Figure 2. The patient with a clinical phenotype diagnostic of enhanced S-cone syndrome (ESCS) underwent targeted genetic testing, which confirmed homozygous (and previously reported) mutations in the NR2E3 gene. A: The fundus photograph shows the typical nummular pigmentary changes throughout the mid-periphery typically associated with ESCS. B: The full-field flash electroretinography (ffERGs) results exhibited non-recordable rod responses with preserved mixed and cone responses showing electronegative waveforms characteristic of ESCS.