TY - JOUR
T1 - Baseline Visual Field Findings in the RUSH2A Study
T2 - Associated Factors and Correlation With Other Measures of Disease Severity
AU - Duncan, Jacque L.
AU - Liang, Wendi
AU - Maguire, Maureen G.
AU - Audo, Isabelle
AU - Ayala, Allison R.
AU - Birch, David G.
AU - Carroll, Joseph
AU - Cheetham, Janet K.
AU - Esposti, Simona Degli
AU - Durham, Todd A.
AU - Erker, Laura
AU - Farsiu, Sina
AU - Ferris, Frederick L.
AU - Heon, Elise
AU - Hufnagel, Robert B.
AU - Iannaccone, Alessandro
AU - Jaffe, Glenn J.
AU - Kay, Christine N.
AU - Michaelides, Michel
AU - Pennesi, Mark E.
AU - Sahel, José Alain
N1 - Publisher Copyright:
© 2020 Elsevier Inc.
PY - 2020/11
Y1 - 2020/11
N2 - Purpose: To report baseline visual fields in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) study. Design: Cross-sectional study within a natural history study. Methods: Setting: multicenter, international. Study population: Usher syndrome type 2 (USH2) (n = 80) or autosomal recessive nonsyndromic retinitis pigmentosa (ARRP) (n = 47) associated with biallelic disease-causing sequence variants in USH2A. Observation procedures: Repeatability of full-field static perimetry (SP) and between-eye symmetry of kinetic perimetry (KP) were evaluated with intraclass correlation coefficients (ICCs). The association of demographic and clinical characteristics with total hill of vision (VTOT) was assessed with general linear models. Associations between VTOT and other functional and morphologic measures were assessed using Spearman correlation coefficients and t tests. Main outcome measures: VTOT (SP) and III4e isopter area (KP). Results: USH2 participants had more severe visual field loss than ARRP participants (P < .001, adjusting for disease duration, age of enrollment). Mean VTOT measures among 3 repeat tests were 32.7 ± 24.1, 31.2 ± 23.4, and 31.7 ± 23.9 decibel-steradians (intraclass correlation coefficient [ICC] = 0.96). Better VA, greater photopic ERG 30-Hz flicker amplitudes, higher mean microperimetry sensitivity, higher central subfield thickness, absence of macular cysts, and higher III4e seeing area were associated with higher VTOT (all r > .48; P < .05). Mean III4e isopter areas for left (4561 ± 4426 squared degrees) and right eyes (4215 ± 4300 squared degrees) were concordant (ICC = 0.94). Conclusions: USH2 participants had more visual field loss than participants with USH2A-related ARRP, adjusting for duration of disease and age of enrollment. VTOT was repeatable and 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.
AB - Purpose: To report baseline visual fields in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) study. Design: Cross-sectional study within a natural history study. Methods: Setting: multicenter, international. Study population: Usher syndrome type 2 (USH2) (n = 80) or autosomal recessive nonsyndromic retinitis pigmentosa (ARRP) (n = 47) associated with biallelic disease-causing sequence variants in USH2A. Observation procedures: Repeatability of full-field static perimetry (SP) and between-eye symmetry of kinetic perimetry (KP) were evaluated with intraclass correlation coefficients (ICCs). The association of demographic and clinical characteristics with total hill of vision (VTOT) was assessed with general linear models. Associations between VTOT and other functional and morphologic measures were assessed using Spearman correlation coefficients and t tests. Main outcome measures: VTOT (SP) and III4e isopter area (KP). Results: USH2 participants had more severe visual field loss than ARRP participants (P < .001, adjusting for disease duration, age of enrollment). Mean VTOT measures among 3 repeat tests were 32.7 ± 24.1, 31.2 ± 23.4, and 31.7 ± 23.9 decibel-steradians (intraclass correlation coefficient [ICC] = 0.96). Better VA, greater photopic ERG 30-Hz flicker amplitudes, higher mean microperimetry sensitivity, higher central subfield thickness, absence of macular cysts, and higher III4e seeing area were associated with higher VTOT (all r > .48; P < .05). Mean III4e isopter areas for left (4561 ± 4426 squared degrees) and right eyes (4215 ± 4300 squared degrees) were concordant (ICC = 0.94). Conclusions: USH2 participants had more visual field loss than participants with USH2A-related ARRP, adjusting for duration of disease and age of enrollment. VTOT was repeatable and 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.
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U2 - 10.1016/j.ajo.2020.05.024
DO - 10.1016/j.ajo.2020.05.024
M3 - Article
C2 - 32446738
AN - SCOPUS:85089292776
SN - 0002-9394
VL - 219
SP - 87
EP - 100
JO - American journal of ophthalmology
JF - American journal of ophthalmology
ER -