Baseline Visual Field Findings in the RUSH2A Study: Associated Factors and Correlation With Other Measures of Disease Severity.



To report baseline visual fields in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) study.


Cross-sectional study within a natural history study.


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).


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).


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.





Published Version (Please cite this version)


Publication Info

Duncan, Jacque L, Wendi Liang, Maureen G Maguire, Isabelle Audo, Allison R Ayala, David G Birch, Joseph Carroll, Janet K Cheetham, et al. (2020). Baseline Visual Field Findings in the RUSH2A Study: Associated Factors and Correlation With Other Measures of Disease Severity. American journal of ophthalmology, 219(7). pp. 87–100. 10.1016/j.ajo.2020.05.024 Retrieved from

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Sina Farsiu

Anderson-Rupp Professor of Biolmedical Engineering

I am the director of the Vision and Image Processing (VIP) Laboratory. Along with my colleagues, we investigate how to improve early diagnostic methods and find new imaging biomarkers of ocular and neurological diseases in adults (e.g. age-related macular degeneration, diabetic retinopathy, Glaucoma, Alzheimer) and children (e.g. retinopathy or prematurity). We also develop automatic artificial intelligence machine learning and deep learning algorithms to detect/segment/quantify anatomical/pathological structures seen on medical images.

On another front, we study efficient signal processing based methods to overcome the theoretical and practical limitations that constrain the achievable resolution of any imaging device. Our approach, which is based on adaptive extraction and robust fusion of relevant information from the expensive and sophisticated as well as simple and cheap sensors, has found wide applications in improving the quality of imaging systems such as ophthalmic SD-OCT, digital X-ray mammography, electronic and optical microscopes, and commercial digital camcorders. We are also interested in pursuing statistical signal processing based projects, including super-resolution, demosaicing, deblurring, denoising, motion estimation, compressive sensing/adaptive sampling, and sensor fusion.


Glenn Jay Jaffe

Robert Machemer M.D. Distinguished Professor of Ophthalmology

I have an active basic and clinical research program. I have been principal investigator on several funded clinical studies including investigations of an oral ganciclovir prodrug to treat CMV retinitis and a study to determine the safety of a cyclosporine sustained drug delivery implant in the treatment of uveitis. I am principal investigator of an ongoing multicenter trial of a fluocinolone sustained drug delivery implant to treat patients with severe uveitis and a trial of this same implant to treat diabetic macular edema. Recently, I have investigated the use of ultrasonography and optical coherence tomography to diagnose macular edema in a variety of ocular diseases. I have maintained an ongoing basic research program to test the hypothesis that cytokines are important in the development of proliferative vitreoretinopathy, an important intraocular wound healing disorder. Education: I am actively involved in resident and fellow education. I give lectures to residents and fellows on a variety of topics related to uveitis and vitreoretinal diseases and train fellows to perform vitreoretinal surgery. I train post-doctoral students and medical students to conduct clinically relevant research. I serve as a mentor for the Duke third year medical school research program. I have served on a yearly basis as course faculty at many national and international meetings.

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