The reliability and validity of decreased sound tolerance scale-screening

Abstract Objectives: Decreased Sound Tolerance (DST) is a negative reaction to a sound that does not cause any reaction in an individual with normal hearing. DST's subclasses include hyperacusis, phonophobia, and misophonia, which are distinct and have therapy variations. There is no diagnostic method or scale that distinguishes them in the literature. This study's purpose was to develop a screening scale that distinguishes these three DSTs. Methods: The study comprised 257 willing participants with normal hearing. Cronbach alpha coefficient, item-total correlation, and item differentiation of the Decreased Sound Tolerance Scale-Screening (DSTS-S) were evaluated. Structural validity of DSTS-S was performed by Varimax rotation with Kaiser normalization using Explanatory Factor Analysis (EFA), and Confirmatory Factor Analysis (CFA) was performed to assess its structural compatibility. The scale's total scores from each section were compared using the Mann-Whitney U test in symptom (+) and symptom (-) participants. Results: The Cronbach alpha value for hyperacusis, phonophobia, and misophonia sections of DSTS-S was calculated as 0.881, 0.775, and 0.938, respectively. The difference between the independent samplet-test and the variables was statistically significant (p<0.01). The Mann-Whitney U test showed a significant difference between the median values of the total groups 'scores with and without hyperacusis, phonophobia, and misophonia (HTS, PTS, and MTS, respectively) (p<0.05). Evaluation by ROC analysis showed that hyperacusis was useful in predicting the presence of hyperacusis, phonophobia was useful in predicting the presence of phonophobia, and misophonia was useful in predicting the presence of misophonia (p< 0.001). Hyperacusis and misophonia sections showed high reliability, and phonophobia section showed a moderately reliable level. The Kappa test showed that the compatibility between test-retest for the total scores was statistically significant (p < 0.001). Conclusion: The study's results indicated that DSTS-S is a valid and reliable scale for identifying subtypes/problems/classes of decreased sound tolerance.

Hiperacusia en trastornos del espectro autista: una revisión de la literatura / Hyperacusis in autism spectrum disorders: a review of the literature

Resumen La hiperacusia se define como la intolerancia a ciertos sonidos cotidianos que causa angustia y discapacidad significativas en las actividades sociales, ocupacionales, recreativas y otras actividades cotidianas. Los sonidos pueden percibirse como incómodamente fuertes, desagradables, atemorizantes o dolorosos. Se encuentra presente en aproximadamente un 3% población general, y aumenta significativamente en trastornos del espectro autista (TEA), alcanzando entre un 15% a 40%. Los mecanismos fisiopatológicos no son del todo claros, pero se ha propuesto, una alteración en el funcionamiento de mecanismos reflejos y de regulación, tanto a nivel de la vía auditiva periférica, como central, incluyendo estructuras no relacionadas directamente con la vía auditiva. El siguiente texto tiene como objetivo analizar la relación entre hiperacusia y TEA, enfatizando en la frecuencia en que se presentan como comorbilidades, en los posibles mecanismos fisiopatológicos, y en actualizaciones en el abordaje diagnóstico y terapéutico. Se realiza una revisión bibliográfica cualitativa en Pubmed con artículos entre los años 2008-2020 utilizando los términos: "hyperacusis autism", "sistema olivococlear", arrojando 39 artículos, de los cuales se seleccionaron en base a la temática de cada uno, evaluada por los autores. A pesar de una significativa relación entre hiperacusia y TEA, los mecanismos fisiopatológicos de ambas patologías siguen siendo un misterio. Existen estudios que sugieren pruebas de screening no invasivas que relacionan ambas patologías, pero debido a los sesgos de selección, todavía no son factibles de usar en forma universal. El abordaje terapéutico ha sido poco explorado, y no se dispone de fármacos que hayan demostrado su efectividad, por el contrario, algunos de ellos empeoran la sintomatología. Se recomienda al tratante, seguir un camino largo, en conjunto con el paciente, donde las terapias no farmacológicas como la terapia cognitivo conductual han mostrado tener buenos resultados.

Abstract Hyperacusis is defined as intolerance to certain sounds that causes significant distress and disability in social, occupational, recreational and other activities. Sounds can be perceived as uncomfortably loud, unpleasant, frightening, or painful. It is present in approximately 3% of the general population, and increases significantly in autism spectrum disorders (ASD), between 15% and 40%. The pathophysiological mechanisms are not entirely clear, but an alteration in the functioning of reflex and regulatory mechanisms has been proposed, both at the peripheral and central auditory pathways, including structures not directly related to the auditory pathway. The therapeutic approach has been little explored as there are no drugs that have demonstrated their effectiveness, on the contrary, some of them worsen the symptoms. The practitioner is recommended to follow a long path, in conjunction with the patient, where non-pharmacological therapies such as cognitive behavioral therapy have been shown to have good results. The following text shows a review of the literature with articles referring to the subject between the years 2008-2019.

Relación entre hiperacusia y desorden del procesamiento auditivo central: Una revisión de la literatura / Relationship between hyperacusis and auditory processing disorder: a literature review

Resumen La hiperacusia corresponde a la excesiva sensibilidad auditiva o intolerancia a ciertos sonidos cotidianos que para la mayoría de las personas parecerían habituales. Considerando los mecanismos fisiológicos involucrados en el origen de la hiperacusia, es lógico pensar que su presencia podría afectar algunas habilidades del procesamiento auditivo central, sin embargo, la evidencia en torno al tema es escasa y no existe actualmente una revisión de la literatura que agrupe las investigaciones sobre esta temática. Por ello, el presente estudio pretende identificar y analizar la evidencia científica disponible sobre la relación entre hiperacusia y desorden del procesamiento auditivo central. Se realizó una revisión de la literatura guiada por protocolo PRISMA en las bases de datos Proquest, Ebsco, Pubmed, ScienceDirect, Cochrane Library y Scielo de acuerdo con términos claves. Fueron incluidos artículos originales de investigación, revisiones sistemáticas y metaanálisis, publicados desde el año 2010, realizados en animales y humanos, escritos en idiomas inglés, español y portugués. Se encontraron 323 estudios relacionados con los términos claves utilizados, de los cuales 13 cumplieron con los criterios de inclusión y fueron analizados. Se puede concluir que la evidencia científica en torno al tema es escasa e incipiente. Estructuras de la vía auditiva central como núcleos cocleares, lemnisco lateral, colículos inferiores, cuerpo geniculado medial y corteza auditiva primaria estarían relacionadas con la hiperacusia, así como también habilidades de procesamiento auditivo de figura/fondo, ordenación temporal y transferencia interhemisférica se verían afectadas.

Abstract Hyperacusis has been defined as the excessive auditory sensitivity or intolerance to certain everyday sounds that seem common for most people. Considering the underlying physiological mechanisms of hyperacusis, it is reasonable to think that it could affect some abilities involved in the central auditory processing. However, there is lack of evidence about this topic, and there is no literature review that gathers all the existing research. Therefore, the current study intends to identify and analyze the available scientific evidence regarding the relationship between hyperacusis and central auditory processing disorder. The review of the literature followed the PRISMA protocol, using key words in Proquest, Ebsco, Pubmed, ScienceDirect, Cochrane Library and Scielo databases. Original research articles, systematic reviews and meta-analyses of studies made with human and animals that have been published since 2010 in English, Spanish and Portuguese were included. Among them, 323 studies were related to the key terms, out of which 13 met the inclusion criteria and were analyzed. It is possible to conclude that there is little and incipient scientific evidence on the topic. Structures of the central auditory pathway such as cochlear nuclei, lateral lemniscus, inferior colliculi, medial geniculate body and primary auditory cortex seem to be related to hyperacusis; auditory processing skills such as figure/ground discrimination, temporal ordering and interhemispheric transfer appear to be affected as well.

Percepção dos pais sobre hipersensibilidade auditiva de crianças com sinais clínicos de risco para o Transtorno do Espectro do Autismo / Parents' perception of auditory hypersensitivity in children with clinical signs of risk for Autism Spectrum Disorder

Resumo Objetivo Averiguar a ocorrência e o tipo de hipersensibilidade auditiva em crianças com sinais clínicos de Transtorno do Espectro do Autismo por meio do relato dos pais no contexto da pandemia da COVID-19. Método Trata-se de um estudo transversal e descritivo, composto por pais de 11 crianças com sinais clínicos de risco para Transtorno do Espectro do Autismo. As crianças eram de ambos os sexos, com média de 44,8 meses de idade. Os pais responderam, por meio de contato telefônico, a um questionário, previamente validado, sobre comportamentos de hipersensibilidade auditiva de seus filhos. Foi considerado como sinalizador de hipersensibilidade a pontuação igual ou superior a oito pontos no escore geral. Resultados 63,6% das crianças apresentaram resultado indicativo de hipersensibilidade e 54,5% obtiveram pontuação máxima nas questões relacionadas à irritabilidade a sons específicos. Os sons citados como geradores de irritabilidade foram: palmas, fogos, gritos, ferramentas de construção, canto e toque de celular. Conclusão Constatou-se ocorrência de hipersensibilidade auditiva, especialmente relacionada à irritabilidade, o que sugere relação com o sistema límbico e, portanto, pode remeter à misofonia. Assim, torna-se importante a equipe multidisciplinar atentar-se à ocorrência e a características de hipersensibilidade dessa população, a fim de maximizar condições favoráveis à reabilitação.

Abstract Objective To investigate the occurrence and type of auditory hypersensitivity in children with clinical signs of Autism Spectrum Disorder through parents' reports in the context of the COVID-19 pandemic. Method This is a cross-sectional and descriptive study, composed of parents of 11 children with clinical signs of risk for Autism Spectrum Disorder. The children were of both sexes, with a mean age of 44.8 months. Parents answered a previously validated questionnaire about their children's auditory hypersensitivity behaviors by telephone. A score equal to or greater than eight points in the general score was considered as a sign of hypersensitivity. Results 63.6% of the children presented results indicative of hypersensitivity and 54.5% obtained maximum scores on questions related to irritability to specific sounds. The sounds cited as generating irritability were: clapping, fireworks, shouting, construction tools, singing and cell phone ringtones. Conclusion Auditory hypersensitivity was observed, especially related to irritability, which suggests a relationship with the limbic system and, therefore, may refer to misophonia. Thus, it is important for the multidisciplinary team to pay attention to the occurrence and characteristics of hypersensitivity in this population, in order to maximize favorable conditions for rehabilitation.

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

RESUMO 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.

ABSTRACT 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.

Evaluation of selective attention in patients with misophonia / Avaliação da atenção seletiva em pacientes com misofonia

Abstract Introduction: Misophonia is characterized by the aversion to very selective sounds, which evoke a strong emotional reaction. It has been inferred that misophonia, as well as tinnitus, is associated with hyperconnectivity between auditory and limbic systems. Individuals with bothersome tinnitus may have selective attention impairment, but it has not been demonstrated in case of misophonia yet. Objective: To characterize a sample of misophonic subjects and compare it with two control groups, one with tinnitus individuals (without misophonia) and the other with asymptomatic individuals (without misophonia and without tinnitus), regarding the selective attention. Methods: We evaluated 40 normal-hearing participants: 10 with misophonia, 10 with tinnitus (without misophonia) and 20 without tinnitus and without misophonia. In order to evaluate the selective attention, the dichotic sentence identification test was applied in three situations: firstly, the Brazilian Portuguese test was applied. Then, the same test was applied, combined with two competitive sounds: chewing sound (representing a sound that commonly triggers misophonia), and white noise (representing a common type of tinnitus which causes discomfort to patients). Results: The dichotic sentence identification test with chewing sound, showed that the average of correct responses differed between misophonia and without tinnitus and without misophonia (p = 0.027) and between misophonia and tinnitus (without misophonia) (p = 0.002), in both cases lower in misophonia. Both, the dichotic sentence identification test alone, and with white noise, failed to show differences in the average of correct responses among the three groups (p ≥ 0.452). Conclusion: The misophonia participants presented a lower percentage of correct responses in the dichotic sentence identification test with chewing sound; suggesting that individuals with misophonia may have selective attention impairment when they are exposed to sounds that trigger this condition.

Resumo Introdução: A misofonia é caracterizada pela aversão a certos sons muito seletivos, que evocam uma forte reação emocional. Tem sido demonstrado que a misofonia, bem como o zumbido, está associada à hiperconectividade entre os sistemas auditivo e límbico. Indivíduos com zumbido incômodo podem ter comprometimento da atenção seletiva, mas isso ainda não foi demonstrado em caso de misofonia. Objetivo: Caracterizar uma amostra de indivíduos misofônicos e compará-la com dois grupos controle, um com indivíduos com zumbido (sem misofonia) e o outro com indivíduos assintomáticos (sem misofonia e sem zumbido) em relação à atenção seletiva. Método: Avaliamos 40 participantes com audição normal: 10 com misofonia, 10 com zumbido (sem misofonia) e 20 sem zumbido e sem misofonia. Para avaliar a atenção seletiva, o teste de identificação de sentenças dicóticas foi usado em três situações: no primeiro, aplicou-se o teste em português do Brasil. Então, o mesmo teste foi aplicado em duas outras situações, combinado com dois sons competitivos: som de mastigação (representa um som que geralmente desencadeia misofonia) e ruído branco (representa um tipo comum de zumbido que causa desconforto nos pacientes). Resultados: No teste de identificação de sentenças dicóticas com som de mastigação, observou-se que a média de respostas corretas diferiu entre os grupos misofonia e sem zumbido e sem misofonia (p = 0,027) e entre misofonia e zumbido (sem misofonia) (p = 0,002) e, em ambos os casos, foi menor em misofonia. Em relação ao teste feito isoladamente e com ruído branco, nenhuma diferença foi observada na média das respostas corretas nos três grupos (p ≥ 0,452). Conclusão: Os participantes do grupo misofonia apresentaram uma menor porcentagem de respostas corretas no teste de identificação de sentenças dicóticas com som de mastigação, sugeriu que indivíduos com misofonia podem ter comprometimento da atenção seletiva quando expostos a sons que desencadeiam a condição.

Severe Temporal Hyper-Activated States Caused by Noise in Tinnitus and Hyperacusis with Normal Hearing

Lots of neuroimaging and animal studies have revealed that tinnitus and hyperacusis share the same patterns in the bottom up central auditory process. The aim was to identify the abnormal central patterns commonly observed in both tinnitus and hyperacusis in humans. We investigated two cases of normal hearing: a tinnitus patient and a hyperacusis patient. We compared the differences between the severe temporal hyper-activated state (STHS), with spikes, fast beta and gamma frequencies after noise exposure, and the mild temporal hyper-activated state (MTHS), in no sound exposed condition. The power of the gamma band in the two cases was increased in both auditory cortices compared to the other brain regions. Our results of human with normal hearing were the first to identify how tinnitus and hyperacusis caused by sound are abnormally active and how they maintain constant pathological states.

Familial misophonia or selective sound sensitivity syndrome: evidence for autosomal dominant inheritance? / Misofonia familiar ou síndrome da sensibilidade seletiva a sons: evidência de herança autossômica dominante?

Abstract Introduction: Misophonia is a recently described, poorly understood and neglected condition. It is characterized by strong negative reactions of hatred, anger or fear when subjects have to face some selective and low level repetitive sounds. The most common ones that trigger such aversive reactions are those elicited by the mouth (chewing gum or food, popping lips) or the nose (breathing, sniffing, and blowing) or by the fingers (typing, kneading paper, clicking pen, drumming on the table). Previous articles have cited that such individuals usually know at least one close relative with similar symptoms, suggesting a possible hereditary component. Objective: We found and described a family with 15 members having misophonia, detailing their common characteristics and the pattern of sounds that trigger such strong discomfort. Methods: All 15 members agreed to give us their epidemiological data, and 12 agreed to answer a specific questionnaire which investigated the symptoms, specific trigger sounds, main feelings evoked and attitudes adopted by each participant. Results: The 15 members belong to three generations of the family. Their age ranged from 9 to 73 years (mean 38.3 years; median 41 years) and 10 were females. Analysis of the 12 questionnaires showed that 10 subjects (83.3%) developed the first symptoms during childhood or adolescence. The mean annoyance score on the Visual Analog Scale from 0 to 10 was 7.3 (median 7.5). Individuals reported hatred/anger, irritability and anxiety in response to sounds, and faced the situation asking to stop the sound, leaving/avoiding the place and even fighting. The self-reported associated symptoms were anxiety (91.3%), tinnitus (50%), obsessive-compulsive disorder (41.6%), depression (33.3%), and hypersensitivity to sounds (25%). Conclusion: The high incidence of misophonia in this particular familial distribution suggests that it might be more common than expected and raises the possibility of having a hereditary etiology.

Resumo Introdução: A misofonia é uma condição recentemente descrita, mal compreendida e negligenciada. É caracterizada por fortes reações negativas de ódio, raiva ou medo quando os indivíduos precisam enfrentar alguns sons repetitivos seletivos e de baixa intensidade. Os mais comuns que desencadeiam tais reações aversivas são aqueles provocados pela boca (mascar goma ou mastigar comida, estalar os lábios) ou nariz (respirando, cheirando e soprando) ou pelos dedos (digitando, amassando papel, clicando a caneta, tamborilando na mesa). Artigos anteriores citam que esses indivíduos geralmente conhecem pelo menos um parente próximo com sintomas semelhantes, sugerindo um possível componente hereditário. Objetivo: Encontramos e descrevemos uma família com 15 membros com misofonia, detalhando suas características comuns e o padrão de sons que desencadeiam um desconforto tão forte. Método: Todos os 15 membros concordaram em nos fornecer seus dados epidemiológicos e 12 concordaram em responder a um questionário específico que investigou os sintomas, sons de gatilho específicos, principais sentimentos evocados e atitudes adotadas por cada participante. Resultados: Os 15 membros pertencem a três gerações da família. A idade variou de 9 a 73 anos (média de 38,3 anos, mediana de 41 anos) e 10 eram mulheres. A análise dos 12 questionários mostrou que 10 indivíduos (83,3%) desenvolveram os primeiros sintomas durante a infância ou a adolescência. A média do escore de irritação na Escala Visual Analógica de 0 a 10 foi de 7,3 (mediana 7,5). Os indivíduos relataram sentimentos de ódio/raiva, irritabilidade e ansiedade em resposta a sons, e enfrentaram a situação pedindo para interromper o som, deixando/evitando o lugar e até mesmo discutindo. Os sintomas associados auto-relatados foram ansiedade (91,3%), zumbido (50%), transtorno obsessivo-compulsivo (41,6%), depressão (33,3%) e hipersensibilidade aos sons (25%). Conclusão: A alta incidência de misofonia nessa distribuição familiar em particular sugere que possa ser mais comum do que o esperado e suscita a possibilidade de haver uma etiologia hereditária.

Tinnitus and sound intolerance: evidence and experience of a Brazilian group / Zumbido e intolerância a sons: evidência e experiência de um grupo brasileiro

Abstract Introduction Tinnitus and sound intolerance are frequent and subjective complaints that may have an impact on a patient's quality of life. Objective To present a review of the salient points including concepts, pathophysiology, diagnosis and approach of the patient with tinnitus and sensitivity to sounds. Methods Literature review with bibliographic survey in LILACS, SciELO, Pubmed and MEDLINE database. Articles and book chapters on tinnitus and sound sensitivity were selected. The several topics were discussed by a group of Brazilian professionals and the conclusions were described. Results The prevalence of tinnitus has increased over the years, often associated with hearing loss, metabolic factors and inadequate diet. Medical evaluation should be performed carefully to guide the request of subsidiary exams. Currently available treatments range from medications to the use of sounds with specific characteristics and meditation techniques, with variable results. Conclusion A review on tinnitus and auditory sensitivity was presented, allowing the reader a broad view of the approach to these patients, based on scientific evidence and national experience.

Resumo Introdução Zumbido e intolerância a sons são queixas frequentes e subjetivas que podem ter impacto na qualidade de vida do paciente. Objetivo Apresentar uma revisão dos principais pontos, inclusive conceitos, fisiopatologia, diagnóstico e abordagem do paciente com zumbido e sensibilidade a sons. Método Revisão da literatura com levantamento bibliográfico na base de dados da LILACS, SciELO, Pubmed e MEDLINE. Foram selecionados artigos e capítulos de livros sobre zumbido e sensibilidade a sons. Os diversos tópicos foram discutidos por um grupo de profissionais brasileiros e as conclusões, descritas. Resultado A prevalência de zumbido tem aumentado ao longo dos anos, muitas vezes associado a perda auditiva, fatores metabólicos e erros alimentares. A avaliação médica deve ser feita minuciosamente no sentido de orientar a solicitação de exames subsidiários. Os tratamentos disponíveis atualmente variam de medicamentos ao uso de sons com características específicas e técnicas de meditação, com resultados variáveis. Conclusão Foi apresentada uma revisão sobre os temas que permitindo ao leitor uma visão ampla da abordagem dos pacientes com zumbido e sensibilidade auditiva baseada em evidências científicas e experiência nacional.

Tinnitus in adolescents: the start of the vulnerability of the auditory pathways / Zumbido em adolescentes: o início da vulnerabilidade das vias auditivas

INTRODUCTION: Although tinnitus is an increasingly common symptom, few studies have assessed its prevalence or incidence among adolescents. PURPOSE: To assess whether the presence of tinnitus in adolescents is associated with minimal hearing damage, evaluated through high-frequency audiometry (HFA), otoacoustic emission (OAE), and loudness discomfort level (LDL). METHODS: The sample comprised 168 adolescents of a private school (61.3% boys; mean age 14.1 years old; standard deviation=2). All of them completed a questionnaire about tinnitus and hypersensitivity to sounds (sound intolerance), and then underwent otoscopy, pure-tone audiometry, HFA, LDL, transient and distortion product otoacoustic emissions (TOAE and DPOAE), and tinnitus pitch/loudness matching (the latter only in those with tinnitus). Participants were later divided into three groups: with no tinnitus (n=73, 43.4%), with sporadic tinnitus (n=47, 28%), and with constant tinnitus (n=48, 28.6%). RESULTS: No significant difference was observed between the groups regarding audiometry thresholds in frequencies from 0.25 to 16 kHz, or TOAE and DPOAE. However, the LDL in adolescents with constant tinnitus was significantly lower than that in other groups, suggesting hypersensitivity to sounds. CONCLUSION: There was no evidence of minimal hearing damage in the audiometry and OAE. Nonetheless, the decreased LDL in adolescents with constant tinnitus suggests that their auditory system is more sensitive. Therefore, this may be the first sign of vulnerability to sounds. Future medium- to long-term monitoring of these students may show whether they will begin a process of functional impairment, altering hearing thresholds, and OAE. .

Embora o zumbido seja um sintoma cada vez mais comum, poucos estudos avaliaram sua prevalência ou incidência entre adolescentes. OBJETIVO: Avaliar se a presença de zumbido em adolescentes está associada a lesões auditivas mínimas, avaliadas por audiometria de altas frequências (AAF), emissões otoacústicas (EOA) e limiar de desconforto a sons (LDL). MÉTODOS: Participaram da amostra 168 alunos adolescentes de uma escola particular, sendo 61,3% do gênero masculino e com média de idade de 14,1 anos (desvio padrão=2). Todos responderam a um questionário sobre zumbido e hipersensibilidade auditiva (intolerância a sons) e foram submetidos à otoscopia, audiometria tonal convencional e de frequências acima de 8.000 Hz, LDL, EOA transientes (EOAT) e produto de distorção (EOAPD) e acufenometria (esta apenas naqueles com zumbido). Em seguida, foram divididos em três grupos: Sem Zumbido (n=73; 43,4%), Zumbido Esporádico (n=47; 28%) e Zumbido Constante (n=48; 28,6%). RESULTADOS: Não houve diferença significativa entre os grupos em relação aos limiares da audiometria nas frequências de 250 a 16.000 Hz, nem nas EOAT ou EOAPD. Entretanto, o LDL dos adolescentes com zumbido constante foi significativamente menor do que o dos demais grupos, sugerindo hipersensibilidade auditiva. CONCLUSÃO: Não houve evidência de alterações auditivas mínimas na audiometria e EOA. Entretanto, a diminuição do LDL em jovens com zumbido constante sugere que suas cócleas sejam mais sensíveis. Portanto, esse pode ser o primeiro sinal de vulnerabilidade a sons, mas o acompanhamento desses adolescentes a médio prazo poderá demonstrar se tais regiões entrarão em processo de comprometimento funcional, alterando os limiares audiométricos e as EOA. .

Estudo da relacao entre o grau de incomodo de pacientes com zumbido e a presenca de hiperacusia / Study of the relationship between the degree of tinnitus annoyance and the presence of hyperacusis

Introdução: A hiperacusia pode ser definida como uma manifestação de ganho central aumentado das vias auditivas, compreendida como um estado pré-zumbido. Em alguns casos, o zumbido pode ser secundário a esse ganho aumentado. Objetivo: Avaliar a prevalência da hiperacusia em pacientes com zumbido e sua associação com o incômodo do zumbido. Materiais e métodos: Estudo retrospectivo envolvendo pacientes do ambulatório de otoneurologia com queixa principal de zumbido que foram submetidos a avaliação clínica, audiológica e a questionário de avaliação da hiperacusia e do zumbido. O grau de incômodo da hiperacusia e do zumbido foi classificado utilizando a Escala Visual Analógica. Resultados: Foram analisados prontuários de 309 pacientes, 169 (54,7%) do sexo feminino e 140 (45,3%) do sexo masculino, com idade média de 53 anos. O grau de incômodo do zumbido apresentou mediana de sete. A hiperacusia esteve presente em 57 (18,4%) pacientes, com mediana de grau de incômodo de cinco. O grau de incômodo pelo zumbido nos pacientes com hiperacusia foi semelhante ao dos pacientes sem hiperacusia. Conclusão: A hiperacusia esteve presente em 18,4% dos pacientes com zumbido. O grau de incômodo do zumbido não teve correlação com a presença da hiperacusia. .

Introduction: Hyperacusis can be defined as a manifestation of an increased of central auditory pathways gain and can be considered a pre-tinnitus state. In some cases tinnitus can be caused by such increased gain. Aim: To evaluate the prevalence of hyperacusis in patients with tinnitus and its relation to the annoyance of tinnitus. Materials and methods: Retrospective study with patients from the neurotology service complaining of tinnitus in the first consultation were submitted to clinical evaluation, a questionnaire and audiological evaluation of tinnitus and hyperacusis. The degree of annoyance of tinnitus and hyperacusis was measured using a visual analog scale. Results: We analyzed medical records of 309 patients, 169 (54.7%) females and 140 (45.3%) males. The mean age was 53 years. The median degree of tinnitus annoyance was 7. Hyperacusis was present in 57 (18.4%) patients, with a median degree of 5. The degree of annoyance due to tinnitus patients with hyperacusis was similar to that of patients without hyperacusis. Conclusion: Hyperacusis was present in 18.4% of patients with tinnitus. The degree of annoyance due to tinnitus had no correlation with the presence of hyperacusis. .

Os pais conhecem as queixas auditivas de seus filhos? / Are parents aware of their children's hearing complaints?

A acurácia dos pais sobre a audição dos filhos é variável e pode diferir das queixas das crianças. OBJETIVO: Investigar as queixas auditivas de crianças e as impressões dos pais a respeito da audição das crianças. MÉTODOS: 477 crianças (2º ao 5º ano do ensino fundamental) foram entrevistadas e seus pais responderam a pesquisa em casa. RESULTADOS: Vinte e nove porcento das crianças referiram dificuldade para entender fala no silêncio, 36,1% tinham história de um a três otites e 12,7% de quatro ou mais otites, 21,7% tinham zumbido contínuo (associação com exposição a sons intensos, p = 0.0007), 3,8% tinham zumbido pulsátil e 2,9% tinham alucinações auditivas. Vinte e oito e meio porcento referiram incômodo com sons intensos (associação com a queixa de zumbido, p = 0,0142, e com gênero, p = 0,0029) 10,4% haviam feito avaliação audiológica, e os fatores determinantes foram história de otites (p < 0,001) e preocupação dos pais com a audição dos filhos (p = 0,043). As respostas dos pais e de seus filhos foram significativamente diferentes. CONCLUSÕES: As queixas auditivas das crianças são prevalentes e relevantes, mas a maioria delas nunca teve a audição avaliada e a maioria dos pais não sabe das queixas de seus filhos. Intolerância a sons e alucinações auditivas deveriam ser consideradas em avaliações clínicas e audiológicas.

The accuracy of parents' impressions about their child's hearing status is variable and may not correspond to the child's complaints. AIM: To investigate children's self-reported hearing symptoms and parents' impressions about it. METHODS: 477 children (2nd to 5th grades of elementary schools) were interviewed and parents answered a survey at home. There were 393 matches between the children's interview and the parent's survey. RESULTS: 29% of the children reported trouble in understanding what people said, 36.1% had history of 1-3 ear infections, 12.7% had four or more ear infections, 21.7% had continuous tinnitus (positive association with history of exposure to loud sounds, p = 0.0007), 3.8% had pulsatile tinnitus and 2.9% had auditory hallucinations. 28.5% of the children were annoyed by loud sounds (associated with tinnitus, p = 0.0142, and gender, p = 0.0029) 10.4% had had audiological tests, and the determinant factors were history of ear infections (p < 0.001) and parents' concern about their child's hearing (p = 0.043). Parents and their own child's responses were significantly different. CONCLUSIONS: Children's auditory complaints were prevalent and relevant, but most of them had never had an audiological evaluation and most parents were not aware of their child's complaints. Sound intolerances and auditory hallucinations should be considered in clinical and audiological examinations.

Ocorrência e efeito de supressão das Emissões Otoacústicas em adultos normo-ouvintes com zumbido e hiperacusia / Occurrence and suppression effect of Otoacoustic Emissions in normal hearing adults with tinnitus and hyperacusis

A associação entre zumbido e hiperacusia é frequente na literatura. OBJETIVOS: Verificar a ocorrência e o efeito de supressão das emissões otoacústicas transientes (EOATs), a existência de associação entre graus de zumbido e de hiperacusia, entre efeito supressor das EOATs e lateralidade, graus de zumbido e de hiperacusia, em adultos normo-ouvintes com queixas de zumbido e hiperacusia. MATERIAIS E MÉTODOS: Foram incluídos, nesta forma de estudo transversal, 25 indivíduos normo-ouvintes com queixas de zumbido e hiperacusia. Utilizou-se o Tinnitus Handicap Inventory (THI) para classificação do grau do zumbido e o Loudness Discomfort Level (LDL) para o da hiperacusia. RESULTADOS: A ocorrência das EOATs variou de 33 por cento a 88 por cento. Houve 63,7 por cento de presença de efeito de supressão na orelha direita e 81,7 por cento na orelha esquerda. Não ocorreu correlação significativa entre os graus de zumbido e os graus de hiperacusia em ambas as orelhas e não houve associação significativa entre efeito de supressão das EOATs e lateralidade, grau de zumbido e de hiperacusia. CONCLUSÃO: A ocorrência de EOATs foi inferior à encontrada em adultos normo-ouvintes. Obteve-se maior percentual de presença do efeito de supressão das EOATs em ambas as orelhas. Não houve associação entre as variáveis analisadas.

The association between tinnitus and hyperacusis is common according to the literature. AIM: To verify the occurrence and the suppression effect of transient otoacoustic emissions (TEOAE), the existence of association between tinnitus degrees and hyperacusis degrees, and between the suppressive effect of TEOAE and laterality, tinnitus and hyperacusis degrees in normal hearing adults with complaints of tinnitus and hyperacusis. MATERIALS AND METHODS: 25 normal hearing subjects with complaints of hyperacusis and tinnitus were studied in this cross-sectional study. The Tinnitus Handicap Inventory (THI) was used for the classification of tinnitus degrees, and the Loudness Discomfort Level (LDL) for the hyperacusis classification. RESULTS: The occurrence of TEOAE ranged from 33 to 88 percent. We observed the presence of TEOAE suppression effect on 63.7 percent in the right ear and 81.7 percent in the left ear. There was no significant correlation between the degrees of tinnitus and hyperacusis in both ears. No statistically significant associations between the TEOAE suppression effect and laterality, tinnitus degrees and hyperacusis degrees were found. CONCLUSION: The occurrence of TEOAE was lower than that found in normal hearing adults. A higher percentage of the presence of TEOAE suppression effect has been found in both ears. No association between the variables was observed.

Características do zumbido e da hiperacusia em indivíduos normo-ouvintes / Characteristics of the tinnitus and hyperacusis in normal hearing individuals

INTRODUÇÃO: O zumbido vem se tornando uma queixa otológica frequente. Outra queixa que é encontrada em portadores de zumbido é a hiperacusia. OBJETIVO: Analisar as características do zumbido e da hiperacusia em indivíduos normo-ouvintes com queixa associada de zumbido e hiperacusia. MÉTODO: 25 indivíduos normo-ouvintes que apresentaram queixas de hiperacusia e zumbido foram pesquisados nesta forma de estudo transversal.Questionou-se sobre a localização e o tipo do zumbido. Utilizou-se o Tinnitus Handicap Inventory brasileiro e a acufenometria para avaliação do zumbido. Foi elaborado um questionário sobre hiperacusia abordando aspectos como: sons considerados desconfortáveis, sensações na presença desses sons e dificuldade de compreensão de fala no ruído. RESULTADOS: Dos 25 indivíduos, 64 por cento eram mulheres e 36 por cento homens. Em relação ao zumbido, 84 por cento referiram localização bilateral e 80 por cento pitch agudo. O grau mais encontrado foi o leve (44 por cento). As mulheres apresentaram grau de zumbido estatisticamente superior ao dos homens. Os sons de forte intensidade e as reações de irritação, ansiedade, necessidade de afastar-se do som foram mais citadas. Dos indivíduos analisados, 68 por cento referiram dificuldade de compreensão de fala no ruído e 12 por cento relataram usar protetores auriculares. As frequências mais encontradas na acufenometria foram 6 e 8 KHz. CONCLUSÃO: Indivíduos normo-ouvintes com queixa de zumbido e hiperacusia apresentaram predomínio de zumbido de pitch agudo, localização bilateral e grau leve. Os sons considerados desconfortáveis foram os de forte intensidade e a reação aos sons mais citada foi à irritação. A dificuldade de compreensão de fala no ruído foi referida pela maioria dos indivíduos.

INTRODUCTION: The tinnitus has become a common otological complaint. Another complaint is found in bearers of the tinnitus is the hyperacusis. OBJECTIVE: Analyze the characteristics of tinnitus and hyperacusis in normal hearing individuals with associated complaints of tinnitus and hyperacusis. METHOD: 25 normal hearing individuals who complained of hyperacusis and tinnitus were surveyed in this form of cross-sectional study. They were questioned about the location and type of the tinnitus. The evaluation of the tinnitus was made using the Brazilian Tinnitus Handicap Inventory and acuphenometry. A questionnaire was made about the hyperacusis covering aspects such as: sounds considered uncomfortable, sensations in the presence of such sounds, and difficulty understanding speech in noise. RESULTS: Of the 25 individuals, 64 percent were women and 36 percent men. Regarding tinnitus, 84 percent referred to bilateral location and 80 percent high pitch. The most common degree found was light (44 percent). The women presented tinnitus degree statistically superior to those of men. The strong intensity sounds and the reactions of irritation, anxiety and the need to move away from the sound were the most mentioned. From the analyzed individuals, 68 percent referred to difficulty understanding speech in noise and 12 percent reported using hearing protection. The most found frequencies at the acuphenometry were 6 and 8 KHz. CONCLUSION: Normal hearing individuals who complain of tinnitus and hyperacusis present mainly high pitch tinnitus, located bilaterally and light degree. The sounds considered uncomfortable were the high intensity ones and the most cited reaction to sound was irritation. The difficulty to understand speech in noise was reported by most of the individuals.

Phonophobia and Hyperacusis: Practical Points from a Case Report

Phonophobia and hyperacusis are two separate but closely related symptoms that are often mistakenly used in clinical practice as the same entity. Here we present a case report to highlight the distinguishing features of both and discuss the steps of management in these conditions. It is vital for the attending doctors to recognise hyperacusis and phonophobia as different entities to manage them successfully.

Clinical Characteristics of Children with Williams Syndrome According to Age

PURPOSE: Williams syndrome (WS) is an autosomal dominant genetic disorder, caused by deletion at chromosome 7q11.23. Although WS is characterized by congenital heart disease (CHD), mental retardation, and distinctive facial dysmorphism, clinical features may vary according to a patient's age, making the diagnosis more difficult. We reviewed clinical, psychosocial, and behavioral developmental manifestations of WS patients according to age. METHODS: We retrospectively studied nine children with WS from March 2003 to February 2009 at NHIC Ilsan Hospital Developmental Disorder Clinic. Patients were divided into three groups by age: infancy, preschool age and school age. Patients' facial dysmorphism, motor, speech, and cognitive development and associated symptoms were analyzed. RESULTS: Prominent clinical features of the infants group were feeding difficulty, hypotonia, constipation, sleep disturbance, hernia with facial features which were not characteristic. The preschool age group showed developmental delays, had very friendly personalities, and hyperacusis. Their typical facial features included long philtrum, prominent lips, and teeth malocclusion. The school age group showed mental retardation, learning disorders, and typical facial dysmorphism, as well as short stature. Although overly sociable, they were not able to adapt to school life. Eight patients out of nine showed CHD. CONCLUSION: WS can be suspected when an infant primarily presents with hypotonia, feeding difficulty, failure to thrive with CHD, and occasional hypercalcemia. When a patient in early childhood presents with delayed development with good verbal language ability and has hyperacusis, WS must be suspected. When an older or adolescent child has unique learning disabilities and has facial dysmorphism, short stature, and a loquacious personality, WS should also be suspected.

Hiperacusia em músicos de banda militar / Hyperacusis in military band musicians

OBJETIVO: Identificar a presença de hiperacusia e investigar as características dos sons desconfortáveis e os comportamentos desencadeados pelo desconforto, em músicos de uma Banda Militar. MÉTODOS: Foram estudados 27 músicos da Banda Militar da Base Aérea de Santa Maria (RS), com idades entre 22 e 50 anos, com tempo de serviço militar entre quatro e 26 anos e com exposição diária ao ruído de trabalho de duas a oito horas. Todos foram submetidos à avaliação audiológica básica, teste do limiar de desconforto sonoro e aplicação de um questionário. Considerou-se presença de hiperacusia, quando a média dos valores obtidos no teste do limiar de desconforto em 250, 500, 1000, 2000, 4000 Hertz foi menor ou igual a 90 decibéis, associado à queixa de desconforto auditivo. RESULTADOS: Verificou-se hiperacusia em 37 por cento dos músicos. Destes, 50 por cento apresentaram audição normal e 50 por cento apresentaram audição normal com presença de entalhe; 80 por cento sentiam o desconforto diariamente e 20 por cento após o trabalho com a banda de música; 70 por cento já evitaram realizar alguma atividade, por acreditarem que o ruído ambiental é desconfortável; 70 por cento faziam uso de protetor auricular regularmente e 90 por cento referiram zumbido. Os sons considerados desconfortáveis pelos hiperacúsicos foram, predominantemente, os de forte intensidade. As reações emocionais mais citadas mediante um som considerado desagradável foram: tensão, ansiedade e necessidade de afastar-se do som. CONCLUSÃO: Com os critérios utilizados neste estudo, 37 por cento dos indivíduos estudados foram classificados como hiperacúsicos, os quais consideraram desagradáveis os sons de forte intensidade, predominantemente. As principais reações emocionais mediante esses sons foram: tensão, ansiedade e necessidade de afastar-se do som. As características comuns entre os sujeitos classificados como hiperacúsicos foram: audição normal, uso de protetor auricular, evitar...

PURPOSE: To identify the presence of hyperacusis and to investigate the characteristics of uncomfortable sounds, as well as the behaviors triggered by the discomfort in musicians of a Military Band. METHODS: Twenty seven musicians of the Military Band of the Santa Maria Airbase (RS) were studied. Their ages ranged from 22 to 50 years old and military work time from four to 26 years, with daily occupational noise exposure time from two to eight hours. All subjects were submitted to a basic audiological evaluation, sound discomfort threshold test and application of a questionnaire. It was considered presence of hyperacusis when the average of the obtained values on the discomfort threshold in 250, 500, 1000, 2000, 4000 Hertz was less than or equal to 90 decibels, associated with hearing discomfort complaint. RESULTS: It was verified presence of hyperacusis in 37 percent of the musicians. From these, 50 percent presented normal hearing and 50 percent had normal hearing with notch; 80 percent of them felt the discomfort daily and 20 percent only after band practice; 70 percent of the musicians had already avoided some activities, believing that the activity noise would be distressing; 70 percent of them used ear plugs regularly and 90 percent referred presence of tinnitus. The sounds that were considered to be distressing by hyperacusics were, predominantly, high intensity sounds. The most common emotional responses towards a distressing sound were tension, anxiety and the urge of repelling from it. CONCLUSION: According to the criteria used in this study, 37 percent of the studied individuals were classified as hyperacusics, which considered predominantly high intensity sounds to be unpleasant. The main emotional responses before these sounds were: tension, anxiety and the need to repel from the sound. The common characteristics among the subjects classified as hyperacusics were: normal hearing, use of ear plugs, avoidance of activities...

Relationship among loudness recruitment, cochlear microphonics and prognosis of hearing loss / 中国耳鼻咽喉头颈外科

75dB SPL or no response. RESULTS The individual differences of CM absolute amplitudes among the normal hearing ones at different frequencies are distinct, but bilateral CM amplitudes in same person are almost uniform and stable. Enlargedand prolongated CM was found in ears with loudness recruitment .Of 104 cases with unilateral hearing loss, CM were enlarged and prolongated at corresponding frequencies with loudness recruitment in 95 cases(91.3 %).The prognosis of cases with CM type Ⅰ was better than those with types Ⅱ and Ⅲ. The effective rates of treatment were 78.19 % in type Ⅰ , 2.5 % in type Ⅱ, and none in type Ⅲ. There were significant differences among them. CM was slightly enlarged during sleep, and the detected threshold of CM is less than that of the awake condition. CONCLUSION CM offers the reliable information for the mechanism of loudness recruitment and is also useful for understanding the relationship among loudness recruitment, cochlear microphonics and prognosis. CM may be taken as a valuable parameter for evaluating prognosis.

The Analysis of Loudness Discomfort Level and Hyperacusis in Tinnitus Patients / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Hyperacusis is an abnormally strong reaction which occurs within auditory pathways resulting from exposure to moderate sound. More patients are inflicted with it than with tinnitus, accounting for a relatively high proportion of the total tinnitus patients. However, no single test could pass as an objective diagnostic test of hyperacusis. Among various psychoacoustic evaluations, loudness discomfort level (LDL) is frequently used in the evaluation of hyperacusis. Thus, this study aimed to demonstrate a proportion of hyperacusis in tinnitus patients and characterize LDL in hyperacusis and tinnitus patients as a diagnostic test. MATERIALS AND METHOD: Using 186 patients with tinnitus as experiment group and 35 acoustically normal persons as control group, pure tone audiometry (PTA), LDL, and dynamic range (DR) were checked. RESULTS: Amongst the total number of tinnitus patients, there were 10 hyperacusis patients (5.38%). Those hyperacusis patients had much lower LDL (73.50 dB) and DR (41.50 dB) than tinnitus patients without hyperacusis (LDL: 100.90 dB, DR: 67.20 dB) as well as the control group (LDL: 104.86 dB, DR: 93.98 dB). CONCLUSION: Hyperacusis patients consist 5.38% (10/186) and have much lower LDL than tinnitus patients without hyperacusis and the control group. There is a positive correlation between hyperacusis and LDL. Therefore, in a diagnosis of hyperacusis, it is essential to check LDL and take a careful look into the history from patients.

Hyperacusis in Patients with Tinnitus-Audiometrical Evaluation & Clinical Characterization / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Hyperacusis, defined as increased sound sensitivity or decreased sound tolerence, has been reported to be related to tinnitus. The purposes of this study are to investigate the audiometrical characterization of hyperacusis and to evaluate the possible effect of hyperacusis on clinical presentation in tinnitus patients. MATERIALS AND METHOD: A total of 128 tinnitus patients, who visited the Tinnitus Clinic, Kangnam St. Mary's Hospital from May, 1999 to September, 2000, were included in this study. Their audiometrical study results were analyzed, and their hearing impairment was compared with those of normal controls. RESULTS: The prevalence of hyperacusis in the patients with tinnitus were as high as 37.8% according to the questionnare. Uncomfortable loudness levels to pure tone stimuli was significantly low in hyperacusis patients. In the tinnitus patients with normal hearing, the tinnitus handicap scores between hyperacusis and non-hyperacusis patients were significantly different, though the results of tinnitogram showed no difference. CONCLUSION: The hyperacusis test using pure tone stimuli may screen hyperacusic patients. It is necessary to consider the presence of hyperacusis in tinnitus patients so as to understand patient discomfort and to seek appropriate treatment modality.