June 2020
Volume 61, Issue 7
ARVO Annual Meeting Abstract  |   June 2020
Baseline visual fields in the RUSH2A study: Associated factors and correlation with measures of disease severity
Author Affiliations & Notes
  • Jacque L Duncan
    Ophthalmology, Univ of California - SF, San Francisco, California, United States
  • Wendi Liang
    Jaeb Center for Health Research, Tampa, Florida, United States
  • Isabelle S Audo
    Institut De La Vision, Paris, France
  • David G Birch
    Retina Foundation of the Southwest, Dallas, Texas, United States
  • Joseph Carroll
    Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Janet K. Cheetham
    Foundation Fighting Blindness, Columbia, Maryland, United States
  • Laura R Erker
    Casey Eye Institute, Oregon Health and Sciences University, Portland, Oregon, United States
  • Frederick L Ferris
    Ophthalmic Research Consultants, Waxhaw, North Carolina, United States
  • Elise Heon
    Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
  • Robert Hufnagel
    National Eye Institute, Bethesda, Maryland, United States
  • Alessandro Iannaccone
    Ophthalmology, Duke University, Durham, North Carolina, United States
  • Glenn J Jaffe
    Duke Eye Center, Duke University, Durham, North Carolina, United States
  • Christine N Kay
    Vitreo Retinal Associates, Gainesville, Florida, United States
  • Maureen Maguire
    University of Pennsylvania, Philadelphia, Pennsylvania, United States
  • Michel Michaelides
    Moorfields Eye Hospital, London, United Kingdom
  • Mark E Pennesi
    Casey Eye Institute, Oregon Health and Sciences University, Portland, Oregon, United States
  • Footnotes
    Commercial Relationships   Jacque Duncan, None; Wendi Liang, None; Isabelle Audo, None; David Birch, None; Joseph Carroll, None; Janet Cheetham, None; Laura Erker, None; Frederick Ferris, None; Elise Heon, None; Robert Hufnagel, None; Alessandro Iannaccone, None; Glenn Jaffe, None; Christine Kay, None; Maureen Maguire, None; Michel Michaelides, None; Mark Pennesi, None
  • Footnotes
    Support  Foundation Fighting Blindness
Investigative Ophthalmology & Visual Science June 2020, Vol.61, 3364. doi:
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    • Get Citation

      Jacque L Duncan, Wendi Liang, Isabelle S Audo, David G Birch, Joseph Carroll, Janet K. Cheetham, Laura R Erker, Frederick L Ferris, Elise Heon, Robert Hufnagel, Alessandro Iannaccone, Glenn J Jaffe, Christine N Kay, Maureen Maguire, Michel Michaelides, Mark E Pennesi; Baseline visual fields in the RUSH2A study: Associated factors and correlation with measures of disease severity. Invest. Ophthalmol. Vis. Sci. 2020;61(7):3364.

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

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Purpose : To address limited data characterizing retinal degeneration related to disease-causing sequence variants in USH2A, the Foundation Fighting Blindness Consortium is conducting a 4-year multicenter, international natural history study: Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A). We report baseline data on visual field testing (including reproducibility) and its relationship with baseline characteristics and other functional and structural measures.

Methods : Intra-class coefficients (ICC) were used to assess reproducibility of triplicate full-field static perimetry (SP) and between-eye symmetry of seeing areas of kinetic perimetry (KP). General linear models were used to assess the association of demographic and clinical characteristics with total hill of vision (VTOT, decibel-steradians) derived from SP. Association between VTOT and other measures (including KP seeing area (deg2), visual acuity (VA), macular sensitivity on fundus-guided microperimetry, ERG amplitudes and latencies, and SD-OCT measures of central subfield thickness (CST) within the center 1mm and presence of intraretinal cysts) was assessed using Spearman correlation coefficients and t-tests.

Results : Participants with Usher syndrome type 2 (N=80) had more severe visual field loss (determined by VTOT) than those with non-syndromic retinitis pigmentosa (RP) (N=47) (22.5 ± 21.5 vs 37.1 ± 24.7, P=.002, adjusting for duration of disease and age of vision loss onset). No other baseline characteristics were associated with mean VTOT. Mean VTOT measures among 3 repeat tests were 32.7 ± 24.1, 31.2 ± 23.4, and 31.7 ± 23.9 (ICC = .97). Better VA, higher photopic ERG 30Hz flicker amplitudes, higher mean sensitivity, higher CST, absence of macular cysts, and higher III4e seeing area were associated with higher VTOT (all r > .48; all P <.05). Mean KP III4e isopter areas for left (4561 ± 4426) and right eyes (4136 ±4249) were concordant (ICC=.92).

Conclusions : After adjusting for duration of disease and age of onset, participants with USH2A-related Usher syndrome had more severe visual field loss than with USH2A-related non-syndromic RP. The volumetric measure of VTOT was highly reproducible and strongly correlated with other functional and structural metrics, suggesting it may be a good summary measure of disease severity in patients with USH2A-related retinal degeneration.

This is a 2020 ARVO Annual Meeting abstract.


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