Dataset Information

The RUSH2A Study: Best-Corrected Visual Acuity, Full-Field Electroretinography Amplitudes, and Full-Field Stimulus Thresholds at Baseline.

ABSTRACT: Purpose:The purpose of this study was to evaluate baseline best corrected visual acuity (BCVA), full-field electroretinography (ERG), full-field stimulus thresholds (FST), and their relationship with baseline demographic and clinical characteristics in the Rate of Progression in Usher syndrome type 2 (USH2A)-related Retinal Degeneration (RUSH2A) multicenter study. Methods:Participants had Usher syndrome type 2 (USH2, N = 80) or autosomal recessive nonsyndromic retinitis pigmentosa (ARRP, N = 47) associated with biallelic variants in the USH2A gene. Associations of demographic and clinical characteristics with BCVA, ERG, and FST were assessed with regression models. Results:In comparison to ARRP, USH2 had worse BCVA (median 79 vs. 82 letters; P < 0.001 adjusted for age), lower rod-mediated ERG b-wave amplitudes (median 0.0 vs. 6.6 µV; P < 0.001) and 30 Hz flicker cone-mediated ERG amplitudes (median 1.5 vs. 3.1 µV; P = 0.001), and higher (white, blue, and red) FST thresholds (means [-26, -31, -23 dB] vs. [-39, -45, -28 dB]; P < 0.001 for all stimuli). After adjusting for age, gender, and duration of vision loss, the difference in BCVA between diagnosis groups was attenuated (P = 0.09). Only diagnosis was associated with rod- and cone-mediated ERG parameters, whereas both genders (P = 0.04) and duration of visual loss (P < 0.001) also were associated with FST white stimulus. Conclusions:USH2 participants had worse BCVA, ERG, and FST than ARRP participants. FST was strongly associated with duration of disease; it remains to be determined whether it will be a sensitive measure of progression. Translational Relevance:Using standardized research protocols in RUSH2A, measures have been identified to monitor disease progression and treatment response and differentiate features of prognostic relevance between USH2 and ARRP participants with USH2A mutations.

SUBMITTER: Birch DG

PROVIDER: S-EPMC7552938 | biostudies-literature | 2020 Oct

REPOSITORIES: biostudies-literature

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The RUSH2A Study: Best-Corrected Visual Acuity, Full-Field Electroretinography Amplitudes, and Full-Field Stimulus Thresholds at Baseline.

Birch David G DG Cheng Peiyao P Duncan Jacque L JL Ayala Allison R AR Maguire Maureen G MG Audo Isabelle I Cheetham Janet K JK Durham Todd A TA Fahim Abigail T AT Ferris Frederick L FL Heon Elise E Huckfeldt Rachel M RM Iannaccone Alessandro A Khan Naheed W NW Lad Eleonora M EM Michaelides Michel M Pennesi Mark E ME Stingl Katarina K Vincent Ajoy A Weng Christina Y CY

Translational vision science & technology 20201008 11

<h4>Purpose</h4>The purpose of this study was to evaluate baseline best corrected visual acuity (BCVA), full-field electroretinography (ERG), full-field stimulus thresholds (FST), and their relationship with baseline demographic and clinical characteristics in the Rate of Progression in Usher syndrome type 2 (<i>USH2A</i>)-related Retinal Degeneration (RUSH2A) multicenter study.<h4>Methods</h4>Participants had Usher syndrome type 2 (USH2, <i>N</i> = 80) or autosomal recessive nonsyndromic retini ...[more]

PMID: 33133772

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Project description:BackgroundSelf-reported questions on blindness and vision problems are collected in many national surveys. Recently released surveillance estimates on the prevalence of vision loss used self-reported data to predict variation in the prevalence of objectively measured acuity loss among population groups for whom examination data are not available. However, the validity of self-reported measures to predict prevalence and disparities in visual acuity has not been established.ObjectiveThis study aimed to estimate the diagnostic accuracy of self-reported vision loss measures compared to best-corrected visual acuity (BCVA), inform the design and selection of questions for future data collection, and identify the concordance between self-reported vision and measured acuity at the population level to support ongoing surveillance efforts.MethodsWe calculated accuracy and correlation between self-reported visual function versus BCVA at the individual and population level among patients from the University of Washington ophthalmology or optometry clinics with a prior eye examination, randomly oversampled for visual acuity loss or diagnosed eye diseases. Self-reported visual function was collected via telephone survey. BCVA was determined based on retrospective chart review. Diagnostic accuracy of questions at the person level was measured based on the area under the receiver operator curve (AUC), whereas population-level accuracy was determined based on correlation.ResultsThe survey question, "Are you blind or do you have serious difficulty seeing, even when wearing glasses?" had the highest accuracy for identifying patients with blindness (BCVA ≤20/200; AUC=0.797). The highest accuracy for detecting any vision loss (BCVA <20/40) was achieved by responses of "fair," "poor," or "very poor" to the question, "At the present time, would you say your eyesight, with glasses or contact lenses if you wear them, is excellent, good, fair, poor, or very poor" (AUC=0.716). At the population level, the relative relationship between prevalence based on survey questions and BCVA remained stable for most demographic groups, with the only exceptions being groups with small sample sizes, and these differences were generally not significant.ConclusionsAlthough survey questions are not considered to be sufficiently accurate to be used as a diagnostic test at the individual level, we did find relatively high levels of accuracy for some questions. At the population level, we found that the relative prevalence of the 2 most accurate survey questions were highly correlated with the prevalence of measured visual acuity loss among nearly all demographic groups. The results of this study suggest that self-reported vision questions fielded in national surveys are likely to yield an accurate and stable signal of vision loss across different population groups, although the actual measure of prevalence from these questions is not directly analogous to that of BCVA.

Project description:PurposeMultifocal electroretinogram (mfERG) shows great utility as a screening tool to detect early hydroxychloroquine (HCQ) retinopathy, but its widespread use is limited by the lack of accessibility and long test duration. In this study, we evaluated a novel concentric 5-ring mfERG stimulus to provide a simplified and rapid protocol for screening HCQ toxicity.MethodsPatients referred for HCQ retinopathy screening were consented to this observational cross-sectional study. Patients with amblyopia, high refractive error (more than 8 diopters), other retinal diseases precluding appropriate evaluation or history of retinal surgery were excluded. The data were collected from patients undergoing HCQ screening at a single center from July 2019 to March 2020. Patients were tested with the new concentric 5-ring mfERG stimulus, standard 61-hexagon mfERG stimulus, spectral domain optical coherence tomography and automated 10-2 visual fields. For the main outcome, the 5-ring mfERG was compared to 61-hexagon stimulus to determine the time-to-test completion and assess the association between ring (R1-R5) amplitude and ring ratio compared against cumulative dose, dose by real body weight and duration of therapy using Pearson correlation.ResultsIn total, 52 patients (104 eyes; 5 males and 47 females) were recruited with a mean age of 59 years (range 23-85 years). The 5-ring protocol was markedly quicker to perform (1.3 ± 0.2 min; mean (SD)) compared to the 61-hexagon protocol (5.2 ± 0.6 min), p < 0.0001; n = 10 patients. The new R2/R5 ring ratio showed a moderate correlation with daily dose (r = - 0.640), cumulative dose (r = - 0.581) and duration of therapy (r = - 0.417). Similar correlations were observed with the new R2/R4 ring ratio which were not significantly different from the new R2/R5 correlation coefficients. The new R2/R5 ring ratio demonstrated a stronger correlation with daily (p = 0.002) and cumulative dose (p = 0.0001) compared to the 61-hexagon stimulus.ConclusionsIn this exploratory study, our novel 5-ring mfERG protocol significantly shortened data acquisition time while providing comparable results to the standard 61-hexagon stimulus for detecting HCQ-induced electrophysiological changes that are correlated with HCQ dosages and treatment duration. Our protocol has the potential to be more clinically practical by simplifying routine screening.

Dataset Information

The RUSH2A Study: Best-Corrected Visual Acuity, Full-Field Electroretinography Amplitudes, and Full-Field Stimulus Thresholds at Baseline.

Publications

The RUSH2A Study: Best-Corrected Visual Acuity, Full-Field Electroretinography Amplitudes, and Full-Field Stimulus Thresholds at Baseline.

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