Auditory Outcomes in Adolescents with Prenatal Alcohol Exposure.

The aim of the study was to investigate three aspects of auditory function (auditory acuity, cochlear dysfunction, and auditory processing) in adolescents with fetal alcohol exposure without phenotypic changes. Fifty-one adolescents with and without intrauterine exposure to alcohol were selected from a cohort study. The summons, evaluation, and analysis of the results were carried out blindly regarding the respective exposure to alcohol. The auditory tests were pure-tone audiometry, transient otoacoustic emissions, and behavioral assessment of auditory processing (speech-in-noise, dichotic digits, and gap-in-noise). After testing, 45 adolescents were included in the evaluation and were divided into exposed (n = 22) and non-exposed (n = 23) groups. Hearing loss was identified in one subject in the exposed group (4.5%). In the absence of hearing loss, there were no significant differences in tonal thresholds or in the magnitudes of the sensory (cochlear) responses between groups (p > 0.05). There was also no difference between the two groups regarding performance on the processing tests (speech-in-noise p = 0.71, dichotic p = 0.94, and gap-in-noise p = 0.33). However, the exposed group had more cases of hearing disorders (hearing loss plus auditory processing disorders) than the non-exposed group (22.7% vs. 4.3%).

Self-reported hearing difficulties and speech-in-noise test performance - what can we find behind a "normal" audiogram? / Autorrelato de dificuldades auditivas e desempenho em teste de fala com ruído - o que podemos encontrar por trás de um audiograma "normal"?

PURPOSE: To investigate complaints of difficulty understanding speech in the presence of noise in subjects without hearing loss and their performance on a speech-in-noise test. METHODS: Thirty-nine subjects aged 18 to 59 years and 11 months were divided into four groups according to their decade of life. They underwent audiometry, tympanometry, auditory processing tests, the Mini-Mental State Examination, a self-report on auditory perception combined with the Amsterdam Inventory for Auditory Disability and Handicap (Pt-AIADH), and a sentence test in silence and in noise. RESULTS: All groups scored high on the Pt-AIADH domains, with the highest average score obtained for the noise intelligibility domain. There were differences between G18 vs. G40, G18 vs. G50, and G30 vs. G50 for auditory self-perception in noise intelligibility, and differences between the youngest and all other groups on the speech-in-noise test in particular, with a lower signal-to-noise ratio for older adults. We also identified a moderate and significant correlation between intelligibility in noise and the speech-in-noise test. CONCLUSION: Normal hearers of all age groups complained of intelligibility in noise. We found that the higher an individual's auditory difficulty in this domain, the worse their performance on the speech-in-noise test; this is especially true for middle-aged adults.

OBJETIVO: Investigar a queixa de dificuldade de inteligibilidade na presença de ruído, em sujeitos sem perda auditiva e compará-la com o desempenho em um teste de fala no ruído. MÉTODO: A casuística foi constituída por 39 sujeitos de 18 a 59 anos e 11 meses foram divididos em quatro grupos em função da década de vida. Estes foram submetidos à audiometria, timpanometria, testes de processamento auditivo, mini exame do estado mental, autorrelato da percepção auditiva com o Pt-AIADH e a um teste de sentenças no silêncio e no ruído. RESULTADOS: Todos os grupos pontuaram para os domínios do Pt-AIADH, com maior pontuação média para o domínio de inteligibilidade no ruído. Houve diferenças entre o G18 vs G40, G18 vs G50, e G30 vs G50 para a autopercepção auditiva na inteligibilidade no ruído; e diferenças entre o G18 e os demais grupos no teste de fala com ruído, com menor relação sinal-ruído para os adultos mais velhos. Houve correlação moderada e significativa para a inteligibilidade no ruído e o teste de fala no ruído. CONCLUSÃO: Normo-ouvintes de todas as faixas etárias estudadas apresentaram queixa de inteligibilidade no ruído. Quanto maior a dificuldade auditiva neste domínio pior o desempenho no teste de fala com ruído, sendo mais significativo em adultos de meia idade.

Behavioral assessment of auditory processing in adulthood: population of interest and tests - a systematic review. / Avaliação comportamental do processamento auditivo na idade adulta: população de interesse e testes - uma revisão sistemática.

PURPOSE: To identify the behavioral tests used to assess auditory processing throughout adulthood, focusing on the characteristics of the target population as an interest group. RESEARCH STRATEGIES: PubMed, CINAHL, Web of Science, and Scielo, databases were searched with descriptors: "auditory perception" or "auditory perception disorders" or "auditory processing" or "central auditory processing" or "auditory processing disorders" or "central auditory processing disorders" with adults OR aging. SELECTION CRITERIA: Studies with humans included, the adult population from 18 to 64 years old, who performed at least one behavioral test to assess auditory processing in the absence of hearing loss. DATA ANALYSIS: Data extraction was performed independently, using a protocol developed by the authors that included different topics, mainly the behavioral auditory tests performed and the results found. RESULTS: Of the 867 records identified, 24 contained the information needed to answer the survey questions. CONCLUSION: Almost all studies were conducted verify performance in one or two auditory processing tests. The target target population was heterogeneous, with the most frequent persons with diabetes, stuttering, auditory processing disorder, and noise exposure. There is little information regarding benchmarks for testing in the respective age groups.

OBJETIVO: Identificar os testes comportamentais utilizados para a avaliação do processamento auditivo ao longo da vida adulta, com enfoque nas características da população alvo enquanto grupo de interesse. ESTRATÉGIA DE PESQUISA: As bases de dados consultadas foram o PubMed, CINAHL, Web of Science e Scielo, a partir dos descritores: "auditory perception" or "auditory perception disorders" or "auditory processing" or "central auditory processing" or "auditory processing disorders" or "central auditory processing disorders" com adults OR aging. CRITÉRIOS DE SELEçÃO: Incluiu-se estudos com humanos, que abordaram a população adulta de 18 a 64 anos, que realizaram pelo menos um teste comportamental para avaliação do processamento auditivo, na ausência de perda auditiva. ANÁLISE DOS DADOS: Realizou-se a extração de dados de forma independente, a partir de um protocolo desenvolvido pelos autores incluindo diferentes tópicos, principalmente os testes auditivos comportamentais realizados e os resultados encontrados. RESULTADOS: Dos 867 registros identificados, 24 foram selecionados como contendo as informações necessárias para responder às perguntas de pesquisa. CONCLUSÃO: Quase a totalidade dos estudos foi conduzida com objetivo de verificar o desempenho em um ou dois testes de processamento auditivo; a população alvo foi heterogênea, as mais frequentes foram diabetes, gagueira, transtorno do processamento auditivo e exposição ao ruído. Há poucas informações sobre os padrões de referência para os testes na respectiva faixa etária.

Does the Number of Stimuli Influence the Formation of the Endogenous Components of the Event-Related Auditory Evoked Potentials?

Introduction The number of stimuli is important to determine the quality of auditory evoked potential records. However, there is no consensus on that number in studies, especially in the sample studied. Objectives To investigate the influence of the number of rare stimuli on forming N2 and P3 components, with different types of acoustic stimuli. Methods Cross-sectional, descriptive, comparative study, approved by the ethics committee of the institution. The sample comprised 20 normal hearing adults of both sexes, aged 18 to 29 years old, with normal scores in the mental state examination and auditory processing skills. The event-related auditory evoked potentials were performed with nonverbal (1 kHz versus 2 kHz) and verbal stimuli (/BA/ versus /DA/). The number of rare stimuli varied randomly in the recordings, with 10, 20, 30, 40, and 50 presentations. Results P3 latency was significantly higher for nonverbal stimuli with 50 rare stimuli. N2 latency did not show any difference between the type and number of stimuli. The absolute P3 and N2-P3 amplitudes showed significant differences for both types of stimuli, with higher amplitude for 10 rare stimuli, in contrast with the other ones. The linear tendency test indicated significance only for the amplitude - as the number of rare stimuli increased, the amplitude tended to decrease. Conclusion The components were identifiable in the different numbers of rare stimuli and types of stimuli. The P3 and N2-P3 latency and amplitude increased with fewer verbal and nonverbal stimuli. Recording protocols must consider the number of rare stimuli.

Differential Cortical Pattern in Auditory Task Oddball Paradigm in Children Exposed to Alcohol during Pregnancy.

The negative effects of fetal alcohol exposure on child development are well documented. This study investigated the electrophysiological processing of cortical level acoustic signals in a group of 21 children prenatally exposed to alcohol. Participants aged 13-14 years at the time of the study were recruited from a longitudinal cohort sample. The study employed an observational, cross-sectional blind design and participants were divided into two groups: with and without fetal exposure to alcohol. Neurophysiological measures recorded N1, P2, N2, P3, P3a, and P3b components using the Oddball paradigm. Our results showed that the mothers' sociodemographic conditions at the time of birth, as well as the children's birth weights were homogeneous between the groups, though the Apgar score was lower in the exposed group (EG). The neurophysiological components that showed different results in the groups were P2 and P3a. P2 amplitude was higher in the midline central electrode (Cz) compared to the midline parietal electrode (Pz), demonstrating a group interaction for exposed children. For P3a there was an interaction of group and electrode position, and the EG showed higher amplitudes in Cz compared to the unEG. However, the Apgar score did not influence these results. In conclusion, children who had fetal exposure to alcohol presented electrophysiological recordings distinct from the control group. These differences occurred both in the P2 component - which reflects a bottom-up mechanism of auditory processing - as well as the P3a component, which may reflect the participation of supra-modal hearing mechanisms.

Cortical auditory evoked potentials with different acoustic stimuli: Evidence of differences and similarities in coding in auditory processing disorders.

OBJECTIVES: The use of cortical auditory evoked potentials allows for the study of the processing of acoustic signals at the cortical level, an important step in the diagnostic evaluation process, and for the monitoring of the therapeutic process associated with auditory processing disorders (APD). The differences and similarities in the acoustic coding between different types of stimuli in the context of APD remain unknown to this date. METHODS: A total of 37 children aged between 7 and 11 years, with and without APDs (identified based on verbal and non-verbal tests), all with a suitable intelligence quotient with respect to their chronological age, were assessed. Components P1 and N1 were studied using verbal and non-verbal stimuli. RESULTS: The comparison between stimuli in each group revealed that the control group had higher latency and amplitude values for speech stimuli, except for the P1 amplitude, while the group with APDs had different results with respect to the amplitudes of P1 and N1, yielding higher values for speech sounds. The differences between the groups varied according to the type of stimulus: the difference was in amplitude for the verbal stimulus and latency for the non-verbal stimulus. CONCLUSION: The records of components P1 and N1 revealed that the children with APDs performed the coding underlying the detection and identification of acoustic signals, whether verbal and non-verbal, according to a different pattern than the children in the control group.

Identification of a microdeletion at the 7q33-q35 disrupting the CNTNAP2 gene in a Brazilian stuttering case.

Speech and language disorders are some of the most common referral reasons to child development centers accounting for approximately 40% of cases. Stuttering is a disorder in which involuntary repetition, prolongation, or cessation of the sound precludes the flow of speech. About 5% of individuals in the general population have a stuttering problem, and about 80% of the affected children recover naturally. The causal factors of stuttering remain uncertain in most cases; studies suggest that genetic factors are responsible for 70% of the variance in liability for stuttering, whereas the remaining 30% is due to environmental effects supporting a complex cause of the disorder. The use of high-resolution genome wide array comparative genomic hybridization has proven to be a powerful strategy to narrow down candidate regions for complex disorders. We report on a case with a complex set of speech and language difficulties including stuttering who presented with a 10 Mb deletion of chromosome region 7q33-35 causing the deletion of several genes and the disruption of CNTNAP2 by deleting the first three exons of the gene. CNTNAP2 is known to be involved in the cause of language and speech disorders and autism spectrum disorder and is in the same pathway as FOXP2, another important language gene, which makes it a candidate gene for causal studies speech and language disorders such as stuttering.

Saethre-Chotzen syndrome, Pro136His TWIST mutation, hearing loss, and external and middle ear structural anomalies: report on a Brazilian family.

OBJECTIVE: To describe the clinical, speech, hearing, and imaging findings in three members of a Brazilian family with Saethre-Chotzen syndrome (SCS) who presented some unusual characteristics within the spectrum of the syndrome. DESIGN: Clinical evaluation was performed by a multidisciplinary team. Direct sequencing of the polymerase chain reaction-amplified coding region of the TWIST1 gene, routine and electrophysiological hearing evaluation, speech evaluation, and imaging studies through computed tomography (CT) scan and magnetic resonance imaging (MRI) were performed. RESULTS: TWIST1 gene analysis revealed a Pro136His mutation in all patients. Hearing evaluation showed peripherial and mixed hearing loss in two of the patients, one of them with severe unilateral microtia. Computed tomography scan showed structural middle ear anomalies, and MRI showed distortion of the skull contour as well as some of the brain structures. CONCLUSIONS: We report a previously undescribed TWIST1 gene mutation in patients with SCS. There is evidence that indicates hearing loss (conductive and mixed) can be related both with middle ear (microtia, high jugular bulb, and enlarged vestibules) as well as with brain stem anomalies. Here we discuss the relationship between the gene mutation and the clinical, imaging, speech, and hearing findings.

Auditory hyper-responsiveness in autism spectrum disorder, terminologies and physiological mechanisms involved: systematic review. / Hiper-responsividade auditiva no transtorno do espectro autista, terminologias e mecanismos fisiológicos envolvidos: revisão sistemática.

PURPOSE: this paper aims to identify the most used terminologies to designate the disproportional behavior to sounds in the autism spectrum disorder (ASD) and its relationship with the respective tools for its investigation, as well as its occurrence and outcomes. RESEARCH STRATEGIES: the databases used were PubMed, PsycINFO, Web of Science, Scielo and Lilacs. The keywords used were "autism", "hyperacusis" and "auditory perception", with the following combinations: "autism AND hyperacusis" and "autism AND auditory perception". SELECTION CRITERIA: individuals diagnosed with ASD of any age group; available abstract; papers in English, Spanish and Brazilian Portuguese; case series, prevalence and incidence studies, cohort and clinical trials. DATA ANALYSIS: we analyzed studies with individuals diagnosed with ASD of any age group; reference in the title and/or summary of the occurrence of disproportional behavior to sounds, accepting the terms hyper-responsiveness, hypersensitivity and hyperacusis; summary available; papers in English, Spanish and Brazilian Portuguese; series of cases, prevalence and incidence studies, cohort and clinical trials. RESULTS: Of the 692 studies resulting from the consultation, 13 studies could achieve the established requirements. CONCLUSION: The term auditory hypersensitivity was the most commonly used to designate disproportional behavior to sounds, followed by hyperacusis. There was no relationship between the terms and the respective research tool, and the questionnaires were the most used to designate the referred behavior, whose reported frequency was from 42.1% to 69.0%. The auditory behavior tests when performed showed the involvement of the auditory, afferent and efferent neural pathways.

OBJETIVO: identificar as terminologias mais utilizadas para designar o comportamento desproporcional a determinados sons (CDS) no TEA e sua relação com as respectivas ferramentas para sua investigação, assim como sua ocorrência e desfechos. ESTRATÉGIA DE PESQUISA: Foram utilizadas as bases de dados: PubMed, PsycINFO, Web of Science, Scielo e Lilacs. As palavras-chave utilizadas foram "autism", "hyperacusis" e "auditoryperception", com as seguintes combinações: "autism AND hyperacusis" e "autism AND auditoryperception". CRITÉRIOS DE SELEÇÃO: Foram incluídos os trabalhos com diagnóstico de TEA, de qualquer faixa etária; resumo disponível; Artigos em inglês, espanhol e português brasileiro; série de casos, estudos de prevalência e incidência, coorte e ensaios clínicos. ANÁLISE DOS DADOS: Foram analisados estudos com sujeitos com diagnóstico de TEA de qualquer faixa etária; referência no título e/ou resumo da ocorrência do CDS, aceitando os termos hiper-responsividade, hipersensibilidade e hiperacusia; resumo disponível; artigos em inglês, espanhol e português brasileiro; série de casos, estudos de prevalência e incidência, coorte e ensaios clínicos. RESULTADOS: Dos 692 estudos resultantes da consulta, foram identificados 13 que atendiam aos requisitos estabelecidos. CONCLUSÃO: O termo hipersensibilidade auditiva foi o mais empregado para designar o CDS, seguido da hiperacusia. Não houve relação entre os termos e a respectiva ferramenta de investigação, sendo os questionários os mais utilizados para designar o referido comportamento, cuja frequência relatada foi de 42,1% a 69,0%. Os testes auditivos, quando realizados, mostraram o envolvimento das vias neurais auditivas, aferente e eferente.

Cross-cultural adaptation of the Amsterdam inventory for auditory disability and handicap to Brazilian Portuguese.

INTRODUCTION: Patient-reported outcome measures, inventory and or questionnaire, allow patients to present their perspective of the impact of their individual condition on a day-to-day basis, independent of the analysis of test results by the expert clinician. Outcome measures are recommended when there is evidence showing their reliability, validity and sensitivity. There are standardized patient-reported outcome measures for hearing in English language; however, other languages lack these instruments. OBJECTIVE: Adapt the Amsterdam inventory for auditory disability and handicap to Brazilian Portuguese and analyze its validation measures. METHODS: We conducted two studies. In Study 1, we translated and adapted the Amsterdam inventory for auditory disability and handicap to Brazilian Portuguese according to good practice guidelines; this included the pre-test stage. In Study 2, we administered the Portuguese version to adults with and without hearing loss (n=31 and 18, respectively) and analyzed the measures of instrument validation, reliability, and reproducibility. Moreover, we calculated the correlation between pure tone thresholds and scores on the questionnaire. RESULTS: The results obtained in Study 1 demonstrated the feasibility of the translation process and the instrument's cultural adaptation, as well as its applicability, resulting in the Portuguese version of the Amsterdam inventory for auditory disability and handicap. In Study 2, the results revealed construct values for the questions and domains, as well as for the total reliable score. The intra-interviewer test-retest condition showed excellent reproducibility (ICC=0.97). Finally, there was a strong positive correlation (r=0.83) between the mean pure tone threshold and the hearing difficulties values, as measured by the instrument's scores. CONCLUSION: The English version of the Amsterdam inventory for auditory disability and handicap could be translated and adapted to Brazilian Portuguese. An analyses of the validation process yielded reliable, consistent, and stable results.

Use of masks in the oral communication of hearing device users / Uso de máscara na comunicação oral para usuários de dispositivos auxiliares à audição

ABSTRACT Purpose: to assess the influence of wearing a mask on auditory-visual speech recognition, in a favorable listening situation, in hearing devices users. Methods: a cross-sectional observational study comprising 52 hearing aid users, whose speech recognition was assessed with six video-recorded lists of sentences with and without masks. The mean test results in the various situations were compared using the Friedman test with Bonferroni post hoc, the significance level being set at 5%. Results: speech recognition assessment results differed between the situations with and without masks and between mask types, with a small effect size. The post hoc, with p-values adjusted with the Bonferroni method, showed a difference between transparent masks and others. The transparent one had a higher mean (77.8%) of auditory-visual sentence recognition between the various situations. There were statistically significant differences, as the transparent mask provided a better performance than the others. Conclusion: the auditory-visual recognition of the hearing-impaired people was better with the transparent mask.

RESUMO Objetivo: avaliar a influência do uso de máscara no reconhecimento auditivo-visual de fala, em situação de escuta favorável, em usuários de dispositivos eletrônicos auxiliares à audição (DEAA). Métodos: estudo observacional transversal. Participaram 52 usuários de dispositivos auxiliares à audição que realizaram avaliação do reconhecimento de fala com seis listas de frases apresentadas em vídeo com e sem máscaras. Para comparar as médias dos resultados do teste nas diferentes situações, foi aplicado o Teste de Friedman, com pos-hoc de Bonferroni e nível de significância 5%. Resultados: houve diferença dos resultados da avaliação de reconhecimento de fala entre as diferentes situações, com e sem máscara, assim como entre os tipos de máscara, com tamanho de efeito de pequeno. O post-hoc, com valor de p ajustado pelo método de Bonferroni, mostrou diferença entre a máscara transparente e as demais. A máscara transparente apresentou maior média (77,8%) do reconhecimento auditivo-visual nas sentenças utilizadas entre as situações. Foram observadas diferenças estatisticamente significantes com desempenho superior da máscara transparente em relação aos outros tipos de máscaras. Conclusão: conclui-se que o reconhecimento auditivo-visual para os deficientes auditivos foi melhor com a máscara transparente.

Central auditory processing and phonological processing in Brazilian Portuguese-English bilingual subjects / Processamento auditivo central e processamento fonológico em bilíngues do português brasileiro - inglês

ABSTRACT Purpose: to compare temporal auditory processing (temporal ordering) and phonological processing skills in bilingual and monolingual subjects. Methods: 100 undergraduate and postgraduate university students divided into a bilingual group (BG; n = 50) and a monolingual group (MG; n = 50). The study assessed the two groups' phonological processing skills (phonological awareness, working memory - phonological loop, and rapid lexicon access) and temporal auditory processing skills (Frequency Pattern and Duration Pattern Tests). The Mann-Whitney test (p = 0.05) was used for statistical analysis. Results: BG performed better than MG in phonological awareness and phonological working memory tasks - nonword repetition and rapid naming tasks, except for color naming, and the two auditory processing tests. Conclusion: Bilinguals performed better on phonological and auditory tests. These skills help them identify and discriminate variations in the phonology of both languages, influencing their performance.

RESUMO Objetivo: comparar as habilidades do processamento auditivo temporal (ordenação temporal) e do processamento fonológico em sujeitos bilíngues e monolíngues. Métodos: participaram 100 estudantes universitários (graduação e pós-graduação) divididos em Grupo Bilíngue (GB n=50) e Grupo Monolíngue (GM n=50). As habilidades do Processamento Fonológico (consciência fonológica, memória de trabalho - alça fonológica e acesso rápido ao léxico) e habilidades temporais do Processamento Auditivo (testes de Padrão de Frequência e Padrão de Duração) dos dois grupos, foram avaliadas. Foi utilizado, para análise estatística, o teste de Mann-Whitney (p=0,05). Resultados: observou-se melhor desempenho do GB em tarefas de consciência fonológica e memória de trabalho fonológica - repetição de não-palavras, em tarefas de nomeação rápida, exceto para nomeação de cores, e melhor desempenho nos dois testes de processamento auditivo, comparado com o desempenho do GM. Conclusão: indivíduos bilíngues apresentam melhor desempenho em testes fonológicos e auditivos. Essas habilidades favorecem a identificação e discriminação de variações na fonologia de ambos os idiomas, influenciando seu desempenho.

Autorrelato de dificuldades auditivas e desempenho em teste de fala com ruído - o que podemos encontrar por trás de um audiograma "normal"? / Self-reported hearing difficulties and speech-in-noise test performance - what can we find behind a "normal" audiogram?

RESUMO Objetivo Investigar a queixa de dificuldade de inteligibilidade na presença de ruído, em sujeitos sem perda auditiva e compará-la com o desempenho em um teste de fala no ruído. Método A casuística foi constituída por 39 sujeitos de 18 a 59 anos e 11 meses foram divididos em quatro grupos em função da década de vida. Estes foram submetidos à audiometria, timpanometria, testes de processamento auditivo, mini exame do estado mental, autorrelato da percepção auditiva com o Pt-AIADH e a um teste de sentenças no silêncio e no ruído. Resultados Todos os grupos pontuaram para os domínios do Pt-AIADH, com maior pontuação média para o domínio de inteligibilidade no ruído. Houve diferenças entre o G18 vs G40, G18 vs G50, e G30 vs G50 para a autopercepção auditiva na inteligibilidade no ruído; e diferenças entre o G18 e os demais grupos no teste de fala com ruído, com menor relação sinal-ruído para os adultos mais velhos. Houve correlação moderada e significativa para a inteligibilidade no ruído e o teste de fala no ruído. Conclusão Normo-ouvintes de todas as faixas etárias estudadas apresentaram queixa de inteligibilidade no ruído. Quanto maior a dificuldade auditiva neste domínio pior o desempenho no teste de fala com ruído, sendo mais significativo em adultos de meia idade.

ABSTRACT Purpose To investigate complaints of difficulty understanding speech in the presence of noise in subjects without hearing loss and their performance on a speech-in-noise test. Methods Thirty-nine subjects aged 18 to 59 years and 11 months were divided into four groups according to their decade of life. They underwent audiometry, tympanometry, auditory processing tests, the Mini-Mental State Examination, a self-report on auditory perception combined with the Amsterdam Inventory for Auditory Disability and Handicap (Pt-AIADH), and a sentence test in silence and in noise. Results All groups scored high on the Pt-AIADH domains, with the highest average score obtained for the noise intelligibility domain. There were differences between G18 vs. G40, G18 vs. G50, and G30 vs. G50 for auditory self-perception in noise intelligibility, and differences between the youngest and all other groups on the speech-in-noise test in particular, with a lower signal-to-noise ratio for older adults. We also identified a moderate and significant correlation between intelligibility in noise and the speech-in-noise test. Conclusion Normal hearers of all age groups complained of intelligibility in noise. We found that the higher an individual's auditory difficulty in this domain, the worse their performance on the speech-in-noise test; this is especially true for middle-aged adults.

Avaliação comportamental do processamento auditivo na idade adulta: população de interesse e testes - uma revisão sistemática / Behavioral assessment of auditory processing in adulthood: population of interest and tests - a systematic review

RESUMO Objetivo Identificar os testes comportamentais utilizados para a avaliação do processamento auditivo ao longo da vida adulta, com enfoque nas características da população alvo enquanto grupo de interesse. Estratégia de pesquisa As bases de dados consultadas foram o PubMed, CINAHL, Web of Science e Scielo, a partir dos descritores: "auditory perception" or "auditory perception disorders" or "auditory processing" or "central auditory processing" or "auditory processing disorders" or "central auditory processing disorders" com adults OR aging. Critérios de seleção Incluiu-se estudos com humanos, que abordaram a população adulta de 18 a 64 anos, que realizaram pelo menos um teste comportamental para avaliação do processamento auditivo, na ausência de perda auditiva. Análise dos dados Realizou-se a extração de dados de forma independente, a partir de um protocolo desenvolvido pelos autores incluindo diferentes tópicos, principalmente os testes auditivos comportamentais realizados e os resultados encontrados. Resultados Dos 867 registros identificados, 24 foram selecionados como contendo as informações necessárias para responder às perguntas de pesquisa. Conclusão Quase a totalidade dos estudos foi conduzida com objetivo de verificar o desempenho em um ou dois testes de processamento auditivo; a população alvo foi heterogênea, as mais frequentes foram diabetes, gagueira, transtorno do processamento auditivo e exposição ao ruído. Há poucas informações sobre os padrões de referência para os testes na respectiva faixa etária.

ABSTRACT Purpose To identify the behavioral tests used to assess auditory processing throughout adulthood, focusing on the characteristics of the target population as an interest group. Research strategies PubMed, CINAHL, Web of Science, and Scielo, databases were searched with descriptors: "auditory perception" or "auditory perception disorders" or "auditory processing" or "central auditory processing" or "auditory processing disorders" or "central auditory processing disorders" with adults OR aging. Selection criteria Studies with humans included, the adult population from 18 to 64 years old, who performed at least one behavioral test to assess auditory processing in the absence of hearing loss. Data analysis Data extraction was performed independently, using a protocol developed by the authors that included different topics, mainly the behavioral auditory tests performed and the results found. Results Of the 867 records identified, 24 contained the information needed to answer the survey questions. Conclusion Almost all studies were conducted verify performance in one or two auditory processing tests. The target target population was heterogeneous, with the most frequent persons with diabetes, stuttering, auditory processing disorder, and noise exposure. There is little information regarding benchmarks for testing in the respective age groups.

Does the Number of Stimuli Influence the Formation of the Endogenous Components of the Event-Related Auditory Evoked Potentials?

Abstract Introduction The number of stimuli is important to determine the quality of auditory evoked potential records. However, there is no consensus on that number in studies, especially in the sample studied. Objectives To investigate the influence of the number of rare stimuli on forming N2 and P3 components, with different types of acoustic stimuli. Methods Cross-sectional, descriptive, comparative study, approved by the ethics committee of the institution. The sample comprised 20 normal hearing adults of both sexes, aged 18 to 29 years old, with normal scores in the mental state examination and auditory processing skills. The event-related auditory evoked potentials were performed with nonverbal (1 kHz versus 2 kHz) and verbal stimuli (/BA/ versus /DA/). The number of rare stimuli varied randomly in the recordings, with 10, 20, 30, 40, and 50 presentations. Results P3 latency was significantly higher for nonverbal stimuli with 50 rare stimuli. N2 latency did not show any difference between the type and number of stimuli. The absolute P3 and N2-P3 amplitudes showed significant differences for both types of stimuli, with higher amplitude for 10 rare stimuli, in contrast with the other ones. The linear tendency test indicated significance only for the amplitude - as the number of rare stimuli increased, the amplitude tended to decrease. Conclusion The components were identifiable in the different numbers of rare stimuli and types of stimuli. The P3 and N2-P3 latency and amplitude increased with fewer verbal and nonverbal stimuli. Recording protocols must consider the number of rare stimuli.

Occipital atretic cephalocele, striking facial anomalies, and large feet in three siblings of a consanguineous union.

Here, we report a newly recognized syndrome in three siblings with occipital atretic cephalocele, striking facial anomalies, and large feet. Specific findings include occipital atretic cephalocele, hypoplastic cerebellar vermis, Dandy-Walker variant, mental deficiency, prominent forehead, midface deficiency, broad nose and nasal root, grooved nasal tip, abnormal nares, narrow malformed ears, severe oligodontia, and large wide feet with a gap between the hallux and the second toe. The phenotype is variable in the three patients. The finding of three affected siblings of a consanguineous couple strongly suggests autosomal recessive inheritance.

A newly recognized syndrome of Marfanoid habitus; long face; hypotelorism; long, thin nose; long, thin hands and feet; and a specific pattern of language and learning disabilities.

Here, we report on a newly recognized syndrome in a Brazilian family with three affected women, who had a Marfanoid habitus; long face; hypotelorism; long, thin nose; long, thin hands and feet; and language and learning disabilities. The disorder is compatible with autosomal dominant inheritance.

What we know of the central auditory disorders in children exposed to alcohol during pregnancy? Systematic review. / O que sabemos das alterações auditivas centrais em crianças expostas ao álcool na gestação? Revisão sistemática.

PURPOSE: To identify the effects of alcohol intake during pregnancy on the central auditory nervous system in relation to their possible diagnosis, Fetal Alcohol Syndrome, partial Fetal Alcohol Syndrome, Alcohol-Related Birth Defects and Alcohol-Related Neurodevelopmental Disorder, his extension and the hearing assessment method. RESEARCH STRATEGY: Systematic and integrative review searched the databases PubMed, LILACS and SciELO, with terms in Portuguese and English "fetal alcohol syndrome", "alcohol-related disorders" associated with "hearing". Selection criteria: We identified 123 abstracts, six were selected and published until May 2015. DATA ANALYSIS: Were listed topics to be answered, characterization of the sample; the diagnosis result of fetal exposure; method of hearing assessment and described results. RESULTS: Among the behavioral assessments, Verbal Dichotic Tests with syllables and sentences and Speech in Noise Test, were used. Among the electrophysiological tests, the Brainstem Auditory Evoked Potential was detected change neural synchrony, and Long-Latency Auditory Evoked Potential - P300, early latency values. CONCLUSION: There is evidence that children exposed to alcohol in utero present central auditory nervous system involvement signals, but it was not possible to identify the influence of different subtypes and their losses. Cortical auditory pathways were the most investigated and the electrophysiological method as used with an unexpected result in two of them, early N2 and P300 latency.

Variables in P300 recording: task type and electrode position.

PURPOSE: To analyze the latency and amplitude of P300 responses obtained with electrodes positioned at Cz and Fz and in different tasks of infrequent stimulus identification in normal hearing individuals. METHODS: Forty adults of both genders participated in the study. Three recordings with three different tasks were obtained for the identification of the infrequent stimulus; simultaneous recordings were obtained from Cz and Fz positions. RESULTS: Cz position showed significantly greater amplitudes and lower latencies than Fz. Regarding the three tasks, only "pronouncing the word 'thin'" was different from "raising a finger", with lowest latencies for the verbal task. Regarding amplitude, significantly higher values were observed for "raising a finger", followed by "pronouncing the word 'thin'" and mental counting. CONCLUSION: Cz obtained the best values, highest amplitude and lowest latency. Lowest latencies were obtained for the task of "pronouncing the word 'thin'" and the highest amplitudes were obtained for "raising a finger".

What Can We Learn about Auditory Processing from Adult Hearing Questionnaires?

BACKGROUND: Questionnaires addressing auditory disability may identify and quantify specific symptoms in adult patients with listening difficulties. PURPOSE: (1) To assess validity of the Speech, Spatial, and Qualities of Hearing Scale (SSQ), the (Modified) Amsterdam Inventory for Auditory Disability (mAIAD), and the Hyperacusis Questionnaire (HYP) in adult patients experiencing listening difficulties in the presence of a normal audiogram. (2) To examine which individual questionnaire items give the worse scores in clinical participants with an auditory processing disorder (APD). RESEARCH DESIGN: A prospective correlational analysis study. STUDY SAMPLE: Clinical participants (N = 58) referred for assessment because of listening difficulties in the presence of normal audiometric thresholds to audiology/ear, nose, and throat or audiovestibular medicine clinics. Normal control participants (N = 30). DATA COLLECTION AND ANALYSIS: The mAIAD, HYP, and the SSQ were administered to a clinical population of nonneurological adults who were referred for auditory processing (AP) assessment because of hearing complaints, in the presence of normal audiogram and cochlear function, and to a sample of age-matched normal-hearing controls, before the AP testing. Clinical participants with abnormal results in at least one ear and in at least two tests of AP (and at least one of these tests to be nonspeech) were classified as clinical APD (N = 39), and the remaining (16 of whom had a single test abnormality) as clinical non-APD (N = 19). RESULTS: The SSQ correlated strongly with the mAIAD and the HYP, and correlation was similar within the clinical group and the normal controls. All questionnaire total scores and subscores (except sound distinction of mAIAD) were significantly worse in the clinical APD versus the normal group, while questionnaire total scores and most subscores indicated greater listening difficulties for the clinical non-APD versus the normal subgroups. Overall, the clinical non-APD group tended to give better scores than the APD in all questionnaires administered. Correlation was strong for the worse-ear gaps-in-noise threshold with the SSQ, mAIAD, and HYP; strong to moderate for the speech in babble and left-ear dichotic digit test scores (at p < 0.01); and weak to moderate for the remaining AP tests except the frequency pattern test that did not correlate. The worse-scored items in all three questionnaires concerned speech-in-noise questions. This is similar to worse-scored items by hearing-impaired participants as reported in the literature. Worse-scored items of the clinical group also included quality aspects of listening questions from the SSQ, which most likely pertain to cognitive aspects of listening, such as ability to ignore other sounds and listening effort. CONCLUSIONS: Hearing questionnaires may help assess symptoms of adults with APD. The listening difficulties and needs of adults with APD to some extent overlap with those of hearing-impaired listeners, but there are significant differences. The correlation of the gaps-in-noise and duration pattern (but not frequency pattern) tests with the questionnaire scores indicates that temporal processing deficits may play an important role in clinical presentation.