ReviewJournal of the American Academy of Audiology2014
Genetics of peripheral vestibular dysfunction: lessons from mutant mouse strains.
Review in Journal of the American Academy of Audiology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 30 papers.
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Who cites it
30 citing papers in PubMed, 42 citations in OpenAlex.
- Auditory contributions to postural and locomotor control: from basic research to clinical applications.Experimental brain research · 2026Review
- Recovery kinetics of dual AAV-mediated human otoferlin expression.Frontiers in molecular neuroscience · 2024Article
- Spontaneous allelic variant in deafness-blindness gene Ush1g resulting in an expanded phenotype.Genes, brain, and behavior · 2023Article
- Vestibular Deficits in Deafness: Clinical Presentation, Animal Modeling, and Treatment Solutions.Frontiers in neurology · 2022Review
- Co-transduction of dual-adeno-associated virus vectors in the neonatal and adult mouse utricles.Frontiers in molecular neuroscience · 2022Article
- Vestibular Hair Cells Require CAMSAP3, a Microtubule Minus-End Regulator, for Formation of Normal Kinocilia.Frontiers in cellular neuroscience · 2022Article
- Otoconia Structure After Short- and Long-Duration Exposure to Altered Gravity.Journal of the Association for Research in Otolaryngology : JARO · 2021Article
- Article
- Current Response in CaFrontiers in neuroscience · 2021Article
- Intense noise exposure alters peripheral vestibular structures and physiology.Journal of neurophysiology · 2020Article
- Quantitative Assessment of Anti-Gravity Reflexes to Evaluate Vestibular Dysfunction in Rats.Journal of the Association for Research in Otolaryngology : JARO · 2019Article
- Spontaneous mutations of the Zpld1 gene in mice cause semicircular canal dysfunction but do not impair gravity receptor or hearing functions.Scientific reports · 2019Article
- Unconventional secretory pathway activation restores hair cell mechanotransduction in an USH3A model.Proceedings of the National Academy of Sciences of the United States of America · 2019Article
- Antisense Oligonucleotides for the Treatment of Inner Ear Dysfunction.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2019Review
- AAV2.7m8 is a powerful viral vector for inner ear gene therapy.Nature communications · 2019Article
- Single Cell and Single Nucleus RNA-Seq Reveal Cellular Heterogeneity and Homeostatic Regulatory Networks in Adult Mouse Stria Vascularis.Frontiers in molecular neuroscience · 2019Article
- Specializations for Fast Signaling in the Amniote Vestibular Inner Ear.Integrative and comparative biology · 2018Review
- Deafness and vestibular dysfunction in a Doberman Pinscher puppy associated with a mutation in the PTPRQ gene.Journal of veterinary internal medicine · 2018Article
- Vestibular short-latency evoked potential abolished by low-frequency noise exposure in rats.Journal of neurophysiology · 2018Article
- Loss of α-Calcitonin Gene-Related Peptide (αCGRP) Reduces Otolith Activation Timing Dynamics and Impairs Balance.Frontiers in molecular neuroscience · 2018Article
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
backgroundA considerable amount of research has been published about genetic hearing impairment. Fifty to sixty percent of hearing loss is thought to have a genetic cause. Genes may also play a significant role in acquired hearing loss due to aging, noise exposure, or ototoxic medications. Between 1995 and 2012, over 100 causative genes have been identified for syndromic and nonsyndromic forms of hereditary hearing loss. Mouse models have been extremely valuable in facilitating the discovery of hearing loss genes and in understanding inner ear pathology due to genetic mutations or elucidating fundamental mechanisms of inner ear development. PURPOSE: Whereas much is being learned about hereditary hearing loss and the genetics of cochlear disorders, relatively little is known about the role genes may play in peripheral vestibular impairment. Here we review the literature with regard to genetics of vestibular dysfunction and discuss what we have learned from studies using mutant mouse models and direct measures of peripheral vestibular neural function.
resultsSeveral genes are considered that when mutated lead to varying degrees of inner ear vestibular dysfunction due to deficits in otoconia, stereocilia, hair cells, or neurons. Behavior often does not reveal the inner ear deficit. Many of the examples presented are also known to cause human disorders.
conclusionsKnowledge regarding the roles of particular genes in the operation of the vestibular sensory apparatus is growing, and it is clear that gene products co-expressed in the cochlea and vestibule may play different roles in the respective end organs. The discovery of new genes mediating critical inner ear vestibular function carries the promise of new strategies in diagnosing, treating, and managing patients as well as predicting the course and level of morbidity in human vestibular disease.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.