineural hearing loss (SNHL). Auditory brainstem response showed poor waveform morphology bilaterally; however, the latencies for waves III and IV were within the normal range. Electronystagmography with rotary chair data showed hypofunction and reduced vestibulo-ocular gain. Ophthalmologic evaluation was refused. Discussion: CMT is an autosomal-dominant disorder manifested by progressive peripheral neuropathy, resulting in distal muscle atrophy and a peripheral sensory neuropathy. SNHL has been described in CMT. The Rosenberg-Chutorian Syndrome involves CMT with SNHL and optic atrophy. Other cranial neuropathies have also been described in CMT. Results: We describe a patient with CMT patient who had hearing loss and imbalance. The combination of vestibulopathy, sensory (proprioceptive) neuropathy, and muscle atrophy produced a devastating balance disorder. The literature on otologic manifestations of CMT is reviewed.