Hearing restoration with auditory brainstem implants after radiosurgery for neurofibromatosis type 2

The auditory brainstem implant (ABI) is designed to restore useful auditory sensations in patients with neurofibromatosis Type 2 (NF2). The implantation is usually performed at the time of tumor removal in patients who do not undergo radiation treatment. The authors evaluated the performance of ABIs in three patients with NF2 in whom vestibular schwannoma continued to grow after radiation treatment. These three patients with NF2 received a 21-channel ABI; a translabyrinthine approach was used for both the tumor removal and the ABI placement. The interval between radiosurgery and the tumor removal plus device implantation ranged from 2 to 11 years. In all cases, the tumor was growing and the patients presented with total deafness. The mean number of active electrodes in these three patients was equivalent to the average results reported in other patients who received ABIs. The patients in this study used the ABI regularly for everyday life and obtained useful levels of environmental sound recognition. It is concluded that hearing function can be rehabilitated using ABIs in patients with NF2, even if radiosurgery fails to control the tumor growth.