Isolated Vestibular Suppression Impairment With Vestibular Migraine: A Phenotypic CANVAS Variant.

Hypothesis: Cerebellar Ataxia with Neuropathy and Vestibular Areflexia (CANVAS) is likely to have a genetic basis. We describe the unique eye movement features of a possible phenotypic CANVAS variant. Background: The patient comes from a large CANVAS kindred (four out of nine siblings) and has sensory neuropathy, cerebellar eye signs, and vestibular migraine (VM), but otherwise normal vestibular function. Methods: We recorded eye and head movements using the gold standard scleral search coil technique: in the patient, a close relative with mild sensory neuropathy, and a normal control. Results: At >=0.8 Hz vestibulo-ocular reflex suppression (VORS) was significantly smaller in the patient. At 1 Hz, the patient's VORS was almost two times worse than the control, and five times worse at 1.6 Hz. The patient's VORS deficiency was observed with the naked eye as an inability to keep the eye stationary during imposed sinusoidal head rotation at ~1 Hz. At

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