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Vestibular Schwannoma (Acoustic Neuroma) and Neurofibromatosis

What is a vestibular schwannoma (acoustic neuroma)?

A vestibular schwannoma (also referred to as 
acoustic neuroma, acoustic neurinoma, or acoustic neurilemoma) may be a benign, usually slow-growing tumor that develops from the balance and hearing nerves supplying the internal ear. The tumor comes from an overproduction of Schwann cells—the cells that normally wrap around nerve fibers like onion skin to assist support and insulate nerves. As the vestibular schwannoma grows, it affects the hearing and balance nerves, usually causing unilateral (one-sided) or asymmetric deafness, tinnitus (ringing within the ear), and dizziness/loss of balance. As the tumor grows, it can interfere with the face sensation nerve (the trigeminal nerve), causing facial numbness. Vestibular schwannomas also can affect the facial (for the muscles of the face) causing facial weakness or paralysis on the side of the tumor. If the tumor becomes large, it'll eventually press against nearby brain structures (such because the brainstem and therefore the cerebellum), becoming life-threatening.


How is a vestibular schwannoma diagnosed?

Unilateral/asymmetric deafness 
and/or tinnitus and loss of balance/dizziness are early signs of a vestibular schwannoma. Unfortunately, early detection of the tumor is usually difficult because the symptoms could also be subtle and should not appear within the beginning stages of growth. Also, hearing loss, dizziness, and tinnitus are common symptoms of the many middle and internal ear problems (the important point here is that unilateral or asymmetric symptoms are the worrisome ones). Once the symptoms appear, a radical ear examination and hearing and balance testing (audiogram, electronystagmography, auditory brainstem responses) are essential for correct diagnosis. Magnetic resonance imaging (MRI) scans are critical within the early detection of a vestibular schwannoma and are helpful in determining the situation and size of a tumor and in planning its microsurgical removal.


How is a vestibular schwannoma treated?

Early diagnosis of a vestibular schwannoma is vital 
to preventing its serious consequences. There are three options for managing a vestibular schwannoma: (1) surgical removal, (2) radiation, and (3) observation. Sometimes, the tumor is surgically removed (excised). The exact sort of operation done depends on the dimensions of the tumor and therefore the level of hearing within the affected ear. If the tumor is little, hearing could also be saved and accompanying symptoms may improve by removing it to stop its eventual effect on the hearing nerve. As the tumor grows larger, surgical removal is more complicated because the tumor may have damaged the nerves that control facial movement, hearing, and balance and should even have affected other nerves and structures of the brain.

The removal of tumors affecting the hearing, balance, or facial nerves can sometimes make the patient’s symptoms worse because these nerves may be injured during tumor removal.

As an alternate 
to standard surgical techniques, radiosurgery (that is, radiation therapy—the “gamma knife” or LINAC) could also be wont to reduce the dimensions or limit the expansion of the tumor. Radiation therapy is usually the well-liked option for elderly patients, patients in poor medical health, patients with bilateral vestibular schwannoma (tumor affecting both ears), or patients whose tumor is affecting their only hearing ear. When the tumor is little and not growing, it's going to be reasonable to “watch” the tumor for growth. MRI scans are used to carefully monitor the tumor for any growth.


What is being done about vestibular schwannoma?

Scientists continue studying the molecular pathways that control normal Schwann cell 
development to raise identify gene mutations that end in vestibular schwannomas. Scientists are working to raised understand how the gene works in order that they can begin to develop new therapies to regulate the overproduction of Schwann cells in individuals with vestibular schwannoma. Learning more about the way genes help control Schwann cell growth may help prevent other brain tumors. In addition, scientists are developing robotic technology to help physicians with acoustic neuroma surgery.



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