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. 2018 Feb 21;13(1):35.
doi: 10.1186/s13023-018-0770-1.

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

Arianna Di Stadio  1 , Valentina Pegoraro  2 , Laura Giaretta  2 , Laura Dipietro  3 , Roberta Marozzo  2 , Corrado Angelini  2
Affiliations

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

Arianna Di Stadio et al. Orphanet J Rare Dis. .

Abstract

Aim: 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.

Material and method: 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.

Results: 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.

Conclusion: We suggest that due to their accuracy and sensitivity, miRs might help monitor the progression of SNHL in patients with MELAS.

Keywords: Auditory brain response; Diagnosis; Hearing impairment; Hearing loss; MELAS; Otoacustic emission; microRNA.

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The authors declare that they have no competing interests.

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Figures

Fig. 1
Fig. 1
The image shows the different shapes as a function of inner ear cells damage as recorded during a pure auditory test. a SensoryNeural Hearing Loss; b Metabolic hearing Loss; c Neural Hearing Loss
Fig. 2
Fig. 2
Two methods are used for investigating the hearing pathways. Cochlear function is investigated by OTOAE/TEOAE/DPOAE (which can identify the hair cells damage). The retro-cochlear portion is investigated by ABR. The table on the left shows the area that generates the specific wave; the image shows the area and the wave
Fig. 3
Fig. 3
The image summarizes the mechanisms regulated by miR 34 a able to determine the damage of the inner ear structures. From top to bottom: Stria Vascularis, Organ of Corti with hair cells, and Spiral Ganglions in the human temporal bone
Fig. 4
Fig. 4
The image shows the details on miR and the structure of the inner ear that,when damaged, can change their levels in blood of patients
See this image and copyright information in PMC

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