Figure 6. ERG from six patients and normal controls. The dark-adapted (DA) 0.01 electroretinogram (ERG) was reduced in all patients tested, and the DA 3.0 ERG showed a reduced b/a ratio in all patients tested except patient L; II-1 (11 years). Three patients [C,II-1 (23 years, c.335G>C); K,II-1 (22 years, c.304C>T); N,II-1 (51 years, c.626G>A)] showed a b/a ratio <1 on the DA 3.0 ERG, while the other three patients [A,II-1 (58 years, c.304C>T); B,II-1 (18 years, c.458T>C); L,II-1 (11 years, c.488G>A)] had a b/a >1. The light-adapted (LA) 3.0 ERG b-waves were delayed and decreased in amplitude in all patients tested. Delayed LA 30 Hz flicker peak time and decreased amplitude were observed in all patients. Solid lines represent average traces of the right eyes, and small dashed lines represent the average traces of the left eyes. Long dashed lines represent eye-blink artifacts [A,II-1 (58 years); B,II-1 (18 years)].