Introduction
Neurofibromatosis is a genetic disorder of the nervous system. This disorder contains three different categories. Neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and Schwannomatosis. Each disorder has specific characteristics that set them apart. These disorders affect growth and nerve cell tissue because tumors grow on nerve tissue throughout the nervous system. Depending on what cells are involved will dictate which disorder is seen. Each disorder has a specific type of tumor associated with it. It is estimated the 100,000 Americans have a neurofibromatosis disorder and they occur in both sexes and in all races and ethnic groups (NINDS).
Disease Characteristics
Neurofibromatosis 1 is commonly known as von Recklinghaus
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If the disease is very severe then evaluations may be more frequent. MRI can reveal tumors of the vestibular nerve as small as a few millimeters in diameter. Surgical options depend on tumor size and if hearing loss will be inflicted. Damaging the auditory nerve is easier as the tumor increases. However sometimes a cochlear implant or an auditory brain stem implant can be implemented to recover hearing. Cataracts or retinal abnormalities are corrected through surgery as well.
Disease Characteristics
Schwannomatosis is a rare form of neurofibromatosis that is just recently being recognized. It rarely affects people before their 20s or 30s making it different from NF1 and NF2. Schwannomatosis causes painful tumors called schwannomas and they develop on cranial, spinal, and peripheral nerves, but not the eighth cranial nerve. Schwannomatosis is the development of multiple schwannomas occurring everywhere in the body except on the vestibular nerve. Therefore, schwannomatosis does not cause hearing loss, it mainly causes chronic pain, occurring anywhere in the body. The pain is caused when the schwannomas enlarge, compresses nerves, or presses on adjacent