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.

A influência das curvas timpanométricas nos resultados das emissões otoacústicas / The influence of tympanometric curves on the results of otoacoustic emissions

RESUMO Objetivo Analisar as emissões otoacústicas evocadas produto de distorção (EOAPD) de indivíduos adultos entre 18 e 50 anos, com audição normal, e associar os resultados com os achados à timpanometria. Métodos Foram selecionados 27 prontuários de adultos com audição dentro dos padrões de normalidade, sem queixa auditiva, com curva timpanométrica do tipo A, Ad ou Ar, presença de reflexos acústicos, sem queixa de zumbido e de exposição frequente a níveis de pressão sonora elevados, com repouso auditivo mínimo de 14 horas no momento do exame e que realizaram o exame de emissões otoacústicas evocadas produto de distorção. Os resultados do exame de emissões otoacústicas foram analisados considerando os resultados das curvas timpanométricas apresentadas por estes indivíduos. Para análise dos dados foram aplicados testes não paramétricos e o nível de significância foi de 5%. Resultados foram analisados os resultados das emissões otoacústicas de 54 orelhas. Observou-se maior ocorrência da curva do tipo A em indivíduos sem queixas auditivas. Independente do lado, a maioria das orelhas que apresentou resposta presente ao exame de EOAPD, apresentou, também, curva timpanométrica normal. Observou-se correlação positiva entre a amplitude das EOAPD e o volume da orelha média para as frequências de 6000 Hz e uma tendência à significância em 4000 Hz Conclusão Foi possível concluir que há maior ocorrência de emissões otoacústicas presentes em indivíduos com audição normal e curva timpanométrica do tipo A e que a amplitude das EOAPD em 6000 Hz mostra-se menor nos indivíduos com audição normal e curva timpanométrica do tipo Ar ou Ad.

ABSTRACT Purpose To analyze distortion product evoked otoacoustic emissions in normal-hearing adults aged between 18 and 50 years old, and to associate the results with the findings of tympanometry. Methods 27 medical records were selected of adults with the following conditions: normal hearing; without auditory complaint; with type A, Ad or Ar tympanometric curve; with presence of acoustic reflexes; with no complaint of tinnitus or frequent exposure to high sound pressure levels; with minimal auditory rest of 14 hours at the time of the test, and who had undergone distortion product evoked otoacoustic emission (DPOAE) testing. The results of otoacoustic emissions were analyzed considering the results of the tympanometric curves presented by these individuals. For the data analysis, non-parametric tests were applied, and the level of significance was 5%. Results The results of otoacoustic emissions of 54 ears were analyzed. There was a greater occurrence of the type A curve in individuals without auditory complaints. Regardless of side, most ears whose response was present in the DPOAE test also presented normal tympanometric curve. There was a positive correlation between DPOAE amplitude and middle ear volume for the 6000Hz frequencies (p = 0.048) and a tendency to significance at 4000Hz (p = 0.054). Conclusion There was a higher occurrence of otoacoustic emissions present in normal-hearing individuals and type A tympanometric curve, and the amplitude of DPOAE at 6000Hz was smaller in normal-hearing individuals and type Ar or Ad tympanometric curve.

Utilidad de mediciones objetivas para la programación en implantes cocleares / Use of objective measures for cochlear implant programming

RESUMEN Introducción: La programación adecuada de los implantes cocleares permiten lograr niveles de estimulación auditivos óptimos. Se realiza de forma individualizada, siendo la detección del umbral de confort ideal un desafío. Se ha descrito la utilidad de los potenciales auditivos del tronco encefálico eléctrico (ePEATC) y el reflejo eléctrico estapedial (eREE) para este propósito. Objetivo: Determinar la posibilidad de realizar ePEATC y eREE en pacientes adultos y pediátricos con implantes cocleares, y evaluar cambios en la programación de los implantes cocleares luego de las mediciones objetivas. Material y método: Se realizó un estudio prospectivo, descriptivo, de pacientes con implante coclear marca MED-EL®, separando los pacientes en dos grupos: el grupo adulto (n =5) y el grupo pediátrico (n =5). Todos los pacientes incluidos presentaron más de 6 meses de encendido del implante. Se evaluaron variables epidemiológicas y tiempo de encendido del implante. Se realizó una otoscopía, prueba básica de funcionamiento del implante, y las mediciones objetivas eléctricas (ePEATC, eREE). Con estos resultados se ajustó el umbral de confort. Resultados: El tiempo promedio de encendido del implante en el grupo adulto fue de 27 meses, y 30 meses en el grupo pediátrico. El ePEATC requiere más tiempo y cooperación al compararlo con eREE. Luego de estas evaluaciones objetivas, fue necesario el ajuste del umbral de confort en tres pacientes adultos, y en dos pacientes pediátricos. Fue necesario realizar una audiometría de campo libre para estimar el umbral de confort en dos pacientes pediátricos que no presentaron respuesta en eREE. Conclusión: Fue posible realizar estas mediciones objetivas en pacientes adultos y pediátricos, siendo mejor tolerado y requiriendo menos tiempo, el eREE.

ABSTRACT Introduction: Mapping a cochlear implant allows for adjusting ideal electrical stimulation limits. It is an individualized process and detecting the most comfortable loudness level can be challenging. The use of electrically evoked auditory brainstem response (ePEATC) and electrically evoked stapedius reflex thresholds (eREE) have been considered for this purpose. Aim: To determine the feasibility of performing ePEATC and eREE on adult and pediatric patients with a cochlear implant, and to evaluate changes in programming following these objective measures. Material and method: A prospective, descriptive study was completed, of patients with MED-EL® cochlear implants, separating patients into two groups: adults (n=5) and children (n=5). All of the patients included had their implants activated for 6 months or longer. Epidemiological variables and duration of implant activation were evaluated. Otoscopy, a basic implant functioning evaluation, and objective measures (ePEATC, eREE) were performed. With these results, comfortable loudness levels were adjusted. Results: Average duration of implant activation was 27 months and 30 months, for adults and children respectively. Performing ePEATC required more time and cooperation as compared to eREE. Following the objective measures, adjustment of the comfortable loudness levels was required for three adult and two pediatric patients. Sound field audiometry was necessary for two pediatric patients in order to estimate the comfortable loudness levels because the eREE responses were absent. Conclusions: It is feasible to perform these objective measures for both adult and pediatric patients, with eREE requiring less time and being better tolerated by patients.

Achados audiológicos em pacientes portadores de disfunção temporomandibular / Audiological findings in patients with temporomandibular dysfunction

RESUMO Objetivo Analisar os achados audiológicos em indivíduos com desordem temporomadibular e comparar esses achados com indivíduos sem desordem temporomanbibular. Métodos A amostra foi composta por 39 participantes adultos, de ambos os gêneros, com diagnóstico prévio de desordem temporomandibular (grupo de estudo) e 39 participantes adultos, sem desordem temporomandibular (grupo controle). Todos os participantes foram submetidos à audiometria tonal limiar em altas frequências, imitanciometria e pesquisa das emissões otoacústicas evocadas por produto de distorção. Resultados Observou-se prevalência de desordem temporomandibular no gênero feminino e a média de idade ficou acima da quarta década de vida. Na audiometria tonal limiar, foi observada ocorrência de perda auditiva do tipo neurossensorial e condutiva, no grupo de estudo, além de piores limiares auditivos em altas frequências. Em ambos os grupos, houve maior ocorrência de curva timpanométrica do tipo A, bem como diferenças entre os grupos na pesquisa do reflexo acústico ipsilateral e contralateral e no registro das emissões otoacústicas evocadas por produto de distorção. Conclusão Indivíduos com desordem temporomandibular apresentam piores resultados nos limiares auditivos, na timpanometria, nos reflexos acústicos ipsilaterais e contralaterais e nas emissões otoacústicas evocadas, quando comparados com o grupo controle.

ABSTRACT Purpose To analyze the audiological findings in individuals with temporomandibular disorder and compare these findings with individuals without temporomandibular disorder. Methods The sample was composed by 39 adult participants, both genders, with previous diagnosis of temporomandibular disorder (study group) and 39 adult participants, without temporomandibular disorder (control group). All the participants were submitted to audiometry including high frequencies, Immittance acoustic (Tympanometry and contralateral acoustic reflexes) and distortion product evoked otoacoustic emissions. Results The prevalence of temporomandibular disorder on female gender was observed, average age was over forty years old. In the audiometry, it was observed the incidence of hearing loss of a sensorineural and conductive type in the Study Group, besides worst hearing thresholds in high frequencies. In both groups, there was a bigger incidence of a type A tympanometric curve, as well as there were differences between the groups in the research of the ipsilateral and contralateral acoustic reflex and in the recording of distortion product evoked otoacoustic emissions. Conclusion It's possible to conclude that individuals with temporomandibular disorder show worst results in the hearing thresholds, in the tympanometry, in the ipsilateral and contralateral acoustic reflexes, in the evoked otoacoustic emissions, when compared to the control group.

Pedigree Analysis and Audiological Investigations of Otosclerosis: An Extended Family Based Study

BACKGROUND AND OBJECTIVES: To analyse the audiometric profile and the pedigree of a large family with otosclerosis to understand the inheritance pattern and its implication in clinical management of the disease. SUBJECTS AND METHODS: Pedigree analysis was performed on the basis of family history and audiometric tests. Pure tone audiometry, tympanometry, and acoustic reflexes were evaluated for the family members. Audiometric analysis was also carried out for the individuals who have already underwent corrective surgery at the time of study. RESULTS: Out of 112 family members, 17 were affected individuals, and 11 of them were surgically confirmed. Hearing loss (HL) started unilaterally and progressed to bilateral form. Otosclerosis was presented in early 20’s in the first and second generations but it was delayed to mid-late 30’s in the fourth generation. An affected female was diagnosed with otosclerosis during her pregnancy. Though the disease was familial, a mother of four affected offspring in this family did not develop otosclerosis until she died at the age of 84. CONCLUSIONS: The five-generation family, which was analysed in the present study, exhibited autosomal dominant inheritance of otosclerosis with reduced penetrance. Bilateral HL and pregnancy-aggravated otosclerosis were observed in this family. It was found for the first time that the age of onset of the disease delayed in the successive generations. The current study indicated the importance of detailed pedigree analysis for better clinical management of otosclerosis.

Influência da retroalimentação auditiva nos parâmetros acústicos vocais de indivíduos sem queixas vocais / Influence of auditory feedback in the vocal acoustic parameters of individuals without vocal complaints

RESUMO Introdução: A qualidade da emissão vocal é dependente da integridade do mecanismo de retroalimentação auditiva. A presença de eventuais falhas nesse mecanismo está relacionada à indução do abuso e/ ou mau uso vocal e, por conseguinte, do surgimento de quadros de disfonia. Objetivo: Avaliar a influência da retroalimentação auditiva na intensidade e na frequência da voz, em indivíduos sem queixas vocais. Métodos: Participaram da pesquisa 40 sujeitos do gênero feminino, sem queixas vocais e com limiares auditivos dentro dos padrões de normalidade. As participantes foram submetidas a uma avaliação auditiva, composta por audiometria tonal liminar, imitanciometria e por uma avaliação acústica vocal dos parâmetros de intensidade e frequência, realizada em três momentos: antes, durante e após a exposição ao ruído branco. Resultados: Houve diferença significativa na relação das médias obtidas na intensidade e entre o limiar dos reflexos acústicos contralaterais e as frequências vocais, nos três momentos da avaliação acústica. Conclusão: Os achados sugerem que a retroalimentação auditiva interfere no controle da intensidade e frequência vocal.

ABSTRACT Introduction: The quality of the vocal emission is dependent on the integrity of the auditory feedback mechanism of the presence of eventual failures is related to the induction of abuse and / or vocal misuse, and therefore of the surgeon of dysphonia. Purpose: To evaluate the influence of auditory feedback in voice intensity and frequency in individuals with no vocal complaints. Methods: Participants were 40 female subjects without vocal and auditory thresholds within normal standards complaints. Participants underwent an auditory evaluation composed of pure tone audiometry, tympanometry and a vocal acoustic assessment of intensity and frequency parameters, carried out in three stages: before, during and after exposure to white noise. Results: There was significant difference in the average of the ratio obtained in intensity and between the threshold of contralateral acoustic reflexes and vocal frequencies in the three moments of acoustic evaluation. Conclusion: Thus, the findings suggest that auditory feedback interferes with the control of the intensity and vocal frequency.

Caracterização do perfil auditivo de frentistas de postos de combustível / Characterization of hearing profile of gas station attendants

RESUMO Introdução Pesquisas voltadas para as populações com baixas exposições a solventes, ou exposições dentro dos níveis de tolerância permitidos em âmbito ocupacional ainda são restritas. Objetivo Caracterizar o perfil auditivo de frentistas de postos de combustíveis. Métodos Estudo transversal, constituído por dois grupos, pareados por gênero e idade: Grupo Controle - 23 indivíduos sem exposição a ruído ou agentes químicos; Grupo Experimental - 21 frentistas de postos de combustíveis. Foi realizada avaliação audiológica, composta por audiometria tonal liminar, logoaudiometria e medidas de imitância acústica. A análise estatística utilizou cálculo de média, desvio padrão, valor mínimo e máximo, teste Qui-quadrado e coeficiente de correlação de Pearson. O nível de significância adotado foi de 5%. Resultados Foram verificadas alterações auditivas nos frentistas, caracterizadas por comprometimento no sistema auditivo periférico, sugerindo ação tóxica da exposição a combustíveis. Houve correlação entre idade e tempo de exposição a solvente. Na comparação entre os grupos, o reflexo acústico demonstrou maior número de alterações no grupo experimental, com diferença para os reflexos acústicos ipsilaterais da orelha direita e contralaterais da orelha esquerda. Conclusão Não houve diferença entre os grupos para os limiares auditivos, porém, a diferença verificada nos reflexos acústicos ipsilaterais e contralaterais no grupo experimental sugere comprometimento retrococlear. Diante das evidências observadas neste estudo, considera-se relevante incluir a pesquisa do reflexo acústico na avaliação auditiva dos frentistas, bem como a integração desta categoria profissional aos programas de prevenção de perda auditiva.

ABSTRACT Introduction Researches into populations with low solvent exposures, or exposures within tolerance levels allowed in the occupational field are still restricted. Purpose To characterize the hearing profile of gas station attendants. Methods Cross-sectional study, constituted of two groups, matched by gender and age: Control Group - 23 subjects without exposure to noise or chemicals; Experimental Group - 21 gas station attendants. An audiological evaluation was performed, composed by pure tone audiometry, speech audiometry and acoustic impedance tests. The statistical analysis used average calculation, standard deviation, minimum and maximum value; Chi-square Test and Pearson’s correlation coefficient. The significance level was 5%. Results Hearing loss was verified in gas station attendants characterized by impairment of the peripheral auditory system, suggesting toxic effects of exposure to fuels. There was a correlation between age and solvent exposure time. Comparing the groups, the acoustic reflex showed more alterations in the experimental group, with a difference for the ipsilateral acoustic reflexes of the right ear and contralateral ones of the left ear. Conclusion There was no difference between the groups for the hearing thresholds; however, the difference observed in the ipsilateral and contralateral acoustic reflexes in the experimental group suggests retrocochlear impairment. Due to the evidence observed in this study, it is considered relevant to include the acoustic reflex research in the auditory evaluation of the gas station attendants, as well as the integration of this professional category into hearing loss prevention programs.

Osteopatia craniana na função auditiva / Cranial osteopathy in hearing function

Osteopatia é a ciência que trata o sistema músculo-ósteo-articular e sua relação com os outros órgãos e sistemas. Poucas são as pesquisa que falam da interação da osteopatia na função auditiva, sendo assim um desafio para a comunidade científica na elaboração de novos trabalhos. O objetivo de nosso estudo foi identificar a influência da osteopatia craniana na função auditiva de indivíduos hígidos. Participaram da pesquisa 17 voluntários que passaram por uma anamnese com o fonoaudiólogo para verificação dos critérios de inclusão e exclusão. Logo após foi realizado o exame de imitanciometria de forma bilateral, em seguida, foi aplicado um protocolo de osteopatia contendo as seguintes manobras: Sutherland contra o movimento, Sutherland a favor do movimento e Ear Pull aplicados na orelha direita e mantendo-se a esquerda como controle. Os dados foram analisados por meio do teste Anova two-way, com post hoc de Bonfferroni e p < 0,05. Foi obtido como resultado a sensibilização dos músculos tensor do tímpano e estapédio evidenciado através da imitanciometria devido a redução do reflexo acústico na frequência auditiva de 500Hz da orelha direita (a frequência média antes foi de 93,53 ± 1,70 e depois foi de 89,41 ± 1,81 (p = 0,0101). Houve uma sensibilização do reflexo com consequente repercussão na função auditiva, de modo que os avaliados foram capazes de disparar o reflexo com níveis sonoros mais baixos. O principal achado da influência da osteopatia na função auditiva a sensibilização dos músculos estapédio e tensor do tímpano. Essa alteração se deu apenas na primeira frequência (500 Hz), frequência principal da fala, no momento inicial da ativação sonora a nível de ouvido médio e sua interface com a cóclea, favorecendo a melhoria da audição. (AU)

Osteopathy is the science which treats the musculoskeletal osteoarticular system and its relationship with other organs and systems. Few researches show interaction of osteopathy in hearing function, and thus is a challenge to the scientific community in developing new works about it. The aim of our study was to identify the influence of cranial osteopathy in hearing function in healthy individuals. Seventeen volunteers were interviewed with the audiologist to check the criteria for inclusion and exclusion. After that was performed bilaterally impedance and the protocol containing the following maneuvers of cranial osteopathy was applied: Sutherland in opposition to the free movement, Sutherland for free movement and ear Pull applied on the right ear and keeping the left as control. Data were analyzed using the Two-way Anova with post hoc Bonfferroni p < 0.05. As a result we obtained the sensitization of tensor tympani and stapedius muscles which was evidenced in the immittancetesting due to a decrease of acoustic reflex in hearing frequency of 500 Hz right ear (the average frequency before was 93.53 ± 1.70 and then was 89.411 ± 1.81 (p = 0.0101). There was a reflex sensitization, with consequent impact on the hearing function, so that the evaluated were capable of firing the reflection with lower sound levels. We can say that we achieved success in our study aims with the primary finding of the influence of hearing function in osteopathy responsiveness of stapedius and tensor tympani. This change occurred only in the first frequency (500 Hz), the speak frequency, at the time of the initial sound activation level of middle ear and its interface with the cochlea, favoring the improvement of hearing. (AU)

Management of children with auditory neuropathy spectrum disorder (ANSD) / Tratamento de crianças com espectro da neuropatia auditiva (ENA)

ABSTRACT INTRODUCTION: ANSD is a challenging problem. OBJECTIVE: To present our experience on management of the children with ANSD with respect to clinical data. METHODS: This retrospective study included all children younger than 16 years of age who applied to the department between 2005 and 2013 (with the exception of newborn hearing screening NHS referrals). The data were derived from pure tone, OAEs and ABR tests, and further medical risk factors of the subjects were evaluated. RESULTS: ANSD was recognized in 74 ears of 40 children (B/U: 34/6) among 1952 children with SNHL (2.04%) detected among 9520 applicants to the department (0.42%). The clinical tests revealed that hearing loss greater than 15 dB was present in both ears of 38 cases. The degree of hearing loss was profound in 48% children, severe in 12% children, moderate in 28% children, mild in 10% children and normal in 5% children. ABRs were absent/abnormal in 37/3 ears and CMs were detected in all. Acoustic reflexes were absent in all ears. Rehabilitation was managed by CI and hearing aids in 15 and 23 cases, respectively. FM system was given to two cases displaying normal hearing but poor speech discrimination in noisy environments. CONCLUSION: ANSD is a relatively challenging problem for the audiology departments because of its various clinical features and difficulties in management. Our patients with ANSD most commonly displayed profound hearing loss. The number of overlooked cases may be minimized by performing ABR and OAE in every case referred with the suspicion of hearing loss.

Resumo Introdução: Espectro da neuropatia auditiva ainda é uma condição clínica desafiadora. Objetivo: Apresentar nossa experiência no tratamento de crianças com espectro da neuropatia auditiva em relação aos dados clínicos. Método: Este estudo retrospectivo incluiu crianças menores de 16 anos de idade que deram entrada no departamento entre 2005 e 2013 (com exceção de encaminhamentos para triagem auditiva neonatal). Foram avaliados os dados obtidos a partir dos exames de audiometria tonal, emissões otoacústicas (EOA), potencial evocado auditivo de tronco encefálico (ABR) e outros fatores de risco. Resultados: Das 1.952 crianças com perda auditiva neurossensorial (2,04%) detectadas dentre os 9.520 candidatos que deram entrada no departamento (0,42%), espectro da neuropatia auditiva foi reconhecida em 74 orelhas de 40 crianças (B/U: 34/6). Os testes clínicos revelaram que uma perda auditiva superior a 15 dB estava presente em ambas as orelhas em 38 casos. O grau de perda auditiva das crianças era profundo em 48%, grave em 12%, moderado em 28%, leve em 10%, e normal em 5%. ABR estava ausente/anormal em 37/3 orelhas e microfonia coclear foi detectado em todas as crianças. Reflexos acústicos estavam ausentes em todas as orelhas. A reabilitação foi tratada com implante coclear e aparelhos auditivos em 15 e 23 casos, respectivamente. Um sistema FM foi utilizado em dois casos que apresentavam audição normal, mas discriminação deficiente da fala em ambientes ruidosos. Conclusão: Espectro da neuropatia auditiva é um problema desafiador para os departamentos de audiologia, devido às suas várias características clínicas e dificuldades no tratamento. Em nossos pacientes a perda auditiva profunda foi a mais frequente. O número de casos negligenciados pode ser diminuído com a realização dos exames ABR e EOA em todos os casos encaminhados com suspeita de perda auditiva.

Auditory Neuropathy/Dyssynchrony in Biotinidase Deficiency

Biotinidase deficiency is a disorder inherited autosomal recessively showing evidence of hearing loss and optic atrophy in addition to seizures, hypotonia, and ataxia. In the present study, a 2-year-old boy with Biotinidase deficiency is presented in which clinical symptoms have been reported with auditory neuropathy/auditory dyssynchrony (AN/AD). In this case, transient-evoked otoacoustic emissions showed bilaterally normal responses representing normal function of outer hair cells. In contrast, acoustic reflex test showed absent reflexes bilaterally, and visual reinforcement audiometry and auditory brainstem responses indicated severe to profound hearing loss in both ears. These results suggest AN/AD in patients with Biotinidase deficiency.

Effect of contralateral stimulation on acoustic reflectance measurements / Efeito da estimulação contralateral nas medidas de reflectância acústica

ABSTRACT INTRODUCTION: Acoustic reflectance is an important tool in the assessment of middle ear afflictions, and the method is considered advantageous in relation to tympanometry. There has been a growing interest in the study of contralateral acoustic stimulation and its effect on the activation of the efferent auditory pathway. Studies have shown that the introduction of simultaneous stimulation in the contralateral ear generates alterations in auditory response patterns. OBJECTIVE: To investigate the influence of contralateral stimulation on acoustic reflectance measurements. METHODS: Case study of 30 subjects with normal hearing, of both genders, aged 18-30 years. The test and retest acoustic reflectance was conducted in the frequency range 200-6000 Hz. The procedure was repeated with the simultaneous presence of contralateral white noise at 30 dBNS. RESULTS: The analysis of the conditions of test, retest, and test with contralateral noise showed statistical difference at the frequency of 2 kHz (p = 0.011 and p = 0.002 in test and retest, respectively) in the right ear. CONCLUSION: The activation of the auditory efferent pathways through contralateral acoustic stimulation produces alterations in response patterns of acoustic reflectance, increasing sound reflection and modifying middle ear acoustical energy transfer.

RESUMO Introdução: A reflectância acústica é citada como uma importante ferramenta na avaliação das afecções da orelha média, sendo um método considerado vantajoso em relação à timpanometria. Tem havido crescente interesse no estudo da estimulação acústica contralateral e seu efeito na ativação da via eferente auditiva. Estudos têm demonstrado que a introdução de estímulo simultâneo na orelha contralateral gera mudanças no padrão de respostas auditivas. Objetivo: Verificar a influência da estimulação contralateral nas medidas de reflectância acústica. Método: Estudo de casos de 30 sujeitos com audição normal, de os gêneros entre 18 a 30 anos. Foi realizado o teste e reteste de reflectância acústica no intervalo de frequência de 200 a 6000 Hz. O procedimento foi repetido com a presença simultânea de ruído branco contralateral à 30 dBNS. Resultados: A análise entre as condições de teste, reteste e teste com ruído contralateral apresentou diferença estatística na frequência de 2 kHz (p = 0,011 em teste e p = 0,002 em reteste) em orelha direita. Conclusão: A ativação da via auditiva eferente por meio da estimulação acústica contralateral produz mudanças nos padrões de respostas da reflectância acústica, aumentando a reflexão do som e, modificando a transferência de energia sonora da orelha média.

High levels of sound pressure: acoustic reflex thresholds and auditory complaints of workers with noise exposure / Níveis elevados de pressão sonora: limiares dos reflexos estapedianos e queixas auditivas de trabalhadores expostos

INTRODUCTION: The clinical evaluation of subjects with occupational noise exposure has been difficult due to the discrepancy between auditory complaints and auditory test results. This study aimed to evaluate the contralateral acoustic reflex thresholds of workers exposed to high levels of noise, and to compare these results to the subjects' auditory complaints.METHODS: This clinical retrospective study evaluated 364 workers between 1998 and 2005; their contralateral acoustic reflexes were compared to auditory complaints, age, and noise exposure time by chi-squared, Fisher's, and Spearman's tests.RESULTS: The workers' age ranged from 18 to 50 years (mean = 39.6), and noise exposure time from one to 38 years (mean = 17.3). We found that 15.1% (55) of the workers had bilateral hearing loss, 38.5% (140) had bilateral tinnitus, 52.8% (192) had abnormal sensitivity to loud sounds, and 47.2% (172) had speech recognition impairment. The variables hearing loss, speech recognition impairment, tinnitus, age group, and noise exposure time did not show relationship with acoustic reflex thresholds; however, all complaints demonstrated a statistically significant relationship with Metz recruitment at 3000 and 4000 Hz bilaterally.CONCLUSION: There was no significance relationship between auditory complaints and acoustic reflexes.

INTRODUÇÃO: A avaliação clínico-ocupacional de trabalhadores expostos a ruído é dificultada pela discrepância entre queixas auditivas e resultados dos exames audiológicos. Este estudo pretende avaliar limiares dos reflexos estapedianos contralaterais em sujeitos expostos a níveis elevados de pressão sonora, relacionando-os com queixas auditivas.MÉTODO: Estudo clínico retrospectivo que analisou 364 trabalhadores e seus limiares de reflexos estapedianos contralaterais, relacionado-os com queixas auditivas, idades e tempos de exposição ao ruído.RESULTADOS: Dos trabalhadores avaliados, com idades de 18 a 50 anos (média 39,6) e tempos de exposição entre um e 38 anos (média 17,3); 15,1% (55) tinham queixa de perda auditiva bilateral, 38,5% (140) zumbidos bilaterais, 52,8% (192) irritação ao ouvir sons intensos e 47,2% (172) dificuldades para reconhecer a fala. As variáveis: perda auditiva, dificuldade para reconhecimento da fala, zumbidos, faixa etária e tempo de exposição ao ruído não se relacionaram significativamente com limiares dos reflexos estapedianos, mas todas as queixas apresentaram relação estatisticamente significante com o recrutamento de Metz nas frequências de 3000 e 4000 Hz, bilateralmente.CONCLUSÃO: Não houve relações significativas entre limiares dos reflexos estapedianos e queixas auditivas.

Acoustic reflex on newborns: the influence of the 226 and 1,000 Hz probes / A influência da sonda de 226 e 1.000 Hz no registro do reflexo acústico em neonatos

PURPOSE: To analyze the occurrence of acoustic reflex and its threshold on newborns using the 226 and 1,000 Hz probes. METHODS: Thirty-six newborns with "PASS" results in newborn hearing screening and tympanogram with one or two peaks for both probe tones were included. Group I comprised 20 full-term newborns without risk indicator for hearing loss, and Group II comprised 16 newborns with at least one risk indicator. The study about ipsilateral acoustic reflex thresholds was conducted in 500, 1,000, 2,000, and 4,000 Hz. RESULTS: The groups presented the acoustic reflex thresholds between 50 and 100 dB for both probe tones. In the comparison between the probes, there were differences in all frequencies evaluated in Group I, with the lowest threshold mean for the 1,000 Hz probe. In Group II, differences were detected at 2,000 Hz. The mean acoustic reflex thresholds were similar in both groups for the 226 Hz probe. There was a difference for the 1,000 Hz probe in all tested frequencies. The percentage of response was higher in both groups for the 1,000 Hz probe. The kappa test showed extremely poor agreement in the comparison of results between both probes. CONCLUSION: The occurrence of acoustic reflex was higher in newborns and its thresholds were lower with the 1,000 Hz probe both for healthy newborns and for newborns at risk. .

OBJETIVO: Verificar a ocorrência e o limiar do reflexo acústico em neonatos utilizando a sonda de 226 e 1.000 Hz. MÉTODOS: Trinta e seis neonatos com resultado "PASS" na triagem auditiva neonatal e timpanogramas com um ou dois picos para os dois tons de sonda. O Grupo I foi composto por 20 neonatos a termo sem indicador de risco para deficiência auditiva e o Grupo II, por 16 neonatos com pelo menos um indicador de risco. A pesquisa dos limiares do reflexo acústico ipsilateral foi realizada em 500, 1.000, 2.000 e 4.000 Hz. RESULTADOS: Os grupos apresentaram limiar do reflexo acústico entre 50 e 100 dB para ambos os tons de sonda. Na comparação entre as sondas, houve diferença em todas as frequências avaliadas no Grupo I, com a média de limiares menor com a sonda de 1.000 Hz. No Grupo II, foi detectada diferença em 2.000 Hz. Com a sonda de 226 Hz, os limiares médios do reflexo acústico foram semelhantes nos dois grupos. Com a sonda de 1.000 Hz, houve diferença em todas as frequências avaliadas. A porcentagem de presença de resposta foi maior para ambos os grupos para a sonda de 1.000 Hz. O teste kappa revelou concordância extremamente pobre na comparação dos resultados entre as duas sondas. CONCLUSÃO: A ocorrência de reflexo acústico em neonatos foi maior e os limiares menores com a utilização da sonda de 1.000 Hz, tanto para neonatos saudáveis como para os neonatos de risco. .

Middle ear impedance studies in elderly patients implications on age-related hearing loss / Estudos de impedância da orelha média em pacientes idosos: implicações na perda auditiva relacionada à idade

INTRODUCTION: Controversies arise with respect to functioning of the middle ear over time. OBJECTIVE: To assess changes in middle ear impedance that may be related to aging, and/or if there was an association of these changes with those of the inner ear in the elderly patients. METHODS: Cross-sectional, comparative study of elderly patients managed in ear, nose and throat clinics. A structured questionnaire was administered to obtain clinical information. Pure tone audiometry, tympanometry, and acoustic reflexes were performed. Comparative analyses were performed to detect intergroup differences between clinico-audiometric findings and middle ear measures, viz. tympanograms and acoustic reflexes. RESULTS: One hundred and three elderly patients participated in the study; 52.4% were male, averagely 70.0 ± 6.3 years old, age-related hearing loss in 59.2%, abnormal tympanograms in 39.3%, absent acoustic reflex in 37.9%. There was no association between age and gender in patients with abnormal tympanograms and absent acoustic reflex. Significantly more patients with different forms and grades of age-related hearing loss had abnormal tympanometry and absent acoustic reflex. CONCLUSION: Some abnormalities were observed in the impedance audiometric measures of elderly patients, which were significantly associated with parameters connected to age-related hearing loss. .

INTRODUÇÃO: Existem controvérsias no que se refere às alterações funcionais da orelha média com o passar dos anos. OBJETIVO: Avaliar as mudanças na impedância da orelha média que podem estar relacionadas ao envelhecimento, bem como qualquer associação dessas alterações com as que ocorrem na orelha interna. MÉTODO: Estudo prospectivo comparativo de pacientes idosos atendidos em ambulatórios espe-cializados em otorrinolaringologia e aplicação de questionário estruturado para obtenção de informações clínicas. Foram realizadas audiometria de tons puros, timpanometria e reflexos acústicos e análise comparativa para detectar as diferenças intergrupos entre os achados clínico-audiométricos. RESULTADOS: Participaram do estudo 103 pacientes idosos: 52,4% do gênero masculino; idade de70 ± 63 anos; perda auditiva relacionada à idade detectada em 59,2%; timpanograma anormal em 39,3%; e reflexo acústico ausente em 37,9%. Não foi encontrada associação entre idade e gênero em pacientes com timpanograma anormal e reflexo acústico ausente. Um número significantemente maior de pacientes com diferentes graus e configurações de perda auditiva relacionada à idade apresentou timpanometria anormal e reflexo acústico ausente. CONCLUSÃO: Algumas anormalidades foram observadas em medidas de impedância audiométrica em pacientes idosos, que foram significantemente associados com os parâmetros ligados à perda auditiva relacionada à idade. .

Evaluation of Hearing and Outer Hair Cell Function of Cochlea in Patients With Psoriatic Arthritis

OBJECTIVES: The aim of this study was to investigate hearing and outer cells function in patients with psoriatic arthritis. Our investigation was a prospective case control study. METHODS: A total of 31 psoriatic arthritis patients (62 ears) and 31 healthy control subjects (62 ears) were enrolled in the study. We investigated hearing changes of patients and controls via pure tone audiometry, speech discrimination scores, tympanometry, acoustic reflex, and transient product otoacoustic emission. RESULTS: The mean age of psoriatic arthritis patients was 36.1+/-8.5 years (range, 14 to 62 years). The average age of the control group was 37.9+/-8.1 years (range, 16 to 62 years). There were statistically significant differences between pure tone audiometry in all frequencies and right and left emission at the 4.0 and 1.0 in psoriatic arthritis patients versus controls (P0.05). Both audiological and otoacoustic emissions were not significantly different between right and left ear (P>0.05). CONCLUSION: Based on the audiological and otoacoustic findings; it is likely that the cochlear outer hair cells become subtly damaged in psoriatic arthritis patients, consequently leading to changes in hearing thresholds. These data suggest that it is important to screen psoriatic arthritis patients for hearing changes with otoacoustic emissions and audiologic tests regularly.

Mass and Stiffness Impact on the Middle Ear and the Cochlear Partition

Mass and stiffness affect on the peculiar characteristics of transmission of the middle ear and the distinctive behavior of the cochlear mechanics. Applying the principle of the mass and stiffness, the band-pass characteristic transfer function of the middle ear has been explained. The greatest transfer function of the middle ear, approximately 24-29 dB, is observed at 1-2 kHz in both cat and human species. However, at lower frequencies, the transfer function was disturbed by the stiffness of the middle ear primarily due to middle ear cavity. At higher frequencies, the transfer function was disturbed by the stiffness of the middle ear primarily due to middle ear bones. Several examples, such as an acoustic reflex, otitis media, and otosclerosis are discussed. For understanding the traveling wave of the basilar membrane, different place tuning at certain stimulus frequencies, contrastingly shaped basilar membrane to the cochlear duct, and the structural and physical characteristics of the whole cochlear partition were reviewed in terms of changing width, mass, and stiffness from the base to apex. Being about ten times wider, more massive, and one hundredfold stiffer at the base than the apex, the nature of the cochlear partition to absorb high-frequency energy changes in fluid pressure declines toward the apex. Consequently, at the base of the cochlea, high frequencies stimuli are decoded while low frequencies stimuli are decoded at the apex of the cochlea. Due to these characteristics of the cochlear partition, the direction of the traveling wave was also proved to be in the fashion of base-to-apex always.

Phylogenic Oto-stomatognathic Connection of the Mammalian Jaw: A Novel Hypothesis for Tensor Tympani Muscle and TMD-related Otologic Symptoms / 체질인류학회지

Otologic complaints, including otalgia, tinnitus, vertigo, and hearing loss, are known to be related to temporomandibular disorders (TMDs). There have been several hypotheses regarding the clinical correlation between otologic complaints and TMDs, based on clinical phenomena with corresponding symptoms, the close neurological relationship between otic and masticatory structures, and anatomical features of the tympanic cavity and jaw joint. Function of the tensor tympani muscle seems to be crucial to understanding TMD-related otologic symptoms. The tensor tympani inserts into the handle of the malleus and it modulates sound transduction in situations of excessive noise. This muscle is innervated by the trigeminal nerve, like the masticatory muscles. Voluntary eardrum movement by pathological tensor tympani contraction results in various otologic symptoms. Thus, co-contraction of the tensor tympani with the masticatory muscle could be a possible cause of TMD-related otologic symptoms. The tensor tympani is rather unrelated to the acoustic reflex, in which the stapedius is strongly involved. The tensor tympani seem to be controlled by proprioceptive information from the trigeminal sensory nucleus. The peripheral innervation pattern of the tensor tympani and masticatory muscles is also supposed to be interconnected. The middle ear structure, including the malleus, incus, and tensor tympani, of mammals had been adapted for acoustic function and lacks the masticatory role seen in non-mammalian jawed vertebrates. The tensor tympani in non-mammals is one of the masticatory muscles and plays a role in the modulation of sound transduction and mastication. After the functional differentiation of the mammalian middle ear, the nervous connection of the tensor tympani with other masticatory apparatus still remains. Through this oto-stomatognathic vestige, the tensor tympani seems to contract unnecessarily in some pathological conditions of the TMD in which the masticatory muscles contract excessively. We hypothesized that the phylogenic relationship between the tensor tympani and masticatory apparatus is a significant and logical reason for TMD-related otologic complaints.

Audiological findings in aphasic patients after stroke / Achados audiológicos em pacientes afásicos após acidente vascular encefálico

Objective To outline the audiological findings of aphasic patients after cerebrovascular accidents. Methods This is a cross-sectional study performed between March 2011 and August 2012 in the Speech, Language, and Hearing Pathology Department of the Universidade Federal de São Paulo. A total of 43 aphasic subjects (27 men) were referred for audiological evaluation after stroke, with mean age of 54.48 years. Basic audiological evaluation tests were performed, including pure tone audiometry, speech audiometry (speech recognition threshold and word recognition score), immittance measures (tympanometry and contralateral acoustic reflex), and transient otoacoustic emissions. Results Sensorineural hearing loss was prevalent (78.6%). Speech recognition threshold and word recognition score were not obtained in some patients because they were unable to perform the task. Hearing loss was a common finding in this population. Conclusion Comprehension and/or oral emission disruptions in aphasic patients after stroke compromised conventional speech audiometry, resulting in the need for changes in the evaluation procedures for these patients. .

Objetivo Avaliar os achados audiológicos em pacientes afásicos após acidente vascular encefálico. Métodos Trata-se de um estudo transversal, realizado entre março de 2011 e agosto de 2012 no Departamento de Fonoaudiologia da Universidade Federal de São Paulo. Participaram do estudo 43 pacientes afásicos (27 homens) após acidente vascular encefálico, com média de idade de 54,48 anos. Foram realizados testes que compõem a bateria da avaliação audiológica básica: audiometria tonal liminar, logoaudiometria (limiar de reconhecimento de fala e índice percentual de reconhecimento de fala), medidas de imitância acústica (timpanometria e pesquisa do reflexo acústico contralateral) e emissões otoacústicas transitórias. Resultados A perda auditiva neurossensorial foi prevalente (78,6%). Não foi possível obter o limiar de reconhecimento de fala e o índice percentual de reconhecimento de fala em todos os pacientes, pois alguns eram incapazes de realizar a tarefa. A perda auditiva foi um achado comum nessa população. Conclusão As alterações de compreensão e/ou emissão oral apresentadas por estes pacientes afetaram a logoaudiometria convencional e apontam para a necessidade de uso de outros procedimentos de avaliação nessa população. .

Effect of caffeine on cervical vestibular-evoked myogenic potential in healthy individuals / O efeito da cafeína no potencial evocado miogênico vestibular cervical em indivíduos saudáveis

INTRODUCTION: Caffeine is the most common psychoactive drug in use around the world and is found at different concentrations in a variety of common food items. Clinically, a strong association between caffeine consumption and diseases of the vestibular system has been established. Cervical vestibular-evoked myogenic potential (cVEMP) is an electrophysiological test that is used to assess the sacculocollic pathway by measuring changes in the vestialibulocollic reflex. AIM: The present study aimed to evaluate the effect of an acute dose of caffeine on the vestibulocollic reflex by using cVEMP. METHOD: A prospective experimental study was performed in which healthy volunteers were submitted to the test before and after the intake of 420 mg of caffeine. The following parameters were compared: p13 and n23 latencies and p13-n23 amplitude. RESULT: No statistically significant difference was found in the test results before and after caffeine use. CONCLUSION: The vestibulocollic reflex is not altered by caffeine intake. .

INTRODUÇÃO: A cafeína é a droga psicoativa mais consumida no mundo e está contida, em diferentes concentrações, em diversos alimentos consumidos no dia a dia. Clinicamente, nota-se um envolvimento importante do seu consumo com as doenças do sistema vestibular. O VEMP cervical é um exame eletrofisiológico que avalia a via sáculo-cólica, determinando alterações no reflexo vestíbulo-cólico. OBJETIVO: O objetivo deste trabalho é avaliar a interferência do uso agudo de cafeína no reflexo vestíbulo-cólico através do cVEMP. MÉTODO: Foi realizado um estudo experimental prospectivo, no qual voluntários saudáveis se submeteram ao exame antes e depois do uso de 420 mg de cafeína, sendo comparados os seguintes parâmetros: latência de p13 e de n23 e interamplitude p13-n23. RESULTADO: Após a comparação dos dados não houve diferença estatisticamente significante entre os exames antes e após o uso da droga. CONCLUSÃO: Não foi observada influência da cafeína no reflexo vestíbulo-cólico. .

Deterioro del reflejo acústico en pacientes con diabetes mellitus tipo I / Acoustics reflex decay in patients with type 1 diabetes mellitus

Introducción: Diversas investigaciones tratan de explicar la asociación entre Diabetes Mellitus (DM) y alteraciones auditivas, relacionándola con el tiempo de evolución, control metabólico, entre otros, encontrando variados resultados. El parámetro menos investigado es el deterioro del reflejo acústico (ARD). Objetivo: Caracterizar el ARD en pacientes con DM tipo 1. Asimismo, relacionar el ARD con el tiempo de evolución de la enfermedad, el grado de control metabólico y la edad. Material y método: Estudio transversal de tipo descriptivo correlacional Se evaluaron 92 oídos en 500 Hz y 99 oídos en 1.000 Hz, con edades entre 4y 68 años, de ambos géneros, con un tiempo de evolución de la enfermedad entre un mes y 44 años. Se realizó un análisis frecuencial del ARD y estadística no paramétrica mediante el coeficiente Rho de Spearman. Resultados: En ambas frecuencias la mayoría no presenta ARD, mientras que 2,2% en 500 Hz y 3,0% en 1.000 Hz presenta ARD patológico. No existiendo correlación entre el ARD con tiempo de evolución de la enfermedad, el grado de control metabólico y la edad de los sujetos. Conclusión: Existe un bajo porcentaje de ARD patológico y una ausencia de correlación con características propias de la enfermedad coincidiendo con otras investigaciones que han utilizado otras herramientas evaluativas.

Introduction: Various studies try to explain the association between diabetes mellitus (DM) and impaired hearing, relating it to the time of evolution, metabolic control, among others, finding mixed results. The parameter under investigation is the acoustic reflex decay (ARD). Aim: To characterize the ARD in patients with type 1 DM. Also relate the ARD with time of evolution of the disease, metabolic control and age. Material and method: Cross-sectional descriptive correlational study. 92 ears were evaluated in 50 0Hz and 99 ears in 1000 Hz, with ages between 4 and 68 years, of both genders, with a time of evolution of the disease from one month to 44 years. Frequency analysis of the ARD were performed and nonparametric statistics by Spearman's Rho coefficient. Results: In both frequencies most ears do not have ARD, while 2.2% in 500 Hz and 3.0% in 1000 Hz showed ARD pathological features. No correlation exists between the ARD with time of evolution of the disease, metabolic control and age of subjects. Conclusion: There is a low percentage of ARD pathological and lack of correlation with disease characteristics, coinciding with other studies that have used differents assessment tools.