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Meniere's Syndrome PATIENT INFORMATION

Meniere’s syndrome is a disorder of the inner ear characterized by the triad of hearing loss, tinnitus and episodic vertigo. It was first recognized to be attributable to an inner ear problem approximately one hundred and fifty years ago by a French physician, Prosper Meniere, and hence the name. Most of the time Meniere’s syndrome affects one ear. However, in about one fifth of all patients it affects both ears. The majority of patients with Meniere’s syndrome can have their symptoms controlled with some simple dietary and medical treatments.

As you can see in the diagram E, the ear is comprised of three parts: the outer, the middle, and the inner ear. Each of these three parts of the ear function very differently. Meniere’s syndrome affects only the inner ear. The inner ear is divided into two parts: the hearing part known as the cochlea, and the balance part known as the vestibular portion. The inner ear is a series of tubular spaces within the bone of the ear. If this tube is divided, there are three chambers seen inside. In Meniere’s syndrome there is a relative imbalance in the fluids between the middle chamber and the outer chambers. The fluid in the middle chamber is very different in chemical composition to the fluids contained in the outer chambers. As the fluid pressure increases in the middle chamber, it presses on the hearing nerve and causes hearing loss and tinnitus, or ringing in the ear. If the pressure becomes great enough, it can rupture the membrane between the middle and outer chambers and cause vertigo, a dizzy spell in which things spin around. (Diagram F)

Meniere’s syndrome can present in many varied fashions. A typical case, however, for Meniere’s syndrome would be as follows. First, one experiences a full feeling in the ear and begins to have ringing or buzzing noise in the ear, known as tinnitus. As this increases, hearing loss is noted. This usually heralds the onset of vertigo, or a spinning sensation. The vertigo is usually accompanied by nausea and vomiting. The onset of the tinnitus and hearing loss can be only a few minutes before the vertigo spell or can last several days. The true spinning sensation usually lasts between thirty minutes and two hours. After the spinning ceases, one feels very washed out and drained. Over the next 24 hours, the hearing usually improves and the tinnitus lessens. This cycle then repeats itself. The cycle can repeat itself several times a day or once every few years. Typically, early in the course of this disease, one experiences infrequent spells that are very severe, and as the disease comes toward its conclusion, one experiences frequent spells that are less severe and more mild in nature. The above scenario has described a typical case. However, there are many variations with Meniere’s syndrome. Sometimes the hearing does not fluctuate much. Sometimes there is a residual constant imbalance and sensitivity to motion. Often the tinnitus persists between spells.

There are also two variations of Meniere’s syndrome in which only one portion of the inner ear is involved, the cochlea or the vestibule. In cochlear hydrops, only the cochlear, or hearing portion of the ear, is involved. These patients experience fluctuating hearing loss and tinnitus but no vertigo. In vestibular hydrops, patients experience only the episodic vertigo and no change in their hearing or tinnitus. Many patients will start with the disease affecting only one portion and then later notice involvement of both portions of the inner ear. It is extremely rare for the person to become totally deaf from Meniere’s syndrome in the affected ear. It is even less common for a patient that has Meniere’s syndrome in both ears to become deaf in both ears.

There are several tests that can be used to help in the diagnosis of Meniere’s syndrome. By far the most important part of making the correct diagnosis of Meniere’s syndrome is the symptoms that you have. A complete hearing test is also a part of every evaluation for Meniere’s syndrome. In addition, a test to measure the balance function of the inner ear known as an electrostagmogram is often ordered. This test involves measurement of the eye movements by placing some electrodes around the eye to measure the movement. Cool and warm water is also placed in the ear canal to stimulate the balance canals. This test does not hurt, but it does take over an hour or so to complete. Sometimes a test known as an electrocochleography is ordered. This test measures the relative amount of fluid in the middle chamber of the inner ear to that in the outer chambers. One of the few conditions that can mimic Meniere’s syndrome is a small growth on the balance nerve known as acoustic neuroma. Because it is important to identify this, many times a test known as a brain stem audiometry or an MRI scan is ordered to make sure that this condition is not present. Often laboratory tests are ordered to evaluate your blood for markers indicative of autoimmune disease. Occasionally allergy testing is recommended to see if this may be the trigger for your Meniere’s syndrome.

The treatment of Meniere’s syndrome is based on efforts to reduce the fluid imbalance in the inner ear. The mainstay of treatment includes a low sodium or low salt diet and use of a fluid pill or diuretic. These two measures work to reduce the production of fluid in the middle ear chamber known as endolymph. Often an anticholinergic medication is prescribed as well. The anticholinergic medicines work to reduce the chemicals in the nerve endings of the inner ear to decrease the sensitivity of these nerve endings. Seventy percent of those persons with Meniere’s syndrome respond very well to these conservative measures. Many times a medication will be prescribed to place under your tongue to work very fast to stop the vertigo. This medication is absorbed very quickly into your body and works to stop the effects of the inner ear on the nausea and dizziness center in the computer part of the brain. It works much faster than a pill. Some people with Meniere’s disease experience a cyclic fashion to their disease. They will go for long periods of time without any problems and then develop severe episodes. In these cases and in the case with a very sudden severe drop in hearing, cortisone is often prescribed. The cortisone acts to decrease the inflammation and fluid imbalance in the inner ear and stop the cycle. Although the cortisone is most often administered orally, occasionally it is administered through the vein. Sometimes in cases of an exacerbation or increase in the cycle or a sudden change in the hearing, the cortisone is placed in the middle ear in an effort for it to move across the tiny membrane separating the inner ear from the middle ear known as the round window membrane. There is evidence to suggest that high concentrations of cortisone can be achieved in the inner ear in this fashion. This usually has no long term effect on Meniere’s syndrome but can be helpful for acute exacerbations such as a sudden sharp drop in the hearing.

About seventy percent of those people affected with Meniere’s syndrome respond well to the above mentioned treatments or a combination thereof. However, about thirty percent of people with Meniere’s syndrome do not respond to these conservative measures and have persistent vertigo spells that have to be treated with more aggressive measures. These treatments can roughly be divided into destructive or nondestructive procedures related to their effect on the vestibular portion of the inner ear. The chart below demonstrates this.

ADVANCED TREATMENT FOR MENIERE’S SYNDROME

 

Setting

Incision

Hearing Loss

Control of dizzy spells

Some Potential Complications

Expected Time Off Work

Destroy the Balance part of inner ear

Endolymphatic Sac Shunt

Outpatient surgery

Behind the ear

1% chance

66% chance

Hearing loss, unsteadiness, facial paralysis, spinal fluid leak, meningitis, infection, bleeding

2 days

No

Intratypmpanic Gentamycin

3 to 7 treatments in office

In eardrum

20% chance

80% chance

Hearing loss, meningitis, unsteadiness, hole in tympanic membrane (eardrum)

For visits

Yes

Vestibular Nerve Section

5 days in hospital, One night in Intensive Care

Behind the ear

10% chance

95% chance

Hearing loss, unsteadiness, facial palsy, stroke, death

2 weeks

Yes

Labryinthectomy

3 to 5 days in hospital

Behind the ear

100% chance

99% chance

Unsteadiness, facial palsy, infection

2 weeks

Yes


As you will note, there is a subdivision of the destructive procedures characterized by their effects on the hearing into two categories as hearing preservation or nonhearing preservation. Treatment choices are often modified based on the amount of hearing that is present.

The most conservative treatment beyond medical treatment is the nondestructive procedure of draining the fluid from the middle chamber of the inner ear. This is known as an endolymphatic sac operation. There is an appendage of the inner ear seen on the ear diagram at the beginning of this pamphlet known as the endolymphatic sac. The endolymphatic sac lies between the main portion of the inner ear and the covering of the brain known as the dura. In fact, the sac itself lies within the dura itself. The operation known as the endolymphatic shunt drains the fluid from this sac into the mastoid or ear sinus. It involves a general anesthetic and takes about forty-five minutes. It is done on an outpatient basis. An incision is made in the crease behind the ear. The mastoid or ear sinus is then opened up to gain access to this sac. The sac is then opened up and a small piece of medical grade Silastic is placed in the sac to shunt the fluid from the sac into the mastoid. A large dressing is wrapped around the head after the wound is closed. The dressing is removed the first postoperative day. For the next several weeks, there is fluid within the middle ear, and this causes some diminution in hearing. However, the fluid eventually resolves and the hearing is restored. This operation is effective in relieving the vertigo spells about two thirds of the time in those people that have failed the medical treatment. However, one third of the time, the operation is not effective at all. The advantage of this procedure is in the fact that it does not destroy any of the balance function of the inner ear. It is also extremely rare for it to affect the hearing in an adverse way. Many times it improves the hearing. However, that is not something to be relied on and it is not recommended for purposes of hearing improvement. It usually has no effect on the tinnitus one way or the other. Occasionally if the hearing is improved, the tinnitus usually improves as well. The surgical risks with the endolymphatic shunt are very rare. In less than one percent of cases, there is a total loss of hearing and extreme dizziness following the surgery, which persists for a couple of weeks. This happens when a portion of one of the balance or semicircular canals is violated. In this complication, the benefit, although unintended, is that there will be no more spells of vertigo as the inner ear is destroyed. Again this particular complication occurs less than one percent of the time. Other even less frequent complications include a leak of spinal fluid through the dura, which could require another operation to repair. The facial nerve, which goes through the ear, is the nerve that moves the face. This nerve could also be injured in this operation. However, that is extremely rare. The chances of this happening are significantly less than one half of one percent. The advantages of this operation are that it is usually successful in controlling the vertigo spells in two thirds of the cases, and it is extremely unlikely to cause any untoward effects. It also retains the balance function of that inner ear.

The destructive procedures all have in common either destruction of the balance portion of the inner ear either in part or whole.

The nonhearing preservation procedure involves complete removal of the inner ear. This is known as a labyrinthectomy. It involves a two or three day hospital stay. The surgery involves an incision behind the ear in the crease behind the ear and takes about forty-five minutes. The balance portion of the inner ear is removed. This always results in a total loss of hearing in that ear. The patient is very dizzy and nauseated right after the surgery. Medications are prescribed to decrease the nausea. The patient is kept in the hospital two or three days as the balance improves. Usually with three days after the surgery, the patient is able to get around with some assistance. Within two weeks after the surgery, the patient is able to take care of himself without any difficulty. They should be able to return to work within two weeks or so. Within two or three months, the patient is able to do almost every activity they had done before including playing tennis, jogging, etc. The age of the patient is critical in determining recovery from destructive procedures. A very young patient will recover very quickly. An elderly patient will recover very slowly and may always be left with some residual imbalance. Sometimes it is difficult to determine whether the residual imbalance is better than the episodic spells of vertigo with nausea and vomiting. The labyrinthectomy is usually not advised if the patient retains serviceable hearing in that ear. The advantage is that it controls the vertigo virtually one hundred percent of the time. The disadvantage is that all remaining hearing is lost one hundred percent of the time.

Hearing conservation procedures allow the possibility to preserve hearing while destroying balance function in the inner ear. Gentamycin profusion of the ear is employed to destroy the balance function and hopefully preserve the hearing. This involves opening the middle ear by cutting a hole in the eardrum after numbing it by placing a cream on it in the office. Once this is accomplished, the Gentamycin is placed in the middle ear and allowed to profuse across the round window membrane into the inner ear. A tube is placed in the eardrum at the same time to allow subsequent infiltration of the medication into the middle ear in the office after the initial profusion. It usually takes at least three treatments lasting thirty minutes each and spaced ten days to two weeks apart. Gentamycin is an antibiotic that is toxic to the nerve endings in the inner ear. It will destroy the balance nerve endings as well as the hearing nerve endings. However, it affects the balance nerve endings preferentially and if the dose can be titrated, you can usually destroy the balance function and preserve the hearing function. This works to stop the dizzy spells about eighty percent of the time. About twenty percent of the time, it does not work. We presume this is due to some problems with transmission of the medication across the round window membrane. Also, about twenty percent of the time when the treatment is effective in relieving the vertigo spells, it causes a significant drop in the hearing. It is rare for this to cause a total hearing loss. The advantages of this procedure are that it can be done with a local anesthetic with very little risk of any problems other than hearing loss. The disadvantage is mainly in its imprecision. It is quite variable how many treatments are needed, and in some cases it is not effective at all.

Another hearing preservation procedure involves a section of the vestibular or balance nerve. The balance and hearing nerves are bound together as they leave the brain and enter toward the inner ear. The balance portion of this nerve can be cut. This results in a disconnection between the inner ear and the brain. Therefore, when the inner ear has a Meniere’s spell, it does not register with the brain and the vertigo spells are therefore relieved. This procedure involves a five day hospital stay. An incision is made two inches behind the ear and a small window is cut and removed from the skull to afford a view into the space between the brain and the inner ear. The nerve is then cut and the window of bone replaced and the incision closed. This surgery is effective in controlling the dizzy spells about ninety-five percent of the time. About one in twenty times, it is not effective because several of the balance fibers travel with the hearing part of the nerve and they are not divided. Ninety percent of the time, the hearing can be saved. However, about ten percent of the time the hearing is lost due to disruption of the blood supply to the hearing portion of the nerve. This surgery involves manipulation of the brain and attendant risk with this manipulation includes the possibility of stroke, spinal fluid leakage, paralysis of the face, infection of the spinal fluid known as meningitis, or death. These complications are extremely rare and occur less than one percent of the time. However, they are very serious. This procedure is usually recommended in patients whose job will not accommodate any dizzy spells, such as an iron worker.

As you can see, there are many treatments for Meniere’s syndrome. These treatments are tailored to meet the specific problems that each patient encounters with Meniere’s syndrome. Most people, as mentioned above, respond very well to simple medical treatments. However, if disabling vertigo ensues, there are more radical treatments that can be used. In extremely rare cases of severe vertigo spells involving both ears, it is necessary to destroy both inner ears. This always leaves the patient with residual imbalance. This destruction is accomplished by systemic injections of antibiotics toxic to the balance portion of the inner ear.

At the Jackson Ear Clinic, we will work diligently with you to tailor a treatment plan that will work effectively for you. We can stop the spells of vertigo almost one hundred percent of the time. Usually patients end up with some degree of permanent hearing loss and many times no amplification is needed as the loss is mild. If the loss is severe, a hearing aid can be used. The tinnitus or noise that accompanies this usually persists to some degree. Most people find that as time goes on, this is not as noticeable and thus it is not so bothersome to them. In the event that it is bothersome, there are things that can be done to help. However, the tinnitus is not something that can be resolved totally. As always, if you have any questions regarding the information contained in this pamphlet or how the treatment applies to you, please feel free to call us.

The content of this site is intended for information purposes only; it is not meant to take the place of seeing a healthcare professional. If you have any concerns regarding your own or someone else's health, we strongly encourage you to consult a physician.

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