SynthesisOrphanet journal of rare diseases2018
Hearing impairment in MELAS: new prospective in clinical use of microRNA, a systematic review.
Synthesis in Orphanet journal of rare diseases, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 2 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
15 citing papers in PubMed, 2 syntheses or guidelines pooled it, 21 citations in OpenAlex.
- Hearing loss and dementia: radiologic and biomolecular basis of their shared characteristics. A systematic review.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2021Pooled it
- Hearing Loss, Tinnitus, Hyperacusis, and Diplacusis in Professional Musicians: A Systematic Review.International journal of environmental research and public health · 2018Pooled it
- Hearing Loss and Alzheimer Disease.Current topics in behavioral neurosciences · 2025Review
- Beyond the cochlea: exploring the multifaceted nature of hearing loss in primary mitochondrial diseases.Brain communications · 2024Article
- The bidirectional brain-cochlea axis: a scaffold for neurologic disease-associated hearing loss.Brain communications · 2024Article
- MELAS: Phenotype Classification into Classic-versus-Atypical Presentations.AJNR. American journal of neuroradiology · 2023Review
- Delay in diagnosing a patient with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome who presented with status epilepticus and lactic acidosis: a case report.Journal of medical case reports · 2022Article
- Recent advances in mitochondrial diseases: From molecular insights to therapeutic perspectives.Saudi pharmaceutical journal : SPJ : the official publication of the Saudi Pharmaceutical Society · 2022Review
- Impaired Hearing in MELAS.The journal of international advanced otology · 2022Article
- Embryology, Malformations, and Rare Diseases of the Cochlea.Laryngo- rhino- otologie · 2021Article
- Audiological and otologic manifestations of glutaric aciduria type I.Orphanet journal of rare diseases · 2020Article
- Temporal bone histopathology in MELAS syndrome.Laryngoscope investigative otolaryngology · 2020Article
- Review
- MicroRNAs are appropriate in mitochondrial related hearing loss? Answer to the skepticism.Orphanet journal of rare diseases · 2018Article
- MicroRNAs are inappropriate for characterising hearing impairment in mitochondrial disorders.Orphanet journal of rare diseases · 2018Article
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 2 countries.
Funding
Abstract
aimTo 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
methodA 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.
resultsIn 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.
conclusionWe suggest that due to their accuracy and sensitivity, miRs might help monitor the progression of SNHL in patients with MELAS.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.