Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 394
Filtrar
1.
Am J Med Genet A ; 185(4): 1131-1141, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33529473

RESUMO

Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive multiple congenital malformation and intellectual disability syndrome resulting from variants in DHCR7. Auditory characteristics of persons with SLOS have been described in limited case reports but have not been systematically evaluated. The objective of this study is to describe the auditory phenotype in SLOS. Age- and ability-appropriate hearing evaluations were conducted on 32 patients with SLOS. A subset of 21 had auditory brainstem response testing, from which an auditory neural phenotype is described. Peripheral or retrocochlear auditory dysfunction was observed in at least one ear of 65.6% (21) of the patients in our SLOS cohort. The audiometric phenotype was heterogeneous and included conductive, mixed, and sensorineural hearing loss. The most common presentation was a slight to mild conductive hearing loss, although profound sensorineural hearing loss was also observed. Abnormal auditory brainstem responses indicative of retrocochlear dysfunction were identified in 21.9% of the patients. Many were difficult to test behaviorally and required objective assessment methods to estimate hearing sensitivity. Individuals with SLOS are likely to have hearing loss that may impact communication, including speech and language development. Routine audiologic surveillance should be conducted to ensure prompt management of hearing loss.


Assuntos
Doenças Auditivas Centrais/genética , Predisposição Genética para Doença , Perda Auditiva Neurossensorial/genética , Síndrome de Smith-Lemli-Opitz/diagnóstico , Adolescente , Adulto , Audiometria , Doenças Auditivas Centrais/fisiopatologia , Criança , Pré-Escolar , Nervo Coclear/fisiopatologia , Potenciais Evocados Auditivos do Tronco Encefálico/genética , Feminino , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Lactente , Masculino , Mutação/genética , Oxirredutases atuantes sobre Doadores de Grupo CH-CH/genética , Fenótipo , Síndrome de Smith-Lemli-Opitz/genética , Síndrome de Smith-Lemli-Opitz/fisiopatologia , Adulto Jovem
2.
Int J Mol Sci ; 21(19)2020 Sep 29.
Artigo em Inglês | MEDLINE | ID: mdl-33003463

RESUMO

Aging is associated with functional and morphological changes in the sensory organs, including the auditory system. Mitophagy, a process that regulates the turnover of dysfunctional mitochondria, is impaired with aging. This study aimed to investigate the effect of aging on mitophagy in the central auditory system using an age-related hearing loss mouse model. C57BL/6J mice were divided into the following four groups based on age: 1-, 6-, 12-, and 18-month groups. The hearing ability was evaluated by measuring the auditory brainstem response (ABR) thresholds. The mitochondrial DNA damage level and the expression of mitophagy-related genes, and proteins were investigated by real-time polymerase chain reaction and Western blot analyses. The colocalization of mitophagosomes and lysosomes in the mouse auditory cortex and inferior colliculus was analyzed by immunofluorescence analysis. The expression of genes involved in mitophagy, such as PINK1, Parkin, and BNIP3 in the mouse auditory cortex and inferior colliculus, was investigated by immunohistochemical staining. The ABR threshold increased with aging. In addition to the mitochondrial DNA integrity, the mRNA levels of PINK1, Parkin, NIX, and BNIP3, as well as the protein levels of PINK1, Parkin, BNIP3, COX4, LC3B, mitochondrial oxidative phosphorylation (OXPHOS) subunits I-IV in the mouse auditory cortex significantly decreased with aging. The immunofluorescence analysis revealed that the colocalization of mitophagosomes and lysosomes in the mouse auditory cortex and inferior colliculus decreased with aging. The immunohistochemical analysis revealed that the expression of PINK1, Parkin, and BNIP3 decreased in the mouse auditory cortex and inferior colliculus with aging. These findings indicate that aging-associated impaired mitophagy may contribute to the cellular changes observed in an aged central auditory system, which result in age-related hearing loss. Thus, the induction of mitophagy can be a potential therapeutic strategy for age-related hearing loss.


Assuntos
Envelhecimento/genética , Mitocôndrias/genética , Mitofagia/genética , Presbiacusia/genética , Envelhecimento/patologia , Animais , Doenças Auditivas Centrais/genética , Doenças Auditivas Centrais/fisiopatologia , DNA Mitocondrial/genética , Modelos Animais de Doenças , Potenciais Evocados Auditivos do Tronco Encefálico/genética , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia , Lisossomos/genética , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Mitocôndrias/metabolismo , Fosforilação Oxidativa , Presbiacusia/fisiopatologia
3.
Rev. otorrinolaringol. cir. cabeza cuello ; 80(3): 344-351, set. 2020. graf
Artigo em Espanhol | LILACS | ID: biblio-1144899

RESUMO

Resumen La hipoacusia unilateral es una alteración prevalente e importante en la población infantil que puede llevar a un deterioro del procesamiento auditivo. Cualquier patología que cause hipoacusia asimétrica (conductiva o sensorioneural) durante ventanas críticas del desarrollo cerebral, puede dar lugar a déficits del procesamiento auditivo que podrían persistir incluso después de la rehabilitación audiológica. Este proceso fisiopatológico se ha denominado "ambliaudia" y los niños que la presentan tienen un mayor riesgo de sufrir retraso en el desarrollo del lenguaje, así como de padecer dificultades académicas, sociales y cognitivas. Por esto, la ambliaudia se presenta como un desafío en audiología, siendo necesario comprender los mecanismos neurobiológicos que la subyacen. Entender de mejor manera cuáles son las consecuencias que tiene la pérdida auditiva asimétrica sobre el desarrollo del sistema nervioso podría ser clave para guiar de mejor forma las intervenciones terapéuticas. En este artículo se revisan trabajos en humanos y en modelos animales que describen las consecuencias neurobiológicas de la deprivación auditiva unilateral durante el desarrollo del sistema nervioso y su posible aplicación a la práctica clínica.


Abstract Unilateral hearing loss is a prevalent and important disorder in children that can lead to an impairment of auditory processing. Any pathology that causes asymmetric (conductive or sensorineural) hearing loss during critical brain development windows can lead to hearing deficits that may persist even after audiological rehabilitation. This pathophysiological process has been referred to as amblyaudia, and children are at greater risk of experiencing language developmental delays as well as academic, social and cognitive impairments. For this reason, amblyaudia presents itself as a challenge in audiology, and it has become necessary to understand its neurobiological mechanisms. A better understanding of the consequences of asymmetric hearing loss on the development of the nervous system may be key to better guiding therapeutic interventions. This article reviews studies performed in humans and animal models that describe the neurobiological consequences of unilateral hearing deprivation during the development of the nervous system and its possible applications to clinical practice.


Assuntos
Humanos , Animais , Criança , Transtornos da Percepção Auditiva/complicações , Perda Auditiva Unilateral/complicações , Doenças Auditivas Centrais , Vias Auditivas/anormalidades , Transtornos do Desenvolvimento da Linguagem/etiologia
4.
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
5.
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
6.
Am J Audiol ; 29(2): 152-164, 2020 Jun 08.
Artigo em Inglês | MEDLINE | ID: mdl-32182128

RESUMO

Purpose Complaints of auditory perceptual deficits, such as tinnitus and difficulty understanding speech in background noise, among individuals with clinically normal audiograms present a perplexing problem for audiologists. One potential explanation for these "hidden" auditory deficits is loss of the synaptic connections between the inner hair cells and their afferent auditory nerve fiber targets, a condition that has been termed cochlear synaptopathy. In animal models, cochlear synaptopathy can occur due to aging or exposure to noise or ototoxic drugs and is associated with reduced auditory brainstem response (ABR) wave I amplitudes. Decreased ABR wave I amplitudes have been demonstrated among young military Veterans and non-Veterans with a history of firearm use, suggesting that humans may also experience noise-induced synaptopathy. However, the downstream consequences of synaptopathy are unclear. Method To investigate how noise-induced reductions in wave I amplitude impact the central auditory system, the ABR, the middle latency response (MLR), and the late latency response (LLR) were measured in 65 young Veterans and non-Veterans with normal audiograms. Results In response to a click stimulus, the MLR was weaker for Veterans compared to non-Veterans, but the LLR was not reduced. In addition, low ABR wave I amplitudes were associated with a reduced MLR, but with an increased LLR. Notably, Veterans reporting tinnitus showed the largest mean LLRs. Conclusions These findings indicate that decreased peripheral auditory input leads to compensatory gain in the central auditory system, even among individuals with normal audiograms, and may impact auditory perception. This pattern of reduced MLR, but not LLR, was observed among Veterans even after statistical adjustment for sex and distortion product otoacoustic emission differences, suggesting that synaptic loss plays a role in the observed central gain. Supplemental Material https://doi.org/10.23641/asha.11977854.


Assuntos
Doenças Auditivas Centrais/fisiopatologia , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia , Ruído Ocupacional , Zumbido/fisiopatologia , Veteranos , Adulto , Audiometria de Tons Puros , Vias Auditivas/fisiopatologia , Estudos de Casos e Controles , Potenciais Evocados Auditivos/fisiologia , Feminino , Células Ciliadas Auditivas Internas , Humanos , Masculino , Emissões Otoacústicas Espontâneas/fisiologia , Sinapses , Adulto Jovem
8.
Undersea Hyperb Med ; 46(3): 245-249, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31394595

RESUMO

Functional magnetic resonance imaging (fMRI) has been available commercially for clinical diagnostic use for many years. However, both clinical interpretation of fMRI by a neuroradiologist and quantitative analysis of fMRI data can require significant personnel resources that exceed reimbursement. In this report, a fully automated computer-based quantification methodology (Enumerated Auditory Response, EAR) has been developed to provide an auditory fMRI assessment of patients who have suffered a mild traumatic brain injury. Fifty-five study participants with interpretable auditory fMRI sequence data were assessed by EAR analysis, as well as both clinical radiologist fMRI interpretation and voxelwise general linear model (GLM) analysis. Comparison between the clinical interpretation and the two computer analysis methods resulted in 67% concordance (identical), 32% nearconcordance (one level difference), and 1% discordant. Comparison between the clinical computer-based quantification (EAR) and GLM analysis yielded significant correlations in right and left ear responses (p⟨0.05) for the full subject group. Automated fMRI quantification analysis equivalent to EAR might be appropriate for both future research projects with constrained resources, as well as possible routine clinical use.


Assuntos
Doenças Auditivas Centrais/diagnóstico por imagem , Concussão Encefálica/fisiopatologia , Diagnóstico por Computador/métodos , Técnicas de Diagnóstico Otológico , Imageamento por Ressonância Magnética/métodos , Doenças Auditivas Centrais/fisiopatologia , Concussão Encefálica/diagnóstico por imagem , Feminino , Humanos , Modelos Lineares , Masculino , Militares , Veteranos
9.
Undersea Hyperb Med ; 46(3): 261-269, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31394597

RESUMO

Auditory processing disorders are common following mild traumatic brain injury (mTBI), but the neurocircuitry involved is not well understood. The present study used functional MRI to examine auditory cortex activation patterns during a passive listening task in a normative population and mTBI patients with and without clinical central auditory processing deficits (APD) as defined by the SCAN-3:A clinical battery. Patients with mTBI had overall patterns of lower auditory cortex activation during the listening tasks as compared to normative controls. A significant lateralization pattern (pairwise t-test; p⟨0.05) was observed in normative controls and in those with mTBI and APD during single-side stimulation. Additionally, baseline connectivity between left and right auditory cortices was lower in mTBI patients than in controls (p=0.01) and significantly reduced in the mTBI with APD group (p=0.008). Correlation was also observed between bilateral task-related activation and competing words subscore of the SCAN-3:A. These findings suggest the passive listening task is well suited to probe auditory function in military personnel with an mTBI diagnosis. Further, the study supports the use of multiple approaches for detecting and assessing central auditory deficits to improve monitoring of short- and long-term outcomes.


Assuntos
Córtex Auditivo/fisiopatologia , Doenças Auditivas Centrais/fisiopatologia , Vias Auditivas/fisiopatologia , Concussão Encefálica/fisiopatologia , Imageamento por Ressonância Magnética , Adolescente , Adulto , Idoso , Análise de Variância , Córtex Auditivo/diagnóstico por imagem , Doenças Auditivas Centrais/diagnóstico , Doenças Auditivas Centrais/etiologia , Vias Auditivas/lesões , Concussão Encefálica/complicações , Estudos de Casos e Controles , Feminino , Audição/fisiologia , Testes Auditivos , Humanos , Processamento de Imagem Assistida por Computador , Masculino , Pessoa de Meia-Idade , Militares , Testes de Função Vestibular , Veteranos , Adulto Jovem
10.
Am J Audiol ; 28(2S): 471-482, 2019 Aug 28.
Artigo em Inglês | MEDLINE | ID: mdl-31461331

RESUMO

Purpose Adults with stuttering (AWS) exhibit compromised phonological working memory abilities, poor central auditory processing, and impaired auditory processing especially during overt speech production tasks. However, these tasks are sensitive to language disturbances already found in them. Thus, in this study, monosyllables were used ruling out the language effects, and auditory working memory ability was evaluated in AWS using the n-back task. In specific, the auditory sensory input of the working memory mechanism was evaluated. Method Thirty-two participants, 16 each of AWS and adults with no stuttering (AWNS), performed behavioral auditory 1-back and 2-back tasks. The long latency responses were also recorded during no-back and 2-back conditions from 64 electrode sites. Results Results revealed no significant differences between the groups in any of the behavioral parameters such as reaction time, accuracy, false alarm rate, or d'. N1 amplitude modulation was noted in AWNS, which was absent in AWS. The segmentation analysis showed a left hemisphere-oriented topographical distribution in the N2 region in AWS irrespective of conditions, whereas the scalp topography was right hemisphere-oriented with the involvement of parietal channels in AWNS. The timing differences existed between AWS and AWNS in the intervals that a topographical distribution lasted in all throughout the time window of analysis. Conclusion The results suggest altered neural pathway and hemispheric differences during auditory working memory tasks in AWS.


Assuntos
Doenças Auditivas Centrais/fisiopatologia , Córtex Cerebral/fisiopatologia , Memória de Curto Prazo/fisiologia , Gagueira/fisiopatologia , Adolescente , Adulto , Estudos de Casos e Controles , Cognição , Eletroencefalografia , Feminino , Humanos , Masculino , Fonética , Tempo de Reação , Fala , Adulto Jovem
11.
Buenos Aires; IECS; jul. 2019.
Não convencional em Espanhol | BRISA/RedTESA | ID: biblio-1178239

RESUMO

CONTEXTO CLÍNICO: Una alteración en el lenguaje se refiere a un impedimento en la capacidad de recibir, enviar, processar y comprender conceptos o sistemas de símbolos verbales, no verbales y gráficos. El desarrollo normal de la comunicación requiere la interacción de un mecanismo intacto con un entorno favorable. Dentro de los componentes responsables de este mecanismo se incluyen la sensibilidad auditiva, percepción, inteligencia, integridad estructural, habilidad motora y estabilidad emocional. Los desórdenes del procesamiento auditivo central (DPAC) se definen como un déficit en el procesamiento de la información relacionada en forma específica a la modalidad auditiva, este déficit puede verse exacerbado en ambientes en donde las condiciones acústicas son desfavorables. Estos déficits pueden estar asociados a dificultades auditivas, de comprensión del habla, desarrollo del lenguaje y aprendizaje. Un DPAC es resultado de una disfunción en el procesamiento de la información auditiva pudiendo o no coexistir con una disfunción global que afecte el desempeño a través de otras modalidades (por ejemplo: déficit de atención, déficit en la representación del lenguaje, entre otros). La prevalencia de este desorden varía levemente según diferentes investigadores, los datos más recientes señalan este rango entre 2% y 3% de los niños a una razón de 2:1 entre hombre y mujeres. TECNOLOGÍA: El profesional a cargo debe seleccionar la batería de pruebas adecuadas para el diagnóstico de DPAC. La selección de éstas debe basarse en los hallazgos de la historia del caso, la evaluación interdisciplinaria (por ejemplo, los resultados del lenguaje y las evaluaciones cognitivas) y la evaluación audiológica periférica. La batería de pruebas se puede usar para diagnosticar una alteración en uno o más processos neurofisiológicos que ocurren cuando las señales auditivas viajan a través del SNAC. La identificación de déficits específicos en los SNAC puede llevar a recomendaciones específicas y planes de tratamiento. OBJETIVO: El objetivo del presente informe es evaluar la evidencia disponible acerca de la capacidad diagnóstica y los aspectos relacionados a las políticas de cobertura del uso de las pruebas diagnósticas en el desorden del procesamiento auditivo central. MÉTODOS: Se realizó una búsqueda en las principales bases de datos bibliográficas, en buscadores genéricos de internet, y financiadores de salud. Se priorizó la inclusión de revisiones sistemáticas (RS), ensayos clínicos controlados aleatorizados (ECAs), evaluaciones de tecnologías sanitarias (ETS), evaluaciones económicas, guías de práctica clínica (GPC) y políticas de cobertura de diferentes sistemas de salud. RESULTADOS: Se incluyeron una RS, siete GPC y siete informes de políticas de cobertura sobre pruebas diagnósticas para el desorden del procesamiento auditivo central. CONCLUSIONES: Evidencia de baja calidad sugiere que las pruebas diagnósticas utilizadas actualmente en el desorden del procesamiento auditivo central (DPAC) podrían ser de utilidad para su abordaje diagnóstico. No se hallaron estudios que evalúen la precisión diagnóstica de un conjunto de pruebas en pacientes con sospecha de DPAC. Las guías de práctica clínica (GPC) relevadas expresan que no existe un patrón de oro para realizar el diagnóstico de DPAC, por lo tanto, la selección de las distintas pruebas debería ajustarse a las características y condiciones del paciente. Las mismas GPC recomiendan el uso de una batería de pruebas (de comportamiento o electrofisiológicas) para el abordaje diagnóstico del DPAC, debido a que en su conjunto mejorarían la precisión diagnóstica. Una GPC intersociedades de Europa recomienda una batería determinada de pruebas para iniciar el abordaje diagnóstico, constituído por la prueba de dígitos dicóticos, las de patrones de frecuencia y de duración, Gaps-In-Noise (GIN), prueba de habla en ruido, diferencia de umbrales binaurales enmascarados, y la prueba de inteligibilidad del habla pediátrica. Los financiadores públicos de salud que han sido relevados no contemplan la cobertura de las pruebas para el diagnóstico de DPAC. Dentro de los financiadores privados de salud de los Estados Unidos, dos no brindan cobertura ya que la consideran en fase experimental, mientras que otros dos otorgan cobertura para el abordaje diagnóstico del DPAC, y en esos casos, la batería de pruebas será seleccionada según criterio del profesional tratante.


Assuntos
Humanos , Doenças Auditivas Centrais/diagnóstico , Testes Auditivos/instrumentação , Avaliação da Tecnologia Biomédica , Análise Custo-Benefício
12.
J Neurophysiol ; 122(2): 451-465, 2019 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-31116647

RESUMO

The CHRNA7 gene that encodes the α7-subunit of the nicotinic acetylcholine receptor (α7-nAChR) has been associated with some autism spectrum disorders and other neurodevelopmental conditions characterized, in part, by auditory and language impairment. These conditions may include auditory processing disorders that represent impaired timing of neural activity, often accompanied by problems understanding speech. Here, we measure timing properties of sound-evoked activity via the auditory brainstem response (ABR) of α7-nAChR knockout mice of both sexes and wild-type colony controls. We find a significant timing delay in evoked ABR signals that represents midbrain activity in knockouts. We also examine spike-timing properties of neurons in the inferior colliculus, a midbrain nucleus that exhibits high levels of α7-nAChR during development. We find delays of evoked responses along with degraded spiking precision in knockout animals. We find similar timing deficits in responses of neurons in the superior paraolivary nucleus and ventral nucleus of the lateral lemniscus, which are brainstem nuclei thought to shape temporal precision in the midbrain. In addition, we find that other measures of temporal acuity including forward masking and gap detection are impaired for knockout animals. We conclude that altered temporal processing at the level of the brainstem in α7-nAChR-deficient mice may contribute to degraded spike timing in the midbrain, which may underlie the observed timing delay in the ABR signals. Our findings are consistent with a role for the α7-nAChR in types of neurodevelopmental and auditory processing disorders and we identify potential neural targets for intervention.NEW & NOTEWORTHY Disrupted signaling via the α7-nicotinic acetylcholine receptor (α7-nAChR) is associated with neurodevelopmental disorders that include impaired auditory processing. The underlying causes of dysfunction are not known but a common feature is abnormal timing of neural activity. We examined temporal processing of α7-nAChR knockout mice and wild-type controls. We found degraded spike timing of neurons in knockout animals, which manifests at the level of the auditory brainstem and midbrain.


Assuntos
Transtornos da Percepção Auditiva/fisiopatologia , Tronco Encefálico/fisiopatologia , Potenciais Evocados Auditivos do Tronco Encefálico/fisiologia , Percepção do Tempo/fisiologia , Receptor Nicotínico de Acetilcolina alfa7/deficiência , Animais , Doenças Auditivas Centrais/fisiopatologia , Transtorno do Espectro Autista/fisiopatologia , Modelos Animais de Doenças , Feminino , Colículos Inferiores/fisiopatologia , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Complexo Olivar Superior/fisiopatologia , Fatores de Tempo
13.
Rev. otorrinolaringol. cir. cabeza cuello ; 79(1): 125-136, mar. 2019. tab, graf
Artigo em Espanhol | LILACS | ID: biblio-1004393

RESUMO

RESUMEN El tinnitus es un síntoma caracterizado por la percepción de un sonido en ausencia de un estímulo externo. Si bien su fisiopatología puede involucrar una alteración a nivel del funcionamiento del oído interno, la percepción de éste y el grado de molestias asociadas dependen de modificaciones de redes cerebrales cognitivas y emocionales. En la presente revisión, se abordan los cambios que existen a nivel coclear, de tronco encefálico, tálamo y la extensa red cerebral que dan cuenta del tinnitus, discutiendo como esta nueva conceptualización tiene importantes implicancias clínicas, permitiendo una mejor comprensión de los síntomas asociados al tinnitus, sus comorbilidades, y el desarrollo de nuevas estrategias terapéuticas.


ABSTRACT Tinnitus is a symptom characterized by the perception of a sound without an external stimulus. Although the pathophysiology of tinnitus initially involves an alteration of the inner ear function, the perception of it and the degree of distress associated with it depends on changes in cognitive and emotional brain networks. In this article, we review the changes that exist at the cochlea, brainstem, thalamus and a widespread cerebral networks that account for tinnitus, discussing how this new conceptualization has significant clinical implications and allows a better understanding of the symptoms associated with tinnitus, its co-morbidities, and how this view has allowed the development of new therapies.


Assuntos
Humanos , Zumbido/fisiopatologia , Cérebro/fisiopatologia , Doenças Auditivas Centrais , Percepção Auditiva/fisiologia , Zumbido/terapia , Perda Auditiva
14.
Brain Inj ; 33(2): 111-128, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30415586

RESUMO

PRIMARY OBJECTIVE: To understand the effects of non-blast-related TBI on peripheral auditory function in adults, as measured through basic and advanced audiological assessments. BACKGROUND: Despite numerous studies demonstrating hearing loss post TBI there has been no systematic investigation of the prevalence, nature and severity of peripheral hearing loss. DATA IDENTIFICATION: An English-language systematic search using MEDLINE, CINAHL, PsychINFO, PubMed and hand-searching of reference lists was conducted from 1 January 1990 to 31 October 2016. STUDY SELECTION: After independent review by the authors, 20 of 281 originally identified articles were retained. DATA EXTRACTION: Audiological findings were extracted and synthesized across studies. RESULTS: Using the Oxford Centre for Evidence Based Medicine levels of evidence (2009), 3b was the highest level of evidence within the review. Sensorineural hearing loss was the most consistent auditory deficit reported post TBI. CONCLUSION: The range and frequency of auditory dysfunction in patients with TBI remain unclear. Future research should focus on understanding the nature, frequency and change of auditory deficits over time following TBI. Knowledge in this area will provide crucial information for clinicians and facilitate the development of diagnostic and best practice guidelines which currently are lacking for the management of this patient population.


Assuntos
Doenças Auditivas Centrais/etiologia , Doenças Auditivas Centrais/fisiopatologia , Lesões Encefálicas Traumáticas/complicações , Lesões Encefálicas Traumáticas/fisiopatologia , Animais , Audiometria , Vias Auditivas/lesões , Vias Auditivas/fisiopatologia , Perda Auditiva Neurossensorial/etiologia , Perda Auditiva Neurossensorial/fisiopatologia , Humanos
15.
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
17.
J Am Acad Audiol ; 29(9): 855-867, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30278870

RESUMO

BACKGROUND: The middle latency response (MLR) first came to light as an auditory evoked potential in 1958. Since then, it has aroused substantial interest and investigation by clinicians and researchers alike. In recent history, its use and popularity have dwindled in tandem with various other auditory evoked potentials in audiology. One area for which MLR research and application has been overlooked is its potential value in measuring the neural integrity of the auditory thalamocortical pathway. In a broader sense, the MLR, when combined with the auditory brain stem response, can provide information concerning the status of much of the central auditory system pathways. This review is intended to provide information concerning the MLR as a measure of central auditory function for the reader to consider. PURPOSE: To review and synthesize the scientific literature regarding the potential value of the MLR in assessing the integrity of the central auditory system and to provide the reader an informed perspective on the value of the MLR in this regard. Information is also provided on the MLR generator sites and fundamental characteristics of this evoked potential essential to its clinical and or research application. RESEARCH DESIGN: A systematic review and synthesis of the literature focusing on the MLR and lesions of the central auditory system. STUDY SAMPLE: Studies and individual cases were reviewed and analyzed that evidenced documented lesions of the central auditory nervous system. DATA COLLECTION AND ANALYSIS: The authors searched and reviewed the literature (journal articles, book chapters, and books) pertaining to central auditory system lesion effects on the MLR. RESULTS: Although findings varied from study to study, overall, the MLR was reasonably sensitive and specific to neurological compromise of the central auditory system. This finding is consistent with the generator sites of this evoked potential. CONCLUSIONS: The MLR is a valuable tool for assessing the integrity of the central auditory system. It should be of interest to the clinician or researcher who focuses their attention on the function and dysfunction of the higher auditory system.


Assuntos
Doenças Auditivas Centrais/fisiopatologia , Potenciais Evocados Auditivos , Tempo de Reação , Humanos , Esclerose Múltipla/fisiopatologia , Doenças Neurodegenerativas/fisiopatologia
18.
Acta pediátr. hondu ; 9(2): 938-942, oct. 2018-mar. 2019.
Artigo em Espanhol | LILACS | ID: biblio-1046293

RESUMO

El síndrome de Alport (SA) es un conjunto de enfermedades que se caracterizan por una afección hereditaria de la membrana basal glomerular con alteraciones en el colágeno tipo IV que la compone. Se presenta con hematuria micro- o macroscópica; además, suele asociarse a alteraciones auditivas y oculares, y es causa de alrededor de 0.3 a 3% de la enfermedad renal terminal en pediatría.Se reporta el caso de adolescente de 14 años, sexo masculino, que consultó por presentar fiebre, debilidad generalizada y palidez de tres días de evolución. Tenía historia de leucocoria en ojo derecho desde el nacimiento, antecedentes familiares de hematuria (padre y hermano), además hipoacusia en oído derecho. Esto sumado a la lesión ocular antes descrita y a insuficiencia renal crónica se sospechó SA, por lo que se le realizó una audiometría y un ultrasonido (USG) ocular los cuales reportaban una hipoacusia de oído derecho y condensaciones vítreas en ojo derecho respectivamente; dada la hematuria familiar y propia del paciente se realizó el diagnóstico de Síndrome de Alport según los criterios de Flinter...(AU)


Assuntos
Humanos , Masculino , Adolescente , Membrana Basal Glomerular , Nefrite Hereditária/diagnóstico , Doenças Auditivas Centrais , Anormalidades do Olho , Nefropatias
19.
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
20.
Vestn Otorinolaringol ; 83(4): 43-50, 2018.
Artigo em Russo | MEDLINE | ID: mdl-30113579

RESUMO

Identification and diagnostics of auditory processing disorders (APD) in the children continues to be a challenge. Screening tools help to decide the need for evaluation. However, none of them are used routinely in Russia, nor are there thus far auditory checklist questionnaires in the Russian language. The aim of his study was to approve Children's Auditory Performance Scale (CHAPS) and Fisher's Auditory Checklist in Russian for the children at the age of 5-10 years. The study included 52 children at the age from 5 to 11 years. All of them were native Russian speakers. Pure tone audiometry, immitancemetry, and the Simplified RuMatrix test were performed for all the children. All of them had normal hearing thresholds, speech and language development, and normal academic achievement at school. The parents filled in the CHAPS and Fisher's Auditory Checklist questionnaires in Russian. The mean value of Fisher's Auditory Checklist was 85.7 ± 9.3, the mean value of CHAPS questionnaire was equal to 0.08 ± 0.26. The results obtained with the use of the Russian language questionnaires are in excellent agreement with the test data yielded by the respective English language versions. It is recommended that the clinical assessment for APD should be performed for children with the result of the Fisher questionnaire less than 67 points and with the result of the CHAPS questionnaire less than -1 point. It is concluded that Fisher's questionnaire was more convenient for the parents to fill in.


Assuntos
Audiometria de Tons Puros/métodos , Doenças Auditivas Centrais , Programas de Rastreamento/métodos , Doenças Auditivas Centrais/diagnóstico , Doenças Auditivas Centrais/epidemiologia , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pediatria/métodos , Federação Russa/epidemiologia , Inquéritos e Questionários
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...