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散发性共济失调患者的纯音听阈和脑干听觉诱发电位

Pure-Tone Hearing Thresholds and Brainstem Auditory Evoked Potentials in Sporadic Ataxia.

作者信息

Zeigelboim Bianca Simone, Scheidt Anylize Wachholz Vom, Kronbauer Kairone Fernandes, Liberalesso Paulo Breno Noronha, José Maria Renata, Fonseca Vinicius Ribas, Teive Hélio Afonso Ghizoni

机构信息

Department of Otoneurology, Universidade Tuiuti do Paraná, Curitiba, PR, Brazil.

Postgraduation Program in Communication Disorders, Universidade Tuiuti do Paraná, Curitiba, PR, Brazil.

出版信息

Int Arch Otorhinolaryngol. 2020 Jan;24(1):e86-e92. doi: 10.1055/s-0039-1693676. Epub 2019 Nov 4.

Abstract

Spinocerebellar ataxia (SCA) is part of a genetic and clinical heterogeneous group of neurodegenerative diseases characterized by progressive cerebellar ataxia.  To describe the results of audiological and electrophysiological hearing evaluations in patients with sporadic ataxia (SA).  A retrospective cross-sectional study was carried out with 11 patients submitted to the following procedures: anamnesis, otorhinolaryngological evaluation, tonal and vocal audiometry, acoustic immittance and brainstem auditory evoked potential (BAEP) tests.  The patients presented with a prevalence of gait imbalance, of dysarthria, and of dysphagia; in the audiometric and BAEPs, four patients presented with alterations; in the acoustic immittance test, five patients presented with alterations, predominantly bilateral.  The most evident alterations in the audiological evaluation were the prevalence of the descending audiometric configuration between the frequencies of 2 and 4 kHz and the absence of the acoustic reflex between the frequencies of 3 and 4 kHz bilaterally. In the electrophysiological evaluation, the patients presented changes with a prevalence of increased I, III and V wave latencies and the interval in the interpeak I-III, I-V and III-V. In the present study, it was observed that auditory complaints did not have a significant prevalence in this type of ataxia, which does not occur in some types of autosomal recessive and dominant ataxia.

摘要

脊髓小脑共济失调(SCA)是一组具有遗传和临床异质性的神经退行性疾病,其特征为进行性小脑共济失调。描述散发性共济失调(SA)患者的听力学和电生理听力评估结果。对11例患者进行了一项回顾性横断面研究,这些患者接受了以下检查:问诊、耳鼻喉科评估、纯音和声导抗测听、声导抗测试以及脑干听觉诱发电位(BAEP)测试。患者中步态失衡、构音障碍和吞咽困难较为普遍;在听力测定和BAEP测试中,4例患者有异常表现;在声导抗测试中,5例患者有异常表现,主要为双侧异常。听力学评估中最明显的异常是2至4kHz频率之间出现下降型听力图,以及双侧3至4kHz频率之间无声反射。在电生理评估中,患者出现变化,主要表现为I、III和V波潜伏期延长以及I-III、I-V和III-V峰间间期延长。在本研究中,观察到在这类共济失调中听觉主诉并不普遍,而在某些常染色体隐性和显性共济失调中则不会出现这种情况。

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