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.

We thank Drs. Vergara Olmos, Dabiri, and Rutka for their thoughtful commentary on our recent publication. These comments reinforce the continually evolving management paradigm for small-and medium-sized VSs. The concept of continued observation of growing VSs 1 and defining clinically significant size thresholds to guide timing of intervention are natural extensions of recent research, and we are grateful for this opportunity to expound on several interesting concepts pertaining to this evolving story.