Here, we show that the unique retinal nummular intraretinal hyperreflective foci (irHRFs) seen on OCT imaging in rd7 mice, which are known to correspond to intraretinal deep photoreceptor cell rosettes and whorls, can be seen on SD-OCT scans in ESCS patients, where nummular deep changes are seen since the earliest disease stages, and persist—and evolve—in later disease stages. This evidence concerns the gene NR2E3 and enhanced S-cone syndrome.