METHODS. Twelve participants from six families with OPA1 ADOA and 16 age-matched controls were recruited. Electrophysiological assessment involved pattern ERGs (PERGs), focal (208) and full-field long-duration (250 ms) flash ERGs using a red light-emitting diode flash on a rod-saturating blue background, and full-field brief (300 ls) xenon flash ERGs using a red filter over a continuous rod saturating blue background. Amplitudes and implicit times of the ERG components were analyzed and the diagnostic potential of each electrophysiological technique was determined by generating receiver operating characteristic (ROC) curves.RESULTS. Mean amplitudes of the N95 and all PhNRs, except the full-field PhNR ON , were significantly reduced in participants with ADOA (P < 0.01). Subtraction of the group-averaged focal ERG of ADOA participants from that of controls showed an equal loss in the focal PhNR ON and PhNR OFF components, whereas in the full-field ERG the loss in the PhNR OFF was greater than that in the PhNR ON component. The areas under the ROC curve (AUC) for the focal PhNR ON (0.92), focal PhNR OFF (0.95), and full-field PhNR OFF (0.83), were not significantly different from that of the PERG N95 (0.99).CONCLUSIONS. In patients with ADOA, the PhNR ON and PhNR OFF components are nearly symmetrically reduced in the long-duration ERG, suggesting that ON-and OFF-RGC pathways may be equally affected.