[Clinical protocol: audiological assessment of infants in Russian Federation. Part II]. / Protokol audiologicheskogo obsledovaniya detei pervogo goda zhizni v Rossiiskoi Federatsii. Chast' II.

This is the second part of the previously published clinical protocol of audiological assessment in infants. The goal of the protocol is unification approaches to audiological diagnosis of the infants. The following sections were included in the second part of the protocol: behavioral testing in infants, testing sequence, duration of the examination and necessity in follow-up, hearing assessment in special cases (premature children, children with congenital infections, after meningitis, with external ear abnormalities, single-sided deafness, with hydrocephalus and shunts, with auditory neuropathy spectrum disorder, with mild hearing loss and otitis media with effusion), medical report.

[Clinical protocol: audiological assessment of infants in Russian Federation. Part I]. / Protokol audiologicheskogo obsledovaniya detei pervogo goda zhizni v Rossiiskoi Federatsii. Chast' I.

The clinical protocol of audiological assessment in infants was prepared by the workgroup of Russian pediatric audiologists from different regions. The goal of the protocol is unification approaches to audiological diagnosis of the infants. The protocol has been developed according the evidence based medicine principles, by reviewing current scientific publications on the topic and taking into account the order of providing medical services and other clinical practice guidelines. When direct evidence was not available, both indirect evidence and consensus practice were considered in making recommendations. This guideline is not intended to serve as a standard to dictate precisely how the child should be diagnosed. This guideline is meant to provide the evidence base from which the clinician can make individualized decisions for each patient. The first part of the protocol covers following sections: equipment, staff requirements, timing of the diagnostics, case history and risk factors, preparing the child for the appointment, sedation and general anesthesia, otoscopy, tympanometry and acoustic reflex, otoacoustic emissions, skin preparing, electrode montage, choosing the stimulators, auditory brainstem responses on broadband and narrow-band stimuli, on bone conducted stimuli, auditory steady-state responses, masking, combined correction factors.

[Hearing loss due to mutations in the genes responsible for Usher syndrome]. / Narushenie slukha pri mutatsiyakh v genakh, otvetstvennykh za sindrom Ashera.

Usher syndrome is characterized by congenital bilateral sensorineural hearing loss and progressive retinitis pigmentosa, and has an autosomal recessive type of inheritance. The purpose of this work is to summarize the modern data of a clinical picture of Usher syndrome and analyse hearing impairment properties. The frequency of the syndrome among children suffering from hearing loss and deafness is from 3 to 10%. The prevalence of the syndrome in the world is estimated as 4.4 per 100.000 population. The complexity of the diagnosis of the syndrome lies in the significant clinical and genetic heterogeneity. Hearing and vision problems begin at different ages. Primary diagnosis begins with the clinical diagnosis of bilateral hearing loss and visual impairment manifests later. In this case the initial diagnosis of nonsyndromal hearing loss will not be definitive. Molecular genetic studies contribute to the early clinical diagnosis of the syndrome. Understanding the cause of the disease allows to conduct correct medical and genetic counseling and get closer to solving treatment problems.

[Ethical analysis of work environment in audiology]. / Analiz eticheskikh aspektov trudovoi deyatel'nosti vrachei-surdologov-otorinolaringologov i vrachei-otorinolaringologov.

The issues of medical ethics concern not only the doctor-patient relationship, but also the ethical aspects of the organization of the labor activity of medical specialists. Identifying and resolving ethical dilemmas can serve as the basis for preventing occupational stress and burnout and improving the effectiveness of medical care in the field of audiology. OBJECTIVE: Ethical analysis of work environment in audiology. MATERIAL AND METHODS: A 14-question questionnaire developed on the basis of demand-control and effort-reward models, an online survey was conducted using the Google Forms service among 111 specialists (43 audiologists, 13 otorhinolaryngologists, and 55 doctors combining work in both specialties).Results and discussion. The differences were revealed on the scales Effort and Reward depending on the specialty and clinic (public or private). The greatest effort was found among doctors combining both specialties, the least - among doctors of private clinics. 58% of the respondents consider the wages to be inadequate to the expended efforts. At the same time, a high respect level among patients, management and colleagues helps to compensate for the identified efforts. CONCLUSION: The analysis made it possible to identify ethical dilemmas in the work of audiologists for effective planning of specialty development.

[Epidemiology of hearing loss in children of the first year of life]. / Épidemiologiia narushenii slukha sredi detei 1-go goda zhizni.

The aim of this study was the investigation of the epidemiology of permanent hearing impairment in the children of first year of life in the Russian Federation after the implementation of the newborn universal hearing screening program. The prevalence of hearing loss in children in the first year of life was estimated at 2.5 per 1,000 based on the official statistical data and reports of hearing rehabilitation centres in 2016. A cohort of 405 children born in 2012 was examined at the age from 0 to 4 years of life. Among them 276 children were diagnosed with permanent congenital and prelingual hearing loss. 88% of the cases were bilateral, sensorineural hearing loss confirmed in 84% of the cases. The genetic cause of hearing loss was revealed in 58% of the patients assessed for the presence of GJB2 gene mutations. In preterm infants, the permanent hearing loss was detected in 70% of the cases. The comprehensive audiological assessment before 3 months of life was conducted only in 32% of the children; this finding is not consistent with the international newborn hearing screening recommendations. Only 70% of the cases of congenital and preverbal hearing loss were diagnosed during the first year of life.

[Changes of the auditory function in he children with chronic kidney failure]. / Izmeneniia slukhovoi funktsii u detei s khronicheskoi bolezn'iu pochek.

The risk factors that contribute to the development of hearing loss in the children with chronic kidney failure (CRF) are complications and/or manifestations of the disease itself as well as the side effects of its treatment. There are many similarities between the anatomical, physiological, and pharmacological features of the nephron and the striavascularis of the cochlea. The objective of the present study was the evaluation of the status of the auditory function in the children presenting with CRF. MATERIAL AND METHODS: A total of 62 children with CRF were examined. 40 patients were on peritoneal dialysis and 9 on hemodialysis whereas 13 children did not receive hemodialysis. Kidney was transplanted in 7 patients. The registration of transient evoked otoacoustic emission (TEOAE) was performed in all the children. For patients without TEOAE complex underwent the audiological examination. RESULTS: The TEOAE was not registered in 17 of the 62 examined children. According to the results of the comprehensive audiological examinations, 13 children suffered from the hearing loss of different forms and severity while 5 children presented with bilateral deafness. As the duration of CRF progressed, the patients became more prone to develop hearing impairment. Hearing loss is possibly genetically determined. Among the etiological factors underlying the development of the hearing loss the high doses of immunosuppressants and aminoglycosides are considered to be the principal ones.

[The Russian and international experience with the implementation of the programs of universal audiological screening of the newborn infants].

The problem of diagnostics of congenital hearing impairment has acquired special importance in the light of new possibilities for the early rehabilitation of the patients presenting with this condition. The implementation of the programs of universal audiological screening into the clinical practice of Russia and many other countries made it possible to significantly reducethe time necessary to confirm congenital impairment of hearing and begin the rehabilitative treatment. The present paper was designed to analyze the international experience with the implementation of the programs of universal audiological screening of the newborn infants as exemplified by such countries as Great Britain, USA, Germany, and Poland. The main indicators of the quality and the efficiency of such programs are considered taking into account the results of the epidemiological studies on the prevalence of congenital hearing impairment. A total of 1.8 mln newborn infants were examined in Russia during 2013. The first stage of screening involved 96.7% of the children, and only 2.9% of them remained uncovered by the examination. As many as 5,659 children were found to present with the congenital loss of hearing,with the prevalence of this condition being 3 per 1.000 newborn infants and the prevalence of deafness 0.6 per 1.000. The principal problem to be resolved for the organization of the management of these patients, both in Russia and other countries, remains the enhancement of the availability of comprehensive diagnostic examination and the timelyreferral of the patients to such examination (if appropriate based on the results of the screening). The successful solution of this problem requires personalized recording of the screening data with the use of the commonly accepted medical information systems.

[The results of cochlear implantation in the patient with hereditary and non-hereditary hearing loss]. / Rezul'taty kokhlearnoi implantatsii u patsientov s nasledstvennymi i nenasledstvennymi formami tugoukhosti.

The objective of the present study was to evaluate the effectiveness of rehabilitation of the patients after cochlear implantation in the early and late periods after operation taking into consideration the etiology of congenital deafness. The comprehensive clinico-audiological examination performed during the period from 2010 to 2015 involved 246 children who had undergone cochlear implantation (CI). All children were operated at the National Research Center for Audiology and Hearing Rehabilitation in the period from 2003 to 2013. 83 (56%) patients were aged 1 to 3 years at the time of surgery. Their age varied from 3 to 18 years when they underwent the clinico-audiological examination. Thus, the study is based on the experience with cochlear implantation varying from 3 to 12 years. The genetic analysis revealed mutations in the GJB2 gene in 49% of the children, in agreement with the data of earlier studies. 85% of all the children with GJB2 deafness surgically treated at the age under 4 years attend ordinary institutions of learning. Within 24 months after the onset of the observations the majority of the children with hereditary deafness (63%) were referred to the groups with good and excellent results of the rehabilitation and only 6 (12%) patients presented with unsatisfactory results. It was shown that the acquired causes of the loss of hearing including severe prenatal pathology have a negative influence on the long-term outcomes of rehabilitation. The results of the genetic analysis for the elucidation of the cause of impaired hearing can be employed as a prognostic criteria not only for the prediction but also for the guarantee of the success of cochlear implantation provided the rehabilitative process was initiated in a proper time.

[Cardiac rhythm disturbances in the children with hearing pathology]. / Narusheniya serdechnogo ritma u detei s patologiei slukha.

The aim of the study was to identify cardiac arrhythmias in children presenting with hearing impairments. Hearing loss in the children can occur concurrently with the disturbances of the cardiac rhythms. The detection of this pathology at the diagnostic stage is as important as it is at the stage of rehabilitation including surgical one. PATIENTS AND METHODS: A retrospective analysis of 100 patients (55 boys and 45 girls of the mean age of 4.8 years) operated in 2013 was made. RESULTS: Cardiac arrhythmias and conduction were recorded on the electrocardiograms obtained from 10 patients (4 boys and 6 girls) of the 100 examined ones, which amounted to 10%. The hereditary long QT syndrome (including Jervell-Lange-Nielsen syndrome) has not been identified. CONCLUSION: The timely diagnostics of congenital arrhythmias in the children with hearing impairment makes it possible to significantly reduce the risk of the sudden cardiac death (for example, in the patients presenting with the Jervell-Lange-Nielsen syndrome) and facilitates the choice of the anesthesiological support for the surgical treatment (including cochlear implantation) taking into consideration possible cardiac rhythm disturbances.

[Hearing disorders in the children presenting with various chronic diseases].

The objective of the present study was to improve the effectiveness of early diagnostics and prophylaxis of hearing disturbances in the children presenting with various chronic diseases. As is known, there are several dozens of neurologic disorders associated with hearing disorders. By way of example, there is a group of nephropathologies responsible for the loss of hearing in the children. Specifically, hearing impairment can be one of the symptoms of diabetes mellitus. The patients presenting with mucopolysaccharidosis make up a group at risk of hearing impairment. Sensorineural loss of hearing is widespread among the children presenting with coeliac disease. The protocols for the treatment of certain pathologies envisage the application of certain medications possessed of the cytotoxic activity, such as preparations for chemo- and radiotherapy or cytostatic agents that suppress cell proliferation when applied for the management of some autoimmune diseases. It is concluded that cooperation between health providers representing different medical disciplines may be instrumental in the organization of efficacious screening for the detection of children with severe chronic pathology.

[The universal audiological screening of newborn infants: achievements and challenges].

The objective of the present study was to estimate the effectiveness of universal audiological screening of newborn infants in Russia based on the results of this procedure obtained in 2011-2012 by the analysis of the activities of surdological centres in 15 regions of the country. It was shown, that the main indicators of the effectiveness of the screening are the technical equipment of maternity houses, newborn coverage at the first stage of screening, and its continuity. The study revealed 3.14% of the infants who failed to be involved in the first stage of screening. Hearing impairment was diagnosed in two of each 1,000 newborn infants at the second stage of screening, the frequency of severe forms of hearing impairment was estimated as three cases per 10,000 infants. The disadvantages of the current system of identification of newborn infants suffering congenital loss of hearing are discussed. The importance of the improvement of data collection methods is emphasized.

[The prevalence of hearing impairment among the elementary school pupils in the city of Sana, the capital of Yemen].

The present study was designed to estimate the prevalence of hearing impairment among the elementary school pupils. The portable tone audiometer in conjunction with otoscopy was used to examine 2.200 children aged from 6 to 9 years attending 12 elementary schools in the city of Sana. The children with negative results of the tests were referred to the University Clinic for the more comprehensive examination. Risk factors were revealed by the questionnaire study involving the parents. As many as 11.6% of the total number of children failed to pass the test. Their number decreased to 10.6% after cleaning the external auditory canal. Conductive hearing impairment was the commonest pathological condition while exudative otitis was its most frequent cause (6% of all cases). The prevalence of hearing impairment among the elementary school pupils in Yemen was shown to exceed that in the developed countries. The high risk of development of this pathology is attributable to the low socio-economic living standards. The importance of audiological screening of elementary school pupils is emphasized.

[Role of electrocochleography in identification of labyrinthine hydrops]. / Rol' élektrokokhleografii v identifikatsii gidropsa labirinta.

We studied potentialities of extratympanic electrocochleography (ETECG) in detection of labyrinthine hydrops (LH) in 50 patients who had an audiological picture of unilateral neurosensory hypoacusis. The patients were divided into two groups: group 1 patients suffered from attacks of systemic vertigo typical for Meniere's disease; group 2 patients had no vestibular complaints. ETECG results were compared to the dynamic range, results of otoacoustic emission and brain stem response, vestibulometry with electronystagmography (data of bithermal caloric test as most informative). The comparison indicated advantages of ETECG used alone and in combination with other tests.