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Neurofibromatosis type 2
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Condition

Neurofibromatosis type 2

Neurofibromatosis type 2 is a genetic disorder characterized by the growth of noncancerous tumors, primarily vestibular schwannomas, in the nervous system, often leading to hearing loss and balance problems.

Overview

Neurofibromatosis type 2 is a disorder characterized by the growth of noncancerous tumors in the nervous system. The most common tumors associated with neurofibromatosis type 2 are called vestibular schwannomas. These growths develop along the nerve that carries information from the inner ear to the brain (the auditory nerve). Tumors that form on the membrane that covers the brain and spinal cord (meninges) are also common in neurofibromatosis type 2. These tumors are called meningiomas. Tumors can also occur on other nerves or tissues in the brain or spinal cord in people with this condition.

The signs and symptoms of neurofibromatosis type 2 usually appear during adolescence or in a person's early twenties, although they can begin at any age. The most frequent early symptoms of vestibular schwannomas are hearing loss, ringing in the ears (tinnitus), and problems with balance. Less commonly, vestibular schwannomas cause facial weakness or paralysis. In most cases, these tumors occur in both ears (bilaterally) by

Frequently Asked Questions

Diagnosis of Neurofibromatosis type 2 (NF2) typically involves a combination of clinical evaluation, imaging, and genetic testing. If early symptoms like hearing loss, tinnitus, or balance problems arise, an MRI of the brain and spinal cord is crucial to detect vestibular schwannomas and other tumors. Audiology tests to assess hearing and eye exams to check for cataracts or retinal abnormalities are also common. Genetic testing can confirm the diagnosis by identifying a mutation in the NF2 gene.
Management of hearing loss and balance problems in NF2 depends on the size and growth of the vestibular schwannomas. Treatment options include watchful waiting with regular MRI scans, surgical removal of the tumors, or radiation therapy (stereotactic radiosurgery). For hearing loss, cochlear implants or auditory brainstem implants may be considered in some cases to restore hearing. Physical therapy can help manage balance issues.
Yes, Neurofibromatosis type 2 is an inherited genetic condition. It is passed down in an autosomal dominant pattern, meaning only one copy of the altered gene is needed to cause the disorder. About half of cases are inherited from an affected parent, while the other half result from new gene mutations. For family planning, genetic counseling is highly recommended to understand the risks of passing the condition to children and to discuss options like preimplantation genetic diagnosis.
Individuals with NF2 require lifelong, multidisciplinary monitoring. This typically includes annual or biannual MRI scans of the brain and spine to track tumor growth, regular audiology evaluations to monitor hearing, and ophthalmology exams to check for eye-related complications. Neurological assessments are also crucial to monitor for any new symptoms or progression of existing ones.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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