Aminoglycoside antibiotics are essential for treating some severe bacterial infections but are notorious for causing irreversible hearing loss in 20%-47% of patients.
Family with sequence similarity 19 member A5 (FAM19A5) is a secretory protein highly expressed in the brain that regulates synapse dynamics through its interaction with leucine-rich repeat-containing 4B (LRRC4B). Noise exposure is one of the most significant causes of synapse loss between inner hair cells and auditory nerve fibers, leading to a decline in hearing sensitivity.
The most common form of hereditary deafness in humans is caused by mutations in the GJB2 gene, which encodes the gap junction protein connexin 26. That regulates the transport of potassium and metabolites between inner ear cells. The coding sequence of this gene fits in an adenovirus-associated vector (AAV), making it an attractive approach for gene therapy.
Primemupharma Co. Ltd. has divulged compounds comprising a novel polyester linker acting as antioxidants reported to be useful for the treatment of hearing loss and Meniere’s disease.
More than half of all hearing loss cases are hereditary. Myelin protein zero-like 2, encoded by MPZL2, is widely expressed in cochlear cells in the inner ear. Mutations in MPZL2 have been identified as the second most prevalent cause of mild to moderate hereditary hearing loss.
Piplartine, also called piperlongumine, had protective effects against hearing loss induced by aminoglycoside antibiotics in zebrafish and mouse models of kanamycin-induced hair cell loss. Kanamycin is a member of the aminoglycosides, a group of antibiotics that have broad-spectrum activity against gram-negative bacteria, including multidrug resistant strains.
A spontaneous homozygous deletion affecting the copy number of the cadherin 23 (Cdh23) gene in a line of laboratory mice gave rise to a new murine model with hereditary hearing loss and vestibular alterations.
Patients with congenital hearing loss could benefit from a gene therapy currently in development. Although there are approaches that could reverse the process in children and young people before it becomes severe, so far, adults do not have any treatment that prevents the progressive deterioration of auditory sensory cells caused by this disease.
It is known that >70% of the genes involved in pathogenic hearing loss are involved in autosomal recessive nonsyndromic hearing loss (ARNSHL), which accounts for 1 case for every 166 individuals in Iran due to the high rate of consanguineous marriage in the country.
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