Neurofibromas
The clinical similarity to neurofibromatosis type 1 arises when subcutaneous, i.e. in the subcutaneous fatty tissue, peripheral nerves are affected, which then impress like neurofibromas. Histologically, i.e. fine-tissue, there is no similarity. About half of those affected show café-au-lait spots. Rarely more than 3 spots appear.
Therapy
Since neurofibromatosis type 2 is also a genetic disease, a therapy to eliminate the cause is not possible. Therefore the therapy is based on the symptoms. By means of surgical procedures on the eye, it is now possible to replace cloudy lenses with artificial lenses.
In order to prevent progressive hearing loss, it is recommended that tumors of the auditory and balance nerve also be surgically removed at an early stage. Even if other cranial or spinal nerves are affected, surgery is recommended in order to preserve residual nerve function. However, such operations also involve risks.
Nerves can be damaged by the operation. In addition, tumors often return. Therefore, regular preventive and control examinations should take place.
It should also be remembered that benign tumors always carry a risk of malignant degeneration. If the hearing loss has progressed to deafness, one should use the possibilities of cochlear or brainstem implants. Electrodes are implanted in the inner ear or brain and the affected person can once again hear or participate in a wide range of communication.
