{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T23:25:56Z","timestamp":1791501956998,"version":"4.3.4"},"reference-count":91,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P30 EY010572"],"award-info":[{"award-number":["P30 EY010572"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100001116","name":"Foundation Fighting Blindness Inc","doi-asserted-by":"publisher","award":["CD-CMM-0224-0873-HSC"],"award-info":[{"award-number":["CD-CMM-0224-0873-HSC"]}],"id":[{"id":"10.13039\/100001116","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000053","name":"National Eye Institute","doi-asserted-by":"publisher","award":["CD-CMM-0224-0873-HSC"],"award-info":[{"award-number":["CD-CMM-0224-0873-HSC"]}],"id":[{"id":"10.13039\/100000053","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000053","name":"National Eye Institute","doi-asserted-by":"publisher","award":["P30 EY005722"],"award-info":[{"award-number":["P30 EY005722"]}],"id":[{"id":"10.13039\/100000053","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000053","name":"National Eye Institute","doi-asserted-by":"publisher","award":["P30 EY002162"],"award-info":[{"award-number":["P30 EY002162"]}],"id":[{"id":"10.13039\/100000053","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000009","name":"Foundation for the National Institutes of Health","doi-asserted-by":"publisher","award":["P30 EY010572"],"award-info":[{"award-number":["P30 EY010572"]}],"id":[{"id":"10.13039\/100000009","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100001818","name":"Research to Prevent Blindness","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001818","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006668","name":"Oregon Health and Science University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006668","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["ajo.com","clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["American Journal of Ophthalmology"],"published-print":{"date-parts":[[2025,8]]},"DOI":"10.1016\/j.ajo.2025.04.034","type":"journal-article","created":{"date-parts":[[2025,5,3]],"date-time":"2025-05-03T18:42:28Z","timestamp":1746297748000},"page":"336-349","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":2,"special_numbering":"C","title":["Natural History of Microperimetry and Optical Coherence Tomography in USH2A-Retinopathy: A Structure-Function Association Study"],"prefix":"10.1016","volume":"276","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6446-3846","authenticated-orcid":false,"given":"Ajoy","family":"Vincent","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1535-1439","authenticated-orcid":false,"given":"Wendi","family":"Liang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maureen G.","family":"Maguire","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9593-6412","authenticated-orcid":false,"given":"Jacque L.","family":"Duncan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1483-7536","authenticated-orcid":false,"given":"Allison R.","family":"Ayala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul S.","family":"Bernstein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8640-1029","authenticated-orcid":false,"given":"Joseph","family":"Carroll","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4603-5375","authenticated-orcid":false,"given":"Janet K.","family":"Cheetham","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Todd A.","family":"Durham","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sina","family":"Farsiu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carel B.","family":"Hoyng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachel M.","family":"Huckfeldt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Glenn J.","family":"Jaffe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5484-9339","authenticated-orcid":false,"given":"Jessica","family":"Loo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark E.","family":"Pennesi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos\u00e9-Alain","family":"Sahel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1749-0088","authenticated-orcid":false,"given":"Mandeep S.","family":"Singh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5800-0690","authenticated-orcid":false,"given":"Zane Z.","family":"Zemborain","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David G.","family":"Birch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8435-521X","authenticated-orcid":false,"given":"Eleonora M.","family":"Lad","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0001","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1097\/WCO.0b013e3283218807","article-title":"Update on usher syndrome","volume":"22","author":"Saihan","year":"2009","journal-title":"Curr Opin Neurol"},{"issue":"10","key":"10.1016\/j.ajo.2025.04.034_bib0002","doi-asserted-by":"crossref","first-page":"1403","DOI":"10.1136\/bjo-2022-321790","article-title":"Inherited causes of combined vision and hearing loss: clinical features and molecular genetics","volume":"107","author":"Guimaraes","year":"2023","journal-title":"Br J Ophthalmol"},{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0003","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1097\/MAO.0000000000002054","article-title":"Genetics of Usher syndrome: new insights from a meta-analysis","volume":"40","author":"Jouret","year":"2019","journal-title":"Otol Neurotol"},{"issue":"7","key":"10.1016\/j.ajo.2025.04.034_bib0004","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1136\/jmg.2009.075143","article-title":"Novel mutations in the long isoform of the USH2A gene in patients with Usher syndrome type II or non-syndromic retinitis pigmentosa","volume":"47","author":"McGee","year":"2010","journal-title":"J Med Genet"},{"key":"10.1016\/j.ajo.2025.04.034_bib0005","doi-asserted-by":"crossref","DOI":"10.1016\/j.exer.2020.108330","article-title":"USH2A-retinopathy: from genetics to therapeutics","volume":"201","author":"Toualbi","year":"2020","journal-title":"Exp Eye Res"},{"issue":"5","key":"10.1016\/j.ajo.2025.04.034_bib0006","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1002\/humu.24365","article-title":"Tissue-specific genotype-phenotype correlations among USH2A-related disorders in the RUSH2A study","volume":"43","author":"Hufnagel","year":"2022","journal-title":"Hum Mutat"},{"issue":"10","key":"10.1016\/j.ajo.2025.04.034_bib0007","doi-asserted-by":"crossref","first-page":"1318","DOI":"10.1038\/ejhg.2014.283","article-title":"A detailed clinical and molecular survey of subjects with nonsyndromic USH2A retinopathy reveals an allelic hierarchy of disease-causing variants","volume":"23","author":"Lenassi","year":"2015","journal-title":"Eur J Hum Genet"},{"issue":"5","key":"10.1016\/j.ajo.2025.04.034_bib0008","doi-asserted-by":"crossref","first-page":"1151","DOI":"10.1016\/j.ophtha.2016.01.021","article-title":"Visual prognosis in USH2A-associated retinitis pigmentosa is worse for patients with Usher Syndrome Type IIa than for those with nonsyndromic retinitis pigmentosa","volume":"123","author":"Pierrache","year":"2016","journal-title":"Ophthalmology"},{"issue":"12","key":"10.1016\/j.ajo.2025.04.034_bib0009","doi-asserted-by":"crossref","first-page":"5532","DOI":"10.1167\/iovs.08-2009","article-title":"Disease course in patients with autosomal recessive retinitis pigmentosa due to the USH2A gene","volume":"49","author":"Sandberg","year":"2008","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"3","key":"10.1016\/j.ajo.2025.04.034_bib0010","doi-asserted-by":"crossref","first-page":"784","DOI":"10.1167\/iovs.03-0906","article-title":"Kinetics of visual field loss in Usher Syndrome type II","volume":"45","author":"Iannaccone","year":"2004","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"5","key":"10.1016\/j.ajo.2025.04.034_bib0011","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1097\/01.iae.0000246675.88911.2c","article-title":"Natural course of visual field loss in patients with type 2 Usher syndrome","volume":"27","author":"Fishman","year":"2007","journal-title":"Retina"},{"key":"10.1016\/j.ajo.2025.04.034_bib0012","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1016\/j.ajo.2020.05.024","article-title":"Baseline visual field findings in the RUSH2A study: associated factors and correlation with other measures of disease severity","volume":"219","author":"Duncan","year":"2020","journal-title":"Am J Ophthalmol"},{"issue":"9","key":"10.1016\/j.ajo.2025.04.034_bib0013","doi-asserted-by":"crossref","first-page":"1143","DOI":"10.1001\/jamaophthalmol.2013.4160","article-title":"Spectral-domain optical coherence tomography measures of outer segment layer progression in patients with X-linked retinitis pigmentosa","volume":"131","author":"Birch","year":"2013","journal-title":"JAMA Ophthalmol"},{"issue":"6","key":"10.1016\/j.ajo.2025.04.034_bib0014","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1001\/jamaophthalmol.2016.0502","article-title":"Quantification of ellipsoid zone changes in retinitis pigmentosa using en face spectral domain\u2013Optical coherence tomography","volume":"134","author":"Hariri","year":"2016","journal-title":"JAMA Ophthalmol"},{"issue":"10","key":"10.1016\/j.ajo.2025.04.034_bib0015","doi-asserted-by":"crossref","first-page":"7141","DOI":"10.1167\/iovs.11-7509","article-title":"Rod sensitivity, cone sensitivity, and photoreceptor layer thickness in retinal degenerative diseases","volume":"52","author":"Birch","year":"2011","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"6","key":"10.1016\/j.ajo.2025.04.034_bib0016","doi-asserted-by":"crossref","first-page":"BIO291","DOI":"10.1167\/iovs.17-21834","article-title":"Correlation between macular integrity assessment and optical coherence tomography imaging of Ellipsoid zone in Macular telangiectasia type 2","volume":"58","author":"Mukherjee","year":"2017","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"11","key":"10.1016\/j.ajo.2025.04.034_bib0017","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1167\/tvst.9.11.9","article-title":"The RUSH2A study: best-corrected visual acuity, full-field electroretinography amplitudes, and full-field stimulus thresholds at baseline","volume":"9","author":"Birch","year":"2020","journal-title":"Transl Vis Sci Technol"},{"issue":"4","key":"10.1016\/j.ajo.2025.04.034_bib0018","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1167\/tvst.10.4.23","article-title":"Tackling the challenges of product development through a collaborative Rare Disease network: The Foundation Fighting Blindness Consortium","volume":"10","author":"Durham","year":"2021","journal-title":"Transl Vis Sci Technol"},{"issue":"12","key":"10.1016\/j.ajo.2025.04.034_bib0019","doi-asserted-by":"crossref","first-page":"3717","DOI":"10.1002\/ajmg.a.62437","article-title":"Auditory and olfactory findings in patients with USH2A-related retinal degeneration-findings at baseline from the rate of progression in USH2A-related retinal degeneration natural history study (RUSH2A)","volume":"185","author":"Iannaccone","year":"2021","journal-title":"Am J Med Genet A"},{"issue":"3","key":"10.1016\/j.ajo.2025.04.034_bib0020","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1167\/iovs.63.3.17","article-title":"The RUSH2A study: dark-adapted visual fields in patients with retinal degeneration associated with biallelic variants in the USH2A gene","volume":"63","author":"Birch","year":"2022","journal-title":"Invest Ophthalmol Vis Sci"},{"key":"10.1016\/j.ajo.2025.04.034_bib0021","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1016\/j.ajo.2022.08.013","article-title":"Baseline microperimetry and OCT in the RUSH2A study: structure-function association and correlation with disease severity","volume":"244","author":"Lad","year":"2022","journal-title":"Am J Ophthalmol"},{"key":"10.1016\/j.ajo.2025.04.034_bib0022","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/j.ajo.2023.02.002","article-title":"Static perimetry in the rate of progression in USH2A-related retinal degeneration (RUSH2A) study: assessment through 2 years","volume":"250","author":"Duncan","year":"2023","journal-title":"Am J Ophthalmol"},{"key":"10.1016\/j.ajo.2025.04.034_bib0023","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.ajo.2023.03.006","article-title":"Change in cone structure over 24 months in USH2A-related retinal degeneration","volume":"252","author":"Duncan","year":"2023","journal-title":"Am J Ophthalmol"},{"issue":"8","key":"10.1016\/j.ajo.2025.04.034_bib0024","doi-asserted-by":"crossref","first-page":"7603","DOI":"10.3390\/ijms24087603","article-title":"A review of CRISPR tools for treating Usher syndrome: Applicability, safety, efficiency, and in vivo delivery","volume":"24","author":"Major","year":"2023","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.ajo.2025.04.034_bib0025","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1016\/j.omtn.2017.08.003","article-title":"USH2A Gene editing using the CRISPR system","volume":"8","author":"Fuster-Garcia","year":"2017","journal-title":"Mol Ther Nucleic Acids"},{"issue":"10","key":"10.1016\/j.ajo.2025.04.034_bib0026","doi-asserted-by":"crossref","first-page":"e381","DOI":"10.1038\/mtna.2016.89","article-title":"Antisense oligonucleotide-based splice correction for USH2A-associated retinal degeneration caused by a frequent deep-intronic mutation","volume":"5","author":"Slijkerman","year":"2016","journal-title":"Mol Ther Nucleic Acids"},{"issue":"5","key":"10.1016\/j.ajo.2025.04.034_bib0027","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1038\/gim.2015.30","article-title":"Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the association for molecular pathology","volume":"17","author":"Richards","year":"2015","journal-title":"Genet Med"},{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0028","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1167\/iovs.11-7640","article-title":"Validated automatic segmentation of AMD pathology including drusen and geographic atrophy in SD-OCT images","volume":"53","author":"Chiu","year":"2012","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"18","key":"10.1016\/j.ajo.2025.04.034_bib0029","doi-asserted-by":"crossref","first-page":"19413","DOI":"10.1364\/OE.18.019413","article-title":"Automatic segmentation of seven retinal layers in SDOCT images congruent with expert manual segmentation","volume":"18","author":"Chiu","year":"2010","journal-title":"Opt Express"},{"issue":"5","key":"10.1016\/j.ajo.2025.04.034_bib0030","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1097\/00006324-199205000-00006","article-title":"Position of the fovea centralis with respect to the optic nerve head","volume":"69","author":"Williams","year":"1992","journal-title":"Optom Vis Sci"},{"issue":"13","key":"10.1016\/j.ajo.2025.04.034_bib0031","first-page":"3318","article-title":"Evaluation of the fatigue effect when testing retinal sensitivity with microperimetry (OPKO SLO\/OCT)","volume":"50","author":"Sabates","year":"2009","journal-title":"Investigative Ophthalmology & Visual Science"},{"issue":"3","key":"10.1016\/j.ajo.2025.04.034_bib0032","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1111\/aos.16780","article-title":"Test-retest variability of mesopic microperimetry-associated parameters in patients with retinitis pigmentosa: REPEAT Study Report No. 2","volume":"103","author":"Karuntu","year":"2025","journal-title":"Acta Ophthalmol"},{"issue":"6","key":"10.1016\/j.ajo.2025.04.034_bib0033","doi-asserted-by":"crossref","first-page":"1030","DOI":"10.1364\/AO.27.001030","article-title":"Fatigue effects in automated perimetry","volume":"27","author":"Johnson","year":"1988","journal-title":"Appl Opt"},{"issue":"3","key":"10.1016\/j.ajo.2025.04.034_bib0034","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1001\/jamaophthalmol.2022.6254","article-title":"Changes in retinal sensitivity associated with cotoretigene Toliparvovec in X-linked retinitis pigmentosa with RPGR gene variations","volume":"141","author":"von Krusenstiern","year":"2023","journal-title":"JAMA Ophthalmol"},{"issue":"4","key":"10.1016\/j.ajo.2025.04.034_bib0035","doi-asserted-by":"crossref","first-page":"1497","DOI":"10.1167\/iovs.08-2843","article-title":"The glaucoma research community and FDA look to the future: a report from the NEI\/FDA CDER Glaucoma Clinical Trial Design and Endpoints Symposium","volume":"50","author":"Weinreb","year":"2009","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"4","key":"10.1016\/j.ajo.2025.04.034_bib0036","doi-asserted-by":"crossref","first-page":"833","DOI":"10.1016\/j.ophtha.2014.11.005","article-title":"Rates of decline in regions of the visual field defined by frequency-domain optical coherence tomography in patients with RPGR-mediated X-linked retinitis pigmentosa","volume":"122","author":"Birch","year":"2015","journal-title":"Ophthalmology"},{"issue":"5","key":"10.1016\/j.ajo.2025.04.034_bib0037","doi-asserted-by":"crossref","first-page":"1106","DOI":"10.1364\/BOE.2.001106","article-title":"Method for deriving visual field boundaries from OCT scans of patients with retinitis pigmentosa","volume":"2","author":"Hood","year":"2011","journal-title":"Biomed Opt Express"},{"issue":"2","key":"10.1016\/j.ajo.2025.04.034_bib0038","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1016\/S0002-9394(02)01825-1","article-title":"A computerized method of visual acuity testing: adaptation of the early treatment of diabetic retinopathy study testing protocol","volume":"135","author":"Beck","year":"2003","journal-title":"Am J Ophthalmol"},{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0039","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/0002-9394(82)90197-0","article-title":"New visual acuity charts for clinical research","volume":"94","author":"Ferris","year":"1982","journal-title":"Am J Ophthalmol"},{"issue":"8","key":"10.1016\/j.ajo.2025.04.034_bib0040","first-page":"1481","article-title":"Six month findings from a phase 1\/2 clinical study of subretinal gene therapy drug AGTC-501 for X-linked retinitis pigmentosa show clinically meaningful improvement in macular sensitivity","volume":"62","author":"Yang","year":"2021","journal-title":"Investig Ophthalmol Vis Sci"},{"issue":"10","key":"10.1016\/j.ajo.2025.04.034_bib0041","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1167\/tvst.13.10.15","article-title":"Endpoints and design for clinical trials in USH2A-related retinal degeneration: results and recommendations from the RUSH2A natural history study","volume":"13","author":"Maguire","year":"2024","journal-title":"Transl Vis Sci Technol"},{"issue":"8","key":"10.1016\/j.ajo.2025.04.034_bib0042","doi-asserted-by":"crossref","first-page":"1670","DOI":"10.1097\/IAE.0b013e318206d155","article-title":"Serial imaging and structure-function correlates of high-density rings of fundus autofluorescence in retinitis pigmentosa","volume":"31","author":"Robson","year":"2011","journal-title":"Retina"},{"issue":"6","key":"10.1016\/j.ajo.2025.04.034_bib0043","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1111\/j.1442-9071.2010.02321.x","article-title":"Changes of fundus autofluorescence, photoreceptor inner and outer segment junction line, and visual function in patients with retinitis pigmentosa","volume":"38","author":"Aizawa","year":"2010","journal-title":"Clin Exp Ophthalmol"},{"issue":"5","key":"10.1016\/j.ajo.2025.04.034_bib0044","doi-asserted-by":"crossref","first-page":"e177","DOI":"10.1111\/j.1755-3768.2010.01926.x","article-title":"Correlation of fundus autofluorescence with photoreceptor morphology and functional changes in eyes with retinitis pigmentosa","volume":"88","author":"Wakabayashi","year":"2010","journal-title":"Acta Ophthalmol"},{"issue":"4","key":"10.1016\/j.ajo.2025.04.034_bib0045","doi-asserted-by":"crossref","first-page":"725","DOI":"10.1007\/s00417-018-04234-6","article-title":"Fundus autofluorescence and ellipsoid zone (EZ) line width can be an outcome measurement in RHO-associated autosomal dominant retinitis pigmentosa","volume":"257","author":"Takahashi","year":"2019","journal-title":"Graefes Arch Clin Exp Ophthalmol"},{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0046","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-019-47251-z","article-title":"Multimodal structural disease progression of retinitis pigmentosa according to mode of inheritance","volume":"9","author":"Jauregui","year":"2019","journal-title":"Sci Rep"},{"key":"10.1016\/j.ajo.2025.04.034_bib0047","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1016\/j.visres.2012.08.017","article-title":"Fundus autofluorescence and optical coherence tomography in relation to visual function in Usher syndrome type 1 and 2","volume":"75","author":"Fakin","year":"2012","journal-title":"Vision Res"},{"issue":"6","key":"10.1016\/j.ajo.2025.04.034_bib0048","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1001\/jamaophthalmol.2016.0502","article-title":"Quantification of ellipsoid zone changes in retinitis pigmentosa using en face spectral domain-optical coherence tomography","volume":"134","author":"Hariri","year":"2016","journal-title":"JAMA Ophthalmol"},{"issue":"S13","key":"10.1016\/j.ajo.2025.04.034_bib0049","first-page":"1","article-title":"Relationship between preserved Ellipsoid zone area and choroidal vascularity index in autosomal dominant retinitis pigmentosa","volume":"13","author":"Verma","year":"2021","journal-title":"J Clin Exp Ophthalmol"},{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0050","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-017-11679-y","article-title":"Electroretinography reveals difference in cone function between syndromic and nonsyndromic USH2A patients","volume":"7","author":"Sengillo","year":"2017","journal-title":"Sci Rep"},{"issue":"2","key":"10.1016\/j.ajo.2025.04.034_bib0051","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1159\/000520329","article-title":"Spectral-domain optical coherence tomography analysis in syndromic and nonsyndromic forms of retinitis pigmentosa due to USH2A genetic variants","volume":"65","author":"Colombo","year":"2022","journal-title":"Ophthalmic Res"},{"issue":"3","key":"10.1016\/j.ajo.2025.04.034_bib0052","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1167\/tvst.7.3.13","article-title":"Intraobserver repeatability and interobserver reproducibility of Ellipsoid zone measurements in Retinitis Pigmentosa","volume":"7","author":"Strampe","year":"2018","journal-title":"Transl Vis Sci Technol"},{"key":"10.1016\/j.ajo.2025.04.034_bib0053","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.ajo.2017.03.012","article-title":"Quantitative analysis of retinal structure using spectral-domain optical coherence tomography in RPGR-associated retinopathy","volume":"178","author":"Tee","year":"2017","journal-title":"Am J Ophthalmol"},{"key":"10.1016\/j.ajo.2025.04.034_bib0054","doi-asserted-by":"crossref","first-page":"1176","DOI":"10.1016\/0002-9394(63)90186-7","article-title":"Symmetrical development of pigmentation as a specific feature of the fundus pattern in retinitis pigmentosa","volume":"55","author":"Biro","year":"1963","journal-title":"Am J Ophthalmol"},{"issue":"2","key":"10.1016\/j.ajo.2025.04.034_bib0055","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1136\/bjo.63.2.90","article-title":"Bilateral symmetry of vision disorders in typical retinitis pigmentosa","volume":"63","author":"Massof","year":"1979","journal-title":"Br J Ophthalmol"},{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0056","doi-asserted-by":"crossref","first-page":"3364","DOI":"10.1038\/s41598-020-60137-9","article-title":"Disease asymmetry and hyperautofluorescent ring shape in retinitis pigmentosa patients","volume":"10","author":"Jauregui","year":"2020","journal-title":"Sci Rep"},{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0057","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1136\/bjophthalmol-2020-315878","article-title":"Prevalence and genetic-phenotypic characteristics of patients with USH2A mutations in a large cohort of Chinese patients with inherited retinal disease","volume":"105","author":"Gao","year":"2021","journal-title":"Br J Ophthalmol"},{"issue":"5","key":"10.1016\/j.ajo.2025.04.034_bib0058","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1136\/bjophthalmol-2019-315786","article-title":"USH2A variants in Chinese patients with Usher syndrome type II and non-syndromic retinitis pigmentosa","volume":"105","author":"Zhu","year":"2021","journal-title":"Br J Ophthalmol"},{"issue":"7","key":"10.1016\/j.ajo.2025.04.034_bib0059","doi-asserted-by":"crossref","first-page":"754","DOI":"10.1016\/S0161-6420(87)33526-2","article-title":"Macular edema in Diabetic Retinopathy Study patients. Diabetic Retinopathy Study report number 12","volume":"94","author":"Ferris","year":"1987","journal-title":"Ophthalmology"},{"key":"10.1016\/j.ajo.2025.04.034_bib0060","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1016\/j.ajo.2016.09.029","article-title":"Outcomes with As-needed aflibercept and macular laser following the phase III VISTA DME trial: ENDURANCE 12-month extension study","volume":"173","author":"Wykoff","year":"2017","journal-title":"Am J Ophthalmol"},{"issue":"2","key":"10.1016\/j.ajo.2025.04.034_bib0061","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1016\/j.ophtha.2006.10.045","article-title":"Subgroup analysis of the MARINA study of ranibizumab in neovascular age-related macular degeneration","volume":"114","author":"Boyer","year":"2007","journal-title":"Ophthalmology"},{"issue":"10","key":"10.1016\/j.ajo.2025.04.034_bib0062","doi-asserted-by":"crossref","first-page":"1804","DOI":"10.1016\/j.ophtha.2007.06.047","article-title":"Visual acuity as an outcome measure in clinical trials of retinal diseases","volume":"114","author":"Beck","year":"2007","journal-title":"Ophthalmology"},{"issue":"2","key":"10.1016\/j.ajo.2025.04.034_bib0063","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1167\/tvst.4.2.14","article-title":"VFMA: topographic analysis of sensitivity data from full-field static perimetry","volume":"4","author":"Weleber","year":"2015","journal-title":"Transl Vis Sci Technol"},{"issue":"10097","key":"10.1016\/j.ajo.2025.04.034_bib0064","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1016\/S0140-6736(17)31868-8","article-title":"Efficacy and safety of voretigene neparvovec (AAV2-hRPE65v2) in patients with RPE65-mediated inherited retinal dystrophy: a randomised, controlled, open-label, phase 3 trial","volume":"390","author":"Russell","year":"2017","journal-title":"Lancet"},{"issue":"12","key":"10.1016\/j.ajo.2025.04.034_bib0065","doi-asserted-by":"crossref","first-page":"1130","DOI":"10.1016\/j.oret.2022.06.013","article-title":"Intravitreal delivery of rAAV2tYF-CB-hRS1 vector for gene augmentation therapy in patients with X-linked retinoschisis: 1-year clinical results","volume":"6","author":"Pennesi","year":"2022","journal-title":"Ophthalmol Retina"},{"issue":"13","key":"10.1016\/j.ajo.2025.04.034_bib0066","doi-asserted-by":"crossref","first-page":"5127","DOI":"10.1167\/iovs.16-20356","article-title":"Report from the NEI\/FDA Diabetic Retinopathy Clinical Trial Design and Endpoints Workshop","volume":"57","author":"Nair","year":"2016","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"9","key":"10.1016\/j.ajo.2025.04.034_bib0067","doi-asserted-by":"crossref","first-page":"3456","DOI":"10.1167\/iovs.17-22339","article-title":"Report from the NEI\/FDA Endpoints Workshop on age-related macular degeneration and inherited retinal diseases","volume":"58","author":"Csaky","year":"2017","journal-title":"Invest Ophthalmol Vis Sci"},{"issue":"7","key":"10.1016\/j.ajo.2025.04.034_bib0068","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1167\/tvst.9.7.2","article-title":"Advancing clinical trials for inherited retinal diseases: recommendations from the Second Monaciano Symposium","volume":"9","author":"Thompson","year":"2020","journal-title":"Transl Vis Sci Technol"},{"key":"10.1016\/j.ajo.2025.04.034_bib0069","unstructured":"Medicine NLo. ClinicalTrials.Gov - search results: microperimetery. https:\/\/clinicaltrials.gov\/search?term=microperimetry. Accessed August 8, 2023."},{"issue":"9923","key":"10.1016\/j.ajo.2025.04.034_bib0070","doi-asserted-by":"crossref","first-page":"1129","DOI":"10.1016\/S0140-6736(13)62117-0","article-title":"Retinal gene therapy in patients with choroideremia: initial findings from a phase 1\/2 clinical trial","volume":"383","author":"MacLaren","year":"2014","journal-title":"Lancet"},{"key":"10.1016\/j.ajo.2025.04.034_bib0071","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1016\/j.ajo.2018.06.011","article-title":"Two-year results after AAV2-mediated gene therapy for Choroideremia: the Alberta experience","volume":"193","author":"Dimopoulos","year":"2018","journal-title":"Am J Ophthalmol"},{"key":"10.1016\/j.ajo.2025.04.034_bib0072","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/j.ajo.2018.09.012","article-title":"Choroideremia Gene Therapy phase 2 clinical trial: 24-month results","volume":"197","author":"Lam","year":"2019","journal-title":"Am J Ophthalmol"},{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0073","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1097\/IAE.0000000000002360","article-title":"Changes in retinal sensitivity after Gene therapy in Choroideremia","volume":"40","author":"Fischer","year":"2020","journal-title":"Retina"},{"issue":"21","key":"10.1016\/j.ajo.2025.04.034_bib0074","doi-asserted-by":"crossref","first-page":"2231","DOI":"10.1056\/NEJMoa0802268","article-title":"Effect of gene therapy on visual function in Leber's congenital amaurosis","volume":"358","author":"Bainbridge","year":"2008","journal-title":"N Engl J Med"},{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0075","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1016\/j.ymthe.2017.09.014","article-title":"Safety and long-term efficacy of AAV4 gene therapy in patients with RPE65 Leber Congenital amaurosis","volume":"26","author":"Le Meur","year":"2018","journal-title":"Mol Ther"},{"issue":"9","key":"10.1016\/j.ajo.2025.04.034_bib0076","doi-asserted-by":"crossref","first-page":"2282","DOI":"10.1016\/j.ymthe.2018.05.025","article-title":"Retinal AAV8-RS1 gene therapy for X-linked retinoschisis: initial findings from a phase I\/IIa trial by Intravitreal delivery","volume":"26","author":"Cukras","year":"2018","journal-title":"Mol Ther"},{"issue":"3","key":"10.1016\/j.ajo.2025.04.034_bib0077","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1038\/s41591-020-0763-1","article-title":"Initial results from a first-in-human gene therapy trial on X-linked retinitis pigmentosa caused by mutations in RPGR","volume":"26","author":"Cehajic-Kapetanovic","year":"2020","journal-title":"Nat Med"},{"issue":"6","key":"10.1016\/j.ajo.2025.04.034_bib0078","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1001\/jamaophthalmol.2020.1032","article-title":"Safety and vision outcomes of subretinal gene therapy targeting cone photoreceptors in achromatopsia: A nonrandomized controlled trial","volume":"138","author":"Fischer","year":"2020","journal-title":"JAMA Ophthalmol"},{"issue":"10","key":"10.1016\/j.ajo.2025.04.034_bib0079","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1167\/tvst.9.10.9","article-title":"Edge of Scotoma sensitivity as a microperimetry clinical trial end point in USH2A retinopathy","volume":"9","author":"Charng","year":"2020","journal-title":"Transl Vis Sci Technol"},{"issue":"3","key":"10.1016\/j.ajo.2025.04.034_bib0080","doi-asserted-by":"crossref","first-page":"652","DOI":"10.3390\/genes14030652","article-title":"Natural disease course in Usher Syndrome patients harboring USH2A variant p.Cys870* in Exon 13, amenable to Exon skipping therapy","volume":"14","author":"\u010cadoni\u010d","year":"2023","journal-title":"Genes (Basel)"},{"issue":"5","key":"10.1016\/j.ajo.2025.04.034_bib0081","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1159\/000515148","article-title":"Clinical perspectives and trends: microperimetry as a trial endpoint in retinal disease","volume":"244","author":"Yang","year":"2021","journal-title":"Ophthalmologica"},{"issue":"5","key":"10.1016\/j.ajo.2025.04.034_bib0082","doi-asserted-by":"crossref","first-page":"714","DOI":"10.1111\/ceo.13753","article-title":"Test-retest repeatability of microperimetry in patients with retinitis pigmentosa caused by mutations in RPGR","volume":"48","author":"Buckley","year":"2020","journal-title":"Clin Exp Ophthalmol"},{"issue":"6","key":"10.1016\/j.ajo.2025.04.034_bib0083","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1167\/tvst.6.6.7","article-title":"Intersession test-retest variability of microperimetry in type 2 macular telangiectasia","volume":"6","author":"Wong","year":"2017","journal-title":"Transl Vis Sci Technol"},{"issue":"4","key":"10.1016\/j.ajo.2025.04.034_bib0084","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1006\/exer.2001.1059","article-title":"Concentric retinitis pigmentosa: clinicopathologic correlations","volume":"73","author":"Milam","year":"2001","journal-title":"Exp Eye Res"},{"key":"10.1016\/j.ajo.2025.04.034_bib0085","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.ajo.2020.08.002","article-title":"Locus-level changes in macular sensitivity in patients with Retinitis Pigmentosa treated with oral N-acetylcysteine","volume":"221","author":"Kong","year":"2021","journal-title":"Am J Ophthalmol"},{"key":"10.1016\/j.ajo.2025.04.034_bib0086","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1016\/j.ajo.2021.07.018","article-title":"Characterization of retinal function using microperimetry-derived metrics in both adults and children with RPGR-associated retinopathy","volume":"234","author":"Anikina","year":"2022","journal-title":"Am J Ophthalmol"},{"issue":"5","key":"10.1016\/j.ajo.2025.04.034_bib0087","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1167\/tvst.11.5.15","article-title":"Characterizing visual fields in RPGR related retinitis pigmentosa using Octopus static-automated perimetry","volume":"11","author":"Buckley","year":"2022","journal-title":"Transl Vis Sci Technol"},{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0088","doi-asserted-by":"crossref","DOI":"10.1117\/1.JBO.19.1.016014","article-title":"Lateral and axial measurement differences between spectral-domain optical coherence tomography systems","volume":"19","author":"Folgar","year":"2014","journal-title":"J Biomed Opt"},{"issue":"1","key":"10.1016\/j.ajo.2025.04.034_bib0089","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.oret.2020.07.006","article-title":"Quantification of geographic atrophy using spectral domain OCT in age-related macular degeneration","volume":"5","author":"Cleland","year":"2021","journal-title":"Ophthalmol Retina"},{"issue":"7","key":"10.1016\/j.ajo.2025.04.034_bib0090","doi-asserted-by":"crossref","first-page":"1347","DOI":"10.1097\/IAE.0000000000003448","article-title":"Validation of a deep learning-based algorithm for segmentation of the ellipsoid zone on optical coherence tomography images of an USH2A-related retinal degeneration clinical trial","volume":"42","author":"Loo","year":"2022","journal-title":"Retina"},{"key":"10.1016\/j.ajo.2025.04.034_bib0091","article-title":"Performance of deep learning models in automatic measurement of ellipsoid zone area on baseline optical coherence tomography (OCT) images from the rate of progression of USH2A-related retinal degeneration (RUSH2A) study","volume":"9","author":"Wang","year":"2022","journal-title":"Front Med (Lausanne)"}],"container-title":["American Journal of Ophthalmology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0002939425002168?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0002939425002168?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,2]],"date-time":"2025-11-02T10:46:29Z","timestamp":1762080389000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0002939425002168"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,8]]},"references-count":91,"alternative-id":["S0002939425002168"],"URL":"https:\/\/doi.org\/10.1016\/j.ajo.2025.04.034","relation":{},"ISSN":["0002-9394"],"issn-type":[{"value":"0002-9394","type":"print"}],"subject":[],"published":{"date-parts":[[2025,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Natural History of Microperimetry and Optical Coherence Tomography in USH2A-Retinopathy: A Structure-Function Association Study","name":"articletitle","label":"Article Title"},{"value":"American Journal of Ophthalmology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ajo.2025.04.034","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}]}}