Algorithm of diagnostics and surgery stage of cochlear implantation in patients with various congenital deafness etiology

Abstract

Over the past few decades, in the world scientific literature devoted to the problems of auditory-speech rehabilitation, there has been an increased interest in studying the etiological features of congenital deafness. A deep understanding of the etiology and mechanisms of deafness development helped optimize the various stages of cochlear implantation and, thereby, increase its effectiveness. Objective. To develop an algorithm for obtaining the earliest possible information about the causative factor of congenital deafness for timely planning of rehabilitation measures, reducing the cost of excessive diagnostic search, and achieving the most effective results in the auditory and speech development of children. Patients and methods. From 2018 to 2021, a study of the etiological spectrum as well as the characteristics of cochlear implantation in patients with various etiologies of congenital deafness was conducted at the Saint Petersburg Research Institute for Ear, Throat, Nose, and Speech. We examined 100 patients under the age of 3 years (mean age 2,15 ± 0,2 years), of which 58 were female, and 42 were male. All patients had congenital bilateral deafness on newborn audiological screening and were candidates for cochlear implantation. Results and discussion. According to the data obtained, 37 patients (16 boys, 21 girls, mean age 2,3 ± 0,3 years) had congenital cytomegalovirus infection as an etiological factor in deafness. The presence of signs of congenital deafness of genetic etiology was detected in 58 patients (24 boys, 34 girls, mean age 1,9 ± 0,2 years): 43 of them with nonsyndromic deafness and 15 patients with syndromic deafness (4 boys, 11 girls, mean age 2,1 ± 0,4 years). In 5 patients, the etiological factor of congenital deafness could not be established even at the present stage of diagnostic search.