Neurofibromatosis type 2 is a genetic disease. It is a non-cancer tumor in the nervous system most commonly located along the auditory nerve that allows for hearing. Teens and adults with neurofibromatosis type 2, have been able to receive Auditory brainstem implants (ABI’s) for more than 30 years. As of November 2015, about 25 children have obtained an ABI in the United States. Many of these were implanted as part of Federal Drug Administration (FDA) approved clinical trials over the past few years.
Children below the age of 12 and without neurofibromatosis type 2, like Colton, may also receive this surgically implanted electronic device. These patients get an off label device. This off label device is called a Humanitarian Use Device. The intent is to treat or diagnose a disease or condition that affects less than 4,000 people in the U.S. each year, according to the FDA.For more information about off-label device use, visit fda.gov.