Definition:
Neurofibromatosis type 2 is an inherited disorder. It is also known as MISME Syndrome (Multiple Inherited Schwannomas, Meningiomas, and Ependymomas). This disease is characterized by the development of symmetric, benign tumors in the portion of the auditory-vestibular nerve. This nerve is the one responsible for conveying sensory information coming from the inner ear to the brain.
Treatment:
The most effective treatment is neurosurgical removal of the tumor; Chemotherapy and radiation therapy may also be necessary for the treatment of Neurofibromatosis type 2, however early detection and diagnosis can be helpful in providing immediate therapy for patients with this disorder.
Surgical treatment of the eye lesion is also effective in case there is occurrence concerning the eye.
Symptoms and Signs:
Symptoms of Neurofibromatosis type 2 include the following:
* Neurofibromas
* Tinnitus or ringing sensation in the ear
* Loss of hearing
* Poor balance
* Headache
* Facial pain
* Numbness in the face
* Swallowing difficulties
* Speech defects
* Eye movement difficulties
* Axillary or inguinal freckles
* Thinning of long bone cortex
* Large head
* Spinal curvature
* Cataract or opacity of the lens of the eye
* Meningiomas
Causes:
Neurofibromatosis type 2 is an autosomal dominant inherited disorder. It is caused by mutations of the Merlin gene in chromosome 22.
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