Texas NF Logo
Donate Now

Texas Neurofibromatosis Foundation - 1

NEUROFIBROMATOSIS TYPE 2 (NF-2)

NF2 is less common than NF1. NF2 was formerly called central neurofibromatosis. Patients with NF2 may have only a few café au lait spots, light patches of skin and skin tumors. There will be tumors that affect hearing and balance called vestibular schwannoma, or acoustic neuroma. Vestibular schwannoma tumors get their name from their location and the types of cells in them that cause pressure damage to neighboring nerves. These tumors are located on the vestibular nerve, another branch of the eighth cranial nerve near the auditory nerve. Meningiomas and schwannomas, which occur in individuals with NF2, are tumors that push on the brain and spinal cord. Tumors are benign but can cause serious problems like weakness or seizures. While the problems can be frightening in people who face NF2, there are important advances in research that will give hope to both those with NF1 and NF2.


WHEN DO SYMPTOMS APPEAR WITH NF2?

For some people, the signs of NF2 are detectable in childhood, but for the majority, NF2 is not discovered until puberty or later. Hearing loss may be noticed in the teens. Changing in hearing may occur in one or both ears, or there may be other early symptoms that may include tinnitus (ringing noise in the ear) and poor balance. Pressure from the tumors may cause headaches, facial pain or facial numbness.


NF2 SYMPTOMS

  • meningioma (and other brain tumors)
  • cataracts (visual impairment/blindness)
  • tinnitus (ringing in the ears)
  • hearing loss (and/or deafness)
  • bilateral vestibular schwannomas (acoustic neuromas)
  • schwannomas of the peripheral nerves and skin tumors
  • spinal tumors
  • balance problems, dizziness, seizures, headaches and general muscle wasting can be associated with NF2

HOW IS NF2 DIAGNOSED?

In diagnosing NF2, a physician looks for two or more of the following:

  1. Bilateral eighth nerve tumors
  2. A parent, sibling or child with NF2 and a unilateral eighth nerve tumor
  3. A parent, sibling or child with NF2 and any two of the following:
    • glioma
    • meningioma
    • neurofibroma
    • schwannoma
    • cataracts at an early age

HOW IS NF2 TREATED?

Treatments for NF2 are also aimed at controlling symptoms. With improved technology, MRIs (magnetic resonance imaging) can detect tumors as small as a few millimeters in diameter, allowing for early treatment. Surgery is an option to remove tumors but may result in hearing loss. Some other options may include partial removal of tumors and radiation. Seizures can be treated with antiepileptic drugs.


MEDICAL EVALUATION AND FOLLOW-UP (NF2)

The first time the NF patient is seen by a specialist in NF, there are certain procedures that should be completed.

  1. A personal medical history
  2. A family history and pedigree construction
  3. A physical examination focusing on the skin, skeleton and nervous system
  4. An examination by an ophthalmologist familiar with NF2
  5. A CT scan or MRI of brain and spinal cord
  6. An audiogram with brainstem auditory-evoked responses and audiologist consultation

As with NF1, follow-up examinations should be done at least at yearly intervals. These follow-up exams should focus on those items found previously and on any items that are to be anticipated based on the patient's age.