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COVID-19 is an acute respiratory coronavirus infection in 2019 caused by the SARS-CoV-2 virus. Currently, the number of neurological complications in the acute or delayed period of coronavirus disease is increasing, including peripheral disorders of the auditory analyzer., Objective: To present clinical cases of sensorineural hearing loss in children under 5 years of age after a novel coronavirus infection., Material and Methods: We report 3 cases of unilateral and 1 case of bilateral acquired deep sensorineural hearing loss, while the association with SARS-CoV-2 has been confirmed anamnetically and/or laboratory., Results: The SARS-CoV-2 virus can have a depressing effect on the cochlea on its own or enhance the toxic effect of viruses during the recovery period after COVID-19. The true frequency of acute sensorineural hearing loss of infectious origin in childhood and, as its outcome, the formation of persistent hearing impairment has not been determined., Conclusion: Viruses are volatile, contagious, and clinically dangerous due to their complications. Vaccination is the most effective measure for the prevention of infectious diseases.

This article describes a rare case of necrotic xanthogranuloma in a 46-year-old patient who presented with the development of periorbital xanthelasms, progressive bilateral sensorineural hearing loss and bilateral vestibulopathy, followed by multiple myeloma and amyloidosis. For several years, the patient underwent standard rehabilitation for chronic sensorineural hearing loss and was fitted with a hearing aid. During hospitalisation for exacerbation of chronic bronchitis, monoclonal gammopathy was identified, and later, after careful examination and repeated biopsies, necrotic xanthogranuloma, multiple myeloma and AL-amyloidosis were confirmed. Targeted immunochemotherapy resulted in improvement of hearing and significant recovery of the vestibuloocular reflex bilaterally.

Background: Cystic fibrosis (CF) is a severe hereditary disease with a multisystem lesion. Manifestations of CF include severe infectious purulent lesions of all parts of the respiratory tract, including purulent rhinosinusitis with nasal polyps. The involvement of the sinonasal region and the need for systemic use of ototoxic drugs (primarily aminoglycosides to treat resistant bacterial infection) potentially create a risk of both conductive and sensorineural hearing loss (SNHL). The available data on the epidemiology of hearing disorders in CF is contradictory. Currently, genetic determinants of the development of aminoglycoside SNHL have been identified., Material and Methods: For 136 CF patients (75 girls, 61 boys) aged 3 to 17 (9.4±3.9) years were performed audiological examination: tympanometry, transient-evoked otoacoustic emission and the pure tone threshold audiometry (standard frequency range) ( n =126). History of systemic therapy with aminoglycosides was evaluated for each patient. Sequencing of c.35delG mutations in the GJB2 gene (nuclear DNA) and A1555G in the 12S rRNA gene (mitochondrial DNA) was performed in 215 patients with cystic fibrosis (the group partially overlaps with the audiological group), and as a control - 106 children with bronchial asthma and 103 healthy children, their age ranged from 3 to 17 (8.8±3.8) years., Results: Audiological examination of CF children reveled a prevalence of conductive hearing loss comparable to the general population (2.4%). The frequency of SNHL was 1.6%, wich exceeds that of non-CF children. A genetic study revealed one case of heterozygous carriage of the c.35delG mutation in the GJB2 gene in a patient with bronchial asthma. In the group of patients with CF ( n =215), mutations in the connexin 26 gene were not detected. No A1555G mutation was detected either in the group of patients with CF or in the control groups., Conclusions: Children with CF are at risk for the development of sensorineural, but not conductive hearing loss. Routine total screening for A1555G and c.35delG mutations probably seems not to be recommended.

The hearing loss is one of the significant problems in the world. The disease can develop both for genetic reasons and under influence of external factors at work. The noise is the main indicator of occupational risk of hearing loss. Due to influence of noise and vibration, in certain crew members of water vessel develops professional sensorineural hearing loss that subsequently prevents signing of employment contract and continuation of work at sea. This circumstance confirms actuality of stated problematic. The article presents types of sensorineural hearing loss depending on classification criterion. The features of professional activities of seafarers in conditions of strong noise exposure are revealed. The methods of identifying hearing defects in ship crew members are determined. The advisable measures to protect hearing health and to prevent deafness are formulated.

The peculiarities of cochlear implant (CI) processor fitting in children with auditory neuropathy spectrum disorders (ANSD) were investigated. At the 1-st fitting of the CI processor a standard protocol of parameters was used in all patients, including patients with cochlear nerve hypoplasia. After the initial fitting session, the behavioral tonal thresholds with CI in 55% of patients were 30-35 dB, in 32.% of patients - 40-50 dB. After 3-6 months, 65% of children with ANSD showed significant progress in auditory-speech development, which made it possible to use the standard protocol of tuning parameters for them with the most comfortable and threshold levels of electrical stimulation adjusted according to the child's reactions. The best dynamics was observed in 2 children with presynaptic ANSD with a confirmed DFNB9 ( OTOF ) gene mutation. In 35% of children, there was no progress in distinguishing speech signals and instability of reactions to sounds persisted after 6 months using of CI and speech therapy training, despite the low tonal thresholds of hearing. In these children the coding strategy was changed, the stimulation frequency was reduced, and the pulse width was increased. This helped to improve the discrimination of sounds with CI and progress in the child's speech development. The results demonstrate that children with ANSD require more frequent correction of CI processor settings: 1st year - every 3 months, then at least 2 times a year until the optimal coding strategy and settings are achieved. To predict the effectiveness of CI and determine the optimal tactics for setting up the CI processor in patients with ANSD, the preoperative examination should include MRI of the cerebellopontine angle to detect anomalies of the cochlear nerve and genetic examination to identify mutations that cause hearing impairment in patients with ANSD.

Congenital anomalies in the inner ear structures development are one of the reasons for unilateral hearing loss in children. Unilateral hearing loss is predominantly congenital, thus children with this pathology are initially deprived of the ability to hear with both ears, which leads to the formation of a specific «individual hearing norm». Due to this, children do not complain of hearing loss, and unsuspecting parents can only guess why their child is poorly studying and lagging behind his peers. Unfortunately, in some situations the detection of malformations of inner ear structures occurs only after the development of complications, namely spontaneous otoliquorrea, leading to bacterial meningitis, and exactly episodes of meningitis (sometimes recurrent) become the cause for examination of patient and diagnosis of this pathology. We present one of this clinical observations.

The article presents modern literary data relating to the expediency of the purpose of glucocorticosteroids in sudden sensorineural hearing loss (SSHL) of various genes. In detail, the radar molecular mechanisms and the anatomo-physiological features of the exposure to the inner ear, side effects, the introduction methods, their comparative efficacy and modern schemes of the SSHL.

The aim of the study was a comparative analysis of subjective assessments and audiological parameters of patients with hearing loss obtained before and after the course of transcranial electrical stimulation (TES). The study group included 99 patients (45 men and 54 women) with chronic mono- (16%) and bilateral (76%) sensorineural hearing loss (SNHL) aged 20 to 85 years. Groups of young and middle age (20-59 years old) amounted to 37 people; elderly (60-74 years old) - 48 and senile (75-90 years old) - 14 people. The observation included: anamnesis, ENT survey, tone threshold audiometry, impedancemetry, and questioning. All patients underwent a course of 10 TES procedures using the «Transair-7» device. The data indicated an improvement in subjective assessments regarding auditory perception in 54%, speech intelligibility in 53%, tinnitus in 24% and general well-being in 36% of patients in the study group. The distribution of hearing thresholds in the region of speech frequencies before and after the course also turned out to be significantly different (p<0,01). At the same time, gender and age-specific features of the effect of TES on hearing and general well-being were revealed, the most pronounced in senile patients who showed minimal positive dynamics. In general, taking into account age restrictions, the results confirm the feasibility of using TES in SNHL, even outside the combined (with audio loading) stimulation.

Modern literature data are presented on the choice of a drug for hormonal therapy in acute neurosensory hearing loss of various origins, the doses used for systemic therapy, the features and methods of intratympanic administration of glucocorticosteroids, and the evaluation of the effectiveness of treatment with this group of drugs.

A survey of 48 victims aged 19-36 years with explosive trauma and combined damage to the auditory system was conducted to assess the level of damage to nerve structures by analyzing the bioelectric activity of the cerebral cortex. All patients underwent electroencephalography (EEG). It is established that akubarotrauma of explosive genesis almost always leads to lesions of the function of the cortical part of the auditory analyzer. Desynchronized activity on the EEG after acubarotrauma is a favorable prognostic sign, indicating only functional disorders of the cortical part of the auditory analyzer. On the contrary, EEG changes of an organic type of cortical or stem nature are an unfavorable prognostic factor, usually accompanied by sensorineural hearing loss with prolonged and incomplete hearing recovery. Promising drugs for the treatment of otoneurological disorders are antihypoxants, in particular, derivatives of triazine indole, which affect the molecular mechanisms of hypoxia development.

After a brief discussion of the problem of idiopathic sensorineural hearing loss and perilymphatic fistulas of the labyrinth windows, audiometric tests are identified that are informative for the preoperative diagnosis of the latter. Taking into account the own results of surgical treatment, attention is drawn to the feasibility of a wider use of exploratory tympanotomy in cases of suspected occurrence of perilymphatic fistulas of the labyrinth windows.

Study of the role of the phenotypic composition of lymphocytes, shifts in which can cause the development of various pathological processes, including sensorineural hearing loss, is an important area of modern research. Purpose of the study. To study the population and subpopulation composition of lymphocytes and their intercellular cooperation in persons with occupational sensorineural hearing loss., Material and Methods: Phenotyping of blood lymphocytes (CD3+, CD4+, CD8+, CD9+, CD16+, CD20+, CD21+, CD23+, CD25+, CD95+) was performed by indirect immunofluorescence., Results: Individuals with sensorineural hearing loss were found to have hyperactivation in both T- and B-cell links immunity., Conclusions: The revealed changes indicate a violation of immunoregulation through factors of intercellular cooperation, and the established relationships between populations and subpopulations of lymphocytes indicate increased activity components of the immune system, their important role in the implementation of the immune response in persons with sensorineural hearing loss.

Objective was to study single-nucleotide polymorphisms (SNP) in CAT, NCL, HSPA1L, PCDH15, and PON2 genes and their associations with hearing impairment among the people working among noise-exposed workers of the mashine-building plant (JSC «Krasmash», Krasnoyarsk, Eastern Siberia, Russia)., Materials and Methods: The 443 employees of Krasmash JSC, who have been working under conditions of increased noise for at least 1 year, were surveyed and examined. A hearing study was performed by speech and tonal audiometry. Tonal audiometry was carried out in accord with according to a standard method in the frequency range 125-8000 Hz. People with chronic hearing impairment, survivors of meningitis and family history of hearing impairment were excluded from the study. The allelic composition of the studied genes was determined in the remaining group of 288 workers (study group). Polymorphisms were detected using bioluminescent method, developed by the authors earlier. The study group comprised 122 people with hearing impairment (experimental group) and 166 people without impairment (control group)., Results: The genotyping results of on allelic variants rs494024 ( CAT ), rs7598759 ( NCL ), rs2227956 ( HSPA1L ), rs7095441 ( PCDH15 ) and rs7785846 ( PON2 ) showed that their frequencies in the study group did not differ and were comparable with those for the European population. No statistically significant differences were revealed in the distribution of the genotypes of the studied mutations between the experimental and control groups. Also no statistically significant associations we found between hearing impairment and availability of two or several SNPs, or these SNPs and clinical characteristics of the disease (degree of hearing impairment, tinnitus). In the group of workers with an experience of 5 to 16 years, an association was found for hearing impairment and SNP rs494024, as well as when it is combined with rs7598759., Conclusions: The associations between SNP rs7598759, rs2227956, and rs7095441 and hearing impairment were not found. In the group of workers with 5-16 year experience, this association was found for SNP rs494024, as well as when it is combined with rs7598759. Discovered associations require further study.

Spatial and speech characteristics of hearing are needed to monitor the rehabilitation of sensorineural hearing loss in patients of older age groups. Using the created «Program for the assessment of speech, spatial and qualitative characteristics of hearing using virtual reality» increases the level of hearing diagnostics using a computerized audiovisual script. The purpose of the study is a comparative analysis of the speech, spatial and qualitative characteristics of hearing before and after using virtual reality in patients of older age groups. The results of the study showed that with good tolerance of virtual reality in 48.3% of patients, the answers to the questions of the SSQrus-12 questionnaire for assessing the spatial and speech characteristics of hearing became more objective. The developed methodology supplemented the group of modern diagnostic methods for spatial and verbal hearing by immersing the patient in a virtual environment in the created audiovisual scenario.

The Aim of the Study: To assess speech recognition in users of mono- and binaural hearing aids (HA) with dynamic environment control system (DECS)., Material and Methods: 25 HA users with symmetrical bilateral moderate to severe sensorineural hearing loss and 10 individuals with normal hearing underwent speech audiometry in a free sound field by listening to an adaptive Russian matrix sentence test with a background noise. The testing was conducted for hearing impaired patients with one and two HA. The Speech Recognition Thresholds in noise (SRT N ) using HA with DESC and using HA without DESC were compared. The dichotic digits test and the rapidly alternating speech perception test were performed to evaluate the central auditory system function., Results: The SRT N in normal hearing listeners was -16.4±1.9 dB SNR. In the case of monaural using HA without DESC the SRT N was -2.5±4.4 dB SNR, using HA with DESC it was -5.8±4.0 dB SNR; in the case of using binaural HAs it was -6.3±3.8 dB SNR and -9.9±3.1 dB SNR accordingly., Conclusions: HAs with Dynamic Environment Control System are highly effective for speech perception in noisy environments. Binaural HA use is more effective than monaural HA use regardless of HA model. Speech recognition in noisy environments in cases of binaural HA use correlates significantly with results of tests assessing the central auditory system function.

Objective: To study the effectiveness of auditory training using virtual reality technologies in people with chronic sensorineural hearing loss in dynamics., Material and Methods: The study included 68 patients aged from 24 to 97 years (mean age 57.63±15.87 years) with latent hearing loss (hearing loss at high frequencies), I degree of hearing loss and its variations. Auditory training was carried out on the basis of the Diagnostic and Rehabilitation System for spatial and speech hearing based on virtual reality using various audiovisual scenarios ReviAudio and control of speech perception, spatial hearing and hearing quality using the SSQRus questionnaire., Results: A comparative analysis of the training results using the ReviAudio software revealed positive trends in the patient's response time and the patient's accuracy in determining the direction of the target signal, regardless of the symmetry and asymmetry of hearing loss. Patients with asymmetric hearing impairment observe an improvement in spatial hearing parameters, patients with symmetric hearing loss - speech perception and hearing quality, patients with latent hearing loss report an improvement in the quality of hearing. The auditory training is effective in individuals with mild chronic sensorineural hearing loss and in individuals with latent hearing loss as an adjunctive treatment.

To determine the electrode impedance dynamics changes in the postoperative period after cochlear implantation as well as the time of their stabilization., Material and Methods: 75 patients with a bilateral sensorineural deafness aged from 1 to 4 years (average age - 1.5 years) were included in the study. 50 patients were implanted with Nucleus cochlear implants (Cochlear - Australia) and other 25 patients - with Advanced Bionics implants (Switzerland)., Results: The impedance dynamics analysis in two groups of patients implanted with «Cochlear» and «Advanced Bionics» cochlear implants demonstrated that statistically significant decrease in impedance ( p <0.05) was observed after the beginning of electrical stimulation and the reduction of postoperative inflammatory process in the inner ear. The stabilization of the impedance levels in both groups of patients was obtained in 3-6 months after the switch-on of speech processor.

The article presents a review of the literature on the treatment of acute sensorineural hearing loss, acoustic trauma in people exposed to impulse noise. Groups of the most used drugs for the pharmacological correction of hearing impairment in this pathology are present, such as glucocorticosteroids, antioxidants, nootropics, antihypoxants, and others, are covered. Particular attention is paid to use of drugs taking into account the main links of pathogenesis.

Studies of the recent years are devoted to using the auditory steady-state responses (ASSR) as an evaluation method of amplification outcomes. In this case stimulation is performed in the sound field via loudspeaker with a hearing aid (HA) in patient's ear., Goal: Estimation the optimal stimulation parameters of ASSR in free field, which provide maximum correlation with behavioral thresholds., Materials and Methods: 26 subjects were included in the study: 10 with normal hearing (4 adults and 6 children) and 16 children (1-16 years old) with sensorineural hearing loss (SNHL). Aided and unaided ASSR thresholds were compared with corresponding behavioral thresholds (BT). All recordings were conducted in free sound field. We used mono-frequency stimulation method at 500-4000 Hz career frequencies. Modulation frequency was set as 40 Hz. Types of stimuli modulation: AM/FM modulation, exponential modulation (AM 2 ), three carrier frequencies and modulated Chirp-tone (Chirp)., Results: In normal hearing subjects the difference between ASSR and BT varied from 0 dB to 35 dB, and the significant correlation between them was observed ( p <0.05). The maximum difference between ASSR and BT in children with SNHL was at 500 Hz (15.6±4.3 dB), minimum - at 2 and 4 kHz (8±2.9 dB and 7±3 dB respectively). In aided condition maximum difference was detected at 500 Hz (10.2±3.8 dB), minimum - at 1 and 2 kHz (7.8±2.8 and 7.3±3.1 dB respectively). We got significantly less difference between ASSR and BT in all test conditions using following types of stimuli: at 500 Hz and 1000 Hz - AM 2 ( p <0.01); at 2 and 4 kHz - Chirp ( p <0.05 and p <0.01 respectively). Thereby, free field ASSR might be used as an objective method of evaluation the amplification outcomes in children with SNHL.

The objective of the present study was to elucidate the specific features of the clinical course of acute otitis media as well as the peculiarities of the vestibular function and the microbial paysage associated with this pathological condition under the present-day conditions. The study included 135 patients presenting with acute otitis media (AOM) at different stages of the disease. The discharge obtained from the tympanic cavity of all the patients was examined with the use of polymerase chain reaction in real time, audiological and vestibulogical methods. The distinctive features of acute otitis medium associated with Streptococcus pneumoniae infection were found to be the intense pain syndrome with the symptoms of intoxication, well apparent inflammatory changes in the tympanic membrane as revealed by otoscopy, the increased frequency of sensorineural impairment of hearing, and the characteristic type B tympanometric curve. Typical of AOM associated with Haemophilus influenza infection are the mild pain syndrome, weak changes in the tympanic membrane as revealed by otoscopy, conductive hearing loss, and the type C tympanometric curve.

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 objective of the present study was to estimate peculiarities of the auditory brainstem evoked potentials (ABR), auditory steady-state responses (ASSR) and cortical auditory evoked potentials (CAEP) in the children presenting with bilateral auditory neuropathy spectrum disorder (ANSD). The study included 100 patients with bilateral ANSD diagnosed based on the positive response of otoacoustic emissions (OAEs) and/or cochlear microphonic (CM) detection, while no synchronous neural activity was detected in the ABR test. Cochlear microphonic was the main clue for the ANSD diagnosing, because OAE was absent in both ears of 49 children. ABR testing revealed no response bilaterally in 72 cases (out of 100). In contrast to ABR, the ASSR thresholds were detectable at all the four main frequencies in both ears in 73 % of the cases (47 out of the 64 tested ones). Both ABR and ASSR in most cases were incomparable with the behavioral audiometric thresholds. 28 children underwent CAEP testing. In 7 cases out of 8 with mild hearing loss detectable CAEP were recorded. CAEP registration in l7 children making use of the hearing aids and in 3 children after cochlear implantation revealed, in the majority of the cases, the concordance between CAEP detectability with behavioral thresholds and rehabilitation outcomes with fairly good speech intelligibility. It is concluded that the ABR registration with CM evaluation is the most informative test for ANSD diagnosis. However, ABR as well as ASSR is useless for the estimation of the behavioral thresholds. The results of this study suggest that the presence or absence of CAEPs can provide some indication of the audibility of a speech sound in the children with ANSD; however this method requires further investigation.

The relevance of the problem of a sensorineural hearing loss (ASNL) arises from the necessity of the special approaches to the diagnostics of this condition, the complications accompanying this pathology, and the difficulties encountered in the implementation of the methods designed for hearing rehabilitation of such patients. The objective of the present study was to estimate the prevalence of ASHL among the adult population. The sensorineural impairment of hearing was diagnosed in a total of 2456 (72%) examined patients presenting with hearing loss. To determine the presence of asymmetry of sensorineural hearing loss, we employed three counting techniques allowing (1) to calculate the difference between the average hearing thresholds at four frequencies within the range from 0.5 to 4 kHz (the difference was found to be 15 dB or more in 17% of the patients), (2) to calculate the difference between the degrees of hearing loss in the right and left ears (the difference was documented in 47% of the patients), (3) to calculate the difference between the hearing thresholds at least at a single frequency within the range from 0.125 to 8 kHz (he difference was found to be 15 dB or more in 71% of the patients). When using the third method to characterize asymmetry of hearing impairment, it was identified in most patients (53%) at one or two frequencies. Moreover, there was a large number of the patients (13%) with asymmetry apparent over the entire frequency range. A high degree of threshold asymmetry (in excess of 40 dB) was more often noted in the mid-frequency range. In the majority of the patients, the asymmetry manifested itself as the different degree of bilateral sensorineural hearing impairment (51%) or unilateral sensorineural hearing loss with the normal hearing ability preserved in the contralateral ear (35%). The results of the present study give evidence of the necessity of developing a reliable method for the identification of clinically significant ACNL.

The objective of the present study was to evaluate to what degree the function of the central auditory pathways influences the intelligibility of the speech in the patients suffering from the sensorineural loss of hearing (SNLH). The study included a total of 20 patients at the age varying from 31 to 80 years presenting with moderate to moderately severe symmetrical SNLN. All the patients were permanent hearing aid users. They underwent the standard audiological examination including pure-tone threshold and suprathreshold audimetries together with impedancemetry, the evaluation of the functional ability of the central auditory pathways with special reference to temporal resolution and differential frequency sensitivity, binaural integration, and the ability to distinguish a sound stimulus against the background noise. It has been found that the impaired intelligibility of the speech and the decreased efficiency of the hearing aids in the patients presenting with SNLH were attributable to the central auditory pathway disorders detected in 50% of the participants in the study. The strong correlation between the results of detection of the stimulus under conditions of tonal signal interference, the binaural integration test, and intelligibility of the speech in the users of hearing aids was documented.

This article describes the main clinical features associated with the development and manifestations of disturbed sound perception in the members of the aeronautical personnel engaged in the Russian civilian aviation. The main expert criteria for the relationship between the diseases of the organs of hearing (as exemplified by chronic sensorineural hearing impairment) and professional occupation have been developed based on the results of the clinical and diagnostic examination with the use of whispered and loud speech acumetry, tonal threshold audiometry, speech audiometry, impedancometry and evoked optoacoustic emission. Civilian aviation sectoral approaches to the evaluation of professional suitability in terms of hearing among the aeronautical personnel are considered in accordance with the criteria adopted by the International Civil Aviation Organization (ICAO), a specialized UN agency that sets the international standards for the civilian aviation and co-ordinates its development with the purpose of enhancing the safety and effectiveness of flights. The criteria are formulated for the solution of the expert problems arising from the relationship between the professional occupation and the diseases of the organs of hearing and for the evaluation of the professional suitability in terms of hearing among the aeronautical personnel engaged in civilian aviation.

The objective of the present study was the prospective analysis of the results of bilateral cochlear implantation (CI) in the children presenting with bilateral ossification of the cochlea after they had survived meningitis. A total of 15 patients underwent the surgical intervention. In those exhibiting bilateral ossification of the basal cochlear helix over the 5 mm segment (up to first bend of the cochlear turn) and partial ossification of the second helix (in 6 children), the affected portions were removed with the placement of two choleostomies, the lower one (from the ossified membrane of the cochlear window) and the upper one (toward the second helix). Activation of the speech processors of the CI systems was carried out within 4-6 weeks after surgery. The hearing abilities of the children were evaluated in accordance with the 'Estimation of the auditory perception categories', 'Estimation of the child's apprehension capacity', and 'Analysis of speech intelligibility rating' guidelines. In all the children with ossification over less than 5 mm of the basal cochlear helix, it proved possible to introduce the whole intracochlear electrode grid whereas only half of the electrode array was implanted in the cases of overall ossification of the basal helix. The first results obtained by telemetry and surdopedagogical testing gave evidence of the possibility of identifying various sources of non-verbal and speech stimuli in all the treated children at a small (up to 3 meters) distance.

Pendred syndrome is an autosomal recessive inherited disorder characterized by a combination of sensorineural hearing impairment and euthyroid goiter; its clinical manifestation in children is hardly distinguishable from nonsyndromic hearing loss. Pendred syndrome is one of the most frequent types of syndromic hearing loss. Hearing impairment is accompanied by abnormal development of the bony labyrinth—enlarged vestibular aqueduct (EVA) and occasionally combined with Mondini dysplasia. Mutations in the SLC26A4 gene, which encodes the pendrin protein, are responsible for both Pendred syndrome and for allelic disorder (nonsyndromic enlarged vestibular aqueduct). The present study for the first time conducted molecular genetic analysis in 20 Russian patients with Pendred syndrome, EVA and/or Mondini dysplasia. As a result, six pathogenic mutations in the SLC26A4 gene were revealed in four patients. The mutation c.222G>T (p.Trp74Cys) was detected for the first time. Mutations were found in patients with Pendred syndrome and nonsyndromic EVA with or without Mondini dysplasia. Mutations were not detected in patients with isolated Mondini dysplasia. One proband with clinical diagnosis Pendred syndrome was homozygous for the c.35delG mutation in the GJB2 gene. The absence of frequent mutations, including well-known ones or “hot” exons in the SLC26A4 gene, was reported. Therefore, the optimal method to search for mutations in the SLC26A4 gene in Russian patients is Sanger sequencing of all exons and exon-intron boundaries in the SLC26A4 gene.

This article presents the data on the frequency, diagnostics, and variants of the surgical treatment of the fistula of the round window of the cochlea. A case of this pathology is reported with the special emphasis placed on the approaches to the diagnostics and surgical management of this condition.

The objectives of the present work were the overview of the results of the modern investigations concerning etiology of idiopathic sensorineural impairment of hearing as well as the analysis of the theory of microthrombus formation and its role in pathogenesis of hearing impairment.

The objective of the present study was to estimate the prevalence of vertigo and to determine the type of the lesion of the vestibular analyzer in the patients presenting with acute sensorineural loss of hearing (ASNLH). The secondary objective was to evaluate the possibility of the restoration of the auditory thresholds. The results of the examination and treatment of 94 patients suffering from ASNLH are presented. It was shown that the development of acute sensorineural loss of hearing was accompanied by unilateral peripheral vestibulopathy in 22.3% of the patients. In 5.3% of these cases, dizziness could be attributed to benign paroxysmal positional vertigo and canalolythias is of the posterior semicircular canal on the side of hearing impairment. It is concluded that the presence of the clinical signs of lesions of the vestibular analyzer and peripheral vestibular dysfunction in the form of latent spontaneous nystagmus without gaze fixation and/or asymmetric nystagmus with unilateral weakness in the caloric test is a negative prognostic factor for the restoration of the auditory thresholds in the patients presenting with acute sensorineural loss of hearing.

The present study was designed to systematize the causes underlying the development of chronic sensorineural impairment of hearing in the aircraft personnel engaged in commercial aviation of the Russian Federation. A detailed clinical and audiological picture of chronic sensorineural loss of hearing in the aircraft personnel is presented with special reference to the criteria accepted in the civil aviationfor the evaluation of professional suitability and occupational selection in terms of hearing conditions. The study has demonstrated the paramount importance of the aviation medical expertise for the flight safety control in civil aviation. We analyzed the results of the audiological examination of the aircraft personnel suffering from chronic sensorineural impairment of hearing and proposed the algorithm for the rehabilitation of such subjects taking into consideration the stage of the chronic process.

The objective of the present study was to improve the effectiveness of cochlear implantation (CI) in the patients presenting with cochlear-vestibular abnormalities based on the development and practical application of the algorithm for the insertion of an electrode arrayinto the spiral (Rosenthal's) canal of the cochlea taking into consideration the specific anatomical features of the middle and inner ears. The study included 25 patients with congenital malformations of the inner ear and bilateral grade IV sensorineural loss of hearing or deafness selected for CI. Indications for drilling a cochleostomy were the high localizationof the jugular bulb and the absence of its bone wall (5 patients, 20%). In the remaining cases, it proved possible to identify the round window and perform the transmembrane insertion of the active electrode. In 15 (69%) patients, the surgical intervention provoked intraoperative leakage of the cerebrospinal fluid that was successfully stopped by the careful tamponade of either the cochleostoma or the round window niche with the use of an automuscular flap. Taken together, good visualization of the round window and the transmembrane insertion of the active electrode into the spiral (Rosenthal's) canal of the cochlea in the patients presenting with cochlear-vestibular abnormalities made it possible to reduce to a minimum the injury to the spiral organ of the cochlea, control liquorrhea, and improve auditory performance in the postoperative period.

The aim of this work was a clinical study of the patients with mutations in the SLC26A4 gene and clinical diagnosis of the Pendred syndrome. The Pendred syndrome is a hereditary autosomal recessive disorder characterized by combined pathology of the inner ear and the thyroid gland. CT of the temporal bones demonstrates the Mondini-type structural anomaly in the inner ear and enlarged vestibular aqueduct. Examination of the thyroid gland reveals hypothyroidism and euthyroid goiter. A total of 20 unrelated children at the age from 2 to 16 years presenting with the hearing loss of different severity were available for the examination. High-resolution CT of the temporal bones demonstrated abnormal development of the inner ear including the Mondini-type structural anomaly and enlarged vestibular aqueduct. Five children with congenital hypothyroidism suffered from bilateral sensorineural impairment of hearing. The routine methods of audiological and molecular genetic examination were used throughout the study., Results: As a result of molecular genetic studies, four out of the 20 patients were found to carry six recessive mutations of the SLC26A4 gene in the compound heterozygous and one such gene in the homozygous state which confirmed the hereditary nature of the disease. The children suffered the hearing loss of varying severity diagnosed at different age. The thyroid hypofunction in one child was identified when it was 2 years of age, and in two children at the age of 8 and 9 years., Conclusion: The first step in the diagnosis of the Pendred syndrome among children with congenital hearing loss was a CT scan of the temporal bones that showed incomplete separation of the curls of the cochlea and enlarged vestibular aqueduct. It is necessary to continue to study epidemiology, clinical and molecular genetics of the Pendred syndrome in the Russian population.

The authors consider the specific features of the vestibular function in the patients with sensorineural hearing loss of vascular genesis. The study included 60 patients at the age from 28 to 75 years presenting with sensorineural impairment of hearing of vascular genesis. All of them were examined with the use of the extended otoneurological method. The data obtained were compared with the structural changes and hemodynamic characteristics of vertebral arteries (VA) and internal carotid arteries (ICA) and with the results of magnetic resonance imaging (MRI) of the brain.

Intrauterine infections are a crucial pathogenic factor exerting an appreciable influence on the development of the fetus. They can provoke intrauterine death, cause multiple lesions in the organs and tissues as well as long-term complications that manifest themselves at the later stages of the growth and development of the child. One of such complications is the sensorineural loss of hearing. The importance of hearing impairment arises from the high prevalence of tis condition and frequent incapacitation it causes in the patients. The present publication is focused on various mechanisms underlying the development of hearing impairment depending on the primary infection. Special attention is given to the methods of diagnostics and treatment of intrauterine infections.

The objective of the present study was to elucidate the specific clinical features of occupational sensorineural impairment of hearing (OSNHI) depending on the origin of this pathology, viz. noise, infrasound and/or their combination. The review of research concerning this problem made it possible to systematize variants of ODNHI based on the clinical signs taking into consideration the influence of these physical factors. Pathophysiological features of the exposure of the organs of hearing to infrasound are described.

The author presents a review of the Russian- and foreign-language literature concerning etiology and pathogenesis of sensorineural hearing impairment with the opinions of domestic and foreign researches on this issue.

The present review of the literature reports the materials concerning the application of the acoustic impedance measurements of normal ears and pathologically charged organs of hearing that are important for the differential diagnostics and monitoring of the hearing system condition during the conservative and surgical treatment.

The article covers implementation of regulatory and legal concepts in prevention of risk caused by hazardous effects of noise in civil aviation flight personnel. The authors analyzed case histories of civil aviation flight personnel patients examined in occupational diseases clinic, with first diagnosed occupational neurosensory deafness.

This publication was designed to describe the clinical manifestations of the enlarged vestibular aqueduct syndrome (EVAS), the currently employed methods for its diagnostics, and the strategy for the rehabilitation of the patients presenting with this pathological condition. In addition, the article provides information about the topographic anatomy and X-ray anatomy of the vestibular aqueduct, the specific clinical features of EVAS, the modern algorithm of its diagnostics, and the facilities for hearing rehabilitation in this group of patients.

The article covers data on prevalence of neurosensory deafness among civil aircraft crew members. The study revealed high level of neurosensory deafness in civil aircraft crew members, averaging to 50.8% in neurosensory deafness structure among noise-related occupations. Arterial hypertension appeared to be the most prevalent concurrent disease in civil aircraft crew members with neurosensory deafness, found in 47.5% of the patients examined - that considers arterial hypertension as a factor of neurosensory deafness progression.

The objective of the present study was to develop the criteria for early diagnostics of hearing disorders in the children presenting with chronic pyelonephritis. The study included 100 children of whom 37 (37%) suffered from the sensorineural hearing loss. The early manifestations of this disorder and the enhancement of the acoustic sensitivity threshold for the high frequencies in the range from 6,000 to 8,000 Hz were documented. The study has demonstrated the dependence of the severity of hearing loss on the duration and severity of chronic pyelonephritis. The criteria for early diagnostics of hearing disorders in the children presenting with chronic pyelonephritis were developed and recommended for the inclusion in the standard examination of the patients with pyelonephritis.

The objective of the present work was to study specific features of the audiological phenotype and the prevalence of GJB2-related sensorineural hearing loss (SNHL) in the infants suffering acoustic disturbances. The study included 264 children with bilateral non-syndromic sensorineural loss of hearing diagnosed during the first year of life by means of detailed audiological examination that included tympanometry, registration of short-latency auditory action potentials (SLAAP), delayed evoked otoacoustic emission (DEOAE), distortion product-frequency otoacoustic emission (DPFOAE), and auditory brain-stem response (ABR). In addition, stationary acoustically evoked responses (SAER) were recorded in 38 children presenting with hearing impairment associated with GJB2-related sensorineural loss of hearing. The follow-up dynamic study involved 113 children subjected to repeated audiological examination. The study revealed the genotype with pathological mutations in 182 (69.0%) children including 171 (64.8%) ones with biallelic mutations and 11 (4.2%) with a single mutation (heterozygous genotype). Eighty two (31.0%) children had genotype without mutations. A total of 21 different mutations and 30 different genotypes were identified. Analysis of the family histories of the children showed that neither the absence of relatives suffering from hearing impairment nor the presence of risk factors of acquired hearing impairment excludes the possibility of GJB2-related sensorineural loss of hearing in the infants. Otoacoustic emission fails to be registered in the majority of the children with the altered genotype (87%) during the stay in the maternity house. Mutations in the GJB2 gene are most frequently diagnosed in the patients with the moderate, moderately severe, and severe loss of hearing. At the same time, almost half of the infants presenting with the mild loss of hearing were found to exhibit changes in the GJB2 gene. The thresholds of registration of short-latency auditory action potentials remain stable in 90.0% of the children presenting with GJB2-related sensorineural loss of hearing which makes it possible to choose the strategy of their rehabilitative treatment (the use of hearing aids or cochlear implantation) during the very first months of life and predict the favourable outcome of cochlear implantation and hearing aid measures. The results of the present work illustrate the importance and practical significance of genetic studies (GJB2 gene tresting) of the infants suffering sensorineural loss of hearing and other acoustic disturbances for the elucidation of etiology of these conditions, prognosis of the disease, and the choice of the strategy for its treatment.

Johanson-Blizzard syndrome is a rare congenital autosomal recessive disease characterized by the association of congenital deficiency of the exocrine pancreatic function and multiple malformations. One of the most common manifestations of this pathology is sensorineural hearing loss of different severity and anomalous development of the inner ear. The case of cochlear implantation in the patient presenting with bilateral sensorineural deafness, congenital malformation of the inner ear, and stenosis of the internal auditory canal is reported.

The clinical features of various subjective and objective methods for the study of the hearing function were evaluated based on the results of examination of 46 children presenting with sensorineural hearing loss and deafness. The factors that influence the effectiveness and quality of diagnostics of sensorineural loss of hearing in the children were elucidated.

The objective of the present study was to estimate the diagnostic value of the characteristics of somatic pathology included in the algorithm for early diagnostics of occupational sensorineural impairment of hearing (OSNIH). The relationship between the development of the main nosological forms of somatic pathology as possible markers of internal ear diseases and professional sensorineural impairment of hearing was elucidated among the members of locomotive crews (n=224). The parameters of interest were evaluated 4 years before, within 1 year, and 4 years after the establishment of diagnosis of OSNIH. It was shown that the members of locomotive crews most frequently suffer atherosclerosis (20.0%), gastrointestinal disorders (34.8%), arterial hypertension (25.0%), osteochondrosis (largely vertebrogenic lumbodynia (21.3%)), and microcirculatory disturbances in the form of vascular spasm in the fundus of the eye (32.9%). 97.6% of the subjects above 35 years of age presented with hypercholesterolemia. Comparative analysis of the occurrence of these nosological forms among the members of locomotive crews with hearing problems on the one hand and the normally functioning organ of hearing on the other hand has demonstrated that none of these pathologies can be regarded as a marker for early diagnosis of OSNIH. At the same time, overweight even in the subjects under 35 years of age should be taken into account when planning and implementing preventive measures.

The present paper is focused on the study of the auditory function and cerebral circulation estimated based on the results of rheoencephalographc examination of 75 patients presenting with disordered sound perception and divided into two groups. Group 1 was comprised of the patients complaining of impaired hearing of the tones only within the extended frequency range (9-16 kHz), group 2 consisted of the patients suffering impaired hearing in the conventional frequency range (0.125-8 kHz). It was shown that the brainstem structures of the auditory analyzer in the patients of both groups remained unaffected whereas the cortical structures thereof in the patients of group 2 exhibited the signs of dysfunction. Cerebral circulation was disturbed in both groups, but its changes were especially well pronounced in the patients of group 2. These findings are of interest in the context of diagnostics and treatment of the patients presenting with early disorders of the sound perception function.

This study was designed to compare different objective methods applied to study the hearing function in 132 children presenting with sensorineural impairment of hearing and deafness. It was focused on the assessment of risk factors of congenital and acquired loss of hearing and deafness. In addition, the factors influencing the efficacy and quality of electroacoustical correction of hearing in the children were considered including those of importance after cochlear implantation.

Aim of the Study: to estimate the efficacy of local therapy of acute sensorinerual deafness by means of intratympanic administration of steroids., Patients and Methods: the study included 73 patients presenting with acute sensorinerual deafness (ACND). 24 of them were given local steroid therapy (through a shunt) during 6 months, another 24 patients received traditional systemic therapy, and 25 ones systemic steroid monotherapy (intravenously) for 10 days. The tonal threshold audiograms obtained before as well as 1, 3, and 6 months after the treatment were compared. It was shown that the efficacy of the intratympanic administration of steroids during 1 month was not significantly different from that of systemic therapy. The long-term therapy (for 6 months) by the intratympanic administration of steroids yielded better results than systemic therapy., Conclusion: the local intratympanic administration of steroids may be used as a self-contained method for the primary treatment of acute sensorinerual deafness.

The objective of the present work was to study the role of GST genes in pathogenesis of various neurodegenerative conditions with special reference to sensorineural impairment of hearing. The DNA samples obtained from 200 subjects were available for the investigation; 109 of them were the patients hospitalized in the otorhinolaryngologcal clinic of S.M. Kirov Military Medical Academy for the treatment of uni- or bilateral impairment of hearing. The polymerase chain reaction with subsequent specific restriction endonuclease analysis was used to study genetic mutations. The analysis was focused on the 35delG mutation in the GJB2 gene (DFNB1). In addition, the MTRNR1 (12S rRNA) and MTTS1 (serine tRNA) genes in the patients with acquired sensorineural impairment of hearing were subjected to analysis. Polymorphism of the second detoxication phase gene (GSTM1) was investigated. It was shown that mitochondrial genes MTRNR1 and MTTS1 contribute to the development of acquired sensorineural impairment of hearing. The frequency of heterozygous 35delG mutations of the GJB2 (DFNB1) gene among the subjects with impaired hearing does not significantly differ from its prevalence in the general population. The allelic mutation of the GSTM1 gene is believed to be a predisposing factor for the development of sensorineural impairment of hearing under the action of intoxication and/or infections.