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Achromatopsia has been proposed to be a morphologically predominately stable retinopathy with rare reports of progression of structural changes in the macula. A five-grade system of optical coherence tomography (OCT) features has been used for the classification of structural macular changes. However, their association with age remains questionable. We characterized the Slovenian cohort of 12 patients with pathogenic variants in

Objective: To establish an automated visual acuity test (AVAT) for infants, based on preferential looking technique and controlled with remote eye tracking. To validate the AVAT in a group of healthy children. To compare AVAT visual acuity (VA) values with corresponding VA values acquired with standard tests (ST). Methods: ST, adapted for age (Keeler Acuity Cards in preverbal children and LEA symbols in verbal children), was performed to obtain monocular VA in a group of 36 healthy children. During AVAT, 9 different stimuli with grating circles that matched spatial frequencies of 9 Keeler Acuity Cards (ranging between 0.29 and 14.5 cycles per degree) were projected on a screen. Three repetitions of each stimulus were shown during 9-s intervals, interchanging with an attention grabber. The remote eye tracker was used to evaluate the proportion of time a child spent looking at each grating circle compared to a homogeneous gray background that matched the grating stimuli in average luminance. From this proportion of time, child’ s binocular VA was evaluated. Results: Ninety-seven percent (35/36) of healthy children successfully completed ST and AVAT. There was an agreement between the results of an ST and AVAT, with Lin’s concordance coefficient being 0.53 (95% CI = 0.31–0.72). A tendency was observed toward VA overestimation on AVAT for children with VA >0.4 logMAR on ST and toward VA underestimation on AVAT for children with VA ≤0.4 logMAR on ST. Conclusions: AVAT requires a minimally skilled investigator. The evaluation of better eye monocular VA on ST and binocular VA on AVAT was comparable for healthy children.