Photo credit: Juan Pablo Serrano Arenas
by Daniel Fink, MD, Chair, The Quiet Coalition
The Science Alert website and multiple other news sources are reporting that an 11 year old boy born with congenital deafness is now able to hear, thanks to new gene therapy. The boy’s deafness was caused by a defective gene for producing a protein, otoferlin, needed for cochlear hair cells to send sound signals to the brain.
In October 2023, delicate surgery allowed a harmless carrier virus carrying the replacement gene to be inserted into the cochlear fluid. The gene infected the cochlear hair cells, and the boy’s ears began making the protein. In just four months, the boy’s hearing improved to the point where he now has only mild to moderate hearing loss. Unfortunately, speech develops before age 5 so the boy may never learn to speak normally.
Using gene therapy to restore hearing in the congenitally and profoundly deaf has been the “holy grail” of hearing loss therapy, according to researchers. This first successful use of gene therapy to restore hearing is a giant step forward. Unfortunately, gene therapy for any condition carries risks, is likely to be extremely expensive for the next several decades and is impractical for most people with hearing loss.
There are many causes of hearing loss — congenital deafness, ototoxic drugs, ear infections and meningitis, among others — but the most common cause of hearing loss is noise exposure. A recent review article from the National Institute of Health’s Library of Medicine estimates that 5% of the world’s population has noise-induced hearing loss. In the United States, about 25% of adults age 20-69 have noise-induced hearing loss, most without significant occupational noise exposure.
The Centers for Disease Control and Prevention states that noise-induced hearing loss is the only type of hearing loss that is 100% preventable. We like to say, “If it sounds loud, it’s too loud and your auditory health is at risk.” To prevent noise-induced hearing loss, avoid loud noise, turn down the volume, leave the noisy environment or use hearing protection, and your ears should last a lifetime.
