In the mature cochlea, each inner hair cell (IHC) is innervated by multiple spiral ganglion neurons of type I (SGNI). SGNIs are morphologically and electro-physiologically diverse. Also, they differ in their susceptibility to noise insult. However, the molecular underpinnings of their identity and physiological differences remain poorly understood. In this study, we developed a novel triple transgenic mouse, which enabled the isolation of pure populations of SGNIs and the analysis of a 96-gene panel single-cell qPCR. We found three distinct populations of Type I SGNs, which were marked by their exclusive expression of , or , respectively, at postnatal days P3, P8, and P12. Our data suggest that afferent SGN subtypes are established genetically before the onset of hearing and that the expression of key physiological markers, such as ion channels, is heterogeneous and may be underlying the heterogeneous firing proprieties of SGNIs.
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http://dx.doi.org/10.3389/fnmol.2020.00083 | DOI Listing |
J Assoc Res Otolaryngol
January 2025
The Bionics Institute, 384-388 Albert St, East Melbourne, VIC, 3002, Australia.
Purpose: Variations in neural survival along the cochlear implant electrode array leads to off-place listening, resulting in poorer speech understanding outcomes for recipients. Therefore, it is important to develop and compare clinically viable tests to identify these patient-specific intra-cochlear neural differences.
Methods: Nineteen experienced cochlear implant recipients (9 males and 10 females) were recruited for this study.
J Otol
October 2024
The Institute of Audiology and Balance Science, Xuzhou Medical University, Xuzhou, Jiangsu, 221004, China.
Objective: This study aims to explore the expression patterns of cysteine string protein alpha (CSPα) and cysteine string protein beta (CSPβ) in the mammalian inner ear, with an emphasis on their temporal dynamics during the developmental stages of C57BL/6 mice.
Methods: We utilized immunofluorescence staining to assess the localization and distribution of CSPα and CSPβ within the inner ears of C57BL/6 mice and miniature pigs. Additionally, this method facilitated the investigation of their temporal expression profiles.
Biomedicines
November 2024
Department of Otorhinolaryngology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430022, China.
Hearing loss (HL) is the most common disorder in newborns with a highly heterogeneous genetic background. Despite significant progress in screening and identifying genes related to congenital hearing loss, there are still candidate genes implicated in HL that remain undiscovered. We investigated HL in 43 Chinese families by segregating bilateral sensorineural HL via whole-exome sequencing (WES) and Sanger sequencing.
View Article and Find Full Text PDFTissue Cell
December 2024
ENT Department, Faculty of Medicine, Suez Canal University, Ismailia, Egypt. Electronic address:
Background: Sensorineural hearing loss (SNHL) is the most common sensory deficit worldwide. Current solutions for SNHL, including hearing aids, cochlear implants, and hearing assistive devices, do not provide consistent results and fail to address the underlying pathology of hair cell and ganglion cell damage. Stem cell therapy is a cornerstone in regenerative medicine.
View Article and Find Full Text PDFJ Otol
July 2024
Department of Biochemistry and Molecular Biology, and Key Laboratory for Genetic Hearing Disorders in Shandong, Binzhou Medical University, 346 Guanhai Road, Yantai, 264003, Shandong, PR China.
Objectives: Deletion of gene in mice has been linked to progressive hearing loss and degeneration of cochlear cells. Cisplatin, an antitumor drug, can cause various side effects, including ototoxicity. The aim of this study was to investigate the effects of on cisplatin-induced hearing impairment in mice and to explore the possible mechanism.
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