Vestibular impairment in patients with Charcot-Marie-Tooth disease

Andrea Poretti, Antonella Palla, Alexander A. Tarnutzer, Jens A. Petersen, Konrad P. Weber, Dominik Straumann, Hans H. Jung

Research output: Contribution to journalArticle

Abstract

Objective: This case-control study aimed to determine whether the imbalance in Charcot-Marie- Tooth (CMT) disease is caused only by reduced proprioceptive input or whether the involvement of the vestibular nerve is an additional factor. Methods: Fifteen patients with CMT disease (aged 48 ± 17 years; 8 women) underwent cervical vestibular-evoked myogenic potentials, which reflect otolith-spinal reflex function, and quantitative horizontal search-coil head-impulse testing, which assesses the high-acceleration vestibulo-ocular reflex of the semicircular canals. Results: Relative to healthy age-matched control subjects, cervical vestibular-evoked myogenic potentials were found to be impaired in 75% of patients (average p13 latency: 23.0 ± 2.7 milliseconds, p = 0.01; average n23 latency: 29.0 ± 1.8 milliseconds, p 5 0.01) and the quantitative head-impulse test in 60% of patients (average gain ± 1 SD: 0.67 ± 0.24, p < 0.001). All patients with head-impulse test impairment also showed cervical vestibular-evokedmyogenic potential abnormalities, while the reverse was not true. Conclusions: We conclude that the neuropathic process in patients with CMT disease frequently involves the vestibular nerve and that cervical vestibular-evoked myogenic potentials may be more sensitive than quantitative head-impulse testing for detecting vestibular involvement, in particular at an early disease stage.

Original languageEnglish (US)
Pages (from-to)2099-2105
Number of pages7
JournalNeurology
Volume80
Issue number23
DOIs
StatePublished - Jun 4 2013

Fingerprint

Charcot-Marie-Tooth Disease
Vestibular Evoked Myogenic Potentials
Head Impulse Test
Vestibular Nerve
Head
Vestibulo-Ocular Reflex
Otolithic Membrane
Semicircular Canals
Reflex
Case-Control Studies

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Poretti, A., Palla, A., Tarnutzer, A. A., Petersen, J. A., Weber, K. P., Straumann, D., & Jung, H. H. (2013). Vestibular impairment in patients with Charcot-Marie-Tooth disease. Neurology, 80(23), 2099-2105. https://doi.org/10.1212/WNL.0b013e318295d72a

Vestibular impairment in patients with Charcot-Marie-Tooth disease. / Poretti, Andrea; Palla, Antonella; Tarnutzer, Alexander A.; Petersen, Jens A.; Weber, Konrad P.; Straumann, Dominik; Jung, Hans H.

In: Neurology, Vol. 80, No. 23, 04.06.2013, p. 2099-2105.

Research output: Contribution to journalArticle

Poretti, A, Palla, A, Tarnutzer, AA, Petersen, JA, Weber, KP, Straumann, D & Jung, HH 2013, 'Vestibular impairment in patients with Charcot-Marie-Tooth disease', Neurology, vol. 80, no. 23, pp. 2099-2105. https://doi.org/10.1212/WNL.0b013e318295d72a
Poretti A, Palla A, Tarnutzer AA, Petersen JA, Weber KP, Straumann D et al. Vestibular impairment in patients with Charcot-Marie-Tooth disease. Neurology. 2013 Jun 4;80(23):2099-2105. https://doi.org/10.1212/WNL.0b013e318295d72a
Poretti, Andrea ; Palla, Antonella ; Tarnutzer, Alexander A. ; Petersen, Jens A. ; Weber, Konrad P. ; Straumann, Dominik ; Jung, Hans H. / Vestibular impairment in patients with Charcot-Marie-Tooth disease. In: Neurology. 2013 ; Vol. 80, No. 23. pp. 2099-2105.
@article{c7ff8327d0944d71a6e8fed3c328a49f,
title = "Vestibular impairment in patients with Charcot-Marie-Tooth disease",
abstract = "Objective: This case-control study aimed to determine whether the imbalance in Charcot-Marie- Tooth (CMT) disease is caused only by reduced proprioceptive input or whether the involvement of the vestibular nerve is an additional factor. Methods: Fifteen patients with CMT disease (aged 48 ± 17 years; 8 women) underwent cervical vestibular-evoked myogenic potentials, which reflect otolith-spinal reflex function, and quantitative horizontal search-coil head-impulse testing, which assesses the high-acceleration vestibulo-ocular reflex of the semicircular canals. Results: Relative to healthy age-matched control subjects, cervical vestibular-evoked myogenic potentials were found to be impaired in 75{\%} of patients (average p13 latency: 23.0 ± 2.7 milliseconds, p = 0.01; average n23 latency: 29.0 ± 1.8 milliseconds, p 5 0.01) and the quantitative head-impulse test in 60{\%} of patients (average gain ± 1 SD: 0.67 ± 0.24, p < 0.001). All patients with head-impulse test impairment also showed cervical vestibular-evokedmyogenic potential abnormalities, while the reverse was not true. Conclusions: We conclude that the neuropathic process in patients with CMT disease frequently involves the vestibular nerve and that cervical vestibular-evoked myogenic potentials may be more sensitive than quantitative head-impulse testing for detecting vestibular involvement, in particular at an early disease stage.",
author = "Andrea Poretti and Antonella Palla and Tarnutzer, {Alexander A.} and Petersen, {Jens A.} and Weber, {Konrad P.} and Dominik Straumann and Jung, {Hans H.}",
year = "2013",
month = "6",
day = "4",
doi = "10.1212/WNL.0b013e318295d72a",
language = "English (US)",
volume = "80",
pages = "2099--2105",
journal = "Neurology",
issn = "0028-3878",
publisher = "Lippincott Williams and Wilkins",
number = "23",

}

TY - JOUR

T1 - Vestibular impairment in patients with Charcot-Marie-Tooth disease

AU - Poretti, Andrea

AU - Palla, Antonella

AU - Tarnutzer, Alexander A.

AU - Petersen, Jens A.

AU - Weber, Konrad P.

AU - Straumann, Dominik

AU - Jung, Hans H.

PY - 2013/6/4

Y1 - 2013/6/4

N2 - Objective: This case-control study aimed to determine whether the imbalance in Charcot-Marie- Tooth (CMT) disease is caused only by reduced proprioceptive input or whether the involvement of the vestibular nerve is an additional factor. Methods: Fifteen patients with CMT disease (aged 48 ± 17 years; 8 women) underwent cervical vestibular-evoked myogenic potentials, which reflect otolith-spinal reflex function, and quantitative horizontal search-coil head-impulse testing, which assesses the high-acceleration vestibulo-ocular reflex of the semicircular canals. Results: Relative to healthy age-matched control subjects, cervical vestibular-evoked myogenic potentials were found to be impaired in 75% of patients (average p13 latency: 23.0 ± 2.7 milliseconds, p = 0.01; average n23 latency: 29.0 ± 1.8 milliseconds, p 5 0.01) and the quantitative head-impulse test in 60% of patients (average gain ± 1 SD: 0.67 ± 0.24, p < 0.001). All patients with head-impulse test impairment also showed cervical vestibular-evokedmyogenic potential abnormalities, while the reverse was not true. Conclusions: We conclude that the neuropathic process in patients with CMT disease frequently involves the vestibular nerve and that cervical vestibular-evoked myogenic potentials may be more sensitive than quantitative head-impulse testing for detecting vestibular involvement, in particular at an early disease stage.

AB - Objective: This case-control study aimed to determine whether the imbalance in Charcot-Marie- Tooth (CMT) disease is caused only by reduced proprioceptive input or whether the involvement of the vestibular nerve is an additional factor. Methods: Fifteen patients with CMT disease (aged 48 ± 17 years; 8 women) underwent cervical vestibular-evoked myogenic potentials, which reflect otolith-spinal reflex function, and quantitative horizontal search-coil head-impulse testing, which assesses the high-acceleration vestibulo-ocular reflex of the semicircular canals. Results: Relative to healthy age-matched control subjects, cervical vestibular-evoked myogenic potentials were found to be impaired in 75% of patients (average p13 latency: 23.0 ± 2.7 milliseconds, p = 0.01; average n23 latency: 29.0 ± 1.8 milliseconds, p 5 0.01) and the quantitative head-impulse test in 60% of patients (average gain ± 1 SD: 0.67 ± 0.24, p < 0.001). All patients with head-impulse test impairment also showed cervical vestibular-evokedmyogenic potential abnormalities, while the reverse was not true. Conclusions: We conclude that the neuropathic process in patients with CMT disease frequently involves the vestibular nerve and that cervical vestibular-evoked myogenic potentials may be more sensitive than quantitative head-impulse testing for detecting vestibular involvement, in particular at an early disease stage.

UR - http://www.scopus.com/inward/record.url?scp=84879116407&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84879116407&partnerID=8YFLogxK

U2 - 10.1212/WNL.0b013e318295d72a

DO - 10.1212/WNL.0b013e318295d72a

M3 - Article

C2 - 23658384

AN - SCOPUS:84879116407

VL - 80

SP - 2099

EP - 2105

JO - Neurology

JF - Neurology

SN - 0028-3878

IS - 23

ER -