Sensorineural hearing loss is currently the most important medical and social problem and is an urgent problem of neurology. According to various researchers, from 6% to 36% of the worlds population suffers from ear pathology, of which 60-80% have sensorineural hearing impairment (damage to the cochleo-vestibular nerve receptor - the cochlea of the inner ear).Deafness is the most common defect in sensory systems in children and occurs in 1: 750 cases, and in adults 4-36% depending on age, which reflects the interaction of environmental factors and individual genetic predisposition (Tranebjaerg L., 2000).About 400 genetic syndromes that include hearing impairment have been described (Friedman T.V., Griffith A J., 2003).