Relationship between hearing function and myasthenia gravis: A contemporary review

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.

Keywords: Myasthenia gravis; contralateral acoustic stimulation; hearing loss; otoacustic emissions; tinnitus.

Publication types

  • Review

MeSH terms

  • Hearing / physiology*
  • Hearing Tests
  • Humans
  • Myasthenia Gravis / physiopathology*
  • Otoacoustic Emissions, Spontaneous / physiology
  • Otolaryngology