30 years of cochlear implantation in Russia

Aim: To discuss recommendations of the World Report on Hearing and World Health Assembly Resolution on prevention of hearing loss and deafness and prioritize the ear and hearing care. Results and Discussion: The results on prevalence of hearing loss and near-term prognosis are analyzed. In accordance with the WHO recommendations it is stressed that all people with hearing loss must have the approach to affordable and effective rehabilitation during the life-span. These approaches include hearing screening and early intervention prevention and management of ear diseases and hearing loss, access to modern hearing technologies, development of the rehabilitation services, improved communication, safe listening and active involvement of the society. Conclusion: The target indicators for the professional society and the national health systems are provided.

Cochlear implantation (CI) is one of the most effective methods of (re)habilitation of patients with profound sensorineural hearing loss and deafness. At present, in the Russian Federation, the number of patients using CI systems has significantly increased. The assessment of hearing and speech rehabilitation outcomes in CI patients is extremely relevant. Despite the technological advances in the CI industry over the past decades the poor speech intelligibility in noisy environments remains the issue. One of the most effective methods for speech intelligibility in noise assessment is speech audiometry in a free sound field using the international phrasal RUMatrix test.

Recently the knowledge about genes involved in the processes of sounds perception considerably extended. Confirmation of the hereditary reason of hearing loss in the child without hearing impaired relatives is possible only thanks to genetic analyses. Knowledge of the hereditary course becomes very important not only for identification, but also for rehabilitation of hearing impaired children. The purpose of this article is to present to speech therapists the common forms of hereditary hearing loss, the peculiarities of their identification and prediction of disease severity.

Identifying and resolving ethical dilemmas in the work of professionals caring for hearing-impaired children can serve as the basis for preventing occupational stress and burnout and improving the effectiveness of audiological care. Aim of the study: Ethical analysis of the professional activities of audiologists and speech-language therapists. Materials and methods: internet survey of 139 specialists (98 audiologists and 41 speech-language therapists) based on the «effort-reward» model. Results and discussion: the average indicator on the «Effort» scale in the group of audiologists was 5,5 ± 1,8 points, in the group of speech-language therapists – 5.9 ± 1,5 points. The average indicator on the «Reward» scale in the group of audiologists was 7,2 ± 1,9 points, in the group of speech-language therapists – 7,4 ± 2,8 points. Salaries are considered inadequate by 58% of audiologists and 22% of speech-language therapists, 59% of audiologists and 25% of speech-language therapists intend to change their job. The revealed tension is compensated by high respect among patients, management and colleagues. At the same time, the level of professional collegiality is higher among audiologists than among speech-language therapists. Conclusion: the analysis made it possible to identify ethical dilemmas in the work of audiologists and speech-language therapists.

Identification and resolution of ethical dilemmas in the implementation of cochlear implantation has practical interest for patients and their relatives, medical specialists and teachers. Goal: ethical analysis of different aspects of cochlear implantation according to regulatory documents. Results and discussion: the attitude towards cochlear implantation underwent certain changes in different conditions and time periods that requires a consensus of different parties. Conclusion: ethical support and legislation changed significantly for over a 30-year period of cochlear implantation in Russia and still remain an issue.

Auditory neuropathy spectrum disorder label incorporates patients with hearing loss of different etiologies and pathogenesis, but united under that label due to the presence of pre-neural cochlear responses such as otoacoustics emission and cochlear microphonics and absent or grossly abnormal neural responses – auditory brainstem responses are usually absent These electrophysiological data indicate that outer hair cells are preserved, but there are some lesions in the auditory pathway on the way from inner hair cells to brainstem. Different options for lesion localization predispose broad spectrum of clinical features and rehabilitation outcomes in these patients. Standard objective tests based on auditory brainstem responses measurement do not predict neither hearing thresholds nor optimal rehabilitation strategy for a child with auditory neuropathy spectrum disorder. Assessing behavioral hearing thresholds and sound discrimination abilities during testing provided by speech therapist are crucial for chousing optimal rehabilitation strategy for children with auditory neuropathy spectrum disorder.

The article concerns the children with impaired hearing and vision after cochlear implantation surgery. Topical issues of post-surgery development of such children are viewed through the lens of the theory and practice of cochlear implantation of deaf children without additional impairments. The necessity of careful consideration of contraindications to surgery, mandatory implementation of post-surgery psychological and pedagogical rehabilitation of children is emphasized. The authors present the arguments for a possibility of using the method of 3R-rehabilitation of a family and a child with CI by the specialists who mastered the new professional competencies.

The aim of the study was to describe a clinical case of medical and pedagogical rehabilitation of a deaf patient with CHARGE syndrome using cochlear implantation - a rare genetic disease with numerous congenital malformations caused in most patients by mutations in the CHD7 gene. A patient observed at the National Research Centre for Audiology and Hearing Rehabilitation with a diagnosis of bilateral deafness and was operated on the left ear with cochlear implant Nucleus CI 512 (Cochlear, Australia) at the age of 1 year 4 months. The difficulties of the surgical stage were caused by multiple anomalies of the middle and inner ear with the dystopia of the facial nerve. A navigation system and facial nerve monitoring were applied during the surgery. Difficulties of postoperative rehabilitation were associated with manifestations of the syndrome, which led to a delay in intellectual and physical development. The speech processor fitting was carried out jointly with the speech therapist based on the registration of objective datas and subjective reactions of the child. Speech therapy were organized individually and systematic. After 3 years and 4 months from the surgery, we observe the child's reactions to non-verbal, speech stimuli and whispers at a distance of 6 m, onomatopoeia, separate words, a short phrase, understanding of simple questions and assignments. Patients with CHARGE syndrome can be effectively rehabilitated by cochlear implantation, taking into account all the risks of surgery and based on the predicted results.