{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T02:00:18Z","timestamp":1760148018208,"version":"build-2065373602"},"reference-count":125,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2023,3,23]],"date-time":"2023-03-23T00:00:00Z","timestamp":1679529600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004587","name":"Sara Borrell\u2019s postdoctoral Fellowship","doi-asserted-by":"publisher","award":["CD20\/00153","DOC_01677","INTRAIBS-2021-07","RH-0073-2021"],"award-info":[{"award-number":["CD20\/00153","DOC_01677","INTRAIBS-2021-07","RH-0073-2021"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Economic Change, Industry, Knowledge and Universities Department","award":["CD20\/00153","DOC_01677","INTRAIBS-2021-07","RH-0073-2021"],"award-info":[{"award-number":["CD20\/00153","DOC_01677","INTRAIBS-2021-07","RH-0073-2021"]}]},{"name":"ibs.GRANADA","award":["CD20\/00153","DOC_01677","INTRAIBS-2021-07","RH-0073-2021"],"award-info":[{"award-number":["CD20\/00153","DOC_01677","INTRAIBS-2021-07","RH-0073-2021"]}]},{"name":"Andalusian Health Department","award":["CD20\/00153","DOC_01677","INTRAIBS-2021-07","RH-0073-2021"],"award-info":[{"award-number":["CD20\/00153","DOC_01677","INTRAIBS-2021-07","RH-0073-2021"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Genetic sensorineural hearing loss and Meniere disease have been associated with rare variations in the coding and non-coding region of the human genome. Most of these variants were classified as likely pathogenic or variants of unknown significance and require functional validation in cellular or animal models. Given the difficulties to obtain human samples and the raising concerns about animal experimentation, human-induced pluripotent stem cells emerged as cellular models to investigate the interaction of genetic and environmental factors in the pathogenesis of inner ear disorders. The generation of human sensory epithelia and neuron-like cells carrying the variants of interest may facilitate a better understanding of their role during differentiation. These cellular models will allow us to explore new strategies for restoring hearing and vestibular sensory epithelia as well as neurons. This review summarized the use of human-induced pluripotent stem cells in sensorineural hearing loss and Meniere disease and proposed some strategies for its application in clinical practice.<\/jats:p>","DOI":"10.3390\/cells12070988","type":"journal-article","created":{"date-parts":[[2023,3,24]],"date-time":"2023-03-24T04:31:05Z","timestamp":1679632265000},"page":"988","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Application of Human Stem Cells to Model Genetic Sensorineural Hearing Loss and Meniere Disease"],"prefix":"10.3390","volume":"12","author":[{"given":"Mar","family":"Lamolda","sequence":"first","affiliation":[{"name":"Otology and Neurotology Group CTS495, Department of Genomic Medicine, GENYO-Centre for Genomics and Oncological Research-Pfizer, University of Granada, Junta de Andaluc\u00eda, PTS, 18016 Granada, Spain"},{"name":"Division of Otolaryngology, Department of Surgery, Instituto de Investigaci\u00f3n Biosanitaria, ibs.GRANADA, Universidad de Granada, 18071 Granada, Spain"},{"name":"Sensorineural Pathology Programme, Centro de Investigaci\u00f3n Biom\u00e9dica en Red en Enfermedades Raras, CIBERER, 28029 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6104-0941","authenticated-orcid":false,"given":"Lidia","family":"Frejo","sequence":"additional","affiliation":[{"name":"Otology and Neurotology Group CTS495, Department of Genomic Medicine, GENYO-Centre for Genomics and Oncological Research-Pfizer, University of Granada, Junta de Andaluc\u00eda, PTS, 18016 Granada, Spain"},{"name":"Division of Otolaryngology, Department of Surgery, Instituto de Investigaci\u00f3n Biosanitaria, ibs.GRANADA, Universidad de Granada, 18071 Granada, Spain"},{"name":"Sensorineural Pathology Programme, Centro de Investigaci\u00f3n Biom\u00e9dica en Red en Enfermedades Raras, CIBERER, 28029 Madrid, Spain"},{"name":"Meniere\u2019s Disease Neuroscience Research Program, Faculty of Medicine & Health, School of Medical Sciences, The Kolling Institute, University of Sydney, Sydney, NSW 2006, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6068-8965","authenticated-orcid":false,"given":"Alvaro","family":"Gallego-Martinez","sequence":"additional","affiliation":[{"name":"Otology and Neurotology Group CTS495, Department of Genomic Medicine, GENYO-Centre for Genomics and Oncological Research-Pfizer, University of Granada, Junta de Andaluc\u00eda, PTS, 18016 Granada, Spain"},{"name":"Division of Otolaryngology, Department of Surgery, Instituto de Investigaci\u00f3n Biosanitaria, ibs.GRANADA, Universidad de Granada, 18071 Granada, Spain"},{"name":"Sensorineural Pathology Programme, Centro de Investigaci\u00f3n Biom\u00e9dica en Red en Enfermedades Raras, CIBERER, 28029 Madrid, Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8583-1430","authenticated-orcid":false,"given":"Jose A.","family":"Lopez-Escamez","sequence":"additional","affiliation":[{"name":"Otology and Neurotology Group CTS495, Department of Genomic Medicine, GENYO-Centre for Genomics and Oncological Research-Pfizer, University of Granada, Junta de Andaluc\u00eda, PTS, 18016 Granada, Spain"},{"name":"Division of Otolaryngology, Department of Surgery, Instituto de Investigaci\u00f3n Biosanitaria, ibs.GRANADA, Universidad de Granada, 18071 Granada, Spain"},{"name":"Sensorineural Pathology Programme, Centro de Investigaci\u00f3n Biom\u00e9dica en Red en Enfermedades Raras, CIBERER, 28029 Madrid, Spain"},{"name":"Meniere\u2019s Disease Neuroscience Research Program, Faculty of Medicine & Health, School of Medical Sciences, The Kolling Institute, University of Sydney, Sydney, NSW 2006, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,3,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1038\/nrm.2015.27","article-title":"Pluripotent Stem Cells in Disease Modelling and Drug Discovery","volume":"17","author":"Avior","year":"2016","journal-title":"Nat. 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