This groundbreaking treatment addresses a rare form of genetic hearing loss caused by changes in the otoferlin (OTOF) gene. Children’s Wisconsin is also one of only four Activated Treatment Centers in the country approved to offer this innovative therapy for OTOF-related hearing loss.

This achievement highlights our commitment to bringing the latest treatments and innovations to the children and families we serve.

What is OTOF-related hearing loss?

OTOF-related hearing loss is a rare genetic condition caused by changes in the OTOF gene. This gene helps send sound signals from the inner ear to the brain. When the gene does not work properly, children can be born with severe hearing loss.

Until now, children with this condition primarily rely on cochlear implants or sign language.

Otarmeni is the first gene therapy for this condition to be approved by the FDA.

“Despite being a rare form of hearing loss, this condition is one that the pediatric audiologists at Children’s Wisconsin have extensive expertise in diagnosing and managing,” said Dr. Brittney Gniedziejko, Manager, Audiology and AMU CRU. “Together with our partners in otology at the Medical College of Wisconsin, we are now able to provide an exciting new treatment option for patients and families. This milestone showcases the extraordinary innovation and forward-thinking care that the Audiology team at Children’s Wisconsin brings to the field every day.”

How does Otarmeni work?

Otarmeni is a one-time surgical procedure that delivers a healthy copy of the OTOF gene directly to the inner ear. The therapy is designed to help restore the ear's ability to send sound signals to the brain. Unlike hearing aids or cochlear implants, which help compensate for hearing loss, Otarmeni aims to address the underlying genetic cause of hearing loss in children with OTOF-related deafness. It's an exciting new treatment that has the potential to help the ear function more naturally.

“This is a remarkable milestone for both our team and the families we serve. To be able to offer the first FDA-approved gene therapy for hearing loss—and to be the first center in the nation to perform this procedure—is a testament to the collaboration, expertise, and dedication of teams across Children's Wisconsin. I'm incredibly proud to be part of the team to make this possible and excited about what it means for children and families facing OTOF-related hearing loss,” said Dr. Michael Harris, Chief Otology & Neurotologic Skull Base Surgery, and co-surgeon on the procedure.

This achievement belongs to our incredible team members—from audiologists and surgeons to nurses, surgical staff and support teams—whose expertise, collaboration and dedication helped make this milestone possible. Special recognition goes to:

• Audiology: Brit Gniedziejko, AuD; Olivia Riemer, AuD; Hayden Koukios, AuD; Monica Barany, AuD; Megan Bertsche, AuD; Sarah Klajbor, AuD; Hilary Gazeley, AuD

• Circulating Nurse: Heather Filocha, RN

• Surgical Technologist: Jennifer Schmid

• Co-Surgeons: Karl W. Doerfer, MD; Michael S. Harris, MD

Thank you for helping Children’s Wisconsin lead the way in pediatric hearing care and innovation.

Wisconsin Academy of Audiology

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