It hit me like a Bergdorf sack of Jimmy Choos. Spinning, I went down and started to spew, totally mucking my new Armani. My ears were roaring. Mother had buried her third husband with a heart attack, so I knew exactly what was going on. The ER doctor ran a complete heart evaluation, got a CT scan to make sure there was no stroke, drew so much blood that I needed a transfusion, and called in a cardiologist and neurologist. What a shock when all those tests came back normal!
This fashion-conscious patient was experiencing her first episode of Ménière’s disease (MD), but she didn’t know it at the time. Ménière’s is a sporadic disorder of the inner ear causing attacks of vertigo and loss of hearing. In the full-blown form, it consists of four symptoms, two major and two minor:
• Major
Episodes of true vertigo
Fluctuating sensorineural hearing loss
• Minor
Tinnitus
Fullness in the ear.
Ménière’s disease has no known cause. However, a related characterization, Ménière’s syndrome, refers to the same four symptoms but this term is reserved for use when there is a known cause, such as low thyroid levels, Lyme disease, or syphilis.
Ménière’s disease is usually first diagnosed when the patient is in middle age, but may be present for many years prior to diagnosis and can begin as early as puberty. The National Institutes of Health estimates that more than 600,000 Americans suffer from MD and that about 45,000 new cases are identified each year. Ménière’s disease initially affects only one ear, but many times both ears become involved over a period of decades.
Symptoms
Vertigo
The most disabling symptom of Ménière’s disease is vertigo, a feeling of whirling out of control that lasts minutes to hours. Vertigo that lasts only seconds is often due to disorders of the cardiovascular system or benign paroxysmal vertigo (chapter 10). If the spinning lasts more than twenty-four hours, it is more likely stemming from a disorder affecting the brain. Similarly, if there is complete loss of consciousness or there is a shaking seizure, the problem is in the brain or heart. One uncommon variation of MD (called Tumarkin’s otologic crisis) is a sudden fall without loss of consciousness.
The spinning vertigo of MD, often accompanied by nausea and vomiting, may occur in attacks lasting minutes to hours. The attacks can occur several days in a row, weekly, monthly, or maybe not for years. Secondary symptoms, the body’s response to vertigo, include sleepiness, paleness, and sweating. The first experience of severe vertigo often causes fright or panic leading to an emergency room visit. It is common to conduct a CT scan and EKG to rule out the possibility of a stroke or heart attack.
Sensorineural Hearing Loss
Nerve deafness associated with Ménière’s disease is caused by damage to the cochlea and often affects low frequency hearing more than high. This is an unusual pattern. Most ear disorders cause high-frequency hearing loss.
The type of hearing loss is sensorineural (nerve deafness) and it fluctuates or tends to come and go. It is typical for the hearing to get worse for a matter of days, but to mostly improve, leaving a small permanent loss. Over a period of years, these small steps of additional hearing loss can build up and become disabling.
Tinnitus
The tinnitus associated with Ménière’s disease also comes and goes and is often compared to roaring or the sound you might hear with a seashell to your ear. Tinnitus may also be a warning, increasing just before an attack of vertigo. It is thought to be caused by a buildup of inner ear fluid pressure.
Fullness
Fullness of the ear can be associated with low-frequency hearing loss or the buildup of fluid pressure in the inner ear. Like tinnitus, ear fullness or pressure may increase before an acute attack of vertigo and provide warning of an attack.
A Typical Ménière’s Disease Attack
Many people experience warning symptoms that an attack of vertigo and hearing loss is about to occur. This may be a vague feeling of unease or, as mentioned, a more specific feeling such as tinnitus or fullness. Learning to recognize a coming attack is important because it provides time for the individual to sit, put down the baby, or pull the car to the side of the road.
The intense vertigo of MD is incapacitating. The hearing fluctuates, usually getting worse but sometimes getting better after an attack. The eyes may shake back and forth (nystagmus) due to inner ear balance system nerve connections with the visual system. This causes blurred vision and the vertigo may be reduced by closing your eyes. Many people experience severe anxiety because the symptoms can mimic those of a heart attack or stroke. If you have any doubt it is wise to call 911 for help.
Following the spinning sensation, most people continue to feel nauseated, fuzzy-headed, and sleepy. Common treatments of vertigo (Antivert, Dramamine) also cause drowsiness and most people sleep for several hours after an attack, waking somewhat refreshed.
After many years of episodes the severity of vertigo and fluctuating hearing loss is reduced, leading to what is commonly called “burned out Ménière’s.” At this stage, constant imbalance may be more prominent than vertigo. Permanent hearing loss is also present in late-stage MD, often requiring hearing aids and sometimes a cochlear implant.
Cause and Mechanism of Ménière’s Disease
By definition, MD is idiopathic (meaning the root cause is unknown). Speculation includes inflammation (such as would be caused by a viral infection), abnormal circulation, genetics, allergy, and autoimmune disorder. It is also thought that acute inner ear pressure increases can be brought on by caffeine, alcohol, tobacco, salt, and stress (known as CATSS) as well as by anxiety and general illness. As mentioned, in those few patients in whom ear specialists can find a basic cause for MD, the term Ménière’s syndrome is usually used.
Regardless of the underlying cause, quite a bit is understood about what happens in the inner ear that results in the symptoms. The basic mechanism is an increase in inner ear fluid called endolymphatic hydrops. In some ways, MD is similar to glaucoma, which is a buildup of inner eye fluid. Like MD, glaucoma can cause nerve damage, has an unknown root cause, but can be effectively treated. The two disorders are not related and having one does not dispose you to having the other.
Making the Diagnosis
Medical History
Classical Ménière’s disease consists of all four symptoms, but in the early phases only some of the symptoms may be present. Clinically, the diagnosis requires either two major symptoms (vertigo and hearing loss) or one major and two minor symptoms (vertigo or hearing loss plus both tinnitus and fullness). For example, hearing loss and tinnitus may be the earliest symptoms, but the diagnosis of Ménière’s disease is not made with certainty unless vertigo or fullness is also present.
Two variations of classical Ménière’s disease have also been identified: cochlear Ménière’s (no vertigo) and vestibular Ménière’s (no hearing loss or tinnitus). Secondary symptoms include vomiting, hyperacusis (sensitivity to louder sound), poor balance, generalized dizziness, sweating, sleepiness, distortion of sounds, and blurred vision.
When you visit your ear specialist, be prepared to answer questions about your symptoms:
• What is the main reason that you came to the office (e.g., an attack of spinning)?
• What did it feel like? How long did it last? What else did you feel?
• What were you doing just before it happened?
• When was the last time you ate or consumed alcohol before the attack?
• If you have had other episodes, when was the first?
• Describe the duration and frequency of the attacks.
• Do you have any other medical problems?
• Describe your diet, stress level, caffeine intake, and medications.
• Have you been checked by your primary doctor; what tests have been done?
• Does anyone in the family have hearing loss, vertigo, tinnitus, ear fullness, or Ménière’s disease?
Even if you fill out a dizziness questionnaire, be sure to tell the doctor or intake nurse your symptoms. The sentence, “Wait, I need to tell you the rest of my symptoms” can sometimes help professionals focus during a busy day. Too often completed forms may be only briefly scanned. Bring notes with you to help you recall details of your symptoms, but avoid long, rambling tales of woe.
Examination
Your ear specialist will check your vital signs (blood pressure, pulse rate, and temperature), examine your ears, nose, throat, head, and neck, and perform a neurological evaluation based on the inner ear. This includes observing your eye movements, standing with feet together and eyes closed, walking in place with your eyes closed, pointing your fingers at the doctor with your eyes closed, touching your fingers alternately from your nose to the doctor’s moving fingers, and a variety of other quick and easy checks.
Hearing and Balance Tests
The hearing test (audiogram) and basic balance test (video nystagmogram, VNG, see chapter 26 for more on balance tests) are nearly always required. The hearing test not only measures your ability to hear, but also the pattern of any hearing loss. Recall that in Ménière’s disease, the hearing loss is usually worse in the low frequencies. The VNG measures eye movement caused by warm and cool water (or air) placed in the ear canals. Ménière’s disease typically causes a weakness in the response to temperature change, so one ear may have a reduced response.
Other tests can also be very helpful in establishing the diagnosis. Electrocochleography (ECoG) measures electrical signals from the cochlea. One particular ECoG pattern is typical of Ménière’s disease, but this pattern is present only during active phases, when the inner ear fluid pressure is elevated.
Less commonly, glycerol or urea dehydration tests have been used to determine the potential reversibility of hearing loss in MD. These two drugs are fast-acting diuretics and temporarily reduce the fluid pressure on hair cells in the inner ear. Improvement in hearing confirms the diagnosis of MD and indicates that hair cell damage may not be permanent, that is, hearing may get better with treatment.
Imaging and Other Tests
Imaging studies are often necessary to make sure that other disorders are not the cause of symptoms. CT and MRI are used to rule out stroke, tumors, inflammatory, autoimmune, and other neurological disease. CT is also used to identify another condition that can cause symptoms similar to MD, superior canal dehiscence. In this disorder the bone protecting the superior semicircular canal (see chapter 1) is eroded by the pulsations of the brain fluid.
Blood tests including complete blood counts, cholesterol levels, immune system tests, and liver, kidney, and thyroid function studies may be necessary. When the patient’s history suggests the possibility of allergy, syphilis, or Lyme disease, these should also be investigated.
Consultation
Your neurotologist may suspect cardiovascular disorders (like abnormal heart rhythm or high blood pressure) or endocrine disease (like low thyroid levels) to be the cause of your symptoms and ask you to consult with your primary doctor, an internist, or other specialist. Primary neurological diseases like multiple sclerosis or stroke would dictate referral to a neurologist.
Treatment
Treatment of Ménière’s disease is personalized based on individual needs and the extent of disability. It is focused on reducing inner ear fluid pressure or blocking abnormal balance nerve signals from getting to the brain. Treatment can be categorized into three stages:
Stage 1—Non-invasive Treatment
Stage 1 treatment is aimed at reducing inner ear fluid buildup and is effective in 80 percent of patients. This treatment begins with a low-salt diet (beginning at 1500 mg of salt per day). Find more information about Ménière’s diet or low-salt diet by searching these terms on the Internet.
Alcohol should be eliminated because it has its own direct effect on the inner ear as well as the brain. It will also be necessary to gradually eliminate caffeine. If you are drinking more than two cups of coffee per day, reduce your consumption gradually over two weeks or more to limit headache and other withdrawal symptoms. Exercise, proper sleep, and stress reduction are also important in managing this disorder. Your doctor will teach you more about MD—understanding what is going on is very important in controlling it.
Stage 1 treatment also includes the use of oral medications when indicated. The most commonly used medications are mild balance nerve suppressants such as Antivert (meclizine) and Dramamine (dimenhydrinate). These work by reducing the vertigo signals from the inner ear to the brain. Diazepam (Valium) is another vestibular nerve suppressant that also works on the balance centers in the brain, making it more effective for many people than other oral medications. Even though the dose required is lower than that necessary to treat anxiety, diazepam should still be restricted to short periods of use in order to avoid habituation.
Diuretics are sometimes helpful. They are thought to work by reducing fluid pressure. These drugs are safe and complement vestibular nerve suppressant treatment. Patients using diuretics will sometimes need to take potassium supplements.
While diuretics and vestibular nerve suppressants are the mainstay of Stage 1 treatment in the United States, many European neurotologists recommend Serc (betahistine), a vasodilator also used by many American neurotologists. Because its mechanism of reducing hydrops is different than other medications, it can have an additive beneficial effect.
Stage 1 treatment of Ménière’s disease is continued for six weeks or longer, unless symptoms are severe. In 80 percent of people, Stage 1 treatment is successful and medications can be tapered off after a period of months. But the restrictions on caffeine, alcohol, tobacco, and stress may need to be continued indefinitely. In the remaining 20 percent of patients, it is necessary to use Stage 2 or 3 treatment methods.
Table 21.1
Summary of Stage 1 Treatment* for Ménière’s Disease
• Use a low-salt diet
• Avoid caffeine, alcohol, and tobacco
• Reduce stress
• Exercise (try for thirty minutes of aerobic exercise daily)
• Learn about Ménière’s disease
• Take medications
• diuretic
• balance nerve suppressant
• vasodilator
• oral steroids
*Recommended for most patients.
Stage 2—Minimally Invasive Treatment
Stage 2 treatment is used only if the more conservative Stage 1 treatment does not relieve the vertigo. In most cases of Stage 1 treatment failure, dietary, general health, and medical management is continued while adding Stage 2 options. The availability of minimally invasive methods has revolutionized treatment of severe cases of MD over the past decades by reducing the number of people who require major surgery to control vertigo.
Two of the Stage 2 treatments are administered by intratympanic (IT, that is, in the middle ear) injection. The eardrum is numbed in the ENT office by placing a drop of local anesthetic on it. Next, using a surgical microscope, the doctor uses a tiny needle to inject medication through the eardrum and into the middle ear space.
Intratympanic gentamicin is the most commonly used Stage 2 injection, controlling vertigo in about 80 percent of MD patients who do not respond to Stage 1 treatment alone. However, it is an ototoxic drug that can also cause hearing loss as a side effect. By using small doses and repeating them only if necessary, the rate of hearing loss caused by gentamicin is limited to about 10 percent of recipients. Studies show use of a fixed dose, that is, not a dose tied to the patient’s response, causes hearing loss in up to 25 percent of people.
The other commonly used intratympanic medications are steroids. There are several possible mechanisms of action, including reducing inflammation and reducing an immune response. Intratympanic steroids do not cause hearing loss, but are less effective than gentamicin.
Another Stage 2 method is air pressure treatment. A hand-held device pumps pulses of air pressure into the ear canal and through a tube in the eardrum to reach the inner ear. The patient applies the device to the ear for five minutes, three times per day and studies have shown this to be safe and probably effective.
Stage 3—Major Surgery
Surgical management of MD is restricted to patients with frequent, incapacitating attacks of vertigo who did not respond to Stage 1 and Stage 2 treatment. Going on to Stage 3 treatment is necessary in fewer than 10 percent of MD patients. Once common, these operations are now infrequent.
Endolymphatic Shunt
This is the most conservative surgical procedure, designed to preserve both hearing and balance. It is non-destructive, theoretically allowing excess fluid to drain from the inner ear into the mastoid cavity where it is absorbed into the blood. It is done to control vertigo and is effective in up to 80 percent of patients who find no relief from all Stage 1 and 2 treatments. Hearing improvement has also been reported. This operation takes one to two hours under general anesthesia as an outpatient. Complications are infrequent but include hearing loss and spinal fluid leakage.
Vestibular Neurectomy
This operation consists of cutting the balance nerve between the inner ear and brain. It preserves hearing but destroys the balance function of one ear. The opposite ear takes over during the following weeks. It is a permanent cure for vertigo arising in the operated ear but has no effect on MD that may be present in the opposite ear. Unfortunately, it cannot be performed in both ears because cutting both balance nerves would cause permanent incapacity. This operation has the risks and complications that accompany any brain surgery because it requires access through the lining of the brain and cerebrospinal fluid to reach the balance nerves. It requires two to three days of hospitalization.
Labyrinthectomy
Labyrinthectomy is a one- to two-hour operation to remove the inner ear. This procedure eliminates vertigo coming from the operated ear but destroys both hearing and balance in that ear. It can only be performed on one side and only when hearing is already severely impaired. It is completely destructive but has the highest rate of vertigo control.
In summary, Stage 1 treatment is non-invasive, consisting of low-salt diet, exercise, and stress reduction at first, then adding diuretics, vasodilators, and balance nerve suppressants when necessary. Stage 2 treatment is semi-invasive, consisting of injections of medication through the eardrum or pressure treatment through a ventilation tube in the eardrum. Stage 3 treatment consists of major surgery and is required in a small minority of cases with disability caused by vertigo.
Table 21.2
Summary of Treatment Stages for Ménière’s Disease
|
Stage 1 (non-invasive) |
Stage 2 (semi-invasive) |
Stage 3 (surgery) |
|
Diet, reduce stress |
IT* Dexamethasone |
Shunt |
|
Exercise |
IT Gentamycin |
Vestibular neurectomy |
|
Oral medication |
Air Pressure |
Labyrinthectomy |
*IT = intratympanic injection.