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听力功能与重症肌无力之间的关系:当代综述

Relationship between hearing function and myasthenia gravis: A contemporary review.

作者信息

Ralli Massimo, Altissimi Giancarlo, Di Stadio A, Mazzei Filippo, Turchetta Rosaria, Cianfrone Giancarlo

机构信息

1 Department of Oral and Maxillofacial Sciences, Sapienza University of Rome, Italy.

2 Department of Sense Organs, Audiology Section, Policlinico Umberto I, Sapienza University of Rome, Italy.

出版信息

J Int Med Res. 2017 Oct;45(5):1459-1465. doi: 10.1177/0300060516672124. Epub 2016 Nov 11.

Abstract

There is increasing evidence of a connection between hearing function and myasthenia gravis (MG). Studies of the pathophysiological basis of this relationship suggest that acetylcholine receptors (AChRs) on outer hair cells (OHCs) play a central role. In patients with MG, autoantibodies against AChRs induce a progressive loss of AChRs on OHCs, decreasing their electromotility. The stapedial reflex decay test can be altered in MG patients, and can be used as an additional tool for diagnosis and monitoring. Transient evoked and distortion product otoacoustic emissions are the main diagnostic tool for monitoring OHC functionality in MG patients, and can be used to record subclinical hearing alterations before the onset of clinically evident hearing loss. Understanding the association between MG and hearing dysfunction requires a multidisciplinary approach. Otolaryngologists should take this relationship into account when approaching patients with a diagnosis of myasthenia gravis and "in patients with MG" with ण128;œin MG patients, and the progress of hearing alterations should always be monitored in patients with MG.

摘要

越来越多的证据表明听力功能与重症肌无力(MG)之间存在关联。对这种关系的病理生理基础的研究表明,外毛细胞(OHC)上的乙酰胆碱受体(AChR)起着核心作用。在MG患者中,针对AChR的自身抗体导致OHC上AChR逐渐丧失,降低其电运动性。镫骨肌反射衰减试验在MG患者中可能会改变,可作为诊断和监测的辅助工具。瞬态诱发耳声发射和畸变产物耳声发射是监测MG患者OHC功能的主要诊断工具,可用于在临床明显听力损失发作之前记录亚临床听力改变。理解MG与听力功能障碍之间的关联需要多学科方法。耳鼻喉科医生在诊治重症肌无力患者时应考虑到这种关系,并且在MG患者中,应始终监测听力改变的进展情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d4b7/5718710/7fc1188ef04f/10.1177_0300060516672124-fig1.jpg

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