ReviewJournal of clinical medicine2023
Current Advances in Gene Therapies of Genetic Auditory Neuropathy Spectrum Disorder.
Review in Journal of clinical medicine, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT07778641 (Genetic Study of OTOF- Related Auditory Neuropathy Spectrum Disorder), which is not on this map. Cited by 10 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
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.
Genetic Study of OTOF- Related Auditory Neuropathy Spectrum Disorder
Who cites it
10 citing papers in PubMed.
- Neural Hearing Loss: Mechanisms, Diagnosis and Treatment Horizons.Journal of the Association for Research in Otolaryngology : JARO · 2026Review
- Evaluation of Intracochlear Pressure and Fluid Distribution in 3D-Printed Artificial Cochlear Models and Human Petrous Bones.Brain sciences · 2025Article
- Identification of genetic mechanisms of non-isolated auditory neuropathy with various phenotypes in Chinese families.Orphanet journal of rare diseases · 2025Article
- A novel therapeutic pathway to the human cochlear nerve.Scientific reports · 2024Article
- Tissue engineering strategies for spiral ganglion neuron protection and regeneration.Journal of nanobiotechnology · 2024Review
- A novel mutation in theIntractable & rare diseases research · 2024Article
- The human OPA1Cellular and molecular life sciences : CMLS · 2024Article
- Modeling of auditory neuropathy spectrum disorders associated with theFrontiers in molecular neuroscience · 2024Article
- Predictors of cochlear implant outcomes in pediatric auditory neuropathy: A matched case-control study.PloS one · 2024Article
- Polygenic Risk Score-Based Association Analysis of Speech-in-Noise and Hearing Threshold Measures in Healthy Young Adults with Self-reported Normal Hearing.Journal of the Association for Research in Otolaryngology : JARO · 2023Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
6 authors.
Funding
Abstract
Auditory neuropathy spectrum disorder (ANSD) refers to a range of hearing impairments characterized by an impaired transmission of sound from the cochlea to the brain. This defect can be due to a lesion or defect in the inner hair cell (IHC), IHC ribbon synapse (e.g., pre-synaptic release of glutamate), postsynaptic terminals of the spiral ganglion neurons, or demyelination and axonal loss within the auditory nerve. To date, the only clinical treatment options for ANSD are hearing aids and cochlear implantation. However, despite the advances in hearing-aid and cochlear-implant technologies, the quality of perceived sound still cannot match that of the normal ear. Recent advanced genetic diagnostics and clinical audiology made it possible to identify the precise site of a lesion and to characterize the specific disease mechanisms of ANSD, thus bringing renewed hope to the treatment or prevention of auditory neurodegeneration. Moreover, genetic routes involving the replacement or corrective editing of mutant sequences or defected genes to repair damaged cells for the future restoration of hearing in deaf people are showing promise. In this review, we provide an update on recent discoveries in the molecular pathophysiology of genetic lesions, auditory synaptopathy and neuropathy, and gene-therapy research towards hearing restoration in rodent models and in clinical trials.
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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.