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

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

Influence of obstructive sleep apnea on auditory event-related potentials.

PURPOSE: To evaluate the influence of obstructive sleep apnea (OSA) on the P300 response of auditory event-related potentials (ERPs) and to correlate the electrophysiological findings with OSA severity. METHODS: Patients with no OSA and mild, moderate, and severe OSA according to polysomnography (PSG) with normal hearing and no comorbidities were studied. Individuals with a body mass index (BMI) ≥ 40 kg/m2, hypertension, diabetes, dyslipidemia, the use of chronic medications, and a risk of hearing loss were excluded. All patients underwent full PSG and auditory ERP measurement using the oddball paradigm with tone burst and speech stimuli. For P300 analysis (latencies and amplitudes), normal multiple linear regression models were adjusted with the groups (No OSA, Mild OSA, Moderate OSA, Severe OSA), age, BMI, and Epworth score as explanatory variables. RESULTS: We studied 54 individuals (47 males) aged 35 ± 8 years with a BMI of 28.4 ± 4.3 kg/m2. Patients were divided according to the apnea-hypopnea index (AHI) derived from PSG into no OSA (n = 14), mild (n = 16), moderate (n = 12), and severe OSA (n = 12) groups. Patients with severe OSA presented prolonged P300 latencies with tone burst stimuli compared to patients with no OSA and those with mild and moderate OSA. CONCLUSION: Severe OSA is associated with impairment of the P300 response of auditory ERPs, suggesting a decrease in the processing speed of acoustic information that may be mediated by the level of somnolence.

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

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

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

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

Peripheral and brainstem auditory evaluation in post-COVID-19 individuals.

PURPOSE: The purpose of this study was to investigate the peripheral and central auditory pathways in adult individuals after COVID-19 infection. METHOD: A total of 44 individuals aged between 19 and 58 years, of both genders, post-COVID-19 infection, confirmed by serological tests, with no previous hearing complaints and no risk factors for hearing loss, were assessed. All the participants underwent the following procedures: pure tone audiometry, logoaudiometry, immitanciometry, and Brainstem Auditory Evoked Potentials (BAEP), in addition to answering a questionnaire about auditory symptoms. RESULTS: Thirteen individuals (29.5 %) had some hearing threshold impairment, mainly sensorineural hearing loss. In the BAEP, 18 individuals (40.9 %) presented longer latencies, mainly in waves III and V. According to the questionnaire answers, 3 individuals (9.1 %) reported worsened hearing and 7 (15.9 %) tinnitus that emerged after the infection. As for the use of ototoxic drugs during treatment, 7 individuals (15.9 %) reported their use, of which 5 showed abnormalities in peripheral and/or central auditory assessments. CONCLUSION: Considering the self-reported hearing complaints after COVID-19 infection and the high rate of abnormalities found in both peripheral and central audiological assessments, it is suggested that the new COVID-19 may compromise the auditory system. Due to the many variables involved in this study, the results should be considered with caution. However, it is essential that audiological evaluations are carried out on post-COVID-19 patients in order to assess the effects of the infection in the short, medium, and long term. Future longitudinal investigations are important for a better understanding of the auditory consequences of COVID-19.

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

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

Auditory central pathways in children and adolescents with multiple sclerosis.

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

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

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

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

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

Cortical auditory evoked potentials in autism spectrum disorder: a systematic review. / Potenciais evocados auditivos corticais no transtorno do espectro do autismo: revisão sistemática.

PURPOSE: To identify and analyze what are the characteristic findings of Cortical Auditory Evoked Potentials (CAEP) in children and / or adolescents with Autism Spectrum Disorder (ASD) compared to typical development, through a systematic literature review. RESEARCH STRATEGIES: Based on the formulation of a research question, a bibliographic survey was carried out in seven databases (Web of Science, Pubmed, Cochrane Library, Lilacs, Scielo, Science Direct, and Google Sholar), with the following descriptors: autism spectrum disorder (transtorno do espectro autista), autistic disorder (transtorno autístico), evoked potentials, auditory (potenciais evocados auditivos), event related potentials, P300 (potencial evocado P300) e child (criança). This review was registered in Prospero, under number 118751. SELECTION CRITERIA: Were selected articles published, without language limitation, between 2007 and 2019. DATA ANALYSIS: The characteristics of the latency and amplitude aspects of the P1, N1, P2, N2 and P3 components present in the CAEP. RESULTS: 193 studies were located; however, 15 original articles were included the inclusion criteria for this study. Although it has not been possible to identify any pattern of response for the P1, N1, P2 and N2 components, the results of the selected studies have demonstrated that individuals with ASD may present a decrease in amplitude and increase in latency of the P3 component. CONCLUSION: Individuals with ASD may present different responses to the components of the CAEP, and the decrease of the amplitude and increase of the latency of the P3 component were the most common characteristics.

OBJETIVO: Identificar e analisar quais são os achados característicos dos Potenciais Evocados Auditivos Corticais (PEAC) em crianças e/ou adolescentes com Transtorno do Espectro do Autismo (TEA) em comparação do desenvolvimento típico, por meio de uma revisão sistemática da literatura. ESTRATÉGIA DE PESQUISA: Após formulação da pergunta de pesquisa, foi realizada uma revisão da literatura em sete bases de dados (Web of Science, Pubmed, Cochrane Library, Lilacs, Scielo, Science Direct, e Google acadêmico), com os seguintes descritores: transtorno do espectro autista (autism spectrum disorder), transtorno autístico (autistic disorder), potenciais evocados auditivos (evoked potentials, auditory), potencial evocado P300 (event related potentials, P300) e criança (child). A presente revisão foi cadastrada no Próspero, sob número 118751. CRITÉRIOS DE SELEÇÃO: Foram selecionados estudos publicados na integra, sem limitação de idioma, entre 2007 e 2019. Análise dos dados: Foram analisadas as características de latência e amplitude dos componentes P1, N1, P2, N2 e P3 presentes nos PEAC. RESULTADOS: Foram localizados 193 estudos; contudo 15 estudos contemplaram os critérios de inclusão. Embora não tenha sido possível identificar um padrão de resposta para os componentes P1, N1, P2, N2 e P3, os resultados da maioria dos estudos demonstraram que indivíduos com TEA podem apresentar diminuição de amplitude e aumento de latência do componente P3. CONCLUSÃO: Indivíduos com TEA podem apresentar respostas diversas para os componentes dos PEAC, sendo que a diminuição de amplitude e aumento de latência do componente P3 foram as características mais comuns.

Auditory hypersensitivity in Williams syndrome.

OBJECTIVES: The objective of this study was to investigate auditory hypersensitivity in WS and to evaluate hyperacusis through standardized protocols, checking if it can be associated with the absence of acoustic reflexes in people with WS. METHOD: The study was performed in 17 individuals with WS, aged between seven and 17 years old (10 males and seven females), and 17 individuals with typical development age- and gender-matched to individuals with WS. Statistical tests were used to analyze the responses collected with the Loudness Discomfort Level (LDL) test as well as ipsilateral and contralateral reflex responses. RESULTS: Auditory hypersensitivity was commonly found. Individuals with WS had phonophobia and were less tolerant to high sound intensity, presenting a reduced discomfort threshold compared to those with typical development. However, hyperacusis was found in 35.29% of individuals with WS and was mild in 50% of cases. There was an association between hyperacusis and acoustic reflex responses, and individuals with absence of the contralateral acoustic reflex were more likely to have hyperacusis. CONCLUSIONS: Individuals with WS have a high prevalence of auditory hypersensitivity, with the presence of phonophobia; however, hyperacusis was not as prevalent and may be associated with the absence of contralateral acoustic reflexes.

Neuronal plasticity of the auditory pathway in children with speech sound disorder: a study of Long-Latency Auditory Evoked Potentials. / Plasticidade neuronal da via auditiva em crianças com transtorno dos sons da fala: estudo dos Potenciais Evocados Auditivos de Longa Latência.

PURPOSE: To analyze the results of Long-latency Auditory Evoked Potentials (LLAEP) in children with Speech Sounds Disorder (SSD) after speech therapy. METHODS: Longitudinal and prospective clinical study at 14 children with SSD, with ages ranging from five to seven years, of both genders. Were applied Picture Naming task and Imitation task, and from these tasks it was calculated the Percentage of Consonants Correct index. For an analysis of the LLAEP with speech stimulus and recorded the latency and amplitude values ​​of P1, N1, P2, N2 and P3 components. Each child was evaluated in two different moments: initial evaluation and after 12 sessions of speech therapy. RESULTS: It was observed that after twelve sessions of speech therapy the value of Percentage of Consonants Correct index increased, and a greater number of components were observed in the LLAEP records of children with SSD, as well as a statistically significant increase in the amplitude of the P3 component, demonstrating that anatomical and physiological changes occurred in the central auditory nervous system after intervention, resulting in improved of the LLAEP results. CONCLUSION: After speech therapy, improvement in the children's phonology was observed, and there was an increase in the number of components present in the LLAEP, as well as an increase in the amplitude of the P3 component, demonstrating that plasticity occurred in the auditory pathway during these three months of therapeutic intervention.

OBJETIVO: Avaliar os achados dos Potenciais Evocados Auditivos de Longa Latência (PEALL) em crianças com Transtorno dos Sons na Fala (TSF) após terapia fonoaudiológica. MÉTODO: Estudo clínico longitudinal e prospectivo em um grupo de 14 crianças com TSF, de cinco a sete anos de idade, de ambos os sexos. Foram aplicadas as provas de Nomeação de Figuras e Imitação de palavras, para as quais foi calculado o índice de gravidade Porcentagem de Consoantes Corretas. Foram registrados os PEALL com estímulo de fala e foram analisados os valores de latência e amplitude dos componentes P1, N1, P2, N2 e P3. Cada criança foi avaliada em dois diferentes momentos: avaliação inicial e após 12 sessões de terapia fonoaudiológica. RESULTADOS: Os resultados mostraram que após terapia fonoaudiológica, o valor do índice de gravidade Porcentagem de Consoantes Corretas aumentou e um maior número de componentes foi observado nos registros dos PEALL nas crianças com TSF. Também foi observado um aumento estatisticamente significativo na amplitude do componente P3, demostrando que modificações anatomofisiológicas ocorreram no sistema nervoso auditivo central após intervenção, proporcionando melhora nos resultados dos PEALL. CONCLUSÃO: Após terapia fonoaudiológica, foi observada melhora no desempenho fonológico das crianças, aumento no número de componentes presentes nos PEALL, bem como aumento na amplitude do componente P3, demonstrando que ocorreu plasticidade na via auditiva após um curto período de intervenção fonoaudiológica.

Central Auditory Nervous System Stimulation through the Cochlear Implant Use and Its Behavioral Impacts: A Longitudinal Study of Case Series.

The purpose of this study was to investigate, over a period of five years, the cortical maturation of the central auditory pathways and its impacts on the auditory and oral language development of children with effective use and without effective use of a Cochlear Implant (CI). A case series study was conducted with seven children who were CI users and seven children with normal hearing, with age- and gender-matched to CI users. The assessment was performed by long-latency auditory evoked potentials and auditory and oral language behavioral protocols. The results pronounced P1 latency decrease in all CI users in the first nine months. Over five years, five children with effective CI use presented decrease or stabilization of P1 latency and a gradual development of auditory and oral language skills, although, for most of the children, the electrophysiological and behavior results remained poor than their hearing peers' results. Two children who stopped the effective use of CI after the first year of activation had worsened auditory and oral language behavioral skills and presented increased P1 latency. A negative correlation was observed between behavioral measures and the P1 latency, the P1 component being considered an important clinical resource capable of measuring the cortical maturation and the behavioral evolution.

Brainstem auditory evoked potentials in children with autism spectrum disorder.

OBJECTIVES: This study aimed to analyze the neural encoding of verbal and nonverbal stimuli in individuals with autism spectrum disorder using brainstem auditory evoked potentials. METHODOLOGY: Thirty individuals between 7 and 12 years of age and of both genders participated in this study. Fifteen were diagnosed with autism spectrum disorder, and 15 had typical development. All subjects had normal hearing and no other impairments. An electrophysiological hearing assessment was performed using brainstem auditory evoked potentials with click and speech stimuli. RESULTS: In the brainstem auditory evoked potentials with click stimuli, the mean wave I latency was longer for the right ear in both groups, and interpeak intervals III-V were greater for the individuals with autism spectrum disorder. For brainstem auditory evoked potentials with speech stimuli, wave V latency was shorter in individuals with autism spectrum disorder. CONCLUSION: These data suggest that individuals with autism spectrum disorder may have a dysfunction of the central auditory nervous system for nonverbal stimuli and faster neural encoding of the initial part of the verbal stimulus, suggesting hypersensitivity to complex sounds such as speech.

Characteristics of auditory evaluation in Williams syndrome: a systematic review. / Características da avaliação auditiva na síndrome de Williams: revisão sistemática.

PURPOSE: Identify the characteristics of the clinical audiological evaluation of individuals with Williams syndrome by means of a systematic literature review. RESEARCH STRATEGIES: The following research question was initially determined: "What are the characteristics of clinical auditory assessment in individuals with Williams syndrome?". From this, a bibliographic search was conducted in four databases using the descriptors: Williams syndrome, Hearing loss, and Audiology. SELECTION CRITERIA: Only full articles with evidence levels 1 or 2, published in Brazilian Portuguese or English, were selected. DATA ANALYSIS: Results obtained in the auditory tests used in the clinical routine, namely: immittance test, pure-tone audiometry, otoacoustic emissions, and brainstem auditory evoked potential were analyzed. RESULTS: Two hundred nine studies were found, but only 12 met the inclusion criteria for the study. It was possible to observe prevalence of type A tympanometry curve, which may occur with absence of acoustic reflexes, mild to moderate sensorineural hearing loss, affecting mainly the high frequencies, absent or less amplified otoacoustic emissions, and brainstem auditory evoked potential without retrocochlear alteration. CONCLUSION: Cochlear impairment is common in individuals with Williams syndrome and the main disorders found in the hearing assessment in this population are absence of otoacoustic emissions and acoustic reflexes, as well as presence of mild to moderate sensorineural hearing loss, mainly in the high-frequency range, observed by audiometry.

OBJETIVO: Identificar por meio de uma revisão sistemática da literatura quais são as características da avaliação audiológica clínica de indivíduos com síndrome de Williams. ESTRATÉGIA DE PESQUISA: Inicialmente foi determinada a seguinte pergunta de pesquisa: "Quais são as características da avaliação auditiva clínica em indivíduos com síndrome de Williams?". A partir desta, foi realizado um levantamento bibliográfico em 4 bases de dados, utilizando-se dos seguintes descritores: síndrome de Williams (Williams syndrome), perda auditiva (hearing loss) e audiologia (audiology). CRITÉRIOS DE SELEÇÃO: Foram selecionados artigos com nível de evidência 1 ou 2, publicados na íntegra nos idiomas português brasileiro ou inglês. ANÁLISE DOS DADOS: Foram analisados os resultados obtidos nos testes auditivos utilizados na rotina clínica, incluindo: imitanciometria, audiometria tonal, emissões otoacústicas e potencial evocado auditivo de tronco encefálico. RESULTADOS: 209 estudos foram encontrados, porém apenas 12 contemplaram os critérios de inclusão para o estudo. Foi possível observar prevalência de curva timpanométrica do tipo A, que pode ocorrer juntamente com ausência de reflexos acústicos, perda auditiva neurossensorial de grau leve a moderado acometendo principalmente as frequências altas, emissões otoacústicas ausentes ou de menor amplitude e potencial evocado auditivo de tronco encefálico sem alteração retrococlear. CONCLUSÃO: O comprometimento coclear é comum em indivíduos com síndrome de Williams e as principais alterações na avaliação auditiva nesta população são a ausência das emissões otoacústicas e dos reflexos acústicos bem como a presença de perda auditiva neurossensorial de grau leve a moderado principalmente nas frequências altas na audiometria tonal.

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

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

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

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

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

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

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

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

Maturation of long latency auditory evoked potentials in hearing children: systematic review. / Maturação dos potenciais evocados auditivos de longa latência em crianças ouvintes: revisão sistemática.

PURPOSE: To analyze how Auditory Long Latency Evoked Potentials (LLAEP) change according to age in children population through a systematic literature review. RESEARCH STRATEGIES: After formulation of the research question, a bibliographic survey was done in five data bases with the following descriptors: Electrophysiology (Eletrofisiologia), Auditory Evoked Potentials (Potenciais Evocados Auditivos), Child (Criança), Neuronal Plasticity (Plasticidade Neuronal) and Audiology (Audiologia). SELECTION CRITERIA: Level 1 evidence articles, published between 1995 and 2015 in Brazilian Portuguese or English language. DATA ANALYSIS: Aspects related to emergence, morphology and latency of P1, N1, P2 and N2 components were analyzed. RESULTS: A total of 388 studies were found; however, only 21 studies contemplated the established criteria. P1 component is characterized as the most frequent component in young children, being observed around 100-150 ms, which tends to decrease as chronological age increases. The N2 component was shown to be the second most commonly observed component in children, being observed around 200-250 ms.. The other N1 and P2 components are less frequent and begin to be seen and recorded throughout the maturational process. CONCLUSION: The maturation of LLAEP occurs gradually, and the emergence of P1, N1, P2 and N2 components as well as their latency values are variable in childhood. P1 and N2 components are the most observed and described in pediatric population. The diversity of protocols makes the comparison between studies difficult.

Cortical maturation of long latency auditory evoked potentials in hearing children: the complex P1-N1-P2-N2. / Maturação dos potenciais evocados auditivos de longa latência em crianças ouvintes: análise do complexo P1-N1-P2-N2.

PURPOSE: The purpose of this study was to monitor the emergence and changes to the components of the Long Latency Auditory Evoked Potentials (LLAEP) in normal hearing children. METHODS: This longitudinal study included children of both genders: seven aged between 10 and 35 months, and eight children between 37 and 63 months. The electrophysiological hearing evaluation consisted of analysis of LLAEP obtained in a sound field generated with loudspeakers positioned at an azimuth of 90°, through which the syllable /ba/ was played at an intensity of 70 dB HL. Each child underwent an initial evaluation followed by two re-evaluations three and nine months later. RESULTS: The emergence of LLAEP components across the nine-month follow-up period was observed. P1 and N2 were the most common components in children of this age range. There was no statistically significant difference regarding the occurrence of P1, N1, P2, and N2 components amongst younger and older children. Regarding latency values, the greatest changes overtime were observed in the P1 component for younger children and in the N2 component for older children. Only the P1 component significantly differed between the groups, with the highest latency values observed in younger children. CONCLUSION: LLAEP maturation occurs gradually and the emergence of complex components appears to be related more to the maturation of the central auditory nervous system than to chronological age.

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

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

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