Central electrical stimulation of the auditory pathway in neurofibromatosis type 2.

The auditory brainstem implant (ABI) is a viable treatment option for patients with neurofibromatosis type 2 (NF2) whom either vestibular schwannomas or the surgery used to remove them has rendered totally deaf. This device stimulates the central auditory pathways in a manner similar to the cochlear implant in individuals with a total hearing loss. A multichannel model with transcutaneous signal transmission is in use experimentally in both the United States and Europe. Of 14 patients implanted with the ABI in a European pilot study, 13 received auditory sensations at initial tuning. Surgical implantation of the ABI is generally, but not always, performed at the same time as tumor removal, with the preferred route being the transmastoid-translabyrinthine approach. After insertion, monitoring of the device is required to optimize the location of its electrode on the cochlear nucleus complex.