Combined Central and Peripheral Degenerative Vestibular Disorders: CANVAS, Idiopathic Cerebellar Ataxia with Bilateral Vestibulopathy (CABV) and Other Differential Diagnoses of the CABV Phenotype

Abstract

Purpose of Review

The traditional dichotomy of central versus peripheral causes of imbalance may be viewed as an obstacle, rather than an aid to diagnosis. This paper aims to review the clinical assessment of conditions with combined central and peripheral vestibular impairment and the more common conditions which may manifest this joint pathology.

Recent Findings

Cerebellar ataxia with bilateral vestibulopathy (CABV) was initially believed to be a distinct syndrome but has more recently been recognised as a phenotype which may be found in a number of disorders including CANVAS, Friedreich's ataxia, spinocerebellar ataxia types 3 and 6 and multiple system atrophy. Oculomotor assessment provides a valuable means of identifying such patients and centres around the identification of an abnormal visually enhanced vestibulo-ocular reflex.

Summary

Identification of the CABV phenotype limits the differential diagnoses and then provides a directed investigation pathway.

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