Charcot-Marie-Tooth (CMT) disease or hereditary motor and sensory neuropathy (HMSN) is a relatively common neurological syndrome, which has seldom been associated with hearing dysfunction, particularly sudden sensorineural hearing loss (SNHL). Families with autosomal dominant, autosomal recessive and X-linked forms of inheritance have been described.

Sudden sensorineural hearing loss is a frustrating and frightening condition, especially if the hearing loss is bilateral. Regarding the site of the lesion, the evidence from the literature on HMSN suggests that either the VIIIth nerve or central auditory pathways are primarily involved in patients with hearing loss.

We report the first case in the English literature of a patient with Charcot-Marie-Tooth type II disease presenting bilateral SNHL in the course of his disease. The patient was hospitalized for 15 days, and undergoing treatment without any audiological improvement. Detailed clinical, audiological and laboratory examination was performed.

The aetiology and prognostic indicators of bilateral SNHL are discussed, as well as, the incidence of hearing loss in CMT patients.