Table 1.
Demographic information by clinical diagnosis (in alphabetical order).
| Diagnosis | N = (%) | Age (Range) |
SD Age | Gender % Male |
Referring EP Findings |
|---|---|---|---|---|---|
| AS | 1 (1.4) | 9 | - | 100% | Flat ERG (i.e., no retinal response) |
| BVMD | 3 (4.3) | 12.67 (5–18) | 6.43 | 67% | EOG: reduced Arden ratio |
| CD | 17 (24.3) | 13.94 (2–19) | 5.55 | 53% | ERG: Unrecordable cone responses without rod involvement |
| CSNB | 9 (12.9) | 18 (7–28) | 6.69 | 67% | ERG: Electronegativity, normal 30 Hz waveform, with residual rod response |
| LCA | 10 (14.3) | 5 (1–9) | 4.16 | 70% | ERG: No recordable retinal function VEP: Residual small amplitude flash VEP response |
| RCD | 18 (25.7) | 16.11 (6–22) | 4.54 | 56% | ERG: Reduced/absent rod response with less pronounced cone attenuation |
| STGD | 5 (7.1) | 19.6 (14–25) | 4.72 | 60% | ERG: Reduced 30 Hz flicker with preservation of rod function |
| XLRS | 7 (10) | 13.14 (7–21) | 4.43 | 100% | ERG: Electronegativity |
AS−Alström syndrome. BVMD−Best vitelliform macular dystrophy. CD−cone dystrophy. CSNB−congenital stationary night blindness. EOG−electro-oculogram. ERG−electroretinogram. LCA−Leber congenital amaurosis. RCD−rod cone dystrophy. STGD−Stargardt disease. VEP−visually evoked potential. XLRS−X-linked retinoschisis.