Irina V. Savenko

Secondary autoimmune sensorineural hearing loss (sASNHL, secondary autoimmune inner ear disease, sAIED) is hearing impairment that might occur in case of systemic or organ-specific autoimmune disorders before or after the main disease manifestation. The onset of sensorineural hearing loss before the specific clinical picture of the underlying disease with a difference of several months and years can lead to serious diagnostic errors. The relevance of the sASNHL issue is due to the risk of developing profound hearing loss that is poorly corrected using traditional methods and may finally require cochlear implantation. All the diagnostic mistakes do happen because of lack of data about sASNHL pathogenesis and clinical features that delay its diagnostics and thus treatment. The significance of sASNHL problem is determined by increase of number of autoimmune pathology in recent decades and their adverse outcomes. The article presents up-to-date information on etiopathogenesis, pathomorphology, clinical features, laboratory, genetic and radiation diagnostic methods, and criteria for the verification of sASNHL. Therapy options using both traditional means of immunosuppression (corticosteroids, cytostatics) and modern biological drugs, as well as progressive and promising semi-surgical methods are described in detail. Gene therapy opportunities are also shown.

Giant cell arteritis (GCA), also called as temporal arteritis, is a systemic vasculitis, primary affecting individuals over 50 years of age and mostly women. The disease can manifest with symptoms of systemic inflammatory response syndrome as well with symptoms of systemic vasculitis affecting mainly medium and large vessels, including branches of the carotid artery, first of all the ophthalmic and temporal. Pathogenetically, arterial damage in GCA is a combination of autoinflammatory and autoimmune processes, resulting in vascular wall remodeling with narrowing of the arterial lumen and subsequent ischemia in the affected organs and tissues. One of the uncommon symptoms of GCA, which in some cases may precede the onset of the disease, is cochleovestibular dysfunction, in particular sensorineural hearing loss, which might occur in 25—60% of GCA cases. The clinical case of untypical GCA was shown. The disease manifested after SARS-Cov-2 infection as the subacute rhinosinusitis and bilateral otitis media with effusion that finally resulted in profound mixed hearing loss. The clinical features of the disease looked like granulomatosis with polyangiitis, however, no increase in antinuclear and antineutrophil cytoplasmic antibody levels was detected. A specific clinical feature with unilateral vision loss after 12 months from the disease onset could help to verify it. Urgently corticosteroid therapy let us relieve both main symptoms and rhinosinusitis with bilateral otitis media with effusion that resulted in significant hearing improvement. It has been suggested that the combination of middle and inner ear lesions in this case could either be a consequence of the simultaneous development of GPA and GCA, or was the result of coincidence of a number of their pathogenesis links, specifically the activation of B cells, as well as excessive proliferation of monocytes/macrophages producing metalloproteinase-9.

Common problems accompanying the development of preterm children include difficulties in speech perception and language use, which can lead to cognitive, communicative, social, and academic difficulties in school age. On the other hand, preterm children are at high risk for developing auditory impairments, which can affect not only the peripheral but also the central auditory system. Any level of auditory lesion, either isolated or in combination, can contribute to the development of central auditory processing disorders (APD). The article discusses the etiopathogenetic link between speech and hearing difficulties and dyslexia with APD. Based on the contemporary concept of the problem, an interdisciplinary approach to the diagnostic process is presented, which allows for identifying the nature and degree of speech and hearing disorders, and subsequently verifying the specific auditory deficit. The results of the diagnostic search enable the optimal (re)habilitation intervention, with a focus on auditory training. Depending on the identified disorders, either a peripheral «bottom-up» approach with correction of acoustic input and/or impaired central auditory functions is used, or a cognitive «top-down» approach aimed at improving higher mental processes, or a combination of both. When choosing the scope of (re)habilitation measures, the age and capabilities of the preterm child are taken into account.

Premature babies are at high risk of developing hearing dysfunction, which can affect all levels of the auditory system and disrupt the development of auditory, language, communicative, emotional, and social skills, thus impairing the child’s overall quality of life. Hearing impairment in premature infants is presented in transient or intermittent forms of conductive hearing loss, mixed hearing loss, as well as sensorineural hearing loss (SNHL) and auditory neuropathy spectrum disorders (ANSD). Auditory function characteristics include unstable hearing thresholds, the possibility of improvement up to complete normalization, less commonly delayed hearing loss, and the transformation of ANSD into SNHL and vice versa. The article presents a possible algorithm for clinical and audiological monitoring of children born prematurely from birth to 10 years old. It involves a two-stage newborn hearing screening within the first 1—2 months after birth and verification of the diagnosis by the time infants reach 3 months corrected age. If hearing loss are detected, children are examined up to the age of 2 years 3—4 times a year; children with initially verified normal hearing are examined 2—3 times in the first year of life, and then annually. Separately, there is an algorithm for diagnostic and habilitation intervention for children with ANSD. Attention is also drawn to the need to involve otolaryngologists for timely identification of conductive hearing loss and special education teachers, speech therapists for early diagnosis of central auditory processing disorders. Long-term monitoring of all aspects of auditory function in premature babies will allow for timely and high-quality diagnostic and (re)habilitation processes, minimizing the negative impact of hearing impairments.

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.

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 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.

Аудиторная (слуховая) нейропатия (АН) представляет собой специфический слуховой дефицит, обусловленный дисфункцией слуховой периферии, при котором сохраняется функция наружных волосковых клеток, но нарушается функция афферентного входа на уровне улитки вследствие патологии внутренних волосковых клеток, нейронов спирального ганглия и/или слухового нерва, а также синаптического контакта между ними. При этом формируется симптомокомплекс, включающий наличие вызванной отоакустической эмиссии и/или микрофонного потенциала улитки, отсутствие или патологию коротколатентных слуховых вызванных потенциалов, несоответствие субъективных порогов слуха данным электрофизиологического обследования, нарушение разборчивости речи, которое не согласуется с порогами тонального слуха. АН — полиэтиологическое заболевание. Одним из факторов риска ее формирования является перинатальная патология, в частности недоношенность. В статье рассмотрены возможные ассоциированные с недоношенностью факторы, провоцирующие возникновение заболевания, а также особенности патогенеза, клинико-аудиологических проявлений АН у недоношенных детей, освещены существующие подходы к абилитации таких пациентов. Обоснована необходимость индивидуального, пациентоориентированного подхода к курации недоношенных детей с АН, который должен основываться как на предположительном генезе расстройства с учетом возможных «точек поражения» слуховой системы, так и на особенностях развития недоношенного ребенка и наличия ассоциированной с недоношенностью сопутствующей патологии. При этом акцентируется внимание на основных направлениях коррекционной работы с такими детьми, в том числе на мультидисциплинарном подходе, на необходимости регулярного динамического наблюдения с тщательным мониторингом слухоречевой функции, интенсивного психолого-педагогического сопровождения, а также на выборе адекватного способа абилитации и его совершенствовании.

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.

Despite the long-term study of exudative otitis media, the disease is not well understood. The wide prevalence of exudative otitis media in children and the increase in the incidence during recent decades explain the interest of specialists in the etiology of the disease. The article presents current views on the role of premature birth, gastroesophageal reflux, systemic and local immunodeficiency, disorders in the antioxidant defense system, mucopolysaccharidosis, genetic predisposition, and other factors in otitis media development. Unfortunately, the cause of exudative otitis media cannot always be established, and it requires further experimental and clinical studies in order to develop effective approaches to etiopathogenetically justified, timely and successful treatment of the disease and its implementation in clinical medicine.