Duncan, Jacque LLiang, WendiMaguire, Maureen GAudo, IsabelleAyala, Allison RBirch, David GCarroll, JosephCheetham, Janet KEsposti, Simona DegliDurham, Todd AErker, LauraFarsiu, SinaFerris, Frederick LHeon, EliseHufnagel, Robert BIannaccone, AlessandroJaffe, Glenn JKay, Christine NMichaelides, MichelPennesi, Mark ESahel, José-AlainFoundation Fighting Blindness Consortium Investigator Group2021-10-012021-10-012020-110002-93941879-1891https://hdl.handle.net/10161/23893<h4>Purpose</h4>To report baseline visual fields in the Rate of Progression in USH2A-related Retinal Degeneration (RUSH2A) study.<h4>Design</h4>Cross-sectional study within a natural history study.<h4>Methods</h4>Setting: multicenter, international.<h4>Study population</h4>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.<h4>Observation procedures</h4>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 (V_TOT) was assessed with general linear models. Associations between V_TOT and other functional and morphologic measures were assessed using Spearman correlation coefficients and t tests.<h4>Main outcome measures</h4>V_TOT (SP) and III4e isopter area (KP).<h4>Results</h4>USH2 participants had more severe visual field loss than ARRP participants (P < .001, adjusting for disease duration, age of enrollment). Mean V_TOT 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 V_TOT (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).<h4>Conclusions</h4>USH2 participants had more visual field loss than participants with USH2A-related ARRP, adjusting for duration of disease and age of enrollment. V_TOT 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.Foundation Fighting Blindness Consortium Investigator GroupRetinaHumansVision DisordersRetinitis PigmentosaDisease ProgressionExtracellular Matrix ProteinsElectroretinographySeverity of Illness IndexLongitudinal StudiesCross-Sectional StudiesVisual AcuityVisual FieldsResearch DesignAdultMiddle AgedFemaleMaleUsher SyndromesVisual Field TestsJournal article2021-10-01