Effectiveness of vestibular rehabilitation therapy in patients with idiopathic Cerebellar Ataxia with Bilateral Vestibulopathy (iCABV)

Abstract

OBJECTIVE: To evaluate the benefit of vestibular rehabilitation therapy (VRT) in the management of patients with idiopathic cerebellar ataxia with bilateral vestibulopathy (iCABV). BACKGROUND: iCABV is a hindbrain degenerative disorder with impairment of both central and peripheral vestibular pathways. There is combined failure of four compensatory eye movement systems including the vestibulo-ocular reflex (VOR), optokinetic reflex, smooth pursuit and the visually enhanced vestibulo-ocular reflex (VVOR). Phenotypic presentation includes postural and gait instability, oscillopsia and dizziness with active head movement. The benefit of VRT in iCABV patients has not been established. METHODS: A retrospective review was performed on a cohort of twelve patients diagnosed with iCABV in a multidisciplinary neuro-otology clinic. All participated in VRT and completed their suggested course of VRT. The following clinical measures were assessed before starting and after finishing VRT: 1) Dizziness Handicap Inventory (DHI), 2) Activities-Specific Balance Confidence (ABC) Scale, 3) Catastrophization scale, 4) Positive Affective Negative Affective Score (PANAS), 5) Dynamic Gait Index (DGI) and 6) Modified Clinical Test of Sensory Interaction in Balance (mCTSIB). The number of falls historically was recorded in addition to gait speed (ft./sec). RESULTS: Following VRT, patients were found to have improved balance on mCTSIB (condition 4 : 7 vs 18 seconds, P = 0.04) and a better postural stability with a reduced number of falls (p = 0.01). No statistically significant improvement was seen in the DHI, ABC, Catastrophization scale, DGI, PANAS and gait speed (p > 0.05). CONCLUSIONS: iCABV patients who underwent VRT were found to have a better postural stability and reduced risk of falls. VRT was not found to significantly improve patients’ overall subjective perception of their symptoms or their psychological status.

Vestibular rehabilitation in cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS)- A case report

“…reported that a 10% change in the ABC was meaningful in persons with vestibular migraine ( Whitney et al., 2000b ). Hassannia reported no difference in ABC scores after vestibular rehab in persons living with CANVAS, which is similar to our finding in Patient X ( Hassannia et al., 2022 ). However, Hassannia et al.…”

“…Persons with CANVAS did not improve their gait speed or their DGI score in a previous study ( Hassannia et al., 2022 ). Patient X's DGI improved from 18/24 to 21/24, which, while an improvement, is not clinically significant.…”

“…Hassannia et al. demonstrated an improvement on the Modified Clinical Test of Sensory Interaction and Balance and decreased number of falls in those with idiopathic cerebellar ataxia with bilateral vestibulopathy following vestibular rehabilitation ( Hassannia et al., 2022 ). To date, no report has shown an improvement in VOR gain or gait outcome measures in someone with CANVAS after a course of vestibular physical therapy.…”

A patient with neuropathy and ataxia: what do I have to consider?

“…Diagnosis of a vestibular component reduces the number of differential diagnoses. In addition, vestibular rehabilitation would add to the standard-of-care neurologic rehabilitation for an isolated somatosensory deficit [10 ▪ ]. Assessment of vestibular function may be performed by a facility that offers formal vestibular function testing, and the modalities that are available to assess semicircular canal function (involved in the perception of radial acceleration of the head) are the video head impulse test (vHIT), rotational chair and bithermal caloric irrigation.…”

Overview of the Clinical Approach to Individuals With Cerebellar Ataxia and Neuropathy

“…4 Formal Vestibular Function Testing Diagnosis of a vestibular component aids both the diagnostic process by potentially reducing the number of differential diagnoses and management, for instance, the addition of vestibular rehabilitation to the standard-of-care neurologic rehabilitation. 13 Assessment of vestibular function may be performed by a facility that offers formal testing, and the modalities that are available to assess semicircular canal function are the video head impulse test (vHIT), rotational chair, and bithermal caloric irrigation. 14 The otolith organs (utricle and saccule) are generally assessed by vestibular-evoked myogenic potentials 15 or the subjective visual vertical (or horizontal) test.…”