Hearing impairment in MELAS: new prospective in clinical use of microRNA, a systematic review

Orphanet Journal of Rare Diseases
A Di StadioCorrado Angelini

Abstract

To evaluate the feasibility of microRNAs (miR) in clinical use to fill in the gap of current methodology commonly used to test hearing impairment in MELAS patients. A literature review was performed using the following keywords, i.e., MELAS, Hearing Loss, Hearing Impairment, Temporal Bone, Otoacustic Emission (OTOAE), Auditory Brain Response (ABR), and microRNA. We reviewed the literature and focused on the aspect of the temporal bone, the results of electrophysiological tests in human clinical studies, and the use of miR for detecting lesions in the cochlea in patients with MELAS. In patients with MELAS, Spiral Ganglions (SG), stria vascularis (SV), and hair cells are damaged, and these damages affect in different ways various structures of the temporal bone. The function of these cells is typically investigated using OTOAE and ABR, but in patients with MELAS these tests provide inconsistent results, since OTOAE response is absent and ABR is normal. The normal ABR responses are unexpected given the SG loss in the temporal bone. Recent studies in humans and animals have shown that miRs, and in particular miRs 34a, 29b, 76, 96, and 431, can detect damage in the cells of the cochlea with high sensitivity. Studies that focus on th...Continue Reading

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Citations

Feb 23, 2020·The International Journal of Neuroscience·Roberta MarozzoArianna Di Stadio
Jun 2, 2018·Orphanet Journal of Rare Diseases·Josef Finsterer, Sinda Zarrouk-Mahjoub
Jul 20, 2018·Orphanet Journal of Rare Diseases·Arianna Di Stadio, Corrado Angelini
Sep 29, 2018·International Journal of Environmental Research and Public Health·Arianna Di StadioMassimo Ralli
Mar 5, 2020·Laryngoscope Investigative Otolaryngology·Ophir HandzelJoseph B Nadol
Dec 2, 2020·Orphanet Journal of Rare Diseases·Yen-Chi ChenYen-Fu Cheng
Jan 8, 2021·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Arianna Di StadioEvanthia Bernitsas
Aug 2, 2021·Metabolism: Clinical and Experimental·T-T TrinhD Bakhos
Aug 6, 2021·Laryngo- rhino- otologie·Athanasia Warnecke, Anja Giesemann

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