Auditory Function in Patients with Charcot-marie-tooth Syndrome

The 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. It strikes one or more neuronals systems and can involve neurons of the central and peripheral motor system, so it can interest the faces of Goll and Burdach or the faces of spine cerebral. The onset may occur during late childhood or adolescence, may become progressive and severe or may spontaneously arrest at any time.Evolutionary trends overlapping was observed in other studies of childhood neurodegenerative diseases relating to immune character. Various conditions have been reported in association with CMT, including Friedreich’s ataxia, myopathy, a combination of nephritis and sensorineural hearing loss and optic atrophy. Features of hereditary cerebellar ataxias, optic atrophy, and other cranial nerve involvement may occur in combination with the previously mentioned classic description. In some cases, it is possible observe bilateral vocal fold paralysis, resulting in disorders of phonation, These changes are based on neurodegenerative disorders, resulting in very similar vocal demonstrations to those found in the larynx senile. Some aspects similar neurodegenerative can also be found in the epileptic disease, even if under the clinical profile diseases are widely different. From the anatomo-pathologic point of view microscopically signs of atrophy are observed with reduction of volume of long spinal lines and of Acta Medica Mediterranea, 2016, 32: 1719