Invited SubmissionUpdate on the Corneal Dystrophies—Genetic Testing and TherapyWeiss, Jayne S. MD*; Willoughby, Colin E. MD†; Abad–Morales, Víctor PhD‡,§; Turunen, Joni A. MD, PhD¶,‖; Lisch, Walter MD** Author Information *Department of Ophthalmology, Pathology and Pharmacology, Louisiana State University School of Medicine, New Orleans, LA; †Genomic Medicine, Biomedical Sciences Research Institute, Ulster University, Coleraine, Northern Ireland, United Kingdom; ‡Fundació de Recerca de l'Institut de Microcirurgia Ocular, Barcelona, Spain; §Department of Genetics, Institut de Microcirurgia Ocular (IMO), Barcelona, Spain; Dr. Abad–Morales is now with the SpliceBio, Barcelona, Spain, Barcelona, Spain; ¶Department of Ophthalmology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland; ‖Folkhälsan Research Center, Helsinki, Finland; and **Department of Ophthalmology, University Medical Center of the Johannes Gutenberg, University Mainz, Mainz, Germany. Correspondence: Jayne S. Weiss, MD, Department of Ophthalmology, Louisiana State University School of Medicine, 533 Bolivar St, Room 459, New Orleans, LA 70112 (e-mail: [email protected]). The authors have no funding or conflicts of interest to disclose. Cornea 41(11):p 1337-1344, November 2022. | DOI: 10.1097/ICO.0000000000002857 Buy Metrics Abstract One major purpose of the IC3D Corneal Dystrophy Nomenclature Revision was to include genetic information with a goal of facilitating investigation into the pathogenesis, treatment, and perhaps even prevention of the corneal dystrophies, an ambitious goal. Over a decade has passed since the first publication of the IC3D Corneal Dystrophy Nomenclature Revision. Gene therapy is available for an early-onset form of inherited retinal degeneration called Leber congenital amaurosis, but not yet for corneal degenerations. We review the current state of affairs regarding our original ambitious goal. We discuss genetic testing, gene therapy [RNA interference (RNAi) and genome editing], and ocular delivery of corneal gene therapy for the corneal dystrophies. Why have gene therapy techniques not yet been introduced for the corneal dystrophies? Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.