Visual Acuity, Full-field Stimulus Thresholds, and Electroretinography for 4 Years in The Rate of Progression of USH2A-related Retinal Degeneration (RUSH2A) Study
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Bibliographic record
Abstract
Purpose To describe progression of best-corrected visual acuity (BCVA), full-field stimulus thresholds (FST), and electroretinography (ERG) over 4 years in the RUSH2A study and to assess their suitability as clinical trial endpoints. Design Prospective natural history study. Participants Participants (n=105) with biallelic disease-causing sequence variants in USH2A and BCVA letter score of ≥54 were included. Methods BCVA, FST, fundus-guided microperimetry, static perimetry and spectral domain optical coherence tomography were performed annually and ERG at baseline and 4 years only. Mixed effects models were used to estimate annual rates of change with 95% confidence intervals (CI). Associations of change from baseline to 4 years between BCVA, FST, ERG and other metrics were assessed with Spearman correlation coefficients (r s ). Main Outcome Measures BCVA, FST, ERG. Results The annual rate of decline in BCVA was 0.83 (95% CI: 0.65, 1.02) letters/year. For FST, the change was 0.09 (0.07, 0.11) log cd.s/m 2 /year for white threshold, 0.10 (0.08, 0.12) log cd.s/m 2 /year for blue threshold and 0.05 (0.04, 0.06) log cd.s/m 2 /year for red threshold. Changes were 22.6 (17.4, 28.2) %/year for white threshold, 26.0 (20.3, 32.1) %/year for blue threshold, and 12.3 (8.7, 16.0) %/year for red threshold. The high percentage of eyes with undetectable ERGs at baseline limited assessment of change. Conclusion BCVA was not a sensitive measure of progression over 4 years. FST was a more sensitive measure; however, additional information on the clinical relevance of changes in FST is needed before this test can be adopted as an endpoint for clinical trials.
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Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it