Signs and Symptoms of NF2:
To be diagnosed with NF2, one must have either:
1. evidence of bilateral internal auditory canal masses(vestibular schwannomas) or
2. a first degree relative with NF2 and either
a unilateral eighth nerve mass or two of the following:
schwannoma
meningioma
ependymoma
neurofibroma
juvenile posterior subscapular lenticular opacity
(a type of cataract)
Disfunction of the eighth cranial nerve:
hearing loss
tinnitus (ringing in the ears)
balance problems
Vestibular Schwannomas
Small skin schwannoms
Treatments:
surgery to remove acoustic tumors, results in deafness
radiation
surgery to remove other tumors with risk of regrowth and
damage to involved nerves
Neurofibromatosis 2 (NF2):
also known as Bilateral Acoustic NF (BAN)
Occurring in 1:30,000 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.
NF2 is caused by a mutation on the 22nd chromosome. This defect reduces the body's ability to produce merlin.
Merlin is one of a number of chemicals in the body that helps to supress tumor growth.
