Patton et al. (1993) reported a family with autosomal dominant congenital nystagmus. The history of the disorder in this family extended over 4 generations with male to male transmission, but the proband's grandfather and great-grandfather were deceased at ... Patton et al. (1993) reported a family with autosomal dominant congenital nystagmus. The history of the disorder in this family extended over 4 generations with male to male transmission, but the proband's grandfather and great-grandfather were deceased at the time of the report. The proband had nystagmus from birth, which did not interfere with his vision. The nystagmus was predominantly horizontal with a small rotary component. The frequency of the nystagmus was 3.5 Hz in the midline and 6 Hz in gaze to either side. Ophthalmic studies showed no abnormality of the retina or optic discs. His father had a similar clinical phenotype. Klein et al. (1998) reported a family in which 3 individuals had congenital nystagmus inherited in an autosomal dominant pattern. All 3 presented with typical and purely horizontal jerk nystagmus with slow-phase waveforms of increasing velocity. As in classic congenital nystagmus, the movements were accentuated by fixation, by lateral gaze, and during smooth pursuit, and they decreased on convergence. There was some intrafamilial variation: 1 individual had unidirectional jerk nystagmus, 1 had jerk nystagmus of periodically alternating direction, and 1 had a mixture of pendular and jerk nystagmus.