The aim of this study was to report relief of optokinetic-triggered vertigo (OKTV) with low-dose gabapentin in three patients with periodic vestibulocerebellar ataxia [episodic ataxia type 4 (EA4); OMIM 606552]. Clinical observations and analysis of video-recorded eye movements were used before and after gabapentin. Gabapentin relieved vertigo of all three treated patients with EA4, particularly during activities that typically would induce vertiginous symptoms. Two patients reported 8-12 hours of sustained relief after the first 100 mg dose. One has benefited from 100-200 mg TID for 7 years. Video analysis of nystagmus revealed improved target tracking on smooth pursuit and a steadier gaze hold. Gabapentin effectively relieved the optokinetic-triggered vertigo in our patients with EA4. Mechanisms are postulated in terms of known tight gabapentin binding to the Purkinje cell voltage-gated calcium channel. The observations may offer insight into this rare disease's neuropathology. © 2021 International Parkinson and Movement Disorder Society.