With more than 466 million people affected, hearing loss represents the most common sensory pathology worldwide. Despite its widespread occurrence, much remains to be explored, particularly concerning the intricate pathogenic mechanisms underlying its diverse phenotypes. In this context, metabolomics emerges as a promising approach. Indeed, lying downstream from molecular biology's central dogma, the metabolome reflects both genetic traits and environmental influences. Furthermore, its dynamic nature facilitates well-defined changes during disease states, making metabolomic analysis a unique lens into the mechanisms underpinning various hearing impairment forms. Hence, these investigations may pave the way for improved diagnostic strategies, personalized interventions and targeted treatments, ultimately enhancing the clinical management of affected individuals. In this comprehensive review, we discuss findings from 20 original articles, including human and animal studies. Existing literature highlights specific metabolic changes associated with hearing loss and ototoxicity of certain compounds. Nevertheless, numerous critical issues have emerged from the study of the current state of the art, with the lack of standardization of methods, significant heterogeneity in the studies and often small sample sizes being the main limiting factors for the reliability of these findings. Therefore, these results should serve as a stepping stone for future research aimed at addressing the aforementioned challenges.
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http://dx.doi.org/10.3390/ijms242015188 | DOI Listing |
Front Neurol
December 2024
Department of Otorhinolaryngology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Objectives: Multiple studies have described the onset and variable incidence of postoperative acute vertigo following cochlear implant (CI) surgery. However, postoperative imaging has not yet been specifically evaluated with special focus on vertigo. The aim of this study is to assess the incidence and causes of new-onset, acute postoperative vertigo following CI surgery using cone beam computed tomography (CBCT).
View Article and Find Full Text PDFBackground: Alport syndrome (AS) is a multifaceted condition that primarily affects the basement membranes of the kidneys, ears, and eyes. AS is considered the second most common cause of hereditary renal failure, exhibiting varied clinical manifestations across different lifespans. The aim of this study is to investigate the clinical features and genetic profile of AS and to elucidate the genotype-phenotype correlation of AS.
View Article and Find Full Text PDFMutations in the gene cause the most common form of human hereditary hearing loss, known as DFNB1. is expressed in two cell groups of the cochlea-epithelial cells of the organ of Corti and fibrocytes of the inner sulcus and lateral wall-but not by sensory hair cells or neurons. Attempts to treat mouse models of DFNB1 with AAV vectors mediating nonspecific expression have not substantially restored function, perhaps because inappropriate expression in hair cells and neurons could compromise their electrical activity.
View Article and Find Full Text PDFBackground: -related schwannomatosis ( -SWN) is a debilitating condition that calls for robust treatment options. The defining feature of -SWN is the presence of bilateral vestibular schwannomas (VSs), which grow over time and can result in irreversible sensorineural hearing loss, significantly affecting the quality of life for those affected. At present, there are no FDA-approved medications specifically for treating VS or related hearing loss.
View Article and Find Full Text PDFActa Otorhinolaryngol Ital
December 2024
Unit of Audiology, Regional Centre of Cochlear Implants and ENT Diseases, Department of Experimental and Clinical Medicine, Magna Graecia University, Catanzaro, Italy.
Objectives: This research aims to validate the digits-in-noise (DIN) test for the Italian language and develop a version capable of independently assessing both ears while maintaining acceptable administration times.
Methods: Individual digits from 0 to 9 in Italian were recorded and adjusted to equalise recognition probabilities. An iOS application (APP) was developed for the independent ear test using triplets in noise.
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