In the United States there are, at present, no drugs that are specifically FDA approved to treat hearing loss. Although several clinical trials are ongoing, including one testing D-methionine that is supported by the US Army, none of these trials directly address the effect of noise exposure on cochlear spiral ganglion neurons. We recently published the first report of a systematic chemical compound screen using primary, mammalian spiral ganglion cultures in which we were able to detect a compound and others in its class that increased neurite elongation, a critical step in restoring cochlear synapses after noise induced hearing loss. Here we discuss the issues, both pro and con, that influenced the development of our approach. These considerations may be useful for future compound screens that target the same or other attributes of cochlear spiral ganglion neurons.
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http://dx.doi.org/10.1016/j.heares.2016.07.019 | 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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