VEMPs and Dysautonomia Assessment in Definite Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome (CANVAS): a Case Series Study.

Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a recently described slowly progressive ataxia with severe imbalance due to the compromise of three of the four sensory inputs for balance, leaving only vision unaffected. Bilateral vestibulopathy is present but saccular and utricular function, measured by vestibular evoked myogenic potentials (VEMPs), has not been widely studied in these patients. Dysautonomia has been reported but is not among the diagnostic criteria. We performed a database analysis to identify patients evaluated between 2003 and 2019 with probable diagnosis of CANVAS by using key words "bilateral vestibulopathy and/or cerebellar ataxia and/or sensory polyneuropathy." Five out of 842 met all conditions. Patients underwent neurological/neurootological exam, brain MRI, visually enhanced vestibulo-ocular reflex (VVOR) exam by high-speed video-oculography using video-Head Impulse Test (vHIT), VEMPs, neurophysiological studies, and genetic tests to exclude other causes of ataxia. Dysautonomia was addressed by the standardized survey of autonomic symptoms. All patients had clinically definite CANVAS as brain MRI showed vermal cerebellar atrophy, neurophysiological studies showed a sensory neuronopathy pattern (absent sensory action potentials), VVOR was abnormal bilaterally, and genetic tests ruled out other causes of ataxia including SCA 3 and Friedreich ataxia. Patients had at least 3 dysautonomic symptoms, including xerostomia/xerophthalmia (5/5). VEMP results varied among patients, ranging from normal to completely abnormal. We found inconsistent results with VEMPs. The utilization of VEMPs in more CANVAS cases will determine its utility in this syndrome. Dysautonomia may be included in the diagnostic criteria.

The vestibulo-ocular reflex assessment in patients with Ménière's disease: examining all semicircular canals.

UNLABELLED: Abstract Conclusion: The distribution of abnormal results is not uniform between different canals in each patient; the most frequent gain reduction is obtained for the posterior canal. Gain reduction reflects the disease duration and amount of hearing loss. OBJECTIVE: To test the hypothesis that the vestibulo-ocular reflex (VOR) evoked after stimulation of each semicircular canal behaves in a different manner in patients with unilateral definite Ménière's disease. METHODS: We studied the VOR evoked by rapid head-impulses in the plane of the 6 semicircular canals in 36 patients. It was evaluated with a video system that analyzes the head and eye velocity and the gain was the objective measure. RESULTS: In 12 (33.3%) patients the examination of both ears was normal for all the semicircular canals, in 12 patients the results from the affected ear were abnormal in at least 1 of the semicircular canals, in 11 (30.5%) patients the results were abnormal in at least 1 of the semicircular canals in both the affected and unaffected ears, and in 1 (2.9%) patient the results were abnormal only in the unaffected ear. The most frequent abnormal result was obtained from the posterior canal of the affected ear and from the coupled superior canal of the unaffected ear. The distribution of abnormal findings was dependent on the disease duration and hearing loss.