Acesso a serviço de saúde e reabilitação auditiva de adultos e idosos durante o período inicial da pandemia COVID-19 no Brasil / Access to health service and hearing rehabilitation for adult and older adults during the initial period of the COVID-19 pandemic in Brazil / Acceso a servicios de salud yrehabilitación auditiva para adultos y ancianos durante el período inicial de la pandemia COVID-19 en Brasil

Introdução: A perda auditiva é uma deficiência comum na população mundial e contribui para dificuldade na comunicação verbal e redução da qualidade de vida, evidenciando a importância da identificação precoce, reabilitação e acompanhamento audiológico dessa deficiência para mitigar suas consequências. Durante a pandemia da COVID-19, as medidas restritivas diminuíram a capacidade de atendimento dos serviços de saúde auditiva e dificultaram a busca de auxílio para resolver problemas relacionados à adaptação aos dispositivos eletrônicos de amplificação sonora (DAES), sendo uma barreira no processo de reabilitação da perda auditiva. Objetivo: Caracterizar os usuários de DEAS e o processo inicial de reabilitação auditiva de adultos e idosos e verificar fatores associados ao retorno para a consulta de monitoramento auditivo durante o período inicial da pandemia da COVID-19.Métodos: Estudo observacional transversal com usuários adultos e idosos de um serviço ambulatorial de saúde auditiva com retorno para consulta de monitoramento auditivo agendada no período inicial da implementação das medidas restritivas da pandemia da COVID-19 no Brasil. Resultados: A maioria dos participantes conseguiu retornou para a consulta de monitoramento auditivo, sendo eles em sua maioria idosos, do sexo feminino e vacinados contra a COVID-19. Houve maior prevalência de adaptação adequada aos DAES. Não houve associação estatística entre as variáveis relacionadas à adaptação aos DAES, COVID-19 e saúde mental e o retorno à consulta de monitoramento auditivo. Conclusão: Os fatores relacionados à adaptação aos DAES, à COVID-19 ou à saúde mental não influenciaram o retorno à consulta de monitoramento auditivo na presente pesquisa. (AU)

Introduction: Hearing loss is a common disability in the world population and contributes to difficulty in verbal communication and reduced quality of life, highlighting the importance of early identification, rehabilitation and audiological monitoring of this disability to mitigate its consequences. During the COVID-19 pandemic, restrictive measures reduced the service capacity of hearing health services and made it difficult to seek help to solve problems related to adaptation to personal sound amplification products (PSAPs), being a barrier in the rehabilitation process of hearing loss. Aim: To characterize PSAPs users and the initial hearing rehabilitation process for adults and elderly people and verify the factors associated with the return to hearing monitoring consultations in the initial period of the COVID-19 pandemic. Methods: Cross-sectional observational study with adults and elderly people: elderly users of an outpatient hearing health service who return for a scheduled hearing monitoring consultation in the initial period of the implementation of restrictive measures of the COVID-19 pandemic in Brazil. Results: Most participants were able to return to the hearing monitoring clinic, the majority of whom were elderly, female and vaccinated against COVID-19. There was a higher prevalence of adequate adaptation to the PSAPs. There was no statistical association between variables related to adaptation to PSAPs, COVID-19 and mental health and return to hearing monitoring consultation. Conclusion: Factors related to adaptation to PSAPs, COVID-19 or mental health did not influence the return to hearing monitoring consultation in the present investigation. (AU)

Introducción: La pérdida auditiva es una discapacidad común en la población mundial y contribuye a la dificultad en la comunicación verbal y a la reducción de la calidad de vida, destacando la importancia de la identificación temprana, rehabilitación y seguimiento audiológico de esta discapacidad para mitigar sus consecuencias. Durante la pandemia de COVID-19, las medidas restrictivas redujeron la capacidad de atención de los servicios de salud auditiva y dificultaron la búsqueda de ayuda para resolver problemas relacionados con la adaptación a dispositivos electrónicos de amplificación del sonido (DEAS), siendo una barrera en el proceso de rehabilitación de la pérdida auditiva. Objetivo: Caracterizar a los usuarios de DEAS y el proceso inicial de rehabilitación auditiva de adultos y ancianos y verificar los factores asociados al retorno a las consultas de monitorización auditiva en el período inicial de la pandemia COVID-19. Métodos: Estudio observacional transversal con adultos y ancianos: ancianos usuarios de un servicio ambulatorio de salud auditiva que regresan para consulta de monitorización auditiva programada en el período inicial de la implementación de medidas restrictivas de la pandemia de COVID-19 en Brasil. Resultados: La mayoría de los participantes pudieron regresar a la clínica de monitorización auditiva, la mayoría de los cuales eran ancianos, mujeres y estaban vacunados contra COVID-19. Hubo mayor prevalencia de adaptación adecuada a la DEAS. No hubo asociación estadística entre variables relacionadas con adaptación a DEAS, COVID-19 y salud mental y retorno a consulta de monitorización auditiva. Conclusión: Los factores relacionados con la adaptación a DEAS, el COVID-19 o la salud mental no influyeron en el retorno a la consulta de monitorización auditiva en la presente investigación. (AU)

Detailed analysis of inner ear malformations in CHARGE syndrome patients - correlation with audiological results and proposal for computed tomography scans evaluation methodology

Abstract Objectives The aim was to describe the spectrum of inner ear malformations in CHARGE syndrome and propose a Computed Tomography (CT) detailed scan evaluation methodology. The secondary aim was to correlate the CT findings with hearing thresholds. Methods Twenty ears of ten patients diagnosed with CHARGE syndrome were subjected to CT analysis focusing on the inner ear and internal acoustic canal. The protocol used is presented in detail. ASSR results were analyzed and correlated with inner ear malformations. Results Cochlear hypoplasia type III was the most common malformation found in 12 ears (60%). Cochlear hypoplasia type II, aplasia with a dilated vestibule, and rudimentary otocyst were also identified. In 20%, no cochlear anomaly was found. The lateral Semicircular Canal (SCC) absence affected 100% of ears, the absence of the posterior SCC 95%, and the superior SCC 65%. Better development of cochlea structures and IAC correlated significantly with the lower hearing thresholds. Conclusion This study demonstrated that rudimentary SCC or a complete absence of these SCCs was universally observed in all patients diagnosed with CHARGE syndrome. This finding supports the idea that inner ear anomalies are a hallmark feature of the CHARGE, contributing to its distinct clinical profile. The presence of inner ear malformations has substantial clinical implications. Audiological assessments are crucial for CHARGE syndrome, as hearing loss is common. Early detection of these malformations can guide appropriate interventions, such as hearing aids or cochlear implants, which may significantly improve developmental outcomes and communication for affected individuals. Recognizing inner ear malformations as a diagnostic criterion presents implications beyond clinical diagnosis. A better understanding of these malformations can advance the knowledge of CHARGE pathophysiology. It may also help guide future research into targeted therapies to mitigate the impact of inner ear anomalies on hearing and balance function. Level of evidence: 4.

Impairments and related social inequalities among adults: a population-based study in São Paulo city, Brazil / Deficiências e desigualdades sociais entre adultos: um estudo de base populacional na cidade de São Paulo, Brasil

Abstract The study of the association of social variables with the prevalence of impairments can provide subsidies for more adequate care and health policies for the most needy people by incorporating social aspects. This article aims to estimate the prevalence of diverse types of impairments, the degree of difficulty, limitations, and the need for help they cause and attest whether this prevalence differ by educational attainment in individuals aged 20 years or older. This is a populational cross-sectional study (2015 Health Survey of São Paulo-ISA Capital). Data from 3184 individuals were analyzed via educational attainment as exposure variable and outcome variables related to visual, hearing, intellectual, and mobility impairments. 19.9% of participants had visual, 7.8%, hearing, 2.7%, intellectual, and 7.4%, mobility impairments. Mobility and intellectual impairments limited participants' daily activities the most, 70.3% and 63.3%, respectively; who, thus, needed the most help: 48.9% and 48.5%, respectively. Lower schooling was associated with a higher prevalence of impairments, greater need for help due to visual and intellectual impairments, and greater limitations due to hearing and visual impairments.

Resumo O estudo da associação de variáveis ​​sociais com a prevalência de deficiências pode fornecer subsídios para uma atenção e políticas de saúde mais adequadas às pessoas mais carentes ao incorporar aspectos sociais. O objetivo deste artigo é estimar a prevalência de diversos tipos de deficiências, o grau de dificuldade, as limitações e a necessidade de ajuda e verificar se essa prevalência difere por escolaridade em indivíduos com 20 anos ou mais. Trata-se de um estudo transversal populacional (Inquérito de Saúde de São Paulo 2015 - ISA-Capital). Os dados de 3.184 indivíduos foram analisados ​​com a escolaridade como variável de exposição relacionada às deficiências visuais, auditivas, intelectuais e de mobilidade. Dezenove vírgula nove por cento dos participantes apresentavam deficiência visual, 7,8% auditiva, 2,7% intelectual e 7,4% de mobilidade. Mobilidade e deficiência intelectual foram as que mais limitaram as atividades diárias, 70,3% e 63,3%, respectivamente, sendo, portanto, as que mais necessitaram de ajuda: 48,9% e 48,5%, respectivamente. Menor nível de escolaridade mostrou associação com maior prevalência de deficiências, maior necessidade de ajuda por deficiência visual e intelectual e maiores limitações por deficiência auditiva e visual.

Idosos com perda auditiva e declínio cognitivo: desempenho da percepção de fala no ruído / Elderly people with hearing loss and cognitive decline: speech perception performance in noise

RESUMO Objetivo Verificar a influência das habilidades intelectuais-cognitivas verbais na percepção de fala no ruído, em idosos com perda auditiva sensorioneural, considerando a escolaridade, a idade e o grau da perda auditiva. Método Participaram 36 idosos entre 60 e 89 anos com perda auditiva sensorioneural bilateral, que após avaliação psicológica por meio do Wechsler Intelligence Scale for Adults (WAIS III), foram divididos em (GI) 24 idosos sem alteração cognitiva e (GII) 12 idosos com risco de alteração cognitiva. Foram submetidos à avaliação otorrinolaringológica, entrevista audiológica, audiometria tonal liminar e a avaliação da percepção de fala no ruído por meio do Hearing in Noise Test (HINT-Brasil). O teste estatístico U de Mann-Whitney comparou os resultados entre os grupos, e a correlação de Spearman verificou as variáveis idade, grau da perda auditiva e nível de escolaridade. Resultados Não houve diferença entre os grupos na habilidade de percepção de fala no ruído, exceto na condição ruído à esquerda, no qual o GII apresentou melhor desempenho no HINT-Brasil. O grau da perda auditiva e o nível de escolaridade influenciaram na percepção de fala no ruído. O nível de escolaridade teve correlação com os resultados do WAIS III. Conclusão O declínio das habilidades intelectuais-cognitivas verbais não interferiu na percepção de fala no ruído nos idosos com perda auditiva. O grau da perda auditiva e o nível de escolaridade influenciaram no desempenho dos idosos no teste de percepção de fala no ruído. O desempenho nas habilidades cognitivas verbais variou com o nível de escolaridade.

ABSTRACT Purpose To verify the influence of verbal intellectual-cognitive skills on speech perception in noise, in elderly with sensorineural hearing loss, considering education, age, and degree of hearing loss. Methods 36 elderly between 60 and 89 years old with bilateral sensorineural hearing loss participated in the study. After psychological assessment using the Wechsler Intelligence Scale for Adults (WAIS-III), they were grouped into (GI) 24 elderly without cognitive alteration and (GII) 12 elderly with risk of cognitive alteration. They underwent otorhinolaryngological assessment, audiological interview, pure tone audiometry, and assessment of speech perception in noise using the Hearing in Noise Test (HINT-Brazil). The Mann-Whitney U statistical test compared the results between the groups, and the Spearman correlation verified the variable's age, degree of hearing loss, and level of education. Results There was no difference between the groups in the ability to perceive speech in noise, except in the noise on the left condition, in which GII showed better performance in HINT-Brazil. The degree of hearing loss and level of education influenced the perception of speech in noise. The level of education was correlated with the WAIS-III results. Conclusion The decline in verbal intellectual-cognitive skills did not affect speech perception of noise in the elderly with hearing loss. The degree of hearing loss and level of education influenced the performance of the elderly in the speech perception test in noise. Performance in verbal cognitive skills varied according to the level of education.

Outcomes of Endoscopic Stapedectomy: Systematic Review

Abstract Introduction Stapes surgery was traditionally performed with the use of microscopy either through postauricular, endaural or transcanal approaches. Endoscopic stapedectomy ushered a revolution as a new technique with less complications. Objective To review the outcomes of endoscopic stapes surgery with an emphasis on intraoperative and postoperative clinical and audiological results. Data Synthesis A literature review on the PubMed, Web of Science, Scopus, the Cochrane Library, and Embase databases was conducted. Endoscopic stapes surgery or stapedotomy were the main keywords used, and we searched for studies and research published from January 2015 to October 2021. Articles on endoscopic stapes surgery were included, and qualitative and descriptive analyses of the studies and outcomes data regarding audiometric changes and postoperative complications were conducted. Articles including patients with cholesteatoma were excluded. A total of 122 studies were retrieved for qualitative and descriptive analyses and to measure the outcomes of endoscopic stapedotomy; only 12 studies met the inclusion criteria, and the rest was excluded. The meta-analysis revealed a statistically significant difference in hearing improvement. The gain in air-bone gap ranged from 9 dB to 16 dB. A low rate of operative and postoperative complications was reported. Conclusions Endoscopic stapes surgery appears to be a reasonable alternative to microscopic stapes surgery, with shorter operative times, low complication rate, and significant hearing improvement. The endoscopic technique enabled a better visualization and less scutum drilling, which was confirmed by all included studies.

Temporal Ordering and Auditory Resolution in Individuals with Sensorineural Hearing Loss

Abstract Introduction Peripheral hearing loss, besides causing inadequate auditory input, can lead to distortions in the tonotopic auditory map and reorganization of neural networks. Therefore, the processing of temporal aspects of a sound stimulus and, consequently, the effectiveness of human communication can be negatively impacted. Objective To test the temporal ordering and auditory resolution of people with mild and moderate sensorineural hearing loss and to compare them with the those of people with normal hearing. Methods A total of 19 right-handed individuals aged 16 to 59 years with mild to moderate postlingually acquired symmetric bilateral sensorineural hearing loss participated in the study. They were submitted to frequency and duration pattern tests and a random gap detection test. Results The mean correct response rate in the frequency pattern test was of 66.3%, and, in the duration pattern test, 71.7%. The mean threshold in the random gap detection test was of 14.1 ms. A comparison with the criteria established for normal subjects without peripheral hearing loss revealed that more than half the subjects had abnormal results in the temporal ordering test, while a smaller fraction had reduced temporal resolution. Conclusions The performance of the subjects with acquired sensorineural hearing loss was poorer than that of the participants without peripheral hearing loss. Their results on the temporal ordering test were also poorer than in the temporal resolution test, demonstrating the importance of analyzing both these auditory skills in people with peripheral hearing loss.

Hearing Performance in Cochlear Implant Users Who Have Facial Nerve Stimulation

Abstract Introduction Facial nerve stimulation (FNS) is a complication in cochlear implant (CI) when the electrical current escapes from the cochlea to the nearby facial nerve. Different management to reduce its effects are available, although changes might result in a less-than-ideal fitting for the CI user, eventually reducing speech perception. Objective To verify the etiologies that cause FNS, to identify strategies in managing FNS, and to evaluate speech recognition in patients who present FNS. Methods Retrospective study approved by the Ethical Board of the Institution. From the files of a CI group, patients who were identified with FNS either during surgery or at any time postoperatively were selected. Data collection included: CI manufacturer, electrode array type, age at implantation, etiology of hearing loss, FNS identification date, number of electrodes that generated FNS, FNS management actions, and speech recognition in quiet and in noise. Results Data were collected from 7 children and 25 adults. Etiologies that cause FNS were cochlear malformation, head trauma, meningitis, and otosclerosis; the main actions included decrease in the stimulation levels followed by the deactivation of electrodes. Average speech recognition in quiet before FNS was 86% and 80% after in patients who were able to accomplish the test. However, there was great variability, ranging from 0% in quiet to 90% of speech recognition in noise. Conclusion Etiologies that cause FNS are related to cochlear morphology alterations. Facial nerve stimulation can be solved using speech processor programming parameters; however, it is not possible to predict outcomes, since results depend on other variables.

Cochlear Implant Activation in the Immediate Postoperative Period in the Operating Room

Abstract Introduction Cochlear implant (CI) activation usually takes place at ∼ 30 days postoperative (PO). In our service, CI surgery is performed with local anesthesia and sedation, so activation is possible with the patient's cooperation, immediately after the CI surgery, still in the operating room (OR). Objective The objective of the present study was to provide the patient with hearing experience with the CI and to assess auditory perception immediately after surgery while still in the OR, as well as to compare impedance telemetry (IT), neural response telemetry (NRT), and comfort (C) level at two moments: in the OR and at the definitive activation, ∼ 30 days PO. Methods Nine adult patients (12 ears) with acquired (postlingual) deafness were included. Auditory perception was evaluated through the Ling Six Sound Check, musical instruments, and clapping, presented in two different programming maps, elaborated using t-NRT, and comparing IT, NRT, and C level between the two moments. Results We observed that while still in the OR, the patient can already present auditory detection and recognition responses. The values of IT, NRT threshold (t-NRT), and C on both dates differed, with statistical significance. Conclusion We concluded that it is possible to provide the patient with an auditory experience with the CI immediately after surgery, and that the auditory experience and the values of electrode IT, NRT, and C vary significantly between the two moments.

Knowledge of and Attitudes Toward Hearing Loss Among Primary Care Physicians in the Public Health Sector of Mauritius

Abstract Introduction Primary care physicians are essential first points of contact for patients with hearing loss. Thus, knowledge of hearing loss and related aspects is essential to ensure the optimal management of individuals with suspected hearing loss. Objective This study aimed to determine the knowledge of and attitudes toward hearing loss among primary care physicians in the public health sector in Mauritius. Methods In this cross-sectional descriptive cohort study, 320 primary care physicians completed an online questionnaire adapted from previous questionnaires on knowledge of and attitudes toward hearing loss. Responses were analyzed using descriptive statistics and cross-sectional analyses. Results Primary care physicians showed limited knowledge of hearing loss in areas such as early identification and intervention, professionals responsible for hearing assessments, and hearing tests used for assessing hearing sensitivity. However, the responses also showed positive attitudes toward hearing loss. Significant associations between knowledge of and attitudes toward hearing loss were obtained regarding the type of physician, length of practice, and department posted in. Ear, nose, and throat specialists, as well as pediatricians, demonstrated significantly higher scores for both knowledge of and positive attitudes toward hearing loss. Conclusions The findings highlight a strong need for ongoing medical education to spread awareness about hearing loss among primary care physicians in the public health sector of Mauritius.

Active Bone Conduction Implant and Adhesive Bone Conduction Device: A Comparison of Audiological Performance and Subjective Satisfaction

Abstract Introduction Atresia of the external auditory canal affects 1 in every 10 thousand to 20 thousand live births, with a much higher prevalence in Latin America, at 5 to 21 out of every 10 thousand newborns. The treatment involves esthetic and functional aspects. Regarding the functional treatment, there are surgical and nonsurgical alternatives like spectacle frames and rigid and softband systems. Active transcutaneous bone conduction implants (BCIs) achieve good sound transmission and directly stimulate the bone. Objective To assess the audiological performance and subjective satisfaction of children implanted with an active transcutaneous BCI for more than one year and to compare the outcomes with a nonsurgical adhesive bone conduction device (aBCD) in the same users. Methods The present is a prospective, multicentric study. The audiological performance was evaluated at 1, 6, and 12 months postactivation, and after a 1-month trial with the nonsurgical device. Results Ten patients completed all tests. The 4-frequency pure-tone average (4PTA) in the unaided condition was of 65 dB HL, which improved significantly to 20 dB HL after using the BCI for 12 months. The speech recognition in quiet in the unaided condition was of 33% on average, which improved significantly, to 99% with the BCI, and to 91% with the aBCD. Conclusion The aBCD demonstrated sufficient hearing improvement and subjective satisfaction; thus, it is a good solution for hearing rehabilitation if surgery is not desired or not possible. If surgery is an option, the BCI is the superior device in terms of hearing outcomes, particularly background noise and subjective satisfaction.

Late Bilateral Sequential Cochlear Implant and Quality of Life

Abstract Introduction Hearing impairment is one of the main disorders that can interfere with the development of speech and language. In an individual, it can cause significant communication difficulties, social isolation, negative feelings, and depressive disorders. The hearing aids (HAs) and cochlear implant (CI) are options for profound and severe hearing loss, and the CI can be indicated for individuals who do not obtain benefits from HAs. Objective To evaluate the quality of life of individuals who underwent sequential bilateral CIs with a long surgical interval between procedures. Methods Fifteen patients, aged 8 to 70 years old, who underwent sequential bilateral CI, with an interval ≥ 4 years between surgeries, were evaluated. Quality of life was evaluated using three questionnaires: WHOQOL-BREF, SSQ-12 and HHIA in Portuguese. Results The WHOQOL-BREF questionnaire showed that the study participants had a good quality of life in all domains assessed. According to the SSQ-12, few reported inability to listen in communication situations. Most individuals were classified as having medium disability by the HHIA, but the social and emotional effects did not significantly affect the quality of life. Conclusion The use of questionnaires to assess the quality of life of patients with hearing impairment is a valuable tool to measure adaptation to CI. Patients undergoing bilateral sequential CI, even with a long interval between procedures, presented high indices of quality of life.

Hearing and functioning: An analysis of the context of a hearing rehabilitation service based on the International Classification of Functioning / Audição e funcionalidade: Análise do contexto de um serviço de reabilitação auditiva com base na Classificação Internacional de Funcionalidade

ABSTRACT Purpose: to verify the association between types and degrees of hearing loss and demographic factors and categories of the International Classification of Functioning, Disability, and Health, related to "Body Structures and Functions" and "Activities and Participation." Methods: a cross-sectional, analytical, observational study with a nonprobabilistic sample, developed with secondary data, according to the International Classification of Functioning, Disability, and Health, obtained from the medical records of patients assessed for hearing rehabilitation at a specialized rehabilitation center. Descriptive and bivariate analyses were performed. Association analyses used Pearson's chi-square test, with the significance level set at 5%. Results: the study analyzed 122 medical records, which revealed a predominance of women, a sensorineural hearing loss of a moderately severe degree and progressive history. The type of hearing loss was associated with three categories of Body Structures and Functions and three categories of Activities and Participation. The degrees of hearing loss were associated with 10 categories of Body Structures and Functions and six categories of Activities and Participation. Conclusion: types and degrees of hearing loss are associated with Body Structures and Functions and Activities and Participation, further impairing communication.

RESUMO Objetivo: verificar a associação do tipo e grau de perda auditiva com os fatores demográficos e as categorias da Classificação Internacional de Funcionalidade, Incapacidade e Saúde relativas a "Estruturas e Funções do Corpo" e "Atividades e Participação". Métodos: trata-se de estudo observacional, analítico e transversal, com amostra não probabilística, desenvolvido com dados secundários, embasados na Classificação Internacional de Funcionalidade, Incapacidade e Saúde, obtidos no prontuário de usuários submetidos à avaliação para reabilitação auditiva, em um Centro Especializado em Reabilitação. Foram realizadas análises descritiva e bivariada. Para as análises de associação, foi utilizado o teste Qui-quadrado de Pearson com o nível de significância de 5%. Resultados: foram analisados 122 prontuários, que revelaram predominância de mulheres, perda auditiva do tipo neurossensorial de grau moderadamente severo e história progressiva. O tipo de perda auditiva apresentou associação com três categorias de Estruturas e Funções do corpo e três categorias de Atividades e Participação. Em relação ao grau da perda auditiva, dez categorias foram associadas com as Estruturas e Funções do corpo e seis categorias com as Atividades e Participação. Conclusão: o tipo e grau da perda auditiva associam-se com as Estruturas e Funções do corpo e com as Atividades e Participação, com maior prejuízo na comunicação.

Characteristics and influencing factors of hearing loss among noise-exposed workers in a large machinery maintenance enterprise / 环境与职业医学

Background The current increasing trend of new cases of occupational noise-induced deafness indicates that the hearing loss of occupational population has not been effectively controlled in China. It is of great significance to study the characteristics of hearing loss among noise-exposed workers and its related factors. Objective To investigate characteristics and influencing factors of hearing loss among occupational noise-exposed workers in a large machinery maintenance enterprise, and to provide a scientific basis to prevent and control noise-induced hearing loss. Methods A cross-sectional survey was conducted to investigate male Han occupational noise-exposed workers in a large mechanical maintenance enterprise. We acquired demographic characteristics, occupational exposure history, and individual life behavior characteristics of the workers through questionnaires, collected occupational exposure level data from annual occupational disease hazard factor surveillance reports, obtained pure tone hearing threshold test data through occupational health examinations, and estimated individual noise exposure levels using cumulative noise exposure (CNE). According to the results of pure tone air conduction hearing threshold test, the workers were divided into a hearing loss group and a normal hearing group. The chi-square test was employed to compare the occupational exposure characteristics and individual life behavior characteristics between the two groups. Additionally, the trend chi-square test was utilized to analyze the changing trends of age, length of service, CNE, and hearing loss rate within the two groups. The relationship between high-frequency hearing loss in both ears and its related influencing factors was assessed by a multiple logistic regression model. Results The M (P25, P75) of CNE for the 2531 occupational noise-exposed workers was 97.51 (95.39, 99.96) dB(A)·year. The incidence of hearing anomaly, binaural high-frequency hearing anomaly, random ear high-frequency hearing anomaly, binaural low-frequency hearing anomaly, and random ear low-frequency hearing anomaly were 22.48%, 16.59%, 22.13%, 2.77%, and 3.52%, respectively. High-frequency hearing threshold increase was the main reason for hearing anomaly (98.42%). In comparison to the CNE ≤ 97 dB(A)·year group, the 97 dB(A)·year<CNE≤ 100 dB(A)·year group and the CNE>100 dB(A)·year group experienced a 36.4% and 52.3% increase in the risk of bilateral high-frequency hearing loss, respectively. The smoking group exhibited a 43.5% elevated risk of bilateral high-frequency hearing loss when compared to the non-smoking group. Conversely, the group frequently wearing hearing protection equipment demonstrated a 23.6% lower risk of bilateral high-frequency hearing loss in comparison to the group occasionally wearing protective equipment. The data suggested that CNE＞97 dB(A)·year and smoking might be independent risk factors for bilateral high-frequency hearing loss, and frequently wearing hearing protection equipment might be an important protective factor. Conclusion Increased CNE and smoking can elevate the risk of high-frequency hearing loss, while personal hearing protection can effectively reduce the risk of hearing loss.

Development of Chinese herbal medicine for sensorineural hearing loss

According to the World Health Organization's world report on hearing, nearly 2.5 billion people worldwide will suffer from hearing loss by 2050, which may contribute to a severe impact on individual life quality and national economies. Sensorineural hearing loss (SNHL) occurs commonly as a result of noise exposure, aging, and ototoxic drugs, and is pathologically characterized by the impairment of mechanosensory hair cells of the inner ear, which is mainly triggered by reactive oxygen species accumulation, inflammation, and mitochondrial dysfunction. Though recent advances have been made in understanding the ability of cochlear repair and regeneration, there are still no effective therapeutic drugs for SNHL. Chinese herbal medicine which is widely distributed and easily accessible in China has demonstrated a unique curative effect against SNHL with higher safety and lower cost compared with Western medicine. Herein we present trends in research for Chinese herbal medicine for the treatment of SNHL, and elucidate their molecular mechanisms of action, to pave the way for further research and development of novel effective drugs in this field.

Clinical features of CAPOS syndrome caused by maternal ATP1A3 gene variation: a case report / 临床耳鼻咽喉头颈外科杂志

CAPOS syndrome is an autosomal dominant neurological disorder caused by mutations in the ATP1A3 gene. Initial symptoms, often fever-induced, include recurrent acute ataxic encephalopathy in childhood, featuring cerebellar ataxia, optic atrophy, areflflexia, sensorineural hearing loss, and in some cases, pes cavus. This report details a case of CAPOS syndrome resulting from a maternal ATP1A3 gene mutation. Both the child and her mother exhibited symptoms post-febrile induction,including severe sensorineural hearing loss in both ears, ataxia, areflexia, and decreased vision. Additionally, the patient's mother presented with pes cavus. Genetic testing revealed a c. 2452G>A（Glu818Lys） heterozygous mutation in theATP1A3 gene in the patient . This article aims to enhance clinicians' understanding of CAPOS syndrome, emphasizing the case's clinical characteristics, diagnostic process, treatment, and its correlation with genotypeic findings.

A case of sudden hearing loss combined with familial hyperlipidemia / 临床耳鼻咽喉头颈外科杂志

Hyperlipidemia is characterized by elevated levels of blood lipids. The clinical manifestations are mainly atherosclerosis caused by the deposition of lipids in the vascular endothelium. The link between abnormal lipid metabolism and sudden hearing loss remains unclear. This article presents a case study of sudden hearing loss accompanied by familial hyperlipidemia. Pure tone audiometry indicated intermediate frequency hearing loss in one ear. Laboratory tests showed abnormal lipid metabolism, and genetic examination identified a heterozygous mutation in theAPOA5 gene. Diagnosis: Sudden hearing loss; hypercholesterolemia. The patient responded well to pharmacological treatment. This paper aims to analyze and discuss thepotential connection between abnormal lipid metabolism and sudden hearing loss.

Genetic and phenotypic analysis of MYO15A rare variants associated with autosomal recessive hearing loss / 临床耳鼻咽喉头颈外科杂志

Objective:To analyze the phenotype and genotype characteristics of autosomal recessive hearing loss caused by MYO15A gene variants, and to provide genetic diagnosis and genetic counseling for patients and their families. Methods:Identification of MYO15A gene variants by next generation sequencing in two sporadic cases of hearing loss at Shanghai Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine. The sequence variants were verified by Sanger sequencing.The pathogenicity of these variants was determined according to the American College of Medical Genetics and Genomics（ACMG） variant classification guidelines, in conjuction with clinical data. Results:The probands of the two families have bilateral,severe or complete hearing loss.Four variants of MYO15A were identified, including one pathogenic variant that has been reported, two likely pathogenic variants,and one splicing variant of uncertain significance. Patient I carries c. 3524dupA（p. Ser1176Valfs*14）, a reported pathogenic variant, and a splicing variant c. 10082+3G>A of uncertain significance according to the ACMG guidelines. Patient I was treated with bilateral hearing aids with satisfactory effect, demonstrated average hearing thresholds of 37.5 dB in the right ear and 33.75 dB in the left ear. Patient Ⅱ carries c. 7441_7442del（p. Leu2481Glufs*86） and c. 10250_10252del（p. Ser3417del）,a pair of as likely pathogenic variants according to the ACMG guidelines. Patient Ⅱ, who underwent right cochlear implantation eight years ago, achieved scores of 9 on the Categorical Auditory Performance-Ⅱ（CAP-Ⅱ） and 5 on the Speech Intelligibility Rating（SIR）. Conclusion:This study's discovery of the rare c. 7441_7442del variant and the splicing variant c. 10082+3G>A in the MYO15A gene is closely associated with autosomal recessive hearing loss, expanding the MYO15A variant spectrum. Additionally, the pathogenicity assessment of the splicing variant facilitates classification of splicing variations.

Genetic characteristic analysis of slight-to-moderate sensorineural hearing loss in children / 临床耳鼻咽喉头颈外科杂志

Objective:To analyze genetic factors and phenotype characteristics in pediatric population with slight-to-moderate sensorineural hearing loss. Methods:Children with slight-to-moderate sensorineural hearing loss of and their parents, enrolled from the Chinese Deafness Genome Project, were studied. Hearing levels were assessed using pure tone audiometry, behavioral audiometry, auditory steady state response（ASSR）, auditory brainstem response（ABR） thresholds, and deformed partial otoacoustic emission（DPOAE）. Classification of hearing loss is according to the 2022 American College of Medical Genetics and Genomics（ACMG） Clinical Practice Guidelines for Hearing Loss. Whole exome sequencing（WES） and deafness gene Panel testing were performed on peripheral venous blood from probands and validations were performed on their parents by Sanger sequencing. Results:All 134 patients had childhood onset, exhibiting bilateral symmetrical slight-to-moderate sensorineural hearing loss, as indicated by audiological examinations. Of the 134 patients, 29（21.6%） had a family history of hearing loss, and the rest were sporadic patients. Genetic causative genes were identified in 66（49.3%） patients. A total of 11 causative genes were detected, of which GJB2 was causative in 34 cases（51.5%）, STRC in 10 cases（15.1%）, MPZL2 gene in six cases（9.1%）, and USH2A in five cases（7.6%）.The most common gene detected in slight-to-moderate hearing loss was GJB2, with c. 109G>A homozygous mutation found in 16 cases（47.1%） and c. 109G>A compound heterozygous mutation in 9 cases（26.5%）. Conclusion:This study provides a crucial genetic theory reference for early screening and detection of mild to moderate hearing loss in children, highlighting the predominance of recessive inheritance and the significance of gene like GJB2, STRC, MPZL2, USH2A.

Genetic counseling for hearing loss today / 临床耳鼻咽喉头颈外科杂志

Genetic counseling for hearing loss today originated from decoding the genetic code of hereditary hearing loss, which serves as an effective strategy for preventing hearing loss and constitutes a crucial component of the diagnostic and therapeutic framework. This paper described the main principles and contents of genetic counseling for hearing loss, the key points of counseling across various genetic models and its application in tertiary prevention strategies targeting hearing impairment. The prospects of an AI-assisted genetic counseling decision system and the envisions of genetic counseling in preventing hereditary hearing loss were introduced. Genetic counseling for hearing loss today embodies the hallmark of a new era, which is inseparable from the advancements in science and technology, and will undoubtedly contribute to precise gene intervention!

Stem Cell-Based Hair Cell Regeneration and Therapy in the Inner Ear / 神经科学通报·英文版

Hearing loss has become increasingly prevalent and causes considerable disability, thus gravely burdening the global economy. Irreversible loss of hair cells is a main cause of sensorineural hearing loss, and currently, the only relatively effective clinical treatments are limited to digital hearing equipment like cochlear implants and hearing aids, but these are of limited benefit in patients. It is therefore urgent to understand the mechanisms of damage repair in order to develop new neuroprotective strategies. At present, how to promote the regeneration of functional hair cells is a key scientific question in the field of hearing research. Multiple signaling pathways and transcriptional factors trigger the activation of hair cell progenitors and ensure the maturation of newborn hair cells, and in this article, we first review the principal mechanisms underlying hair cell reproduction. We then further discuss therapeutic strategies involving the co-regulation of multiple signaling pathways in order to induce effective functional hair cell regeneration after degeneration, and we summarize current achievements in hair cell regeneration. Lastly, we discuss potential future approaches, such as small molecule drugs and gene therapy, which might be applied for regenerating functional hair cells in the clinic.