1- The Ear
The ear is a complex structure that affects not only the ability to hear, but also impacts a person’s sense of equilibrium, affecting balance. The structure of the ear consists of three different regions: the outer ear, the middle ear and the inner ear.
The outer ear is the portion of the ear anatomy that is external to the world. It consists of the pinna, also called the auricle, and the external auditory meatus. The auricle consists of skin and cartilage and is responsible for protecting the inner portions of the ear, as well as for gathering sounds and steering them into the ear as part of the hearing process. Although unseen from the outside of the body, the external auditory meatus is considered a portion of the outer ear. This is the canal that leads from the auricle in toward the inner portions of the ear. The inner portion of the external ear ends at the eardrum.
The middle ear connects the eardrum to the inner ear. The middle ear contains ossicles, three different tiny bones. They are known as the malleus, the incus and the stapes and they move when sound waves come through the eardrum. This process converts the sound waves into vibrations to send forward into the inner ear. The Eustachian tube runs from the base of the middle ear toward the nasopharynx. This tube normalizes the pressure in the middle ear; for example, when flying in an airplane, passengers may clear the pressure that builds up in their ears by swallowing.
The inner ear consists of the bones that are necessary for hearing and balance. The cochlea is a coiled, fluid-filled tube that contains tiny hairs that work as hearing receptors. The vibrations created in the middle ear are pushed against a membrane known as the oval window, moving fluid through the cochlea, stimulating the hair cells and creating nerve signals. These signals are transmitted through the auditory nerve, which connects the cochlea to the brain. Nerve signals travel through the auditory nerve to the brain where they are perceived as sounds.
The auditory nerve actually plays two distinct roles: sending nerve signals for sounds and regulating balance for the body. An acoustic neuroma begins to grow on this auditory nerve, which is also referred to as the vestibulocochlear nerve. The tumor typically affects only one ear. An acoustic neuroma often grows slowly and as it does, it emerges through the auditory canal, the tube in which the auditory nerve, as well as the facial nerve is located. As the acoustic neuroma continues to grow, it expands behind the temporal bone of the skull. It may grow large enough to press on brain tissue, although this type of tumor does not actually infiltrate brain matter. It may also affect other cranial nerves, frequently the fifth cranial nerve, also known as the trigeminal nerve.
In most cases, an acoustic neuroma is typically not inherited, although it may occur manifesting as part of a genetic disorder. Most acoustic neuromas develop on their own, and there is not one exact cause of tumor formation. Research continues as to the reasons for the spontaneous development of this type of tumor, and several studies have indicated possible causes, including a history of radiation to the head or neck, a previous parathyroid tumor or defects in tumor suppressor genes. There is currently not one environmental factor that has been proven to cause acoustic neuroma.
2- Symptoms of Acoustic Neuroma
Additional symptoms include:
- Tinnitus
- Vertigo
- Loss of balance
- Facial weakness
Over 90 percent of patients diagnosed with acoustic neuroma complain of hearing loss in the affected ear. Hearing loss begins to manifest as difficulty hearing conversations or other sounds, particularly in noisy situations. While hearing can begin to diminish as people age, hearing loss associated with acoustic neuroma is distinguished by a reduction of hearing in only one ear. For five percent of patients, hearing loss is sudden and obvious, while the remainder of patients describe their hearing loss as a gradual process. Hearing may be affected by an inability to distinguish certain sounds or a full feeling in the affected ear.
Hearing loss is caused by pressure of the tumor on the acoustic nerve. This pressure can affect the work of the hairy cells and the body’s ability to transmit nerve signals to the brain. The auditory nerve may also become impaired when the acoustic neuroma disrupts blood flow to the site. Hearing loss may begin by an inability to perceive high-pitched sounds, although left untreated; the condition can progress to complete deafness in the affected ear.
Tinnitus occurs as a ringing or buzzing sound in the ear, even if there is no other noise present. Patients may complain of a variety of sounds from ringing to cracking, hissing, whistling or roaring noises that may be constant or intermittent. The sound volume varies from soft and barely perceptible to overwhelming and disruptive. Tinnitus related to acoustic neuroma is typically localized to the affected ear and may be high-pitched.
Because the tumor affects the inner portion of the ear, the patient with acoustic neuroma may complain of vertigo and may have difficulty with balance. For many patients, vertigo is an early sign of acoustic neuroma, and occurs when the tumor is a small size. As the tumor grows, balance problems and dizziness may resolve as a compensation mechanism. Vertigo is the sensation that the environment is moving or spinning when it is actually still. The patient may feel as if he or she is also spinning or moving, and severe cases may result in nausea or vomiting.
Loss of balance is more common than vertigo among patients with an acoustic neuroma. This condition occurs in approximately 50 percent of patients with this type of tumor. As the size of the neuroma increases, loss of balance, also referred to as dysequilibrium, may increase. Balance issues can result in difficulties with standing or walking and the patient may lean or bend toward one side of the body, putting him at greater risk of falls and injuries.
If an acoustic neuroma begins to press on the facial nerve, the patient may experience facial weakness and difficulty with forming some expressions. The facial nerve supplies the muscles of the face, particularly the eyebrows, eyelids and forehead. The patient may have difficulty with eye closure or with making expressions that involve eyebrow or forehead movement, particularly as the tumor grows. There may be a decrease in the ability to produce tears and the patient complains of changes in taste. Large tumors may compress the trigeminal nerve, which can result in facial numbness or tingling, a condition that can be constant or intermittent.
3- Diagnostic Procedures
Acoustic neuromas are often found as part of testing for other medical conditions. Some patients present for treatment with unilateral hearing loss or complaints of vertigo or tinnitus and after testing are diagnosed with the tumor. There are several types of tests that may pinpoint an acoustic neuroma:
- Audiometry
- Brainstem auditory evoked response
- Magnetic resonance imaging or computed tomography
Audiometry is a type of hearing test that is one of the first forms of diagnosis for acoustic neuroma. Approximately 95 percent of patients with this condition will have abnormal audiometry results. Audiometry tests both the intensity of sounds, measured in decibels, as well as sound tones, which are considered pitch. Many people with acoustic neuroma begin with hearing loss associated with high-pitched sounds.
Electronystagmography tests the acoustic nerve to determine if a tumor is affecting a patient’s sense of balance. The patient is fitted with electrodes around the eye to record eye movements. The ear is flushed with cold and then warm water to stimulate the inner ear and surrounding nerves. Normally, the patient experiences nystagmus, or rapid side-to-side eye movements; typically away from the cold water and toward the warm water. Abnormal responses indicate a potential problem with the auditory nerve.
Brainstem auditory evoked response (BAER) measures brain activity and may be used if an acoustic neuroma is affecting the brainstem. The patient is given earphones and electrodes are placed on the scalp and earlobes. The earphones play a series of clicks or tones and the electrodes record the brain’s response. This procedure tests cochlear function, which, if diminished, also indicates auditory impairment that could be related to acoustic neuroma.
Magnetic resonance imaging (MRI) or computed tomography (CT) scans may provide images of the brain to determine the presence of a tumor. These types of scans may be done after taking a patient history and physical, listing prominent symptoms and performing other initial tests, such as audiometry.
4- Classifications
After diagnosis of an acoustic neuroma, a physician stages the tumor according to one of three different classifications. Stage I means that the tumor is small and is contained within the auditory canal. It may be no bigger than the size of a bean. Stage II of diagnosis means that the tumor has extended beyond the auditory canal and into the intracranial space, but remains relatively small. At this stage, the tumor is usually no larger than 2cm. Stage III of tumor diagnosis means the tumor has invaded the intracranial space, it is larger than 2 cm and it is pressing on the brain.
5- Treatment Methods
Treatment options for acoustic neuroma depend on tumor classification, as well as the patient’s symptoms. Because most acoustic neuromas grow slowly, an early stage classification may warrant further observation.
6- Monitoring
7- Stereotactic Radiation
One type of stereotactic surgery used for the treatment of acoustic neuroma is the Gamma Knife. While the word “knife” is part of the name, the procedure actually does not involve cutting. For this process, the physician uses computer technology to determine the exact location of the neuroma in order to control the radiation. The patient is then placed in the Gamma Knife machine, which is similar in appearance to a helmet. The radiation is then directed at the tumor with scalpel precision. The overall effect of the procedure is to shrink the size of the neuroma by damaging the tumor cells so that they do not replicate and continue to grow. The Gamma Knife is up to 95 percent effective in shrinking skull tumors such as acoustic neuroma.
8- Surgical Procedures
The translabrynthine approach works to preserve the function of the facial nerve, but patients who undergo this type of surgery often have permanent hearing loss in the affected ear. This procedure involves cutting away the bone of the mastoid process behind the ear to expose the tumor. It is then removed and the tissue behind the ear is replaced with abdominal fat and a plate covering.
The middle fossa approach is often used for smaller neuromas that are still contained within the auditory canal. This procedure involves temporarily removing a small piece of skull bone above the affected ear to reach the tumor. After the neuroma is removed, the bone of the skull is replaced to its former position. The facial nerve is preserved in this type of procedure but unilateral hearing loss can occur in 30 to 40 percent of cases.
During the retrosigmoid approach, the surgeon makes an incision far behind the ear so the tumor can be exposed at about the level of the brainstem. Part of the tumor is removed in order to see the auditory canal and the facial and auditory nerves. The skull bone next to the nerves is then drilled away and the remainder of the tumor is removed. During this procedure, there is a risk that some of the tumor may remain behind. Hearing is preserved in 50 to 70 percent of cases.
Although an acoustic neuroma is a benign tumor, this diagnosis can dramatically change a patient’s quality of life. While there is little chance of metastasis, an acoustic neuroma can do enough damage at its original location to significantly impact a patient’s sense of balance and hearing abilities. Patients diagnosed with these types of tumors need guidance about the best types of treatment for their situations as well as reassurance that there is a bright outlook for the future.
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The ear is a complex structure that affects not only the ability to hear, but also impacts a person’s sense of equilibrium, affecting balance. The structure of the ear consists of three different regions: the outer ear, the middle ear and the inner ear.
The outer ear is the portion of the ear anatomy that is external to the world. It consists of the pinna, also called the auricle, and the external auditory meatus. The auricle consists of skin and cartilage and is responsible for protecting the inner portions of the ear, as well as for gathering sounds and steering them into the ear as part of the hearing process. Although unseen from the outside of the body, the external auditory meatus is considered a portion of the outer ear. This is the canal that leads from the auricle in toward the inner portions of the ear. The inner portion of the external ear ends at the eardrum.
The middle ear connects the eardrum to the inner ear. The middle ear contains ossicles, three different tiny bones. They are known as the malleus, the incus and the stapes and they move when sound waves come through the eardrum. This process converts the sound waves into vibrations to send forward into the inner ear. The Eustachian tube runs from the base of the middle ear toward the nasopharynx. This tube normalizes the pressure in the middle ear; for example, when flying in an airplane, passengers may clear the pressure that builds up in their ears by swallowing.
The inner ear consists of the bones that are necessary for hearing and balance. The cochlea is a coiled, fluid-filled tube that contains tiny hairs that work as hearing receptors. The vibrations created in the middle ear are pushed against a membrane known as the oval window, moving fluid through the cochlea, stimulating the hair cells and creating nerve signals. These signals are transmitted through the auditory nerve, which connects the cochlea to the brain. Nerve signals travel through the auditory nerve to the brain where they are perceived as sounds.
The auditory nerve actually plays two distinct roles: sending nerve signals for sounds and regulating balance for the body. An acoustic neuroma begins to grow on this auditory nerve, which is also referred to as the vestibulocochlear nerve. The tumor typically affects only one ear. An acoustic neuroma often grows slowly and as it does, it emerges through the auditory canal, the tube in which the auditory nerve, as well as the facial nerve is located. As the acoustic neuroma continues to grow, it expands behind the temporal bone of the skull. It may grow large enough to press on brain tissue, although this type of tumor does not actually infiltrate brain matter. It may also affect other cranial nerves, frequently the fifth cranial nerve, also known as the trigeminal nerve.
In most cases, an acoustic neuroma is typically not inherited, although it may occur manifesting as part of a genetic disorder. Most acoustic neuromas develop on their own, and there is not one exact cause of tumor formation. Research continues as to the reasons for the spontaneous development of this type of tumor, and several studies have indicated possible causes, including a history of radiation to the head or neck, a previous parathyroid tumor or defects in tumor suppressor genes. There is currently not one environmental factor that has been proven to cause acoustic neuroma.
2- Symptoms of Acoustic Neuroma
The most common symptom associated with acoustic neuroma is hearing loss, although other signs may appear either in conjunction with hearing impairment or separately.
Additional symptoms include:
- Tinnitus
- Vertigo
- Loss of balance
- Facial weakness
Over 90 percent of patients diagnosed with acoustic neuroma complain of hearing loss in the affected ear. Hearing loss begins to manifest as difficulty hearing conversations or other sounds, particularly in noisy situations. While hearing can begin to diminish as people age, hearing loss associated with acoustic neuroma is distinguished by a reduction of hearing in only one ear. For five percent of patients, hearing loss is sudden and obvious, while the remainder of patients describe their hearing loss as a gradual process. Hearing may be affected by an inability to distinguish certain sounds or a full feeling in the affected ear.
Hearing loss is caused by pressure of the tumor on the acoustic nerve. This pressure can affect the work of the hairy cells and the body’s ability to transmit nerve signals to the brain. The auditory nerve may also become impaired when the acoustic neuroma disrupts blood flow to the site. Hearing loss may begin by an inability to perceive high-pitched sounds, although left untreated; the condition can progress to complete deafness in the affected ear.
Tinnitus occurs as a ringing or buzzing sound in the ear, even if there is no other noise present. Patients may complain of a variety of sounds from ringing to cracking, hissing, whistling or roaring noises that may be constant or intermittent. The sound volume varies from soft and barely perceptible to overwhelming and disruptive. Tinnitus related to acoustic neuroma is typically localized to the affected ear and may be high-pitched.
Because the tumor affects the inner portion of the ear, the patient with acoustic neuroma may complain of vertigo and may have difficulty with balance. For many patients, vertigo is an early sign of acoustic neuroma, and occurs when the tumor is a small size. As the tumor grows, balance problems and dizziness may resolve as a compensation mechanism. Vertigo is the sensation that the environment is moving or spinning when it is actually still. The patient may feel as if he or she is also spinning or moving, and severe cases may result in nausea or vomiting.
Loss of balance is more common than vertigo among patients with an acoustic neuroma. This condition occurs in approximately 50 percent of patients with this type of tumor. As the size of the neuroma increases, loss of balance, also referred to as dysequilibrium, may increase. Balance issues can result in difficulties with standing or walking and the patient may lean or bend toward one side of the body, putting him at greater risk of falls and injuries.
If an acoustic neuroma begins to press on the facial nerve, the patient may experience facial weakness and difficulty with forming some expressions. The facial nerve supplies the muscles of the face, particularly the eyebrows, eyelids and forehead. The patient may have difficulty with eye closure or with making expressions that involve eyebrow or forehead movement, particularly as the tumor grows. There may be a decrease in the ability to produce tears and the patient complains of changes in taste. Large tumors may compress the trigeminal nerve, which can result in facial numbness or tingling, a condition that can be constant or intermittent.
3- Diagnostic Procedures
- Audiometry
- Electronystagmography
- Brainstem auditory evoked response
- Magnetic resonance imaging or computed tomography
Audiometry is a type of hearing test that is one of the first forms of diagnosis for acoustic neuroma. Approximately 95 percent of patients with this condition will have abnormal audiometry results. Audiometry tests both the intensity of sounds, measured in decibels, as well as sound tones, which are considered pitch. Many people with acoustic neuroma begin with hearing loss associated with high-pitched sounds.
Electronystagmography tests the acoustic nerve to determine if a tumor is affecting a patient’s sense of balance. The patient is fitted with electrodes around the eye to record eye movements. The ear is flushed with cold and then warm water to stimulate the inner ear and surrounding nerves. Normally, the patient experiences nystagmus, or rapid side-to-side eye movements; typically away from the cold water and toward the warm water. Abnormal responses indicate a potential problem with the auditory nerve.
Brainstem auditory evoked response (BAER) measures brain activity and may be used if an acoustic neuroma is affecting the brainstem. The patient is given earphones and electrodes are placed on the scalp and earlobes. The earphones play a series of clicks or tones and the electrodes record the brain’s response. This procedure tests cochlear function, which, if diminished, also indicates auditory impairment that could be related to acoustic neuroma.
Magnetic resonance imaging (MRI) or computed tomography (CT) scans may provide images of the brain to determine the presence of a tumor. These types of scans may be done after taking a patient history and physical, listing prominent symptoms and performing other initial tests, such as audiometry.
4- Classifications
5- Treatment Methods
Treatment options for acoustic neuroma depend on tumor classification, as well as the patient’s symptoms. Because most acoustic neuromas grow slowly, an early stage classification may warrant further observation.
6- Monitoring
In some situations, no treatment may be necessary in the initial stages, and the physician may decide only to monitor the tumor for changes. The patient may be monitored periodically for new symptoms, such as an increase in hearing loss or balance problems that could indicate that the tumor is growing. Frequent observation may be a viable option for patients who are not suffering from disruptive symptoms of acoustic neuroma or those who want to avoid surgical intervention. Because the tumor grows slowly, there may be little change in growth, even over the course of several years. Additionally, a small percentage of patients have had acoustic neuromas that have actually diminished in size. Alternatively, there are some patients whose tumors end up growing rapidly, warranting further intervention. Careful monitoring is necessary to ensure prompt treatment if the patient does have the latter condition.
7- Stereotactic Radiation
An acoustic neuroma may be removed through a process of stereotactic radiation. Under normal circumstances, radiation involves using a beam of energy that is directed at a specific tumor or location to kill specific tissue. Stereotactic radiation involves directing radiation in an exact dose through narrow beams. This type of radiation focuses more on killing the tumor and has less of an effect on the surrounding tissues. The physician delivering the radiation beam has much more control over targeting the tumor, and is less prone to injuring surrounding cells, which is important because of the adjacent brain tissue.
One type of stereotactic surgery used for the treatment of acoustic neuroma is the Gamma Knife. While the word “knife” is part of the name, the procedure actually does not involve cutting. For this process, the physician uses computer technology to determine the exact location of the neuroma in order to control the radiation. The patient is then placed in the Gamma Knife machine, which is similar in appearance to a helmet. The radiation is then directed at the tumor with scalpel precision. The overall effect of the procedure is to shrink the size of the neuroma by damaging the tumor cells so that they do not replicate and continue to grow. The Gamma Knife is up to 95 percent effective in shrinking skull tumors such as acoustic neuroma.
8- Surgical Procedures
Some patients may require surgery for treatment of acoustic neuroma. The goal of surgery is to remove the tumor while preserving hearing function for the patient. In many cases, the entire tumor can be removed; the likelihood of tumor regrowth is less than 0.1 percent. There are several different surgical procedures that may be performed.
The translabrynthine approach works to preserve the function of the facial nerve, but patients who undergo this type of surgery often have permanent hearing loss in the affected ear. This procedure involves cutting away the bone of the mastoid process behind the ear to expose the tumor. It is then removed and the tissue behind the ear is replaced with abdominal fat and a plate covering.
The middle fossa approach is often used for smaller neuromas that are still contained within the auditory canal. This procedure involves temporarily removing a small piece of skull bone above the affected ear to reach the tumor. After the neuroma is removed, the bone of the skull is replaced to its former position. The facial nerve is preserved in this type of procedure but unilateral hearing loss can occur in 30 to 40 percent of cases.
During the retrosigmoid approach, the surgeon makes an incision far behind the ear so the tumor can be exposed at about the level of the brainstem. Part of the tumor is removed in order to see the auditory canal and the facial and auditory nerves. The skull bone next to the nerves is then drilled away and the remainder of the tumor is removed. During this procedure, there is a risk that some of the tumor may remain behind. Hearing is preserved in 50 to 70 percent of cases.
Although an acoustic neuroma is a benign tumor, this diagnosis can dramatically change a patient’s quality of life. While there is little chance of metastasis, an acoustic neuroma can do enough damage at its original location to significantly impact a patient’s sense of balance and hearing abilities. Patients diagnosed with these types of tumors need guidance about the best types of treatment for their situations as well as reassurance that there is a bright outlook for the future.
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