WIKIMEDIA, HENRY GRAYMutations in the gene PJVK and resulting deficiencies in the gene’s protein product, pejvakin, impair inner ear cells’ ability to respond to oxidative stress brought about by noise, making them vulnerable to damage, according to a study published today (November 4) in Cell. The results present a new mechanism for the most common environmental cause of hearing loss—injury from noise.
The findings also offer opportunities to explore therapies for people affected by these mutations, study coauthor Christine Petit of the Institut Pasteur and the Collège de France told The Scientist. “I think what should be done immediately is propose the use of antioxidants,” she said, and look into developing a gene therapy to correct for the loss of pejvakin.
About a decade ago, Petit’s team identified the PJVK gene as being associated with deafness in certain families. It was another in a growing heap of genes associated with hearing loss, but this one was peculiar in that the clinical manifestations of its mutations were all over the map. Some individuals were profoundly deaf, while others had only a slight impairment. Some family members showed progressive ...
