Neurofibromatosis type 2: Hearing preservation and rehabilitation.

The main manifestation of neurofibromatosis type 2 (NF2) is the development of bilateral vestibular schwannomas (VS). Consequently, one of the most severe functional sequelae is bilateral sensorineural hearing loss, caused by spontaneous tumor progression and/or treatment-related damage (surgery or radiosurgery). Preserving or restoring hearing is still challenging in NF2 no matter the strategy applied to each individual based on the natural history of VS. In this review, the different strategies for hearing preservation or rehabilitation are discussed and illustrated by several cases. A decisional algorithm for NF2 patients with VS is proposed that takes into consideration the tumor size and hearing level.