A New Hope for Childhood Deafness

Cassidy DeMasi

Dr. Barbato

12/7/2025

                                                      A New Hope for Childhood Deafness

The OTOF gene allows the inner ear send sound signals to the brain. We first learn about whether this gene is functioning appropriately when a child is first born, because we learn that they fail their newborn hearing test. This is because when this gene has a mutation, the ear can hear sound but can’t send the signal correctly. This causes auditory neuropathy. It is typically inherited from both parents and is often best treated with a cochlear implant. However, cochlear implants require surgery, and the last thing that a parent wants their young kid to go through is surgery. 

However, there is a new genetic treatment on the market presented by the University of Cincinnati. The study states, "The treatment, which targets a mutation in the otoferlin (OTOF) gene, has been effective in most of the tested children. The rare mutation causes hearing loss in an estimated 20 to 50 babies born in the U.S. each year. Deafness linked to OTOF mutations is a type of auditory neuropathy, a spectrum of disorders in which the inner ear can detect sound but struggles sending that information to the brain," (Burgasser, 2025) This discovery brings new hope to families that just want their children to hear their voice for the first time. It also contributes to discoveries in new findings for potentially curing genetic deafness.

Resources 

Burgasser, M. (2025, December 8). The gene therapy poised to rewrite childhood deafness. UC News. https://www.uc.edu/news/articles/2025/12/the-gene-therapy-poised-to-rewrite-childhood-deafness.html

OTOF otoferlin [Homo sapiens (human)] - Gene - NCBI. (n.d.). Www.ncbi.nlm.nih.gov. https://www.ncbi.nlm.nih.gov/gene/9381