Neurofibromatosis 2 (NF2) is a rare condition occurring in 1 in 40,000 live births. NF2 is characterized by multiple tumors on the cranial and spinal nerves, and by other lesions of the brain and spinal cord. Tumors affecting both of the auditory nerves are the hallmark. Hearing loss beginning in the teens or early twenties is generally the first symptom.
The early symptoms of NF2 are symptoms of dysfunction of the acoustic (hearing) nerve, which carries information about sound to the brain, and the vestibular nerve, which carries balance information to the brain. Consequently, hearing loss, ringing in the ears (called tinnitus) and problems with balance, beginning in the teens or early twenties, are generally the first symptoms of NF2.
Although tumors on the eighth cranial nerve are most common, persons with NF2 can develop tumors on other nerves as well.
Other symptoms of NF2 may include facial weakness, headache, change in vision, and a lump or swelling under the skin caused by the development of a neurofibroma. In a family member at risk for NF2, a positive diagnosis is suspect if mild signs of NF are found elsewhere, such a small lump under the scalp or skin.