[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.

[Autoimmune hearing loss and a chance of its development in children: literature review and own observations]. / Autoimmunnaya tugoukhost' i veroyatnost' ee formirovaniya v detskom vozraste: obzor literatury i sobstvennye nablyudeniya.

Autoimmune sensorineural hearing loss (AiSNHL) is an uncommon auditory disorder characterized by rapidly progressive bilateral hearing loss and a positive clinical response to treatment with corticosteroids and cytostatics. The prevalence of the disease in the adult population is less than 1% among all cases of subacute and permanent sensorineural hearing loss (precise data are unknown), it is even rarer in children. AiSNHL can be primary (isolated, organ-specific) or secondary (manifestation of another systemic autoimmune disease). The pathogenesis of AiSNHL is based on the proliferation of autoaggressive T cells and the pathological production of autoantibodies to the protein structures of the inner ear, which leads to damage to various parts of the cochlea (possibly also to the retrocochlear parts of the auditory system), less frequently to the vestibular labyrinth. Pathologically, the disease is most often represented by cochlear vasculitis with degeneration of the vascular stria, damage to hair cells and spiral ganglion cells, and endolymphatic hydrops. In 50% of cases, the result of autoimmune inflammation may be fibrosis and/or ossification of the cochlea. The most characteristic symptoms of AiSNHL at any age are episodes of sudden progression of hearing loss, fluctuations of hearing thresholds, and bilateral, often asymmetric impairments. The article presents contemporary ideas of the clinical and audiological manifestations of AiSNHL, the possibilities of diagnosing and treating the disease, and highlights the current approaches to (re)habilitation. Along with literature data, two own clinical cases of an extremely rare pediatric AiSNHL are given.

[Auditory neuropathy and prematurity: modern view of the issue (literature review)]. / Auditornaya neiropatiya i nedonoshennost': sovremennyi vzglyad na problemu (obzor literatury).

Auditory neuropathy spectrum disorder (ANSD) is a specific auditory disorder caused by dysfunction of periphery part of the auditory system, in which the function of the outer hair cells is preserved, but the afferent input at the cochlear level suffers due to the pathology of the inner hair cells, neurons of the spiral ganglion and/or the auditory nerve, as well as synaptic contact between them. As a result, a specific condition is formed, in which a patient's otoacoustic emissions and/or cochlear microphonics are present, auditory brainstem responses are abnormal or absent, the discrepancy between the hearing level and the electrophysiological data, poor speech perception which may not correlate with the hearing thresholds. ANSD is a multifactorial disease. One of the main risk factors is perinatal pathology and, in particular, prematurity. The possible factors associated with prematurity that provoke the onset of the disease, features of the pathogenesis, clinical and audiological peculiarities of ANSD in premature infants, contemporary approaches to the habilitation of such patients are discussed in the article. The necessity of an individual, patient-oriented approach to the treatment of premature infants with ANSD is substantiated; such an approach should be based both on the genesis of the disorder, taking into account possible points of lesion in the auditory system, and the developmental peculiarities of a premature baby considering the presence of concomitant diseases associated with prematurity. In the article attention is focused on the main directions of habilitation work with such children, including a multidisciplinary approach, regular careful monitoring of the auditory, speech and language skills, intensive psychological and speech therapist support, the choice of an adequate way of intervention and its improvement as necessary.

[The issue of auditory neuropathy: from origins to the present]. / Problema auditornoi (slukhovoi) neiropatii: ot istokov k sovremennosti.

The issue of auditory neuropathy spectrum disorders (ANSD) has been in a focus of specialists attention for a relatively short time, but during this time a huge amount of scientific and practical knowledge about this hearing disorder has been accumulated. ANSD is a specific auditory deficit caused by dysfunction of periphery part of the auditory system, which may affect the inner hair cells, the spiral ganglion neurons and the auditory nerve, as well as the area of synaptic contact between them, while the outer hair cells, as a rule, remain intact. As a result, a specific condition is formed, in which a patient's otoacoustic emissions and/or cochlear microphonics are present, auditory brainstem responses are abnormal or absent, electrophysiological data may not correlate with hearing level, the discrepancy between pure tone audiometry and speech discrimination is observed. ANSD prevalence, epidemiology, contemporary views on its etiology, including detailed information on hereditary forms of the disorder and its risk factors are considered in the review. The data on the basic rungs of the ANSD pathogenesis, which underlie the development of various forms of the disorder and mainly determine the rehabilitation approach, are presented. The detailed clinical and audiological characteristics of ANSD are presented; contemporary approach to ANSD diagnosis and rehabilitation, including indications for surgical treatment, are considered.

[Childhood hearing screening: achievements, difficulties, and possible ways to improve]. / Audiologicheskii skrining v detskom vozraste: dostizheniya, problemy, vozmozhnosti povysheniya effektivnosti.

Hearing impairment is the most common sensory impairment that is seen among adults and children. The frequency of congenital hearing loss is well-known due to implementation of newborn hearing screening. Hearing may change throughout a lifetime due to different factors and, therefore, the number of hearing impaired children increases with age. Introduction of universal newborn hearing screening has enabled earlier detection of hearing loss including unilateral and minimal disorders. Nevertheless, despite significant progress made in this field, there is still a group of hearing impairments that stay undiagnosed timely. Auditory neuropathy spectrum disorders, late-onset hearing loss, low-frequency and minimal hearing impairment are sometimes missed in newborn hearing screening or they manifest later. These types of hearing disorders are covered in detail in this review as well as possible ways of increasing the effectiveness of early diagnosis.

[Psychoacoustic methods in diagnosis of central auditory processing disorders in prematurely born children]. / Psikhoakusticheskie metody v diagnostike narushenii tsentral'noi slukhovoi obrabotki u detei, rodivshikhsya nedonoshennymi.

INTRODUCTION: The presence of numerous perinatal risk factors and comorbid pathology in prematurely born children, even in the absence of peripheral auditory deficit, can lead to disruptions in the processes of higher nervous sound information processing with the formation of central auditory disorders. OBJECTIVE: Audiological assessment of the functional state of auditory system central parts in prematurely born children. MATERIAL AND METHODS: The study involved 54 deeply premature born infants, which were divided into 3 groups depending on age (6-7, 8-9, and 10-11 years), 18 subjects in each group, and 70 healthy, term-born children of the corresponding age. In addition to the traditional audiological examination, all children underwent an assessment of functional state of the central parts of auditory system through a pause detection test (Random Gap Detection Test, RGDT); the perception of fast rhythmic sequences of stimuli, monaural low excess speech testing, binaural interaction test in alternating binaural speech format (ABS ), dichotic presentation of pairs of single digits, single digits and monosyllables, two-digit numerals, Russian matrix phrasal test in noise (RUMatrix) were studied. RESULTS: Prematurely born infants of all age groups were significantly worse compared to control group (p<0.01) while having RGDT, a test for assessing the perception of fast rhythmic sequences of stimuli and dichotic binaural integration tests. Monaural intelligibility of monosyllabic words in silence in children of all three groups did not differ from normal values, but it suffered from contralateral use of noise interference in children aged 6-7. According to the RUMatrix test, legibility of phrases in noise was impaired in 65% of subjects. Test results in the ABS format revealed a significant violation of speech intelligibility (p<0.01) only in children of the younger age group. CONCLUSION: In prematurely born children, there is a dysfunction of the central parts of the auditory system, which is multilevel in nature, partially leveling as children grow older. Moreover, the processes of temporary processing of acoustic information suffer to the greatest extent, not being compensated up to adolescence.

[Age-specific dynamics of the auditory function in the extremely premature babies]. / Vozrastnaia dinamika slukhovoi funktsii u detei, rodivshikhsia gluboko nedonoshennymi.

Extremely early premature babies (i.e. those born before the 32nd week of pregnancy) constitute a group at high risk of development of the perceptive forms of hearing impairment represented mostly by chronic sensorineural hearing loss (CSNHL) and hearing neuropathy (HN). The timely diagnostics of these hearing disorders in premature children and, accordingly, their early and adequate rehabilitation provide a basis for the prevention of hearing and speech problems. At the same time, the hearing function in prematurely born babies is known to be highly unstable and can undergo substantial changes with age; these changes are most frequently observed during the early childhood. This paper was designed to report the results of observations of the hearing function dynamics in a cohort of the extremely early premature children. A total of 186 babies were available for the examination. It was shown that 14 (7.5%) of them had hearing problems. They disappeared by the 12th and 24th months of life in 3 children. Three other babies experienced transformation of NH into CSNHL at the age of 12, 14, and 18 months. NH transformed into CSNHL in three children by the age of 12, 24, and 48 months. Five children developed delayed CSNHL by the 10th, 15th, 24th, 28th, and 36th months of the actual life. These findings give evidence that the extremely premature children may experience both the improvement of the hearing function due largely to the processes of maturation in the auditory system and its deterioration or delayed formation of hearing impairment. It is concluded that the extremely early premature children born before the 32nd week of pregnancy should remain under the dynamic audiological observation at least as long as the third or fourth years of life.

[The patulous Eustachian tube syndrome: the current state-of-the-art and an original clinical observation. Second communication]. / Sindrom ziiaiushchei slukhovoi truby: sovremennoe sostoianie problemy i sobstvennye klinicheskie nabliudeniia. Soobshchenie 2.

The choice of the treatment strategy for the patients presenting with the patulous Eustachian tube syndrome constitutes a serious challenge for an otorhinolaryngologist. Despite the considerable progress achieved during the past years in the development of modern pharmacotherapy and functional endoscopic surgery, the specialists often encounter difficulties in the management of the patients suffering from this disease. The present article (continuing the previous communication) contains information concerning the historical and modern approaches to the treatment of the patients with the patulous Eustachian tube syndrome. The authors describe the conservative and surgical methods employed for the treatment of this condition and the approaches to the choice of the treatment strategies depending on the etiology, pathogenesis, and severity of the disorder being considered, its duration, and the character of the morphological changes identified during the diagnostic procedures. The authors emphasize the importance of the wait-and-see strategy for the treatment of the patients presenting with the patulous Eustachian tube syndrome. In conclusion, they present the concrete results of four original observations of such patients.

[The patulous Eustachian tube syndrome: the current state of the problem and our own clinical observation. Part 1]. / Sindrom ziiaiushchei slukhovoi truby: sovremennoe sostoianie problemy i sobstvennye klinicheskie nabliudeniia. Soobshchenie 1.

The patulous Eustachian tube syndrome was described more than 150 years ago, but up to the present time some of its aspects continue to be a challenge for both fundamental and clinical medicine. Certain issues of etiology and pathogenesis of this condition have until recently remained unclear which probably accounts for the failure of choosing the adequate methods for its medical correction. Despite the seemingly broad spectrum of the modern tools for the purpose including first and foremost the endoscopic and radiological techniques, verification of the patulous Eustachian tube may encounter difficulties for the specialists. In connection with this, we have undertaken to summarize the views and opinions as regards the syndrome of interest available in the relevant world literature. In the first part of this communication, the authors discuss the modern concepts of etiology and pathogenesis of the patulous Eustachian tube syndrome with special reference to its clinical manifestations and methods of diagnostics.

[Intrauterine infections as a risk factor of the development of sensorineural impairment of hearing ].

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.

[Antimicrobial Properties Surfactants Synthesized under Different Cultivation Conditions of Acinetobacter calcoaceticus EMV B-7241].

Aim: To study the antimicrobial properties of the surface-active agents (surfactants) Acinetobacter calcoaceticus IMV B-7241 depending on the availability of yeast autolysate and trace elements in the composition of ethanol-, n-hexadecane- and glycerol-containing media. Methods: Antimicrobial against bacteria (Escherichia coli IEM-1, Bacillus subtilis BT-2), and yeast (Candida albicans D-6) properties of the surfactant was determined by index of the minimum inhibitory concentration (MIC). Surfactants were extracted from supernatant of cultural liquid by mixture of chloroform and methanol (2:1). Results: The removal from cultivation medium yeast autolysate and trace element mix and replacing them by copper sulfate and iron sulphate in the medium with ethanol and n-hexadecane, and in the medium with glycerol - by potassium chloride, zinc sulfate and copper sulfate accompanied by decreasing antimicrobial properties of surfactants. The most effective antimicrobial agents were surfactant synthesized on ethanol in the presence of yeast autolysate and trace elements (MIC 9-20 µ/ml), whereas the surfactant obtained under similar cultivation conditions on glycerol and n-hexadecane, inhibited growth of tested bacteria and yeast at higher (9-68 and 27-54 µ/ml, respectively) concentrations. The minimum inhibitory concentration of surfactant, synthesized in a medium with ethanol (glycerol, n-hexadecane), yeast autolysate and trace elements, correlated with the activity of NADP+-dependent glutamate dehydrogenase - a key enzyme of aminolipids biosynthesis (610 ± 30, 395 ± 24, 397 ± 24 nM min-1·mg-1 protein, respectively). Conclusions: The higher activity of NADP+-dependent glutamate dehydrogenase when growing the strain IMV B-7241 in a medium with ethanol (n-hexadecane), yeast autolysates and trace elements compared to that in a medium with copper sulfate and iron sulfate, as well as an increase enzyme activity in the presence of zinc cations suggests the possibility of increasing synthesis aminolipids by introducing Zn2+ in the medium with ethanol and n-hexadecan. The obtained data indicate the need for studies depending on biological properties of surfactants of the cultivation conditions of producer.

[EFFECT OF CULTIVATION CONDITION OF ACINETOBACTER CAL COACETICUS IMV B-7241 ON ANTIADHESIVE PROPERTIES OF SURFACTANTS].

AIM: To study the effect of growth factors and microelements in composition of ethanol-, n-hexadecane- and glycerol-containing media on antiadhesive properties of A cinetobacter calcoaceticus IMV B-7241 surfactants. METHODS: Surfactants were extracted from supernatant of cultural liquid by mixture of chloroform and methanol (2:1). The number (%) of attached cells (adhesion) was determined as a ratio of the optical density of the suspension obtained from the materials treated with surfactants to the optical density of the control samples (100 %). RESULTS: Dependence of surfactants antiadhesive properties on presence in the medium of A. calcoaceticus IMB B-7241 cultivation of growth factors and certain microelements, as well as the nature of the carbon source was established. Adhesion of bacteria (Escherichia coli IEM- 1, Bacillus subtilis BT-2) and yeast (Candida albicans D-6) on plastic, dutch tile, linoleum, and steel was a minimal (25-35 %) after surface treatment with surfactant (0.005 mg/ml) synthesized on ethanol in the presence of yeast autolysate and microelements. Replacement the yeast autolysate and microelement mixture in the composition of ethanol- and n-hexadecane-containing media by copper sulfate and iron sulfate and in the medium with glycerol by KCl, zinc sulfate and copper sulfate accompanied by decreasing antiadhesive properties of synthesized surfactants. CONCLUSIONS: The obtained data indicate that the increasing surfactant synthesis does not always the accompanied by the formation of product with the required biological properties and indicate the need for studies depending on biological properties of surfactants of the cultivation conditions of producer.

[The hearing function in the premature infants].

The present review of the literature is focused on the morphological and functional aspects of antenatal ontogenesis of the hearing system with special reference to the peculiar features of its formation during the post-natal period in the premature infants. In this context, the results of the objective psychoacoustic investigation s are considered (including those obtained by such methods as threshold audiometry, verbal and non-verbal tests, evaluation of central auditory processing of the sound information). Also presented are the results of the objective methods of hearing investigations (acoustic impedancometry, registration of optoacoustic emission and auditory evoked potentials). The underdevelopment of the hearing system in the premature infants manifests itself as the specific functional features of all its components. Their parameters are presented with reference to dynamics of their formation. In addition, the problem of central auditory processing of the disorders that are very likely to be present in the premature infants are discussed.

[ANTIMICROBIAL ACTION OF NOCARDIA VACCINII IMV B-7405 SURFACTANTS].

AIM: To study the effect of Nocardia vaccinii IMV B-7405 surfactants on some bacteria (including pathogens of genera Proteus, Staphylococcus, Enterobacter), yeast of Candida species and fungi (Aspergillus niger R-3, Fusarium culmorum T-7). METHODS: The antimi- crobial properties of surfactant were determined in suspension culture by Koch method and also by index of the minimum inhibitory concentration. Surfactants were extracted from supernatant of cultural liquid by mixture of chloroform and methanol (2:1). RESULTS: It is shown that the antimicrobial properties of N. vaccinii IMV B-7405 surfactant depended on the degree of purification (supernatant, solution of surfactant), concentration and exposure. Survival of Escherichia coli IEM-1 and Bacillus subtilis BT-2 (both vegetative cells and spores) after treatment for 1-2 hours with surfactants solution and the supernatant (the surfactant concentration 21 µg/ml) was 3-28%. Minimum inhibitory concentrations of N. vaccinii IMV B-7405 surfactants on studied bacteria, yeast and micromycetes were 11.5-85.0; 11.5-22.5 and 165.0-325.0 µ/ml respectively. CONCLUSIONS: Minimum inhibitory concentrations of N. vaccinii IMV B-7405 surfactants are comparable to those of the known microbial surfactants. The possibility of using the supernatant of culture liquid as an effective antimicrobial agent noticeably simplifies and reduces the cost of the technology of its obtaining.

[Modern aspects of diagnosis of presbycusis and its treatment in elderly patients].

This study was designed to evaluate the efficacy of tanakan used to treat tympanophonia in elderly women. The entire spectrum of modern audiological technique was employed to examine the patients. It was shown that tanakan therapy decreases intensity of typmanytis and improves speech hearing in aged patients. The results of the study give reason to recommend the treatment with tanakan for the elderly patients presenting with either presbiacusis or normal tonal hearing.

[Epstein-Barr virus infection as etiological and pathogenetic factor of exudative otitis media in childhood].

A detailed examination of 40 children with recurrent exudative otitis media (EOM) using enzyme immunoassay and polymerase chain reaction suggested that ENT pathology in the above children (EOM, adenoiditis, tonsillopharyngitis, sinusitis) may be a complication of acute or chronic Epstein-Barr virus infection (EBVI) because primary EBVI infection or its long-term persistence followed secondary immunodeficiency resulting in lymphoid system impairment and damage of upper airway epithelium. This causes a recurrent and persistent course of EOM. Etiotropic and pathogenetically sound treatment of children with recurrent EOM includes antiviral therapy, immunocorrection, rehabilitation with participation of pediatrician, immunologist, infection therapist.

[Use of immunomocorrection in the combined treatment of laryngeal papillomatosis in children]. / Ispol'zovanie sredstv immunokorrektsii v kompleksnom lechenii papillomatoza gortani u detei.

Original immunological studies allowed the authors to design a combined treatment of laryngeal papillomatosis (LP) with administration of immunomodulators and cytostatics. Surgery was performed on demand. Thymic hormone preparation (T-activin, thymalin, thymogen) and prospidin served as immunomodulators and cytostatic, respectively. To create lymphotropism, prospidin was delivered by means of electrophoresis following subcutaneous introduction of 8--16 U lidase. Sensitivity of patients to thymalin was tested in vitro by thymalin-induced changes in the number of CD2--DR+ lymphocytes. Adjuvant modalities were age-adjusted dibasol as inductor of interferon genesis and adaptogens (eleutherococcal fluid extract, B and C vitamins). Such treatment was given to 39 children. Remission duration increased 1.6--2.6-fold, 6 of 7 children with primary LP achieved persistent remission. Positive changes in immunological indices occurred in 84% of children. The above treatment outcomes support the efficacy of the method in childhood LP.

[Structure of immunological defect in laryngeal papillomatosis in children]. / Struktura immunologicheskogo defekta pri papillomatoze gortani u detei.

81 children aged from 10 months to 15 years with laryngeal papillomatosis (LP) were studied immunologically. Two groups of the patients were formed: by age and frequency of LP recurrences. Multifactorial estimation covered T and B immunity, factors of nonspecific defense. It was found that LP patients had T-cell immunodeficiency indicated by a reduction in the number of CD2--DR+, CD2, CD3, CD4 lymphocytes and suppression of nonspecific defense as shown by decreased C3--component of complement, number of NK-cells, lymphocyte enzyme activity. LP correlated clinically with immunogenetic abnormalities which proved most severe in older children with more frequent recurrences and least severe in the same age group with rare recurrences. The authors infer on interdependence of immunodeficiency and human papillomatosis virus infection. It is thought valid to enter immunomodulation in LP treatment.

[Indicators of immunologic reactivity in children with chronic laryngostenosis]. / Pokazateli immunologicheskoi reaktivnosti u detei s khronicheskimi stenozami gortani.

Immunological examinations of 40 children with papillomatosis and chronic cicatricial stenosis of the larynx included measurements of circulating immune complexes (CIC) and lysozyme in the saliva and serum as well as complement activity in serum. The examinations were performed regularly during treatment. It was found that immunobiological factors played an important part in the pathogenesis of papillomatosis and cicatricial stenosis of the larynx in children. It was shown that CIC and lysozyme concentrations in the saliva and serum as well as complement activity in serum increased. In the course of treatment all the groups displayed an increase of lysozyme in the saliva; patients with papillomatosis showed a decrease of the CIC concentration and those with cicatricial stenosis a decline of complement activity in serum. Involvement of the autoimmune component in the pathogenesis of the above diseases is discussed. Applicability of the results for controlling the therapeutic efficacy and predicting the outcome of diseases is considered.