Foveation dynamics in congenital nystagmus III: Vestibulo-ocular reflex

Abstract

It has been shown that, during fixation of a stationary target with a fixed head, an individual with congenital nystagmus (CN) can repeatedly (beat-to-beat) foveate (within 13 minarc) and maintain low retinal slip velocities (less than 4°/sec). With the head in motion, vestibuloocular reflex (VOR) data showed eye velocities during these foveation periods that approximation head veloicty. Despite some claims that the VOR of CN subjects was deficient or absent, individuals with CN hardly ever complain of oscillopsia or exhibit any of the symptoms that would accompany such deficits in the VOR, whether during simple walking and running or while skiing down a mogul field. We developed and describe several different and unrelated methods to accurately assess the function of the VOR in an individual with typical idiopathic CN. We investigated the dynamics of CN foveation periods during head rotation to test the hypothesis that eye velocities would match head velocities during these periods. At about 1 Hz, horizontal VOR instantaneous (beat-to-beat) gains were 0.96 in the light and 0.94 in the dark while imagining a stationary target. Vertical VOR gains were 1.00 and 0.99 for these two conditions at the same frequency; the CN was horizontal. Also, during the VOR there is a CN neutral-zone shift comparable to that found during smooth pursuit. Our methods demonstrated that gaze velocity was held constant during foveation periods and we conclude that the VOR in this subject is functioning normally in the presence of the CN oscillation. Based on our findings in this and previous studies, we hypothesize that CN may be due to a peripheral instability.