{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T14:35:24Z","timestamp":1792852524516,"version":"4.5.0"},"reference-count":158,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2023,11,6]],"date-time":"2023-11-06T00:00:00Z","timestamp":1699228800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Inherited retinal dystrophies encompass a diverse group of disorders affecting the structure and function of the retina, leading to progressive visual impairment and, in severe cases, blindness. Electrophysiology testing has emerged as a valuable tool in assessing and diagnosing those conditions, offering insights into the function of different parts of the visual pathway from retina to visual cortex and aiding in disease classification. This review provides an overview of the application of electrophysiology testing in the non-macular inherited retinal dystrophies focusing on both common and rare variants, including retinitis pigmentosa, progressive cone and cone-rod dystrophy, bradyopsia, Bietti crystalline dystrophy, late-onset retinal degeneration, and fundus albipunctatus. The different applications and limitations of electrophysiology techniques, including multifocal electroretinogram (mfERG), full-field ERG (ffERG), electrooculogram (EOG), pattern electroretinogram (PERG), and visual evoked potential (VEP), in the diagnosis and management of these distinctive phenotypes are discussed. The potential for electrophysiology testing to allow for further understanding of these diseases and the possibility of using these tests for early detection, prognosis prediction, and therapeutic monitoring in the future is reviewed.<\/jats:p>","DOI":"10.3390\/jcm12216953","type":"journal-article","created":{"date-parts":[[2023,11,6]],"date-time":"2023-11-06T13:24:53Z","timestamp":1699277093000},"page":"6953","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Application of Electrophysiology in Non-Macular Inherited Retinal Dystrophies"],"prefix":"10.3390","volume":"12","author":[{"given":"Yulia","family":"Haraguchi","sequence":"first","affiliation":[{"name":"Department of Ophthalmology, University Hospitals, Case Western Reserve University, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1400-135X","authenticated-orcid":false,"given":"Tsun-Kang","family":"Chiang","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University Hospitals, Case Western Reserve University, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3115-8930","authenticated-orcid":false,"given":"Minzhong","family":"Yu","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University Hospitals, Case Western Reserve University, Cleveland, OH 44106, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,11,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e004015","DOI":"10.1136\/bmjopen-2013-004015","article-title":"A comparison of the causes of blindness certifications in England and Wales in working age adults (16\u201364 years), 1999\u20132000 with 2009\u20132010","volume":"4","author":"Liew","year":"2014","journal-title":"BMJ Open"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1384","DOI":"10.1016\/j.ophtha.2020.04.008","article-title":"Genetic Basis of Inherited Retinal Disease in a Molecularly Characterized Cohort of More Than 3000 Families from the United Kingdom","volume":"127","author":"Pontikos","year":"2020","journal-title":"Ophthalmology"},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Chay, J., Tang, R.W.C., Tan, T.E., Chan, C.M., Mathur, R., Lee, B.J.H., Chan, H.H., Sim, S., Farooqui, S., and Teo, K.Y.C. 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