Publication Date
6-1-2023
Journal
American Journal of Ophthalmology
DOI
10.1016/j.ajo.2023.02.002
PMID
36764426
PMCID
PMC10705000
PubMedCentral® Posted Date
6-1-2024
PubMedCentral® Full Text Version
Author MSS
Published Open-Access
yes
Keywords
Humans, Usher Syndromes, Retinal Degeneration, Visual Field Tests, Prospective Studies, Visual Fields, Visual Acuity, Tomography, Optical Coherence, Extracellular Matrix Proteins
Abstract
PURPOSE: To evaluate disease progression using static perimetry (SP) in patients with USH2A-related retinal degeneration, including Usher syndrome type 2 (USH2) and nonsyndromic autosomal recessive retinitis pigmentosa.
DESIGN: Prospective, observational cohort study.
METHODS: A total of 102 patients with biallelic disease-causing sequence variants in USH2A with baseline best-corrected visual acuity (BCVA) letter score ≥54 were recruited from 16 clinical sites in Europe and North America. SP, BCVA, full-field stimulus thresholds, spectral domain optical coherence tomography macular scans, and fundus-guided mesopic microperimetry were performed at baseline and annually. The main outcome measures were total hill of vision (V
RESULTS: The average decline (95% CI) was 2.05 (1.40, 2.70) decibel-steradian (dB-sr)/y for V
CONCLUSIONS: Quantitative measures of SP declined significantly over 2 years in USH2A-related retinal degeneration. The annual percentage rate of change was greatest for V