Examples of fundus findings characteristic of rod–cone dystrophy. A, Fundus of a 54-year-old woman with USH2A mutation shows optic nerve head pallor, arteriolar attenuation, macular atrophy, and widespread peripheral pigmentary alteration. Corrected distance visual acuity (CDVA) was hand motions. B, Fundus of a 66-year-old man with USH2A mutation shows extensive peripheral retinal pigment epithelium (RPE) atrophy but relative preservation of the optic nerve, macula, and arterioles. CDVA was 20/30. Note the wide differences in phenotypes between panels A and B, despite involvement of the same gene. C, Fundus of a 45-year-old woman with mutations in the PCARE and CRB1 genes shows arteriolar attenuation, prominent RPE hyperplasia (so-called bone spicules) posterior to the equator, and lacunar RPE atrophy in the periphery. A tiny island of healthy retina remains in the fovea. CDVA was 20/100.