Vestibular Performance During High-Acceleration Stimuli Correlates with Clinical Decline in SCA6

Young Eun Huh, Ji Soo Kim, Hyo Jung Kim, Seong Ho Park, Beom Seok Jeon, Jong Min Kim, Jin Whan Cho, David S. Zee

Research output: Contribution to journalArticlepeer-review

19 Scopus citations


In spinocerebellar ataxia type 6 (SCA6), the vestibular dysfunction and its correlation with other clinical parameters require further exploration. We determined vestibular responses over a broad range of stimulus acceleration in 11 patients with SCA6 (six men, age range=33–72 years, mean age±SD=59±12 years) using bithermal caloric irrigations, rotary chair, and head impulse tests. Correlations were also pursued among disability scores, as measured using the International Cooperative Ataxia Rating Scale, disease duration, age at onset, cytosine-adenine-guanine (CAG) repeat length, and the gain of the vestibulo-ocular reflex (VOR). In response to relatively low-acceleration, low-frequency rotational and bithermal caloric stimuli, the VOR gains were normal or increased regardless of the severity of disease. On the other hand, with relatively high-acceleration, high-frequency head impulses, there was a relative increase in gain in the mildly affected patients and a decrease in gain in the more severely affected patients and gains were negatively correlated with the severity of disease (Spearman correlation, R=−0.927, p<0.001). Selective decrease of the vestibular responses during high-acceleration, high-frequency stimuli may be ascribed to degeneration of either the flocculus or vestibular nuclei. The performance of the VOR during high-acceleration, high-frequency head impulses may be a quantitative indicator of clinical decline in SCA6.

Original languageEnglish (US)
Article number650
Pages (from-to)284-291
Number of pages8
Issue number3
StatePublished - Jun 26 2015


  • Cerebellum
  • Head impulse test
  • Spinocerebellar ataxia
  • Vertigo
  • Vestibulo-ocular reflex

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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