This review presents some of the major historical events that advanced the body of knowledge of the anatomy of the inner ear and its sensory receptors as well as the biology of these receptors that underlies the sensory functions of hearing and balance. This knowledge base of the inner ear's structure/function has been an essential factor for the design and construction of prosthetic devices to aid patients with deficits in their senses of hearing and balance. Prosthetic devices are now available for severely hearing impaired and deaf patients to restore hearing and are known as cochlear implants and auditory brain stem implants. A prosthetic device for patients with balance disorders is being perfected and is in an animal model testing phase with another prosthetic device for controlling intractable dizziness in Meniere's patients currently being evaluated in clinical testing. None of this would have been possible without the pioneering studies and discoveries of the investigators mentioned in this review and with the work of many other talented investigators to numerous to be covered in this review.
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http://dx.doi.org/10.1002/ar.22598 | DOI Listing |
The recent ACHIEVE study (https://www.achievestudy.org/) demonstrated the substantial benefit of hearing aid use in those with mild-moderate hearing loss and at increased risk for cognitive decline.
View Article and Find Full Text PDFOrphanet J Rare Dis
January 2025
Senior Department of Otolaryngology Head and Neck Surgery, The 6th Medical Center of Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, 100048, China.
Background: Non-isolated auditory neuropathy (AN), or syndromic AN, is marked by AN along with additional systemic manifestations. The diagnostic process is challenging due to its varied symptoms and overlap with other syndromes. This study focuses on two mitochondrial function-related genes which result in non-isolated AN, FDXR and TWNK, providing a summary and enrichment analysis of genes associated with non-isolated AN to elucidate the genotype-phenotype correlation and underlying mechanisms.
View Article and Find Full Text PDFJ Neurosci
January 2025
Flaum Eye Institute, Department of Ophthalmology, University of Rochester Medical Center, Rochester, New York, 14642, USA;
The inner ear houses both hearing and balance sensory modalities. The hearing and balance organs consist of similar cell types, including sensory hair cells and associated supporting cells. Previously we showed that is required for maintaining supporting cell survival during cochlear maturation.
View Article and Find Full Text PDFJ Otol
October 2024
Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine, Aurora, CO, USA.
Objective: To better understand the clinical phenotype of Ménière's disease (MD), we examined family history, thyroid disorder, migraine, and associated disorders in complaints of people living with MD.
Method: We designed the study as a retrospective and examined data gathered from 912 participants with MD. Their data were originally collected by the Finnish Ménière Federation (FMF).
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.
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