A rare genetic disease called neurofibromatosis 2 affects the inner ear and eventually leads to complete deafness. The condition causes tumours to form in the person’s hearing nerves, so they slowly lose their hearing. Cochlear implants cannot help them hear again and are also not an option for people who lose their hearing from other rare inner ear abnormalities.

An auditory brainstem implant is the only treatment that may help people who have become deaf due to these conditions. Electrodes are surgically implanted into the base of the person’s brain. An ear-piece worn by the person picks up sounds and sends them to the implant. This does not restore normal hearing but may allow people to hear some sounds and to recognize speech.

Ontario Health looked at how safe and effective auditory brainstem implants are for adults with neurofibromatosis 2 or severe inner ear abnormalities who cannot use a cochlear implant. We also looked at the budget impact of publicly funding these devices in Ontario, and at the experiences, preferences, and values of adults with these two conditions.