Congenital malformations of ear structures can occur in various combinations and be combined with aplasia/hypoplasia of the vestibulocochlear nerve. Cochlear implantation in cases of severe combined ear anomalies is associated with a high risk of intra and postoperative complications and unsatisfactory results. Complex combined malformations of the temporal bone structures occur in genetic syndromes, the structure of which may also include intellectual disability and autism spectrum disorder. This combination of pathology further worsens the prognosis of cochlear implantation. A correct assessment of the condition of the ear structures and the clinical picture determines the fate of a deaf child. When determining indications for cochlear implantation and assessing its prospects in children with complex anomalies, it is necessary to assess the condition of all ear structures in CT and MR (3 Tesla) images. In this case, the most critical is the condition of the following structures: cochlea - modiolus - cochlear aperture - diameter of the internal auditory canal - presence and condition of the auditory nerve. The absence of an image of the auditory nerve with complete bony obliteration of the cochlear aperture makes cochlear implantation unpromising.

Liia Toropchina has completed her PHD at the age of 30 years from Research Institute of Clinical Otorhinolaryngology, Russia. She is a clinical audiologist and associate professor at the Department of Pediatric Otorhinolaryngology at the Russian Medical Academy of Continuing Professional Education, Russia. She has over 30 publications that have been cited over 112 times, and her h-index of publications is 4. She was the main pediatric audiologist in the Moscow region for 6 years. Area of professional interests: rehabilitation of children with deafness and complex structure of the defect, genetics of hearing, congenital anomalies  and autoimmune diseases of the ear.