Jonathan Hazell FRCS
Director, London Tinnitus and Hyperacusis Centre.
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In 1861 Dr Prosper Ménière wrote a now classic description of the condition which now bears his name. As the doctor in charge of the Imperial Institute for Deaf Mutes in Paris, he realised that what had previously been thought of as a form of apoplexy was due to a disturbance of the inner ear.
"A man, young and robust, suddenly without reason, experienced vertigo, nausea and vomiting. He had a state of inexpressible anguish and prostration. The face was pale and bathed in sweat as if about to faint. Often, and at the same time, the patient, after seeming to stagger in a dazed state, fell on the ground unable to get up. Lying on his back he could not open his eyes without his environment becoming a whirlpool. The smallest movements of the head worsened the feeling of vertigo and nausea."
Ménière's description of the vertigo which accompanies a severe attack of Ménière's disease, cannot be bettered, although many people do not experience this extreme form.
We now know that the condition is caused by an increase in the pressure of fluids in the inner ear. The cochlea (concerned with hearing) and semi-circular canals (concerned with balance) are filled with fluid which is called endolymph. Periodic increases in the pressure of the endolymph (sometimes called endolymphatic hydrops) produce a dramatic disturbance of the hearing and balance at the same time. In addition to the giddiness or vertigo there is a loss of hearing in the affected ear, together with tinnitus which is generally low pitched or rushing. After the attack the hearing and tinnitus can improve, and there may be long periods of time when the patient is entirely free of symptoms.
The full blown condition affects about 1 in 20,000 of the population. It is more common in men than women. If the condition is untreated, the hearing tends to become progressively worse, although in the early stages the hearing often returns to near normal levels.
Although tinnitus can be a distressing part of Ménière's disease, particularly in the later stages, it is usually the vertigo and vomiting which trouble the majority of patients. The attacks are unpredictable, and finding someonene lying on the floor, in a public place, being sick, does not always bring out the most charitable feelings in other people.
Many patients with Ménière's disease are successfully diagnosed and treated by their general practitioner. There are, however, many more common causes for vertigo which can be misdiagnosed as Ménière's disease. Sometimes the term is used quite wrongly as a diagnosis for any kind of balance disturbance. It is important to make sure that you do have this condition, and not something else, as there are some very specific treatments for Ménière's disease which do not work in other conditions, and vice versa. Here are some specific features of Ménière's disease:
1.It first appears in relatively young people (usually around the age of 30)
2.If your first attack of vertigo is in your 70s then it is likely to be something else.
3.It is usually, but not invariably, associated with hearing symptoms in one ear, for example, fluctuating hearing in the low frequencies, tinnitus and sensitivity to
4.The hearing symptoms should occur at or around the time of the attacks of vertigo.
5.The hearing symptoms are usually experienced in one ear, not equally in both ears.
6.It is quite common to have a feeling of pressure in the affected ear before or during the attack. Sometimes this is the worst symptom.
7.The attacks of vertigo usually last from two to twenty-four hours. The spinning is often very fast and is often aggravated by moving the head. It is often
accompanied by vomiting, and sometimes diarrhoea, although these symptoms may get better as time passes.
8.There are often other "autonomic" effects such as sweating, palpitations and anxiety which can be the results of the release of too much adrenaline associated with
the attacks. These symptoms can accompany any severe vertigo.
9.There are periods of "remission" when patients feel quite normal. These may be as short as a few days or longer than ten years.
If you have many or all of these symptoms, then it is very probable that you do have Ménière's disease. If you have none of these symptoms then it is still possible to make a diagnosis of Ménière's disease, but only in a specialist hospital department, and usually as a result of further tests and investigations. If your symptoms of vertigo are very different, then it is important to question the diagnosis of Ménière's disease. Where possible, it helps to have some special tests performed to be quite certain that the diagnosis is correct. A pure tone audiogram measures the hearing in each ear, at different frequencies and is used to diagnose Meniere's disease. Many who have Ménière's disease suffer from severe discomfort from loud sounds, although their hearing is impaired, and this feature (sometimes called recruitment or hyperacusis) can be measured (by audiological tests).
Investigation of the balance disorder is complicated and takes a long time. One test which is commonly performed is the caloric test. Each ear is gently irrigated with water (or air), which is at a slightly different temperature from that of the body. This changes the temperature in the inner ear fluids, causing them to move in one or other direction. Examination of eye movements during this procedure can show how well the balance mechanism in each ear is working. In Ménière's disease there is often a reduction in the function of the affected ear on caloric testing. Many patients have an understandable fear of this investigation, as it might produce slight giddiness for a minute or so. However, it is an important investigation. It is not distressing if it is performed with care and it yields important diagnostic information which can help in the patient's further management.
Management of Ménière's disease
Many forms of treatment are very effective, and may bring about long periods of freedom from the condition. After a while many patients cease to have disabling vertigo. But as treatment may be needed over a long period of time, it is important to find a doctor with an interest in the condition, and heed his or her advice about what may be a continuing programme of treatment and care.
Because there is an increase in fluid pressure in the inner ear, most patients benefit from reducing salt intake which can cause fluid retention. Some specialists recommend keeping the general fluid intake down as much as possible, and also steering clear of caffeine (although this is not proven). Salt substitute can be obtained at chemists and used in cooking, but should not contain any sodium.
No two patients with Ménière's disease are alike, and as the frequency of attacks and course of the condition are very unpredictable, it is often hard to say whether the treatment is being effective. As there are often emotional factors at work, even the calm assurance of a competent practitioner sometimes produces periods of remission.
Serc (betahistine) probably helps more Ménière's patients than any other drug, and is said to have a direct action on the endolymph production in the inner ear - usually 16mgs three times a day. Most patients with Ménière's disease will have tried it, and it can be taken for long periods of time without ill-effect. Betahistine should be taken in combination with a salt-free diet and should be given initially over a period of some months and occasionally years. In resistant cases 'high dose' Serc has been very effective (up to 32 mgs three times a day). Some patients who have severe attacks of vertigo need strong anti-vertigo drugs such as Stemetil. It is often useful to have these available as a suppository, as tablets may not be absorbed during an attack. If attacks occur very infrequently it is much better not to take Stemetil-like preparations on a regular basis, but to rely on tablets or suppositories which can be used to give rapid relief as soon as the onset of an attack can be predicted. Another newer method of taking Stemetil is by a buccal preparation (Buccastem) which is placed inside the upper lip and is absorbed rapidly through the mucous membrane. Many patients feel a greatly increased confidence if they have a current supply of suppositories or buccal preparation which are effective in rapidly getting rid of the unpleasant symptoms of Ménière's. There is a very large number of different anti vertigo tablets, many of which may be helpful at one time or another, and successful treatment is often a matter of identifying the drug most helpful to the individual.
This usually affects only one ear, and while there is one normal ear there may well be no difficulty in hearing in normal situations. In a minority of patients, Ménière's disease may develop in the second ear. A trial of a suitable hearing aid should always be offered to anyone with a hearing difficulty, and because of loudness discomfort or recruitment, this may need to incorporate a device for reducing the amplification of uncomfortable loud sounds and be fitted on an 'open' or well vented-mould. National Health hearing aids are available which incorporate these devices. Occasionally loudness discomfort is a serious problem, and when one ear is affected, a good fitting earplug (such as the EAR plug) may be helpful in very noisy environments. Ear plugs should not be used however for sensitivity to normal every-day sounds.
Although tinnitus is not usually the most troublesome symptom, it is often relatively simple to treat in Ménière's disease. The tinnitus in Ménière's disease may be easily managed by a suitable white noise generator and counselling (TRT), and often by a hearing aid alone. Patients with Ménière's disease whose vertigo responds to drug treatment may also experience a reduction in tinnitus; this is one of the few examples we have of successful treatment of tinnitus with tablets. However, drugs such as Serc do not have any effect on the tinnitus (or vertigo) associated with conditions other than Ménière's disease.
If all hearing has been lost in one ear and vertigo persists, a destructive operation can be performed down the ear canal (labyrinthectomy). This should not be contemplated when any useful hearing remains in the affected ear, in view of the possibility of second ear involvement. Other operations reduce the pressure of endolymph (for example drainage of the endolymphatic sac - preferred by the author) or they may involve cutting the nerve of balance, where intractable
vertigo persists. The endolymphatic sac is a small cul-de-sac coming from the inner ear, which acts as a "kidney" to the inner ear, helping to remove its waste products and control pressure change. The drainage or decompression of this sac is often effective at controlling vertigo, and sometimes results in an improvement in the hearing and tinnitus, at least in the short term. This operation can be repeated (sometimes after a few years) if the drainage tube becomes blocked, with subsequent further improvement in the vertigo. Because true Ménière's is such a rare condition, not all ear surgeons have experience of doing this operation. It is well worth going to a centre where there is a special interest in treating Ménière's disease.