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Neurofibromatosis type 2

Neurofibromatose de type 2

Neurofibromatosis type 2 (NF2) is a rare genetic disease, characterized by the almost systematic development of bilateral vestibular schwannomas, i.e., tumours located next to the auditory nerves that can lead to progressive or sudden hearing loss.

In half of the cases, patients with NF2 also harbour meningiomas, which are benign tumours of the meninges (coverings of the brain) and may also present with benign tumours compressing the spinal cord (meningiomas, schwannomas) that can lead to walking disorders or weakness in the arms or legs.

Symptoms vary from one individual to another, even within the same family, ranging from minor to more severe forms. The evolution of tumours and symptoms is not predictable, and therefore the possible treatment must be adapted on a case-by-case basis. Deafness can be improved by sophisticated equipment that sometimes requires care by specialised teams.

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