What is neurofibromatosis type 2 (NF2)?

Neurofibromatosis type 2 (NF2) is a rare inherited genetic condition. It is caused by a change (mutation) in the NF2 gene on chromosome 22, which normally helps stop tumours from forming. People with NF2 develop multiple, usually benign (non-cancerous) tumours of the nervous system. The hallmark is vestibular schwannomas on both hearing-and-balance nerves (bilateral acoustic neuromas). Meningiomas, spinal schwannomas, and certain eye changes are also common. NF2 is different from the more common neurofibromatosis type 1 (NF1) — they are separate conditions caused by different genes. NF2 affects roughly 1 in 25,000 to 1 in 33,000 people (source: EANO / published estimates).

Are NF2 brain tumours cancer?

In the vast majority of cases, no. The tumours seen in NF2 — vestibular schwannomas (acoustic neuromas) and meningiomas — are almost always benign. They do not spread to other organs the way cancer does. The problem in NF2 is not spread — it is that many tumours grow in tight, delicate spaces around the hearing nerves, brainstem, and spinal cord, and can press on important structures. This is why NF2 is managed by a specialist team over many years rather than with the aggressive chemotherapy used for malignant cancers. Malignant transformation is rare.

What is the first sign of NF2?

For most adults, the first sign is gradual hearing loss in one ear, often with ringing (tinnitus) or a feeling of imbalance. This reflects a slow-growing vestibular schwannoma on the hearing-and-balance nerve. Because it comes on slowly, people may notice it as trouble on the phone or difficulty following conversations in noise. In younger patients the first clue may instead be a cataract-type eye change or a skin/spinal tumour found on a scan. One-sided hearing loss has many common, non-serious causes — but persistent, unexplained one-sided hearing loss should always be checked with a hearing test and, if needed, an MRI.

Is NF2 inherited? Will my children get it?

NF2 follows an autosomal dominant pattern. If one parent carries an NF2 mutation, each child has about a 50% chance of inheriting it. However, roughly half of all NF2 cases are new (de novo) mutations — meaning there is no family history and the change happened for the first time in that person. Some people are also mosaic (the mutation is in only some cells), which can affect how the condition shows up and the risk to children. Genetic counselling and testing give the clearest answer for your family. CION coordinates referral to accredited genetics services for confirmation and family planning support.

How are NF2 acoustic neuromas and meningiomas treated?

Treatment is highly individual and led by a specialist team. Many small, slow-growing NF2 tumours are safely monitored with regular MRI (active surveillance) rather than treated immediately. When a tumour grows or causes symptoms, options include stereotactic radiosurgery / focused radiation and, when surgery is needed, neurosurgery coordinated with accredited neurosurgical partners — with a strong focus on preserving hearing and facial-nerve function. For growing vestibular schwannomas, an anti-angiogenic (VEGF-blocking) drug class is sometimes used to slow growth and protect hearing. Hearing rehabilitation (including hearing devices) is planned alongside. CION delivers the radiation, medical/systemic therapy, imaging, and supportive care, and coordinates surgery with neurosurgical partners.

Can NF2 hearing loss be prevented or reversed?

Hearing loss in NF2 cannot always be prevented, but early, planned care gives the best chance of protecting hearing for as long as possible. Timely surveillance means tumours are found while still small, when hearing-preservation strategies — careful monitoring, focused radiation, or hearing-sparing surgery — are most likely to succeed. When natural hearing is lost, hearing rehabilitation options can help, and these are discussed as part of your long-term plan. There are no guarantees, and we will always be honest with you about what is realistic for your specific tumours and hearing status.

Why does NF2 need a coordinated, long-term team?

NF2 is a lifelong condition where several tumours may need attention at different times. No single specialty can manage it alone. The right care blends neuro-radiology, radiation oncology, medical oncology, ENT/audiology, genetics, and neurosurgery. At CION, every NF2 patient is discussed by a multidisciplinary tumour board so that decisions balance tumour control against hearing, facial-nerve function, and quality of life. We deliver radiation, systemic therapy, imaging, and supportive care directly, and coordinate neurosurgery with accredited neurosurgical partners — so your care stays joined-up across every appointment.