Hearing and Visual Impairment in Children with Cerebral Palsy, A Study in Tertiary Level Hospital in Bangladesh

Background: Cerebral palsy (CP) is a diagnostic term used to describe a group of motor syndromes resulting from disorders of early brain development. CP remains unexplained in most cases and is typically diagnosed outside the neonatal period. Visual impairment decreases the quality of life and hearing impairment hampers linguistic development. This study aimed to observe the visual and hearing impairment of children with cerebral palsy associated with developmental disabilities. Material & Methods: This is a cross-sectional observational descriptive study carried out in the department of Paediatric Neurology CMH Dhaka from March 2018 to February 2022. The participants (N=120) were from birth to 12 years of age. Detailed information was obtained in each case according to protocol. The pediatric neurologist based on the study definition crosschecked the diagnosis. The hearing assessment was done with the help of an Otolaryngologist in the department of Otolaryngology and the ophthalmological evaluation was done with the help of an ophthalmologist of the department of ophthalmology of CMH Dhaka. Hearing impairment, Ophthalmological motor disturbance, and developmental disabilities were correlated. A convenience sampling technique was used in this study. Relevant data were collected from hospital records. All the information was recorded in the fixed protocol. Collected data were classified, edited, coded, and entered into the computer for statistical analysis by using SPSS 2021. Results: In this study, among the 120 children with CP, the mean age of the study was 5.57(SD±3.89) age range of 2-12 years Males were 82(68%) and females were 38(32%), male-female ratio (sex M: F 2.15: 1). Spastic quadriplegia constitutes the predominant group 59(49%), followed by Spastic diplegia 21(18%), Spastic hemiplegia 20(17.5%), dyskinetic CP 09(8%), 7(6%) were mixed CP and 4(3%) were hypotonic CP. Hearing impairment was found in 87 (72%) cases and visual impairment was detected in 92(76%). Among them, the child with Spastic Quadriplegic (83%) and diplegic CP (62%) children had a hearing impairment. Regarding vision, no fix and follow were observed in 69(57%) cases. Among the studied CP child (n=120) 16 children (13.33%) had normal eye finding and 104 (86%) had abnormal eye findings. Refractive error (32.5%) and Squint (19%) were the most common ocular defect among studied patients. Other ocular defect were nystagmus(10%), optic atrophy(12.5%), microphthalmia(10%), corneal opacity(3%), optic hypoplasia(4.1%), cataract(2.5%), pigmentary retinopathy(5%), retro-lental fibroplasias(3.3%) and ptosis(2.5%). In children with hypotonic (athetoid) and mixed CP, almost all (11 of 11) children had visual impairment. Spastic quadriplegic CP children (80%) and Spastic diplegic CP children (90%) had one or more domains of visual impairment. Conclusion: Cerebral Palsy is a non-progressive various form of neurological disorder in children. Early visual screening and hearing assessment can help CP children to minimize mental retardation, learning difficulties, and speech delay.

The mediating effect of self-regulation fatigue between acceptance of hearing impairment and work withdrawal behavior in young and middle-aged patients with sudden deafness / 中国实用护理杂志

Objective:To explore the mediating effect of self-regulation fatigue between acceptance of hearing impairment and work withdrawal behavior in young and middle-aged patients with sudden deafness, so as to provide reference for the formulation of intervention measures for job withdrawal behavior.Methods:A cross-sectional survey was conducted to facilitate the selection of 326 young and middle-aged patients with sudden deafness who were treated in the Department of Otolaryngology, Head and Neck Surgery in the First Affiliated Hospital and the Second Affiliated Hospital of Air Force Military Medical University of the Chinese PLA from February 2021 to January 2022. The survey was carried out by general information questionnaire, the Revised Acceptance Disability Scale, the Self-Regulatory Fatigue Scale, and the Work Withdrawal Behavior Scale. Structural equation model method was used to analyze the mediating effect.Results:The acceptance of hearing impairment score in young and middle-aged patients with sudden deafness was (59.82 ± 10.99) points, the self-regulation fatigue score was (60.38 ± 8.84) points, and the work withdrawal behavior score was (39.06 ± 6.51) points. Self-regulation fatigue was negatively correlated with acceptance of hearing impairment ( r=-0.541, P<0.01). Work withdrawal behavior was negatively correlated with acceptance of hearing impairment ( r=-0.488, P<0.01), and was positively correlated with self-regulation fatigue ( r=0.587, P<0.01). Self-regulation fatigue played a partial mediating effect between the acceptance of hearing impairment and work withdrawal in young and middle-aged patients with sudden deafness, and the mediating effect ratio was 56.30%. Conclusions:The acceptance of hearing impairment can not only directly affect the work withdrawal behavior of young and middle-aged patients with sudden deafness, but also indirectly affect their work withdrawal behavior through self-regulation fatigue. Medical staff should pay attention to the internal relationship between the acceptance of hearing impairment, self-regulation fatigue and work withdrawal behaviors, so as to improve the acceptance of hearing impairment of patients, relieve their self-regulation fatigue, and avoid the occurrence of work withdrawal behaviors.

Deafness genetic mutation spectrum in nonsyndromic hearing impairment associated with enlarged vestibular aqueducts / 中国康复理论与实践

ObjectiveTo investigate the deafness genetic mutation spectrum in nonsyndromic hearing impairment (NSHI) associated with enlarged vestibular aqueducts (EVA). MethodsFrom October, 2015 to August, 2016, 85 patients with NSHI from Hubei Yichang Special Education School were examined with temporal bone CT, and 20 deafness-related gene mutations in GJB2, GJB3, SLC26A4 and mtDNA 12S rRNA were detected with matrix-assisted laser desorption/ionization time-of-flight mass spectrometry. ResultsA total of 31 patients were found EVA with temporal bone CT. Compared with non-EVA patients, the proportion of deafness-related gene mutations was more in patients with EVA (χ2 = 11.160, P = 0.001), especially for c.919-2A>G mutation of SLC26A4 (χ2 = 23.870, P < 0.001). ConclusionThe deafness gene mutation spectrum is different in NSHI patients with or without EVA. It is needed to optimize genetic testing scheme for deafness for early diagnosis and intervention of NSHI associated with EVA.

Teleintervenção guiada por videofeedback à família de uma criança usuária de implante coclear: estudo de caso / Videofeedback-guided teleintervention for the family of a child with a cochlear implant: a case study

RESUMO Intervenções para a capacitação parental de famílias de crianças com deficiência auditiva, incluindo as usuárias de implante coclear, são apontadas como otimizadoras em seus resultados de desenvolvimento. Neste estudo de intervenção de caso único, temos por objetivos descrever o uso do videofeedback em ambiente remoto, bem como identificar a sua efetividade, a partir da análise da interação da mãe e criança, tanto para os comportamentos comunicativos da mãe como para os comportamentos da linguagem receptiva e expressiva da criança. Medidas pré e pós-intervenção foram realizadas, a partir da análise de vídeos de interação da mãe com a criança, por juízes cegos, bem como pela aplicação de instrumentos de avaliação da criança e da mãe. Foram realizadas 13 sessões, sendo 3 de avaliação nos momentos pré e pós-intervenção e 10 sessões de teleconsulta em que se empregou a ferramenta de videofeedback com a mãe. Os dados foram analisados de forma descritiva e inferencial, por meio do método JT, que determinou o Índice de Mudança Confiável (IMC) e a Significância Clínica. Houve mudança positiva confiável na linguagem receptiva e expressiva da criança, bem como mudança positiva confiável e mudança clínica significativa na interação da mãe com a criança após as 10 sessões de intervenção remota com videofeedback. A partir das mudanças confiáveis observadas neste estudo, apresentamos este modelo (Televideofeedback), como potencial para otimizar os recursos e esforços para o sucesso terapêutico na reabilitação auditiva infantil, o qual deve ser estudado em pesquisas com método rigoroso, para a ampla recomendação de seu uso.

ABSTRACT Interventions for parental training for families of hard of hearing children, including cochlear implant users, are identified as optimizing their developmental outcomes. In this single-case intervention study, we aim to describe the use of videofeedback in a remote environment, as well as to identify its effectiveness, based on the analysis of mother-child interaction, both for the mother's communicative behaviors and for the behaviors of the mother, receptive and expressive language of the child. Pre- and post-intervention measurements were performed, based on video analysis of the mother's interaction with the child, by blind judges, as well as through the application of assessment instruments for the child and the mother. There were 13 sessions, 3 of which were for evaluation before and after the intervention and 10 of teleconsultation sessions in which the videofeedback tool was used with the mother. Data were analyzed descriptively and inferentially, using the JT method, which determined the Reliable Change Index (BMI) and Clinical Significance. There was reliable positive change in the child's receptive and expressive language, as well as reliable positive change and clinically significant change in mother-child interaction after the 10 sessions of remote videofeedback intervention. Based on the reliable changes observed in this study, we present this model (televideofeedback) as a potential to optimize resources and efforts for therapeutic success in children's auditory rehabilitation, which should be studied in research with a rigorous method, for the broad recommendation of its use.

Impacto da protetização auditiva na qualidade de vida do adulto com deficiência auditiva: revisão de escopo / Impact of hearing aids on the quality of life of adults with adult disabilities: scope review

RESUMO Objetivo Verificar os benefícios da protetização auditiva na qualidade de vida do adulto com deficiência auditiva. Estratégia de pesquisa Revisão de escopo, guiada pelas recomendações PRISMA. Critérios de seleção A busca foi realizada com o auxílio dos unitermos "auxiliares de audição", "aparelho auditivo", "implante coclear", "fonoaudiologia", "deficiência auditiva", "perda auditiva", "qualidade de vida", e seus respectivos em inglês e espanhol, nos bancos virtuais de dados: LILACS, SciELO, PubMed, Scopus, Web of Science e OpenGrey. Os dados foram analisados qualitativamente. Resultados Foram identificados 1.312 registros e selecionados 6 artigos, com população protetizada auditivamente, dos 18 aos 92 anos, de ambos os gêneros, usuários de implante coclear e aparelho de amplificação sonora individual. Foram utilizados os seguintes instrumentos: Questionário de avaliação das expectativas do adulto/idoso novo usuário de próteses auditivas, International Outcome Inventory For Hearing Aids, Questionário Nijmegen de Implantes Cocleares, Questionário de Satisfação do Cliente, Caregiver Strain Questionnaire, Formulário de Questionário Relativo ao Índice, World Health Organization Quality of Life-bref, Glasgow Health Status Inventory, Abbreviated Profile of Hearing Aid Benefit, Cochlear Implant Quality of Life, Caregiver Burden Scale, Escala Quantificada de Denver e Escala Visual Analógica. Os resultados indicaram melhor qualidade de vida nos domínios testados, tanto na visão dos adultos surdos quanto na de seus parceiros. Conclusão Apesar da heterogeneidade dos protocolos de qualidade de vida utilizados nos estudos selecionados, foi possível verificar que a protetização auditiva melhora a qualidade de vida do adulto com deficiência auditiva.

ABSTRACT Purpose To verify the benefits of hearing aids in the quality of life of adults with hearing loss. Research strategy Scoping review guided by PRISMA recommendations. Selection criteria with the help of the keywords "hearing aids", "hearing aid", "cochlear implant", "speech therapy", "hearing impairment", "hearing loss", "quality of life", and their respective in English and Spanish in the virtual databases: LILACS, SciELO, PubMed, SCOPUS, Web of Science and Open Grey. Qualitatively analyzed. Results 1,312 records were identified and six articles were selected, with a hearing aided population from 18 to 92 years old, from both genders, cochlear implant and individual sound amplification device users. Questionnaires were used (assessment of expectations of adults/elderly users of hearing aids, International Outcome Inventory For Hearing Aids, Nijmegen Cochlear Implant, Satisfaction, Caregiver Strain Questionnaire, Relative to the Index, World Health Organization Quality of Life-bref, Glasgow Health Status Inventory, Abbreviated Profile of Hearing Aid Benefit, Cochlear Implant Quality of Life and scales (Care Giving Burden Scale), Denver Quantified and visual analog). The results indicated a better quality of life in the domains tested, both in the view of deaf adults and their partners. Conclusion Despite the heterogeneity of the quality of life protocols used in the selected studies, it was possible to verify that hearing aids increase the quality of life of adults with hearing loss.

Beginner's Intelligibility Test (BIT): translation, cultural adaptation to Brazilian Portuguese and validation

Abstract Objective: Translate and validate the Beginner's Intelligibility Test (BIT) speech intelligibility assessment instrument into Brazilian Portuguese. Method: Study developed in two stages: 1st translation and cross-cultural adaptation of the Beginner's Intelligibility Test instrument into Brazilian Portuguese; 2nd application of the instrument. The second stage of the study involved 20 children using cochlear implants, aged between 4 and 11 years old, enrolled in the Cochlear Implant Program at Hospital das Clínicas de Ribeirão Preto, Faculty of Medicine of Ribeirão Preto, University of São Paulo. All data collection procedures were video, and audio recorded for analysis by judges. After orthographic transcription of each sentence repeated by the child, the judge was asked to score the participant's speech intelligibility and classify it according to criteria established in the literature. Results: The translation stage took place for the four lists and 40 sentences of the Beginner's Intelligibility Test (BIT) instrument into Brazilian Portuguese, the semantic, idiomatic, experimental and conceptual equivalences were considered, and it took place without any difficulties identified by the translators. The terms used in the Portuguese language were similar and those that presented differences among the translators did not bring significant divergences to its understanding. In the inter-evaluator analysis, there was reliability between the classification and the score obtained. Different judges evaluated the same children and a concordance was observed in classification and scoring. Conclusions: Face validity of the BIT was confirmed through the understanding of each sentence of the four lists by the majority of children using cochlear implants participating in the pretest phase. The content validity among experts was unanimous for the four lists of sentences. The Brazilian Portuguese adapted version maintained the semantic, idiomatic, conceptual, and cultural equivalence, according to the evaluation of the expert committee. Evidence level: 02.

Influência da escolaridade de famílias de crianças e adolescentes com deficiência auditiva em suas necessidades de informação: estudo descritivo / Influence of educational level of families of hard of hearing children and adolescents on their informational needs: descriptive study

RESUMO Objetivo avaliar a influência da escolaridade de responsáveis por crianças com deficiência auditiva com níveis de educação formal do ensino fundamental ao ensino médio, em relação às suas necessidades de informação no contexto de um serviço de reabilitação auditiva do Sistema Único de Saúde. Métodos estudo transversal, observacional, com amostra de conveniência de 58 responsáveis por crianças com deficiência auditiva. Aplicou-se o Inventário de Necessidades Familiares traduzido e adaptado para o português brasileiro e foram coletados os dados de escolaridade dos responsáveis e as variáveis idade da criança e idade no diagnóstico. Foi realizada análise descritiva e inferencial. Resultados todas as famílias apresentaram necessidades de informação, sendo que, para as famílias com menor escolaridade, a necessidade de informações sobre a audição e os dispositivos auditivos foi mais frequente. Na análise de regressão, não se observou influência da escolaridade na quantidade de necessidades de informação, mesmo considerando no modelo a idade cronológica da criança e a idade no seu diagnóstico. Conclusão famílias de todas as escolaridades analisadas apresentaram necessidades de informação, sendo que a escolaridade não influenciou a quantidade de informações requeridas. Foi possível observar diferença qualitativa nos tópicos de necessidades de informação, o que alerta para a importância de investigações sobre as necessidades das famílias em programas de reabilitação auditiva infantil, de modo a se efetivar abordagens mais centradas nas famílias.

ABSTRACT Purpose To evaluate the influence of education of guardians of hard of hearing children with formal education levels from elementary to high school, in relation to their information needs in the context of an auditory rehabilitation service. Methods Cross-sectional, observational study, with a convenience sample of 58 guardians of children with hearing loss. The Family Needs Inventory (INF) was applied, translated, and adapted into Brazilian Portuguese, and parents' education was collected, in addition to the variables age of the child and age at diagnosis. Results In the quantitative analysis of the "yes" responses from the INF, all the families presented need for information , and for families with less education, the need for information about hearing and hearing devices was greater. In the regression analysis, there was no influence of schooling on the amount of information needed, even considering the chronological age and diagnosis of the child in the model. As limitations of this study, we highlight the absence of sufficient number of families with higher education for the analysis of the final model, as well as the impossibility of including other variables in the analysis. Conclusion The analyzed families with different levels of schooling showed need for information , and schooling did not influence the amount of information required by them. It was possible to observe a qualitative difference in the topics of needed information, which alerts to the importance of investigation about the needs of families in child auditory rehabilitation programs, to implement more family-centered approaches.

Desenvolvimento e validação do BATUTA: um teste para avaliação da percepção musical de pessoas com deficiência auditiva / Development and validation of BATUTA: a test to evaluate the musical perception of people with hearing impairment

RESUMO Objetivo Descrever o desenvolvimento e a validação de um teste de percepção musical, denominado BATUTA, destinado a avaliar a percepção musical de pessoas com deficiência auditiva, usuárias de aparelhos de amplificação sonora individual (AASI). O BATUTA é um teste computadorizado composto por 35 subtestes divididos nos módulos ritmo, pitch e timbre para os quais os participantes devem responder se as amostras sonoras e/ou os trechos musicais apresentados, aos pares, são iguais ou diferentes. Método O processo de construção do BATUTA foi composto por quatro etapas: desenvolvimento do teste, submissão da versão inicial ao comitê de especialistas para validação de conteúdo; aplicação do piloto em 51 participantes com audição normal e reteste para validação da confiabilidade, fundamentadas em reconhecidas recomendações para elaboração e validação de instrumentos de avaliação. Diretrizes relacionadas à natureza das amostras sonoras utilizadas, aos atributos musicais avaliados, ao ambiente de testagem e ao tipo de resposta indicada para a finalidade do BATUTA foram amplamente investigadas, com o propósito de lhe conferir fidedignidade. Resultados Os índices de Validação de Conteúdo (IVC) e de concordância entre os especialistas, quando analisados juntamente com as recomendações do comitê, resultaram em correções e em novas gravações dos áudios para garantir o cumprimento do teste. Os escores da aplicação do teste piloto indicaram boa consistência interna e o reteste confirmou a confiabilidade do BATUTA. Conclusão Os resultados demonstraram a viabilidade do BATUTA para avaliar a percepção musical de pessoas com deficiência auditiva usuárias de AASI.

ABSTRACT Purpose To describe the development and validation of a test, called BATUTA, that assesses the musical perception of people with hearing impairment that are hearing aid (HA) users. BATUTA is a computerized test with 35 subtests, divided into the rhythm, pitch, and timbre modules, and the participants must answer whether the sound samples and/or parts of the songs, presented in pairs, are the same or not. Methods The BATUTA creation process consisted of four stages: test development, submission to the expert committee for content validation; pilot application with 51 normal hearing participants and retest to validate reliability. The process was based on several recommendations for the development and validation of musical assessment instruments. A deep investigation of the guidelines related to sound samples used, musical attributes evaluated, testing environment and the most appropriate response method was undertaken to ensure dependability. Results The Content Validity Index (CVI) and expert agreement rates, when analyzed with the committee's recommendations, resulted in corrections and new audio recordings to ensure compliance to the test. The pilot test scores indicated internal consistency and the retest confirmed the reliability of BATUTA. Conclusion The results demonstrated the viability of BATUTA to assess the musical perception of people with hearing impairment that are HA users.

Effect of psychological intervention on anxiety or depression in children and adolescents with hearing impairment: a systematic review / 中国康复理论与实践

ObjectiveTo systematically review the effect of psychological intervention on anxiety and depression in hearing-impaired children and adolescents. MethodsThe literature was researched from the databases of PubMed, Web of Science, Embase, CNKI, and Wanfang Database on psychological intervention on anxiety and depression for hearing-impaired children and adolescents until July, 2023. After screening, author, country, study object, study design, intervention mode, outcome, and measurement tools were extracted from the literature. ResultsEight articles from three countries were included, including eight randomized controlled trials, involving 404 participants, publishing mainly from 2013 to 2023. The types of psychological intervention included family support education, emotional intelligence training, cognitive behavioral therapy, self-confidence group training, and play therapy, and so on, which improved social phobia, and relieved the levels of anxiety and depression of children and adolescents with hearing impairment. ConclusionPsychological intervention can effectively alleviate the anxiety and depression of children and adolescents with hearing impairment.

Assessment of Wave V latency and Wave I-V Interpeak latency in preterm babies by Brainstem Evoked Response Audiometry

Background: Early screening of hearing impairment optimizes communication, social, academic, and vocational outcomes for each child with hearing loss measurement of the auditory brain stem response which is considered the most sensitive method of assessing the auditory activity of neonates. Aims and Objectives: This study aims to compare wave V latency and interpeak I-V latency by brainstem auditory evoked response in preterm babies (32 weeks–36 weeks) with age-specific normal response and intergroup comparison (Group 1–32 weeks, Group 2–34 weeks, and Group 3–36 weeks) for the identification of hearing impairment if any. Materials and Methods: The present study was done on 50 preterm newborn, and after satisfying inclusion and exclusion criteria, preterm babies were subdivided into three groups on the basis of gestational age (Group A: 32 weeks [n = 12], Group B: 34 weeks [n = 18], and Group C: 36 weeks [n = 20]). Babies were subjected to brainstem evoked response audiometry (BERA) test on RMS EMG EP MARK-II machine in the neurophysiology unit of the Department of Physiology, Gandhi Medical College, Bhopal. Interpretation of the data as compared to normal values was done. Results: A trend toward decrease in absolute peak latencies of wave V with advancing gestational age was observed indicating progressive maturation, but the values recorded in all the three groups were longer as compared to the normal term values suggesting impairment in the maturation process. Wave I-V interpeak latencies values showed appreciable prolongation in all groups as compared to normal term values. Conclusion: The present study has shown that the preterm babies had altered BERA findings signifying hearing impairment.

Assessment Protocol for Candidates for Bone-Anchored Hearing Devices

Abstract Introduction The technology regarding bone-anchored hearing devices has been advancing. Nevertheless, complications are still often reported, which can impair treatment adherence and lead to discontinuation of use. There is a lack of studies conducted in tropical countries, where complications can be even greater, as well as standardized protocols for selection, indication and evaluation. Objective To characterize implanted patients from a Brazilian public institution and describe the medical and audiological assessment protocols to which they were submitted during the selection process and in the follow-up after surgery. Method An observational, cross-sectional study evaluating the medical records of patients with hearing loss and ear malformations and describing the care protocol through which they were treated. Results The medical records of 15 patients were reviewed: 6 received transcutaneous implants, and 9, percutaneous implants; 9 patients reported some type of skin lesion, 2 reported pain on the follow-up visit, and 3 had osseointegration failure. The time between surgery and activation ranged from 2 to 9 months. The median scores on the sentences, Sentences in Noise and Monosyllable tests were 100%, 60% and 80%, respectively. Conclusion It was possible to characterize the patients who received implants at the institution. The patients performed well in silence and had greater difficulty in noise. Even patients who had complications did not complain about the audibility and sound quality. It is essential to develop a model and to standardize the assessment and follow-up methods aimed at the benefit of users of bone-anchored hearing devices, as well as to enable the technico-scientific development in this field.

Orientación a la familia del niño con necesidad educativa sensorial de tipo auditiva

RESUMEN En la orientación a la familia con niño de la primera infancia con necesidad educativa sensorial de tipo auditiva es vital prestar atención a la estimulación que debe recibir el menor y que propicia el desarrollo, con lo cual se previenen posibles retrasos y discapacidades. El objetivo del presente texto es exponer los resultados de la constatación del estado actual de la orientación que recibe la familia del niño con necesidad educativa sensorial de tipo auditiva durante sus dos primeros años de vida para que el proceso de atención temprana en el hogar sea eficiente desde la etapa prediagnóstica. Los resultados son parte del proyecto de investigación Perfeccionamiento del trabajo preventivo y comunitario en los diferentes niveles educativos de la provincia de Camagüey, que se desarrolló en la Universidad de Camagüey en el período comprendido entre diciembre de 2018 y marzo de 2020. Se aplicaron métodos y técnicas como análisis de documentos, observación, encuesta, entrevista y el Test de percepción del funcionamiento familiar. Los resultados del diagnóstico permiten disponer de datos cuantitativos y cualitativos que permiten mejorar la orientación familiar desde el programa Educa a Tu Hijo con la participación del médico y la enfermera de la familia.

ABSTRACT In the orientation of the family with a child of early childhood with an auditory sensory educational need, it is vital to pay special attention to the stimulation that the child should receive and that promotes development, thereby preventing possible delays or disabilities. Hence, the objective of this text is to present the results of the verification of the current state of the orientation that the child´s family receives with an auditory sensory educational need during the first two years of life so that the early care process at home is effective from the pre-diagnosis stage. The results are part of research project: Improvement of preventive and community work in the different educational levels of the province of Camagüey which takes place at the University of Camagüey in the period between December 2018 and March 2020. In carrying out this work, methods and techniques such as documents analysis, observation, survey, interview and the Family Performance Perception Test. The results of the diagnosis allow quantitative and qualitative data to be available to improve family orientation of the Educate Your Child Program with the participation of the family doctor and nurse.

Follow-up of children diagnosed with deafness in a neonatal hearing screening program in Manaus / Seguimento de crianças com diagnóstico de surdez em programa de triagem auditiva neonatal em Manaus

ABSTRACT OBJECTIVE To evaluate the follow-up of children diagnosed with deafness in neonatal hearing screening and risk factors for hearing loss. METHODS Quantitative, cross-sectional, and retrospective study to evaluate factors associated with hearing loss and the follow-up of cases of children diagnosed with audiological dysfunction, by analyzing electronic medical records of 5,305 children referred to a Specialized Center in Type I Rehabilitation, from January/2016 to February/2020, in the city of Manaus, Amazonas. The statistical study used Pearson's chi-square test and binary logistic regression in which odds ratio scans were obtained with reliability intervals of 95%. RESULTS Of the 5,305 children referred for the otoacoustic emission retest, 366 (6.9%) failed the retest. Children diagnosed with sensorineural hearing loss continued in the study, totaling 265 (72.4%). Only 58 (21.9%) children continued in the study to its end, of these 39 had received hearing aids at that point; and 16 (41%) had surgical indication for cochlear implants, of which only 3 (18.7%) had undergone surgery. Among the risk factors for hearing loss, we found 2.6 times more chance of failure in the otoacoustic emissions retest in those children who had a family history of hearing loss and ICU stay. CONCLUSION Although the screening flow reaches a large part of live births, the dropout rates during the process are high, therefore, the socioeconomic and geographic characteristics of regions such as the Amazon should be considered as relevant factors to the evasion of rehabilitation programs of these children. Hospitalization in the neonatal ICU and family history of hearing loss in the investigations could be identified as the main and most important factors for alteration of the otoacoustic emissions retests.

RESUMO OBJETIVO Avaliar o seguimento das crianças com diagnóstico de surdez na triagem auditiva neonatal e fatores de risco para deficiência auditiva. MÉTODOS Estudo quantitativo, transversal e retrospectivo para avaliação de fatores associados à perda auditiva e o seguimento dos casos de crianças diagnosticadas com disfunção audiológica, por meio da análise de prontuários eletrônicos de 5.305 crianças encaminhadas a um Centro Especializado em Reabilitação Tipo I, no período de janeiro/2016 a fevereiro/2020, na cidade de Manaus, Amazonas. O estudo estatístico utilizou o teste qui-quadrado de Pearson e por regressão logística binária nos quais foram obtidos odds ratio com intervalos de confiabilidade de 95%. RESULTADOS Das 5.305 crianças encaminhadas para realização do reteste da orelhinha, 366 (6,9%) falharam no reteste. Prosseguiram no estudo as crianças com diagnóstico de perda auditiva neurossensorial, totalizando 265 (72,4%). Permanecendo, no final da pesquisa, apenas 58 (21,9%) crianças, destas 39 receberam aparelho auditivo até o presente estudo; e 16 (41%) já tinham indicação cirúrgica para implante coclear, sendo que apenas três (18,7%) haviam realizado a cirurgia. Dentre os fatores de risco para deficiência auditiva encontramos 2,6 vezes mais chance de falha no reteste da orelhinha naquelas crianças que tinham história familiar de perda auditiva e internação em UTI. CONCLUSÕES Embora o fluxo de triagem alcance boa parte dos nascidos vivos, as taxas de evasão durante o processo são altas, portanto, as características socioeconômicas e geográficas de regiões como a Amazônia devem ser consideradas como fatores relevantes à evasão dos programas de reabilitação dessas crianças. Foi possível identificar que a internação em UTI neonatal e o histórico familiar de perda auditiva presentes nas investigações compõem os principais e mais importantes fatores para alteração dos retestes da orelhinha.

Ensino da Língua Brasileira de Sinais durante a graduação em Medicina: a percepção dos futuros médicos / Teaching Brazilian Sign Language during medical students' training: the perception of future physicians

RESUMO Objetivo Avaliar a percepção dos estudantes de Medicina em relação à oferta da disciplina Língua Brasileira de Sinais (Libras) durante a sua formação acadêmica. Métodos Estudo transversal, descritivo e analítico, incluindo estudantes de Medicina da cidade de Salvador, Bahia, maiores de 18 anos. Aplicou-se um questionário virtual, semiestruturado, contendo aspectos sociodemográficos, acadêmicos e sobre a Libras (comunicação, aprendizado, importância na formação médica e oferta curricular). Resultados Dos 240 estudantes avaliados, 82,9% não sabe se comunicar através de Libras, entretanto 95,8% acreditam que a disciplina é necessária no currículo médico. Quanto à oferta curricular, os estudantes consideram que esta deveria ser obrigatória (55,2%) e na modalidade presencial (75,7%). Os principais motivos para não cursar a disciplina foram por esta ser optativa (41,7%) e por falta de tempo (33,3%). Identificou-se que as mulheres dão mais importância a esta formação (p=0,0013) e essa percepção independe de idade, natureza administrativa da instituição e ciclo acadêmico em curso. Conclusão Os estudantes de Medicina têm uma percepção favorável ao ensino de Libras na educação médica. Entretanto, a maioria desses estudantes não cursou a disciplina em seus percursos acadêmicos e acredita ser desafiador o atendimento a este público.

ABSTRACT Purpose To assess the perception of medical students regarding the discipline Brazilian Sign Language (Libras) during their academic training. Methods Cross-sectional, descriptive and analytical study, including medical students from the city of Salvador, Bahia, over 18 years old. A virtual, semi-structured questionnaire was applied, containing sociodemographic, academic and Libras (communication, learning, importance in medical training and curriculum offer) aspects. Results Of the 240 students evaluated, 82.9% do not know how to communicate through Libras, however 95.8% believe that the discipline is necessary in the medical curriculum. As for the curricular offer, students consider that it should be mandatory (55.2%) and in the face-to-face modality (75.7%). The main reasons for not taking the course were because it was optional (41.7%) and lack of time (33.3%). It was identified that women give more importance to this training (p=0.0013) and this perception is independent of age, administrative nature of the institution and ongoing academic cycle. Conclusion Medical students have a favorable perception of the teaching of Libras in medical education. However, most of these students did not attend the discipline in their academic careers and believe that serving this public is challenging.

Hearing Impairment in Mucopolysaccharidosis: A Systems Biology Approach

Abstract Mucopolysaccharidoses (MPS) are lysosomal diseases caused by deficiencies in lysosomal enzymes involved in the degradation of glycosaminoglycans (GAGs). Sensorineural hearing impairment is a common feature in MPS patients, but there is no consensus on its etiology. For this reason, we aimed to identify genes and pathways related to hearing loss and to correlate them with gene expression data in MPS. We used HPO and Disgenet to identify candidate genes. We constructed the network with string and Cytoscape, and hub genes were identified in Cytohubba. Expression data were obtained from the MPSBase website. We found the NDUFA gene family as the major hub genes and 114 enriched pathways related to hearing loss. These genes and biological pathways may serve as potential candidates for clinical studies to better understand hearing impairment mechanisms in lysosomal storage diseases like mucopolysaccharidosis.

Physical activity during school recess and physical education among deaf school children1 / Atividade física durante o recreio escoiar e educação física em crianças surdas

ABSTRACT: The objective of this study was to determine comparatively the physical activity in Physical Education and school recess of hearing-impaired students in Primary Education. Fifty-eigth Physical Education classes and recess in Primary Education were evaluated in a representative way, in specialized educational institutions for people with hearing disabilities. Physical activity was determined using the system for observing fitness instruction time (SOFIT). Inferential statistics, using the Student's t-test, reported significant differences with more moderate to vigorous physical activity performed during recess than in Physical Education. The same pattern was identified in schoolchildren without diagnosed disabilities. It is recommended that teachers guide the content for the development of coordinating skills that favor the gross and fine motor conditions of this population, implementing didactic strategies that increase the amount of moderate to vigorous activity in Physical Education that is greater than that performed during recess.

RESUMO: O objetivo deste estudo foi determinar comparativamente a atividade física na Educação Física e no recreio escolar de alunos com deficiência auditiva no Ensino Fundamental. Foram avaliadas 58 aulas de Educação Física e o recreio no Ensino Fundamental, de forma representativa, em instituições de ensino especializadas para pessoas com deficiência auditiva. A atividade física foi determinada usando o sistema de observação do tempo de instrução de fitness (SOFIT). A estatística inferencial, usando o teste t de Student, relatou diferenças significativas com atividades físicas mais moderadas a vigorosas realizadas durante o recreio do que na Educação Física. O mesmo padrão foi identificado em alunos sem deficiência diagnosticada. Recomenda-se aos professores orientar o conteúdo para o desenvolvimento de coordenar capacidades que favoreçam as condições motoras grossas e finas dessa população, implementando estratégias didáticas que aumentem a quantidade de atividade moderada a vigorosa na Educação Física que seja maior do que a realizada no recreio.

Treinamento de habilidades sociais educativas com mães de pré-adolescentes com deficiência auditiva / Entrenamiento de habilidades sociales educativas con madres de preadolescentes con deficiencia auditiva / Training of educational social skills in mothers of preadolescents with hearing impairment

RESUMO. Desenvolveu-se um Programa de Treinamento de Habilidades Sociais Educativas com mães de pré-adolescentes com deficiência auditiva. Duas mães constituíram o Grupo Experimental (GE) e duas, o Controle (GC). Buscou-se avaliar os efeitos do Programa no repertório de comportamentos das mães e dos filhos por meio do Roteiro de Entrevista de Habilidades Sociais Educativas Parentais (RE-HSE-P) e o do Child Behavior Checklist (CBCL). Foi utilizado o método Jacobson Truax para análise dos dados do RE-HSE-P, obtendo mudanças confiáveis em relação ao incremento das habilidades sociais educativas positivas e das habilidades sociais infantis para o GE. Os dados do CBCL indicaram problemas de comportamento internalizantes no GE e em um participante do GC e problemas de comportamento externalizantes em um participante do GC na pré e na pós-intervenção e na avaliação follow up (para o GE). Acredita-se que o desenvolvimento de Programas nestes moldes pode se constituir em um importante aliado da fonoterapia.

RESUMEN. Se desarrolló un Programa de Entrenamiento de Habilidades Sociales Educativas con madres de preadolescentes con deficiencia auditiva. Dos madres constituyeron el Grupo Experimental (GE) y dos el Grupo Control (GC). Se buscó evaluar los efectos del Programa en el repertorio de comportamientos de las madres y de los hijos por medio de la Guía de Entrevista de Habilidades Sociales Educativas Parentales (GE-HSE-P) y de la Child Behavior Checklist (CBCL). Se utilizó el método Jacobson Truax para análisis de los datos del GE-HSE-P, obteniéndose cambios confiables en relación al incremento de las habilidades sociales educativas positivas y de las habilidades sociales infantiles para el GE. Los datos del CBCL indicaron problemas de comportamiento internalizantes en el GE y en un participante del GC y problemas de comportamiento externalizantes en un participante del GC en la pre y en la postintervención y en la evaluación follow up (para el GE). Se cree que el desarrollo de estos modelos de Programas pueda constituirse en un importante aliado de la fonoterapia.

ABSTRACT. A Parental Social Skills Program was conducted with mothers of preadolescents with hearing impairment. Two mothers were included in the Experimental Group (EG) and two in the Control Group (CG). The study evaluated the effects of the Program on the repertoire of behaviors of mothers and children using the Interview Script for Parental Social Skills (IS-PSS) and the Child Behavior Checklist (CBCL). The Jacobson Truax method was applied to analyze data of the RE-HSE-P, achieving reliable changes concerning the increment of positive educational social skills and child social skills for the EG. Data of the CBCL indicated internalizing behavior disorders in the EG and in one participant of CG, and externalizing behavior disorders in one participant of the CG before and after intervention and on the follow-up evaluation (for the EG). It is believed that the development of programs like this may constitute an important aid in auditory rehabilitation.

Retomando los sonidos: Prevención de la hipoacusia y rehabilitación auditiva en las personas mayores / Bringing back sounds: prevention of hearing loss and auditory rehabilitation in the elderly / Recuperando os sons: prevenção da perda auditiva e reabilitação auditiva em pessoas idosas

RESUMEN La hipoacusia representa una de las principales causas de discapacidad a nivel mundial y su prevalencia aumenta a medida que se envejece. Representa un tipo de discapacidad que, en la mayoría de los casos, puede ser potencialmente prevenible y tratable, por lo que su prevención, identificación temprana y rehabilitación integral deberían ser algunos de los puntos de acción a nivel local ya que se ha relacionado con numerosos desenlaces adversos en salud, incluyendo los dominios físico, mental, social y económico de las personas mayores. El objetivo de este artículo es resaltar la importancia de la prevención de la hipoacusia y promover su rehabilitación a lo largo del curso de vida, especialmente en personas mayores, y describir estrategias que se han planteado desde la Organización Mundial de la Salud y la Organización Panamericana de la Salud.

ABSTRACT Hearing loss is one of the leading causes of disability worldwide and its prevalence increases with age, though in most cases, this disability is potentially preventable and treatable. Thus, its prevention, early identification, and comprehensive rehabilitation should be points of action at the local level, since hearing loss has been linked to numerous adverse health outcomes affecting the physical, mental, social, and economic conditions of older people. The objective of this article is to highlight the importance of hearing loss prevention, promote rehabilitation throughout the life course, especially in older people, and describe strategies put forth by the World Health Organization and Pan American Health Organization.

RESUMO A perda auditiva representa uma das principais causas de incapacidade em todo o mundo e sua prevalência aumenta com a idade. Representa um tipo de deficiência que, na maioria dos casos, é potencialmente prevenível e tratável e, por essa razão, sua prevenção, identificação precoce e reabilitação integral devem ser alguns dos pontos de ação no âmbito local, considerando que está relacionada a inúmeros desfechos adversos de saúde, incluindo os domínios físico, mental, social e econômico das pessoas idosas. O objetivo deste artigo é ressaltar a importância da prevenção da perda auditiva e de sua reabilitação ao longo da vida, especialmente nas pessoas idosas, e descrever estratégias propostas pela Organização Mundial da Saúde e pela Organização Pan-Americana da Saúde.

Correlation between Hearing Handicap Inventory for the Elderly Screening (HHIE-S) and Pure Tone Audiometry (PTA) Test among Malaysian Elderly

@#Introduction: The study aims to determine the correlation of the Hearing Handicap Inventory for Elderly Screening (HHIE-S) questionnaire with the pure tone audiometry (PTA) in the Malaysian older adult population. Methods: This cross-sectional study took a random sample of adults ≥60 years in a tertiary hospital. A total of 202 participants completed both the HHIE-S questionnaire and the PTA test. The total HHIE-S score and the PTA result were explored for the correlation using a Kruskal–Wallis and the validity using the receiver operating characteristic curve. Results: A Kruskal–Wallis analysis indicated a significant correlation for the level of hearing impairment (r =.704, p < 0.001) between the HHIE-S score and the PTA result. Comparison between HHIE-S score of 8 and the PTA >25db results gave sensitivity: 87.9%, specificity: 78.4%, positive predictive value: 80.3%, and negative predictive value: 86.6%. Conclusion: This study suggests that the HHIE-S questionnaire is a good and valid screening instrument for hearing impairment detection in the Malaysian older adult population.

Factores de Riesgo para la Salud Sexual de Jóvenes Sordos: Evaluación con un Cuestionario Computarizado / Risk Factors for the Sexual Health of Young Deaf: Evaluation with a Computed Questionnaire

Resumen El objetivo del estudio fue analizar un conjunto de factores de riesgo para la salud sexual de jóvenes mexicanos con discapacidad auditiva. Se utilizó un cuestionario computarizado con instrumentos validados y adaptados para la población sorda. En 112 jóvenes con edad promedio de 19.7 años se aplicaron los siguientes instrumentos: comunicación sexual con padres y con madres, apoyo y supervisión parental, conocimientos sobre infecciones de transmisión sexual y sobre el uso del preservativo, así como prácticas y abuso sexuales. En los jóvenes sexualmente activos se identificó un patrón de conducta sexual de riesgo, con proporciones importantes que han vivido abuso sexual. Se encontraron bajos niveles tanto en comunicación y supervisión parental, como en las pruebas de conocimientos. La comunicación con los padres se relacionó con el uso del preservativo y fue mayor en quienes no han vivido abuso sexual. En los varones sin experiencia sexual, las variables parentales se asociaron con ambas pruebas de conocimientos.

Abstract The objective of the study was to analyze a set of risk factors for the sexual health of young Mexicans with hearing disabilities. We used a computerized questionnaire with instruments adapted and validated to the deaf population. In 112 young people with an average age of 19.7, we applied the following instruments: sexual communication with parents, parental support and supervision, knowledge about sexually transmitted infections and condom use, sexual practices and sexual abuse. We identified a pattern of sexual risk in sexually active young people, with important proportions that have experienced sexual abuse. Low levels were found in both communication and parental supervision, as well as in knowledge tests. Communication with parents was related to the use of condoms and it was greater in those subjects who have not experienced sexual abuse. In men without sexual experience, the parental variables were associated with both knowledge tests.