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1.
Medicina (Kaunas) ; 56(7)2020 Jul 13.
Artigo em Inglês | MEDLINE | ID: mdl-32668569

RESUMO

Background and objectives: The cochlear implant is not only meant to restore auditory function, but it also has a series of benefits on the psychomotor development and on the maturation of central auditory pathways. In this study, with the help of neuropsychological tests and cortical auditory potentials (CAEPs), we intend to identify a series of instruments that allow us to monitor children with a cochlear implant, and later on, to admit them into an individualized rehabilitation program. Materials and methods: This is a longitudinal study containing 17 subjects (6 boys and 11 girls) diagnosed with congenital sensorineural hearing loss. The average age for cochlear implantation in our cohort is 22 months old. Each child was tested before the cochlear implantation, tested again 3 months after the implant, and then 6 months after the implant. To test the general development, we used the Denver Developmental Screening Test (DDST II). CAEPs were recorded to assess the maturation of central auditory pathways. Results: The results showed there was progress in both general development and language development, with a significant statistical difference between the overall DQ (developmental quotient) and language DQ before the cochlear implantation and three and six months later, respectively. Similarly, CAEP measurements revealed a decrease of positive-going component (P1) latency after cochlear implantation. Conclusion: CAEPs and neuropsychological tests prove to be useful instruments for monitoring the progress in patients with cochlear implants during the rehabilitation process.


Assuntos
Córtex Auditivo/crescimento & desenvolvimento , Doenças Auditivas Centrais/complicações , Implantes Cocleares/normas , Doenças Auditivas Centrais/cirurgia , Pré-Escolar , Implante Coclear/métodos , Implantes Cocleares/efeitos adversos , Feminino , Crescimento e Desenvolvimento , Humanos , Lactente , Estudos Longitudinais , Masculino , Transtornos do Neurodesenvolvimento/complicações , Transtornos do Neurodesenvolvimento/cirurgia
2.
BMJ Open ; 10(6): e033342, 2020 06 22.
Artigo em Inglês | MEDLINE | ID: mdl-32571854

RESUMO

INTRODUCTION: A number of studies have reported an association between peripheral hearing impairment, central auditory processing and Alzheimer's disease (AD) and its preclinical stages. Both peripheral hearing impairment and central auditory processing disorders are observed many years prior to the clinical manifestation of AD symptoms, hence, providing a long window of opportunity to investigate potential interventions against neurodegenerative processes. This paper outlines the protocol for a systematic review of studies examining the central auditory processing functions in AD and its preclinical stages, investigated through behavioural (clinical assessments that require active participation) central auditory processing tests. METHODS AND ANALYSIS: We will use the keywords and Medical Subject Heading terms to search the following electronic databases: MEDLINE, PsychINFO, PubMed, Scopus, EMBASE and CINAHL Plus. Studies including assessments of central auditory function in adults diagnosed with dementia, AD and its preclinical stages that were published before 8 May 2019 will be reviewed. This review protocol will be reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses Protocols guidelines. Data analysis and search results will be reported in the full review. This manuscript has designed the protocols for a systematic review that will identify the behavioural clinical central auditory processing measures that are sensitive to the changes in auditory function in adults with AD and its preclinical stages. Such assessments may subsequently help to design studies to examine the potential impact of hearing and communication rehabilitation of individuals at risk of AD. ETHICS AND DISSEMINATION: Ethical approval is not required as this manuscript only reports the protocols for conducting a systematic review as primary data will only be reviewed and not be collected. The results of this systematic review will be disseminated through publication and in scientific conferences. PROSPERO REGISTRATION NUMBER: CRD42017078272.


Assuntos
Doença de Alzheimer/complicações , Doenças Auditivas Centrais/complicações , Doenças Auditivas Centrais/diagnóstico , Perda Auditiva Neurossensorial/complicações , Perda Auditiva Neurossensorial/diagnóstico , Humanos , Projetos de Pesquisa , Revisões Sistemáticas como Assunto
3.
Nat Rev Neurol ; 15(1): 11-24, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30532084

RESUMO

The vast increase in Alzheimer disease (AD) worldwide has grave implications for individuals, family support systems and the health-care systems that will attempt to cope with the disease. Early markers of the disease are essential for efficient selection of clinical trial participants for drug development and for timely treatment once an intervention becomes available. There is avid interest in noninvasive, inexpensive markers that have the potential to identify prodromal AD. This Review considers sensory impairments that have the potential to serve as early indicators of AD, with a focus on olfaction, hearing and vision. Current evidence regarding the potential markers of AD in each modality is examined, with a particular emphasis on olfaction and current findings that olfactory function is associated with prodromal AD. Research suggests that olfactory impairment is associated with other markers that signal the emergence of prodromal AD. Auditory impairment is associated with dementia in epidemiological studies and visual system deficits have been reported in AD; however, the emergence of these deficits in prodromal AD is unclear. Further research is necessary to address the relative sensitivity and specificity of olfactory, auditory and visual measures for the detection of prodromal AD.


Assuntos
Doença de Alzheimer/complicações , Doença de Alzheimer/diagnóstico por imagem , Transtornos do Olfato/complicações , Transtornos do Olfato/diagnóstico por imagem , Sintomas Prodrômicos , Doença de Alzheimer/metabolismo , Doenças Auditivas Centrais/complicações , Doenças Auditivas Centrais/diagnóstico por imagem , Doenças Auditivas Centrais/metabolismo , Biomarcadores/líquido cefalorraquidiano , Biomarcadores/metabolismo , Humanos , Transtornos do Olfato/metabolismo , Transtornos da Visão/complicações , Transtornos da Visão/diagnóstico por imagem , Transtornos da Visão/metabolismo
4.
Dement. neuropsychol ; 12(3): 314-320, July-Sept. 2018. tab
Artigo em Inglês | LILACS | ID: biblio-952978

RESUMO

Abstract Auditory deprivation associated with early otitis media with effusion has been considered a risk factor for central auditory processing (CAP), as well as for the development of a number of cognitive functions. Objective: To study the neuropsychological functions of attention, working memory and executive function in adolescents with and without non-cholesteatomatous chronic otitis media (NCCOM) and analyze their interrelationships with the behavioral evaluation of CAP. Methods: Sixty-eight adolescents were recruited, 34 were diagnosed with NCCOM (study group - SG), and 34 had no otological history (control group - CG). The Neupsilin Brief Neuropsychological Assessment Instrument was used. CAP was assessed by: Masking Level Difference, Synthetic Sentence Identification, Random Gap Detection Test, Duration Pattern Sequence Test and Dichotic Digits Test. Results: The results of Neupsilin showed lower scores in the study group when compared to the control group on the following tests: digit sequence repetition, ascending digit ordering, auditory sentence span, and phonemic verbal fluency. An association was found between central auditory processing tests and Neupsilin subtests. Conclusion: The effects of NCCOM on attention, memory and executive function related to central auditory processing disorder in adolescents seem to be enhanced by the severity of the disease.


Resumo A privação auditiva associada à otite média precoce com efusão tem sido considerada um fator de risco para o processamento auditivo central (CAP), bem como para o desenvolvimento de diversas funções cognitivas. Objetivo: Estudar as funções neuropsicológicas da atenção, memória de trabalho e função executiva em adolescentes com e sem otite média crônica não-colesteatomatosa (NCCOM) e analisar suas inter-relações com a avaliação comportamental da PAC. Métodos: Sessenta e oito adolescentes foram recrutados, 34 foram diagnosticados com NCCOM (grupo de estudo - GE) e 34 não tinham história otológica (grupo controle - GC). Utilizou-se o Instrumento de Avaliação Neuropsicológica Breve de Neupsilin. O CAP foi avaliado por: Diferença de Nível de Mascaramento, Identificação de Frase Sintética, Teste de Detecção de Intervalo Aleatório, Teste de Sequência de Padrão de Duração e Teste de Dígitos Dicóticos. Resultados: Os resultados de Neupsilin mostraram menores escores no grupo de estudo quando comparados ao grupo controle nos seguintes testes: repetição de sequência de dígitos, ordenação de dígitos ascendentes, extensão de sentenças auditivas e fluência verbal fonêmica. Foi encontrada uma associação entre os testes do processamento auditivo central e os subtestes de Neupsilin. Conclusão: Os efeitos do NCCOM na atenção, memória e função executiva relacionados ao transtorno do processamento auditivo central em adolescentes parecem ser potencializados pela gravidade da doença.


Assuntos
Humanos , Adolescente , Doenças Auditivas Centrais/complicações , Percepção Auditiva , Adolescente , Cognição
5.
Early Interv Psychiatry ; 10(2): 129-36, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24861468

RESUMO

AIM: In the recent literature, there has been growing interest in assessment methods for detecting increased risk of developing psychosis. Self-report methods are popular but may lead to different results compared to clinical interviews. METHODS: The difference in psychosis risk scores was tested between self-reported psychosis risk symptoms (PROD-SR) and self-reported symptoms additionally confirmed by interview (PROD-SR + I). The symptom categories were derived from 12 common psychosis risk symptoms included in the PROD screening instrument. The data were collected by questionnaires and interviews conducted with 395 adolescents (mean age 15.3 years) in an early intervention and detection team, JERI, at Helsinki University Central Hospital, Finland. RESULTS: The results show a significant difference between the PROD-SR risk symptom sum scores and the PROD-SR + I risk symptom sum scores (N = 395; Z = -15.123; P < 0.001). In an item-by-item analysis, the item 'Disorders in connection with hearing' had the strongest kappa value (0.827) agreement between an interviewed and self-report psychosis risk item. Agreement in most items remained between slight and substantial (kappa values from 0.082 to 0.649). CONCLUSIONS: The results suggest that there is a significant difference between psychosis risk symptom responses collected by self-report and self-report responses which are additionally confirmed by interview. Auditory disorders are the most reliably reported item with self-report.


Assuntos
Doenças Auditivas Centrais/diagnóstico , Diagnóstico Precoce , Entrevista Psicológica , Transtornos Psicóticos/diagnóstico , Autorrelato , Adolescente , Doenças Auditivas Centrais/complicações , Criança , Feminino , Humanos , Masculino , Escalas de Graduação Psiquiátrica , Transtornos Psicóticos/complicações , Fatores de Risco , Avaliação de Sintomas , Adulto Jovem
6.
Handb Clin Neurol ; 129: 537-56, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25726289

RESUMO

Central auditory processing disorders (CAPD) can affect children and adults of all ages due to a wide variety of causes. CAPD is a neurobiologic deficit in the central auditory nervous system (CANS) that affects those mechanisms that underlie fundamental auditory perception, including localization and lateralization; discrimination of speech and non-speech sounds; auditory pattern recognition; temporal aspects of audition, including integration, resolution, ordering, and masking; and auditory performance with competing and/or degraded acoustic signals (American Speech-Language-Hearing Association, 2005a, b). Although it is recognized that central auditory dysfunction may coexist with other disorders, CAPD is conceptualized as a sensory-based auditory disorder. Administration of behavioral and/or electrophysiologic audiologic tests that have been shown to be sensitive and specific to dysfunction of the CANS is critical for a proper diagnosis of CAPD, in addition to assessments and collaboration with a multidisciplinary team. Intervention recommendations for CAPD diagnosis are based on the demonstrated auditory processing deficits and related listening and related complaints. This chapter provides an overview of current definitions and conceptualizations, methods of diagnosis of, and intervention for, CAPD. The chapter culminates with a case study illustrating pre- and posttreatment behavioral and electrophysiologic diagnostic findings.


Assuntos
Doenças Auditivas Centrais , Transtornos da Percepção Auditiva/etiologia , Transtornos da Linguagem/etiologia , Transtornos da Memória/etiologia , Estimulação Acústica , Adolescente , Adulto , Doenças Auditivas Centrais/complicações , Doenças Auditivas Centrais/diagnóstico , Doenças Auditivas Centrais/etiologia , Feminino , Testes Auditivos , Humanos , Imageamento por Ressonância Magnética
7.
Otolaryngol Clin North Am ; 45(5): 959-81, 2012 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22980678

RESUMO

Over the past 30 years, hearing care clinicians have increasingly relied on cochlear implants to restore auditory sensitivity in selected patients with advanced sensorineural hearing loss. This article examines the impact of intervention with cochlear implantation in children and adults. The authors report a range of clinic-based results and patient-based outcomes reflected in the reported literature on cochlear implants. The authors describe the basic assessment of the physiologic response to auditory nerve stimulation; measures of receptive and productive benefit; and surveys of life effects as reflected measures of quality of life, educational attainment, and economic impact.


Assuntos
Estimulação Acústica/métodos , Doenças Auditivas Centrais , Implante Coclear/efeitos adversos , Implantes Cocleares , Perda Auditiva Neurossensorial , Tempo para o Tratamento , Idade de Início , Idoso , Doenças Auditivas Centrais/complicações , Doenças Auditivas Centrais/psicologia , Doenças Auditivas Centrais/cirurgia , Pré-Escolar , Implante Coclear/métodos , Implantes Cocleares/economia , Implantes Cocleares/psicologia , Implantes Cocleares/estatística & dados numéricos , Nervo Coclear/patologia , Nervo Coclear/fisiopatologia , Análise Custo-Benefício , Intervenção Educacional Precoce , Escolaridade , Perda Auditiva Neurossensorial/diagnóstico , Perda Auditiva Neurossensorial/epidemiologia , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/fisiopatologia , Perda Auditiva Neurossensorial/psicologia , Perda Auditiva Neurossensorial/cirurgia , Humanos , Desenvolvimento da Linguagem , Avaliação de Resultados em Cuidados de Saúde/métodos , Qualidade de Vida , Anos de Vida Ajustados por Qualidade de Vida , Resultado do Tratamento
8.
Med Hypotheses ; 78(6): 802-10, 2012 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-22520337

RESUMO

In this paper, we aimed to present a hypothesis that would explain the mechanism of auditory hallucinations, one of the main symptoms of schizophrenia. We propose that auditory hallucinations arise from abnormalities in the predictive coding which underlies normal perception, specifically, from the absence or attenuation of prediction error. The suggested deficiencies in processing prediction error could arise from (1) abnormal modulation of thalamus by prefrontal cortex, (2) absence or impaired transmission of external input, (3) dysfunction of the auditory and association cortex, (4) neurotransmitter dysfunction and abnormal connectivity, and (5) hyperactivity activity in auditory cortex and broad prior probability. If there is no prediction error, the initially vague prior probability develops into an explicit percept in the absence of external input, as a result of a recursive pathological exchange between auditory and prefrontal cortex. Unlike existing explanations of auditory hallucinations, we propose concrete mechanisms which underlie the imbalance between perceptual expectation and external input. Impaired processing of prediction error is reflected in reduced mismatch negativity and increased tendency to report non-existing meaningful language stimuli in white noise, shown by those suffering from auditory hallucinations. We believe that the expectation-perception model of auditory hallucinations offers a comprehensive explanation of the underpinnings of auditory hallucinations in both patients and those not diagnosed with mental illness. Therefore, our hypothesis has the potential to fill the gaps in the existing knowledge about this distressing phenomenon and contribute to improved effectiveness of treatments, targeting specific mechanisms.


Assuntos
Doenças Auditivas Centrais/fisiopatologia , Alucinações/etiologia , Alucinações/fisiopatologia , Modelos Biológicos , Neurotransmissores/efeitos adversos , Doenças Auditivas Centrais/complicações , Humanos , Córtex Pré-Frontal/fisiopatologia , Tálamo/fisiopatologia
9.
Schizophr Res ; 135(1-3): 15-22, 2012 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-22226903

RESUMO

Brain circuits involved in language processing have been suggested to be compromised in patients with schizophrenia. This does not only include regions subserving language production and perception, but also auditory processing and attention. We investigated resting state network connectivity of auditory, language and attention networks of patients with schizophrenia and hypothesized that patients would show reduced connectivity. Patients with schizophrenia (n = 45) and healthy controls (n = 30) underwent a resting state fMRI scan. Independent components analysis was used to identify networks of the auditory cortex, left inferior frontal language regions and the anterior cingulate region, associated with attention. The time courses of the components where correlated with each other, the correlations were transformed by a Fisher's Z transformation, and compared between groups. In patients with schizophrenia, we observed decreased connectivity between the auditory and language networks. Conversely, patients showed increased connectivity between the attention and language network compared to controls. There was no relationship with severity of symptoms such as auditory hallucinations. The decreased connectivity between auditory and language processing areas observed in schizophrenia patients is consistent with earlier research and may underlie language processing difficulties. Altered anterior cingulate connectivity in patients may be a correlate of habitual suppression of unintended speech, or of excessive attention to internally generated speech. This altered connectivity pattern appears to be present independent of symptom severity, and may be suggestive of a trait, rather than a state characteristic.


Assuntos
Transtorno do Deficit de Atenção com Hiperatividade/etiologia , Doenças Auditivas Centrais/complicações , Transtornos da Linguagem/etiologia , Vias Neurais/patologia , Esquizofrenia/complicações , Esquizofrenia/patologia , Adulto , Transtorno do Deficit de Atenção com Hiperatividade/patologia , Mapeamento Encefálico , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Transtornos da Linguagem/patologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Vias Neurais/irrigação sanguínea , Estatísticas não Paramétricas , Fatores de Tempo , Adulto Jovem
10.
Laryngoscope ; 121(1): 202-13, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-21181985

RESUMO

OBJECTIVES/HYPOTHESIS: Otoacoustic emissions (OAE) can be suppressed with activation of the medial olivocochlear neural pathway by stimulation of the contralateral ear. The primary objective of this study was to assess the feasibility of using olivocochlear mediated OAE suppression to test neonatal hearing with a novel device that detects changes in distortion product (DP)OAE level with high temporal resolution. The secondary objective was to investigate whether temporal parameters of the response can be determined with this technique and used in the assessment of neonates at risk of auditory neuropathy spectrum disorder (ANSD). STUDY DESIGN: Prospective translational study of novel hearing assessment technique. METHODS: There were 46 neonates tested in a clinic or neonatal intensive care unit (NICU). DPOAE were recorded in real time with narrow band pass digital filtering (1 ms temporal resolution) during presentation of an intermittent contralateral broadband noise stimulus. Magnitude and latency of the contralateral suppression response were compared with hearing outcome (auditory brainstem response screen and clinical follow-up) and risk factors for hearing loss, particularly hyperbilirubinemia as a risk factor for ANSD. RESULTS: : Contralateral suppression was identified in all of 38 neonates with detectable DPOAE and normal hearing, most reliably at f(2) = 4.4 kHz (average values = 1 dB suppression from DP level of 14 dB SPL using 0.55 s contralateral stimulus at 50 dB SPL). Sensorineural hearing loss was identified in three cases (6.5%) and ANSD in five cases (11% of all neonates tested). Contralateral suppression was absent in two of the ANSD cases (one associated with cochlear nerve aplasia, the other with hyperbilirubinemia) and present in three. The median latency for onset of contralateral suppression was 60 ms and offset latency 83 ms. The latency for offset of suppression was longer in neonates who required treatment for hyperbilirubinemia at 123 ms (P = .02, Mann-Whitney rank sum test). Latency measurements were determined with high intraobserver reliability (Pearson product moment correlation coefficient > 0.96). CONCLUSIONS: Contralateral suppression of real-time DPOAE can reliably be identified in neonates. This is likely a manifestation of olivocochlear activity, although middle ear muscle reflexes might contribute to suppression in some circumstances. The technique provides a feasible objective test of hearing in neonates that can be applied in the NICU setting without sedation. The presence of a response indicates detection of sound by the contralateral ear and effective brainstem transmission of neural signals, therefore providing a more sensitive test of hearing than OAE alone. The high temporal resolution of the technique allows measurement of latency of the response. These benefits help to identify neonates at risk of ANSD and have the potential to provide prognostic information that will assist in the management of this unpredictable disorder. Further development of the technique is indicated with regard to determination of hearing threshold, frequency specific testing, and automation of response detection.


Assuntos
Doenças Auditivas Centrais/diagnóstico , Cóclea/fisiologia , Triagem Neonatal , Núcleo Olivar/fisiologia , Emissões Otoacústicas Espontâneas , Doenças Auditivas Centrais/complicações , Doenças Auditivas Centrais/fisiopatologia , Vias Auditivas , Cóclea/inervação , Perda Auditiva Neurossensorial/diagnóstico , Testes Auditivos , Humanos , Hiperbilirrubinemia Neonatal/complicações , Lactente , Recém-Nascido
11.
Rev. logop. foniatr. audiol. (Ed. impr.) ; 30(4): 206-212, oct.-dic. 2010.
Artigo em Espanhol | IBECS | ID: ibc-128972

RESUMO

El trastorno de neuropatía auditiva (ANSD) es un tipo de disfunción auditiva en la que los procesos periféricos pueden ser normales, pero la transmisión neural a través del par craneal VIII y el tronco encefálico auditivo está perturbada. Las consecuencias perceptivas del ANSD difieren de las pérdidas auditivas cocleares y, por lo general, incluyen distorsión de las claves temporales (sincronización) y discriminación de frecuencias alterada. Estos déficit básicos de procesamiento pueden, a su vez, afectar a la audición funcional, lo que da lugar a deficiencias graves en la percepción del habla, sobre todo en la presencia de ruido de fondo. Este artículo abordará los mecanismos subyacentes en el ANSD, ilustrará sus efectos mediante un detallado estudio de caso y presentará pruebas sobre el uso de un sistema FM personal en un oyente afectado (AU)


Auditory neuropathy spectrum disorder (ANSD) is a form of auditory dysfunction in which peripheral processes can be normal, but neural transmission through the VIIIth nerve and auditory brainstem is disrupted. The perceptual consequences of ANSD are distinct from those of cochlear hearing loss and most commonly include distortion of temporal (timing) cues and altered frequency discrimination. These basic processing deficits can, in turn, affect functional hearing, resulting in severe impairment of speech perception particularly in the presence of background noise. This article will address the mechanisms underlying ANSD, illustrate it's effects in a detailed case study and show evidence for the use of personal FM devices in affected listeners (AU)


Assuntos
Humanos , Masculino , Feminino , Audiologia/métodos , Fonoaudiologia/tendências , Doenças Auditivas Centrais/complicações , Doenças Auditivas Centrais/diagnóstico , Doenças Auditivas Centrais/psicologia , Testes Auditivos/métodos , Doenças do Nervo Vestibulococlear/complicações , Doenças do Nervo Vestibulococlear/diagnóstico , Transtornos da Articulação/complicações , Transtornos da Articulação/diagnóstico , Doenças do Nervo Vestibulococlear/psicologia , Implantes Cocleares/psicologia , Transtornos da Articulação/psicologia , Audiometria da Fala/métodos , Audiometria da Fala/tendências , Audiometria da Fala , Transtornos do Desenvolvimento da Linguagem/psicologia , Testes de Articulação da Fala , Distúrbios da Fala/complicações , Distúrbios da Fala/diagnóstico
12.
Mol Vis ; 16: 650-64, 2010 Apr 13.
Artigo em Inglês | MEDLINE | ID: mdl-20405026

RESUMO

PURPOSE: To define the phenotype and elucidate the molecular basis for an autosomal recessively inherited optic atrophy and auditory neuropathy in a consanguineous family with two affected children. METHODS: Family members underwent detailed ophthalmologic, electrophysiological, and audiological assessments. An autozygosity mapping strategy using high-density single nucleotide polymorphism microarrays and microsatellite markers was used to detect regions of genome homozygosity that might contain the disease gene. Candidate genes were then screened for mutations by direct sequencing. RESULTS: Both affected subjects had poor vision from birth and complained of progressive visual loss over time. Current visual acuity ranged from 6/60 to 6/120. Fundus examination revealed bilateral temporal optic nerve pallor in both patients with otherwise normal retinal findings. International-standard full-field electroretinograms were normal in both individuals, with no evidence of generalized retinal dysfunction. Pattern cortical visual evoked potentials were grossly abnormal bilaterally in both cases. The pattern electroretinogram N95:P50 ratio was subnormal, and the P50 was of shortened peak time bilaterally in both patients. The electrophysiological findings were consistent with bilateral retinal ganglion cell/optic nerve dysfunction. Audiological investigation in both siblings revealed abnormalities falling within the auditory neuropathy/dysynchrony spectrum. There were no auditory symptoms and good outer hair cell function (as demonstrated by transient evoked otoacoustic emissions) but impaired inner hair cell/neural function with abnormal stapedial reflex thresholds and abnormal or absent auditory brainstem-evoked responses. The single nucleotide polymorphism microarray data demonstrated a 24.17 Mb region of homozygosity at 11q14.1-11q22.3, which was confirmed by microsatellite marker analysis. The candidate target region contained the transmembrane protein 126A (TMEM126A) gene, and direct sequencing identified a previously described nonsense mutation (c.163C>T; p.Arg55X). CONCLUSIONS: We describe the first detailed phenotyping of patients with autosomal recessive TMEM126A-associated optic atrophy and auditory neuropathy. These findings will facilitate the identification of individuals with this recently described disorder.


Assuntos
Doenças Auditivas Centrais/complicações , Doenças Auditivas Centrais/genética , Códon sem Sentido/genética , Genes Recessivos/genética , Proteínas de Membrana/genética , Atrofias Ópticas Hereditárias/complicações , Atrofias Ópticas Hereditárias/genética , Adolescente , Audiometria de Tons Puros , Doenças Auditivas Centrais/fisiopatologia , Limiar Auditivo/fisiologia , Sequência de Bases , Análise Mutacional de DNA , Eletrorretinografia , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia , Potenciais Evocados Visuais/fisiologia , Feminino , Fundo de Olho , Ligação Genética , Humanos , Masculino , Dados de Sequência Molecular , Fibras Nervosas/patologia , Atrofias Ópticas Hereditárias/fisiopatologia , Emissões Otoacústicas Espontâneas/fisiologia , Linhagem , Neurônios Retinianos/patologia , Adulto Jovem
13.
Behav Brain Res ; 209(1): 123-30, 2010 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-20109499

RESUMO

The left auditory cortex (AC) in humans is involved in the processing of the temporal parameters of acoustical signals, specifically in speech perception, whereas the right AC plays the dominant role in pitch and melody perception. The hemispheric lateralization of acoustical signal processing in non-human mammals is less explored. The present study examined the ability of rats to detect or discriminate a series of gaps in continuous noise under conditions of unilateral or bilateral reversible inactivation of the AC. The results showed that muscimol-induced reversible inactivation of the left AC suppresses the ability of rats to discriminate between acoustical stimuli of different temporal parameters (duration or repetition rate), whereas inactivation of the right AC results in no change or only a mild decrease in discrimination ability. Hemispheric asymmetry was observed only in the case of gap discrimination tasks, but not in a gap detection task. Our findings demonstrate that, similarly as in humans, the left AC in the rat plays the dominant role in temporal discrimination. These data provide further evidence for the functional asymmetry of the mammalian brain, which appears in a relatively early phase of evolution.


Assuntos
Doenças Auditivas Centrais/complicações , Transtornos da Percepção Auditiva/etiologia , Discriminação Psicológica/fisiologia , Lateralidade Funcional/fisiologia , Estimulação Acústica/métodos , Animais , Córtex Auditivo/efeitos dos fármacos , Córtex Auditivo/fisiologia , Doenças Auditivas Centrais/induzido quimicamente , Condicionamento Clássico/fisiologia , Modelos Animais de Doenças , Eletrochoque/efeitos adversos , Feminino , Agonistas GABAérgicos/efeitos adversos , Muscimol/efeitos adversos , Ratos
14.
Acta Otorrinolaringol Esp ; 60(6): 415-21, 2009.
Artigo em Espanhol | MEDLINE | ID: mdl-19909717

RESUMO

OBJECTIVES: The aim is to assess the ability to discriminate words, using two psychoacoustic verbal tests of central auditory processes in Spanish: Binaural Fusion Test (BFT in its Spanish version) and Filtered Word Test (FWT in its Spanish version) in children with dyslexia and controls. METHODS: One group of 40 dyslexic children was receiving therapy for dyslexia at the time of the tests. 40 children without dyslexia were selected as controls, out of 298 children who attended a public school. RESULTS: The rate of males to females was 2/1 in the dyslexic group. The average correct answers for the BFT were 65-66% in dyslexic group and 75-80% in the control group. For the FWT they were 50-54% in the dyslexic group and 67-71% in the control group (student t <0.05). CONCLUSIONS: These results contribute to make evident disorders in central auditory processing in children with dyslexia. We suggest using the tests with each patient in order to elaborate a rehabilitation plan.


Assuntos
Doenças Auditivas Centrais/diagnóstico , Dislexia/fisiopatologia , Transtornos do Desenvolvimento da Linguagem/diagnóstico , Testes de Discriminação da Fala , Doenças Auditivas Centrais/complicações , Doenças Auditivas Centrais/fisiopatologia , Transtornos da Percepção Auditiva/complicações , Transtornos da Percepção Auditiva/diagnóstico , Transtornos da Percepção Auditiva/fisiopatologia , Estudos de Casos e Controles , Criança , Estudos Transversais , Dislexia/complicações , Feminino , Humanos , Idioma , Transtornos do Desenvolvimento da Linguagem/complicações , Transtornos do Desenvolvimento da Linguagem/fisiopatologia , Masculino , Reconhecimento Fisiológico de Modelo , Estudos Prospectivos , Espanha , Percepção da Fala
15.
J Am Acad Audiol ; 20(10): 621-8, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-20503800

RESUMO

BACKGROUND: The relation between the auditory steady-state response (ASSR) and behavioral audiometric thresholds requires further clarification in the case of adults with auditory neuropathy/auditory dys-synchrony (AN/AD). PURPOSE: The aim of this study was to compare pure-tone audiometric threshold (PTAT) and ASSR in adults with AN/AD. STUDY SAMPLE: Sixteen adult participants (32 ears) with AN/AD, ranging in age from 14 to 34 years. DATA COLLECTION AND ANALYSIS: PTAT and ASSR with high-rate stimulus modulation were measured at four octave frequencies, 500, 1000, 2000, and 4000 Hz, in each ear. The behavioral auditory thresholds were compared with ASSR estimated thresholds at each frequency. Analyses included comparison of group means and coefficients of correlation. RESULTS: The average pure-tone thresholds revealed a moderate hearing loss in the AN/AD patients with a focus on the low frequencies. Low-frequency loss audiograms were observed in almost two-thirds of the participants. The estimated auditory thresholds measured by ASSR at all frequencies were substantially higher than the PTAT measures. There were no significant correlations between the PTAT and ASSR measurements at the 1000, 2000, and 4000 Hz frequencies (p > .05); the correlation between the two measures at 500 Hz (p = .029, r = 0.39) was weak but significant. CONCLUSION: There was no significant correlation between the PTAT and ASSR results at the majority of the frequencies usually tested in adults with AN/AD. Although ASSR is not a suitable method to estimate auditory thresholds in this group of patients, perhaps it can be utilized as an adjunct technique for the differential diagnosis of this disorder.


Assuntos
Audiometria de Resposta Evocada/métodos , Doenças Auditivas Centrais/diagnóstico , Limiar Auditivo/fisiologia , Potenciais Evocados Auditivos/fisiologia , Perda Auditiva/fisiopatologia , Adolescente , Adulto , Audiometria de Tons Puros , Doenças Auditivas Centrais/complicações , Doenças Auditivas Centrais/fisiopatologia , Feminino , Perda Auditiva/diagnóstico , Perda Auditiva/etiologia , Humanos , Masculino , Valor Preditivo dos Testes , Reprodutibilidade dos Testes , Adulto Jovem
16.
Braz J Otorhinolaryngol ; 74(4): 606-12, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18852990

RESUMO

UNLABELLED: The association between hearing and balance disorders with migraine is known since the times of the ancient Greeks, when Aretaeus from Cappadocia in 131 B.C, made an accurate and detailed description of this occurrence during a migraine episode. We present a broad review of migraine neurotological manifestations, using the most recent publications associated with epidemiology, clinical presentation, pathophysiology, diagnostic methods and treatment for this syndrome. AIM: To describe the clinical entity: "Migraine associated with auditory-vestibular dysfunction" in order to help otorhinolaryngologists and neurologists in the diagnosis and management of such disorder. FINAL REMARKS: There is a strong association between neurotological symptoms and migraine, and the auditory-vestibular dysfunction-associated migraine is the most common cause of spontaneous episodic vertigo (non-positional). Symptoms may vary broadly among patients, making it a diagnostic challenge to the otorhinolaryngologist. This entity usually presents with positional or spontaneous vertigo spells, lasting for seconds or days, associated with migraine symptoms. A better understanding of the relationship between central vestibular mechanisms and migraine mechanisms, besides the discovery of ionic channel disorders in some cases of migraine, ataxia and vertigo, may lead to a better understanding of migraine pathophysiology associated with audio-vestibular disorder.


Assuntos
Doenças Auditivas Centrais/complicações , Transtornos de Enxaqueca/complicações , Doenças Vestibulares/complicações , Doenças Auditivas Centrais/diagnóstico , Doenças Auditivas Centrais/terapia , Diagnóstico Diferencial , Humanos , Transtornos de Enxaqueca/diagnóstico , Transtornos de Enxaqueca/terapia , Síndrome , Doenças Vestibulares/diagnóstico , Doenças Vestibulares/terapia
17.
Praxis (Bern 1994) ; 97(14): 765-9, 2008 Jul 09.
Artigo em Alemão | MEDLINE | ID: mdl-18717457

RESUMO

Coxsackieviruses are responsible for numerable diseases in man. This is also the reason for the high prevalence of endemic infection rates in the population. Our analysis (working hypothesis) will focus on the participation of Coxsackieviruses in chronic decompensated, complex tinnitus. Examination of the Coxsackievirus antibody titers might reveal the extent to which a Coxsackieviruses-triggered disease of the central nervous system participates in the direct sequelae of tinnitus disorders. A spread of Coxsackieviruses to the auditory pathway might lead to an overstimulation of the auditory pathway, comparable to an epileptic lesion. Based on this assumption, treatment with an antiepileptic would make sense. The reasoning behind this working hypothesis is to find a potentially new diagnostic and therapeutic roadmap as a further guide for specialized clinics. The authors are well aware that previous results bear little relevance as they have been based on small case numbers.


Assuntos
Anticonvulsivantes/uso terapêutico , Doenças Auditivas Centrais/diagnóstico , Vias Auditivas , Infecções por Coxsackievirus/diagnóstico , Enterovirus Humano B , Zumbido/tratamento farmacológico , Zumbido/etiologia , Idoso , Doenças Auditivas Centrais/complicações , Doença Crônica , Infecções por Coxsackievirus/complicações , Diagnóstico Diferencial , Humanos , Masculino , Pleurodinia Epidêmica/complicações , Pleurodinia Epidêmica/diagnóstico
18.
Rev. bras. otorrinolaringol ; 74(4): 606-612, jul.-ago. 2008. ilus, tab
Artigo em Inglês, Português | LILACS | ID: lil-494432

RESUMO

A associação de distúrbios da audição e equilíbrio com enxaqueca é reconhecida desde a Grécia antiga quando Aretaeus da Capadócia em 131 a.C., fez uma descrição precisa e com detalhes desta ocorrência durante uma crise de enxaqueca. Uma revisão ampla das manifestações otoneurológicas da enxaqueca é apresentada, usando as mais recentes publicações com respeito à epidemiologia, apresentação clínica, fisiopatologia, métodos diagnósticos e manejo desta síndrome. OBJETIVO: Descrever a entidade clínica "Enxaqueca associada a Disfunção Auditivo-vestibular" no intuito de ajudar médicos otorrinolaringologistas e neurologistas no diagnóstico e no manejo clínico dessa doença. COMENTÁRIOS FINAIS: Uma forte associação existe entre sintomas otoneurológicos e enxaqueca, sendo a enxaqueca associada a disfunção auditivo-vestibular a causa mais comum de vertigem episódica espontânea (não-posicional). Os sintomas podem variar bastante entre pacientes tornando um desafio diagnóstico para o otorrinolaringologista. Esta entidade geralmente se apresenta com ataques de vertigem espontâneos ou posicionais, durando de segundos a dias com sintomas de enxaqueca associados. Uma melhor elucidação da ligação entre os mecanismos vestibulares centrais e os mecanismos da enxaqueca em si, além da descoberta de defeitos em canais iônicos em algumas causas de enxaqueca, ataxia e vertigem, podem levar a um entendimento maior da fisiopatologia da enxaqueca associada a disfunção auditivo-vestibular.


The association between hearing and balance disorders with migraine is known since the times of the ancient Greeks, when Aretaeus from Cappadocia in 131 B.C, made an accurate and detailed description of this occurrence during a migraine episode. We present a broad review of migraine neurotological manifestations, using the most recent publications associated with epidemiology, clinical presentation, pathophysiology, diagnostic methods and treatment for this syndrome. AIM: to describe the clinical entity: "Migraine associated with auditory-vestibular dysfunction" in order to help otorhinolaryngologists and neurologists in the diagnosis and management of such disorder. FINAL REMARKS: There is a strong association between neurotological symptoms and migraine, and the auditory-vestibular dysfunction-associated migraine is the most common cause of spontaneous episodic vertigo (non-positional). Symptoms may vary broadly among patients, making it a diagnostic challenge to the otorhinolaryngologist. This entity usually presents with positional or spontaneous vertigo spells, lasting for seconds or days, associated with migraine symptoms. A better understanding of the relationship between central vestibular mechanisms and migraine mechanisms, besides the discovery of ionic channel disorders in some cases of migraine, ataxia and vertigo, may lead to a better understanding of migraine pathophysiology associated with audio-vestibular disorder.


Assuntos
Humanos , Doenças Auditivas Centrais/complicações , Transtornos de Enxaqueca/complicações , Doenças Vestibulares/complicações , Doenças Auditivas Centrais/diagnóstico , Doenças Auditivas Centrais/terapia , Diagnóstico Diferencial , Transtornos de Enxaqueca/diagnóstico , Transtornos de Enxaqueca/terapia , Síndrome , Doenças Vestibulares/diagnóstico , Doenças Vestibulares/terapia
19.
Pró-fono ; 19(4): 352-356, out.-dez. 2007. tab
Artigo em Inglês, Português | LILACS | ID: lil-471305

RESUMO

TEMA: potenciais evocados auditivos de longa latência. OBJETIVO: caracterizar os potenciais evocados auditivos de longa latência (Peall) de indivíduos com HIV/Aids comparando com os obtidos no grupo controle. MÉTODO: a casuística foi composta por 21 indivíduos com HIV/Aids pertencentes ao grupo pesquisa (14 do gênero masculino e sete do gênero feminino) com idade entre 31 e 48 anos e 21 indivíduos saudáveis pertencentes ao grupo controle (cinco do gênero masculino e 16 do gênero feminino) com idade entre 19 e 36 anos. Foram analisados os valores de latência e amplitude da onda P300, latência das ondas N1 e P2 e amplitude N1-P2. Os eletrodos foram colocados nas posições A1, A2, Cz e Fpz. RESULTADOS: no P300 observou-se que o grupo com HIV/Aids apresentou maiores valores de latência (p-valor = 0,010) e menores de amplitude (p-valor = 0,021) quando comparados com o grupo controle. Na análise do complexo N1-P2, ao comparar os grupos, verificou-se que o grupo pesquisa apresentou maiores valores de latência tanto para a onda N1 (p-valor = 0,035) como para a onda P2, porém esta última sem diferença estatisticamente significante. Com relação à análise da amplitude N1-P2, verificou-se que o grupo controle apresentou maiores valores, sendo esta diferença estatisticamente significante quando comparada ao grupo pesquisa. CONCLUSÃO: os achados do presente estudo mostraram que indivíduos com HIV/Aids apresentam alterações nos Peall, sugerindo comprometimento nas áreas corticais do sistema auditivo e mostrando a importância destes testes na avaliação audiológica de indivíduos com HIV/Aids.


BACKGROUND: Long Latency Auditory Evoked Potentials. AIM: to characterize the Long Latency Auditory Evoked Potentials (LLAEP) in individuals with HIV/AIDS in comparison to a control group. METHOD: the research sample was composed by 21 individuals with HIV/AIDS - research group (14 male and 7 female), with ages ranging from 31 to 48 years, and 21 healthy individuals - control group (5 male and 16 female), with ages ranging from 19 to 36 years. The latency and amplitude values of the P300 wave were analyzed; latency of N1 and P2 waves, and amplitude N1-P2. The electrodes were placed on the following positions: A1, A2, Cz and Fpz. RESULTS: the T-student test was used to analyze the results and the adopted significance level was of 5 percent. In the analyzes of P300 it was observed that the group with HIV/AIDS presented greater latency values (p-value = 0,010) and lower amplitude values (p-value = 0,021) when compared to the control group. The analysis of the N1-P2 complex revealed that the research group presented higher latency values for both, N1 wave (p-value = 0,035) and P2 wave, however for this last one, there was no significant statistical difference when compared to the control group. Concerning the amplitude analysis of the N1-P2 complex, it was verified that the control group presented significantly higher values when compared to the research group. CONCLUSION: the findings of this study indicates that individuals with HIV/AIDS present alterations in the Long Latency Auditory Evoked Potentials (higher latencies and lower amplitudes of N1, P2 and P300 waves), suggesting a disorder in the cortical regions of the auditory pathway, and therefore stressing the importance of such tests in the evaluation of these individuals.


Assuntos
Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Auditivas Centrais/diagnóstico , /fisiologia , Potenciais Evocados Auditivos/fisiologia , Infecções por HIV/fisiopatologia , Estimulação Acústica , Síndrome da Imunodeficiência Adquirida/fisiopatologia , Doenças Auditivas Centrais/complicações , Estudos de Casos e Controles , Tempo de Reação
20.
Acta otorrinolaringol. esp ; 58(9): 401-407, nov. 2007. tab
Artigo em Es | IBECS | ID: ibc-057219

RESUMO

Objetivo: Comprobar si se produce traumatismo acústico en los músicos de música clásica. Material y método: Se estudió a 65 músicos voluntarios de la Orquesta Sinfónica de Castilla y León empleando las escalas de corrección para la presbiacusia ISO 7029:2000 y ELI. También se estudió las intensidades que emiten los distintos instrumentos que componen una orquesta. Resultados: Comprobamos que las intensidades que los músicos soportan se encuentran por encima de lo que la ley establece como riesgo para la audición, que el porcentaje de músicos con pérdida auditiva en 4.000 Hz supone más del doble de lo esperado para la edad en el percentil 5, y que el oído izquierdo es el más afectado en los instrumentistas de violín y viola. Conclusiones: La música clásica produce un traumatismo acústico que se debería reconocer como enfermedad profesional. Los músicos están obligados a protegerse los oídos y a realizarse revisiones periódicas


Objective: To confirm the existence of acoustic trauma in classical musicians. Material and method: Sixty-five volunteers from the Castilla and León Symphony Orchestra were studied. The hearing thresholds of each musician were age-corrected using the ELI and ISO 7029:2000 scales. Furthermore the sound levels of the instruments in this symphony orchestra were studied. Results: We observed that the sound level of the symphony orchestra instruments is higher than the level permitted by law, ie it constitutes a risk for hearing loss. We also found that 4 kHz hearing loss in the 5th percentile among musicians was double the rate that would be expected for age, and that violinists and viola players showed poorer hearing in the left ear. Conclusions: Classical music causes acoustic trauma in musicians and should be recognized as a professional illness. Musicians are obliged to protect their hearing and to undergo regular check-ups


Assuntos
Masculino , Feminino , Adulto , Humanos , Perda Auditiva Provocada por Ruído/diagnóstico , Perda Auditiva Provocada por Ruído/epidemiologia , Doenças Auditivas Centrais/complicações , Doenças Profissionais/diagnóstico , Doenças Profissionais/epidemiologia , Música , Audiometria/métodos
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