Brainstem Implant Can Preserve Hearing in Neurofibromatosis Type 2 Patients

According to a story from Newswise, neurosurgeons at UC San Diego Health can now provide patients with an auditory brainstem implant (ABI) that can preserve some degree of hearing in people with neurofibromatosis type 2. Usually, people with this disorder can completely lose their hearing because of tumors called acoustic neuromas that appear. The ABI allows patients to detect some environmental sounds, such as a door closing or a phone ringing.
Neurofibromatosis type 2 (NF2) is a genetic mutation that can be an inherited or may also appear spontaneously. The main symptom is the development of acoustic neuroma tumors, which can eventually lead to deafness but can also cause problems with vision such as eye lesions. Neurofibromatisis type 2 can be difficult to diagnose, and many patients experience hearing loss for nearly a decade before receiving an accurate diagnosis. Early surgical intervention can help preserve long term hearing, but in more severe cases, the tumors will recur even after surgery. Patients often begin to lose their hearing after already learning how to communicate with speech. It is recommended that patients learn sign language or use hearing assistive technology. To learn more about neurofibromatosis type 2, click here.

The ABI has the potential to have a major impact on quality of life for people with NF2. In some cases, the combination of the ABI supplemented by lip reading can even allow patients to retain a useful understanding of spoken language. The device is especially useful for those patients that are not capable of benefiting from more traditional hearing aids. Many hearing aids are not effective if hearing loss is due to nerve damage. The implant is recommended for patients that have already lost most of their hearing or whose tumors have grown so large that the surgery required to remove them would not allow for hearing ability to be saved.

Any patients aged 12 or older are eligible for the ABI. A sound processor attached to the ear picks up sounds with a mic and converts them into electrical signals that are sent directly to the brainstem. The caregiver and the patient learn to adjust the device over the course of a number of appointments as the brain adapts in order to interpret the signals.

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