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 = 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-evoked myogenic 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.
authors: Departments of Neurology, University Hospital Zurich, Switzerland.
authors: Andrea
authors: A
authors: Poretti
authors: Antonella
authors: A
authors: Palla
authors: Alexander A
authors: AA
authors: Tarnutzer
authors: Jens A
authors: JA
authors: Petersen
authors: Konrad P
authors: KP
authors: Weber
authors: Dominik
authors: D
authors: Straumann
authors: Hans H
authors: HH
authors: Jung
mesh_terms: D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D002607:Charcot-Marie-Tooth Disease / Q000145:classification / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010037:Otolithic Membrane / Q000503:physiopathology; D012027:Reflex, Vestibulo-Ocular / Q000502:physiology*; D012665:Semicircular Canals / Q000503:physiopathology; D058585:Vestibular Evoked Myogenic Potentials / Q000502:physiology*; D014724:Vestibular Function Tests / Q000295:instrumentation / Q000379:methods*; D055815:Young Adult