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

Overview of attention for article published in Orphanet Journal of Rare Diseases, February 2018
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Title
Hearing impairment in MELAS: new prospective in clinical use of microRNA, a systematic review
Published in
Orphanet Journal of Rare Diseases, February 2018
DOI 10.1186/s13023-018-0770-1
Pubmed ID
Authors

Arianna Di Stadio, Valentina Pegoraro, Laura Giaretta, Laura Dipietro, Roberta Marozzo, Corrado 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 the temporal bone aspects have reported that miRs increase is correlated with the death of specific cells of the inner ear. MiR - 9/9* was identified as a biomarker of human brain damage, miRs levels increase might be related to damage in the central auditory pathways and these increased levels could identify the damage with higher sensitivity and several months before than electrophysiological testing. We suggest that due to their accuracy and sensitivity, miRs might help monitor the progression of SNHL in patients with MELAS.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 29 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 29 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 8 28%
Student > Master 3 10%
Student > Ph. D. Student 3 10%
Other 2 7%
Librarian 1 3%
Other 3 10%
Unknown 9 31%
Readers by discipline Count As %
Medicine and Dentistry 8 28%
Biochemistry, Genetics and Molecular Biology 4 14%
Agricultural and Biological Sciences 2 7%
Nursing and Health Professions 1 3%
Sports and Recreations 1 3%
Other 3 10%
Unknown 10 34%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 28 February 2018.
All research outputs
#18,589,103
of 23,025,074 outputs
Outputs from Orphanet Journal of Rare Diseases
#2,163
of 2,642 outputs
Outputs of similar age
#257,279
of 331,231 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#31
of 35 outputs
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