It has been shown that, during fixation of a stationary target with a
fixed head, an individual with congenital nystagmus (CN) can repeatedl
y (beat-to-beat) foveate (within 13 minarc) and maintain low retinal s
lip velocities (less than 4-degrees/sec). With the head in motion, ves
tibuloocular reflex (VOR) data showed eye velocities during these fove
ation periods that approximation head veloicty. Despite some claims th
at the VOR of CN subjects was deficient or absent, individuals with CN
hardly ever complain of oscillopsia or exhibit any of the symptoms th
at would accompany such deficits in the VOR, whether during simple wal
king 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. W
e investigated the dynamics of CN foveation periods during head rotati
on to test the hypothesis that eye velocities would match head velocit
ies 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 t
hat found during smooth pursuit. Our methods demonstrated that gaze ve
locity was held constant during foveation periods and we conclude that
the VOR in this subject is functioning normally in the presence of th
e CN oscillation. Based on our findings in this and previous studies,
we hypothesize that CN may be due to a peripheral instability.