Table 3.
Demographic, clinical and morpho-functional data of the patients.
| N | Sex | Age | Onset | BCVA | VF | EZ | CS | Grade | SRI 5 mm Circle Area (mm2) | SRI Fovea Distance (mm) | Focal ERG (μV) | Submicrovolt 30 Hz (μV) | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| USH2A | 1 | F | 21 | 8 | 0 | 2 | 3 | 5 | 2 | 0 | 0 | 0.28 | 0.93 |
| 2 | F | 59 | 16 | 5 | 5 | 5 | 15 | 5 | 0 | 2.6 | 0.11 | 0.2 | |
| 3 | F | 62 | 25 | 5 | 5 | 5 | 15 | 5 | 7.2 | 0.3 | 0.11 | 0.07 | |
| 4 | M | 18 | 13 | 1 | 1 | 5 | 7 | 3 | 3.2 | 1.2 | 0.39 | 0.05 | |
| 5 | M | 49 | 30 | 3 | 5 | 5 | 13 | 5 | 0.3 | 1.6 | 0.26 | 0.08 | |
| 6 | F | 21 | 19 | 3 | 0 | 4 | 7 | 3 | 0 | 0 | 0.51 | 2.79 | |
| 7 | M | 17 | 15 | 0 | 0 | 2 | 2 | 1 | 0 | 0 | 1.07 | 0.73 | |
| 8 | M | 48 | 28 | 3 | 5 | 4 | 12 | 4 | 3.6 | 0.9 | 0.29 | 0.49 | |
| 9 | F | 27 | 21 | 0 | 0 | 1 | 1 | 1 | 0.1 | 2.3 | 1.32 | 1.68 | |
| 10 | M | 32 | 22 | 0 | 0 | 5 | 5 | 2 | 1.8 | 1.7 | 0.27 | 0.69 | |
| 11 | M | 31 | 20 | 0 | 1 | 3 | 4 | 2 | 0 | 2.6 | 0.63 | 0.4 | |
| 12 | M | 55 | 54 | 0 | 1 | 5 | 6 | 2 | 0 | 2.7 | 0.14 | 0.69 | |
| 13 | F | 36 | 17 | 3 | 1 | 5 | 9 | 3 | 0 | 3.6 | 0.12 | 0.69 | |
| 14 | M | 62 | 20 | 4 | 5 | 5 | 14 | 5 | 2.1 | 0.7 | 0.27 | 0.75 | |
| 15 | F | 47 | 44 | 4 | 5 | 5 | 14 | 5 | 1.1 | 1.1 | 0.21 | 0.4 | |
| 16 | F | 30 | 23 | 0 | 3 | 2 | 5 | 2 | 0 | 2.5 | 1.49 | 1.87 | |
| 17 | M | 42 | 15 | 3 | 5 | 5 | 13 | 5 | 2.4 | 0.4 | 0.19 | 0.59 | |
| 18 | M | 55 | 22 | 5 | 3 | 5 | 13 | 5 | 2.8 | 0.5 | 0.1 | 0.59 | |
| 19 | F | 59 | 58 | 0 | 2 | 1 | 3 | 1 | 0 | 0 | 1.99 | 1.93 | |
| 20 | M | 72 | 43 | 4 | 2 | 5 | 11 | 4 | 9.8 | 0.3 | 0.12 | 2.17 | |
| NSRP | 21 | M | 38 | 37 | 3 | 0 | 4 | 7 | 3 | 0 | 0 | 0.23 | 0.96 |
| 22 | F | 52 | 20 | 3 | 3 | 5 | 11 | 4 | 0.2 | 2 | 0.15 | 0.38 | |
| 23 | F | 35 | 12 | 1 | 3 | 4 | 8 | 3 | 0 | 3.5 | 0.12 | 0.79 |
BCVA: best-corrected visual acuity; CS: cumulative score; ERG: electroretinogram; EZ: ellipsoid zone; F: female; M: male; N: number; NSRP: non-syndromic retinitis pigmentosa; SRI: sub-RPE illumination; USH2A; Usher syndrome (USH) with variants in USH2A gene; VF: visual field.