Jacque L Duncan, Maureen G Maguire, Lee S McDaniel, Nicole R Doucet, Isabelle Audo, Allison R Ayala, Janet K Cheetham, Peiyao Cheng, Todd A Durham, Rachel M Huckfeldt, Robert B Hufnagel, K Thiran Jayasundera, Naheed Khan, Brett Malbin, Ramiro S Maldonado, Michel Michaelides, Mark E Pennesi, Christina Y Weng, Alex Zmejkoski, Shobana Aravind, Hiroshi Ishikawa, David G Birch
{"title":"Characterization of Visual Field Loss Over 4 Years in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) Study.","authors":"Jacque L Duncan, Maureen G Maguire, Lee S McDaniel, Nicole R Doucet, Isabelle Audo, Allison R Ayala, Janet K Cheetham, Peiyao Cheng, Todd A Durham, Rachel M Huckfeldt, Robert B Hufnagel, K Thiran Jayasundera, Naheed Khan, Brett Malbin, Ramiro S Maldonado, Michel Michaelides, Mark E Pennesi, Christina Y Weng, Alex Zmejkoski, Shobana Aravind, Hiroshi Ishikawa, David G Birch","doi":"10.1016/j.ajo.2025.03.039","DOIUrl":null,"url":null,"abstract":"<p><strong>Purpose: </strong>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.</p><p><strong>Design: </strong>Prospective, observational cohort study.</p><p><strong>Subjects, participants, and/or controls: </strong>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.</p><p><strong>Methods: </strong>Participants were examined annually through 4 years. Mixed effects models were used to estimate the annual, standardized rate, and percentage rates of change.</p><p><strong>Main outcome measures: </strong>SP measures included hill of vision (total: V<sub>TOT</sub>, central 30°: V<sub>30</sub>, and peripheral: V<sub>PERIPH</sub>) 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.</p><p><strong>Results: </strong>The average decline with SP (95% CI) was 1.94 (1.62, 2.25) dB-sr/year for V<sub>TOT</sub>, 0.54 (0.45, 0.62) dB-sr/year for V<sub>30</sub>, 1.37 (1.11, 1.63) dB-sr/year for V<sub>PERIPH,</sub> and 0.56 (0.48, 0.64) dB/year for MS<sub>cw</sub>. Average percentage decline per year was 8.6% (7.2, 10.0) for V<sub>TOT</sub>, 6.4% (5.3, 7.5) for V<sub>30</sub>, 13.6% (10.4, 16.7) for V<sub>PERIPH</sub>, and 5.6% (4.7, 6.4) for MS<sub>cw</sub>. The standardized rate of change was greatest at -1.35 for MS<sub>cw</sub>. Rates were higher in the preserved cohorts. Progression exceeding the CoR was 18% (11, 28) for V<sub>TOT</sub>, 21% (13, 31) for V<sub>30</sub>, 21% (13, 31) for V<sub>PERIPH</sub> and 17% (10, 27) for MS<sub>cw</sub>. 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.</p><p><strong>Conclusions: </strong>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 MS<sub>cw</sub>. These measures are useful characterizations of vision loss in USH2A-related retinal degeneration.</p>","PeriodicalId":7568,"journal":{"name":"American Journal of Ophthalmology","volume":" ","pages":""},"PeriodicalIF":4.1000,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"American Journal of Ophthalmology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.ajo.2025.03.039","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"OPHTHALMOLOGY","Score":null,"Total":0}
引用次数: 0
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.
期刊介绍：
The American Journal of Ophthalmology is a peer-reviewed, scientific publication that welcomes the submission of original, previously unpublished manuscripts directed to ophthalmologists and visual science specialists describing clinical investigations, clinical observations, and clinically relevant laboratory investigations. Published monthly since 1884, the full text of the American Journal of Ophthalmology and supplementary material are also presented online at www.AJO.com and on ScienceDirect.
The American Journal of Ophthalmology publishes Full-Length Articles, Perspectives, Editorials, Correspondences, Books Reports and Announcements. Brief Reports and Case Reports are no longer published. We recommend submitting Brief Reports and Case Reports to our companion publication, the American Journal of Ophthalmology Case Reports.
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