June 2022
Volume 63, Issue 7
Open Access
ARVO Annual Meeting Abstract  |   June 2022
Visual acuity and full-field stimulus thresholds (FST) over 2 years in the RUSH2A study: annual rates of change from mixed effects modeling
Author Affiliations & Notes
  • Peiyao Cheng
    Jaeb Center for Health Research, Tampa, Florida, United States
  • David G Birch
    Retina Foundation of the Southwest, Dallas, Texas, United States
  • Jacque L Duncan
    Department of Ophthalmology, University of California San Francisco, San Francisco, California, United States
  • Frederick L Ferris
    Ophthalmic Research Consultants, Waxhaw, North Carolina, United States
  • Maureen G Maguire
    Jaeb Center for Health Research, Tampa, Florida, United States
  • Allison R. Ayala
    Jaeb Center for Health Research, Tampa, Florida, United States
  • Janet K Cheetham
    Foundation Fighting Blindness Inc, Columbia, Maryland, United States
  • Todd Durham
    Foundation Fighting Blindness Inc, Columbia, Maryland, United States
  • Abigail T Fahim
    University of Michigan, Ann Arbor, Michigan, United States
  • Rachel M Huckfeldt
    Massachusetts Eye and Ear, Boston, Massachusetts, United States
  • Michel Michaelides
    Moorfields Eye Hospital NHS Foundation Trust, London, London, United Kingdom
  • Mark E Pennesi
    Oregon Health & Science University Casey Eye Institute, Portland, Oregon, United States
  • Jose Alain Sahel
    Department of Ophthalmology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States
  • Katarina Stingl
    Eberhard Karls Universitat Tubingen, Tubingen, Baden-Württemberg, Germany
  • Ajoy Vincent
    The Hospital for Sick Children, Toronto, Ontario, Canada
  • Christina Y Weng
    Baylor College of Medicine, Houston, Texas, United States
  • Footnotes
    Commercial Relationships   Peiyao Cheng None; David Birch Nacuity, ProQR, Editas, AGTC, Iveric, 4D Therapeutics, Novartis, Foundation Fighting Blindness, Code C (Consultant/Contractor), ProQR, AGTC, 4D Molecular Therapeutics, Code F (Financial Support); Jacque Duncan AGTC, DTx Therapeutics, Editas, Eyevensys, Gyroscope, Helios, Nacuity, Spark Therapeutics, SparingVision, ProQR Therapeutics, PYC Therapeutics, Verdere Bio II, Code C (Consultant/Contractor), Acucela, Allergan/Abbvie, Second Sight Medical Products, Biogen/Nightstarx Therapeutics, Neurotech USA , Code F (Financial Support), RxSight, Inc (spouse), Code I (Personal Financial Interest); Frederick Ferris Foundation Fighting Blindness, JAMA Ophthalmology, Diabetic Retinopathy Clinical Research Network, Norvo Nordisk, Apellis, Genentech, Roche, Novartis, Eyevensys, Kodiak, 4D Molecular Therapeutics, Adverum, Annexon , Code C (Consultant/Contractor), Bausch and Lomb, Code P (Patent); Maureen Maguire None; Allison Ayala None; Janet Cheetham FFB, Code C (Consultant/Contractor), Abbvie, Code I (Personal Financial Interest); Todd Durham Novartis, Code C (Consultant/Contractor); Abigail Fahim Ionis, Code I (Personal Financial Interest); Rachel Huckfeldt ProQR, Code C (Consultant/Contractor), ProQR, Code F (Financial Support); Michel Michaelides None; Mark Pennesi 4D Molecular Therapeutics, Abbvie, Adverum, AGTC, Astellas Pharmaceuticals, Astena, Bayer, Biogen, Blue Rock, DTx Therapeutics, Editas, Endogena, Eyevensys,Horama, IVERIC, Janssen, Nacuity Pharmaceuticals, Novartis, Ocugen, Ora, ProQR, PYC Therapeutics, RegenexBio, Roche, Sanofi, Saliogen, Sparing Vision, Viewpoint Therapeutics, Vedere, Code C (Consultant/Contractor), AGTC, Biogen, Editas, FFB, ProQR, Sanofi, Code F (Financial Support), Astena, DTx Therapeutics, Endogena, Nacuity Pharmaceuticals, Ocugen, Code I (Personal Financial Interest), Astena, DTx Therapeutics, Eyevensys, Horama, Nacuity Pharmaceuticals, Ocugen, Sparking Vision, Vedere, Akous, Gensight, Code S (non-remunerative); Jose Sahel LabEx LIFESENSES (ANR-10-LABX-65), IHU FOReSIGHT (ANR-18-IAHU-01), RHU LIGHT4DEAF (ANR-15-RHUS-0001), Foundation Fighting Blindness Center Grant, Code F (Financial Support), Pixium Vision, GenSight Biologics, Sparing Vision, Prophesee, Chronolife, Tilak Healthcare, Vegavect, Newsight, Replay Therapeutics, SharpEye , Code I (Personal Financial Interest); Katarina Stingl ProQR, ViGeneron, Novartis, SANTEN, Rejuveron, Lightning health, Code C (Consultant/Contractor), ProQR, Code F (Financial Support), Novartis, CRA, Code R (Recipient); Ajoy Vincent None; Christina Weng Alcon, Allergan/AbbVie, Alimera Sciences, DORC, Novartis, Regeneron, REGENXBIO, Genentech, Code C (Consultant/Contractor)
  • Footnotes
    Support  Foundation Fighting Blindness
Investigative Ophthalmology & Visual Science June 2022, Vol.63, 4480 – F0267. doi:
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    • Get Citation

      Peiyao Cheng, David G Birch, Jacque L Duncan, Frederick L Ferris, Maureen G Maguire, Allison R. Ayala, Janet K Cheetham, Todd Durham, Abigail T Fahim, Rachel M Huckfeldt, Michel Michaelides, Mark E Pennesi, Jose Alain Sahel, Katarina Stingl, Ajoy Vincent, Christina Y Weng; Visual acuity and full-field stimulus thresholds (FST) over 2 years in the RUSH2A study: annual rates of change from mixed effects modeling. Invest. Ophthalmol. Vis. Sci. 2022;63(7):4480 – F0267.

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      © ARVO (1962-2015); The Authors (2016-present)

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Purpose : Best corrected visual acuity (BCVA) and FST are quantitative measures affected by inherited retinal degenerations (IRDs). There are few available estimates for rates of change in IRDs. Here we provide estimates of change over 2 years from the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) study using mixed effects models.

Methods : Participants with BCVA ≥ 20/80, stable fixation, and kinetic visual field of ≥ 10° at baseline in the study eye had BCVA (ETDRS letter score) and FST measured at baseline, 1, and 2 years. FST was measured with white, blue and red stimuli in triplicate and was performed only in centers having the required equipment. Mixed effects models were used to estimate the annual rates of change and percentage rates of change using log transformed data with 95% confidence intervals (95% CI). Sensitivity analyses were performed by down-weighting results from eyes with outlier rates of change. Spearman correlation coefficients (r) were calculated among BCVA and FST measures.

Results : The average decline of BCVA score [N=102] was 0.87 (0.48, 1.26) letters/year or 1.1 (0.6,1.6) %/year (Table 1). For FST measures [N=73], the average increase of thresholds was 1.28 (0.85, 1.71) dB/year for white stimulus, 1.25 (0.82, 1.67) dB/year for blue stimulus and 0.76 (0.42, 1.10) dB/year for red stimulus. Models that down-weighted outliers yielded lower rates of decline for BCVA and lower rates of increase for FST measures compared to models that weighted equally all data points. There was no significant correlation between 2-year changes in BCVA letter score and the three FST measures (r between -0.08 and -0.12; Table 2). 2-year changes in the three FST measures were highly correlated with each other (r between 0.61 and 0.77; p<0.001). Among eyes [N=65] that had FST testing at baseline and year 2, the response was cone mediated (FST white > -30 dB) in 31 (48%) at both times, rod mediated (FST white ≤ -30 dB) in 29 (45%) at both times and switched from rod- to cone-mediated in 5 (8%).

Conclusions : Based on 2 years of follow-up in RUSH2A, average decline in BCVA was small. Average white and blue FST values deteriorated a similar degree. Sensitivity changed from rod- to cone-mediated in 8% of patients.

This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.




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