Publication Date
1-1-2025
Journal
Ophthalmology Science
DOI
10.1016/j.xops.2024.100648
PMID
39811264
PMCID
PMC11730847
PubMedCentral® Posted Date
11-8-2024
PubMedCentral® Full Text Version
Post-print
Published Open-Access
yes
Keywords
Electroretinography (ERG), Full-field stimulus thresholds (FST), Best-corrected visual acuity (BCVA), Retinal degeneration, USH2A
Abstract
PURPOSE: To describe progression of best-corrected visual acuity (BCVA), full-field stimulus thresholds (FST), and electroretinography (ERG) over 4 years in the
DESIGN: Prospective natural history study.
PARTICIPANTS: Participants (n = 105) with biallelic disease-causing sequence variants in USH2A and BCVA letter scores of ≥54 were included.
METHODS: BCVA, FST, fundus-guided microperimetry, static perimetry, and spectral domain OCT 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. Associations of change from baseline to 4 years between BCVA, FST, ERG, and other metrics were assessed with Spearman correlation coefficients (r
MAIN OUTCOME MEASURES: Best-corrected visual acuity, FST, and ERG.
RESULTS: The annual rate of decline in BCVA was 0.83 (95% confidence interval: 0.65-1.02) letters/year. For FST, the change was 0.09 (0.07-0.11) log cd.s/m
CONCLUSIONS: Best-corrected visual acuity was not a sensitive measure of progression over 4 years. Full-field stimulus threshold 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.
FINANCIAL DISCLOSURES: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
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