It is not your eyes

Devon A. Cohen, M. Tariq Bhatti, John Chen, Michelle M Mauermann, Daniel R. Gold

Research output: Contribution to journalArticle


A 55-year-old woman reported blurred vision while walking, numbness and tingling, contact allodynia, and gait imbalance. Visual acuity was 20/20 in both eyes, but there was a loss of 4 lines with horizontal dynamic visual acuity testing. Ocular motility examination demonstrated spontaneous downbeat nystagmus that increased in lateral gaze with a torsional component and impaired smooth pursuits. Head impulse test was positive, and electromyography demonstrated a sensory neuropathy/neuronopathy. Vestibular testing confirmed both central ocular motor pathway and bilateral peripheral vestibular system involvement. The constellation of clinical findings and paraclinical testing was consistent with a recently recognized neurodegenerative disorder termed cerebellar ataxia with neuropathy and vestibular areflexia syndrome.

Original languageEnglish (US)
JournalSurvey of ophthalmology
StatePublished - Jan 1 2019



  • cerebellar ataxia
  • dynamic visual acuity
  • head impulse test
  • nystagmus
  • vestibulo-ocular reflex

ASJC Scopus subject areas

  • Ophthalmology

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