[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.

[Prospects for cochlear implantation in patients with auditory nerve dysplasia]. / Perspektivy kokhlearnoi implantatsii u patsientov s displaziei slukhovogo nerva.

Auditory nerve dysplasia (AND) can encompass various conditions of the auditory nerve (AN), ranging from true aplasia to hypoplasia. The purpose of this review is to discuss the prospect of cochlear implantation (CI) and subsequent auditory speech rehabilitation for AN abnormality. Studies of different authors when working with this category of children, possible results and methods of diagnostics of the AN condition are presented.

[Epidemiological characteristics of the pediatric population of cochlear implant users in St. Petersburg]. / Epidemiologicheskaya kharakteristika detskoi populyatsii pol'zovatelei kokhlearnykh implantatov Sankt-Peterburga.

An epidemiological analysis of the pediatric population of cochlear implant (CI) users in St. Petersburg, the second largest metropolis of Russian Federation, was carried out. CI users are 14% of the total number of hearing impaired children, and 26% of children with hearing devices. Only 43% of children who are candidates for cochlear implantation in according to audiological criteria use CI. Since 2012, unilateral cochlear implantation has been carried out for all children in need - their annual number ranges from 25 to 45. Bilateral implantation, mostly sequential with an interval of 1-3 years, was performed in 21% of children The average age at the time of the surgery was 3 years, more than 50% of children were implanted between 1 and 3 years. In recent years, the number of children implanted before 2 years has increased, as well as the number of children implanted at school age. 27% of children with CI continue wearing a hearing aid on the opposite ear. 23% of implanted children have additional disabilities. It has been established that the proportion of children attending mainstream educational institutions is significantly higher among children with CI, compared with children using hearing aids. Some directions have been developed to improve care for CI children and to increase the effectiveness of cochlear implantation as a medical-technical-pedagogical technology for hearing restoration in a regional centre for hearing impaired children.

[Peculiarities of CI processor fitting in children with auditory neuropathy spectrum disorders]. / Osobennosti nastroiki protsessora kokhlearnogo implantata u detei so slukhovoi (auditornoi) neiropatiei.

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.

[Results of the audit of the first stage newborn hearing screening in St. Petersburg]. / Rezul'taty audita pervogo etapa audiologicheskogo skrininga novorozhdennykh v Sankt-Peterburge.

OBJECTIVE: Analysis of the first stage of universal newborn hearing screening in St. Petersburg and increasing its effectiveness. RESULTS: In result of the audit, it was found that screening performs in all maternity wards, children's hospitals and at the majority of pediatric clinics. Meanwhile only 14% of institutions meet criteria of good practice in providing screening. In other clinics improving the procedure of screening is needed: equipment update and calibration, correction of the method and conditions of examination, results documenting, etc. The set of activities for increasing the effectiveness of newborn hearing screening is proposed. The work which carried out with institutions during and after audit allowed to reach 100% involving clinics in screening system in 6 months. Due to information support of institutions coverage of newborns by the first stage of screening increased on 8.5% (matched to target) and the number of referred children who come to the second stage of screening increased on 18%. Results suggest that the audit of the first stage of newborn hearing screening by the district pediatric audiological centers is an effective tool for finding problems in the screening system and its solving.

[Telerehabilitation of hearing-impaired children during COVID-19 pandemic]. / Distantsionnaya reabilitatsiya detei s tugoukhost'yu v period pandemii COVID-19 v usloviyakh surdologicheskogo tsentra.

OBJECTIVE: To evaluate advantages and effectiveness of remote rehabilitation services for hearing-impaired children at Center of Pediatric Audiology during COVID-19 pandemic. MATERIAL AND METHODS: 181 children with different types and degrees of permanent hearing loss, their parents and 10 hearing care professionals (audiologists, speech-language therapists) were included in the study. 2115 rehabilitation services were provided during 3 months: video- and text consultations, video lessons with child, sending homework to parents, etc. RESULTS: The results of questionnaires showed that, on specialists' and parents' opinion, remote rehabilitation care is effective tool for hearing impaired children during emergency situations. TeleCare allowed to improve parents' abilities to manage with children by themselves, their understanding goals and methods of rehabilitation, improving child's hearing and speech skills. 95% of parents were satisfied by remote rehabilitation. Advantages and problems of remote hearing rehabilitation were analyzed from the sides of professionals and parents. The most challenging activities for professionals during TeleCare were: evaluation of HA/CI effectiveness, diagnosis and developing of hearing and speech. CONCLUSIONS: The experience of remote hearing rehabilitation in emergency situation allows to conclude that this type of care could be useful in clinical practice after pandemic for parents consulting and for children with motor problems.

[Epidemiological study of hearing impairments in children: prevalence, structure, amplification, and social factors]. / Epidemiologiya narushenii slukha u detei: rasprostranennost', struktura, aspekty slukhoprotezirovaniya i sotsial'nye faktory.

For the first time, a detailed epidemiological study of hearing impairments (HI) in the children's population of St. Petersburg (n=3098) was conducted. The data on the incidence of HI in children, the distribution of children depending on the degree of hearing loss, HI type, the presence of additional disorders, risk factors for hearing loss were obtained. Only 19% of children with HI were diagnosed in accordance with modern requirements of early care - at the age of 3 months. In children who underwent universal newborn hearing screening the average age of diagnosis was significantly lower than in children who were not screened. Only 54% of HI children use hearing aids (HA) or cochlear implants. Among the children who do not use HA, the main number is children with mild and moderate hearing loss, unilateral hearing loss and otitis media with effusion. A significant part of them should be considered as candidates for amplification according to current recommendations. The average interval between diagnosis and amplification was 15.7 months. Only 5% of children from the entire population of children with HI received early intervention in accordance with the modern time standard: 1 month (detection) - 3 months (diagnosis) - 6 months (amplification). The problems of organizing an effective care for HI children and the directions of its development are discussed: improving newborn hearing screening; introduction the system of informing the citizens and doctors of related specialties (pediatricians, neurologists) in the field of prevention, diagnosis and rehabilitation of HI in children; expansion of genetic testing of children; reducing time of waiting and improving the quality of HA provided to handicapped children.

The benefits of underwater vibrostimulation in the rehabilitation of children with impaired hearing.

INTRODUCTION: Early intervention is crucial for the optimal speech and language development of children with impaired hearing. Underwater vibrostimulation could help develop behavioural reactions to low-frequency stimuli immediately after diagnosis and facilitate aural rehabilitation after hearing aid (HA) or cochlear implant (CI) activation. GOALS: To determine the limits of underwater vibrotactile stimuli perception and to measure the effect of vibrostimulation training on the aural rehabilitation of young children. METHODS: Two adults and three children with congenital hearing loss participated in the first part of the study. Pure tones between 100 and 4000 Hz and natural broadband sounds were delivered under water while the participants were sitting in a pool. The lower thresholds of perception and the maximum comfortable levels were measured and the subjective sensations were recorded. In the second part of the study, 15 children <3 years old were presented with the same stimuli until they developed stable conditioned reactions to the stimuli. The time until the development of "hearing behaviour" and the number of fitting sessions after HA or CI activation were compared between the vibrostimulation group and a control group who did not receive such training. RESULTS: In the first part of the study, participants were most sensitive to 100-400 Hz stimuli, while the stimuli >1000 Hz did not evoke any sensations. The vibrations were felt across all body parts and produced hearing-like sensations in the ear. In the second part of the study, children in the vibrostimulation group required fewer fitting sessions and developed "hearing behaviour" sooner than the control group. CONCLUSIONS: Underwater vibrostimulation is a promising new method of early aural rehabilitation that could be recommended for implementation in paediatric audiology centres.

[Psychoacoustic tests for perceptual assessment of processor fitting in patients with cochlear implants]. / Ispol'zovanie psikhoakusticheskikh testov dlya pertseptivnoi otsenki nastroiki protsessora kokhlearnogo implanta u glukhikh patsientov.

THE AIM OF THE STUDY: Was to study the possibility of using a battery of psychoacoustic tests to assess the tuning of the cochlear implant processor (CI) in deaf patients. METHODOLOGY: The study involved 60 prellingually deaf patients aged 10 to 23 years with oral speech skills. To assess the quality of the CI processor tuning, in addition to traditional methods, a special battery of psychoacoustic tests was used. The first block of tests assessed the perception of the basic characteristics of sound signals (duration, temporal structure, spectrum, timbre) and was used to assess the initial setting. The second block of tests, intended for patients with experience using CI, included tasks to distinguish acoustically similar and dynamically changing signals, etc. RESULTS: At the end of the initial CI setup session, patients with short signal perception problems were identified. Adjusting the frequency of electrical stimulation in patients has increased their ability to distinguish between sounds. During the second tuning session of the CI processor, 6 months later, a group of patients with difficulties in perceiving acoustic information in the low-frequency range was identified - distinguishing melodic intervals, changing the pitch of sounds, highlighting the voice of the target speaker. The «problem¼ patients underwent additional correction of the CI processor setting and the corresponding auditory training, which improved the test performance and subjective perception of sounds. CONCLUSION: The use of psychoacoustic tests expands the possibilities of fine tuning the CI processor, taking into account the individual characteristics of the patient's auditory perception at different stages of CI use, especially in «problem¼ patients.

[Comparative Analysis of MPTP Neurotoxicity in Mice with a Constitutive Knockout of the α-Synuclein Gene].

Aggregated forms of α-synuclein are core components of pathohistological inclusions known as Lewy bodies in substantia nigra (SN) neurons of patients with Parkinson's disease (PD). The role of α-synuclein in selective loss of SN dopaminergic neurons (DNs) in PD is studied in mice knocked out in the α-synuclein gene. The new mouse strain delta flox KO with a constitutive knockout of the α-synuclein gene models the end point of in vivo deletion of the α-synuclein gene in mice with a conditional knockout and has no foreign sequence in the modified genomic locus, thus differing from all other α-synuclein knockout mouse strains. The effect of the neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), which is used to model PD, was compared between delta flox KO mice and mice of the well-known α-synuclein knockout strain AbKO. Subchronic MPTP administration, which models early PD, was found to reduce the dopamine content and to change the ratio of dopamine metabolites in the striatum to the same levels in delta flox KO, АbKO, and wild-type mice. Overt locomotor defects were not observed after MPTP treatment, but gait testing in a CatWalk XT (Noldus) system revealed identical gait deviations in mice of the two strains and control wild-type mice. Based on the findings, a similar mechanism of neurotoxic damage to DNs was assumed for delta flox KO and AbKO mice.

[Complex disorders in children with sensorineural hearing loss - influence on the diagnosis of hearing pathology and hearing aid]. / Kompleksnye narusheniya u detei s sensonevral'noi tugoukhost'yu ­ vliyanie na diagnostiku patologii slukha i slukhoprotezirovanie.

The presence of additional disabilities (AD) in children with sensorineural hearing loss (SNHL) and influence of AD on age of hearing loss diagnosis and amplification were investigated. It was shown that 41% children with SNHL have different AD - pathology of the central neural system (including mental retardation, autism spectrum disorders, attention deficits), vision, motor and articulation impairments, cardiovascular, respiratory, urinary, digestive, endocrine diseases etc. 59% children among them have 2-3 AD. The most often combination of AD was pathology of central neural and motor systems. The genetic factor was revealed rarely in the group of children with AD than in children with SNHL only. The diagnosis of hearing loss before age 4 months was rarely in the group of children with AD. Possibly it is because of the most of such children got treatment in neonatal intensive care unit and didn't have access to universal newborn hearing screening. Moreover, SNHL might develop later as the outcome of their AD or their treatment. Suggestion about more late amplification in children with SNHL was confirmed. The cause might be wary relation of audiologists to amplification of children with AD and difficulties of this process.

[Organizing the medical care for the COVID-19 patients in non-infectious Moscow hospital: reassignment experience].

AIM: To present the results of work of National Medical Research Center of Treatment and Rehabilitation, reassigned for COVID-19 patients treatment during pandemic. Run-up methodology, procedures and working process organization are detailed. MATERIALS AND METHODS: 354 COVID-19 patients were treated from 13.04.2020 to 10.06.2020 [age 59 (470) years, 56% women, body mass index 28.5 (24.932.2) kg/m2]. Patients were admitted at 8 (611) day of sickness. In-hospital stay was 16 (1420) days. RESULTS: NEWS scale at the day of admittance was 2 (14); 2 (13) in patients discharged alive and 6 (47) in died patients, p=0.0001. So prognostic accuracy of NEWS scale was confirmed as very well (area under ROC-curve = 0.819). 69 patients (19.5%) were treated at intensive care department for 7 (413) days. 13 patients died, 11 of them had COVID-19 as direct or indirect cause of death. Total in-hospital mortality was 3.67%, in-hospital mortality of COVID-19 patients 3.1%. 17 healthcare workers (HCW), contacted with COVID-19 patients were infected (2.67%). 4 HCW, who had no direct contact with patients were also infected and 7 HCW were infected before the first patient was admitted. No one of them died. CONCLUSION: Complex tasks of healthcare organization during COVID-19 pandemic can be solved quickly with acceptable quality, characterized by low levels of patients; mortality and HCW infection.

[Sequential bilateral cochlear implantation with long interval between surgeries in deaf-blind patient]. / Posledovatel'naia bilateral'naia kokhlearnaia implantatsiia s dlitel'nym intervalom mezhdu operatsiiami u slepoglukhogo patsienta.

The efficacy of sequential bilateral cochlear implantation with long interval between surgeries (18 years) was investigated in deaf-blind patient (22 years old man, lost hearing and vision after meningitis at the age 2.5 years). At the age 4 years he got cochlear implant at right ear. His speech skills completely disappeared before the implantation. Auditory-speech rehabilitation with cochlear implant was successful - patient has good auditory, language, speech, cognitive skills. At the age 22 years patient made decision to get cochlear implant at the left ear after breakdown of internal part of cochlear implant at right ear in spite of successful reoperation. After activation of new processor (all electrodes were activated) the patient got daily auditory training with cochlear implant at left ear (Concerto/Opus 2, Medel) on the base of 'auditory' method, in daily life patient uses 2 devices. After 1 month of using cochlear implant at left ear the patient recognized environmental sounds and music instruments, words and sentences in close set tests EARS battery, the score for OLSA test in quiet was 90%, but the perception in noise was complicated. The balance of parameters of fitting for 1-st and 2-nd processors and special auditory training with two cochlear implants provided the development of initial binaural interaction. Perilinqually deaf patients can achieve high effect after sequential bilateral cochlear implantation with long interval between surgeries. The necessary conditions are - preservation of electrical activity of auditory nerve, intensive structured auditory training with new 'ear', patient's motivation of using of cochlear implant at 'new' ear.

[The characteristics of microbiota of periodontal recesses in smoking patients with chronic generalized periodontitis].

The clinical examination of 36 tobacco smokers with chronic generalized periodontitis of light, average and severe degree was carried out. The examination established poor hygienic condition of oral cavity, less expressed inflammatory reaction of tissues of periodont and predominance of occurrences of destruction of alveolar portion of bone as compared with the group of 59 non-smoking patients with chronic generalized periodontitis of light, average and severe degree. The study demonstrated higher rate of detection of T. forsythia in smokers as compared with non-smoking patients at all stages of of development of chronic generalized periodontitis. Under light stage of chronic generalized periodontitis increasing of rate of detection of T. forsythia more than twice was registered. P.gigngivalis and P.intermedia were detected in smoking patients with light stage of chronic generalized periodontitis either in the same values or more rarely as compared with non-smokers. In the group of smokers with average stage of chronic generalized periodontitis increasing of rate of occurrence of association of T. forsythia-P. gigngivalis-P. intermedia occurred more than five times in comparison with non-smokers. The obtained results indicate on relationship between alterations of microbiota and aggressive development of chronic generalized periodontitis in smoking patients and on development in periodontal recesses of smokers of favorable conditions for growth of T. forsythia. The presence of T. forsythia is a significant factor of development of destructive processes in tissues of periodont.

[Intranasal co-administration of recombinant streptococcus group B polypeptides and influenza deltaNS1 vaccine].

In the present work, the immunoadjuvant properties of the influenza deltaNS1 vaccine virus after intranasal administration in combination with recombinant GBS polypeptides was tested in mice. According to our data, co-administration of recombinant GBS polypeptides and influenza deltaNS1 vaccine resulted in the increase in the immunogenicity and protective efficacy of bacterial proteins. Combined vaccination with the GBS polypeptides and influenza deltaNS1 vaccine has a potential to be used not only for prophylaxis infections caused by SGB, but also for prevention of the bacterial complications of influenza.

[Radiation mapping of the short arm of swine chromosome 2]. / Radiatsionnaia karta korotkogo plecha khromosomy 2 svin'i.

In recent years, maps of mammalian genomes have been acquiring increasingly higher resolution. Integration of maps of different types has become possible. As a tool in integrating maps of mammalian genomes of different types, high-resolution mapping with radiation-induced hybrids (RH) is used. Here, we present an RH6000 map of the short arm of porcine chromosome 2. The map contains 15 microsatellites and five genes (for parathyroid hormone, lactate dehydrogenase A, myogenic factor, follicle-stimulating hormone beta, and calpain I). The RH panel was obtained on the basis of a hybrid cell line bearing the single porcine chromosome 2 against the background of mink chromosomes. The mean frequency of preserving markers examined in the panel was 18.3%. Integration of four genes in the panel and a comparison of gene order in homologous regions of human and porcine chromosomes are presented.

Effects of 17 beta-estradiol and its isomer 17 alpha-estradiol on learning in rats with chronic cholinergic deficiency in the brain.

It was shown for the first time that estrogens 17 beta- and 17 alpha-estradiols compensate impaired cognitive functions in rats with partial chronic deprivation of cholinergic functions in the central nervous system induced by intracerebral administration of selective cholinergic neurotoxin AF64A. 17 beta-Estradiol produced strong dose-dependent changes in the weights of hormone-sensitive endocrine glands, while 17 alpha-estradiol did not affect the weight of the gonads and slightly influenced (in high concentration) the weights of the adrenal glands and thymus. The positive effects of exogenous 17 beta- and 17 alpha-estradiols on cognitive functions are due to their antioxidant properties, rather than due to specific action on hormone-sensitive endocrine glands.

[Rehabilitation and assessment of aural-oral speech development in children with cochlear implants]. / Reabilitatsiia i otsenka slukhorechevogo razvitiia detei s kokhlearnymi implantami.

Updated models of cochlear implants provide good speech audibility and thus complete rehabilitation of children who have lost hearing after learning speech. All the children who lost hearing before learning speech can hear sounds of normal loudness and orient in sound media by means of cochlear implant. However, they need long-term audio-vocal rehabilitation the results of which depend on the age of the child's operation and hi(her) individual traits. Cochlear implants in children aged under 3 years are most perspective. Russian language methodology including 7 tests and 2 questionnaires is described. It is intended for assessment of audio-vocal development in children with cochlear implants and results of audio-vocal rehabilitation as well as of effectiveness of using cochlear implant in children over 2 years of age. Establishment of centers for cochlear implantation and introduction of cochlear implantation state program are recommended.