Internal disorder pattern as a predictor of adaptation of disabled adults

The article is devoted to the problem of adaptation of disabled adults. The influence of personal characteristics on adaptation and the problems of including people in the Societas are usually studied. This publication examines the influence of the internal disorder pattern on adaptation. The internal disorder pattern is a complex of sensations, knowledge, experiences, motivational changes associated with the violation. The results of an empirical study involving 75 disabled adults are presented. The methods of conversation, survey and statistical processing were used. It was found that the severity of the components of the internal disorder pattern differs in groups of adults with different variants of dysontogeny. The greatest number of correlations was found between the adaptation parameters and the physical component of the internal disorder pattern. The physical component is a predictor of the overall level of adaptability, internality and emotional comfort. This is important for people with severe speech and hearing disorders. The cognitive component is a predictor of internal control in a group of people with hearing impairments. There was no influence of the components of the internal disorder pattern on adaptation in groups of people with visual impairment and mobility disabilities. It is suggested that this influence is mediated through personal characteristics and self-attitude.

THE RELATIONSHIP BETWEEN NOISE INTENSITY AND WORKER CHARACTERISTICS WITH HEARING DISORDERS IN DENTAL LABORATORY JAKARTA

Background: Along with the development of processing goods using machine tools and equipment as well as the use of chemicals, one of the impacts is loud or noisy sound, which can be interpreted as unwanted sound or exceeding threshold and has an opportunity to damage hearing so that can be detrimental. Purpose: To analyze the relationship between the noise intensity and worker’s characteristics with hearing disorders at the Dental Engineering Laboratory in the Jakarta area in 2020. Method: This research was an analytical study with a descriptive cross-sectional design with a sample of 30 dental technicians who work in a dental engineering laboratory in the Jakarta area. Each area is taken 6 people. Measurement of noise in the laboratory with a sound level meter and measurement of hearing loss with audiometry. Result: Workers whose noise intensity is >85 dBA have hearing loss, workers whose noise intensity is 85 dBA, 4 people who have hearing loss, workers aged >40 years have hearing loss, while among workers aged ≤40 years, there are 7 people experiencing hearing disorders. workers with >5 years of service experience hearing loss, while among workers 5 years of service, none have hearing loss. Conclusion: There is a significant between noise intensity with hearing loss (p-value = 0.011), it isn’t significant between age with hearing loss (p-value = 0.0563), and it isn’t significant between tenure with hearing loss (p-value = 0.06).

The Ganglioside Monosialotetrahexosylganglioside Protects Auditory Hair Cells Against Neomycin-Induced Cytotoxicity Through Mitochondrial Antioxidation: An in vitro Study

Neomycin is a common ototoxic aminoglycoside antibiotic that causes sensory hearing disorders worldwide, and monosialotetrahexosylganglioside (GM1) is reported to have antioxidant effects that protect various cells. However, little is known about the effect of GM1 on neomycin-induced hair cell (HC) ototoxic damage and related mechanism. In this study, cochlear HC-like HEI-OC-1 cells along with whole-organ explant cultures were used to establish an in vitro neomycin-induced HC damage model, and then the apoptosis rate, the balance of oxidative and antioxidant gene expression, reactive oxygen species (ROS) levels and mitochondrial membrane potential (MMP) were measured. GM1 could maintain the balance of oxidative and antioxidant gene expression, inhibit the accumulation of ROS and proapoptotic gene expression, promoted antioxidant gene expression, and reduce apoptosis after neomycin exposure in HEI-OC-1 cells and cultured cochlear HCs. These results suggested that GM1 could reduce ROS aggregation, maintain mitochondrial function, and improve HC viability in the presence of neomycin, possibly through mitochondrial antioxidation. Hence, GM1 may have potential clinical value in protecting against aminoglycoside-induced HC injury.

Vertigo in Systemic Lupus Erythematosus: A Case Report

Introduction: Systemic lupus erythematosus (SLE) is a chronic inflammatory disease with multiorgan involvement based on an autoimmune process. SLE, although rare, is associated with comorbid vertigo. Vertigo in SLE is caused by a disturbance in the balance system in the inner ear. Few journals discuss SLE related to vertigo. We will report a case SLE with complaints of recurrent vertigo. Case: A-37-year-old woman came with complaints of recurrent vertigo since 1 day ago with a duration of about 15 minutes associated with nausea, vomiting and nystagmus. Patient did not complain tinnitus or hearing disorders. The patient has been diagnosed as SLE since two years ago. The physical examination showed normal and Neuro-otological examination revealed nystagmus horizontal unidirectional, negative skew deviation test, positive Head Impulse Test (HIT). Conclusion: Patients with a diagnosis of SLE can find comorbid peripheral vestibular disorders such as vertigo where there is an antibody mechanism that can damage the inner ear. Treatment of audiovestibular symptoms is usually strongly associated with systemic conditions and in patients with vertigo used betahistine to treatment. Keywords: SLE, Inner Ear, Vertigo.

Prevalence of Hyperacusis in the General and Special Populations: A Scoping Review

Objectives: To study the prevalence of hyperacusis in the general population and the special population, and to determine the effect of population differences on hyperacusis.Methods: The two authors followed a scoping review methodology and screened nearly 30 years of English literature in Pubmed, Web of Science, OVID, and EBSCO. Then, the extracted results of each study were discussed in groups and subgroups.Results: The authors selected 42 pieces of scientific literature that met the requirements, studying a total of 34,796 subjects, including the general population (28,425 subjects), the special occupation population (2,746 subjects), and the patients with concomitant diseases (5,093 subjects). The prevalence was 0.2–17.2% in the general population, 3.8–67% in the special occupation population, and 4.7–95% in the patients with special diseases. It was found that in the general population, the high prevalence occurs in adolescents and older adults. The prevalence of hyperacusis in women is significantly higher than in men. In people with hearing disorders, the prevalence of hyperacusis is significantly higher than in people with normal hearing. Various diseases (such as Williams syndrome, tinnitus, and autism), as well as various occupations (musicians, music students, teachers, and others), have been found to be high risk factors for hyperacusis.Conclusion: The high prevalence of hyperacusis and the large differences between reported prevalence in different studies deserves our great attention. Additionally, in order to increase the comparability of the studies, a standardized set of criteria are needed to study the prevalence of hyperacusis.

Genetic Heterogeneity in GJB2, COL4A3, ATP6V1B1 and EDNRB Variants Detected Among Hearing Impaired Families in Morocco

Deafness has a very variable disease. It may occur as a result of external auditory canal involvement or a deficiency in the sound conduction mechanism (transmission deafness) or impairment of the cochlear, cochlear nerve or central auditory perception. Genetics is the most common cause, as approximately 70% of hearing disorders are of hereditary origin. 1/3 of hereditary deafness is syndromic (associated with other symptoms) and 2/3 are non-syndromic (isolated deafness). At this date, 173 loci of deafness gene have been reported in the literature (69 DFNA, 94 DFNB, 6 X-linked DFN, 2 DFNM, 1 DFNY and 1 AUNA1). For syndromic deafness, approximately 400 syndromes associated with hearing disorders are already described. Thus, the determination of causal mutations is a valuable aid for accurate and early diagnosis. This makes it possible to better guide the management since forms of deafness respond better to the cochlear implant than others. The correct diagnosis also gives an idea of ​​the evolutionary profile of deafness and whether it is a syndromic deafness requiring special surveillance. In this study, we have examined the genetic causes of sensorineural hearing loss in Moroccan patients through whole exome sequencing (WES) to identify candidate genes for six severely deaf Moroccan families. The results revealed four genetic variants in the genes GJB2, COL4A3, ATP6V1B1 and EDNRB, which are therefore common causes of syndromic and non-syndromic deafness.

Central auditory deficits associated with genetic forms of peripheral deafness

Since the 1990s, the study of inherited hearing disorders, mostly those detected at birth, in the prelingual period or in young adults, has led to the identification of their causal genes. The genes responsible for more than 140 isolated (non-syndromic) and about 400 syndromic forms of deafness have already been discovered. Studies of mouse models of these monogenic forms of deafness have provided considerable insight into the molecular mechanisms of hearing, particularly those involved in the development and/or physiology of the auditory sensory organ, the cochlea. In parallel, studies of these models have also made it possible to decipher the pathophysiological mechanisms underlying hearing impairment. This has led a number of laboratories to investigate the potential of gene therapy for curing these forms of deafness. Proof-of-concept has now been obtained for the treatment of several forms of deafness in mouse models, paving the way for clinical trials of cochlear gene therapy in patients in the near future. Nevertheless, peripheral deafness may also be associated with central auditory dysfunctions and may extend well beyond the auditory system itself, as a consequence of alterations to the encoded sensory inputs or involvement of the causal deafness genes in the development and/or functioning of central auditory circuits. Investigating the diversity, causes and underlying mechanisms of these central dysfunctions, the ways in which they could impede the expected benefits of hearing restoration by peripheral gene therapy, and determining how these problems could be remedied is becoming a research field in its own right. Here, we provide an overview of the current knowledge about the central deficits associated with genetic forms of deafness.

Congenital Hypothyroidism and the Deleterious Effects on Auditory Function and Language Skills: A Narrative Review

Congenital hypothyroidism (CH) is an endocrine disease commonly found in newborns and is related to the absence or reduction of thyroid hormones (THs), which are essential for development since intrauterine life. Children with CH can develop hearing problems as THs are crucial for the auditory pathway’s development and maturation. Sensory deprivations, especially in hearing disorders at early ages of development, can impair language skills, literacy, and behavioral, cognitive, social, and psychosocial development. In this review we describe clinical and molecular aspects linking CH and hearing loss.