ArticleHearing research2014
Connexin 26 null mice exhibit spiral ganglion degeneration that can be blocked by BDNF gene therapy.
Article in Hearing research, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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Who cites it
40 citing papers in PubMed, 57 citations in OpenAlex.
- Recent Progress in Mechanism-Based Therapies forInternational journal of molecular sciences · 2026Review
- Exploring Embryonic and Postnatal Gene Therapy Approaches for GJB2-Related Deafness: A Scoping Review.Audiology research · 2026Review
- Cell-specific delivery of GJB2 restores auditory function in mouse models of DFNB1 deafness and mediates appropriate expression in NHP cochlea.Nature communications · 2025Article
- Visualization of the Spiral Ganglion Neuron in Vivo Using a NovelAdvanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025Article
- Early control of cochlear viral load limits cochlear inflammation and prevents virus-induced sensorineural hearing loss.Journal of neuroinflammation · 2025Article
- Article
- An α7 nicotinic and GABAThe Journal of physiology · 2025Article
- Abnormal Innervation, Demyelination, and Degeneration of Spiral Ganglion Neurons as Well as Disruption of Heminodes are Involved in the Onset of Deafness in Cx26 Null Mice.Neuroscience bulletin · 2024Article
- Cochlear Health and Cochlear-implant Function.Journal of the Association for Research in Otolaryngology : JARO · 2023Review
- Connexin Mutations and Hereditary Diseases.International journal of molecular sciences · 2022Review
- No Protective Effects of Hair Cells or Supporting Cells in Ototoxically Deafened Guinea Pigs upon Administration of BDNF.Brain sciences · 2021Article
- Article
- Impact of Systemic versus Intratympanic Dexamethasone Administration on the Perilymph Proteome.Journal of proteome research · 2021Article
- Advances and challenges in adeno-associated viral inner-ear gene therapy for sensorineural hearing loss.Molecular therapy. Methods & clinical development · 2021Review
- Advances in genome editing for genetic hearing loss.Advanced drug delivery reviews · 2021Review
- Mechanism and Prevention of Spiral Ganglion Neuron Degeneration in the Cochlea.Frontiers in cellular neuroscience · 2021Review
- Research progress on flat epithelium of the inner ear.Physiological research · 2020Review
- Advances in Inner Ear Therapeutics for Hearing Loss in Children.Current otorhinolaryngology reports · 2020Article
- Article
- Growth factor and receptor malfunctions associated with human genetic deafness.Clinical genetics · 2020Review
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 3 countries.
Funding
Abstract
Mutations in the connexin 26 gene (GJB2) are the most common genetic cause of deafness, leading to congenital bilateral non-syndromic sensorineural hearing loss. Here we report the generation of a mouse model for a connexin 26 (Cx26) mutation, in which cre-Sox10 drives excision of the Cx26 gene from non-sensory cells flanking the auditory epithelium. We determined that these conditional knockout mice, designated Gjb2-CKO, have a severe hearing loss. Immunocytochemistry of the auditory epithelium confirmed absence of Cx26 in the non-sensory cells. Histology of the organ of Corti and the spiral ganglion neurons (SGNs) performed at ages 1, 3, or 6 months revealed that in Gjb2-CKO mice, the organ of Corti began to degenerate in the basal cochlear turn at an early stage, and the degeneration rapidly spread to the apex. In addition, the density of SGNs in Rosenthal's canal decreased rapidly along a gradient from the base of the cochlea to the apex, where some SGNs survived until at least 6 months of age. Surviving neurons often clustered together and formed clumps of cells in the canal. We then assessed the influence of brain derived neurotrophic factor (BDNF) gene therapy on the SGNs of Gjb2-CKO mice by inoculating Adenovirus with the BDNF gene insert (Ad.BDNF) into the base of the cochlea via the scala tympani or scala media. We determined that over-expression of BDNF beginning around 1 month of age resulted in a significant rescue of neurons in Rosenthal's canal of the cochlear basal turn but not in the middle or apical portions. This data may be used to design therapies for enhancing the SGN physiological status in all GJB2 patients and especially in a sub-group of GJB2 patients where the hearing loss progresses due to ongoing degeneration of the auditory nerve, thereby improving the outcome of cochlear implant therapy in these ears.
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Registered trials
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.