An Implant for Hearing Loss Due to Removal of Neurofibromatosis 2 Tumors

Penetrating Auditory Brainstem Implant for Neurofibromatosis 2

This is a study to gather some information on the safety and efficacy of the penetrating auditory brainstem implant (PABI) in patients with neurofibromatosis type 2.

Neurofibromatosis Type 2 (NF2) is characterized by multiple tumors of the cranial and spinal nerves, and, in particular, by bilateral acoustic tumors. Surgical resection of these tumors generally results in the transection of the VIIIth nerve, destruction of the cochlea and auditory nerve, and total hearing loss. Due to the loss of the auditory nerve, these patients are not candidates for cochlear implantation. The Auditory Brainstem Implant (ABI) provides the only available intervention to restore some auditory sensation to these patients. An ABI is an implantable prosthesis that produces sound sensation by electrical stimulation of the ascending auditory pathway at the level of the cochlear nucleus.

The PABI may be placed surgically during tumor resection. Efficacy will be assessed using psychophysical and standard speech reception measures.

Inclusion criteria:

• Diagnosis of neurofibromatosis type 2
• Speak English as a primary language

Exclusion criteria:

• Physical, psychological, or medical conditions that contraindicate the surgical procedure