{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T17:49:55Z","timestamp":1792518595960,"version":"4.5.0"},"reference-count":82,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"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","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Hearing Research"],"published-print":{"date-parts":[[2026,4]]},"DOI":"10.1016\/j.heares.2026.109590","type":"journal-article","created":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T00:15:47Z","timestamp":1772151347000},"page":"109590","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":1,"special_numbering":"C","title":["The development of inner ear gene therapy for DFNB9: From bench to bedside"],"prefix":"10.1016","volume":"474","author":[{"given":"Meghan C.","family":"Drummond","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3865-3918","authenticated-orcid":false,"given":"Wade","family":"Chien","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.heares.2026.109590_bib0001","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1016\/j.omtm.2023.08.004","article-title":"Selection of viral capsids and promoters affects the efficacy of rescue of Tmprss3-deficient cochlea","volume":"30","author":"Aaron","year":"2023","journal-title":"Mol. Ther. Methods Clin. Dev."},{"issue":"10","key":"10.1016\/j.heares.2026.109590_bib0002","doi-asserted-by":"crossref","first-page":"4496","DOI":"10.1073\/pnas.1817537116","article-title":"Dual AAV-mediated gene therapy restores hearing in a DFNB9 mouse model","volume":"116","author":"Akil","year":"2019","journal-title":"Proc. Natl. Acad. Sci. USA"},{"issue":"10","key":"10.1016\/j.heares.2026.109590_bib0003","doi-asserted-by":"crossref","first-page":"4496","DOI":"10.1073\/pnas.1817537116","article-title":"Dual AAV-mediated gene therapy restores hearing in a DFNB9 mouse model","volume":"116","author":"Akil","year":"2019","journal-title":"Proc. Natl. Acad. Sci. USA"},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0004","doi-asserted-by":"crossref","DOI":"10.15252\/emmm.201809396","article-title":"A dual-AAV approach restores fast exocytosis and partially rescues auditory function in deaf otoferlin knock-out mice","volume":"11","author":"Al-Moyed","year":"2019","journal-title":"EMBo Mol. Med."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0005","doi-asserted-by":"crossref","DOI":"10.15252\/emmm.201809396","article-title":"A dual-AAV approach restores fast exocytosis and partially rescues auditory function in deaf otoferlin knock-out mice","volume":"11","author":"Al-Moyed","year":"2019","journal-title":"EMBO Mol. Med."},{"key":"10.1016\/j.heares.2026.109590_bib0006","doi-asserted-by":"crossref","DOI":"10.1016\/j.heares.2020.107947","article-title":"Adeno-associated virus gene replacement for recessive inner ear dysfunction: progress and challenges","volume":"394","author":"Askew","year":"2020","journal-title":"Hear. Res."},{"issue":"4","key":"10.1016\/j.heares.2026.109590_bib0007","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1016\/j.ajhg.2018.08.006","article-title":"Genomic landscape and mutational signatures of deafness-associated genes","volume":"103","author":"Azaiez","year":"2018","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.heares.2026.109590_bib0008","unstructured":"Bruno Arabian, N.E., 2025. Sensorion completes patient enrollment of the second cohort in Audiogene phase 1\/2 gene therapy clinical trial. https:\/\/www.businesswire.com\/news\/home\/20250728154446\/en\/Sensorion-Completes-Patient-Enrollment-of-the-Second-Cohort-in-Audiogene-Phase-12-Gene-Therapy-Clinical-Trial."},{"issue":"9","key":"10.1016\/j.heares.2026.109590_bib0009","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1001\/jamaneurol.2025.2053","article-title":"Gene therapy vs cochlear implantation in restoring hearing function and speech perception for individuals with congenital deafness","volume":"82","author":"Cheng","year":"2025","journal-title":"JAMa Neurol."},{"issue":"4","key":"10.1016\/j.heares.2026.109590_bib0010","doi-asserted-by":"crossref","DOI":"10.1016\/j.omtm.2025.101577","article-title":"Functional, sustained recovery of hearing in Otoferlin-deficient mice using DB-OTO, a hair-cell-specific AAV-based gene therapy","volume":"33","author":"Chung","year":"2025","journal-title":"Mol. Ther. Methods Clin. Dev."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0011","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1038\/s41551-024-01235-1","article-title":"A base editor for the long-term restoration of auditory function in mice with recessive profound deafness","volume":"9","author":"Cui","year":"2025","journal-title":"Nat. Biomed. Eng."},{"issue":"10","key":"10.1016\/j.heares.2026.109590_bib0012","doi-asserted-by":"crossref","first-page":"2337","DOI":"10.1016\/j.ymthe.2018.07.011","article-title":"Systemic AAV micro-dystrophin gene therapy for Duchenne muscular dystrophy","volume":"26","author":"Duan","year":"2018","journal-title":"Mol. Ther."},{"issue":"2","key":"10.1016\/j.heares.2026.109590_bib0013","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1089\/hgtb.2013.212","article-title":"Dual adeno-associated virus vectors result in efficient in vitro and in vivo expression of an oversized gene, MYO7A","volume":"25","author":"Dyka","year":"2014","journal-title":"Hum Gene Ther Methods"},{"issue":"10","key":"10.1016\/j.heares.2026.109590_bib0014","doi-asserted-by":"crossref","first-page":"1429","DOI":"10.1007\/s00439-023-02595-5","article-title":"The natural history, clinical outcomes, and genotype-phenotype relationship of otoferlin-related hearing loss: A systematic, quantitative literature review","volume":"142","author":"Ford","year":"2023","journal-title":"Hum. Genet."},{"key":"10.1016\/j.heares.2026.109590_bib0015","doi-asserted-by":"crossref","DOI":"10.1016\/j.heares.2020.107932","article-title":"Disease mechanisms and gene therapy for Usher syndrome","volume":"394","author":"Geleoc","year":"2020","journal-title":"Hear. Res."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0016","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1089\/hum.2010.122","article-title":"Efficient transgene reconstitution with hybrid dual AAV vectors carrying the minimized bridging sequences","volume":"22","author":"Ghosh","year":"2011","journal-title":"Hum. Gene Ther."},{"key":"10.1016\/j.heares.2026.109590_bib0017","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1016\/j.omtm.2021.09.009","article-title":"GJB2 gene therapy and conditional deletion reveal developmental stage-dependent effects on inner ear structure and function","volume":"23","author":"Guo","year":"2021","journal-title":"Mol. Ther. Methods Clin. Dev."},{"key":"10.1016\/j.heares.2026.109590_bib0018","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.omtm.2018.11.003","article-title":"Gene transfer with AAV9-PHP.B rescues hearing in a mouse model of Usher Syndrome 3A and transduces hair cells in a non-human primate","volume":"13","author":"Gyorgy","year":"2019","journal-title":"Mol. Ther. Methods Clin. Dev."},{"issue":"6","key":"10.1016\/j.heares.2026.109590_bib0019","doi-asserted-by":"crossref","first-page":"952","DOI":"10.3390\/audiolres13060083","article-title":"Gene therapy for inherited hearing loss: updates and remaining challenges","volume":"13","author":"Hahn","year":"2023","journal-title":"Audiol. Res."},{"issue":"5","key":"10.1016\/j.heares.2026.109590_bib0020","doi-asserted-by":"crossref","first-page":"961","DOI":"10.1007\/s10995-017-2287-y","article-title":"What you don't know can hurt you: the risk of language deprivation by impairing sign language development in deaf children","volume":"21","author":"Hall","year":"2017","journal-title":"Matern. Child Health J."},{"issue":"2","key":"10.1016\/j.heares.2026.109590_bib0021","doi-asserted-by":"crossref","first-page":"F81","DOI":"10.1136\/fn.71.2.F81","article-title":"Development of fetal hearing","volume":"71","author":"Hepper","year":"1994","journal-title":"Arch. Dis. Child Fetal. Neonatal. Ed."},{"issue":"Pt 4","key":"10.1016\/j.heares.2026.109590_bib0022","first-page":"966","article-title":"Analysis of the 'angulin' proteins LSR, ILDR1 and ILDR2\u2013tricellulin recruitment, epithelial barrier function and implication in deafness pathogenesis","volume":"126","author":"Higashi","year":"2013","journal-title":"J. Cell Sci."},{"key":"10.1016\/j.heares.2026.109590_bib0023","first-page":"0341","article-title":"Engineering of the AAV-compatible hair cell-specific small-size Myo15 promoter for gene therapy in the inner ear","volume":"7","author":"Hu","year":"2024","journal-title":"Res. (Wash. D. C)"},{"issue":"9","key":"10.1016\/j.heares.2026.109590_bib0024","doi-asserted-by":"crossref","first-page":"2783","DOI":"10.1016\/j.ymthe.2023.07.014","article-title":"Combined AAV-mediated gene replacement therapy improves auditory function in a mouse model of human DFNB42 deafness","volume":"31","author":"Isgrig","year":"2023","journal-title":"Mol. Ther."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0025","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1038\/s41467-018-08243-1","article-title":"AAV2.7m8 is a powerful viral vector for inner ear gene therapy","volume":"10","author":"Isgrig","year":"2019","journal-title":"Nat. Commun."},{"issue":"3","key":"10.1016\/j.heares.2026.109590_bib0026","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1016\/j.ymthe.2017.01.007","article-title":"Gene therapy restores balance and auditory functions in a mouse model of Usher syndrome","volume":"25","author":"Isgrig","year":"2017","journal-title":"Mol. Ther."},{"key":"10.1016\/j.heares.2026.109590_bib0027","doi-asserted-by":"crossref","DOI":"10.1016\/j.heares.2023.108735","article-title":"Immune responses in the mammalian inner ear and their implications for AAV-mediated inner ear gene therapy","volume":"432","author":"Ishibashi","year":"2023","journal-title":"Hear. Res."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0028","doi-asserted-by":"crossref","first-page":"216","DOI":"10.1016\/j.ymthe.2025.10.005","article-title":"Gene supplementation with precise transgene expression rescues hearing loss in a mouse model with an Mpzl2 East Asian founder variant","volume":"34","author":"Jang","year":"2026","journal-title":"Mol. Ther."},{"issue":"8","key":"10.1016\/j.heares.2026.109590_bib0029","doi-asserted-by":"crossref","first-page":"e240","DOI":"10.1097\/MAO.0000000000001448","article-title":"What does music sound like for a Cochlear implant user?","volume":"38","author":"Jiam","year":"2017","journal-title":"Otol. Neurotol."},{"key":"10.1016\/j.heares.2026.109590_bib0030","doi-asserted-by":"crossref","DOI":"10.1038\/srep44450","article-title":"Effects of Cdh23 single nucleotide substitutions on age-related hearing loss in C57BL\/6 and 129S1\/sv mice and comparisons with congenic strains","volume":"7","author":"Johnson","year":"2017","journal-title":"Sci. Rep."},{"issue":"3","key":"10.1016\/j.heares.2026.109590_bib0031","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1038\/nbt.3781","article-title":"A synthetic AAV vector enables safe and efficient gene transfer to the mammalian inner ear","volume":"35","author":"Landegger","year":"2017","journal-title":"Nat. Biotechnol."},{"key":"10.1016\/j.heares.2026.109590_bib0032","doi-asserted-by":"crossref","DOI":"10.1016\/j.heares.2020.107882","article-title":"Efficient viral transduction in mouse inner ear hair cells with utricle injection and AAV9-PHP.B","volume":"394","author":"Lee","year":"2020","journal-title":"Hear. Res."},{"issue":"1\u20132","key":"10.1016\/j.heares.2026.109590_bib0033","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/j.heares.2011.05.021","article-title":"Hereditary hearing loss: from human mutation to mechanism","volume":"281","author":"Lenz","year":"2011","journal-title":"Hear. Res."},{"issue":"4","key":"10.1016\/j.heares.2026.109590_bib0034","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1016\/j.tins.2018.01.008","article-title":"Why do hearing aids fail to restore normal auditory perception?","volume":"41","author":"Lesica","year":"2018","journal-title":"Trends. Neurosci."},{"issue":"1\u20132","key":"10.1016\/j.heares.2026.109590_bib0035","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/j.heares.2007.09.005","article-title":"A missense mutation in the conserved C2B domain of otoferlin causes deafness in a new mouse model of DFNB9","volume":"234","author":"Longo-Guess","year":"2007","journal-title":"Hear. Res."},{"issue":"10441","key":"10.1016\/j.heares.2026.109590_bib0036","doi-asserted-by":"crossref","first-page":"2317","DOI":"10.1016\/S0140-6736(23)02874-X","article-title":"AAV1-hOTOF gene therapy for autosomal recessive deafness 9: A single-arm trial","volume":"403","author":"Lv","year":"2024","journal-title":"Lancet"},{"issue":"3","key":"10.1016\/j.heares.2026.109590_bib0037","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1038\/nm1358","article-title":"Successful transduction of liver in hemophilia by AAV-Factor IX and limitations imposed by the host immune response","volume":"12","author":"Manno","year":"2006","journal-title":"Nat. Med."},{"issue":"3","key":"10.1016\/j.heares.2026.109590_bib0038","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1080\/14992027.2021.1883197","article-title":"Estimating the global costs of hearing loss","volume":"60","author":"McDaid","year":"2021","journal-title":"Int. J. Audiol."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0039","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1182\/blood-2013-01-306647","article-title":"Immune responses to AAV vectors: overcoming barriers to successful gene therapy","volume":"122","author":"Mingozzi","year":"2013","journal-title":"Blood"},{"issue":"6","key":"10.1016\/j.heares.2026.109590_bib0040","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1016\/j.otc.2021.08.008","article-title":"Genetics of childhood hearing loss","volume":"54","author":"Mitchell","year":"2021","journal-title":"Otolaryngol. Clin. North Am."},{"issue":"3","key":"10.1016\/j.heares.2026.109590_bib0041","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1093\/hmg\/ddu474","article-title":"ILDR1 null mice, a model of human deafness DFNB42, show structural aberrations of tricellular tight junctions and degeneration of auditory hair cells","volume":"24","author":"Morozko","year":"2015","journal-title":"Hum. Mol. Genet."},{"issue":"20","key":"10.1016\/j.heares.2026.109590_bib0042","doi-asserted-by":"crossref","first-page":"2151","DOI":"10.1056\/NEJMra050700","article-title":"Newborn hearing screening\u2013a silent revolution","volume":"354","author":"Morton","year":"2006","journal-title":"N. Engl. J. Med."},{"issue":"2","key":"10.1016\/j.heares.2026.109590_bib0043","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1002\/mrdd.10067","article-title":"The genetics of deafness","volume":"9","author":"Nance","year":"2003","journal-title":"Ment. Retard. Dev. Disabil. Res. Rev."},{"issue":"4","key":"10.1016\/j.heares.2026.109590_bib0044","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1007\/s40259-017-0234-5","article-title":"Adeno-associated virus (AAV) as a vector for gene therapy","volume":"31","author":"Naso","year":"2017","journal-title":"BioDrugs"},{"key":"10.1016\/j.heares.2026.109590_bib0045","doi-asserted-by":"crossref","DOI":"10.1016\/j.heares.2025.109278","article-title":"C57BL\/6-derived mice and the Cdh23(ahl) allele - background matters","volume":"462","author":"Newton","year":"2025","journal-title":"Hear. Res."},{"key":"10.1016\/j.heares.2026.109590_bib0046","doi-asserted-by":"crossref","DOI":"10.1016\/j.heares.2020.107958","article-title":"Genome and base editing for genetic hearing loss","volume":"394","author":"Niggemann","year":"2020","journal-title":"Hear. Res."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0047","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1038\/s41467-018-08264-w","article-title":"Improved TMC1 gene therapy restores hearing and balance in mice with genetic inner ear disorders","volume":"10","author":"Nist-Lund","year":"2019","journal-title":"Nat. Commun."},{"issue":"5","key":"10.1016\/j.heares.2026.109590_bib0048","doi-asserted-by":"crossref","first-page":"367","DOI":"10.2471\/BLT.13.128728","article-title":"The global burden of disabling hearing impairment: A call to action","volume":"92","author":"Olusanya","year":"2014","journal-title":"Bull. World Health Organ."},{"key":"10.1016\/j.heares.2026.109590_bib0049","unstructured":"Organization, W.H., 2021. Deafness and hearing loss. https:\/\/www.who.int\/news-room\/fact-sheets\/detail\/deafness-and-hearing-loss."},{"issue":"7","key":"10.1016\/j.heares.2026.109590_bib0050","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1038\/nn.2578","article-title":"Hearing requires otoferlin-dependent efficient replenishment of synaptic vesicles in hair cells","volume":"13","author":"Pangrsic","year":"2010","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.heares.2026.109590_bib0051","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/B978-0-444-62630-1.00001-9","article-title":"Auditory pathways: anatomy and physiology","volume":"129","author":"Pickles","year":"2015","journal-title":"Handb. Clin. Neurol."},{"issue":"11","key":"10.1016\/j.heares.2026.109590_bib0052","article-title":"AAV-mediated gene therapy restores hearing in patients with DFNB9 deafness","volume":"11","author":"Qi","year":"2024","journal-title":"Adv. Sci. (Weinh)"},{"issue":"9","key":"10.1016\/j.heares.2026.109590_bib0053","doi-asserted-by":"crossref","first-page":"2917","DOI":"10.1038\/s41591-025-03773-w","article-title":"AAV gene therapy for autosomal recessive deafness 9: A single-arm trial","volume":"31","author":"Qi","year":"2025","journal-title":"Nat. Med."},{"issue":"3","key":"10.1016\/j.heares.2026.109590_bib0054","article-title":"Preclinical efficacy and safety evaluation of AAV-OTOF in DFNB9 mouse model and nonhuman primate","volume":"11","author":"Qi","year":"2024","journal-title":"Adv. Sci. (Weinh)"},{"key":"10.1016\/j.heares.2026.109590_bib0055","article-title":"Overloaded adeno-associated virus as a novel gene therapeutic tool for otoferlin-related deafness","volume":"13","author":"Rankovic","year":"2020","journal-title":"Front. Mol. Neurosci."},{"issue":"2","key":"10.1016\/j.heares.2026.109590_bib0056","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1016\/j.cell.2006.08.040","article-title":"Otoferlin, defective in a human deafness form, is essential for exocytosis at the auditory ribbon synapse","volume":"127","author":"Roux","year":"2006","journal-title":"Cell"},{"issue":"5","key":"10.1016\/j.heares.2026.109590_bib0057","first-page":"365","article-title":"The cost of biotech innovation: exploring research and development costs of cell and gene therapies","volume":"37","author":"Sabatini","year":"2023","journal-title":"Pharmaceut. Med."},{"key":"10.1016\/j.heares.2026.109590_bib0058","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.heares.2017.12.010","article-title":"Communication pathways to and from the inner ear and their contributions to drug delivery","volume":"362","author":"Salt","year":"2018","journal-title":"Hear. Res."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0059","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1146\/annurev-virology-031413-085355","article-title":"AAV-mediated gene therapy for research and therapeutic purposes","volume":"1","author":"Samulski","year":"2014","journal-title":"Annu Rev. Virol."},{"key":"10.1016\/j.heares.2026.109590_bib0060","doi-asserted-by":"crossref","DOI":"10.1016\/j.ijporl.2021.110852","article-title":"Round window anatomy predicts ease of cochlear implantation in children","volume":"149","author":"Shim","year":"2021","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0061","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1037\/h0077648","article-title":"Ontogeny of the acoustic startle response in C57BL\/6J mouse pups","volume":"94","author":"Shnerson","year":"1980","journal-title":"J. Comp. Physiol. Psychol."},{"issue":"4","key":"10.1016\/j.heares.2026.109590_bib0062","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1007\/s00439-016-1648-8","article-title":"Comprehensive genetic testing in the clinical evaluation of 1119 patients with hearing loss","volume":"135","author":"Sloan-Heggen","year":"2016","journal-title":"Hum. Genet."},{"issue":"4","key":"10.1016\/j.heares.2026.109590_bib0063","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1007\/s00439-016-1648-8","article-title":"Comprehensive genetic testing in the clinical evaluation of 1119 patients with hearing loss","volume":"135","author":"Sloan-Heggen","year":"2016","journal-title":"Hum. Genet."},{"key":"10.1016\/j.heares.2026.109590_bib0064","doi-asserted-by":"crossref","DOI":"10.3389\/fncel.2021.677543","article-title":"Otoferlin is required for proper synapse maturation and for maintenance of inner and outer hair cells in mouse models for DFNB9","volume":"15","author":"Stalmann","year":"2021","journal-title":"Front. Cell Neurosci."},{"issue":"7","key":"10.1016\/j.heares.2026.109590_bib0065","doi-asserted-by":"crossref","first-page":"3006","DOI":"10.1016\/j.ymthe.2025.03.029","article-title":"Combined AAV-mediated specific Gjb2 expression restores hearing in DFNB1 mouse models","volume":"33","author":"Sun","year":"2025","journal-title":"Mol. Ther."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0066","doi-asserted-by":"crossref","first-page":"3733","DOI":"10.1038\/s41467-019-11687-8","article-title":"AAV-ie enables safe and efficient gene transfer to inner ear cells","volume":"10","author":"Tan","year":"2019","journal-title":"Nat. Commun."},{"issue":"2","key":"10.1016\/j.heares.2026.109590_bib0067","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1007\/s00439-022-02504-2","article-title":"Hearing of Otof-deficient mice restored by trans-splicing of N- and C-terminal otoferlin","volume":"142","author":"Tang","year":"2023","journal-title":"Hum. Genet."},{"issue":"2","key":"10.1016\/j.heares.2026.109590_bib0068","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1002\/emmm.201302948","article-title":"Effective delivery of large genes to the retina by dual AAV vectors","volume":"6","author":"Trapani","year":"2014","journal-title":"EMBo Mol. Med."},{"key":"10.1016\/j.heares.2026.109590_bib0069","article-title":"DB-OTO gene therapy for inherited deafness","author":"Valayannopoulos","year":"2025","journal-title":"N. Engl. J. Med."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0070","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1136\/jmg.40.1.45","article-title":"Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene","volume":"40","author":"Varga","year":"2003","journal-title":"J. Med. Genet."},{"issue":"4","key":"10.1016\/j.heares.2026.109590_bib0071","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1002\/mrdd.10082","article-title":"Infants and children with hearing loss\u2013part 2: overview","volume":"9","author":"Vohr","year":"2003","journal-title":"Ment. Retard. Dev. Disabil. Res. Rev."},{"issue":"2","key":"10.1016\/j.heares.2026.109590_bib0072","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1002\/mrdd.10070","article-title":"Overview: infants and children with hearing loss-part I","volume":"9","author":"Vohr","year":"2003","journal-title":"Ment. Retard. Dev. Disabil. Res. Rev."},{"issue":"7","key":"10.1016\/j.heares.2026.109590_bib0073","doi-asserted-by":"crossref","first-page":"1898","DOI":"10.1038\/s41591-024-03023-5","article-title":"Bilateral gene therapy in children with autosomal recessive deafness 9: single-arm trial results","volume":"30","author":"Wang","year":"2024","journal-title":"Nat. Med."},{"issue":"13\u201314","key":"10.1016\/j.heares.2026.109590_bib0074","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1089\/hum.2020.182","article-title":"Moving forward after two deaths in a gene therapy trial of myotubular myopathy","volume":"31","author":"Wilson","year":"2020","journal-title":"Hum. Gene Ther."},{"issue":"3","key":"10.1016\/j.heares.2026.109590_bib0075","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1016\/j.ymthe.2020.11.016","article-title":"Single and dual vector gene therapy with AAV9-PHP.B rescues hearing in Tmc1 mutant mice","volume":"29","author":"Wu","year":"2021","journal-title":"Mol. Ther."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0076","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1038\/mt.2009.255","article-title":"Effect of genome size on AAV vector packaging","volume":"18","author":"Wu","year":"2010","journal-title":"Mol. Ther."},{"issue":"12","key":"10.1016\/j.heares.2026.109590_bib0077","doi-asserted-by":"crossref","first-page":"3520","DOI":"10.1016\/j.ymthe.2023.10.019","article-title":"RNA base editing therapy cures hearing loss induced by OTOF gene mutation","volume":"31","author":"Xue","year":"2023","journal-title":"Mol. Ther."},{"issue":"3","key":"10.1016\/j.heares.2026.109590_bib0078","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1086\/303049","article-title":"OTOF encodes multiple long and short isoforms: genetic evidence that the long ones underlie recessive deafness DFNB9","volume":"67","author":"Yasunaga","year":"2000","journal-title":"Am. J. Hum. Genet."},{"issue":"4","key":"10.1016\/j.heares.2026.109590_bib0079","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1038\/7693","article-title":"A mutation in OTOF, encoding otoferlin, a FER-1-like protein, causes DFNB9, a nonsyndromic form of deafness","volume":"21","author":"Yasunaga","year":"1999","journal-title":"Nat. Genet."},{"issue":"1","key":"10.1016\/j.heares.2026.109590_bib0080","doi-asserted-by":"crossref","first-page":"2980","DOI":"10.1038\/s41598-018-21233-z","article-title":"Enhanced viral-mediated cochlear gene delivery in adult mice by combining canal fenestration with round window membrane inoculation","volume":"8","author":"Yoshimura","year":"2018","journal-title":"Sci. Rep."},{"issue":"7","key":"10.1016\/j.heares.2026.109590_bib0081","doi-asserted-by":"crossref","first-page":"1457","DOI":"10.1038\/s41562-025-02184-8","article-title":"Preliminary evidence for enhanced auditory cortex activation and mental development after gene therapy in children with autosomal recessive deafness 9","volume":"9","author":"Zhang","year":"2025","journal-title":"Nat. Hum. Behav."},{"key":"10.1016\/j.heares.2026.109590_bib0082","doi-asserted-by":"crossref","DOI":"10.1016\/j.omtm.2023.101154","article-title":"Preclinical evaluation of the efficacy and safety of AAV1-hOTOF in mice and nonhuman primates","volume":"31","author":"Zhang","year":"2023","journal-title":"Mol. Ther. Methods Clin. Dev."}],"container-title":["Hearing Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0378595526000663?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0378595526000663?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,4,7]],"date-time":"2026-04-07T11:10:43Z","timestamp":1775560243000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0378595526000663"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4]]},"references-count":82,"alternative-id":["S0378595526000663"],"URL":"https:\/\/doi.org\/10.1016\/j.heares.2026.109590","relation":{},"ISSN":["0378-5955"],"issn-type":[{"value":"0378-5955","type":"print"}],"subject":[],"published":{"date-parts":[[2026,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"The development of inner ear gene therapy for DFNB9: From bench to bedside","name":"articletitle","label":"Article Title"},{"value":"Hearing Research","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.heares.2026.109590","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 Published by Elsevier B.V.","name":"copyright","label":"Copyright"}],"article-number":"109590"}}