Acoustic Neuroma Treatment Options - Overview

Acoustic Neuroma Surgical Options

Translabyrinthine Approach

Doctors may use translabyrinthine surgery for any size of tumor that has caused significant hearing loss or where hearing preservation is not possible. During this procedure, the surgeon makes an incision behind the ear and opens the mastoid bone, as well as a portion of the inner ear, which contains structures important for hearing and balance. This gives the surgeon access to the tumor in the internal auditory canal, which acts as the passageway for the eighth cranial nerve—the nerve that runs from the brain to the inner ears—and provides a good view of the nerves so the surgeon can preserve facial function.

The surgeon removes the entire tumor, or as much of it as is safely possible. To reach the tumor, surgeons occasionally remove the cochlea, the part of the inner ear that processes sound, or the otic capsule, which is the bony structure that surrounds the inner ear.

Because a portion of the inner ear is removed during this procedure, hearing is lost in that ear. Balance is usually not a problem because the opposite ear can take over this function, although rehabilitation therapy may be necessary to help you compensate for some loss of balance.

In general, the translabyrinthine approach is the best option when hearing has already been severely affected from the tumor or when tumors are large and hearing preservation is not possible.

Pros:

• Oldest approach - longest history
• Approach facilitates identification of facial nerve for preservation
• Allows for excellent exposure of the internal auditory canal and tumor
• Any size tumor can be removed with this approach.

Con:

• Results in permanent and complete hearing loss on tumor side

Retrosigmoid Approach

Surgeons may use a retrosigmoid approach for smaller acoustic neuromas when hearing preservation is possible. They use this approach for tumors that are growing out of the internal auditory canal and approaching the brainstem.

During this surgery, a surgeon makes an incision further behind the ear to open a portion of the skull called the occipital bone, located behind the mastoid. The cerebellum, a part of the brain located above the brain stem, falls back out of the way, and surgeons remove the bone over the internal auditory canal to fully access the tumor. The surgeons can view the facial nerve, the hearing nerve, and the brainstem.

If removing the entire tumor could damage nerves or brain tissue, the doctor may leave some small bits of the tumor behind. The section of the skull opened to perform this surgery is replaced after tumor removal. Fat from the periumbilical region, meaning the area surrounding the belly button, may be removed and used to seal the closure to prevent spinal fluid leaks.

Pros:

• Possible preservation of hearing 
• Approach provides a good view of the AN in relation to brainstem
• Possible preservation of facial nerve
• Any size tumor can be removed with this approach.

Cons:

• Hearing preservation decreases if the tumor is large.
• Headaches are a more prevalent post-op side effect.

Middle Fossa Approach

The middle fossa approach is an option for smaller tumors that have not grown beyond the internal auditory canal. As with the retrosigmoid approach, it is used to help preserve hearing. The surgeon makes an incision above the ear in the lateral skull bone, and then uncovers the internal auditory canal, and removes the acoustic neuroma. This approach is the best for saving hearing, which is possible in the majority of people who have the procedure. Then surgeons replace the skull bone and use fat from elsewhere in the body to help close the opening.

Pro:

• Possible preservation of hearing with small tumors in the right location, typically confined to the internal auditory canal

Con:

• Most often used only with small tumors, typically confined to the internal auditory canal.

Radiation Treatment Options

Stereotactic radiation can either be delivered as single-fraction radiosurgery (SRS) or by dividing the radiation dose over multiple sessions which is termed fractionated stereotactic radiotherapy (FRS). Both forms of radiation (SRS and FSR) work similarly by damaging the DNA within the tumor cells. The cells can no longer divide and eventually die over time, a process called necrosis. Both techniques are performed in the outpatient setting and do not require either general anesthesia or a hospital stay.

Over the last several decades, as the technologies for delivering stereotactic radiation have improved, an increasing number of patients have chosen to receive stereotactic radiosurgery as the primary treatment for their acoustic neuroma. There are several different commercially-available machines that are used to treat acoustic neuromas with the technologies differing in their source of radiation and how the radiation is precisely delivered.

• Gamma Knife® machines derive their radiation from a fixed-array of Cobalt-60 sources. These machines are typically used to deliver SRS in a single-session, although the newest platform (Leksell Gamma Knife® Icon™) allows for fractionated delivery (FRS).
• Linear Accelerator (LINAC) machines work by accelerating electrons to produce high-energy X-rays. The beam of X-rays (photons) is then shaped to the tumor as it exits the machine using a series of collimators and by rotating either the patient or the machine. LINAC machines are produced by a variety of different manufacturers with common trade names including CyberKnife®, Trilogy®, Novalis Tx™, and TruBeam™ among others, with each machine available for both single-session (SRS) and multi-session (FSR) treatment.
• Proton Beam machines use a particle accelerator to generate radiation energy in the form of protons which can be delivered as either SRS or FSR.

Despite their differences, similar results in terms of effectively treating acoustic neuromas and avoiding side effects have been reported for each of the stereotactic radiation machines. The treatment team should consist of a neurosurgeon and/or a neurotologist and a radiation oncologist. The patient and the treatment team typically consider a number of factors before determining whether radiation therapy is appropriate including the size of the acoustic neuroma and the rate at which it is growing, the patient’s age and overall health, and the patient’s symptoms including the degree of hearing loss, balance problems, and vertigo. Typically, acoustic neuromas that are greater than 2.5 – 3cm in size are not considered ideal candidates for radiation therapy as these larger tumors often compress the surrounding brainstem and the potential for side effects from the radiation is increased.

Advantages of Surgery

• Surgery removes the tumor for those who want it "out of their body."
• Some patients have a fear of very rare, long term effects of radiation, such as induced malignancy.
• Size and/or position of the tumor may make radiation inadvisable, due to post-treatment swelling.
• Radiation may not be recommended for tumors larger than 2.5 to 3 cm.
• Younger age is generally another determining factor for choosing surgery.
• Sub or near-total tumor resection followed by radiation may be considered.

Advantages of Radiation

• Good option for patients in their mid-50's and older or with health issues.
• Radiation is typically an outpatient procedure, though some patients may stay in the hospital overnight. The radiation session itself is relatively quick. Some procedures are done in one session and others take several sessions.
• There is usually no need to take time off from work. Some people are treated on their way to or from work when having multiple sessions.
• There is no recuperation or convalescence time immediately after treatment.
• There are usually no immediate complications. In the medium term, there may occasionally be complications as radiation takes time to fully present symptoms.

Advantages of Observation

• Good option for small tumors, especially in older individuals; AN may not grow and may not require treatment.
• Hearing may be preserved longer in cases where the tumor presents on the only hearing side.
• All medical treatments, surgical or radiation, carry some risks. As ANs are benign and grow very slowly, many physicians will recommend having a second MRI at least 6 months after the first, to establish the growth rate. If the tumor is not growing, avoiding treatment altogether is a possibility.
• In time, safer treatments for acoustic neuromas, other than surgery or radiation, may be found.
• There are no know ways to cure or shrink an acoustic neuroma naturally.

What To Expect

Due to expanded use of MRIs, many acoustic neuroma tumors are discovered when they are relatively small. Because of this and the fact that acoustic neuromas are non-cancerous, the patient and caregivers typically have time to do thoughtful research on treatment options and medical providers. ANA recommends getting more than one medical opinion about treatment whenever possible. It is also important to learn how various treatments will affect short and long-term quality of life. Depending on a variety of factors, a patient may be more concerned with hearing preservation, facial function, or other side effects.

Surgery

Surgery for an acoustic neuroma is performed during general anesthesia and involves removing the tumor through the inner ear or through a window in your skull. The entire tumor may not be able to be completely removed in some cases. For example, if the tumor is too close to important parts of the brain or the facial nerve.

Surgery can create complications, including worsening of symptoms, if certain nerve or cranial structures are affected during the operation. These risks are often based on the size of the tumor and the surgical approach used.

After surgery, you may spend a few days recovering in the hospital while your doctor monitors you and manages any pain, dizziness, and other symptoms you may be experiencing. If your hearing has been affected by the surgery, your doctor can work with you to explore your options for hearing rehabilitation. Balance is recovered slowly, and most people can return to work in 8 to 12 weeks. (Included with permission by NYU Langone www.nyulangone.com.)

Radiation

If the decision is made to move forward with radiation, the treatment process usually begins by defining the radiation target using an MRI with gadolinium to visualize the acoustic neuroma. The stereotactic radiation planning software is then used to create a 3-dimensional model of the tumor and the surrounding structures. The treatment team along with a radiation physicist then creates dosimetry maps showing the level of radiation to be received by the tumor and the normal tissues and the treatment plan is optimized to focus the radiation as precisely as possible to the acoustic neuroma. The patient’s head is then stabilized with either a molded mask shield or a metal frame that is pinned to the skull. The type of stabilization device and the length of treatment depend on the stereotactic radiation machine being used, but treatments generally last between 30 -120 minutes for SRS and approximately 10-15 minutes for each treatment fraction with FSR.

Once radiation has been used to treat the acoustic neuroma, surveillance imaging, typically with MRI scans, should be performed for at least 10 years after the treatment to ensure that the tumor does not continue to show any signs of growth that would require further treatment.

Observation

Observation has become an accepted ‘treatment’ option for acoustic neuroma when the tumor is small, the patient is asymptomatic, is elderly, or the patient is averse to treatment.

According to the 2014 ANA Patient Survey:

• the percentage of respondents reporting surgery as their first treatment has fallen from 100% in 1983 to 51% in 2014
• the percentage of respondents reporting “Watch and Wait”, or observation, as their first treatment has risen from 0% in 1983 to 20% in 2014
• 29% have been in observation mode for 1 year or less
• 27% have been in observation mode between 1 and 3 years
• 17% have been in observation mode between 5 and 10 years
• 8% have been in observation mode for more than 10 years

Monitoring will likely continue with periodic MRIs and other tests; surgery or radiation treatment may be recommended if there is tumor growth and/or an increase in symptoms. Your doctor may also recommend treatment to preserve hearing, facial function, or other factors.

The Newly Diagnosed Handbook patient booklet has more detailed information about treatment options.