Neurofibromatosis type 2 (NF 2) typically results in deafness due to disruption of the cochlear nerves, making peripheral devices such as cochlear implants ineffective. Auditory brainstem implants (ABIs), for direct electrical stimulation of the cochlear nucleus, have been used to provide auditory stimulation in this group of patients. Currently, 141 patients have been implanted in our institution, most recently using an advanced multichannel device. We report results of a recent series of 86 patients who received ABIs. Of this group, 60 had successful implantation, recovered from surgery, responded successfully to stimulation and underwent a full course of device programming and audiologic testing. This group had significant improvement in scores on several audiologic tests compared to baseline. When used to augment lip reading, improvement was also seen. The degree of improvement varied considerably among patients. ABI is a useful device for deaf patients with NF 2. As measured by audiologic testing, many patients receive substantial benefit with regard to sound and speech comprehension.

Keywords:
Neurofibromatosis type 2, Auditory brainstem implant, Acoustic neuroma, Deafness
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© 2003 S. Karger AG, Basel
2004
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