Vestibular schwannoma
A vestibular schwannoma often called an acoustic neuroma is a benign primary intracranial tumor of the myelin-forming cells of the vestibulocochlear nerve (8th cranial nerve). A type of schwannoma this tumor arises from the Schwann cells responsible for the myelin sheath that helps keep peripheral nerves insulated. Approximately 3000 cases are diagnosed each year in the United States with a prevalence of about 1 in 100000 worldwide.
Until recently the cause was unknown except for a small group of patients who have Neurofibromatosis type 2 which is genetically determined. Recent research has discovered the following:- Neurofibromatosis type 2 (NF-2) is a dominantly inherited disease characterized by the formation of bilateral acoustic schwannomas and other benign tumours associated with the central nervous system. The NF-2 protein, also known as merlin or schwannomin is a tumour-suppressor protein whose loss or absence is clearly linked to the development of certain types of brain tumours, in particular, acoustic neuromas.
“Acoustic Neuromas appear to be caused by a malfunctioning gene on chromosome 22. Normally, this gene produces a protein that helps control the growth of Schwann cells covering the nerves. What makes this gene malfunction isn’t clear”.- Links
- https://en.wikipedia.org/wiki/Vestibular schwannoma
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