Objective: To describe the experience of auditory brainstem implantation (ABI) in patients with Neurofibromatosis type 2 (NF2). Results: There were 50 primary ABI insertions in 49 patients, including 16 inserted at the time of first side tumor removal as a sleeper, and two revision repositionings which failed to improve outcome. Postoperatively three patients had cerebrospinal fluid leaks which did not require reoperation, one patient had meningitis, and eight patients suffered either temporary or permanent lower cranial nerve dysfunction. Twenty-nine patients became full time users; a further 12 patients became non-users. Three patients died while their device was inactive. Five patients retain serviceable contralateral hearing. Audiological open set testing of users showed means of: environmental sounds discrimination 51%; phoneme discrimination: with ABI alone 22%/lip reading (LR) 45%/ABI with LR 65%; sentence testing: with ABI alone 13%/LR 19%/ABI with LR 54%. Conclusions: The majority of patients with NF2 implanted with an ABI find the device a useful aid to communication in conjunction with LR and in recognizing common environmental sounds. A small proportion gain open set discrimination. Almost a third of patients may end up as non-users. There is probably an increased risk of postoperative lower cranial nerve dysfunction so careful preoperative assessment is advised.Key Words: Auditory brainstem implantBilateral deafness-Postoperative complications-SurgeryVestibular schwannoma.Otol Neurotol 37:xxx-xxx, 2016.The auditory brainstem implant (ABI) evolved from cochlear implant technology to rehabilitate those individuals with total bilateral deafness who are unsuitable for cochlear implantation because of non-functional cochlear nerves or unimplantable cochleas. The majority of candidates suffer from the genetic disorder neurofibromatosis type 2 (NF2) which causes bilateral vestibular schwannomas leading to deafness either by direct damage from the tumors themselves or from the surgery to remove them (1). The ABI stimulates the cochlear nucleus complex directly, which is readily accessible after vestibular schwannoma removal.The Manchester programme participated in the original European pilot study (2), and since then has routinely offered it to appropriate patients. This article reviews our experience in NF2 patients.
MATERIAL AND METHODS
PatientsPatients were included in the study if they met the Manchester criteria for NF2 disease (3), and had been implanted with an ABI and had continued rehabilitation by the Manchester team between 1994 and 2009.