Peripheral Auditory Pathway and ABR Characterization in Adults with Williams Syndrome.

Introduction Williams syndrome (WS) is a genetic disorder caused by a microdeletion in chromosome 7, affecting ∼ 28 genes. Studies have demonstrated conductive losses seemingly related to the absence of the elastin gene and mild to profound sensorineural losses due to cochlear fragility. Objective To characterize and compare the peripheral auditory system and auditory brainstem response (ABR) of adults with WS and neurotypical adults matched by age and gender. Methods We conducted a cross-sectional observational study with 30 individuals of both sexes, aged 18 to 37 years - 15 of them with WS (study group) and 15 with neither the syndrome nor hearing complaints (control group), matched for sex and age. The subjects underwent pure-tone and speech audiometry, acoustic immittance, transient-evoked otoacoustic emissions (TEOAEs), and ABR. Results Early-onset sensorineural hearing loss was found in 53.3% of the study sample, mostly mild, occurring above 3 kHz. The TEOAEs were absent in 53.3% of assessed subjects; for those in whom they were present, the signal-to-noise responses were significantly lower than in the control group. In the ABR, increased absolute latencies were observed in waves I and III. Conclusion Individuals with WS have early and progressive cochlear impairments, mainly affecting the basal region of the cochlea. They may have low brainstem changes which seem to begin in adulthood.

The Relationship between Speech Sound Disorder and Cortical Auditory Evoked Potential.

INTRODUCTION: Speech sound disorder (SSD) is a speech and language disorder associated with difficulties in motor production, perception, and phonological representation of sounds and speech segments. Since auditory perception has a fundamental role in forming and organizing sound representation for its recognition, studies that evaluate the cortical processing of sounds are required. Thus, the present study aimed to verify the relation between SSD severity measured by the percentage of correct consonants (PCCs) with the cortical auditory evoked potentials (CAEPs) using speech stimulus. METHODS: Twenty-nine children with normal hearing participated in this research and were grouped into three groups by SSD level measured by the PCC index. In addition, the groups were subdivided according to the children's age group: between 60-71 months, 72-83 months, and 83-94 months. The CAEP with speech stimulus was carried out in all children. RESULTS: Older children had longer P1 and N1 latencies. In P2 latency, there was an interference of age only in the severe group. The N2 latency was affected by age, where older children had longer latency. CONCLUSION: The amplitude of CAEP has not suffered any interference with the age, or severity of SSD. For the latency, older children generally presented longer averages than younger ones.

Study of the peripheral and central auditory pathways in patients with mucopolysaccharidosis.

OBJECTIVE: To investigate the peripheral and central auditory pathways in mucopolysaccharidosis (MPS) individuals. METHOD: The research sample comprised 15 individuals (one female and 14 males), aged 8 to 46 years. The following procedures were used: medical history survey, otoscopy, speech and pure-tone threshold audiometry, acoustic immittance measures, and central auditory pathway assessment with brainstem auditory evoked potentials (BAEP) and long-latency auditory evoked potentials (LLAEP). RESULTS: The pure-tone audiometry identified hearing loss in 13 individuals, and more than 90 % of the hearing loss was sensorineural. The degree of hearing loss was between mild to moderately severe with descendent configuration. Type A tympanogram predominated, and acoustic reflexes were present according to the types and degrees of hearing loss. Among the individuals with abnormal BAEP, longer wave III and V absolute latencies were the main findings. In addition, the unilateral absence of wave I was observed in two cases. In the LLAEP, longer latencies were observed in 14 individuals, and the most impaired components were the P1 and P3 in children and adolescents and the P2, N2 and P3 in adult individuals. CONCLUSION: The peripheral auditory pathway assessment revealed a predominantly sensorineural hearing loss, affecting mainly high frequencies, and in the central pathway was observed abnormal brainstem and cortical auditory processing in individuals with MPS.

Auditory central pathways in children and adolescents with multiple sclerosis.

BACKGROUND: Multiple sclerosis (MS) is an inflammatory demyelinating disease. Auditory evoked potential studies have demonstrated conduction and neural processing deficits in adults with MS, but little is known about the electrophysiological responses in children and adolescents. OBJECTIVE: to evaluate the central auditory pathway with brainstem auditory evoked potentials (BAEP) and long-latency auditory evoked potentials (LLAEP) in children and adolescents with MS. METHODS: The study comprised 17 individuals with MS, of both sexes, aged 9 to 18 years, and 17 healthy volunteers, matched for age and sex. All individuals had normal hearing and no middle ear impairments. They were assessed with click-BAEP and LLAEP through oddball paradigm and tone-burst stimuli. RESULTS: Abnormal responses were observed in 60% of electrophysiologic assessments of individuals with MS. In BAEP, 58.82% of MS patients had abnormal responses, with longer wave V latency and therefore longer III-V and I-V interpeak latencies than healthy volunteers. In LLAEP, 52.94% of MS patients had abnormal responses. Although statistical differences were found only in P2-N2 amplitude, MS patients had longer latencies and smaller amplitudes than healthy volunteers in all components. CONCLUSION: Children and adolescents with MS had abnormal BAEP responses, with delayed neural conduction between the cochlear nucleus and the lateral lemniscus. Also, abnormal LLAEP results suggest a decrease in neural processing speed and auditory sensory discrimination response.

ANTECEDENTES: A esclerose múltipla (EM) é uma doença inflamatória desmielinizante. Estudos com potenciais evocados auditivos têm demonstrado déficits de condução e processamento neural em adultos com EM, mas pouco se sabe sobre as respostas electrofisiológicas em crianças e adolescentes. OBJETIVO: avaliar a via auditiva central por meio dos potenciais evocados auditivos de tronco encefálico (PEATE) e dos potenciais evocados auditivos de longa latência (PEALL) em crianças e adolescentes com EM. MéTODOS: Foram avaliados17 indivíduos com EM, de ambos os sexos, com idades entre 9 e 18 anos, e 17 voluntários saudáveis, pareados por sexo e idade. Todos os indivíduos tinham audição normal sem alterações de orelha média. Os indivíduos foram avaliados por meio do PEATE com estímulo clique e do PEALL com paradigma de oddball e estímulo tone-burst. RESULTADOS: Foram observadas alteração em 60% das avaliações dos indivíduos com EM. No PEATE, 58,82% dos pacientes com EM apresentaram alteração, com aumento da latência da onda V, e interpicos III-V e I-V aumentados em comparação aos voluntários saudáveis. No PEALL, 52,94% dos pacientes com EM apresentaram alteração. Embora diferenças estatísticas foram observadas apenas na amplitude P2-N2, os pacientes com EM apresentaram latências prolongadas e amplitudes menores em comparação aos voluntários saudáveis para todos os componentes. CONCLUSãO: Crianças e adolescentes com EM apresentaram alteração das respostas do PEATE, com atraso de condução neural entre o núcleo coclear e o lemnisco lateral. Além disso, os resultados alterados do PEALL sugeriram uma diminuição na velocidade de processamento neural e de discriminação sensorial da audição.

Translation and cross-cultural adaptation of the Noise Exposure Questionnaire (NEQ) to Brazilian Portuguese. / Tradução e adaptação transcultural do Noise Exposure Questionnaire (NEQ) para o português brasileiro.

OBJECTIVE: To translate and cross-culturally adapt the Noise Exposure Questionnaire (NEQ) and 1-Minute Noise Screen (NEQ-S) instruments to Brazilian Portuguese. METHODS: Procedures widely known in health research were used in the translation and cross-cultural adaptation process, comprising the following steps: initial translation, synthesis of translations, back-translation, expert committee, pretest, and content and layout validation. Altogether, 60 workers participated in the pretest by answering the questionnaires and then evaluating them in terms of understandability, layout, clarity, and writing. Reliability was verified with Cohen's kappa test, and the internal consistency was analyzed with Cronbach's alpha coefficient. RESULTS: The translated and adapted versions of NEQ and NEQ-S were similar in terms of general and referential meanings. However, some modifications and adaptations were made to adapt them to the Brazilian reality. The kappa test indicated moderate agreement and Cronbach's alpha coefficient, substantial internal consistency. CONCLUSION: The translation and cross-cultural adaptation were carried out according to the methodology recommended in the national and international literature, performing the necessary equivalences to maintain the face and content validity with the original instrument. The availability of NEQ and NEQ-S in Brazilian Portuguese opens new fields of research to quantify yearly noise exposure more in-depth.

OBJETIVO: Traduzir e realizar a adaptação transcultural dos instrumentos Noise Exposure Questionnaire (NEQ) e 1-Minute Noise Screen (NEQ-S) para a versão em português brasileiro. MÉTODO: O processo de tradução e adaptação transcultural seguiu procedimentos amplamente utilizados em pesquisas da área da saúde, compostos pelas seguintes etapas: tradução inicial, síntese das traduções, retrotradução, comitê de especialistas, pré-teste, e validação do conteúdo e aparência. Na etapa de pré-teste, participaram 60 trabalhadores, que responderam aos questionários e, posteriormente, os avaliaram quanto ao: entendimento, aparência, clareza e escrita. Para a verificação da confiabilidade, foi utilizado o teste kappa de Cohen e para a análise da consistência interna foi aplicado o coeficiente alfa de Cronbach. RESULTADOS: As versões traduzidas e adaptadas dos questionários NEQ e NEQ-S mostraram-se semelhantes em relação ao significado geral e referencial. Entretanto, algumas modificações e adaptações foram realizadas, para adequação à realidade brasileira. O teste kappa indicou concordância moderada e o coeficiente alfa de Cronbach, consistência interna substancial. CONCLUSÃO: A tradução e a adaptação transcultural foram realizadas de acordo com a metodologia preconizada na literatura nacional e internacional, incluindo-se as equivalências necessárias para a manutenção da validade de face e conteúdo com o instrumento original. A disponibilização dos questionários NEQ e NEQ-S na versão da língua portuguesa brasileira abre novos campos de pesquisa para aprofundamento da quantificação de exposição anual ao ruído.

Learning challenges in Physical Therapy, Speech-Language-Hearing Sciences, and Occupational Therapy undergraduate programs during the COVID-19 pandemic.

PURPOSE: COVID-19 posed numerous challenges to educational programs that had to quickly adapt to remote online learning (ROL) to ensure the continuity of health professional training over the pandemic. We aimed to assess the students' and professors' perceptions of the teaching-learning process in the Physical Therapy, Speech-Language-Hearing Sciences, and Occupational Therapy undergraduate programs at a Brazilian public university. METHODS: We used an electronic self-reported questionnaire with multiple-choice questions on a Likert scale ranged 1-5; higher the score, higher the level of agreement/importance/satisfaction. RESULTS: Most of undergraduate students and teachers had previous experience using information and communication technologies, and 85% stated their preference for in-person learning. Students expressed their appreciation for more active learning methodologies with clear objectives, accessible content, and visualization of abstract concepts. Regarding benefits and barriers, some similar perceptions were observed between students and teachers with ROL favoring time management, benefits in the teaching-learning process, satisfaction and motivation with the course content, and low attendance rates to general academic activities due to absent or poor access to technological resources. CONCLUSION: ROL is an alternative learning mode when the in-person classes cannot be carried out, as in the case of the COVID-19 pandemic. ROL is believed to be unfit to replace in-person learning, although it can complement the traditional classroom-based education in a hybrid model, respecting the nature of each program in the field of health that requires in-person practical training.

Evaluation of Subtle Auditory Impairments with Multiple Audiological Assessments in Normal Hearing Workers Exposed to Occupational Noise.

Recent studies involving guinea pigs have shown that noise can damage the synapses between the inner hair cells and spiral ganglion neurons, even with normal hearing thresholds-which makes it important to investigate this kind of impairment in humans. The aim was to investigate, with multiple audiological assessments, the auditory function of normal hearing workers exposed to occupational noise. Altogether, 60 workers were assessed (30 in the noise-exposure group [NEG], who were exposed to occupational noise, and 30 in the control group [CG], who were not exposed to occupational noise); the workers were matched according to age. The following procedures were used: complete audiological assessment; speech recognition threshold in noise (SRTN); speech in noise (SN) in an acoustic field; gaps-in-noise (GIN); transient evoked otoacoustic emissions (TEOAE) and inhibitory effect of the efferent auditory pathway; auditory brainstem response (ABR); and long-latency auditory evoked potentials (LLAEP). No significant difference was found between the groups in SRTN. In SN, the NEG performed worse than the CG in signal-to-noise ratio (SNR) 0 (p-value 0.023). In GIN, the NEG had a significantly lower percentage of correct answers (p-value 0.042). In TEOAE, the NEG had smaller amplitude values bilaterally (RE p-value 0.048; LE p-value 0.045) and a smaller inhibitory effect of the efferent pathway (p-value 0.009). In ABR, the NEG had greater latencies of wave V (p-value 0.017) and interpeak intervals III-V and I-V in the LE (respective p-values: 0.005 and 0.04). In LLAEP, the NEG had a smaller P3 amplitude bilaterally (RE p-value 0.001; LE p-value 0.002). The NEG performed worse than the CG in most of the assessments, suggesting that the auditory function in individuals exposed to occupational noise is impaired, even with normal audiometric thresholds.

A longitudinal study of the peripheral and central auditory pathways in individuals with acute lymphoid leukemia.

OBJECTIVE: To characterize the peripheral and central auditory pathways in individuals with Acute Lymphoid Leukemia (ALL) and compare assessment results before and during chemotherapy. METHOD: The study included 17 subjects with ALL, divided into two age groups: 3 to 6 (11 individuals) and 7 to 16 years old (6 individuals). Each subject was evaluated twice (before and 3 to 6 months after chemotherapy treatment) with the following procedures: medical history survey, otoscopy, Pure-Tone Threshold (PTA) and speech audiometry, acoustic immittance measures, Brainstem Auditory Evoked Potentials (BAEP) and Long-Latency Auditory Evoked Potentials (LLAEP). RESULTS: PTA was normal. Tympanometry was abnormal in the second assessment in 2 individuals aged 3 to 6 years. One subject in each age group had absent ipsilateral acoustic reflexes. In high-frequency audiometry, 1 individual had abnormal results. BAEP was abnormal in 5 (first assessment) and 7 individuals (second assessment) aged 3 to 6 years and 2 (first assessment) and 1 individual (second assessment) aged 7 to 16 years. As for LLAEP, P1 latency was increased in 5 (first assessment) and 7 individuals (second assessment) aged 3 to 6 years. CONCLUSION: No hearing loss was identified in the behavioral audiological assessment. BAEP was more affected in the 3-to-6-year-old group, with greater impairment in the lower brainstem in the first and second assessments. In LLAEP, P1 was the most impaired component in children aged 3 to 6 years, and P2 and N2 were so for those 7 to 16 years old, especially in the second assessment.

Dangerous Decibels Program in Elementary and Middle School Students: Effectiveness After a 6-Month Follow-Up.

PURPOSE: The purpose of this article was to assess the effectiveness of the Dangerous Decibels (DD) program in students in the short- and medium-term follow-ups (up to 6 months), including an online game. METHOD: A randomized trial was conducted between two interventions (DD and placebo). The research included 58 participants who were divided into two groups: the study group (SG) and the control group. The following phases were developed: intervention (DD or placebo), post-3-month assessment and availability of the online game, and post-6-month assessment. A questionnaire was administered to assess their performance. Overall total scores and category scores were obtained. RESULTS: Improved overall scores were found in the SG in the immediate post-intervention (p = .004), post-3-month (p = .022), and post-6-month (p = .002) questionnaires, as well as the knowledge and behavior categories. CONCLUSIONS: The DD program effectively improved the knowledge and behavior of 10- to 12-year-old children regarding noise in the short- and medium-term follow-ups. However, no significant changes were achieved in terms of barriers only by using the program and the online game. Adding a second intervention (the online game) to the program seems to be a good option to maintain the changes achieved with the interactive class.

Potenciais evocados auditivos de tronco encefálico em indivíduos com doença de Parkinson: revisão de literatura / Brainstem auditory evoked potentials in individuals with Parkinson's disease: literature review

Objetivo: investigar a integridade da via auditiva por meio do Potencial Evocado Auditivo de Tronco Encefálico (PEATE) em indivíduos com Doença de Parkinson (DP) por meio de uma revisão integrativa da literatura. Métodos: foram utilizadas as bases de dados Embase, Google acadêmico, Scielo, bem como o portal eletrônico completo da Biblioteca Virtual da Saúde, com os seguintes descritores: DP (Parkinson Disease ou Parkinsonian Disorders) e PEATE (Evoked Potentials, Auditory, Brain Stem ou Evoked Potentials, Auditory). Foram selecionados artigos nacionais e internacionais, sem limitação de ano ou idioma. Os estudos foram descritos e analisados qualitativamente e quantitativamente quanto à latência absoluta e à amplitude das ondas I, III e V, e quanto aos interpicos I-III, III-V e I-V. Resultados: após exclusão dos títulos repetidos, 420 artigos foram encontrados, sendo que 17 atenderam aos critérios de inclusão. Nos resultados do PEATE, estudos descreveram o aumento da latência de todas as ondas, principalmente das ondas III e V e, também, aumento dos interpicos I-V e III-V. Conclusão: indivíduos com DP apresentam neurodegeneração das vias auditivas centrais com diminuição na velocidade de transmissão neural do estímulo acústico.

Purpose: investigate the integrity of the auditory pathway through the Brainstem Auditory Evoked Potential (BAEP) in individuals with Parkinson's Disease (PD) through an integrative literature review. Methods: Embase, Academic Google, Scielo databases were used, as well as the complete electronic portal of the Virtual Health Library, with the following descriptors: PD (Parkinson Disease or Parkinsonian Disorders) and BAEP (Evoked Potentials, Auditory, Brain Stem or Evoked Potentials, Auditory). National and international articles were selected, without limitation of year or language. The studies were described and analyzed qualitatively and quantitatively regarding the absolute latency and amplitude of waves I, III and V, and regarding the interpeaks I-III, III-V and I-V. Results: After excluding repeated titles, 420 articles were found, however only 17 met the inclusion criteria. In the ABR results, studies described the increase in latency of all waves, especially waves III and V, and also an increase in interpeaks I-V and III-V. Conclusion: individuals with PD present neurodegeneration of the central auditory pathways with a decrease in the neural transmission speed of the acoustic stimulus.

Tradução e adaptação transcultural do Noise Exposure Questionnaire (NEQ) para o português brasileiro / Translation and cross-cultural adaptation of the Noise Exposure Questionnaire (NEQ) to Brazilian Portuguese

RESUMO Objetivo Traduzir e realizar a adaptação transcultural dos instrumentos Noise Exposure Questionnaire (NEQ) e 1-Minute Noise Screen (NEQ-S) para a versão em português brasileiro. Método O processo de tradução e adaptação transcultural seguiu procedimentos amplamente utilizados em pesquisas da área da saúde, compostos pelas seguintes etapas: tradução inicial, síntese das traduções, retrotradução, comitê de especialistas, pré-teste, e validação do conteúdo e aparência. Na etapa de pré-teste, participaram 60 trabalhadores, que responderam aos questionários e, posteriormente, os avaliaram quanto ao: entendimento, aparência, clareza e escrita. Para a verificação da confiabilidade, foi utilizado o teste kappa de Cohen e para a análise da consistência interna foi aplicado o coeficiente alfa de Cronbach. Resultados As versões traduzidas e adaptadas dos questionários NEQ e NEQ-S mostraram-se semelhantes em relação ao significado geral e referencial. Entretanto, algumas modificações e adaptações foram realizadas, para adequação à realidade brasileira. O teste kappa indicou concordância moderada e o coeficiente alfa de Cronbach, consistência interna substancial. Conclusão A tradução e a adaptação transcultural foram realizadas de acordo com a metodologia preconizada na literatura nacional e internacional, incluindo-se as equivalências necessárias para a manutenção da validade de face e conteúdo com o instrumento original. A disponibilização dos questionários NEQ e NEQ-S na versão da língua portuguesa brasileira abre novos campos de pesquisa para aprofundamento da quantificação de exposição anual ao ruído.

ABSTRACT Objective To translate and cross-culturally adapt the Noise Exposure Questionnaire (NEQ) and 1-Minute Noise Screen (NEQ-S) instruments to Brazilian Portuguese. Methods Procedures widely known in health research were used in the translation and cross-cultural adaptation process, comprising the following steps: initial translation, synthesis of translations, back-translation, expert committee, pretest, and content and layout validation. Altogether, 60 workers participated in the pretest by answering the questionnaires and then evaluating them in terms of understandability, layout, clarity, and writing. Reliability was verified with Cohen's kappa test, and the internal consistency was analyzed with Cronbach's alpha coefficient. Results The translated and adapted versions of NEQ and NEQ-S were similar in terms of general and referential meanings. However, some modifications and adaptations were made to adapt them to the Brazilian reality. The kappa test indicated moderate agreement and Cronbach's alpha coefficient, substantial internal consistency. Conclusion The translation and cross-cultural adaptation were carried out according to the methodology recommended in the national and international literature, performing the necessary equivalences to maintain the face and content validity with the original instrument. The availability of NEQ and NEQ-S in Brazilian Portuguese opens new fields of research to quantify yearly noise exposure more in-depth.

Learning challenges in Physical Therapy, Speech-Language-Hearing Sciences, and Occupational Therapy undergraduate programs during the COVID-19 pandemic / Desafios de aprendizagem nos cursos de graduação em Fisioterapia, Fonoaudiologia e Terapia Ocupacional durante a pandemia de COVID-19

ABSTRACT Purpose COVID-19 posed numerous challenges to educational programs that had to quickly adapt to remote online learning (ROL) to ensure the continuity of health professional training over the pandemic. We aimed to assess the students' and professors' perceptions of the teaching-learning process in the Physical Therapy, Speech-Language-Hearing Sciences, and Occupational Therapy undergraduate programs at a Brazilian public university. Methods We used an electronic self-reported questionnaire with multiple-choice questions on a Likert scale ranged 1-5; higher the score, higher the level of agreement/importance/satisfaction. Results Most of undergraduate students and teachers had previous experience using information and communication technologies, and 85% stated their preference for in-person learning. Students expressed their appreciation for more active learning methodologies with clear objectives, accessible content, and visualization of abstract concepts. Regarding benefits and barriers, some similar perceptions were observed between students and teachers with ROL favoring time management, benefits in the teaching-learning process, satisfaction and motivation with the course content, and low attendance rates to general academic activities due to absent or poor access to technological resources. Conclusion ROL is an alternative learning mode when the in-person classes cannot be carried out, as in the case of the COVID-19 pandemic. ROL is believed to be unfit to replace in-person learning, although it can complement the traditional classroom-based education in a hybrid model, respecting the nature of each program in the field of health that requires in-person practical training.

RESUMO Objetivo O COVID-19 impôs inúmeros desafios aos programas educacionais que tiveram que se adaptar rapidamente ao aprendizado remoto on-line (ARO) para garantir a continuidade da formação dos profissionais de saúde durante a pandemia. O objetivo do estudo foi avaliar a percepção de alunos e professores sobre o processo ensino-aprendizagem dos cursos de graduação em Fisioterapia, Fonoaudiologia e Terapia Ocupacional de uma universidade pública brasileira. Métodos Foi utilizado um questionário eletrônico autoaplicável com questões de múltipla escolha em escala Likert de 1 a 5; quanto maior a pontuação, maior o nível de concordância/importância/satisfação. Resultados A maioria dos alunos de graduação e professores tinha experiência anterior no uso de tecnologias de informação e comunicação, e 85% afirmaram preferir o ensino presencial. Os alunos expressaram preferência por metodologias de aprendizagem mais ativas, com objetivos claros, conteúdo acessível e visualização de conceitos abstratos. Em relação aos benefícios e barreiras, algumas percepções semelhantes foram observadas entre alunos e professores com ARO favorecendo a gestão do tempo, benefícios no processo ensino-aprendizagem, satisfação e motivação com o conteúdo do curso e baixa frequência às atividades acadêmicas gerais por ausência ou dificuldade de acesso aos recursos tecnológicos. Conclusão O ARO pode ser uma modalidade alternativa de aprendizado quando as aulas presenciais não podem ser realizadas, como no caso da pandemia do COVID-19. Porém, o ARO é inadequado para substituir a aprendizagem presencial, embora possa complementar a educação presencial tradicional em um modelo híbrido, respeitando a natureza de cada programa na área da saúde que exige formação prática presencial.

A longitudinal study of the peripheral and central auditory pathways in individuals with acute lymphoid leukemia

Abstract Objective To characterize the peripheral and central auditory pathways in individuals with Acute Lymphoid Leukemia (ALL) and compare assessment results before and during chemotherapy. Method The study included 17 subjects with ALL, divided into two age groups: 3 to 6 (11 individuals) and 7 to 16 years old (6 individuals). Each subject was evaluated twice (before and 3 to 6 months after chemotherapy treatment) with the following procedures: medical history survey, otoscopy, Pure-Tone Threshold (PTA) and speech audiometry, acoustic immittance measures, Brainstem Auditory Evoked Potentials (BAEP) and Long-Latency Auditory Evoked Potentials (LLAEP). Results PTA was normal. Tympanometry was abnormal in the second assessment in 2 individuals aged 3 to 6 years. One subject in each age group had absent ipsilateral acoustic reflexes. In high-frequency audiometry, 1 individual had abnormal results. BAEP was abnormal in 5 (first assessment) and 7 individuals (second assessment) aged 3 to 6 years and 2 (first assessment) and 1 individual (second assessment) aged 7 to 16 years. As for LLAEP, P1 latency was increased in 5 (first assessment) and 7 individuals (second assessment) aged 3 to 6 years. Conclusion No hearing loss was identified in the behavioral audiological assessment. BAEP was more affected in the 3-to-6-year-old group, with greater impairment in the lower brainstem in the first and second assessments. In LLAEP, P1 was the most impaired component in children aged 3 to 6 years, and P2 and N2 were so for those 7 to 16 years old, especially in the second assessment.

Auditory central pathways in children and adolescents with multiple sclerosis / Sistema auditivo central em crianças e adolescentes com esclerose múltipla

Abstract Background Multiple sclerosis (MS) is an inflammatory demyelinating disease. Auditory evoked potential studies have demonstrated conduction and neural processing deficits in adults with MS, but little is known about the electrophysiological responses in children and adolescents. Objective to evaluate the central auditory pathway with brainstem auditory evoked potentials (BAEP) and long-latency auditory evoked potentials (LLAEP) in children and adolescents with MS. Methods The study comprised 17 individuals with MS, of both sexes, aged 9 to 18 years, and 17 healthy volunteers, matched for age and sex. All individuals had normal hearing and no middle ear impairments. They were assessed with click-BAEP and LLAEP through oddball paradigm and tone-burst stimuli. Results Abnormal responses were observed in 60% of electrophysiologic assessments of individuals with MS. In BAEP, 58.82% of MS patients had abnormal responses, with longer wave V latency and therefore longer III-V and I-V interpeak latencies than healthy volunteers. In LLAEP, 52.94% of MS patients had abnormal responses. Although statistical differences were found only in P2-N2 amplitude, MS patients had longer latencies and smaller amplitudes than healthy volunteers in all components. Conclusion Children and adolescents with MS had abnormal BAEP responses, with delayed neural conduction between the cochlear nucleus and the lateral lemniscus. Also, abnormal LLAEP results suggest a decrease in neural processing speed and auditory sensory discrimination response.

Resumo Antecedentes A esclerose múltipla (EM) é uma doença inflamatória desmielinizante. Estudos com potenciais evocados auditivos têm demonstrado déficits de condução e processamento neural em adultos com EM, mas pouco se sabe sobre as respostas electrofisiológicas em crianças e adolescentes. Objetivo avaliar a via auditiva central por meio dos potenciais evocados auditivos de tronco encefálico (PEATE) e dos potenciais evocados auditivos de longa latência (PEALL) em crianças e adolescentes com EM. Métodos Foram avaliados17 indivíduos com EM, de ambos os sexos, com idades entre 9 e 18 anos, e 17 voluntários saudáveis, pareados por sexo e idade. Todos os indivíduos tinham audição normal sem alterações de orelha média. Os indivíduos foram avaliados por meio do PEATE com estímulo clique e do PEALL com paradigma de oddball e estímulo tone-burst. Resultados Foram observadas alteração em 60% das avaliações dos indivíduos com EM. No PEATE, 58,82% dos pacientes com EM apresentaram alteração, com aumento da latência da onda V, e interpicos III-V e I-V aumentados em comparação aos voluntários saudáveis. No PEALL, 52,94% dos pacientes com EM apresentaram alteração. Embora diferenças estatísticas foram observadas apenas na amplitude P2-N2, os pacientes com EM apresentaram latências prolongadas e amplitudes menores em comparação aos voluntários saudáveis para todos os componentes. Conclusão Crianças e adolescentes com EM apresentaram alteração das respostas do PEATE, com atraso de condução neural entre o núcleo coclear e o lemnisco lateral. Além disso, os resultados alterados do PEALL sugeriram uma diminuição na velocidade de processamento neural e de discriminação sensorial da audição.

Central auditory processing in children after traumatic brain injury.

INTRODUCTION: Traumatic brain injury can impair the central auditory pathways and auditory cortex. Hence, individuals who suffered a traumatic brain injury may be at risk of central auditory processing disorders, which can be identified with behavioral tests that assess central auditory function. OBJECTIVE: To characterize and compare the performance of children and adolescents with and without a history of traumatic brain injury in behavioral tests that assess central auditory processing. METHOD: The sample comprised 8- to 18-year-old individuals of both sexes who suffered moderate or severe closed traumatic brain injury 3 to 24 months before their participation in the study and whose hearing thresholds were normal. These individuals were matched for sex and age with other subjects without a history of traumatic brain injury and submitted to behavioral assessment of the central auditory processing with special tests to assess hearing skills (namely, auditory closure, figure-ground, and temporal processing), selected according to their chronological age and response-ability. RESULTS: The study group performed statistically worse than the comparison group in auditory closure, figure-ground in verbal dichotic listening, and temporal ordering. The central auditory processing tests with abnormal results in the comparison group were different from those in the study group. CONCLUSION: Central auditory processing disorders were identified in all subjects of the study group, especially involving auditory closure and temporal processing skills, in comparison with subjects without a history of traumatic brain injury.

Effects of Noise and Chemical Exposure on Peripheral and Central Auditory Pathways in Normal-hearing Workers.

Objectives: To assess the effects of noise and chemical exposure on peripheral and central auditory pathways in normal-hearing workers exposed to chemicals or high noise levels and compare the groups with each other and with workers not exposed to either of these agents. Methods: A total of 54 normal-hearing workers were divided into three groups (chemical, noise, control) and submitted to the following assessments: conventional and extended high-frequency pure-tone audiometry; transient and distortion-product otoacoustic emissions, the inhibitory effect of the efferent auditory pathway; and Staggered Spondaic Word (SSW) and Pitch Pattern Sequence (PPS) test. Results: There were no significant differences between the groups in extended high-frequency hearing thresholds. Significantly lower amplitudes were observed in the noise group for otoacoustic emissions. There were significantly more absences of the inhibitory effect of the efferent system in the noise group. There was no difference between the groups in the SSW test, while in PPS, the noise group performed worse than the control group. Conclusion: These findings suggest that noise exposure produced deleterious effects on the workers' peripheral and central auditory systems, despite their normal hearing thresholds. The chemical group did not have significantly different results from those of the control group. It is important that individuals exposed to noise or chemicals have their auditory pathways monitored with complementary assessments.

Impact of ERT and follow-up of 17 patients from the same family with a mild form of MPS II.

BACKGROUND: Mucopolysaccharidosis type II, also known as Hunter syndrome, is a rare X-linked recessive disorder caused by deficiency of the lysosomal enzyme Iduronate-2- Sulfatase (IDS), leading to progressive accumulation of Glycosaminoglycans (GAGs) in several organs. Over the years, Enzyme Replacement Therapy (ERT) has provided significant benefits for patients, retarding the natural progression of the disease. RESULTS: The authors evaluated 17 patients from the same family with a mild form of MPS type II; the proband had developed acute decompensated heart failure refractory to clinical measurements at 23 years and needed a rather urgent heart transplant; however, he died from surgical complications shortly after the procedure. Nevertheless, subsequent to his tragic death, 16 affected male relatives were detected after biochemical tests identifying the low or absent activity of the IDS enzyme and confirmed by molecular analysis of the IDS gene. Following diagnosis, different options of treatment were chosen: 6 patients started ERT with Elaprase® (Idursulfase) soon after, while the other 10 remained without ERT. Eventually, 4 patients in the latter group began ERT with Hunterase® (Idursulfase Beta). None presented adverse effects to either form of the enzyme. Among the 6 individuals without any ERT, two died of natural causes, after reaching 70 years. Despite the variable phenotype within the same family (mainly heart dysfunctions and carpal tunnel syndrome), all 14 remaining patients were alive with an independent lifestyle. CONCLUSION: Here, the authors report the variable progress of the disease with and without ERT in a large Brazilian family with a slowly progressive form of MPS II, harboring the same missense variant in the IDS gene.

Audiological profile and cochlear functionality in Williams syndrome. / O perfil audiológico e a funcionalidade coclear na síndrome de Williams.

PURPOSE: to evaluate cochlear functionality in Williams syndrome (WS) individuals. METHODS: a study with 39 individuals, being 22 with WS aged between 7 and 17 years, 15 male and 7 female, and 17 individuals with typical development and normal hearing. All individuals were evaluated using pure tone audiometry, acoustic immittance measurements, and Transient Evoked Otoacoustic Emissions (TEOAE). The audiological profile in individuals with WS was analyzed, and TEOAE responses were compared between WS individuals without hearing loss and typical developmental individuals. RESULTS: The hearing loss was observed in 50% of patients, being 78.95% sensorineural and 21.05% mixed. This hearing loss was predominantly mild to moderate, affecting mainly frequencies above 3 kHz. As for TEOAE, there was a higher incidence of absence and lower amplitude responses in individuals with WS. CONCLUSION: WS individuals have hair cell dysfunction, mainly in the basal region of the cochlea. Thus, TEOAE analysis is an important clinical resource to be considered in the routine audiological evaluation.

OBJETIVO: avaliar o perfil audiológico e a funcionalidade coclear em indivíduos com SW. MÉTODO: estudo com 39 indivíduos, sendo 22 indivíduos com SW com idade entre 7 e 17 anos, sendo 15 do sexo masculino e 7 do sexo feminino e 17 indivíduos com desenvolvimento típico e normo-ouvintes. Todos os indivíduos foram avaliados por meio da audiometria tonal limiar, medidas de imitância acústica e análise das Emissões Otoacústicas Transientes (EOAT). Foi avaliado o perfil audiológico dos indivíduos com SW, e também foram comparadas as respostas das EOAT entre os indivíduos com SW sem perda auditiva e indivíduos controles. RESULTADOS: perda auditiva foi observada em 50% dos pacientes, sendo 78,95% neurossensorial e 21,05% mista. Esta perda foi predominantemente de grau leve a moderado, acometendo principalmente as frequências a partir de 3 kHz. Quanto às EOAT, observou-se maior incidência de ausência e de respostas de menor amplitude em indivíduos com SW. CONCLUSÃO: indivíduos com SW apresentam disfunção das células ciliadas, principalmente da região basal da cóclea. Assim, a análise das EOAT é um recurso clínico importante a ser considerada na avaliação audiológica de rotina.

Auditory evoked potentials in children and adolescents with multiple sclerosis and neuromyelitis optica spectrum disorders.

BACKGROUND: In children, an acute demyelinating disease may evolve as a multiphasic disease with multiple relapses, such as multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). The Brainstem Auditory Evoked Potentials (BAEP) and Long-Latency Auditory Evoked Potentials (LLAEP) contribute to the identification of either retrocochlear changes or other central auditory nervous system (CANS) changes. OBJECTIVES: To characterize BAEP and LLAEP in children and adolescents with MS and NMOSD and verify the diagnostic values of these potentials in each of the demyelinating diseases. METHODS: The 40 participants were divided into two study groups (SG1 - MS, SG2 - NMOSD) and two comparison groups (CG1 and CG2), matched for age (9 years-17 years and 11 months) and sex. Electrophysiological hearing assessment was performed with BAEP and LLAEP. RESULTS: When SG1 and SG2 were compared with CG1 and CG2 regarding BAEP and LLAEP, both SG1 and SG2 presented a higher occurrence of changes. Also, individuals with MS had higher occurrences of BAEP changes, whereas individuals with NMOSD had a higher occurrence of LLAEP changes. CONCLUSIONS: BAEP and LLAEP in children and adolescents with MS or NMOSD showed higher latencies and lower amplitudes of some components when these individuals were compared with their peers. These procedures were highly accurate to identify demyelinating diseases. BAEP results were more abnormal in individuals with MS, while LLAEP was so with NMOSD. These findings indicate that the auditory evoked potentials are important instruments for the differential diagnosis of MS and NMOSD, and valuable to monitor disease evolution.