Duncan, Jacque L; Maguire, Maureen G; McDaniel, Lee S; Doucet, Nicole R; Audo, Isabelle; Ayala, Allison R; Cheetham, Janet K; ... Foundation Fighting Blindness Clinical Consortium Investigator G; + view all Duncan, Jacque L; Maguire, Maureen G; McDaniel, Lee S; Doucet, Nicole R; Audo, Isabelle; Ayala, Allison R; Cheetham, Janet K; Cheng, Peiyao; Durham, Todd A; Huckfeldt, Rachel M; Hufnagel, Robert B; Jayasundera, K Thiran; Khan, Naheed; Malbin, Brett; Maldonado, Ramiro S; Michaelides, Michel; Pennesi, Mark E; Weng, Christina Y; Zmejkoski, Alex; Aravind, Shobana; Ishikawa, Hiroshi; Birch, David G; Foundation Fighting Blindness Clinical Consortium Investigator G; - view fewer (2025) Characterization of Visual Field Loss Over 4 Years in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) Study. American Journal of Ophthalmology 10.1016/j.ajo.2025.03.039. (In press).

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Abstract

PURPOSE: To report visual field loss using static perimetry (SP) and kinetic perimetry (KP) over 4 years in the Rate of Progression of USH2A-related Retinal Degeneration (RUSH2A) study. DESIGN: Prospective, observational cohort study. SUBJECTS, PARTICIPANTS, AND/OR CONTROLS: Participants had USH2A-related rod-cone degeneration, visual acuity ≥ 20/80, and KP III4e ≥ 10° at baseline in the study eye. Preserved cohorts with baseline visual fields sufficient to detect progression were identified. METHODS: Participants were examined annually through 4 years. Mixed effects models were used to estimate the annual, standardized rate, and percentage rates of change. MAIN OUTCOME MEASURES: SP measures included hill of vision (total: VTOT, central 30°: V30, and peripheral: VPERIPH) and centrally weighted mean sensitivity (MScw). Percentages with 4-year progression exceeding the coefficient of repeatability (CoR) and with change meeting FDA-recommended criteria were estimated. KP seeing area (dB-steradian (sr)/degree) for I4e, III4e, and V4e isopters was calculated. RESULTS: The average decline with SP (95% CI) was 1.94 (1.62, 2.25) dB-sr/year for VTOT, 0.54 (0.45, 0.62) dB-sr/year for V30, 1.37 (1.11, 1.63) dB-sr/year for VPERIPH, and 0.56 (0.48, 0.64) dB/year for MScw. Average percentage decline per year was 8.6% (7.2, 10.0) for VTOT, 6.4% (5.3, 7.5) for V30, 13.6% (10.4, 16.7) for VPERIPH, and 5.6% (4.7, 6.4) for MScw. The standardized rate of change was greatest at -1.35 for MScw. Rates were higher in the preserved cohorts. Progression exceeding the CoR was 18% (11, 28) for VTOT, 21% (13, 31) for V30, 21% (13, 31) for VPERIPH and 17% (10, 27) for MScw. Progression exceeding an FDA-recommended threshold was 5% (2%, 12%) for all SP points and 45% (35%, 55%) for functional transition points. Average KP annual percentage decline was 13.1% (7.5, 18.5) for I4e, 12.1% (8.1,15.9) for III4e, and 9.2% (6.3,12.0) for V4e. CONCLUSIONS: All quantitative perimetry measures declined over 4 years. Progression was greater than the CoR in a relatively low percentage of eyes (17-21%); 45% exceeded the FDA-recommended threshold when only functional transition points were considered. Standardized rate of change was greatest for MScw. These measures are useful characterizations of vision loss in USH2A-related retinal degeneration.

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