Despite the rare incidence of Van der Hoeve syndrome in the population, the problem of treating patients with this type of disease is important for modern science and practical medicine. One of the most difficult tasks in treatment is to improve the quality of hearing. The world scientific community lacks a unified coordinated approach to the methods of auditory rehabilitation of patients with Van der Hoeve syndrome. In recent years, there have been tendencies in the scientific literature to increase the frequency of non-surgical approach due to the low incidence of satisfactory results of surgical treatment. In this regard, we present our experience of complex treatment of patients with Van der Hove syndrome, based on the use of modern surgical technologies and conservative pathogenetically substantiated treatment.