OTOF-related hearing loss is a rare genetic condition that affects how sound signals travel from the inner ear to the brain.

Children with this condition may be able to detect sounds in some situations, but their ears have difficulty sending those sounds to the brain clearly and consistently.

This condition:

• Is a rare genetic form of hearing loss.
• Often causes severe or profound hearing loss from birth.
• Accounts for approximately 2-4% of non-syndromic congenital deafness.
• Is usually inherited when both parents pass down a changed copy of the OTOF gene.

Not every child with hearing loss is a candidate for Otarmeni. A child may be considered for treatment if they:

• Have hearing loss caused by changes in both copies of the OTOF gene.
• Have severe to profound hearing loss.
• Show evidence that important inner-ear structures are functioning.
• Have typical inner-ear anatomy.
• Can safely undergo anesthesia and surgery.

It is important to note that children cannot receive Otarmeni gene therapy treatment in an ear that already has a cochlear implant. 

A Team of Experts: Children’s Wisconsin was one of the leading enrollment sites in the Otarmeni clinical trial and cared for more children receiving the investigational therapy than almost any other participating center.

Cutting-Edge Care: Children’s Wisconsin is one of only five Activated Treatment Centers in the United States approved to offer this treatment An Activated Treatment Center is a hospital that has been approved to evaluate patients and provide Otarmeni gene therapy.

Care Closer to Home: Families now have access to one of the most advanced treatments available for genetic hearing loss closer to home. Instead of traveling long distances for evaluation and treatment, eligible patients can receive care from a nationally recognized team at Children’s Wisconsin.