Allen (1942) described a family in which many affected members had congenital nystagmus, presumably inherited in an autosomal dominant pattern. Congenital nystagmus has also been observed as a probably dominant trait among the Old Order Amish of Holmes ... Allen (1942) described a family in which many affected members had congenital nystagmus, presumably inherited in an autosomal dominant pattern. Congenital nystagmus has also been observed as a probably dominant trait among the Old Order Amish of Holmes Co., Ohio (McKusick, 1986). Dell'Osso et al. (1993) studied a family containing 4 sibs with congenital nystagmus to test the hypothesis that instability of the neural integrator responsible for gaze holding is its cause. They concluded that there is no gaze-holding abnormality. The parents and 5 other sibs had no visual problems or clinically apparent motility disorders. However, the father and 2 of the other sibs had saccadic instabilities. Kerrison et al. (1998) reported a large African American family in which 21 of 54 living family members had congenital nystagmus inherited in an autosomal dominant pattern. Clinical examinations were performed on 14 individuals. Among 7 affected persons whose parents were able to report whether the nystagmus was present congenitally, onset at birth was noted in 2 and between 3 and 6 months in 5. Best-corrected binocular Snellen visual acuity ranged from 20/30 to 20/100, with a mode of 20/50. Strabismus was present in 14 examined patients (36%). Eye movement recordings, performed on 5 persons, included asymmetric pendular (3), asymmetric pendular combined with dual waveform jerk (1), and unidirectional jerk nystagmus (1).