Neurofibromatosis Type 2 (NF2)

Neurofibromatosis type 2 (NF2) is a genetic disorder that causes noncancerous tumors to grow in the nervous system.

There are two additional types of neurofibromatosis:

• Neurofibromatosis type 1 (NF1)
• Schwannomatosis

Neurofibromatosis Type 2 (NF2) Symptoms

NF2 symptoms can appear at any age, but they most often begin between the ages of 18 and 22. NF2 symptoms can include the following:

• Hearing loss
• Ringing in the ears (tinnitus)
• Loss of balance
• Vertigo (dizziness)
• Vision loss
• Arm or leg weakness
• Seizures
• Skin tumors

Neurofibromatosis Type 2 (NF2) Diagnosis at Rush

Rush specialists in ENT and neurology and neurosurgery diagnose and treat NF2. To make a diagnosis, they look for the following:

• Vestibular schwannoma in both ears or
• A family history of NF2 plus a vestibular schwannoma in one ear before age 30 or
• Any two of the following:
  • Glioma, a tumor that begins in the brain or spine
  • Meningioma, a tumor that starts in the membranes surrounding the brain or spine
  • Schwannoma, a tumor on the outside of a nerve sheath
  • Cataracts on the lens of one or both eyes (in children and young adults)

If Rush providers suspect you have NF2, they may perform the following tests:

• Hearing tests
• Vision tests
• Magnetic resonance imaging (MRI) to look for tumors
• Genetic testing to find a change in the gene that causes NF2

Neurofibromatosis Type 2 (NF2) Treatment at Rush

Otolaryngologists, neurotologists and neurosurgeons at Rush collaborate to treat NF2. Every case of NF2 is different, so your treatment will be tailored to meet your needs.

Your plan may involve one or more of the following:

Addressing Complications of NF2

• Our treatment goal is to prevent (when possible) complications from NF2 and address them when they exist.
• You will visit with your provider on a regular basis to monitor your symptoms; together, we will determine when they need to be addressed.
• Complications of NF2 can include the following:
  • Deafness
  • Severe balance problems
  • Facial nerve paralysis
  • Spinal cord compression
  • Swallowing difficulties (dysphagia)
  • Vision problems

Surgery

• You may need surgery to remove vestibular schwannoma (also known as acoustic neuroma) or other tumors.
• The timing and type of surgery used for NF2 varies depending on your symptoms and how quickly they progress.
• Based on monitoring your symptoms, we will discuss with you whether surgery is needed, and, if so, which type.

Radiation Therapy

• Your providers may recommend radiation therapy to shrink tumors. This decision will depend on where your tumors are and how much they affect your quality of life.

Rush Excellence in Neurofibromatosis Type 2

• Acoustic neuroma expertise: Hearing loss and balance problems (two common NF2 symptoms) are caused by a noncancerous, slow-growing tumor called a vestibular schwannoma. Vestibular schwannoma (also known as acoustic neuroma) grows on the nerve that transmits sound and balance sensations from the inner ear to the brain. Acoustic neuroma providers at Rush work together to help you address the complex issues arising from the location of these tumors.
• Coordinated care: Depending on your symptoms and/or complications of NF2, you may need care from other specialists at Rush. Your doctors will work together to coordinate every aspect of your care so you have a seamless experience.
• Nationally recognized expertise: Neurology and neurosurgery and ear, nose and throat services at Rush University Medical Center are consistently ranked among the best in the nation by U.S. News & World Report.