Departments of Neurology, University Hospital Zurich, Switzerland.
Neurology. 2013 Jun 4;80(23):2099-105. doi: 10.1212/WNL.0b013e318295d72a. Epub 2013 May 8.
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.
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.
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.
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.
本病例对照研究旨在确定夏科-马里-图病（CMT）的失衡是否仅由本体感觉传入减少引起，还是前庭神经的参与是另一个因素。
15 例 CMT 病患者（年龄 48 ± 17 岁；8 名女性）接受了颈性前庭诱发肌源性电位检查，该检查反映了耳石-脊髓反射功能，以及定量水平搜索线圈头脉冲测试，该测试评估了半规管的高加速度前庭眼反射。
与健康年龄匹配的对照组相比，75%的患者存在颈性前庭诱发肌源性电位异常（平均 p13 潜伏期：23.0 ± 2.7 毫秒，p = 0.01；平均 n23 潜伏期：29.0 ± 1.8 毫秒，p = 0.01），60%的患者定量头脉冲测试异常（平均增益 ± 1 标准差：0.67 ± 0.24，p < 0.001）。所有头脉冲测试异常的患者均存在颈性前庭诱发肌源性电位异常，而反之则不然。
我们得出结论，CMT 病患者的神经病变过程常累及前庭神经，颈性前庭诱发肌源性电位可能比定量头脉冲测试更敏感，可用于检测前庭受累，尤其是在疾病早期。
