Introduction to Acoustic Neuromas

Neurofibromatosis-2 and Acoustic Neuromas

Neurofibromatosis-2 (NF-2) is an inherited autosomal dominant condition which affects approximately 1 in 40,000 people and is caused by a mutation in the NF2 gene located on chromosome 22. It is associated with:

• Multiple inherited schwannomas
• Meningiomas - tumors that grow from the meninges (dural membrane covering the brain and spinal cord)
• Ependymomas - tumors that line the passageways in the brain where cerebrospinal fluid is produced and stored
• Ocular (eye) abnormalities - including cataracts

Symptoms of acoustic neuroma in patients with NF-2 typically appear in the teenage years and early adulthood. Bilateral presence of acoustic neuromas is a principle feature of NF-2. It is estimated that approximately 5% of people with this condition develop acoustic neuromas. Approximately 2-4% of all acoustic neuromas are considered to be associated with NF-2.