Neurofibromatosis Type-2 (NF-2) occurs in approximately 1 in 25,000 people. One distinguishing feature of NF-2 from NF-1 is tumor growth on the 8 th cranial nerve, which is called the vestibulocochlear nerve. This nerve plays a vital role in hearing and balance. Tumors that grow on the 8th cranial nerve are called schwannomas, because they are caused by an overgrowth of Schwann cells, which help nerve cells conduct information as well as protect the nerve cell.
To be diagnosed with NF-2, the Children’s Tumour Foundation (2016) states that an individual
MUST have ONE of the following:
1. Tumors noted on both of the vestibulocochlear nerves (8th cranial nerve) OR
2. Family history of NF-2 AND
a) Tumor noted on one of the vestibulocochlear nerves (8 th cranial nerve) in a person under the age of 30
b) Any two of the following: meningioma (tumor that grows in the membrane surrounding your brain and spinal cord), glioma (tumor that arises from glial tissue), schwannoma or juvenile/early onset cataracts
Hearing loss and issues with balance usually appear in the late teen to early adult years. Like NF-
1, treatment is usually aimed at controlling symptoms. Surgery can be done to remove tumors
growing on the vestibulocochlear nerve, but there is a risk of losing hearing completely during the
surgery. Chemotherapy and radiation can also be used in individuals with NF-2, but if no significant
symptoms/complications are present medical professionals typically monitor tumor growth using
scans such as MRI’s and CT scans.