OBJECTIVE

To describe clinical observations of patients with syndromic deafness.

RESULTS

Deaf patients with CHARGE, Crouzon, and Wildervanck syndromes were monitored at the Russian Research Clinical Center for Audiology and Hearing Rehabilitation (Moscow) in different years. All of them were diagnosed having bilateral congenital deafness. After collecting anamnesis, evaluating the results of computed tomography of the temporal bones, and audiological examination, it was decided to conduct the cochlear implantation.

CONCLUSION

The only method that allows patients with bilateral congenital deafness to gain hearing is the cochlear implantation. The malformations of the tympanic cavity structures, an abnormal course of the facial nerve canal lead to technical difficulties during the surgical stage of cochlear implantation. The navigation equipment, monitoring of the facial nerve makes it easier to find anatomical structures, as well as to avoid injuries.