Cortical Deafness and Tinnitus Following Sequential Bilateral Putaminal Hemorrhage

No abstract available.

Ensino por múltiplos exemplares e integração de comportamentos de ouvinte e falante com unidades sintáticas substantivo-adjetivo em crianças com DENA e IC / Multiple exemplar instruction and integration of listening and speaking behaviors with substantive-adjective syntactic units in children with ANSD and CI

RESUMO Objetivo Verificar o efeito do ensino por múltiplos exemplares na aquisição e integração dos comportamentos de ouvinte e falante, com substantivos e combinações substantivo-adjetivo, em crianças com Desordem do Espectro da Neuropatia Auditiva (DENA) e implante coclear (IC). Método Participaram duas crianças com DENA que usavam IC. Foram adotados estímulos ditados e figuras que correspondiam a palavras (substantivo) e unidades sintáticas substantivo+adjetivo. O estudo foi organizado em passos de ensino que foram intercalados por avaliações dos comportamentos de ouvinte e falante, com todos os estímulos. O ensino por múltiplos exemplares apresentou tarefas de imitação oral (ecoico), reconhecimento auditivo (ouvinte) e nomeação de figuras (tato) de maneira rotativa; os substantivos foram ensinados primeiro e, em seguida, as combinações substantivo-adjetivo. Resultados No pré-teste, os participantes mostraram variabilidade e discrepância nas porcentagens de acertos de ouvinte e de falante. Todos alcançaram primeiro 100% de acertos nas tarefas de ouvinte e os desempenhos de falante ficaram próximos aos de ouvinte após o ensino. Todos estenderam a aprendizagem dos substantivos para as unidades sintáticas substantivo-adjetivo. Conclusão Crianças com DENA e IC podem aprender e integrar comportamentos de ouvinte e de falante por meio do ensino por múltiplos exemplares, de palavras a unidades sintáticas.

ABSTRACT Purpose To verify the effect of the multiple exemplar instruction at the acquisition and integration of listening and speaking behaviors, with substantives and substantive-adjective combinations, in children with Auditory Neuropathy Spectrum Disorder (ANSD) and cochlear implant (CI). Methods Participants were two children with ANSD that were users of CI. We adopted dictated stimulus and pictures that corresponded to words (substantive) and substantive-adjective syntactic units. The study was arranged in teaching steps that were intercalated with listening and speaking behaviors probes, with all stimuli. The multiple exemplar instruction presented oral imitation (echoic), auditory recognition (listening) and pictures naming (touch) tasks, on a rotating way; the substantives were taught first and, after that, the substantive-adjective combinations. Results In the pre-test, the participants showed variability and discrepancy in the correct responses percentages of listening and speaking. All achieved firstly 100% correct responses in the listening task and the speaking performances were close to listening after the teaching. All extended substantive learning to substantive-adjective syntactic units. Conclusion Children with ANSD and CI can learn and integrate listening and speaking behaviors by multiple exemplar instruction, from words to syntactic units.

The importance of central auditory evaluation in Friedreich's ataxia / A importância da avaliação auditiva central na ataxia de Friedreich

ABSTRACT Objective To assess central auditory function in Friedreich's ataxia. Methods A cross-sectional, retrospective study was carried out. Thirty patients underwent the anamnesis, otorhinolaryngology examination, pure tone audiometry, acoustic immittance measures and brainstem auditory evoked potential (BAEP) assessments. Results The observed alterations were: 43.3% in the pure tone audiometry, bilateral in 36.7%; 56.6% in the BAEP test, bilateral in 50%; and 46.6% in the acoustic immittance test. There was a significant difference (p < 0.05) in the comparison between the tests performed. Conclusion In the audiological screening, there was a prevalence of the descending audiometric configuration at the frequency of 4kHz, and absence of the acoustic reflex at the same frequency. In the BAEP test, there was a prevalence of an increase of the latencies in waves I, III and V, and in the intervals of interpeaks I-III, I-V and III-V. In 13.3% of the patients, wave V was absent, and all waves were absent in 3.3% of patients.

RESUMO Objetivo Avaliar a função auditiva central na ataxia de Friedreich (AFRD). Métodos Foi realizado um estudo retrospectivo de corte transversal. 30 pacientes realizaram anamnese, avaliações otorrinolaringológica, audiológica, imitanciométrica e do potencial evocado auditivo de tronco encefálico (PEATE). Resultados As alterações observadas foram: 43,3% no exame audiométrico sendo 36,7% dos casos, bilateralmente; 56,6% na avaliação do PEATE com 50% dos casos, bilateralmente e 46,6% no exame imitanciométrico. Houve diferença significativa (p < 0,05) na comparação entre os exames realizados. Conclusão No exame audiológico, ocorreu uma preponderância maior da configuração audiométrica descendente a partir da freqüência de 4kHz e ausência do reflexo acústico na mesma frequência. No exame do PEATE, houve prevalência do aumento das latências nas ondas I, III e V, e nos intervalos dos interpicos I-III, I-V e III-V. Em 13,3% dos casos, a onda V estava ausente, e em 3,3% dos casos, todas as ondas estavam ausentes.

A Case of Auditory Neuropathy Caused by Pontine Hemorrhage in an Adult

A pontine hemorrhage can evoke several neurological symptoms because the pons contains various nuclei and nerve fibers. Hearing loss can develop as a result of a pontine hemorrhage because there is an auditory conduction pathway in the cochlear nucleus of the pons. However, very few cases of hearing loss caused by pontine lesions have been reported, and there have been no reports of auditory neuropathy that developed following a pontine hemorrhage. Recently we had a patient who experienced a nontraumatic pontine hemorrhage who was diagnosed with auditory neuropathy. The 34-year-old male patient was admitted to the emergency department with sudden alteration of mental status. His brain computed tomographic imaging revealed a hemorrhage in the central pons. He complained of hearing difficulties after his mental status recovered through conservative treatment, but a pure-tone audiogram showed very mild hearing loss in both ears. Further hearing tests using otoacoustic emissions, which showed normal responses, and auditory brainstem responses, which showed no waveforms at maximum stimulus intensity, revealed that his hearing difficulties were caused by auditory neuropathy. This case implies that the threshold of sound detection can be preserved in patients with pontine hemorrhage who complain of hearing difficulties. Auditory neuropathy should be considered as a possible cause of hearing difficulties in these patients and appropriate hearing tests should be performed.

Study of cochlear microphonic potentials in auditory neuropathy / Estudo do microfonismo coclear na neuropatia auditiva

Abstract Introduction: Auditory Neuropathy/Dyssynchrony is a disorder characterized by the presence of Otoacoustic Emissions and Cochlear Microphonic Potentials, an absence or severe alteration of Brainstem Evoked Auditory Potential, auditory thresholds incompatible with speech thresholds and altered acoustic reflexes. The study of the Cochlear Microphonic Potential appears to be the most important tool for an accurate diagnosis of this pathology. Objective: Determine the characteristics of the Cochlear Microphonic in Auditory Neuropathy/Dyssynchrony using an integrative review. Methods: Bibliographic survey of Pubmed and Bireme platforms and MedLine, LILACS and SciELO data banks, with standardized searches up to July 2014, using keywords. Criteria were established for the selection and assessment of the scientific studies surveyed, considering the following aspects: author, year/place, degree of recommendation/level of scientific evidence, objective, sample, age range, mean age, tests, results and conclusion. Results: Of the 1959 articles found, 1914 were excluded for the title, 20 for the abstract, 9 for the text of the article, 2 for being repeated and 14 were selected for the study. Conclusion: The presence of the Cochlear Microphonic is a determining finding in the differential diagnosis of Auditory Neuropathy/Dyssynchrony. The protocol for the determination of Cochlear Microphonic must include the use of insert earphones, reverse polarity and blocking the stimulus tube to eliminate electrical artifact interference. The amplitude of the Cochlear Microphonic in Auditory Neuropathy/Dyssynchrony shows no significant difference from that of normal individuals. The duration of the Cochlear Microphonic is longer in individuals with Auditory Neuropathy/Dyssynchrony.

Resumo Introdução: A Neuropatia/Dessincronia Auditiva é uma doença caracterizada pela presença das Emissões Otoacústicas e do Microfonismo Coclear, com ausência ou grave alteração do Potencial Evocado Auditivo de Tronco Encefálico, limiares auditivos incompatíveis com limiares vocais e reflexos acústicos alterados. O estudo do Microfonismo Coclear parece ser a ferramenta mais importante para um diagnóstico preciso desta patologia. Objetivo: Verificar por meio de uma revisão integrativa as características do Microfonismo Coclear na Neuropatia/Dessincronia Auditiva. Método: Levantamento bibliográfico nas plataformas Pubmed e Bireme e nas bases de dados MedLine, LILACS e SciELO, com buscas padronizadas até julho de 2014, utilizando-se palavraschave. Para a seleção e avaliação dos estudos científicos levantados, foram estabelecidos critérios, contemplando os aspectos: autor, ano/local, grau de recomendação/nível de evidência científica, objetivo, amostra, faixa etária, média de idade em anos, testes, resultados e conclusão. Resultados: Dos 1959 artigos encontrados, 1914 foram excluídos pelo título, 20 pelo resumo, nove pela leitura do artigo, dois eram repetidos e 14 foram selecionados para o estudo. Conclusão: A presença do Microfonismo Coclear é um achado determinante no diagnóstico diferencial da Neuropatia/Dessincronia auditiva. O protocolo de registro do Microfonismo Coclear deve contar com o uso de fones de inserção, a inversão da polaridade e o bloqueio do tubo do estímulo para impedir a interferência de artefato elétrico. A amplitude do Microfonismo Coclear na Neuropatia/Dessincronia auditiva não apresenta diferença significante entre a amplitude do Microfonismo Coclear em ouvintes normais. A duração do Microfonismo Coclear é maior em indivíduos com Neuropatia/Dessincronia auditiva.

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.

Percepção de fala em deficientes auditivos pré-linguais com desordem do espectro da neuropatia auditiva usuários de aparelho auditivo de amplificação sonora / Speech perception in users of hearing aid with auditory neuropathy spectrum disorder

RESUMO Objetivo: Analisar a percepção de fala em crianças portadoras de deficiência auditiva pré-lingual com desordem do espectro da neuropatia auditiva (DENA) usuárias de aparelho de amplificação sonora individual (AASI) bilateral. Métodos: Trata-se de um estudo descritivo e exploratório realizado no Centro de Pesquisas Audiológicas da Sessão de Implante Coclear do Hospital de Reabilitação de Anomalias Craniofaciais (HRAC/USP). Foram avaliadas 4 crianças com idade variando entre 8 anos e 3 meses e 12 anos e 2 meses. Foram utilizados: listas de palavras monossílabas, dissílabas, palavras sem sentido e sentenças, Escala de Integração Auditiva Significativa para Crianças Pequenas (IT-MAIS) e Questionário de Avaliação da Linguagem Oral (MUSS), categorias de linguagem e audição. Todas as listas foram aplicadas em cabine acústica, à viva-voz, em campo livre, no silêncio. Resultados: Os resultados apresentaram média de 69,5% para a lista de palavras monossílabas, 87,75% para a lista de palavras dissílabas, 89,92% para a lista de sílabas sem sentido e 92,5% para a lista de sentenças. Conclusão: O processo terapêutico aplicado, que incluiu o uso do AASI bilateral, foi extremamente satisfatório, uma vez que possibilitou o desenvolvimento máximo das habilidades auditivas.

ABSTRACT Purpose: To analyze speech perception in children with pre-lingual hearing loss with auditory neuropathy spectrum disorder users of bilateral hearing aid. Methods: This is a descriptive and exploratory study carried out at the Research Center Audiological (HRAC/USP). The study included four children aged between 8 years and 3 months and 12 years and 2 months. Lists of monosyllabic words, two syllables, nonsense words and sentences, the Infant Toddler-Meaningful Auditory Integration Scale (IT-MAIS) and the Meaningful Use of Speech Scale (MUSS), hearing, and language categories were used. All lists were applied in acoustic booth, with speakers, in free field, in silence. Results: The results showed an average 69.5% for the list of monosyllabic words, 87.75% for the list of two-syllable words, 89.92% for the list of nonsense syllables, and 92.5% for the list of sentences. Conclusion: The therapeutic process that includes the use of bilateral hearing aid was extremely satisfactory, since it allowed the maximum development of auditory skills.

Molecular approach of auditory neuropathy / Abordagem molecular da neuropatia auditiva

INTRODUCTION: Mutations in the otoferlin gene are responsible for auditory neuropathy. OBJECTIVE: To investigate the prevalence of mutations in the mutations in the otoferlin gene in patients with and without auditory neuropathy. METHODS: This original cross-sectional case study evaluated 16 index cases with auditory neuropathy, 13 patients with sensorineural hearing loss, and 20 normal-hearing subjects. DNA was extracted from peripheral blood leukocytes, and the mutations in the otoferlin gene sites were amplified by polymerase chain reaction/restriction fragment length polymorphism. RESULTS: The 16 index cases included nine (56%) females and seven (44%) males. The 13 deaf patients comprised seven (54%) males and six (46%) females. Among the 20 normal-hearing subjects, 13 (65%) were males and seven were (35%) females. Thirteen (81%) index cases had wild-type genotype (AA) and three (19%) had the heterozygous AG genotype for IVS8-2A-G (intron 8) mutation. The 5473C-G (exon 44) mutation was found in a heterozygous state (CG) in seven (44%) index cases and nine (56%) had the wild-type allele (CC). Of these mutants, two (25%) were compound heterozygotes for the mutations found in intron 8 and exon 44. All patients with sensorineural hearing loss and normal-hearing individuals did not have mutations (100%). CONCLUSION: There are differences at the molecular level in patients with and without auditory neuropathy. .

INTRODUÇÃO: Mutações no gene da otoferlina (OTOF) são responsáveis pela neuropatia auditiva. OBJETIVO: Investigar a prevalência de mutações no gene OTOF em pacientes com e sem neuropatia auditiva. MÉTODO: Estudo de casos em corte transversal sendo avaliados 16 casos índice com neuropatia auditiva, 13 pacientes com deficiência auditiva sensorioneural (DASN) e 20 indivíduos ouvintes. DNA foi extraído de leucócitos do sangue periférico e regiões do gene OTOF foram analisadas pela técnica PCR-RFLP. RESULTADOS: Dos 16 casos índice, 9 (56%) são do gênero feminino e 7 (44%) do masculino. Dos 13 pacientes com DASN, 7 (54%) são masculinos e 6 (46%) femininos. Dos 20 ouvintes, 13 (65%) são masculinos e 7 (35%) femininos. Treze (81%) casos índice apresentam o genótipo selvagem (AA) e 3 (19%) o genótipo heterozigoto AG para a mutação IVS8-2A-G (intron 8). A mutação 5473C-G (exon 44) foi encontrada em heterozigose (CG) em 7 (44%) dos casos índice e 9 (56%) apresentam o genótipo selvagem (CC). Destes mutantes, dois (25%) são heterozigotos compostos para as mutações encontradas no intron 8 e exon 44. Os pacientes com DASN e os ouvintes não apresentam mutações (100%). CONCLUSÃO: Existem diferenças, ao nível molecular, em pacientes com e sem neuropatia audi tiva. .

Performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implant: a systematic review, / Resultados do desempenho das habilidades auditivas em crianças com o espectro da neuropatia auditiva usuárias de implante coclear: revisão sistemática

Introduction: Currently, there are no doubts about the benefits of cochlear implants for the development of children with severe or profound hearing loss. However, there is still no consensus among researchers and professionals regarding the benefits for the improvement of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implants. Objective: Review the available evidence in the literature to answer the following: "What is the performance of hearing skills in children with auditory neuropathy spectrum disorder using cochlear implants?" Methods: Systematic review of the literature through electronic database consultation, considering publications in the period 2002-2013. Results: Twenty-two studies met the criteria and were included in the systematic review. Conclusion: The analyzed studies demonstrated that after cochlear implant surgery, individuals with auditory neuropathy spectrum disorder improved their performance of hearing skills and had similar performance to that of children with sensorineural hearing loss using cochlear implant. .

Introdução: Atualmente não restam dúvidas quanto aos benefícios do uso do implante coclear no desenvolvimento da população infantil com perda auditiva de grau severo e/ou profundo. Entretanto, ainda não há um consenso entre pesquisadores e profissionais sobre os seus benefícios para a melhora das habilidades auditivas em crianças com o espectro da neuropatia auditiva usuárias de implante coclear. Objetivo: Revisar a evidência disponível na literatura para responder ao questionamento: "Quais os resultados do desempenho das habilidades auditivas em crianças com o espectro da neuropatia auditiva usuárias de implante coclear?" Método: Revisão sistemática da literatura, a partir da consulta de bases de dados eletrônicas, considerando publicações no período de 2002 a 2013. Resultados: Vinte e dois estudos contemplaram os critérios e foram incluídos na revisão sistemática. Conclusão: Os estudos analisados demonstraram que, após a cirurgia de IC, os indivíduos como espectro da neuropatia auditiva melhoraram o desempenho das habilidades auditivas e apresentaram desempenho semelhante ao de crianças com perda auditiva sensorioneural usuárias de implante coclear. .

Lexical tone perception in sensorineural hearing-impaired and auditory neuropathy spectrum disorder / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#This study was aimed at investigating the ability of lexical tone perception in listeners with sensorineural hearing impairment and auditory. neuropathy spectrum disorder.@*METHOD@#Three groups of subjects were recruited in this study, including 11 subjects with normal hearing, 14 subjects with sensorineural hearing impairment, and 25 subjects with auditory neuropathy spectrum disorder. Ten monosyllabic syllables were selected, and combined with four lexical tones which were made up of 40 tone tokens as the original test materials. Then, these original words were recorded using one adult male and one adult female native Beijing Mandarin speaker. The speakers were asked to record these 40 monosyllabic words multiple times, and the 80 tokens in which the durations of four tones in each monosyllabic word were within 5ms precision were chosen as the test tone tokens. The subjects were asked to perform a four-alternative forced-choice study and select which tone they had heard.@*RESULT@#The mean and standard deviation of the tone perception correct scores for normal-hearing subjects, subjects with sensorineural hearing impairment, and subjects with auditory neuropathy spectrum disorder were (97.3 ± 2.8)%, (88.0 ± 9.9)%, and (65.7 ± 17.1)%, respectively. Significant differences in tone perception scores were found to be between subjects with normal hearing and subjects with sensorineural hearing impairment (P < 0.01) and between subjects with auditory neuropathy spectrum disorder (P < 0.01). In addition, a significant difference was found to be between subjects with sensorineural hearing impairment and auditory neuropathy spectrum disorder (P < 0.05). A significantly negative correlation was observed between tone perception score and pure tone hearing thresholds for both subjects with sensorineural hearing loss (r = -0.756, P < 0.01) and subjects with auditory neuropathy spectrum disorder (r = -0.546, P < 0.01).@*CONCLUSION@#As the hearing loss became more severe, the ability to perceive lexical tone for both subjects with sensorineural hearing loss and auditory neuropathy spectrum disorder reduced. Subjects with auditory neuropathy spectrum disorder had more degraded ability to perceive lexical tone and a larger individual difference, in comparison with subjects with sensorineural hearing loss.

O uso do P300 com estímulo de fala para monitoramento do treinamento auditivo / The use of P300 with speech stimulus for monitoring the auditory training / Uso del P300 con estímulo de habla para monitoramento de la intervencion auditiva

Introdução: Uma vez confirmado o diagnóstico do Transtorno do Processamento Auditivo torna-se necessária realização do treinamento auditivo, para trabalhar as habilidades auditivas que se encontram alteradas. O fundamento chave para o treinamento auditivo está embasado na plasticidade neuronal e nas mudanças benéficas no comportamento auditivo e no sistema nervoso auditivo central. Tais mudanças podem ser monitoradas por meio de testes eletrofisiológicos, como o potencial cognitivo P300. Objetivo: Avaliar a efetividade do treinamento auditivo em crianças com o diagnóstico de transtorno do processamento auditivo, por meio do P300 com estímulo de fala. Métodos: Realizada a avaliação do P300 em 10 indivíduos, com idades entre sete e 14 anos, de ambos os gêneros, antes e após o treinamento auditivo. O programa de intervenção foi composto por nove sessões individuais. Todos os participantes foram submetidos ao P300 com estímulos acústicos binaurais (sílabas ? BA e GA) e intensidade de 75 dB NA. Foram utilizados 500 estímulos, dentre os quais 80% foram requentes (sílaba BA) e 20% foram raros (sílaba GA). Os estímulos raros e frequentes foram apresentados de forma aleatória (paradigma oddball). Resultados: Foi observada redução da latência e aumento da amplitude do P300 no instante pós-treinamento auditivo, de forma estatisticamente significante. Conclusão: Considerando as mudanças verificadas em relação à latência e amplitude do P300, eliciado com estímulo de fala após o programa terapêutico proposto, é possível concluir que este teste eletrofisiológico é uma ferramenta objetiva sensível para monitorar a efetividade do treinamento auditivo.

Introduction: Once confirmed the diagnosis of Auditory Processing Disorder, it is necessary to provide auditory training to improve the auditory skills. The basis for auditory training is based on neuronal plasticity and beneficial changes in auditory behavior and the central auditory pathways. These changes can be monitored by electrophysiological tests, as the the long latency auditory evoked potentials (P300). Objective: To assess the effectiveness of auditory training in children with auditory processing disorder through P300 with speech stimuli. Methods: The P300 was performed in 10 subjects, aged betweenseven and 14 years (both genders) of Clinical Speech Pathology, University of Guarulhos, before and after auditory training. The intervention program consisted of nine individual sessions. All participants underwent the P300. The test was performed with 500 binaural acoustic stimuli (syllables /BA/ for high- probability sounds and /GA/ for low-probability sounds), at 75 dB HL. The rare and frequent stimuli were presented in random order (oddball paradigm). Results: After the auditory training it was observed an improvement in the results of P300, by a statistically significant latency reduction and increased amplitude of the P300 at the instant post-auditory training. Conclusion: P300 elicited by speech was considered a sensitive tool to monitor the effectiveness of auditory training.

Introducción: Una vez confirmado el diagnóstico de Trastorno del Procesamiento Auditivo se hace necesaria la realización del entrenamiento auditivo, para trabajar las habilidades auditivas que se encuentran alteradas. El fundamento llave para el entrenamiento auditivo está embasado en la plasticidad neuronal y en los cambios benéficos para el comportamiento auditivo y el sistema nervioso auditivo central. Tales cambios pueden ser monitoreados por medio de pruebas eletrofisiológicas, como el potencial cognitivo P300. Objetivo: Evaluar la efectividad del entrenamiento auditivo en niños con el diagnóstico de trastorno del procesamiento auditivo, por medio del lo P300 con estímulo de habla. Métodos: Realizar la evaluación del P300 en 10 individuos, con edades entre los siete y 14 años, de ambos géneros, antes y después del entrenamiento auditivo. El programa de intervención fue compuesto por nueve sesiones individuales. Todos los participantes fueron sometidos al P300 con estímulos acústicos binaurales (sílabas ? BA y GA), a una intensidad de 75 dBNA. Fueron utilizados 500 estímulos entre los cuales 80% fueron frecuentes (sílaba BA) y 20% fueron raros (sílaba GA). Los estímulos raros y frecuentes fueron presentados de forma aleatoria (paradigma oddball). Resultados: Fue observada reducción de la latencia y aumento de la amplitud del lo P300 en el instante post-entrenamiento auditivo, de forma estadísticamente significante. Conclusión: Considerando los cambios verificados en la latencia y amplitud del P300, suscitados con estímulo de habla después del programa terapéutico propuesto, es posible concluir que esta prueba eletrofisiológica es una herramienta objetiva sensible para monitorear la efectividad del entrenamiento auditivo.

Early diagnosis and intervention in 0-9 months old infants with hearing loss / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the current situation of early diagnosis and intervention in 0-9 months old infants with hearing loss and analysis factors that will affect early diagnosis and intervention.@*METHOD@#One hundred and eighty-six infants referred to the West China hospital from February 2014 to September 2014 were included. All 186 children were referred due to the fact that either they failed infant hearing screening or outer ear malformation. Early diagnosis and/or intervention were performed on those 186 children and their records of early diagnosis and intervention were analyzed.@*RESULT@#Among the 186 infants, 167 (89.8%) were diagnosed with an average age at (4.0 ± 1.4) months. Among the 167 infants with final diagnosis, there were 31 (18.6%) infants diagnosed as conductive hearing loss (CHL), and 99 cases (59.3%) diagnosed as sensorineural hearing loss (SNHL), among whom, there were 75 (44.9%) bilateral SNHL and 24 (14.4%) unilateral SNHL. There were 2 cases (1.20%) with SNHL on one side and atresia on the other side. 5 (2.99%) of all conductive hearing loss cases with unilateral atresia and 2 cases with auditory neuropathy (AN) were found. 33 infants (19.8%) were found to have normal hearing. 30.7% (23/75) infants diagnosed as bilateral SNHL and 8.3% (2/24) infants diagnosed as unilateral SNHL were fitted with hearing aids. The fitting rate in infants with bilateral SNHL with mild, moderate, severe to profound degrees were 0 (0/23), 24.0% (6/25), 66.7% (6/9), 61.1% (11/18) respectively. The average intervention age was (5.0 ± 2.1) months.@*CONCLUSION@#Although the early diagnosis and intervention situation in this study are very close to international standard, there are still infants without final diagnosis and infants with hearing loss without hearing aid fitting. Further studies and efforts to promote early diagnosis and intervention in infants with hearing loss are needed.

The curative effect analysis of combined medical therapy for auditory neuropathy spectrum disorder / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the efficacy of combined medical treatment on auditory neuropathy spectrum disorder and the effect of related factors on the prognosis.@*METHOD@#Eleven cases (22 ears) diagnosed as auditory neuropathy spectrum disorder using multiple criteria including pure tone auditory threshold, impedance audiometry, acoustic reflexes, distortion products otoacoustic emission (DPOAE) and auditory brainstem response (ABR) were subjected to combined medical treatment . Eleven auditory neuropathy spectrum disorder patients diagnosed during the corresponding period but refused treatment were selected as control group. The change of pure tone auditory threshold and speech discrimination score after treatment or follow-up were evaluated for both 2 groups, and the relationship between the patients' gender, age, accompanying symptoms and curative effect were also analyzed. Data were analyzed by SPSS 19.0 statistical software using pared-sample t-test, independent-sample test and Pearson's chi-square test.@*RESULT@#The effective rate of combined medical therapy was 59.09% (13/22) in the therapy group. PTA levels before and after-treatment were (53.92 +/- 18.86) dB HL and (47.44 +/- 14.98) dB HL respectively in 22 ears with the combined medical therapy, the improvement of which showed statistically significance (t = 5.20, P 0.05). Speech discrimination score before and after-treatment were (29.20 +/- 25.80)% and (41.60 +/- 22.90)% respectively for the treatment group. The average improvement of speech discrimination score was (12.40 +/- 13.80)% with statistically significant difference (t = 4.02, P 0.05).@*CONCLUSION@#The combined medical therapy with glucocorticoids helps improving the pure tone auditory threshold and speech discrimination score of auditory neuropathy spectrum disorder. In light of our findings we support the combined medical therapy as an option for patients with auditory neuropathy spectrum disorder.

Analysis of characteristics of tinnitus in patients with auditory neuropathy spectrum disorder / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To investigate the characteristics of tinnitus in patients with auditory neuropathy spec- trum disorder (ANSD).@*METHOD@#This study recruited 14 ANSD patients with tinnitus. All the ANSD patients un- derwent detailed history taking, audiological examinations and assessments of tinnitus. This study analyzed the correlation of tinnitus status and hearing loss, and discussed the effects of sex, age, and the course of disease on tinnitus in ANSD patients.@*RESULT@#(1) In the ANSD patients, tinnitus often occurred in 3 years after the onset of hearing loss; (2) Tinnitus was highly prevalent in ANSD patients, and the severity of tinnitus was mostly from mild to moderate; (3) There was no obvious correlation between the subjective grading of tinnitus and hearing loss de- gree, and the impact of curve patterns of hearing loss on the level of tinnitus need much more evidence-based proof; (4) Along with the course extension, the impact of tinnitus on the quality of life was much more obvious; (5) Some risk factors such as noise exposure could be the reasons of aggravating the degree of tinnitus.@*CONCLUSION@#Tinnitus in ANSD patients has its unique clinical features. The study of Tinnitus in ANSD patients can provide clinical basis for further research in ANSD.

Audiological evaluation in Chinese patients with mitochondrial encephalomyopathies / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Hearing impairment has been reported to be common in patients with mitochondrial disorders, a group of diseases characterized by pleiomorphic clinical manifestations due to defects in oxidative phosphorylation of mitochondria. This study aimed to investigate the audiological characteristics in a large cohort of patients with mitochondrial disease.</p><p><b>METHODS</b>Comprehensive audiological evaluations, including pure tone audiometry, tympanometry, speech audiometry, otoacoustic emissions, electrocochleography and auditory brainstem evoked potentials, were performed in 73 Chinese patients with mitochondrial encephalomyopathy and with confirmed mitochondrial DNA (mtDNA) defects.</p><p><b>RESULTS</b>Among the patients, 71% had hearing impairment. However, the incidence rate and severity of hearing impairment were much less in the chronic progressive external ophthalmoplegia (CPEO) subtype than in the mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), myoclonic epilepsy with ragged red fibers (MERRF) and Kearns-Sayre syndrome (KSS) subtypes. While most of our patients had a predominantly cochlea origin for the hearing deficit, five patients had an auditory neuropathy spectrum disorder and three patients had impairment of both cochlea and auditory cortex.</p><p><b>CONCLUSIONS</b>Various portions of the auditory system could be involved in patients with mitochondrial diseases, including cochlea, auditory nerve, auditory pathway and cortex. Hearing loss was more associated with multisystem involvement. Genotype, mutant load of mtDNA and other unknown factors could contribute to heterogeneity of hearing impairment in mitochondrial disease.</p>

Auditory Late Responses in Asperger Syndrome: two case study

INTRODUCTION: Auditory Late Responses (ALR) assess central auditory processing by neuroelectric activity of the auditory pathway and analyse the activities involved in cortical abilities of discrimination, attention and integration of the brain. Individuals withAsperger Syndromeexperience changesinthese skills, so it is importanttoresearchthesepotentialthis population. The objective of this paper was to describe the auditory late responses of two patients with Asperger Syndrome. METHODS: The study included two male patients with Asperger Syndrome, of 7 and 12 years of age, treated in a study centre. The patients did not present any auditory complaint detected by anamnesis. The external auditory canal was inspected and audiological and auditory late responses assessed. After evaluation the components P2, N2 and P3 were analysed. RESULTS: In both patients, the latency of the components P2, N2 and P3 were elongated in both ears. Regarding the amplitude of the P2 component, reduced values were found for the left ear of patient 1 and the right ear of patient 2. The N2 amplitude was reduced for both ears of patient 1 and only the right ear of patient 2. The two patients showed a decrease in the amplitude of the P3 only in the right ear. CONCLUSION: This study concludes that there were changes in the ALR results in both patients with Asperger Syndrome, suggesting alteration of the auditory function at the cortex level...

INTRODUÇÃO: Os Potenciais Evocados Auditivos de Longa Latência avaliam o processamento auditivo central através da atividade neuroelétrica da via auditiva, analisando as atividades corticais envolvidas nas habilidades de discriminação, integração e atenção do cérebro. Indivíduos com Síndrome de Asperger apresentam alterações em tais habilidades, sendo importante a investigação desses potenciais nessa população. O objetivo do trabalho foi descrever os achados dos Potenciais Evocados Auditivos de Longa Latência de dois pacientes com Síndrome de Asperger. MÉTODO: Participaram do estudo dois pacientes com Síndrome de Asperger do sexo masculino, com idade de 7 e 12 anos, atendidos em um centro de estudos, isentos de alterações auditivas, detectadas por meio de anamnese, inspeção do conduto auditivo externo e avaliação audiológica. Foi aplicado o potencial evocado auditivo de longa latência e analisado os componentes P2, N2 e P3. RESULTADOS: A latência dos componentes P2, N2 e P3 mostrou-se alongada nas duas orelhas nos dois pacientes. Quanto à amplitude, o componente P2 estava com valores reduzidos apenas para a orelha esquerda do paciente 1 e direita do paciente 2. O N2 estava com amplitude reduzida nas duas orelhas do paciente 1 e apenas na orelha direita do paciente 2, e o P3 apresentou diminuição de amplitude apenas na orelha direita nos dois pacientes. CONCLUSÃO: Pode-se concluir que há alteração no funcionamento da informação auditiva ao nível do córtex nas duas crianças com Síndrome de Asperger atendidas neste centro...

Prevalência do espectro da neuropatia auditiva em um serviço de saúde auditiva / Prevalence of auditory neuropathy spectrum disorder in an auditory health care service

Oespectro da neuropatia auditiva (ENA) caracteriza-se pelo acometimento do nervo auditivo, associado à preservação da função das células ciliadas externas cocleares. OBJETIVO: Determinar a prevalência do ENA em sujeitos com perda auditiva neurossensorial (PANS). MÉTODO: Estudo retrospectivo realizado de 2010 a 2012, pela análise dos prontuários de 2.292 sujeitos com PANS. Foram coletados dados das avaliações otorrinolaringológica e audiológica, por meio da audiometria tonal e vocal, imitanciometria, Emissões Otoacústicas (EOAs) e Potencial Evocado Auditivo de Tronco Encefálico (PEATE). Critérios de inclusão: presença das EOAs e/ou Microfonismo Coclear (MC); ausência ou alterações nas ondas do (PEATE) e ressonância nuclear magnética cerebral normal. RESULTADOS: Entre os 2.292 sujeitos com PANS, 27 (1,2%) apresentaram ENA, sendo 37% masculino e 63% feminino. Entre os sujeitos com ENA, 29,6% tinham PANS leve; 55,5% moderada; 7,4% grave e 7,5% profunda. Em relação à faixa etária, 14,8% estavam entre 0 e 20 anos, 33,4% entre 21 e 40 anos, 44,4% entre 41 e 60 anos e 7,4% acima de 60 anos. CONCLUSÃO: A prevalência do ENA neste estudo foi de 1,2% em sujeitos com PANS.

Auditory neuropathy spectrum disorder (ANSD) is characterized by impairment of the auditory nerve associated with preservation of outer hair cell function. OBJECTIVE: To establish the prevalence of ANSD in subjects with sensorineural hearing loss (SNHL). METHOD: This retrospective study was carried out between 2010 and 2012 and included the charts of 2,292 individuals with SNHL. Data from otolaryngological and audiological examinations based on pure-tone and speech audiometry, impedance tests, otoacoustic emissions (OAEs), and brainstem auditory evoked potentials (BAEPs) were collected. Inclusion criteria: presence of OAEs and/or cochlear microphonic (CM); absent or altered BAEPs, and normal MRI scans of the brain. RESULTS: Twenty-seven (1.2%) of the 2,292 subjects with SNHL had ANSD (37% males; 63% females). Mild SNHL was seen in 29.6% of the individuals with ANSD; 55.5% had moderate SNHL; 7.4% had severe SNHL; and 7.5% had profound SNHL. In terms of age, 14.8% were aged between zero and 20 years, 44.1% were 41 to 60 years old, and 7.4% were above the age of 60. CONCLUSION: ANSD was seen in 1.2% of the individuals with SNHL included in this study.

Efeitos da exposição a agrotóxicos sobre o sistema auditivo periférico e central: uma revisão sistemática / Peripheral and central auditory effects of pesticide exposure: a systematic review / Efectos de la exposición a los pesticidas en el sistema auditivo periférico y central: una revisión sistemática

De acordo com a Organização Mundial da Saúde, as intoxicações agudas por agrotóxicos são da ordem de 3 milhões anuais, com 2,1 milhões de casos só nos países em desenvolvimento. Na última década, no Brasil, o uso de agrotóxicos assumiu proporções assustadoras. Entre 20012008 a venda desses produtos saltou, quando o país alcançou a posição de maior consumidor mundial de venenos. O objetivo deste estudo foi avaliar por meio de uma revisão sistemática se a exposição ao agrotóxico causa alterações auditivas no sistema auditivo periférico/central, atentando assim para a importância da avaliação auditiva em populações expostas de forma crônica ou aguda. Trata-se de uma revisão sistemática dos estudos publicados sobre os efeitos da exposição ao agrotóxico no sistema auditivo. Analisaram-se os trabalhos contemplados na íntegra e também sua qualidade metodológica. A pesquisa identificou 143 estudos sobre o tema, sendo que 16 se enquadraram nos critérios de inclusão. Todos os artigos analisados evidenciaram que a exposição ao agrotóxico é ototóxica e induz ao dano às vias auditivas.

The World Health Organization reports a total of 3 million annual cases of acute pesticide poisoning (2.1 million cases in the developing countries alone). Pesticide use has reached alarming proportions in Brazil in the last decade. Pesticide sales skyrocketed from 2001 to 2008, making Brazil the world's leading consumer of poisons. This study aimed to assess whether pesticide exposure causes peripheral or central auditory disorders and thus focused on the importance of hearing tests in populations with acute or chronic exposure. This was a systematic review of studies on the effects of pesticide exposure on the auditory system. The context and methodological quality of the full texts were analyzed. The review identified 143 studies on the theme, 16 of which met the inclusion criteria. All articles showed that pesticide exposure is ototoxic and leads to hearing loss.

El objetivo de este artículo es presentar un estudio sobre el uso de la historia oral de vida como estrategia de aproximación entre cuidador y anciano, con el fin de contribuir a la humanización en la relación entre el profesional de la salud y el paciente. Se trata de una investigación cualitativa y descriptiva. Hemos reunido a siete ancianos, varones y mujeres, con más de 65 años que, a partir de entrevistas abiertas y semi-estructuradas, hicieron posible la producción de relatos de vida que, una vez finalizados, fueron devueltos a los colaboradores en forma de cuadernos personalizados para que ellos dispusieran de ellos como quisieran. Como resultado ha sido posible percibir que tal metodología contribuye a la generación de un vínculo entre enfermero y anciano, presentándose no solamente como elemento humanizador, sino también terapéutico.

Effectiveness of cochlear implantation in children with auditory neuropathy and cochlear nerve aplasia / 中华耳鼻咽喉头颈外科杂志

<p><b>OBJECTIVE</b>The aim of this study is to assess of cochlear implantation in children with auditory neuropathy and cochlear nerve aplasia by using Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR).</p><p><b>METHODS</b>Twenty one children with cochlear implants participated in this study. They all received cochlear implant surgery at our hospital from January 2004 to October 2010. All children had hearing aid trial and hearing and speech rehabilitation before surgery at least three months.Nine children (7 male, 2 female) were diagnosed with auditory neuropathy, twelve (7 male, 5 female) with cochlear nerve aplasia. Twenty children (10 male, 10 female) with sensorineural hearing loss served as a control group. All the children received cochlear implant for more than six months. Forty two children with normal hearing served as another control group which were divided into three subgroups according to their age.Group A included 18 children aged under two yrs, group B consisted of 16 children aged from two to four yrs and group C comprised eight children aged above four yrs. CAP and SIR were used to evaluate among all the children and the scores were compared.</p><p><b>RESULTS</b>The CAP scores of children with auditory neuropathy, cochlear nerve aplasia, sensorial neural hearing loss and the three subgroups children with normal hearing were 4.44 ± 1.50, 4.83 ± 1.69, 4.55 ± 1.66, 5.22 ± 1.11, 6.75 ± 0.45 and 7.00 ± 0.00 respectively, and SIR scores were 2.66 ± 1.11, 2.33 ± 1.15, 2.40 ± 0.75, 2.56 ± 1.04, 4.12 ± 0.81 and 5.00 ± 0.00 respectively. There were significant differences among the six groups for CAP scores(χ(2) = 35.481, P < 0.001) and SIR scores(χ(2) = 40.549, P < 0.001).No significant differences for CAP and SIR scores were observed between children with auditory neuropathy/cochlear nerve aplasia and sensorial neural hearing loss as well as group A (P > 0.05 for each), and there were significant differences were shown between children with auditory neuropathy/cochlear nerve aplasia and group B as well as group C (P < 0.01 for each aplasia).</p><p><b>CONCLUSIONS</b>The auditory and speech capabilities of children with auditory neuropathy and cochlear nerve deficiency can can get benefits from cochlear implants as children with sensorineural hearing loss, however not achieve the level of those with normal hearing after cochlear implantation. The long term effects still need follow-up and evaluation.</p>

Cochlear implantation in patients with auditory neuropathy assisted by intra-operative EABR and the therapeutic effect evaluation / 临床耳鼻咽喉头颈外科杂志

OBJECTIVE@#To evaluate the pathological position of auditory neuropathy and investigate the hearing and speech rehabilitation results of cochlear implantation in patients with auditory neuropathy.@*METHOD@#In our hospital, among the patients received cochlear implantation, 8 cases with auditory neuropathy were selected, and 8 cases of non-auditory neuropathy patients with profound deafness were selected as matched control group with the background close to the study group. The preoperative hearing data of these two groups were retrospectively analyzed. During operation, the homemade stimulation electrodes were inserted to test the electric evoked auditory brainstem response(EABR) for assessing the auditory pathway; EABR and neural response telemetry(NRT) were tested after implantation, and T, C value were acquired 1 month later. CAP, SIR and speech recognition rate were used to assess hearing and speech rehabilitation effect 12 months after booting.@*RESULT@#Intra-operative EABR wave can be derived in 8 cases of auditory neuropathy, but the wave pattern exhibited variations compared with normal wave. It needed increased stimulation or adjusted parameters, with variable V latency. After cochlear implantation, the waveforms of NRT and EABR were similar between the two groups, and the post-operative V waveform was close to the intra-operative EABR. 8 pairs of patients can present listening response after booting. There was no statistically significant difference in T, C value, CAP (6.50 +/- 0.94 and 6.90 +/- 0.77) and speech recognition rate (85.00% +/- 11.66% and 89.50% +/- 9.02%) between the auditory neuropathy group and the control group 1 year after booting.@*CONCLUSION@#Pre-operative EABR can be used as an effective tool to assess the auditory pathway of auditory neuropathy patients, improving the pre-operative examination and helping with selecting the suitable cochlear implant patient. Cochlear implantation can help patients with auditory neuropathy to improve hearing and speech.