AK-OTOF for treating sensorineural hearing loss

AK-OTOF is a gene therapy in development for the treatment of sensorineural hearing loss (SNHL) due to otoferlin (OTOF) gene mutations. SNHL is the rapid loss of hearing in the ear. It is caused by damage to the cells in the inner ear, or by damage to the hearing nerves. OTOF is a protein essential for sending sound signals from the ear to the brain. People missing the OTOF gene typically have serious hearing loss. Common causes of SNHL include exposure to loud noises, genetic factors, or the natural aging process. Symptoms include noticeable hearing loss for most sounds, typically in one ear and distortion of sounds in the poorer hearing ear. SNHL cannot be cured, though it can be managed in ways that can address its symptoms and improve quality of life. SNHL can interfere with the individual’s ability to communicate if not properly managed. The main treatment for OTOF-related hearing loss is surgical introduction of an electronic implant called a cochlear implant which improves, but does not fully restore, hearing.