Meniere's disease

Introduction

Meniere’s disease is a disorder of the balance and hearing organs in the inner ear.

Patients with Meniere’s disease report symptoms of:

  • Vertigo - the illusion of motion when one is stationary (like the room is spinning)
  • Fluctuations in hearing
  • Tinnitus – ringing of the ears
  • Pressure in the ear

The attacks of dizziness typically last for several minutes to several hours and sometimes cause nausea and vomiting. Meniere’s disease, however, does not produce constant vertigo that lasts for days.

Rarely, patients may develop sudden drop (Tumarkin) attacks in which they suddenly lose control of their posture and fall to the ground. These drop attacks generally occur in patients who have suffered from Meniere’s disease for several years.

Hearing loss, which may or may not be present in Meniere’s disease, usually will worsen in patients with active disease and frequent attacks.

The unpredictability of the vertigo spells can be troublesome, particularly when driving or in situations that require good balance function. In many cases, the incapacitating nature of this disorder and its unpredictability creates anxiety and stress and leads to avoidance behaviors (such as not wanting to go out due to fear of an attack) as a coping mechanism.

Balance disorders may lead to a lack concentration, fatigue, or difficulty in situations with significant motion in the field of vision such as driving, shopping, and action movies.

Diagnosis

A doctor will diagnose Meniere’s disease based on the combination of symptoms and by ruling out other possible neurological problems.

The symptoms of Meniere’s disease can be caused by other diseases so it is important to exclude other problems that may mimic Meniere’s disease. In fact, misdiagnosis of Meniere’s disease is relatively common.

  • In most cases, a hearing test will be ordered.
  • Depending on the results of the examination and hearing test, other tests may be requested including an MRI to visualize the hearing and balance nerves and the brain and an ENG (electronystagmography) to assess the inner ear balance function.
  • Sometimes a referral to a neurologist is helpful to exclude migraines or other neurological diseases that may mimic Meniere’s disease.

Not all tests are necessarily needed and each is ordered when the specific information that they produce is helpful in confirming the cause, the amount of damage, and, in some cases, to determine which ear is affected. In addition to helping confirm the diagnosis, the tests also help guide decisions about which therapies are most appropriate.

Meniere’s Disease and the inner ear

The inner ear has six sensory organs: the cochlea, which senses sound, and five vestibular organs that sense head motion.

Hair cells are the sensory cells in the inner ear that detect sound or head motion. The tips of the hair cells project into a compartment with specialized fluid known as endolymph. The correct composition of this fluid is critical to the function of both the hearing and vestbular hair cells. Abnormalities of the endolymph lead to hearing loss and dizziness.

Details of inner ear anatomy

One of the main functions of the vestibular system is to maintain images steady on the back of the eye during head motion through the vestibulo-ocular reflex (VOR). This reflex stabilizes images on the retinas by sensing head motion and then inducing eye movements in the direction opposite to head movement. For example, if the head moves to the right, the eyes move to the left, and vice versa.

Abnormal activation or inhibition of the vestibular organs in the inner ear forces the eyes to move even though the head is stationary. This creates the illusion that the room or environment is moving, or vertigo.

Causes of Meniere’s disease

Most cases of Meniere’s disease have no specific cause identified. Other cases likely result from prior ear injury or surgery, autoimmune problems, viral infections, or genetic causes.

As the condition progresses, there is increased swelling of the fluid compartment of the inner ear (endolymphatic hydrops). It is believed that vertigo attacks and hearing loss result from rupture of delicate inner ear membranes due to the increased fluid pressure.

Because there is an excess of fluid in the inner ear compartment, factors that affect fluid retention in the body are felt to exacerbate Meniere’s disease. Total body salt (sodium) content is very important since increased sodium content in tissues results in increased fluid retention.

Details of inner ear fluid chambers

Treatment with diet

The mainstay of treatment for Meniere’s disease involves a low sodium diet and drugs to limit fluid overload of the inner ear. Together, these help reduce the frequency and severity of vertigo attacks.

We recommend no more than 1500-2000 milligrams of sodium daily. Even if you limit the salt that you add to food, it is important to check the sodium amounts in pre-made and restaurant foods since they are typically very high in sodium.

As for caffeine or alcohol, their effects on Meniere’s disease remain unclear and are often anecdotal. You might wish to experiment by avoiding them for a period of time to see if they particularly affect you.

Treatment with medications

Most patients benefit from diuretics or fluid pills in addition to a low salt diet. The diuretics that are most commonly prescribed include hydrochlorothiazide (HCTZ), triamterene, aldactone, furosemide, or acetazolamide.

Because some diuretics also cause potassium to be lost in the urine, it’s important to monitor a patient’s potassium levels. Eating fresh bananas, oranges, cantaloupe, or other foods high in potassium is often sufficient to maintain normal potassium levels.

If you develop leg cramps or other symptoms of low potassium, blood levels should be checked. Some patients need to take supplemental potassium to prevent potassium levels from getting too low.

In some patients a short course of steroids, such as prednisone, may be recommended. These seem particularly helpful in patients with a recent exacerbation of the disease. The side effects of prolonged steroid use are significant, so they are typically only prescribed for several days up to a few weeks.

If vertigo does occur, vestibular suppressants such as Compazine, Dramamine, Antivert (meclizine), Phenergan, and Valium (diazepam) are helpful in reducing the severity of the attack. These provide symptomatic relief rather than preventing the attack from occurring, so they usually are needed only when severe dizziness occurs. In addition to relieving vertigo, all vestibular suppressants typically cause drowsiness. Further, when taken on a daily basis they can limit overall balance function since they suppress normal balance reflexes.

Dietary vigilance and medical therapy controls vertigo attacks in approximately 70 percent of patients with Meniere’s disease. Despite control or reduction in the number of vertigo attacks with diet and medical therapy, the hearing loss, tinnitus, and ear pressure often persist. Thus, the primary goal of medical therapy is reduction in the frequency and severity of vertigo attacks.

Surgical treatment

In the minority of patients who fail medical therapy and continue to experience incapacitating  attacks of dizziness, patients and doctors may consider surgical options to help control the dizzy attacks.

Patients with Meniere’s disease should not have to suffer from continued severe vertigo, as it can nearly always be remedied. The types of surgery depend on the hearing level, the amount of balance function in the affected ear and good ear, the age and general health of the patient, and the lifestyle demands placed on the patient.

There are two general classes of surgical options, conservative and aggressive. The conservative approache is non-destructive and involves adjusting fluid levels in the inner ear. The aggressive approach is referred to as destructive and involves removal of certain affected tissues that create the balance function of that ear. Overall balance can be maintained as the brain will compensate and use the other ear’s balance control for overall perception and response to head motion. 

Non-destructive procedures

Endolymphatic Sac Surgery

The endolymphatic sac is the part of the inner ear that is responsible for resorbing inner ear fluid. This procedure aims to facilitate further drainage of fluid from the inner ear. It is a conservative, nondestructive procedure that is often recommended as a first step in attempting to control vertigo attacks.

An incision is made behind the ear and the mastoid bone overlying the endolymphatic sac is removed. The endolymphatic sac is decompressed and opened. Typically a piece of plastic is placed in the sac to facilitate ongoing drainage.

The operation is done under general anesthesia and takes approximately two to three hours. Some patients stay overnight while many feel well enough to go home the same day.

The procedure carries risks associated with any inner ear operation including hearing loss, dizziness, facial nerve weakness, and cerebrospinal fluid leakage, yet these are fortunately rare.

Endolymphatic sac surgery has a ~70% success rate in control of vertigo attacks. It usually does not worsen or improve hearing loss, tinnitus, or pressure.

Following endolymphatic sac surgery, patients are typically asked to maintain a low-sodium diet and continue diuretic therapy until they have gone six months without a severe vertigo attack.

Intratympanic steroid injection

Steroids are felt to relieve vertigo attacks in some patients with Meniere’s disease, especially those with a recent increase in the frequency or severity of attacks.

This is a simple in-office procedure. Using a small needle, a small quantity of steroid solution is injected into the middle ear space through the eardrum. The steroid is then absorbed into the inner ear.

While the long-term effectiveness of the procedure in controlling vertigo attacks has not been firmly established, there is some evidence that it is effective in providing at least temporary relief.

Destructive procedures

Vestibular Nerve Sectioning

This procedure requires an intracranial approach, meaning an opening into the skull. It takes four to six hours and requires a three- to five-day hospital stay.

In this microsurgery, the facial, auditory (hearing), and vestibular (balance) nerves are exposed and the vestibular nerve is cut. The auditory and facial nerves are preserved.

Rarely the separation between the hearing and vestibular nerves is not distinct. In this case, surgeons usually recommend taking more of the nerve rather than less, in order to ensure relief from vertigo, even at the expense of some hearing.

The success rate for relief of attacks of vertigo is 90-95 percent. The risks of the surgery include: deafness, persistent dizziness, facial nerve weakness, meningitis, cerebrospinal fluid leakage, stroke, and bleeding. While these risks are rare, they nevertheless need to be considered.

This operation is typically best for patients who have good hearing, severe vertigo, and who either have failed the endolymphatic sac procedure or wish to undergo a single, definitive operation.

Labyrinthectomy

This is an excellent, safe, definitive destructive procedure in which the balance organs are systematically removed.

Hospital stay is usually two to four days. Hearing will be lost from the procedure so it is recommended for patients with severe vertigo and poor hearing. It has a high cure rate for vertigo (greater than 95 percent), but in elderly with poor vision, or any preexistent brain dysfunction, there may be a problem adjusting to the loss of balance function.

Risks are rare and include persistent dizziness, tinnitus, facial nerve weakness, and cerebrospinal fluid leak. In some patients, a cochlear implant can be placed in the inner ear at the time of surgery to help restore hearing to the affected ear.

Gentamicin Chemical Labyrinthectomy

Gentamicin is an antibiotic that is toxic to the sensory hair cells in the inner ear. It is more toxic to the vestibular hair cells involved with balance compared to the hair cells for hearing.

The drug is injected into the middle ear through the eardrum using a small needle. The antibiotic is absorbed into the inner ear and destroys the hair cells in the vestibular organs.

This procedure is performed in the office and often repeated two to three times to achieve a permanent destruction of the vestibular function in the affected ear.

Because gentamicin also has the potential to destroy hearing and cause tinnitus, it must be used with caution in ears with hearing. Because absorption of the drug into the inner ear is unpredictable and susceptibility to the drug varies, the end results vary from patient to patient. Therefore, some patients may still have instability because of the incomplete loss of vestibular function.

This is a good technique in patients too ill to undergo surgery or who have poor hearing. It is still a destructive procedure and the compensation issues raised above pertain to this procedure.

A final thought

Each of these procedures is designed to alleviate vertigo in patients with intractable Meniere’s disease. Treatment of Meniere’s disease depends on many individual factors and a frank discussion with your doctor is necessary to determine the best treatment strategy in your personal case. The information presented here is to reiterate and supplement discussions you have had with your doctor.

 

Last reviewed: 
April 2018

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