[Management of Neurofibromatosis Type 2].

Abstract

Surgical and radiotherapeutic interventions for vestibular schwannomas in patients with neurofibromatosis type 2 (NF2) generally yield less favorable outcomes than in sporadic unilateral cases, often hindering hearing preservation. Managing NF2 entails a lifelong series of complex decisions, repeatedly weighing functional preservation against impairment and surveillance against invasive therapeutic interventions. For both patients and their physicians, a critical issue lies in how to maximize functional preservation while simultaneously securing long-term survival. Auditory reconstruction using implantable auditory devices should be considered in cases of severe bilateral hearing loss. In patients with preserved cochlear nerve integrity, cochlear implantation is effective, and treatment strategies should be planned with this option in mind. Clinical trials of bevacizumab for pharmacological treatment are currently ongoing in Japan, and the clinical application of anti-vascular endothelial growth factor receptor vaccine therapy is anticipated.