Neurofibromatosis type 2 (NF2) is a hereditary condition most commonly associated with bilateral vestibular schwannomas (also known as acoustic neuromas). These are benign (noncancerous) tumors that occur on the nerves for balance leading to the inner ear. Although these tumors are benign, they can cause hearing and balance problems.
People with NF2 also have an increased risk of other tumors of the nervous system. These tumors are generally not cancerous, but they may still lead to significant medical problems, especially if there are multiple tumors in or next to the brain. Other nervous system tumor types include:
- Schwannomas of other nerves
- Meningiomas
- Gliomas (including Ependymoma of the brain or spinal cord)
People with NF2 are at an increased risk of developing cataracts in the eyes and benign skin tumors. They may have café-au-lait spots (light brown pigmentation, like the color of "coffee with milk") like individuals with NF1, but people with NF2 usually have fewer café-au-lait spots than people with NF1.
Signs of NF2 usually develop in the late teenage years or early 20s. Multiple features have been associated with NF2, but the overall cancer risk is low. The number of features present and the severity of symptoms can vary among people with NF2, even within the same family.