Vestibular Schwannoma Specialists in New Jersey

Vestibular schwannomas are benign (noncancerous) tumors, but they can still cause a host of problems by pressing against nearby nerves. Vestibular schwannomas originate in the brainstem then grow into the inner ear, known as the internal auditory canal. They interfere with the ability to hear, balance, control facial muscles, and can become life-threatening if allowed to grow. Treatment from the right neurosurgical specialist is essential to avoid long-term nerve injury and other health consequences.

Centers for Neurosurgery, Spine & Orthopedics (CNSO) features a team of specialists that includes neurosurgeons, interventional pain management physicians, physiatrists, rehabilitation specialists, and certified physical therapists. This team provides surgical and non-surgical care for many conditions, including vestibular schwannoma, in New Jersey, helping patients to a full recovery.

What Is Vestibular Schwannoma?

A schwannoma is a benign type of tumor that forms from the cells that cover and protect the nerves, called Schwann cells. A vestibular schwannoma is a benign brain tumor that forms from the Schwann cells that cover and protect nerves that originate in the brainstem and extend into the internal auditory canal of the inner ear. The nerves that enter into the inner ear include the cochlear nerve (hearing), superior and inferior vestibular nerves (balance), and the facial nerve (facial motion and movement). The vast majority of vestibular schwannomas, over 90%, arise from the inferior vestibular nerve.

Vestibular schwannomas were originally called acoustic neuromas, due to the prevalence of hearing loss and the belief that the tumor actually arose from the nerve fibers (neuroma) of the cochlear (acoustic or hearing) nerve, hence “acoustic neuroma”. There are two distinct types of vestibular schwannomas based on their origin.

• Sporadic, unilateral vestibular schwannoma: This tumor grows on only one side of the body. It may develop at any age, and results from sporadic, nonhereditary mutation.
• Genetic, bilateral vestibular schwannoma: This tumor grows on both sides of the body and occurs only in people who have a genetic disorder called neurofibromatosis type 2.

Although vestibular schwannomas are benign tumors, meaning they grow slowly and do not spread to other tissues or parts of the body, benign does not mean harmless. Due to their location, vestibular schwannomas press against the nerves responsible for hearing, balance, and facial movement and function. Vestibular schwannomas typically grow very slowly, however, occasionally they begin to grow rapidly. As the schwannoma grows, it puts pressure on the brain, which can impact vital functions – potentially creating serious, even life-threatening, health complications.

Vestibular Schwannoma Symptoms

Symptoms of a vestibular schwannoma typically appear slowly, classically with subtly progressive one-sided hearing loss and tinnitus. During the early stages when the tumor is small, the signs are subtle and easy to miss. They develop and become more noticeable as the tumor grows. This growth puts increasing pressure on the nerves that process hearing and balance, resulting in neurological symptoms that worsen over time. These may include:

• Hearing loss: Patients with vestibular schwannoma will gradually lose their hearing over a period of months or years. The loss usually only occurs on one side.
• Ear fullness: Patients may experience a sensation of fullness in the ear, as if there is water or a blockage in it, even when the ear canal is empty.
• Tinnitus: Some patients may hear a tone in the affected ear. It may be intermittent or constant, quiet or loud, and may sound like hissing, buzzing, or roaring.
• Vertigo: Patients with schwannoma may feel a sense of dizziness, or unsteadiness as the tumor interferes with their sense of balance.

Large schwannomas sometimes compress the nerves that control facial muscles and sensation. When this occurs, the patient may experience additional symptoms, such as:

• Facial Weakness: Pressure on the facial nerve leads to weakness of one side of the face with asymmetric movement, which can appear similar to Bell’s palsy.
• Facial twitching: Pressure on the facial nerve often causes twitching, tics, or spasms in the eye, eyebrow, forehead, or mouth muscles.
• Numbness: A tumor that presses on the trigeminal nerve, which transmits sensory information from the face, may create numbness or a tingling sensation on one side of the face.
• Difficulty swallowing: The patient may have difficulty swallowing due to loss of sensation in the throat or loss of movement in the vocal cords or tongue.
• Hoarse voice: Due to a loss of movement of the vocal cords and tongue
• Changes in taste: Less commonly, vestibular schwannoma creates changes in taste perception. Food may be less flavorful or taste different.
• Dry eye: Changes in tear production are another rare symptom of schwannoma, resulting in dry, irritated eyes or sometimes unexpected tears.
• Headache: Very large schwannomas can press against the dura, which lines the inside of the skull. This creates a sense of pressure or a dull ache on one side of the head. A headache can also be due to increased intracranial pressure from a very large brain tumor compressing the fourth ventricle, one of the fluid passages, in the brain resulting in obstructive hydrocephalus. This is a life-threatening complication of a vestibular schwannoma.

These symptoms are not unique to patients with vestibular schwannomas and may be caused by other conditions. Individuals experiencing neurological symptoms should seek an evaluation and diagnosis from a medical neurological specialist such as a neurosurgeon. Accurately identifying the cause is necessary to determine an appropriate treatment.

Causes and Risk Factors for Vestibular Schwannoma

In most cases, there is no clear cause as to why a patient develops a vestibular schwannoma. These types of brain tumors are rare, occurring in fewer than 200,000 people. While they can occur at any age, including in children, they are most common in people ages 50 to 60.

In other cases, the cause is a genetic disorder known as neurofibromatosis type 2. This inherited mutation affects the gene responsible for producing Schwann cells, resulting in a higher likelihood of tumors. Vestibular schwannomas caused by neurofibromatosis type 2 occur bilaterally, or on both sides of the body. Patients may also have other tumors elsewhere in their body. Their condition often requires a different approach to treatment.

How Is Vestibular Schwannoma Diagnosed?

The most common presenting symptoms of a patient with a vestibular schwannoma are hearing loss and tinnitus. When a patient exhibits signs of a vestibular schwannoma, most frequently hearing loss, their primary care physician often either orders imaging directly, or refers the patient to an Ear, Nose and Throat surgeon (ENT) who typically orders imaging which leads to the diagnosis. If a diagnosis of a vestibular schwannoma is either confirmed or suspected, a patient will be referred to CNSO for further evaluation and management. The team of brain tumor specialists will review the patient’s medical history, which may include health conditions that influence their choice of a treatment plan. The team then performs a series of tests to diagnose the cause of the symptoms. Tests for diagnosing schwannoma include:

• Audiometry: This test is also called a hearing test. Sounds are directed into one ear at a time, in a range of volumes and tones. The patient is asked to indicate each time they hear a sound. Various words may also be presented to test hearing comprehension. The results help specialists determine how severely a patient’s hearing has been affected.
• Neurological exam: This is a physical exam designed to check for neurological deficits and injury. The tester directs the patient to perform tasks that assess their hearing, balance, strength, sensation, coordination, and motor control. Results can tell the doctors what area of the brain is affected.
• Imaging tests: Once a neurological issue is identified, and the symptoms are consistent with a vestibular schwannoma, imaging tests will be requested to determine the cause. Computed tomography (CT) and magnetic resonance imaging (MRI) are the gold standard imaging studies to detect a vestibular schwannoma.

Treatments for Vestibular Schwannoma

After diagnosing a vestibular schwannoma, the complex brain tumor and skull base specialists decide on a treatment plan. The priority of treatment is to preserve and restore as much facial function and hearing as possible while maintaining a high quality of life for the patient. Factors that affect overall goals and outcome of treatment include the size of the tumor, presenting symptoms including hearing loss and facial weakness, as well as age and overall health of the patient.

CNSO maintains a conservative philosophy toward treatment and prioritizes minimally invasive treatments when possible. Its specialists are experienced in administering a variety of surgical and non-surgical treatments, including:

Surgery

Vestibular schwannoma surgery is a highly effective treatment for most cases. The surgeon may use one of three approaches depending on the tumor’s size and the patient’s hearing:

• Middle fossa: This technique is best for small tumors in patients with good hearing. The incision is made above the ear, and some bone is removed.
• Suboccipital (retrosigmoid): This technique is best for any size tumor, in patients with good hearing. The incision is made behind the ear, and some bone is removed.
• Translabyrinthine: This technique is best for large tumors in patients who have already lost their hearing. The incision is made behind the ear, and part of the inner ear structure is removed. This gives the surgeon excellent access to the tumor.

After exposing the tumor, the surgeon uses an operative microscope and microsurgical techniques to remove as much of the brain tumor as possible before closing the incision. Neurological deficits prior to surgery, including hearing loss and facial weakness cannot always be reversed, however, the goal is to preserve pre-operative function. Though not guaranteed, surgery will at times improve hearing and often reverse facial nerve weakness, facial numbness, and tingling and balance issues.

Radiation Therapy

Radiation Therapy is offered in the form of Stereotactic Radiosurgery (SRS) or Fractionated Radiotherapy (Radiation):

• Stereotactic Radiosurgery (SRS): commonly known as Gamma Knife or CyberKnife radiosurgery, is a very focused type of radiation therapy that is a non-invasive alternative to traditional surgical methods. During this treatment, the patient’s head may be placed in a frame or a mask, and the patient is positioned in a machine that delivers very focused, high-dose radiation to just the tumor. The surgeon uses advanced technology to precisely target and focus the radiation on the tumor while avoiding damage to surrounding tissue. Due to the one-time high-dose radiation employed during SRS, only smaller tumors are appropriate for this treatment.
• Fractionated Radiotherapy (Radiation): a type of radiation treatment for patients who have large tumors but otherwise, due to age or underlying medical conditions, cannot tolerate an operation. This is a focused type of radiation, that breaks up the radiation treatments over a period of a few weeks so any one particular treatment only delivers a low dose of radiation, however, cumulatively a high dose of radiation is delivered to the tumor. This minimizes damage to surrounding structures including the cranial nerves and the brain, by spreading out the radiation over a number of sessions but still allowing a focused and high cumulative dose of radiation.

Radiation treatment in the form of SRS or Fractionated Radiotherapy does not remove the tumor, and it will not address any damage or symptoms caused by the tumor. Instead, the goal is to kill tumor cells and prevent the Vestibular Schwannoma from growing and causing new symptoms from developing.

Monitoring

Since a vestibular schwannoma is typically slow-growing, immediate intervention is not always necessary. If the tumor is small, and is not causing symptoms, often neurosurgeons recommend regular monitoring. In this approach, neurosurgeons will order regular scans of the tumor site, called an MRI scan of the brain and IACs. This allows the neurosurgeon to keep an eye on the tumor in case it starts growing rapidly. Patients should also watch for symptoms of increased growth.

Monitoring is typically recommended for patients with very small tumors that do not cause symptoms. It may also be recommended for older patients and those with serious medical problems, for whom other treatments might be too risky. However, doctors may recommend attempting surgery or radiation therapy if the tumor shows signs of significant growth.

Trust Vestibular Schwannoma Treatment at CNSO

A vestibular schwannoma can severely impact a person’s quality of life. At Centers for Neurosurgery, Spine & Orthopedics, patients can find comprehensive and coordinated treatment to relieve symptoms and improve their well-being. Patients work with a wide-ranging team of specialists who provide personalized surgical and non-surgical care. The comprehensive treatment model supports patients at every stage from diagnosis to treatment. CNSO offers convenient locations across New Jersey, including Bergen, Passaic, Essex, Morris, and Hudson counties. To learn more about vestibular schwannoma care, contact CNSO today.

Frequently Asked Questions About Vestibular Schwannoma

What is vestibular schwannoma?

Vestibular schwannoma, also called acoustic neuroma, is a benign and slow-growing tumor that most commonly originates from the Schwann cells covering the inferior vestibular nerve. These are cells that produce myelin, a fatty substance that insulates neurons.

What causes vestibular schwannoma?

Vestibular schwannoma occurs when Schwann cells start multiplying rapidly. Doctors are not always sure what causes this change. It is sometimes linked to neurofibromatosis type 2, a genetic disorder that creates mutations in Schwann cells and a higher likelihood of tumors.

Who is most at risk of vestibular schwannoma?

Anybody can develop a vestibular schwannoma. However, it is most common in individuals between the ages of 50 and 60. Other risk factors include chronic exposure to loud noises and ionizing radiation.

What are the symptoms of vestibular schwannoma?

Symptoms of vestibular schwannoma are caused by the tumor pressing against nerves that originate at the brainstem and course into the inner ear, which are responsible for facial movement, hearing and balance. The patient may experience a range of symptoms including:

• Hearing loss on one side of the head, often developing gradually
• Tinnitus, or a high-pitched ringing, buzzing, humming, or roaring sound
• A sensation of fullness in one ear
• Vertigo and loss of balance
• Dizziness
• Facial weakness
• Twitching, tics, or spasms in the eye, eyebrow, forehead, or mouth
• Tingling or numbness on one side of the face
• Difficulty swallowing
• Hoarse or scratchy voice
• Rarely, changes in taste perception
• Rarely, dry eyes or excess tears

Is surgery always the treatment for vestibular schwannoma?

The recommended treatment for vestibular schwannoma depends on its size and rate of growth. Small tumors may not require immediate intervention. If treatment is required, some tumors may be addressed using non-invasive methods such as radiation therapy. Surgery is the only treatment that has a chance of curing the tumor and has favorable outcomes with respect to facial nerve function and hearing preservation as compared to radiation therapy. Surgery has typically been reserved for larger tumors, but more patients are electing to have smaller tumors removed with excellent results. Surgery cannot restore lost hearing, but it can address other symptoms including balance problems, facial weakness and numbness.