{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T12:25:15Z","timestamp":1792758315182,"version":"4.5.0"},"reference-count":257,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,4,4]],"date-time":"2022-04-04T00:00:00Z","timestamp":1649030400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["HearInNoise, ANR-17-CE16-0017"],"award-info":[{"award-number":["HearInNoise, ANR-17-CE16-0017"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["LabEx LIFESENSES, ANR-10-LABX-65"],"award-info":[{"award-number":["LabEx LIFESENSES, ANR-10-LABX-65"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001665","name":"Agence Nationale de la Recherche","doi-asserted-by":"publisher","award":["light4deaf, ANR-15-RHUS-0001"],"award-info":[{"award-number":["light4deaf, ANR-15-RHUS-0001"]}],"id":[{"id":"10.13039\/501100001665","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:p>The inner ear is responsible for both hearing and balance. These functions are dependent on the correct functioning of mechanosensitive hair cells, which convert sound- and motion-induced stimuli into electrical signals conveyed to the brain. During evolution of the inner ear, the major changes occurred in the hearing organ, whereas the structure of the vestibular organs remained constant in all vertebrates over the same period. Vestibular deficits are highly prevalent in humans, due to multiple intersecting causes: genetics, environmental factors, ototoxic drugs, infections and aging. Studies of deafness genes associated with balance deficits and their corresponding animal models have shed light on the development and function of these two sensory systems. Bilateral vestibular deficits often impair individual postural control, gaze stabilization, locomotion and spatial orientation. The resulting dizziness, vertigo, and\/or falls (frequent in elderly populations) greatly affect patient quality of life. In the absence of treatment, prosthetic devices, such as vestibular implants, providing information about the direction, amplitude and velocity of body movements, are being developed and have given promising results in animal models and humans. Novel methods and techniques have led to major progress in gene therapies targeting the inner ear (gene supplementation and gene editing), 3D inner ear organoids and reprograming protocols for generating hair cell-like cells. These rapid advances in multiscale approaches covering basic research, clinical diagnostics and therapies are fostering interdisciplinary research to develop personalized treatments for vestibular disorders.<\/jats:p>","DOI":"10.3389\/fneur.2022.816534","type":"journal-article","created":{"date-parts":[[2022,4,4]],"date-time":"2022-04-04T07:33:52Z","timestamp":1649057632000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":34,"title":["Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions"],"prefix":"10.3389","volume":"13","author":[{"given":"Audrey","family":"Maudoux","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandrine","family":"Vitry","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aziz","family":"El-Amraoui","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,4,4]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"143","DOI":"10.1002\/neu.10098","article-title":"Development and evolution of inner ear sensory epithelia and their innervation","volume":"53","author":"Fritzsch","year":"2002","journal-title":"J Neurobiol."},{"key":"B2","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1016\/S0959-4388(02)00287-8","article-title":"Revisiting cell fate specification in the inner ear","volume":"12","author":"Fekete","year":"2002","journal-title":"Curr Opin Neurobiol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"4119","DOI":"10.1242\/dev.01339","article-title":"From placode to polarization: new tunes in inner ear development","volume":"131","author":"Barald","year":"2004","journal-title":"Development."},{"key":"B4","doi-asserted-by":"publisher","first-page":"a008409","DOI":"10.1101\/cshperspect.a008409","article-title":"Molecular mechanisms of inner ear development","volume":"4","author":"Wu","year":"2012","journal-title":"Cold Spring Harb Perspect Biol."},{"key":"B5","doi-asserted-by":"publisher","first-page":"1410","DOI":"10.1002\/dvdy.260","article-title":"Uncovering the secreted signals and transcription factors regulating the development of mammalian middle ear ossicles","volume":"249","author":"Ankamreddy","year":"2020","journal-title":"Dev Dyn."},{"key":"B6","doi-asserted-by":"publisher","first-page":"395","DOI":"10.1016\/j.conb.2009.07.010","article-title":"Development of form and function in the mammalian cochlea","volume":"19","author":"Kelly","year":"2009","journal-title":"Curr Opin Neurobiol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"220","DOI":"10.1016\/j.smrv.2018.09.001","article-title":"The balance of sleep: role of the vestibular sensory system","volume":"42","author":"Besnard","year":"2018","journal-title":"Sleep Med Rev."},{"key":"B8","first-page":"1131","article-title":"Prevalence and presentation of dizziness in a general practice community sample of working age people","volume":"48","author":"Yardley","year":"1998","journal-title":"Br J Gen Pract."},{"key":"B9","doi-asserted-by":"publisher","first-page":"552","DOI":"10.1111\/j.1749-4486.2009.02025.x","article-title":"Dizziness and vertigo in an older population: the Blue Mountains prospective cross-sectional study","volume":"34","author":"Gopinath","year":"2009","journal-title":"Clin Otolaryngol."},{"key":"B10","doi-asserted-by":"publisher","first-page":"417","DOI":"10.1016\/j.jpsychores.2008.09.012","article-title":"Dizziness: anxiety, health care utilization and health behavior\u2013results from a representative German community survey","volume":"66","author":"Wiltink","year":"2009","journal-title":"J Psychosom Res."},{"key":"B11","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1186\/s13561-019-0258-2","article-title":"Economic burden of vertigo: a systematic review","volume":"9","author":"Kovacs","year":"2019","journal-title":"Health Econ Rev."},{"key":"B12","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1186\/s12873-020-00361-8","article-title":"Characteristics and resource needs in patients with vestibular symptoms: a comparison of patients with symptoms of unknown versus determined origin","volume":"20","author":"Muller","year":"2020","journal-title":"BMC Emerg Med."},{"key":"B13","doi-asserted-by":"publisher","first-page":"3076","DOI":"10.1007\/s00415-019-09525-4","article-title":"Frequency, aetiology, and impact of vestibular symptoms in the emergency department: a neglected red flag","volume":"266","author":"Goeldlin","year":"2019","journal-title":"J Neurol."},{"key":"B14","doi-asserted-by":"publisher","first-page":"395","DOI":"10.1016\/j.ijporl.2010.12.015","article-title":"Dizziness in 10 year old children: an epidemiological study","volume":"75","author":"Humphriss","year":"2011","journal-title":"Int J Pediatr Otorhinolaryngol."},{"key":"B15","doi-asserted-by":"publisher","first-page":"240","DOI":"10.1016\/j.jpeds.2015.12.002","article-title":"Epidemiology of dizziness and balance problems in children in the United States: a population-based study","volume":"171","author":"Li","year":"2016","journal-title":"J Pediatr."},{"key":"B16","doi-asserted-by":"publisher","first-page":"148","DOI":"10.1016\/j.ijporl.2016.06.020","article-title":"Balance disorders in childhood: main etiologies according to age. Usefulness of the video head impulse test","volume":"87","author":"Sommerfleck","year":"2016","journal-title":"Int J Pediatr Otorhinolaryngol."},{"key":"B17","doi-asserted-by":"publisher","first-page":"229","DOI":"10.1055\/s-0038-1666815","article-title":"Epidemiology of vestibular impairments in a pediatric population","volume":"39","author":"Wiener-Vacher","year":"2018","journal-title":"Semin Hear."},{"key":"B18","doi-asserted-by":"publisher","first-page":"38","DOI":"10.14336\/AD.2014.0128","article-title":"Dizziness and imbalance in the elderly: age-related decline in the vestibular system","volume":"6","author":"Iwasaki","year":"2015","journal-title":"Aging Dis."},{"key":"B19","doi-asserted-by":"publisher","first-page":"1195","DOI":"10.1016\/j.ijporl.2003.07.004","article-title":"A clinical test of dynamic visual acuity for children","volume":"67","author":"Rine","year":"2003","journal-title":"Int J Pediatr Otorhinolaryngol."},{"key":"B20","doi-asserted-by":"publisher","first-page":"2732","DOI":"10.1093\/brain\/awh617","article-title":"Vestibular loss causes hippocampal atrophy and impaired spatial memory in humans","volume":"128","author":"Brandt","year":"2005","journal-title":"Brain."},{"key":"B21","doi-asserted-by":"publisher","first-page":"246","DOI":"10.1016\/j.jpeds.2012.02.009","article-title":"Vestibular impairment after bacterial meningitis delays infant posturomotor development","volume":"161","author":"Wiener-Vacher","year":"2012","journal-title":"J Pediatr."},{"key":"B22","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1097\/WCO.0000000000000286","article-title":"The vestibular system: balancing more than just the body","volume":"29","author":"Lopez","year":"2016","journal-title":"Curr Opin Neurol."},{"key":"B23","doi-asserted-by":"publisher","first-page":"19","DOI":"10.3233\/RNN-2010-0509","article-title":"Plastic events in the vestibular nuclei during vestibular compensation: the brain orchestration of a \u201cdeafferentation\u201d code","volume":"28","author":"Lacour","year":"2010","journal-title":"Restor Neurol Neurosci."},{"key":"B24","doi-asserted-by":"publisher","first-page":"28","DOI":"10.1111\/j.1749-6632.2009.04484.x","article-title":"Development and regeneration of the inner ear","volume":"1170","author":"Kwan","year":"2009","journal-title":"Ann N Y Acad Sci."},{"key":"B25","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1242\/dev.067074","article-title":"Shaping sound in space: the regulation of inner ear patterning","volume":"139","author":"Groves","year":"2012","journal-title":"Development."},{"key":"B26","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1016\/j.ydbio.2017.08.034","article-title":"Gene, cell, and organ multiplication drives inner ear evolution","volume":"431","author":"Fritzsch","year":"2017","journal-title":"Dev Biol."},{"key":"B27","doi-asserted-by":"publisher","first-page":"660748","DOI":"10.3389\/fncel.2021.660748","article-title":"Transcription factor reprogramming in the inner ear: turning on cell fate switches to regenerate sensory hair cells","volume":"15","author":"Iyer","year":"2021","journal-title":"Front Cell Neurosci."},{"key":"B28","doi-asserted-by":"publisher","first-page":"4189","DOI":"10.3390\/ijms22084189","article-title":"Development in the mammalian auditory system depends on transcription factors","volume":"22","author":"Elliott","year":"2021","journal-title":"Int J Mol Sci."},{"key":"B29","doi-asserted-by":"publisher","first-page":"801","DOI":"10.1111\/joa.13459","article-title":"Development and evolution of the vestibular apparatuses of the inner ear","volume":"239","author":"Mackowetzky","year":"2021","journal-title":"J Anat."},{"key":"B30","doi-asserted-by":"publisher","first-page":"1837","DOI":"10.1126\/science.284.5421.1837","article-title":"Math1: an essential gene for the generation of inner ear hair cells","volume":"284","author":"Bermingham","year":"1999","journal-title":"Science."},{"key":"B31","doi-asserted-by":"publisher","first-page":"312","DOI":"10.1016\/j.celrep.2019.06.028","article-title":"Atoh1 directs regeneration and functional recovery of the mature mouse vestibular system","volume":"28","author":"Sayyid","year":"2019","journal-title":"Cell Rep."},{"key":"B32","doi-asserted-by":"publisher","first-page":"e3000326","DOI":"10.1371\/journal.pbio.3000326","article-title":"Uncoordinated maturation of developing and regenerating postnatal mammalian vestibular hair cells","volume":"17","author":"Wang","year":"2019","journal-title":"PLoS Biol."},{"key":"B33","doi-asserted-by":"publisher","first-page":"dev186015","DOI":"10.1242\/dev.186015","article-title":"GFI1 functions to repress neuronal gene expression in the developing inner ear hair cells","volume":"147","author":"Matern","year":"2020","journal-title":"Development."},{"key":"B34","doi-asserted-by":"publisher","first-page":"21397","DOI":"10.1038\/s41598-020-78167-8","article-title":"Combinatorial Atoh1 and Gfi1 induction enhances hair cell regeneration in the adult cochlea","volume":"10","author":"Lee","year":"2020","journal-title":"Sci Rep."},{"key":"B35","doi-asserted-by":"publisher","first-page":"441","DOI":"10.1097\/MOO.0b013e32833e0586","article-title":"Structure and function of cochlear afferent innervation","volume":"18","author":"Meyer","year":"2010","journal-title":"Curr Opin Otolaryngol Head Neck Surg."},{"key":"B36","doi-asserted-by":"publisher","first-page":"488","DOI":"10.1016\/j.pneurobio.2011.01.004","article-title":"Connecting the ear to the brain: molecular mechanisms of auditory circuit assembly","volume":"93","author":"Appler","year":"2011","journal-title":"Prog Neurobiol."},{"key":"B37","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1007\/s00441-014-2031-5","article-title":"Inner ear development: building a spiral ganglion and an organ of Corti out of unspecified ectoderm","volume":"361","author":"Fritzsch","year":"2015","journal-title":"Cell Tissue Res."},{"key":"B38","doi-asserted-by":"publisher","first-page":"157","DOI":"10.1016\/j.heares.2015.07.015","article-title":"Cochlear afferent innervation development","volume":"330","author":"Delacroix","year":"2015","journal-title":"Hear Res."},{"key":"B39","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1016\/0378-5955(86)90089-4","article-title":"Functional structure of the organ of Corti: a review","volume":"22","author":"Lim","year":"1986","journal-title":"Hear Res"},{"key":"B40","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1038\/nrn1828","article-title":"The sensory and motor roles of auditory hair cells","volume":"7","author":"Fettiplace","year":"2006","journal-title":"Nat Rev Neurosci."},{"key":"B41","doi-asserted-by":"publisher","first-page":"2407","DOI":"10.1073\/pnas.74.6.2407","article-title":"Sensitivity, polarity, and conductance change in the response of vertebrate hair cells to controlled mechanical stimuli","volume":"74","author":"Hudspeth","year":"1977","journal-title":"Proc Natl Acad Sci USA."},{"key":"B42","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1016\/j.crvi.2004.12.003","article-title":"How the ear's works work: mechanoelectrical transduction and amplification by hair cells","volume":"328","author":"Hudspeth","year":"2005","journal-title":"C R Biol"},{"key":"B43","doi-asserted-by":"publisher","first-page":"dev162263","DOI":"10.1242\/dev.162263","article-title":"Development of the cochlea","volume":"147","author":"Driver","year":"2020","journal-title":"Development."},{"key":"B44","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1007\/0-387-31706-6_8","article-title":"Mammalian vestibular hair cells","volume-title":"Vertebrate Hair Cells","author":"Eatock","year":"2006"},{"key":"B45","doi-asserted-by":"publisher","first-page":"419","DOI":"10.1002\/(SICI)1096-9861(19971222)389:3<419::AID-CNE5>3.0.CO;2-33.0.CO;2-3","article-title":"A regional ultrastructural analysis of the cellular and synaptic architecture in the chinchilla cristae ampullares","volume":"389","author":"Lysakowski","year":"1997","journal-title":"J Comp Neurol."},{"key":"B46","doi-asserted-by":"publisher","first-page":"172","DOI":"10.1016\/S0194-5998(98)70052-X","article-title":"Hair cells in mammalian utricles","volume":"119","author":"Eatock","year":"1998","journal-title":"Otolaryngol Head Neck Surg."},{"key":"B47","doi-asserted-by":"publisher","first-page":"341","DOI":"10.1093\/icb\/icy069","article-title":"Specializations for fast signaling in the amniote vestibular inner ear","volume":"58","author":"Eatock","year":"2018","journal-title":"Integr Comp Biol"},{"key":"B48","doi-asserted-by":"publisher","first-page":"109358","DOI":"10.1016\/j.celrep.2021.109358","article-title":"Spatiotemporal dynamics of inner ear sensory and non-sensory cells revealed by single-cell transcriptomics","volume":"36","author":"Jan","year":"2021","journal-title":"Cell Rep."},{"key":"B49","doi-asserted-by":"publisher","first-page":"2309","DOI":"10.3390\/jcm9072309","article-title":"Inner ear gene therapies take off: current promises and future challenges","volume":"9","author":"Delmaghani","year":"2020","journal-title":"J Clin Med."},{"key":"B50","doi-asserted-by":"publisher","first-page":"509","DOI":"10.1146\/annurev-neuro-061010-113705","article-title":"The auditory hair cell ribbon synapse: from assembly to function","volume":"35","author":"Safieddine","year":"2012","journal-title":"Annu Rev Neurosci."},{"key":"B51","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1146\/annurev.cb.04.110188.000431","article-title":"Hair cells: transduction, tuning, and transmission in the inner ear","volume":"4","author":"Roberts","year":"1988","journal-title":"Annu Rev Cell Biol."},{"key":"B52","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1016\/j.cell.2009.09.010","article-title":"Mechanotransduction by hair cells: models, molecules, and mechanisms","volume":"139","author":"Gillespie","year":"2009","journal-title":"Cell."},{"key":"B53","doi-asserted-by":"publisher","first-page":"13336","DOI":"10.1073\/pnas.97.24.13336","article-title":"High-resolution structure of hair-cell tip links","volume":"97","author":"Kachar","year":"2000","journal-title":"Proc Natl Acad Sci USA."},{"key":"B54","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1016\/0896-6273(88)90139-0","article-title":"Compliance of the hair bundle associated with gating of mechanoelectrical transduction channels in the bullfrog's saccular hair cell","volume":"1","author":"Howard","year":"1988","journal-title":"Neuron."},{"key":"B55","doi-asserted-by":"publisher","first-page":"254","DOI":"10.1016\/0166-2236(92)90066-H","article-title":"Mechanoelectrical transduction by hair cells","volume":"15","author":"Pickles","year":"1992","journal-title":"Trends Neurosci."},{"key":"B56","doi-asserted-by":"publisher","first-page":"1721","DOI":"10.1242\/jcs.106120","article-title":"The composition and role of cross links in mechanoelectrical transduction in vertebrate sensory hair cells","volume":"126","author":"Hackney","year":"2013","journal-title":"J Cell Sci."},{"key":"B57","doi-asserted-by":"publisher","first-page":"553","DOI":"10.1038\/nn.2295","article-title":"Localization of inner hair cell mechanotransducer channels using high-speed calcium imaging","volume":"12","author":"Beurg","year":"2009","journal-title":"Nat. Neurosci."},{"key":"B58","doi-asserted-by":"publisher","first-page":"347","DOI":"10.1007\/s00441-006-0276-3","article-title":"Hair cell ribbon synapses","volume":"326","author":"Moser","year":"2006","journal-title":"Cell Tissue Res."},{"key":"B59","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1016\/j.semcdb.2016.11.001","article-title":"Development and regeneration of vestibular hair cells in mammals","volume":"65","author":"Burns","year":"2017","journal-title":"Semin Cell Dev Biol."},{"key":"B60","doi-asserted-by":"publisher","first-page":"a033241","DOI":"10.1101\/cshperspect.a033241","article-title":"A functional perspective on the evolution of the cochlea","volume":"9","author":"Koppl","year":"2019","journal-title":"Cold Spring Harb Perspect Med."},{"key":"B61","doi-asserted-by":"publisher","first-page":"88","DOI":"10.1016\/j.tins.2019.12.003","article-title":"Diverse mechanisms of sound frequency discrimination in the vertebrate cochlea","volume":"43","author":"Fettiplace","year":"2020","journal-title":"Trends Neurosci."},{"key":"B62","doi-asserted-by":"publisher","first-page":"7487","DOI":"10.1523\/JNEUROSCI.18-18-07487.1998","article-title":"Postnatal development of type I and type II hair cells in the mouse utricle: acquisition of voltage-gated conductances and differentiated morphology","volume":"18","author":"Rusch","year":"1998","journal-title":"J Neurosci."},{"key":"B63","doi-asserted-by":"publisher","first-page":"bio038083","DOI":"10.1242\/bio.038083","article-title":"Characterization of spatial and temporal development of Type I and Type II hair cells in the mouse utricle using new cell-type-specific markers","volume":"7","author":"McInturff","year":"2018","journal-title":"Biol Open."},{"key":"B64","doi-asserted-by":"publisher","first-page":"251","DOI":"10.1152\/jn.00746.2003","article-title":"Comparative morphology of rodent vestibular periphery. I. Saccular utricular maculae","volume":"93","author":"Desai","year":"2005","journal-title":"J Neurophysiol."},{"key":"B65","doi-asserted-by":"publisher","first-page":"771","DOI":"10.1523\/JNEUROSCI.4690-06.2007","article-title":"Neural variability, detection thresholds, and information transmission in the vestibular system","volume":"27","author":"Sadeghi","year":"2007","journal-title":"J Neurosci."},{"key":"B66","doi-asserted-by":"publisher","first-page":"501","DOI":"10.1146\/annurev-neuro-061010-113710","article-title":"Vestibular hair cells and afferents: two channels for head motion signals","volume":"34","author":"Eatock","year":"2011","journal-title":"Annu Rev Neurosci."},{"key":"B67","doi-asserted-by":"publisher","first-page":"695179","DOI":"10.3389\/fnins.2021.695179","article-title":"Similarities and differences between vestibular and cochlear systems - a review of clinical and physiological evidence","volume":"15","author":"Curthoys","year":"2021","journal-title":"Front Neurosci."},{"key":"B68","doi-asserted-by":"publisher","first-page":"807","DOI":"10.1083\/jcb.96.3.807","article-title":"Actin filaments, stereocilia, and hair cells of the bird cochlea. I. Length, number, width, and distribution of stereocilia of each hair cell are related to the position of the hair cell on the cochlea","volume":"96","author":"Tilney","year":"1983","journal-title":"J Cell Biol."},{"key":"B69","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1016\/0378-5955(88)90079-2","article-title":"New observations on the stereocilia of hair cells of the chick cochlea","volume":"37","author":"Tilney","year":"1988","journal-title":"Hear Res."},{"key":"B70","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1242\/dev.116.1.213","article-title":"Actin filaments, stereocilia and hair cells of the bird cochlea. VI. How the number and arrangement of stereocilia are determined","volume":"116","author":"Tilney","year":"1992","journal-title":"Development."},{"key":"B71","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1146\/annurev.cb.08.110192.001353","article-title":"Actin filaments, stereocilia, and hair cells: how cells count and measure","volume":"8","author":"Tilney","year":"1992","journal-title":"Annu Rev Cell Biol."},{"key":"B72","doi-asserted-by":"publisher","first-page":"718","DOI":"10.1152\/jn.00831.2007","article-title":"Architecture of the mouse utricle: macular organization and hair bundle heights","volume":"99","author":"Li","year":"2008","journal-title":"J Neurophysiol."},{"key":"B73","doi-asserted-by":"publisher","first-page":"132","DOI":"10.1152\/jn.00068.2019","article-title":"Multiscale modeling of mechanotransduction in the utricle","volume":"122","author":"Nam","year":"2019","journal-title":"J Neurophysiol."},{"key":"B74","doi-asserted-by":"publisher","first-page":"164","DOI":"10.1111\/j.1749-6632.1996.tb15700.x","article-title":"Synaptic organization of the crista ampullaris in vertebrates","volume":"781","author":"Lysakowski","year":"1996","journal-title":"Ann NY Acad Sci"},{"key":"B75","doi-asserted-by":"publisher","DOI":"10.1101\/2021.11.18.469197","article-title":"A biophysical model of nonquantal transmission at the vestibular hair cell-calyx synapse: KLV currents modulate fast electrical and slow K+ potentials in the synaptic cleft","author":"Govindaraju","year":"2021","journal-title":"bioRxiv [Preprint]."},{"key":"B76","doi-asserted-by":"publisher","first-page":"252","DOI":"10.3389\/fncel.2018.00252","article-title":"Evolutionary and developmental biology provide insights into the regeneration of organ of corti hair cells","volume":"12","author":"Elliott","year":"2018","journal-title":"Front Cell Neurosci."},{"key":"B77","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1016\/j.heares.2010.05.004","article-title":"Sensory regeneration in the vertebrate inner ear: differences at the levels of cells and species","volume":"273","author":"Warchol","year":"2011","journal-title":"Hear Res."},{"key":"B78","doi-asserted-by":"publisher","first-page":"1772","DOI":"10.1126\/science.3381100","article-title":"Regeneration of sensory hair cells after acoustic trauma","volume":"240","author":"Corwin","year":"1988","journal-title":"Science."},{"key":"B79","doi-asserted-by":"publisher","first-page":"633","DOI":"10.1387\/ijdb.072408js","article-title":"Hair cell regeneration in the avian auditory epithelium","volume":"51","author":"Stone","year":"2007","journal-title":"Int J Dev Biol."},{"key":"B80","doi-asserted-by":"publisher","first-page":"15093","DOI":"10.1523\/JNEUROSCI.1709-12.2012","article-title":"Hair cell replacement in adult mouse utricles after targeted ablation of hair cells with diphtheria toxin","volume":"32","author":"Golub","year":"2012","journal-title":"J Neurosci."},{"key":"B81","doi-asserted-by":"publisher","first-page":"396","DOI":"10.1002\/cne.21849","article-title":"Reinforcement of cell junctions correlates with the absence of hair cell regeneration in mammals and its occurrence in birds","volume":"511","author":"Burns","year":"2008","journal-title":"J Comp Neurol."},{"key":"B82","doi-asserted-by":"publisher","first-page":"20","DOI":"10.3389\/fnmol.2018.00020","article-title":"Junctional E-cadherin\/p120-catenin is correlated with the absence of supporting cells to hair cells conversion in postnatal mice cochleae","volume":"11","author":"Luo","year":"2018","journal-title":"Front Mol Neurosci."},{"key":"B83","doi-asserted-by":"publisher","first-page":"11855","DOI":"10.1523\/JNEUROSCI.2525-11.2011","article-title":"The postnatal accumulation of junctional E-cadherin is inversely correlated with the capacity for supporting cells to convert directly into sensory hair cells in mammalian balance organs","volume":"31","author":"Collado","year":"2011","journal-title":"J Neurosci."},{"key":"B84","doi-asserted-by":"publisher","first-page":"1430","DOI":"10.1002\/cne.23250","article-title":"Specializations of intercellular junctions are associated with the presence and absence of hair cell regeneration in ears from six vertebrate classes","volume":"521","author":"Burns","year":"2013","journal-title":"J Comp Neurol."},{"key":"B85","doi-asserted-by":"publisher","first-page":"52","DOI":"10.1016\/j.heares.2013.01.005","article-title":"A historical to present-day account of efforts to answer the question: \u201cwhat puts the brakes on mammalian hair cell regeneration?","volume":"297","author":"Burns","year":"2013","journal-title":"Hear Res."},{"key":"B86","doi-asserted-by":"publisher","first-page":"637779","DOI":"10.3389\/fcell.2021.637779","article-title":"Single-cell sequencing applications in the inner ear","volume":"9","author":"Wu","year":"2021","journal-title":"Front Cell Dev Biol."},{"key":"B87","doi-asserted-by":"publisher","first-page":"156","DOI":"10.3389\/fgene.2018.00156","article-title":"Intracellular regulome variability along the organ of corti: evidence, approaches, challenges, and perspective","volume":"9","author":"Booth","year":"2018","journal-title":"Front Genet."},{"key":"B88","doi-asserted-by":"publisher","first-page":"660812","DOI":"10.3389\/fncel.2021.660812","article-title":"Navigating hereditary hearing loss: pathology of the inner ear","volume":"15","author":"Nicolson","year":"2021","journal-title":"Front Cell Neurosci."},{"key":"B89","doi-asserted-by":"publisher","first-page":"633207","DOI":"10.3389\/fneur.2021.633207","article-title":"Genetics and the individualized therapy of vestibular disorders","volume":"12","author":"Mei","year":"2021","journal-title":"Front Neurol."},{"key":"B90","doi-asserted-by":"publisher","first-page":"70","DOI":"10.1016\/S0194-5998(96)70139-0","article-title":"Vestibular deficits in deaf children","volume":"115","author":"Selz","year":"1996","journal-title":"Otolaryngol Head Neck Surg."},{"key":"B91","doi-asserted-by":"publisher","first-page":"1814","DOI":"10.1097\/MLG.0b013e31817fadfa","article-title":"Evidence of vestibular and balance dysfunction in children with profound sensorineural hearing loss using cochlear implants","volume":"118","author":"Cushing","year":"2008","journal-title":"Laryngoscope."},{"key":"B92","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1016\/j.ijporl.2008.10.024","article-title":"Vestibular impairments pre- and post-cochlear implant in children","volume":"73","author":"Jacot","year":"2009","journal-title":"Int J Pediatr Otorhinolaryngol."},{"key":"B93","doi-asserted-by":"publisher","first-page":"259","DOI":"10.1007\/s40136-017-0172-2","article-title":"Vestibular assessment in cochlear implanted children: how to do? When to do? A review of literature","volume":"5","author":"Coudert","year":"2017","journal-title":"Curr Otorhinolaryngol Rep."},{"key":"B94","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1055\/s-0038-1666817","article-title":"Quantitative vestibular function testing in the pediatric population","volume":"39","author":"Janky","year":"2018","journal-title":"Semin Hear."},{"key":"B95","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1159\/000368399","article-title":"The mouse as a model for age-related hearing loss - a mini-review","volume":"61","author":"Bowl","year":"2015","journal-title":"Gerontology."},{"key":"B96","doi-asserted-by":"publisher","first-page":"493","DOI":"10.1007\/s10162-016-0589-1","article-title":"Application of mouse models to research in hearing and balance","volume":"17","author":"Ohlemiller","year":"2016","journal-title":"J Assoc Res Otolaryngol."},{"key":"B97","doi-asserted-by":"publisher","first-page":"S10","DOI":"10.1007\/s00415-015-7909-y","article-title":"Vestibular animal models: contributions to understanding physiology and disease","volume":"263","author":"Straka","year":"2016","journal-title":"J Neurol."},{"key":"B98","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1007\/s10162-018-0676-6","article-title":"Characterization of adult vestibular organs in 11 CreER mouse lines","volume":"19","author":"Stone","year":"2018","journal-title":"J Assoc Res Otolaryngol."},{"key":"B99","first-page":"26","volume-title":"Assessing Hearing, Vision and Balance in Mice. What's Wrong With My Mouse? New Interplays Between Mouse Genes and Behavior.","author":"Steel","year":"1996"},{"key":"B100","doi-asserted-by":"publisher","first-page":"299","DOI":"10.1016\/S0168-0102(00)00228-5","article-title":"Dynamic properties, interactions and adaptive modifications of vestibulo-ocular reflex and optokinetic response in mice","volume":"39","author":"Iwashita","year":"2001","journal-title":"Neurosci Res."},{"key":"B101","doi-asserted-by":"publisher","first-page":"489","DOI":"10.1007\/s00221-010-2521-y","article-title":"Characterization of the 3D angular vestibulo-ocular reflex in C57BL6 mice","volume":"210","author":"Migliaccio","year":"2011","journal-title":"Exp Brain Res."},{"key":"B102","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1016\/S0165-0270(02)00125-5","article-title":"Stimulus and recording variables and their effects on mammalian vestibular evoked potentials","volume":"118","author":"Jones","year":"2002","journal-title":"J Neurosci Methods."},{"key":"B103","doi-asserted-by":"publisher","first-page":"289","DOI":"10.3766\/jaaa.25.3.8","article-title":"Genetics of peripheral vestibular dysfunction: lessons from mutant mouse strains","volume":"25","author":"Jones","year":"2014","journal-title":"J Am Acad Audiol."},{"key":"B104","doi-asserted-by":"publisher","first-page":"34","DOI":"10.3389\/fnsys.2017.00034","article-title":"Electrophysiological measurements of peripheral vestibular function-a review of electrovestibulography","volume":"11","author":"Brown","year":"2017","journal-title":"Front Syst Neurosci."},{"key":"B105","doi-asserted-by":"publisher","first-page":"708395","DOI":"10.3389\/fneur.2021.708395","article-title":"Understanding the pathophysiology of congenital vestibular disorders: current challenges and future directions","volume":"12","author":"Peusner","year":"2021","journal-title":"Front Neurol."},{"key":"B106","doi-asserted-by":"publisher","first-page":"782","DOI":"10.1086\/511981","article-title":"Etiology of severe sensorineural hearing loss in children: independent impact of congenital cytomegalovirus infection and GJB2 mutations","volume":"195","author":"Ogawa","year":"2007","journal-title":"J Infect Dis."},{"key":"B107","doi-asserted-by":"publisher","first-page":"e887","DOI":"10.1542\/peds.2015-0908","article-title":"Vestibular disorders in children with congenital cytomegalovirus infection","volume":"136","author":"Bernard","year":"2015","journal-title":"Pediatrics."},{"key":"B108","doi-asserted-by":"publisher","first-page":"763","DOI":"10.1007\/s00401-011-0895-y","article-title":"Inner ear lesions in congenital cytomegalovirus infection of human fetuses","volume":"122","author":"Teissier","year":"2011","journal-title":"Acta Neuropathol."},{"key":"B109","doi-asserted-by":"publisher","first-page":"413","DOI":"10.1016\/j.anorl.2016.03.004","article-title":"Audiovestibular consequences of congenital cytomegalovirus infection","volume":"133","author":"Teissier","year":"2016","journal-title":"Eur Ann Otorhinolaryngol Head Neck Dis."},{"key":"B110","doi-asserted-by":"publisher","first-page":"917","DOI":"10.1086\/656409","article-title":"Factors associated with the occurrence of hearing loss after pneumococcal meningitis","volume":"51","author":"Worsoe","year":"2010","journal-title":"Clin Infect Dis."},{"key":"B111","doi-asserted-by":"publisher","first-page":"876","DOI":"10.1288\/00005537-199108000-00012","article-title":"Otologic sequelae after pneumococcal meningitis: a survey of 164 consecutive cases with a follow-up of 94 survivors","volume":"101","author":"Rasmussen","year":"1991","journal-title":"Laryngoscope."},{"key":"B112","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1016\/B978-0-444-63437-5.00017-0","article-title":"Bilateral vestibulopathy","volume":"137","author":"Strupp","year":"2016","journal-title":"Handb Clin Neurol."},{"key":"B113","doi-asserted-by":"publisher","first-page":"956","DOI":"10.1097\/01.mao.0000231590.57348.4b","article-title":"Vestibular evoked myogenic potentials in acute acoustic trauma","volume":"27","author":"Wang","year":"2006","journal-title":"Otol Neurotol."},{"key":"B114","doi-asserted-by":"publisher","first-page":"118","DOI":"10.1016\/j.heares.2016.03.002","article-title":"Effects of high intensity noise on the vestibular system in rats","volume":"335","author":"Stewart","year":"2016","journal-title":"Hear Res."},{"key":"B115","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1016\/j.otohns.2007.05.005","article-title":"Vestibular-evoked myogenic potentials in chronic noise-induced hearing loss","volume":"137","author":"Wang","year":"2007","journal-title":"Otolaryngol Head Neck Surg."},{"key":"B116","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1016\/S0196-0709(99)90076-5","article-title":"Aging effects on auditory and vestibular responses: a longitudinal study","volume":"20","author":"Enrietto","year":"1999","journal-title":"Am J Otolaryngol."},{"key":"B117","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1177\/00034894931020S101","article-title":"Cochlear pathology in presbycusis","volume":"102","author":"Schuknecht","year":"1993","journal-title":"Ann Otol Rhinol Laryngol."},{"key":"B118","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1177\/0394632015570819","article-title":"Genetics of presbycusis and presbystasis","volume":"28","author":"Ciorba","year":"2015","journal-title":"Int J Immunopathol Pharmacol."},{"key":"B119","doi-asserted-by":"publisher","first-page":"5072582","DOI":"10.1155\/2018\/5072582","article-title":"Update on vertigo in autoimmune disorders, from diagnosis to treatment","volume":"2018","author":"Girasoli","year":"2018","journal-title":"J Immunol Res."},{"key":"B120","doi-asserted-by":"publisher","first-page":"675","DOI":"10.1007\/s12026-018-9023-6","article-title":"Autoimmune vertigo: an update on vestibular disorders associated with autoimmune mechanisms","volume":"66","author":"Russo","year":"2018","journal-title":"Immunol Res."},{"key":"B121","doi-asserted-by":"publisher","first-page":"5798103","DOI":"10.1155\/2018\/5798103","article-title":"Audiovestibular symptoms in systemic autoimmune diseases","volume":"2018","author":"Ralli","year":"2018","journal-title":"J Immunol Res."},{"key":"B122","first-page":"601","article-title":"Pharmacological drugs inducing ototoxicity, vestibular symptoms and tinnitus: a reasoned and updated guide","volume":"15","author":"Cianfrone","year":"2011","journal-title":"Eur Rev Med Pharmacol Sci."},{"key":"B123","doi-asserted-by":"publisher","first-page":"4909237","DOI":"10.1155\/2021\/4909237","article-title":"Hair cell protection from ototoxic drugs","volume":"2021","author":"Wu","year":"2021","journal-title":"Neural Plast."},{"key":"B124","doi-asserted-by":"publisher","first-page":"571","DOI":"10.1177\/019459989411100506","article-title":"Gentamicin vestibulotoxicity","volume":"111","author":"Halmagyi","year":"1994","journal-title":"Otolaryngol Head Neck Surg."},{"key":"B125","doi-asserted-by":"publisher","first-page":"517","DOI":"10.3349\/ymj.2003.44.3.517","article-title":"Comparative vestibulotoxicity of different aminoglycosides in the Guinea pigs","volume":"44","author":"Selimoglu","year":"2003","journal-title":"Yonsei Med J."},{"key":"B126","doi-asserted-by":"publisher","first-page":"2613","DOI":"10.1007\/s00204-021-03067-3","article-title":"Progress in protecting vestibular hair cells","volume":"95","author":"Jiang","year":"2021","journal-title":"Arch Toxicol."},{"key":"B127","doi-asserted-by":"publisher","first-page":"327","DOI":"10.1016\/0892-0362(93)90034-L","article-title":"Effects of toluene, styrene, trichloroethylene, and trichloroethane on the vestibulo-and opto-oculo motor system in rats","volume":"15","author":"Niklasson","year":"1993","journal-title":"Neurotoxicol Teratol."},{"key":"B128","unstructured":""},{"key":"B129","doi-asserted-by":"publisher","first-page":"21011","DOI":"10.1038\/s41598-020-78049-z","article-title":"Vestibular Infant Screening (VIS)-Flanders: results after 1.5 years of vestibular screening in hearing-impaired children","volume":"10","author":"Martens","year":"2020","journal-title":"Sci Rep."},{"key":"B130","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1016\/j.anorl.2019.05.018","article-title":"Guidelines (short version) of the French Society of Otorhinolaryngology (SFORL) on pediatric cochlear implant indications","volume":"136","author":"Simon","year":"2019","journal-title":"Eur Ann Otorhinolaryngol Head Neck Dis."},{"key":"B131","doi-asserted-by":"publisher","first-page":"S45","DOI":"10.1007\/s00415-015-7988-9","article-title":"Genetics of vestibular disorders: pathophysiological insights","volume":"263","author":"Frejo","year":"2016","journal-title":"J Neurol."},{"key":"B132","doi-asserted-by":"publisher","first-page":"12444","DOI":"10.1038\/ncomms12444","article-title":"Novel gene function revealed by mouse mutagenesis screens for models of age-related disease","volume":"7","author":"Potter","year":"2016","journal-title":"Nat Commun."},{"key":"B133","unstructured":""},{"key":"B134","doi-asserted-by":"publisher","first-page":"345","DOI":"10.1038\/nm.3106","article-title":"Rescue of hearing and vestibular function by antisense oligonucleotides in a mouse model of human deafness","volume":"19","author":"Lentz","year":"2013","journal-title":"Nat Med."},{"key":"B135","doi-asserted-by":"publisher","first-page":"3482","DOI":"10.1093\/hmg\/ddx234","article-title":"Rescue of peripheral vestibular function in Usher syndrome mice using a splice-switching antisense oligonucleotide","volume":"26","author":"Vijayakumar","year":"2017","journal-title":"Hum Mol Genet."},{"key":"B136","doi-asserted-by":"publisher","first-page":"76","DOI":"10.1016\/j.bbr.2017.10.012","article-title":"Antisense oligonucleotide therapy rescues disruptions in organization of exploratory movements associated with Usher syndrome type 1C in mice","volume":"338","author":"Donaldson","year":"2018","journal-title":"Behav Brain Res."},{"key":"B137","doi-asserted-by":"publisher","first-page":"2662","DOI":"10.1016\/j.ymthe.2020.08.002","article-title":"Direct delivery of antisense oligonucleotides to the middle and inner ear improves hearing and balance in Usher Mice","volume":"28","author":"Lentz","year":"2020","journal-title":"Mol Ther."},{"key":"B138","doi-asserted-by":"publisher","first-page":"5065","DOI":"10.1093\/nar\/gkaa194","article-title":"Fetal antisense oligonucleotide therapy for congenital deafness and vestibular dysfunction","volume":"48","author":"Wang","year":"2020","journal-title":"Nucleic Acids Res."},{"key":"B139","doi-asserted-by":"publisher","first-page":"264","DOI":"10.1038\/nbt.3801","article-title":"Gene therapy restores auditory and vestibular function in a mouse model of Usher syndrome type 1c","volume":"35","author":"Pan","year":"2017","journal-title":"Nat Biotechnol."},{"key":"B140","doi-asserted-by":"publisher","first-page":"697","DOI":"10.1038\/s41467-020-20808-7","article-title":"Gene therapy via canalostomy approach preserves auditory and vestibular functions in a mouse model of Jervell and Lange-Nielsen syndrome type 2","volume":"12","author":"Wu","year":"2021","journal-title":"Nat Commun."},{"key":"B141","doi-asserted-by":"publisher","first-page":"379","DOI":"10.1016\/j.ymthe.2016.12.010","article-title":"Rescue of hearing by gene delivery to inner-ear hair cells using exosome-associated AAV","volume":"25","author":"Gyorgy","year":"2017","journal-title":"Mol Ther."},{"key":"B142","doi-asserted-by":"publisher","first-page":"7184","DOI":"10.7150\/thno.38032","article-title":"Gene therapy for hereditary hearing loss by SLC26A4 mutations in mice reveals distinct functional roles of pendrin in normal hearing","volume":"9","author":"Kim","year":"2019","journal-title":"Theranostics."},{"key":"B143","doi-asserted-by":"publisher","first-page":"17979","DOI":"10.1038\/s41598-019-54262-3","article-title":"Prenatal electroporation-mediated gene transfer restores Slc26a4 knock-out mouse hearing and vestibular function","volume":"9","author":"Takeda","year":"2019","journal-title":"Sci Rep."},{"key":"B144","doi-asserted-by":"publisher","first-page":"9695","DOI":"10.1073\/pnas.1708894114","article-title":"Local gene therapy durably restores vestibular function in a mouse model of Usher syndrome type 1G","volume":"114","author":"Emptoz","year":"2017","journal-title":"Proc Natl Acad Sci USA."},{"key":"B145","doi-asserted-by":"publisher","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."},{"key":"B146","doi-asserted-by":"publisher","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":"B147","doi-asserted-by":"publisher","first-page":"223","DOI":"10.1097\/MAO.0b013e31802b3225","article-title":"Vestibular hair cell regeneration and restoration of balance function induced by math1 gene transfer","volume":"28","author":"Staecker","year":"2007","journal-title":"Otol Neurotol."},{"key":"B148","doi-asserted-by":"publisher","first-page":"884","DOI":"10.1038\/gt.2011.33","article-title":"Selective atonal gene delivery improves balance function in a mouse model of vestibular disease","volume":"18","author":"Schlecker","year":"2011","journal-title":"Gene Ther."},{"key":"B149","doi-asserted-by":"publisher","first-page":"254","DOI":"10.1159\/000192953","article-title":"Bcl-2 gene therapy prevents aminoglycoside-induced degeneration of auditory and vestibular hair cells","volume":"14","author":"Pfannenstiel","year":"2009","journal-title":"Audiol Neurootol."},{"key":"B150","doi-asserted-by":"publisher","first-page":"834","DOI":"10.1038\/mt.2013.18","article-title":"siRNA targeting Hes5 augments hair cell regeneration in aminoglycoside-damaged mouse utricle","volume":"21","author":"Jung","year":"2013","journal-title":"Mol Ther."},{"key":"B151","doi-asserted-by":"publisher","first-page":"819","DOI":"10.3109\/00016489.2012.673233","article-title":"Type I hair cell regeneration induced by Math1 gene transfer following neomycin ototoxicity in rat vestibular sensory epithelium","volume":"132","author":"Xu","year":"2012","journal-title":"Acta Otolaryngol."},{"key":"B152","doi-asserted-by":"publisher","first-page":"1046","DOI":"10.1038\/sj.gt.3301180","article-title":"Effect of transgenic GDNF expression on gentamicin-induced cochlear and vestibular toxicity","volume":"7","author":"Suzuki","year":"2000","journal-title":"Gene Ther."},{"key":"B153","doi-asserted-by":"publisher","first-page":"955","DOI":"10.1038\/nature02484","article-title":"Mutations in cadherin 23 affect tip links in zebrafish sensory hair cells","volume":"428","author":"Sollner","year":"2004","journal-title":"Nature."},{"key":"B154","doi-asserted-by":"publisher","first-page":"10188","DOI":"10.1523\/JNEUROSCI.1096-15.2015","article-title":"Zebrafish models for the mechanosensory hair cell dysfunction in Usher Syndrome 3 reveal that clarin-1 is an essential hair bundle protein","volume":"35","author":"Gopal","year":"2015","journal-title":"J Neurosci."},{"key":"B155","doi-asserted-by":"publisher","first-page":"915","DOI":"10.1007\/s00439-020-02254-z","article-title":"A biallelic variant in CLRN2 causes non-syndromic hearing loss in humans","volume":"140","author":"Vona","year":"2021","journal-title":"Hum Genet."},{"key":"B156","doi-asserted-by":"publisher","first-page":"215","DOI":"10.1007\/s10162-021-00798-z","article-title":"How zebrafish can drive the future of genetic-based hearing and balance research","volume":"22","author":"Sheets","year":"2021","journal-title":"J Assoc Res Otolaryngol."},{"key":"B157","doi-asserted-by":"publisher","first-page":"1882","DOI":"10.1093\/hmg\/ddaa004","article-title":"Clinical and preclinical therapeutic outcome metrics for USH2A-related disease","volume":"29","author":"Toms","year":"2020","journal-title":"Hum Mol Genet."},{"key":"B158","doi-asserted-by":"publisher","first-page":"42","DOI":"10.1097\/WCO.0b013e32834ef8b2","article-title":"Usher syndrome (sensorineural deafness and retinitis pigmentosa): pathogenesis, molecular diagnosis and therapeutic approaches","volume":"25","author":"Bonnet","year":"2012","journal-title":"Curr Opin Neurol."},{"key":"B159","doi-asserted-by":"publisher","first-page":"107932","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."},{"key":"B160","doi-asserted-by":"publisher","first-page":"220","DOI":"10.1016\/j.tins.2011.10.007","article-title":"Sensing sound: molecules that orchestrate mechanotransduction by hair cells","volume":"35","author":"Kazmierczak","year":"2012","journal-title":"Trends Neurosci."},{"key":"B161","doi-asserted-by":"publisher","first-page":"375","DOI":"10.1016\/j.neuron.2009.04.006","article-title":"Harmonin mutations cause mechanotransduction defects in cochlear hair cells","volume":"62","author":"Grillet","year":"2009","journal-title":"Neuron."},{"key":"B162","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1007\/s00424-009-0711-x","article-title":"Harmonin-b, an actin-binding scaffold protein, is involved in the adaptation of mechanoelectrical transduction by sensory hair cells","volume":"459","author":"Michalski","year":"2009","journal-title":"Pfl\u00fcgers Arch."},{"key":"B163","doi-asserted-by":"publisher","first-page":"406","DOI":"10.1016\/j.bbadis.2014.11.020","article-title":"Usher syndrome: hearing loss, retinal degeneration and associated abnormalities","volume":"1852","author":"Mathur","year":"2015","journal-title":"Biochim Biophys Acta."},{"key":"B164","doi-asserted-by":"publisher","first-page":"3921","DOI":"10.1093\/hmg\/ddi416","article-title":"Usherin, the defective protein in Usher syndrome type IIA, is likely to be a component of interstereocilia ankle links in the inner ear sensory cells","volume":"14","author":"Adato","year":"2005","journal-title":"Hum Mol Genet."},{"key":"B165","doi-asserted-by":"publisher","first-page":"6478","DOI":"10.1523\/JNEUROSCI.0342-07.2007","article-title":"Molecular characterization of the ankle link complex in cochlear hair cells and its role in the hair bundle functioning","volume":"27","author":"Michalski","year":"2007","journal-title":"J Neurosci."},{"key":"B166","doi-asserted-by":"publisher","first-page":"421","DOI":"10.1038\/ng1208","article-title":"Defects in whirlin, a PDZ domain molecule involved in stereocilia elongation, cause deafness in the whirler mouse and families with DFNB31","volume":"34","author":"Mburu","year":"2003","journal-title":"Nat Genet."},{"key":"B167","doi-asserted-by":"publisher","first-page":"203","DOI":"10.1007\/s00439-006-0304-0","article-title":"A novel gene for Usher syndrome type 2: mutations in the long isoform of whirlin are associated with retinitis pigmentosa and sensorineural hearing loss","volume":"121","author":"Ebermann","year":"2007","journal-title":"Hum Genet."},{"key":"B168","doi-asserted-by":"publisher","first-page":"401","DOI":"10.1093\/hmg\/ddi036","article-title":"Myosin XVa and whirlin, two deafness gene products required for hair bundle growth, are located at the stereocilia tips and interact directly","volume":"14","author":"Delprat","year":"2005","journal-title":"Hum Mol Genet."},{"key":"B169","doi-asserted-by":"publisher","first-page":"148","DOI":"10.1038\/ncb1219","article-title":"Myosin-XVa is required for tip localization of whirlin and differential elongation of hair-cell stereocilia","volume":"7","author":"Belyantseva","year":"2005","journal-title":"Nat Cell Biol."},{"key":"B170","doi-asserted-by":"publisher","first-page":"951","DOI":"10.1152\/physrev.00038.2013","article-title":"The physiology of mechanoelectrical transduction channels in hearing","volume":"94","author":"Fettiplace","year":"2014","journal-title":"Physiol Rev."},{"key":"B171","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1016\/j.heares.2019.02.007","article-title":"Usher syndrome and non-syndromic deafness: functions of different whirlin isoforms in the cochlea, vestibular organs, and retina","volume":"375","author":"Mathur","year":"2019","journal-title":"Hear Res."},{"key":"B172","doi-asserted-by":"publisher","first-page":"935","DOI":"10.1016\/j.celrep.2016.03.081","article-title":"Alternative splice forms influence functions of whirlin in mechanosensory hair cell stereocilia","volume":"15","author":"Ebrahim","year":"2016","journal-title":"Cell Rep."},{"key":"B173","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-021-02324-w","article-title":"Genetics, pathogenesis and therapeutic developments for Usher syndrome type 2","author":"Stemerdink","year":"2021","journal-title":"Hum Genet."},{"key":"B174","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1038\/sj.ejhg.5200831","article-title":"USH3A transcripts encode clarin-1, a four-transmembrane-domain protein with a possible role in sensory synapses","volume":"10","author":"Adato","year":"2002","journal-title":"Eur J Hum Genet."},{"key":"B175","doi-asserted-by":"publisher","first-page":"2748","DOI":"10.1093\/hmg\/ddp210","article-title":"Usher syndrome IIIA gene clarin-1 is essential for hair cell function and associated neural activation","volume":"18","author":"Geng","year":"2009","journal-title":"Hum Mol Genet."},{"key":"B176","doi-asserted-by":"publisher","first-page":"9485","DOI":"10.1523\/JNEUROSCI.0311-12.2012","article-title":"The mechanosensory structure of the hair cell requires clarin-1, a protein encoded by Usher syndrome III causative gene","volume":"32","author":"Geng","year":"2012","journal-title":"J Neurosci."},{"key":"B177","doi-asserted-by":"publisher","first-page":"3382","DOI":"10.1172\/JCI94351","article-title":"Clarin-1 gene transfer rescues auditory synaptopathy in model of Usher syndrome","volume":"128","author":"Dulon","year":"2018","journal-title":"J Clin Invest."},{"key":"B178","doi-asserted-by":"publisher","first-page":"9208","DOI":"10.1523\/JNEUROSCI.23-27-09208.2003","article-title":"A receptor-like inositol lipid phosphatase is required for the maturation of developing cochlear hair bundles","volume":"23","author":"Goodyear","year":"2003","journal-title":"J Neurosci."},{"key":"B179","doi-asserted-by":"publisher","first-page":"2762","DOI":"10.1523\/JNEUROSCI.3635-11.2012","article-title":"Hair bundle defects and loss of function in the vestibular end organs of mice lacking the receptor-like inositol lipid phosphatase PTPRQ","volume":"32","author":"Goodyear","year":"2012","journal-title":"J Neurosci."},{"key":"B180","doi-asserted-by":"publisher","first-page":"61","DOI":"10.1002\/cm.21159","article-title":"CLIC5 stabilizes membrane-actin filament linkages at the base of hair cell stereocilia in a molecular complex with radixin, taperin, myosin VI","volume":"71","author":"Salles","year":"2014","journal-title":"Cytoskeleton."},{"key":"B181","doi-asserted-by":"publisher","first-page":"297","DOI":"10.1007\/s10162-005-0009-4","article-title":"A quantitative survey of gravity receptor function in mutant mouse strains","volume":"6","author":"Jones","year":"2005","journal-title":"J Assoc Res Otolaryngol."},{"key":"B182","doi-asserted-by":"publisher","first-page":"504","DOI":"10.1016\/j.neuron.2013.06.019","article-title":"TMC1 and TMC2 are components of the mechanotransduction channel in hair cells of the mammalian inner ear","volume":"79","author":"Pan","year":"2013","journal-title":"Neuron."},{"key":"B183","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1007\/s00424-014-1582-3","article-title":"Transmembrane channel-like (TMC) genes are required for auditory and vestibular mechanosensation","volume":"467","author":"Kawashima","year":"2015","journal-title":"Pflugers Arch."},{"key":"B184","doi-asserted-by":"publisher","first-page":"a033506","DOI":"10.1101\/cshperspect.a033506","article-title":"Function and dysfunction of TMC channels in inner ear hair cells","volume":"9","author":"Corey","year":"2019","journal-title":"Cold Spring Harb Perspect Med."},{"key":"B185","doi-asserted-by":"publisher","first-page":"a033167","DOI":"10.1101\/cshperspect.a033167","article-title":"Molecular structure of the hair cell mechanoelectrical transduction complex","volume":"9","author":"Cunningham","year":"2019","journal-title":"Cold Spring Harb Perspect Med."},{"key":"B186","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/j.heares.2014.01.001","article-title":"TMC function in hair cell transduction","volume":"311","author":"Holt","year":"2014","journal-title":"Hear Res."},{"key":"B187","doi-asserted-by":"publisher","first-page":"736","DOI":"10.1016\/j.neuron.2018.07.033","article-title":"TMC1 forms the pore of mechanosensory transduction channels in vertebrate inner ear hair cells","volume":"99","author":"Pan","year":"2018","journal-title":"Neuron."},{"key":"B188","doi-asserted-by":"publisher","first-page":"2131","DOI":"10.1016\/j.neuron.2021.05.007","article-title":"CIB2 and CIB3 are auxiliary subunits of the mechanotransduction channel of hair cells","volume":"109","author":"Liang","year":"2021","journal-title":"Neuron."},{"key":"B189","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1146\/annurev-biophys-062420-081842","article-title":"The mechanosensory transduction machinery in inner ear hair cells","volume":"50","author":"Zheng","year":"2021","journal-title":"Annu Rev Biophys."},{"key":"B190","doi-asserted-by":"publisher","first-page":"1283","DOI":"10.1016\/j.cell.2012.10.041","article-title":"TMHS is an integral component of the mechanotransduction machinery of cochlear hair cells","volume":"151","author":"Xiong","year":"2012","journal-title":"Cell."},{"key":"B191","doi-asserted-by":"publisher","first-page":"954","DOI":"10.1016\/j.neuron.2014.10.041","article-title":"TMIE is an essential component of the mechanotransduction machinery of cochlear hair cells","volume":"84","author":"Zhao","year":"2014","journal-title":"Neuron."},{"key":"B192","doi-asserted-by":"publisher","first-page":"1265","DOI":"10.1038\/ng.2426","article-title":"Alterations of the CIB2 calcium- and integrin-binding protein cause Usher syndrome type 1J and nonsyndromic deafness DFNB48","volume":"44","author":"Riazuddin","year":"2012","journal-title":"Nat Genet."},{"key":"B193","doi-asserted-by":"publisher","first-page":"1711","DOI":"10.15252\/emmm.201708087","article-title":"CIB2, defective in isolated deafness, is key for auditory hair cell mechanotransduction and survival","volume":"9","author":"Michel","year":"2017","journal-title":"EMBO Mol Med."},{"key":"B194","doi-asserted-by":"publisher","first-page":"401","DOI":"10.3389\/fnmol.2017.00401","article-title":"Loss of CIB2 causes profound hearing loss and abolishes mechanoelectrical transduction in mice","volume":"10","author":"Wang","year":"2017","journal-title":"Front Mol Neurosci."},{"key":"B195","doi-asserted-by":"publisher","first-page":"812","DOI":"10.1111\/cge.13170","article-title":"Variants in CIB2 cause DFNB48 and not USH1J","volume":"93","author":"Booth","year":"2018","journal-title":"Clin Genet."},{"key":"B196","doi-asserted-by":"publisher","first-page":"43","DOI":"10.1038\/s41467-017-00061-1","article-title":"CIB2 interacts with TMC1 and TMC2 and is essential for mechanotransduction in auditory hair cells","volume":"8","author":"Giese","year":"2017","journal-title":"Nat Commun."},{"key":"B197","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1016\/j.heares.2011.05.009","article-title":"Hearing loss associated with enlargement of the vestibular aqueduct: mechanistic insights from clinical phenotypes, genotypes, mouse models","volume":"281","author":"Griffith","year":"2011","journal-title":"Hear Res."},{"key":"B198","doi-asserted-by":"publisher","DOI":"10.1007\/s00439-021-02311-1","article-title":"Genetic architecture and phenotypic landscape of SLC26A4-related hearing loss","author":"Honda","year":"2021","journal-title":"Hum Genet."},{"key":"B199","doi-asserted-by":"publisher","first-page":"153","DOI":"10.1093\/hmg\/10.2.153","article-title":"Targeted disruption of mouse Pds provides insight about the inner-ear defects encountered in Pendred syndrome","volume":"10","author":"Everett","year":"2001","journal-title":"Hum Mol Genet."},{"key":"B200","doi-asserted-by":"publisher","first-page":"e12635","DOI":"10.1111\/gbb.12635","article-title":"A mouse model for benign paroxysmal positional vertigo with genetic predisposition for displaced otoconia","volume":"19","author":"Dror","year":"2020","journal-title":"Genes Brain Behav."},{"key":"B201","doi-asserted-by":"publisher","first-page":"239","DOI":"10.1002\/dvdy.24195","article-title":"Mechanisms of otoconia and otolith development","volume":"244","author":"Lundberg","year":"2015","journal-title":"Dev Dyn."},{"key":"B202","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1016\/j.brainres.2006.02.083","article-title":"Assembly of the otoconia complex to the macular sensory epithelium of the vestibule","volume":"1091","author":"Lundberg","year":"2006","journal-title":"Brain Res."},{"key":"B203","doi-asserted-by":"publisher","first-page":"529","DOI":"10.1073\/pnas.96.2.529","article-title":"Characterization of otoconin-95, the major protein of murine otoconia, provides insights into the formation of these inner ear biominerals","volume":"96","author":"Verpy","year":"1999","journal-title":"Proc Natl Acad Sci USA."},{"key":"B204","doi-asserted-by":"publisher","first-page":"508","DOI":"10.1016\/j.ydbio.2007.01.013","article-title":"Gene targeting reveals the role of Oc90 as the essential organizer of the otoconial organic matrix","volume":"304","author":"Zhao","year":"2007","journal-title":"Dev Biol."},{"key":"B205","doi-asserted-by":"publisher","first-page":"1137","DOI":"10.1242\/dev.116632","article-title":"Otolith tethering in the zebrafish otic vesicle requires Otogelin and alpha-Tectorin","volume":"142","author":"Stooke-Vaughan","year":"2015","journal-title":"Development."},{"key":"B206","doi-asserted-by":"publisher","first-page":"151","DOI":"10.1016\/S0378-5955(01)00312-4","article-title":"Spatiotemporal expression of otogelin in the developing and adult mouse inner ear","volume":"158","author":"El-Amraoui","year":"2001","journal-title":"Hear Res."},{"key":"B207","doi-asserted-by":"publisher","first-page":"14450","DOI":"10.1073\/pnas.94.26.14450","article-title":"Otogelin: a glycoprotein specific to the acellular membranes of the inner ear","volume":"94","author":"Cohen-Salmon","year":"1997","journal-title":"Proc Natl Acad Sci USA."},{"key":"B208","doi-asserted-by":"publisher","first-page":"212","DOI":"10.1002\/neu.10097","article-title":"Extracellular matrices associated with the apical surfaces of sensory epithelia in the inner ear: molecular and structural diversity","volume":"53","author":"Goodyear","year":"2002","journal-title":"J Neurobiol."},{"key":"B209","doi-asserted-by":"publisher","first-page":"6240","DOI":"10.1073\/pnas.082515999","article-title":"Otoancorin, an inner ear protein restricted to the interface between the apical surface of sensory epithelia and their overlying acellular gels, is defective in autosomal recessive deafness DFNB22","volume":"99","author":"Zwaenepoel","year":"2002","journal-title":"Proc Natl Acad Sci USA."},{"key":"B210","doi-asserted-by":"publisher","first-page":"139","DOI":"10.1038\/72793","article-title":"Targeted disruption of otog results in deafness and severe imbalance","volume":"24","author":"Simmler","year":"2000","journal-title":"Nat Genet."},{"key":"B211","doi-asserted-by":"publisher","first-page":"703","DOI":"10.1038\/nn.2330","article-title":"Linking deafness genes to hair-bundle development and function","volume":"12","author":"Petit","year":"2009","journal-title":"Nat Neurosci."},{"key":"B212","doi-asserted-by":"publisher","first-page":"108327","DOI":"10.1016\/j.heares.2021.108327","article-title":"CACHD1-deficient mice exhibit hearing and balance deficits associated with a disruption of calcium homeostasis in the inner ear","volume":"409","author":"Tian","year":"2021","journal-title":"Hear Res."},{"key":"B213","doi-asserted-by":"publisher","first-page":"3439","DOI":"10.1152\/jn.00525.2010","article-title":"Regulation of cellular calcium in vestibular supporting cells by otopetrin 1","volume":"104","author":"Kim","year":"2010","journal-title":"J Neurophysiol."},{"key":"B214","doi-asserted-by":"publisher","first-page":"274","DOI":"10.1093\/hmg\/ddab153","article-title":"Advances in mouse genetics for the study of human disease","volume":"30","author":"Brown","year":"2021","journal-title":"Hum Mol Genet."},{"key":"B215","doi-asserted-by":"publisher","first-page":"416","DOI":"10.1038\/s41588-021-00825-y","article-title":"A resource of targeted mutant mouse lines for 5,061 genes","volume":"53","author":"Birling","year":"2021","journal-title":"Nat Genet."},{"key":"B216","doi-asserted-by":"publisher","first-page":"R130","DOI":"10.1093\/hmg\/ddy158","article-title":"Genetic landscape of auditory dysfunction","volume":"27","author":"Bowl","year":"2018","journal-title":"Hum Mol Genet."},{"key":"B217","doi-asserted-by":"publisher","first-page":"886","DOI":"10.1038\/s41467-017-00595-4","article-title":"A large scale hearing loss screen reveals an extensive unexplored genetic landscape for auditory dysfunction","volume":"8","author":"Bowl","year":"2017","journal-title":"Nat Commun."},{"key":"B218","doi-asserted-by":"publisher","first-page":"e3000194","DOI":"10.1371\/journal.pbio.3000194","article-title":"Mouse screen reveals multiple new genes underlying mouse and human hearing loss","volume":"17","author":"Ingham","year":"2019","journal-title":"PLoS Biol."},{"key":"B219","doi-asserted-by":"publisher","first-page":"119","DOI":"10.3233\/VES-130494","article-title":"Neuropharmacological basis of vestibular system disorder treatment","volume":"23","author":"Soto","year":"2013","journal-title":"J Vestib Res."},{"key":"B220","doi-asserted-by":"publisher","first-page":"26","DOI":"10.2174\/157015910790909511","article-title":"Neuropharmacology of vestibular system disorders","volume":"8","author":"Soto","year":"2010","journal-title":"Curr Neuropharmacol."},{"key":"B221","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1159\/000274957","article-title":"Central compensation of vestibular dysfunction. I. Peripheral lesions","volume":"32","author":"Pfaltz","year":"1970","journal-title":"Pract Otorhinolaryngol."},{"key":"B222","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1016\/0165-0173(89)90013-1","article-title":"Mechanisms of recovery following unilateral labyrinthectomy: a review","volume":"14","author":"Smith","year":"1989","journal-title":"Brain Res Brain Res Rev."},{"key":"B223","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1097\/00019052-200002000-00006","article-title":"Vestibular compensation and substitution","volume":"13","author":"Curthoys","year":"2000","journal-title":"Curr Opin Neurol."},{"key":"B224","doi-asserted-by":"publisher","first-page":"S54","DOI":"10.1007\/s00415-015-7903-4","article-title":"Vestibular compensation: the neuro-otologist's best friend","volume":"263","author":"Lacour","year":"2016","journal-title":"J Neurol."},{"key":"B225","doi-asserted-by":"publisher","first-page":"184","DOI":"10.3988\/jcn.2011.7.4.184","article-title":"Vestibular rehabilitation therapy: review of indications, mechanisms, key exercises","volume":"7","author":"Han","year":"2011","journal-title":"J Clin Neurol."},{"key":"B226","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1016\/j.neucli.2013.10.138","article-title":"Vestibular compensation and vestibular rehabilitation. Current concepts and new trends","volume":"44","author":"Deveze","year":"2014","journal-title":"Neurophysiol Clin."},{"key":"B227","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1097\/NPT.0000000000000120","article-title":"Vestibular rehabilitation for peripheral vestibular hypofunction: an evidence-based clinical practice guideline: from The American Physical Therapy Association Neurology Section","volume":"40","author":"Hall","year":"2016","journal-title":"J Neurol Phys Ther."},{"key":"B228","doi-asserted-by":"publisher","first-page":"Doc01","DOI":"10.3205\/cto000140","article-title":"Recent surgical options for vestibular vertigo","volume":"16","author":"Volkenstein","year":"2017","journal-title":"GMS Curr Top Otorhinolaryngol Head Neck Surg."},{"key":"B229","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1177\/000348941112000202","article-title":"Eye movements in response to electrical stimulation of the lateral and superior ampullary nerves","volume":"120","author":"Guyot","year":"2011","journal-title":"Ann Otol Rhinol Laryngol."},{"key":"B230","doi-asserted-by":"publisher","first-page":"66","DOI":"10.3389\/fneur.2014.00066","article-title":"Artificial balance: restoration of the vestibulo-ocular reflex in humans with a prototype vestibular neuroprosthesis","volume":"5","author":"Perez Fornos","year":"2014","journal-title":"Front Neurol."},{"key":"B231","doi-asserted-by":"publisher","first-page":"600","DOI":"10.3389\/fneur.2017.00600","article-title":"The video head impulse test to assess the efficacy of vestibular implants in humans","volume":"8","author":"Guinand","year":"2017","journal-title":"Front Neurol"},{"key":"B232","doi-asserted-by":"publisher","first-page":"577","DOI":"10.3389\/fnins.2016.00577","article-title":"Restoring visual acuity in dynamic conditions with a vestibular implant","volume":"10","author":"Guinand","year":"2016","journal-title":"Front Neurosci."},{"key":"B233","doi-asserted-by":"publisher","first-page":"e128397","DOI":"10.1172\/jci.insight.128397","article-title":"Continuous vestibular implant stimulation partially restores eye-stabilizing reflexes","volume":"4","author":"Boutros","year":"2019","journal-title":"JCI Insight."},{"key":"B234","doi-asserted-by":"publisher","first-page":"521","DOI":"10.1056\/NEJMoa2020457","article-title":"Posture, gait, quality of life, and hearing with a vestibular implant","volume":"384","author":"Chow","year":"2021","journal-title":"N Engl J Med."},{"key":"B235","doi-asserted-by":"publisher","first-page":"789","DOI":"10.1097\/MAO.0b013e318254ec24","article-title":"Implantation of the semicircular canals with preservation of hearing and rotational sensitivity: a vestibular neurostimulator suitable for clinical research","volume":"33","author":"Rubinstein","year":"2012","journal-title":"Otol Neurotol."},{"key":"B236","doi-asserted-by":"publisher","first-page":"3866","DOI":"10.1152\/jn.00171.2013","article-title":"Vestibular implantation and longitudinal electrical stimulation of the semicircular canal afferents in human subjects","volume":"113","author":"Phillips","year":"2015","journal-title":"J Neurophysiol."},{"key":"B237","doi-asserted-by":"publisher","first-page":"68","DOI":"10.1097\/MAO.0000000000002463","article-title":"Results from a second-generation vestibular implant in human subjects: diagnosis may impact electrical sensitivity of vestibular afferents","volume":"41","author":"Rubinstein","year":"2020","journal-title":"Otol Neurotol."},{"key":"B238","doi-asserted-by":"publisher","first-page":"154","DOI":"10.5152\/iao.2017.4216","article-title":"Vestibular response to electrical stimulation of the otolith organs. Implications in the development of a vestibular implant for the improvement of the sensation of gravitoinertial accelerations","volume":"13","author":"Ramos de Miguel","year":"2017","journal-title":"J Int Adv Otol."},{"key":"B239","doi-asserted-by":"publisher","first-page":"307","DOI":"10.1097\/MOO.0000000000000477","article-title":"Molecular therapy for genetic and degenerative vestibular disorders","volume":"26","author":"Sayyid","year":"2018","journal-title":"Curr Opin Otolaryngol Head Neck Surg."},{"key":"B240","doi-asserted-by":"publisher","first-page":"107947","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."},{"key":"B241","doi-asserted-by":"publisher","first-page":"1241062","DOI":"10.1126\/science.1241062","article-title":"Sound strategies for hearing restoration","volume":"344","author":"Geleoc","year":"2014","journal-title":"Science."},{"key":"B242","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1016\/j.omtm.2021.03.005","article-title":"Advances and challenges in adeno-associated viral inner-ear gene therapy for sensorineural hearing loss","volume":"21","author":"Bankoti","year":"2021","journal-title":"Mol Ther Methods Clin Dev."},{"key":"B243","doi-asserted-by":"publisher","first-page":"737632","DOI":"10.3389\/fgeed.2021.737632","article-title":"Progress in gene editing tools and their potential for correcting mutations underlying hearing and vision loss","volume":"3","author":"Botto","year":"2021","journal-title":"Front Gene Edit."},{"key":"B244","doi-asserted-by":"publisher","first-page":"1123","DOI":"10.1038\/s41591-019-0500-9","article-title":"Allele-specific gene editing prevents deafness in a model of dominant progressive hearing loss","volume":"25","author":"Gyorgy","year":"2019","journal-title":"Nat Med."},{"key":"B245","doi-asserted-by":"publisher","first-page":"R65","DOI":"10.1093\/hmg\/ddz129","article-title":"Gene therapy for hearing loss","volume":"28","author":"Omichi","year":"2019","journal-title":"Hum Mol Genet."},{"key":"B246","doi-asserted-by":"publisher","first-page":"107958","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."},{"key":"B247","doi-asserted-by":"publisher","first-page":"999","DOI":"10.1080\/00016489.2016.1183169","article-title":"Regenerative therapy for vestibular disorders using human induced pluripotent stem cells (iPSCs): neural differentiation of human iPSC-derived neural stem cells after in vitro transplantation into mouse vestibular epithelia","volume":"136","author":"Taura","year":"2016","journal-title":"Acta Otolaryngol."},{"key":"B248","doi-asserted-by":"publisher","first-page":"583","DOI":"10.1038\/nbt.3840","article-title":"Generation of inner ear organoids containing functional hair cells from human pluripotent stem cells","volume":"35","author":"Koehler","year":"2017","journal-title":"Nat Biotechnol."},{"key":"B249","doi-asserted-by":"publisher","first-page":"dev177188","DOI":"10.1242\/dev.177188","article-title":"Inner ear organoids: new tools to understand neurosensory cell development, degeneration and regeneration","volume":"146","author":"Roccio","year":"2019","journal-title":"Development."},{"key":"B250","doi-asserted-by":"publisher","first-page":"108646","DOI":"10.1016\/j.celrep.2020.108646","article-title":"Greater epithelial ridge cells are the principal organoid-forming progenitors of the mouse cochlea","volume":"34","author":"Kubota","year":"2021","journal-title":"Cell Rep."},{"key":"B251","doi-asserted-by":"publisher","first-page":"673","DOI":"10.1002\/stem.3353","article-title":"Human induced pluripotent stem cells and CRISPR\/Cas-mediated targeted genome editing: platforms to tackle sensorineural hearing loss","volume":"39","author":"Stojkovic","year":"2021","journal-title":"Stem Cells."},{"key":"B252","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1038\/s41418-020-00678-8","article-title":"Building inner ears: recent advances and future challenges for in vitro organoid systems","volume":"28","author":"van der Valk","year":"2021","journal-title":"Cell Death Differ."},{"key":"B253","doi-asserted-by":"publisher","first-page":"7","DOI":"10.3389\/fneur.2020.00007","article-title":"Prediction of vestibular dysfunction by applying machine learning algorithms to postural instability","volume":"11","author":"Kamogashira","year":"2020","journal-title":"Front Neurol."},{"key":"B254","doi-asserted-by":"publisher","first-page":"326","DOI":"10.21053\/ceo.2020.00654","article-title":"Recent advances in the application of artificial intelligence in otorhinolaryngology-head and neck surgery","volume":"13","author":"Tama","year":"2020","journal-title":"Clin Exp Otorhinolaryngol."},{"key":"B255","doi-asserted-by":"publisher","first-page":"377","DOI":"10.1007\/s00415-020-10139-4","article-title":"Diagnosing vestibular hypofunction: an update","volume":"268","author":"Starkov","year":"2021","journal-title":"J Neurol."},{"key":"B256","doi-asserted-by":"publisher","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":"B257","doi-asserted-by":"publisher","first-page":"107882","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."}],"container-title":["Frontiers in Neurology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2022.816534\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,4]],"date-time":"2022-04-04T07:34:02Z","timestamp":1649057642000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2022.816534\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,4]]},"references-count":257,"alternative-id":["10.3389\/fneur.2022.816534"],"URL":"https:\/\/doi.org\/10.3389\/fneur.2022.816534","relation":{},"ISSN":["1664-2295"],"issn-type":[{"value":"1664-2295","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4,4]]},"article-number":"816534"}}