page_title: Neurofibromatosis type II
section_title: Pathology
hierarchical_section_title: Neurofibromatosis type II / Pathogenesis / Pathology
caption_attribution_description: Deutsch: Winziges de:Schwannom des rechten de:Nervus vestibularis, inkorrekte aber verbreitete Bezeichnung: de:Akustikusneurinom, im inneren Gehörgang rechts (im Bild links, mit Pfeil markiert). Transversale T1-gewichtete de:Kernspintomographie nach Applikation von Kontrastmittel. Tumoren dieser Größe sind ausschließlich mit der Kernspintomographie nachweisbar.
context_page_description: Neurofibromatosis type II is a genetic condition which may be inherited or may arise spontaneously. The main manifestation of the condition is the development of bilateral benign brain tumors in the nerve sheath of the cranial nerve VIII, which is the "auditory-vestibular nerve" that transmits sensory information from the inner ear to the brain. Besides, other benign brain and spinal tumors occur. Symptoms depend on the presence, localisation and growth of the tumor. Many people with this condition also experience visual problems. Neurofibromatosis type II is caused by mutations of the "Merlin" gene, which seems to influence the form and movement of cells. The principal treatments consist of neurosurgical removal of the tumors and surgical treatment of the eye lesions. Historically the underlying disorder has not had any therapy due to the cell function caused by the genetic mutation.
context_section_description: The so-called acoustic neuroma of NF2 is in fact a schwannoma of the nervus vestibularis, or vestibular schwannoma. The misnomer of acoustic neuroma is still often used. The vestibular schwannomas grow slowly at the inner entrance of the internal auditory meatus (meatus acousticus internus). They derive from the nerve sheaths of the upper part of the nervus vestibularis in the region between the central and peripheral myelin (Obersteiner-Redlich-Zone) within the area of the porus acousticus, 1 cm from the brainstem.
caption: Schwannoma of the N. Vestibularis
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