Table 2.
Visual Acuity and Spectral-Domain OCT Measurements for Each Phenotype and Control Participant
| Bothnia Dystrophy (n = 4 Eyes) | Newfoundland Rod–Cone Dystrophy (n = 34 Eyes) | Retinitis Punctata Albescens (n = 4 Eyes) | Control Participants (n = 28 Eyes) | P Value∗ | P Value† | |
|---|---|---|---|---|---|---|
| Visual acuity (logMAR) | ||||||
| Initial | 0.35 ± 0.31 | 0.53 ± 0.50 | –0.03 ± 0.05 | — | — | 0.27 |
| Final | 0.63 ± 0.59 | 0.52 ± 0.45 | 0.18 ± 0.24 | — | — | 0.43 |
| Foveal thickness | ||||||
| Initial | 109.25 ± 42.91 | 167.50 ± 38.22 | 204.75 ± 3.30 | 226.79 ± 20.71 | <0.01 | 0.05‡ |
| Final | 94.25 ± 40.06 | 148.22 ± 49.15 | 204.75 ± 2.75 | — | — | 0.13§ |
| EZ width | ||||||
| Initial | 3096 ± 2552 | 6229 ± 2771 | 7306 ± 353 | — | — | 0.16 |
| Final | 2944 ± 2481 | 6331 ± 2675 | 7276 ± 360 | — | — | 0.24 |
| Nasal | ||||||
| At 1500 | ||||||
| Initial | 243.50 ± 13.38 | 268.77 ± 22.19 | 316.50 ± 49.57 | 347.21 ± 19.29 | <0.01 | 0.04‡ |
| Final | 240.50 ± 15.33 | 258.83 ± 20.63 | 312.75 ± 42.52 | — | — | 0.05‡ |
| At 3000 μm | ||||||
| Initial | 307.00 ± 42.61 | 243.14 ± 30.05 | 265.50 ± 29.49 | 293.32 ± 24.27 | <0.01 | 0.03|| |
| Final | 266.25 ± 20.47 | 233.94 ± 28.71 | 248.00 ± 36.15 | — | — | 0.28 |
| Temporal | ||||||
| At 1500 μm | ||||||
| Initial | 209.25 ± 19.87 | 241.64 ± 33.92 | 308.00 ± 46.05 | 321.46 ± 19.03 | <0.01 | 0.03¶ |
| Final | 209.75 ± 12.66 | 229.78 ± 28.04 | 303.00 ± 43.95 | — | — | 0.02¶ |
| At 3000 μm | ||||||
| Initial | 183.75 ± 16.56 | 212.41 ± 39.32 | 236.25 ± 28.63 | 257.89 ± 17.08 | <0.01 | 0.34 |
| Final | 181.75 ± 16.36 | 205.78 ± 35.99 | 231.25 ± 27.54 | — | — | 0.33 |
EZ = ellipsoid zone; logMAR = logarithm of the minimum angle of resolution; – = not apllicable.
Values represented as mean±standard deviation, unless otherwise indicated.
Comparisons between control participants and the 3 pooled phenotypes using a linear mixed model adjusted for age with participant as the random effect.
Comparisons among the 3 phenotypes using a linear mixed model adjusted for age with participant as the random effect. Tukey’s correction was applied for pairwise comparisons.
Retinitis punctata albescens > Newfoundland rod–cone dystrophy.
Bothnia dystrophy < retinitis punctata albescens.
Newfoundland rod–cone dystrophy < Bothnia dystrophy.
Retinitis punctata albescens > Bothnia dystrophy and Newfoundland rod–cone dystrophy.