She also reported having bilateral tinnitus, chronic dizziness, and right-sided facial paresthesia.īecause the ABI is placed on the brainstem, a thorough head and neck examination is important to evaluate the cranial nerves, which includes a fiberoptic laryngoscopy to evaluate vocal cord mobility. In 2015, she received CyberKnife radiosurgery for right-sided tumor growth and developed progressive HL. Surveillance imaging in 2010 showed a new right VS, which was followed over time. Although her tumor had remained stable in size, she developed profound HL on the left side after radiation. In 1996, she underwent a subtotal resection and Gamma Knife radiosurgery for a left VS at an outside institution. CASE REPORTĪ 64-year-old woman with a history of NF2 was referred to our clinics for VS surgery and ABI placement. In this article, we describe our institution's ABI process and the experience and hearing outcomes of the first implanted patient. Thus far, we have implanted three NF2 patients with an ABI. Other specialists include anesthesiologists, neuro-monitoring specialists, implant company representatives, neurointensivists, audio-verbal therapists, psychologists, and social workers. The multidisciplinary team consists of: (1) neuro-otologists and skull base neurosurgeons who remove the VS and place the ABI, (2) an electrophysiologist who verifies placement of the ABI by reading electrically evoked auditory brainstem responses (eABR), and (3) audiologists who evaluate and counsel patients pre-operatively, help confirm ABI placement, program the ABI, and evaluate hearing outcomes. Neurofibromatosis, audiology, surgery.īecause of a growing need to provide NF2 patients with better hearing options, the University of Miami (UM) developed a comprehensive ABI program in 2019. Test scores using lipreading and auditory brainstem implant (ABI) were higher than those with ABI or lipreading alone. Testing was performed in quiet using monitored live voice in Spanish. 3 The ABI is FDA-approved to improve the hearing of NF2 patients 12 years of age and older. When the ABI electrodes stimulate the cochlear nucleus, information is transmitted to the auditory cortex that will allow NF2 patients to hear. The auditory brainstem implant (ABI) is a surgically implantable device that delivers electrical stimulation to the cochlear nucleus of the brainstem, bypassing the inner ear and cochlear nerves. 2 When the cochlear nerves are severely damaged, hearing aids and cochlear implants (CI) may not provide patients with useful hearing. Hearing loss (HL) in this patient population can occur from the tumor itself or a result of treatment (i.e., microsurgical resection or radiosurgery). 1 In particular, NF2 patients develop bilateral vestibular schwannomas (VS), which are benign intracranial neoplasms that affect the cochleovestibular nerve. This rare condition affects approximately one in 25,000 individuals. Neurofibromatosis type 2 (NF2) is a hereditary tumor disorder that predisposes patients to develop multiple cranial and spinal tumors.
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