Decision-making in neurofibromatosis type 2

Abstract

Defined by the presence of bilateral vestibular schwannomas (VS) in the great majority of cases, NF2 is a complex genetic disease with unpredictable behavior. Since the 1980s, VS surgery outcomes have dramatically improved, and large VS were surgically removed to save lives or prevent disabling sequelae as well as small-/mid-sized tumors to avoid functional adverse events such as facial paralysis and deafness. Then, rehabilitation of hearing could be achieved by either auditory brainstem or cochlear implantations. However, new technologies such as radiosurgery have shown that conservative treatments could be applied to NF2-related VS, and more recently, chemotherapy has demonstrated that VS growth could be stopped and hearing preserved for some time at least. Today, the management of NF2-related VS should be tailored in function not only to the VS size and behavior and/or hearing level and evolution but also to the genetic status, tumor burden, symptom-induced disabilities, and the patient's feelings to preserve as long as possible his/her quality of life.

Keywords: Auditory brainstem implant; Chemotherapy; Cochlear implant; Microsurgery; Neurofibromatosis type 2; Radiosurgery; Vestibular schwannomas.