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Vestibular impairment in Charcot–Marie–Tooth disease

Abstract

Objective

To find out if Charcot–Marie–Tooth (CMT) patients, who have peripheral vestibular as well as peripheral somatosensory impairment, have worse postural balance than those who do not.

Methods

We studied 32 patients with various CMT phenotypes and genotypes. Vestibular function was measured with the video head impulse test (vHIT) which tests vestibulo-ocular reflex (VOR) gain from each of the six semicircular canals in response to rapid head rotations. Postural balance was evaluated with a battery of four postural tests with emphasis on the modified clinical test of sensory integration in balance (mCTSIB).

Results

Half of the 32 patients had some impairment of vestibular function ranging from mild, affecting only 1–2 semicircular canals, to almost total affecting all 6 semicircular canals. Their mCTSIB scores correlated with VOR gain from the vertical rather than from the lateral semicircular canals. The worse the vertical VOR gain the worse the mCTSIB score.

Conclusion

We propose that any CMT patient could have clinically inapparent vestibular impairment that can be easily measured with the vHIT. This vestibular impairment could be contributing to their imbalance and could respond to a focused vestibular rehabilitation program.

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Acknowledgements

Authors thank Prof. Dr. G. M.Halmagyi for linguistic help and comments, Mr Leigh McGarvie for help with video head impulse testing.

Funding

Study funded by the Scientific and Technological Research Council of Turkey (TÜBİTAK) under Grant no. (216S658).

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Correspondence to Gülden Akdal.

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Conflicts of interest

None of the authors have conflict of interest.

Ethics approval

Local ethics committee approved the study. All study procedures have been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Akdal, G., Koçoğlu, K., Tanrıverdizade, T. et al. Vestibular impairment in Charcot–Marie–Tooth disease. J Neurol 268, 526–531 (2021). https://doi.org/10.1007/s00415-020-10186-x

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Keywords

  • CMT disease
  • Modified clinical test of sensory integration in balance
  • Vestibulo-ocular reflex
  • Video head impulse test