Table 3

Photopic ERG properties (SD)

StimulusPropertyControl (n = 19)NYX gene mutations ( n = 11)CACNA1F gene mutations (n = 4)
SFMean a-wave latency (ms)13 (6)16 (1)16 (1)
Response prolonged in011 (100%)3 (75%)
Mean a-wave amplitude (μV)−62 (15)−66 (18)−38 (20)
Response subnormal in002 (50%)
Mean b-wave amplitude (μV)129 (41)100 (30)46 (15)
Response abnormal in002 (50%)
30 Hz flickerMean latency (ms)11 (2)15 (5)15 (3)
Response prolonged in08 (73%)1 of 2 recordable (50%)
Mean amplitude (μV)96 (28)84 (23)25 (13)
Response subnormal in002 of 2 recordable (100%)
ON-OFF response Control (n = 15) CSNB1 (n = 2) CSNB2 (n = 2)
Mean a-wave latency (ms)22 (1)28 (5)31 (2)
Response prolonged in02 (100%)2 (100%)
Mean a-wave amplitude (μV)−36 (8)−32 (0)−17 (7)
Response subnormal in001 (50%)
Mean b-wave amplitude (μV)67 (14)19 (9)18 (2)
Response subnormal in02 (100%)2 (100%)
Mean d-wave amplitude (μV)49 (16)52 (24)16 (1)
Response subnormal in002 (100%)