Objective: To describe the alterations observed in the vestibular testing in patients with CANVAS.

Background: The Cerebellar Ataxia with Neuropathy and Bilateral Vestibular Areflexia Syndrome (CANVAS) is a slowly progressive neurodegenerative disease, with its onset in adulthood. It is characterized by neurosensory, cerebellar ataxia and bilateral vestibulopathy. The most specific clinical sign is impairment of the visually enhanced vestibulo-ocular reflex (VOR).

Method: 15 patients who met the clinical criteria for CANVAS were assessed by using the following procedures: clinical history, otorhinolaryngological assessment and vestibular testing

Results: The main complaints found in the clinical history were gait disturbances (86,7%), headache (73,3%), dizziness (60%), paresthesia, dysarthria and fatigue, both with (53.3%). In the vestibular testing, by means of the caloric test (80%), gaze nystagmus, dysmetric saccades and smooth pursuit deficit, both with (53.3%), presented the highest rates of abnormalities, with prevalence of central vestibulopathy in 60% of the cases.

Conclusion: The vestibular testing can be a significant tool to help investigate CANVAS, in addition to an accurate clinical approach and molecular genetic testing. We point out that the possibility of CANVAS should be considered in patients with bilateral vestibular deficit.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/neurotological-evaluation-on-cerebellar-ataxia-with-neuropathy-and-bilateral-vestibular-areflexia-syndrome-canvas/