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Published on: October 11, 2024
Neurofibromatosis type 2: Hearing preservation and rehabilitation
H Jia1, M M El El Sayed2, M Smail3
1Inserm, UMRS 1159, "unité de réhabilitation chirurgicale mini-invasive robotisée de l'audition", Sorbonne Universités, université Pierre et Marie-Curie Paris 6, 75013 Paris, France; Service d'ORL, otologie, implants auditifs et chirurgie de la base du crâne, GHU Pitié Salpêtrière, AP-HP, 75651 Paris, France; Department of Otolaryngology Head and Neck Surgery, Shanghai Ninth People's Hospital, Shanghai Jiaotong University School of Medicine, 200011 Shanghai, PR China; Sino-French Joint Clinical Research Center of Otology and Neuro-otology, 200011 Shanghai, PR China.
Abstract:
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.
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