[Electrically evoked ABR through cochlear implant in children with auditory neuropathy spectrum disorder].

Objective: To estimate the applicability of electrically evoked auditory brainstem response (eABR) registration for the estimation of neural integrity after cochlear implantation (CI) in children with auditory neuropathy spectrum disorder (ANSD) and to compare the eABR data with patient's hearing performance.

Material And Methods: 4 children, Nucleus (Cochlear) CI users, with ANSD were enrolled in the study. Hearing performance in these children ranged from successful to unsatisfied.

[OTOF-related auditory neuropathy spectrum disorder].

Unlabelled: Otoferlin () gene mutations are the most common cause of hereditary ANSD according to investigations in several countries.

The Aim: Of this study was to estimate the prevalence of mutations in Russian children with ANSD and evaluate audiological and clinical features of -related ANSD.

Patients And Methods: 28 children with bilateral ANSDwere enrolled in the investigation.

[The effectiveness of the speech therapist and audiologist collaboration during speech processor fitting].

The aim of the study was to present the first clinical experience of clinical trials of new methodology of the CI speech processor fitting with the new software Nucleus Fitting Software - NFS. At all 4 fittings sessions there were 15 children. During the fitting sessions 2 techniques were used - with the new NFS software and classical technique were used.

Update of the GJB2/DFNB1 mutation spectrum in Russia: a founder Ingush mutation del(GJB2-D13S175) is the most frequent among other large deletions.

Although mutations in the GJB2 gene sequence make up the majority of variants causing autosomal-recessive non-syndromic hearing loss, few large deletions have been shown to contribute to DFNB1 deafness. Currently, genetic testing for DFNB1 hearing loss includes GJB2 sequencing and DFNB1 deletion analysis for two common large deletions, del(GJB6-D13S1830) and del(GJB6-D13S1854). Here, we report frequency in Russia, clinical significance and evolutionary origins of a 101 kb deletion, del(GJB2-D13S175), recently identified by us.

[Application of the mathematical model for prognosis in the rehabilitation of children after cochlear implantation].

Unlabelled: Despite the variety of etiological factors, cochlear implantation (CI) remains the only effective method for the rehabilitation of the patients presenting with total deafness. The aim of this study was the enhancement of the efficiency of selection of the candidates for CI, the improvement of the quality of rehabilitation of the patients with cochlear implants, and the determination of the prognostic criteria for clinical trials.

Patients And Methods: (CI).

[The Russian-language version of the matrix test (RUMatrix) in free field in patients after cochlear implantation in the long term].

Unlabelled: The deterioration of speech intelligibility in the patients using cochlear implantation (CI) systems is especially well apparent in the noisy environment. It explains why phrasal speech tests, such as a Matrix sentence test, have become increasingly more popular in the speech audiometry during rehabilitation after CI. The Matrix test allows to estimate speech perception by the patients in a real life situation.

[The analysis of acoustic activity unsynchronized with the stimulus with the use of TEOAE].

Unlabelled: The presence of ambient noise or the troubled child's behavior during the registration of transiently induced optoacoustic emission (TEOAE) can lead to false positive results at the first stage of hearing screening. The aim of this study is to evaluate the effectiveness of the noise reduction method based on weighted averaging of the data in the frequency and time slot, to check TEOAE, and to improve the effectiveness of universal hearing screening. Particular attention is paid to the analysis of the origin of noise recorded during the UAE.

[The impact of implantable technologies on the quality of life in the patients with hearing loss].

The improvement of the effectiveness of the rehabilitation of the patients with hearing disorders is a priority task for an audiologist. One part of the present work was to assess satisfaction of the patients treated with the use of the hearing aids, drug therapy, and the surgical operations. The aim of the study was to evaluate the influence of the implantation of the Baha bone conduction system on the quality of life of the treated patients.

[The role of computer and magnetic resonance imaging in patients with deafness].

Unlabelled: The objective of the present study was to evaluate the potential of CT and MRI for diagnostics of congenital and acquired pathology of the inner ear in the deaf patients. Two groups of the patients were examined. The first group consisted of 75 patients with congenital or acquired deafness etiology.

[Reoperations after cochlear implantation].

Unlabelled: The objective of the present study: the improvement of the quality and effectiveness of the surgical stage of cochlear implantation on the basis of the comprehensive analysis of performed reoperations.

Patients: 84 patients of the 2413 ones who had undergone cochlear implantation were re-examined by revision surgery within various periods after the first intervention.

Main Results: In certain patients, the correction of the intracochlear electrode array position was performed and in 72 cases the replacement of the implant was done.

[The history of cochlear implantation].

The creation of cochlear implants has become possible owing to the collaboration in the field of neurophysiology, otorhinolaryngology, audiology, engineering, and psychoacoustics. The experiments carried out in the 1930s were not directly associated with the electrical stimulation of the auditory nerve but had a significant influence on the development of cochlear implants. The first attempt at direct electrical stimulation of the auditory nerve was performed in 1957 using a device that consisted of an active electrode and an implantable induction coil.

Phenotype in a patient with p.D50N mutation in GJB2 gene resemble both KID and Clouston syndromes.

Keratitis-ichthyosis-deafness (KID) syndrome (OMIM 148210) is a rare ectodermal dysplasia syndrome characterized by vascularizing keratitis, congenital profound sensorineural hearing loss, and progressive erythrokeratoderma. We have found a 148G-A transition in the GJB2 gene, resulting in an asp50-to-asn (D50N) substitution in a girl with congenital deafness. This finding allowed us to diagnose а KID syndrome.