Parekh, B; Duncan, JL; Samarakoon, L; Melia, M; Abalem, MF; Andrews, CA; Audo, I; ... Jayasundera, KT; + view all Parekh, B; Duncan, JL; Samarakoon, L; Melia, M; Abalem, MF; Andrews, CA; Audo, I; Ayala, AR; Bradley, C; Cheetham, JK; Dagnelie, G; Durham, TA; Huckfeldt, RM; Lacy, GD; Malbin, B; Michaelides, M; Musch, DC; Peck-Dimit, N; Sting, K; Weng, CY; Zmejkoski, AZ; Jayasundera, KT; - view fewer (2024) Self-Reported Functional Vision in USH2A-Associated Retinal Degeneration as Measured by the Michigan Retinal Degeneration Questionnaire. Investigative Ophthalmology & Visual Science , 65 (6) , Article 5. 10.1167/iovs.65.6.5.

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Abstract

Purpose: The purpose of this study was to evaluate self-reported functional vision (FV) and the impact of vision loss in patients with USH2A-associated retinal degeneration using a patient-reported outcome (PRO) measure, the Michigan Retinal Degeneration Questionnaire (MRDQ), to correlate MRDQ scores with well-established visual function measurements. // Design: An observational cross-sectional study (n = 93) of participants who had Usher Syndrome Type 2 (USH2, n = 55) or autosomal recessive non-syndromic retinitis pigmentosa (ARRP; n = 38) associated with biallelic variants in the USH2A gene. // Methods: The study protocol was approved by all ethics boards and informed consent was obtained from each participant. Participants completed the MRDQ at the 48-month study follow-up visit. Disease duration was self-reported by participants. One-way ANOVA was used to compare subgroups (clinical diagnosis, age, disease duration, and full-field stimulus threshold [FST] Blue-Red mediation) on mean scores per domain. Spearman correlation coefficients were used to assess associations between MRDQ domains and visual/retinal function assessments. // Results: Of the study sample, 58% were female participants and the median disease duration was 13 years. MRDQ domains were sensitive to differences between subgroups of clinical diagnosis, age, disease duration, and FST Blue-Red mediation. MRDQ domains correlated with static perimetry, microperimetry, full-field stimulus testing, and best-corrected visual acuity (BCVA). // Conclusions: Self-reported FV measured by the MRDQ, when applied to USH2 and ARRP participants, had good distributional characteristics and correlated well with visual function tests. MRDQ adds a new dimension of understanding on vision-related functioning and establishes this PRO tool as an informative measure in evaluating USH2A outcomes.

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