%0 Journal Article
%T Self-Reported Functional Vision in USH2A-Associated Retinal Degeneration as Measured by the Michigan Retinal Degeneration Questionnaire.
%A Parekh B
%A Duncan JL
%A Samarakoon L
%A Melia M
%A Abalem MF
%A Andrews CA
%A Audo I
%A Ayala AR
%A Bradley C
%A Cheetham JK
%A Dagnelie G
%A Durham TA
%A Huckfeldt RM
%A Lacy GD
%A Malbin B
%A Michaelides M
%A Musch DC
%A Peck-Dimit N
%A Stingl K
%A Weng CY
%A Zmejkoski AZ
%A Jayasundera KT
%A  
%J Invest Ophthalmol Vis Sci
%V 65
%N 6
%D 2024 Jun 3
%M 38833260
%F 4.925
%R 10.1167/iovs.65.6.5
%X <B>UNASSIGNED: </B>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.<BR><B>UNASSIGNED: </B>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.<BR><B>UNASSIGNED: </B>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.<BR><B>UNASSIGNED: </B>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).<BR><B>UNASSIGNED: </B>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.