More than 150 years after it was first recognised as an inner ear problem, Meniere’s Disease is still misdiagnosed and misunderstood, writes David Becconsall, B.App.Sc. Physiotherapy, vertigo specialist physiotherapist.
What is Meniere’s Disease?
French physician Prosper Meniere was the first to theorize that attacks of vertigo, ringing in the ear (tinnitus) and hearing loss, came from the inner ear rather than from the brain. This idea, formed in the 1860s, was finally accepted and Dr Meniere’s name has been synonymous with the condition ever since.
Meniere’s disease is a chronic, incurable vestibular (inner ear) disorder that causes repeated attacks of dizziness due to increased pressure caused by excess fluid build-up in the inner ear. Fluids in the inner ear chambers are constantly being produced and absorbed by the circulatory system.
Any disturbance in this delicate relationship resulting in over-production or under-absorption of these fluids can lead to increased pressure producing dizziness, hearing loss and tinnitus (ringing or buzzing in the ears).
Who gets it and why?
Meniere’s disease can develop at any age, but it is more likely to happen to adults between 40 and 60 years of age. The exact number of people with Meniere’s is difficult to measure because no official reporting system exists. Numbers used by researchers differ from one report to the next and from one country to the next. Support group Meniere’s Australia reports that around one in every 600 people have the condition. Around 90 per cent have it in one ear while 50 per cent can expect to develop it in both. The National Institutes of Health estimates around 615,000 people in the US have it and 45,500 new cases are diagnosed there each year.
Despite extensive research, the exact cause and reason why Meniere’s starts is unknown. Factors thought to increase its likelihood include genetics, circulatory and metabolic functions, viral infection, allergies, an autoimmune reaction, migraine, toxic or emotional unbalance.
The leading theory is that attacks result from increased pressure from excessive fluid in the inner ear and/or from the presence of potassium in this area where it doesn’t belong. Some people with Meniere’s find that certain triggers set off attacks. These include stress, overwork, fatigue, emotional distress, illnesses, pressure changes, certain foods and too much salt in the diet.
Meniere’s disease may start with fluctuating hearing loss and is often characterized by attacks of dizziness that vary in frequency and duration from once or more a day to once a year, lasting a few hours to 24 hours. It is this unpredictability that makes it challenging to manage.
Hearing loss and head noise usually accompany attacks, which can occur without warning. Violent spinning, whirling and falling, associated with nausea, vomiting and a sensation of ear pressure or fullness are also common.
If attacks do recur, they are usually less severe and shorter than the first. In between episodes, sufferers tend to be asymptomatic making diagnosis difficult as Meniere’s markers are only present at the time of symptoms.
Occasionally hearing impairment, head noise and ear pressure occur without dizziness. This type of Meniere’s disease is called Cochlear Hydrops.
Similarly, episodic dizziness and ear pressure may occur without hearing loss and tinnitus; this is called Vestibular Hydrops.
During an attack of early-stage Meniere’s disease, symptoms can include any of the following symptoms:
- spontaneous, severe vertigo
- fluctuating hearing loss
- aural fullness and/or tinnitus
- blurry vision
- nausea and vomiting
- cold sweat
- palpitations or rapid pulse
Oncoming attacks are often preceded by an “aura,” or the specific set of warning symptoms including balance issues, dizziness, light-headedness, headache, hearing loss or increased tinnitus and sound sensitivity. Paying attention to these can allow a person to move to a safe or more comfortable situation before an attack.
After the attack, a period of extreme fatigue or exhaustion often occurs, prompting the need for hours of sleep. The periods between attacks are symptom-free for some people and symptomatic for others.
Many symptoms have been reported after and between attacks including concentration difficulty, fatigue, palpitations, cold sweat, light-headedness, nausea, neck ache or stiffness, unsteadiness, particularly in dim light and vision problems.
Late-stage Meniere’s disease refers to a set of symptoms rather than a point in time. Hearing loss is more significant and is less likely to fluctuate. Tinnitus and/or aural fullness may be stronger and more constant. Attacks of vertigo may be replaced by more constant struggles with vision and balance, including difficulty walking in the dark or in visually stimulating surroundings and occasional sudden loss of balance. Sometimes, drop attacks (Tumarkin’s otolithic crisis) occur characterized by a sudden brief loss of posture without losing consciousness.
Unfortunately, no treatment currently exists to cure Meniere’s. However, it can be eased by medication or surgery as well as diet modification, stress reduction, exercise and natural therapies.
Medical options are aimed at improving the inner ear circulation and controlling fluid pressure changes in this area. Some medical treatments aim to reduce the severity of an attack while it is occurring while others attempt to reduce their severity and frequency in the long term.
Surgical options block the movement of information from the affected ear to the brain. In the most severe cases, destroying the inner ear so that it does not generate balance information to send to the brain or destroying the vestibular nerve so this information is not transmitted to the brain, can relieve the patient of disabling symptoms.
Medical and dietary management
Medical treatment is effective in decreasing the frequency and severity of attacks in 80% of patients. Treatment may consist of medication to stimulate inner ear circulation, decrease inner ear fluid pressure or prevent inner ear allergic reactions. Vasodilating drugs are used to stimulate inner ear circulation and are prescribed together with anti-dizziness medication such as Serc (betahistine HCl) as a vestibular suppressant for Meniere’s disease.
Vasoconstricting substances have the opposite effect and should be avoided. These include caffeine (coffee) and nicotine (cigarettes).
Some medications are prescribed for attacks to reduce vertigo, nausea/vomiting or both. These include diazepam (Valium), lorazepam (Ativan), promethazine (Phenergan), dimenhydrinate (Dramamine Original Formula), and meclizine hydrochloride (Antivert, Dramamine Less Drowsy Formula).
Meniere’s may also be caused or aggravated by metabolic or allergic disorders. Special diets (reduced sodium) or drug therapy (diuretics to control water retention) are good for conservative, long-term treatment.
Vestibular rehabilitation therapy (VRT) is widely used to help imbalance that can plague people between attacks. Its goal is to help retrain the body and brain to process balance information.
The success of such programs is dependent on the patient’s compliance and practitioner’s experience to monitor and moderate a tailored exercise program. This needs to take account of the patient’s tolerances and limitations with movement, head motion, imbalance, visual dependency and postural intolerances.
Other non-surgical options
Around 20% of people do not respond to medication or diet modification. In these cases, a physician may recommend a treatment that involves more physical risk such as intratympanic injections. The aminoglycoside antibiotic (Gentamicin) is injected into the ear which selectively destroys vestibular tissue.
This procedure is usually reserved for patients with Meniere’s in their only hearing ear, or in both ears and when other medical options have not been successful. Recently, intratympanic steroid injections have been used which have led to less risk of hearing loss and persistent imbalance.
Surgery is successful in relieving acute attacks of dizziness in the majority of patients. Residual hearing and tinnitus may improve, stay the same or worsen.
Two categories of surgery are available. The goal of the first type is to relieve the pressure on the inner ear such as ‘endolymphatic shunt’ and ‘cochleosacculotomy surgery’. However, these are not as widely used now due to unfavourable statistics on long-term effectiveness. The goal of the second type of surgery is to block the movement of information from the affected ear to the brain. The process involves either destroying the inner ear so that the ear does not generate balance information to send to the brain, or destroying the vestibular nerve so that balance information is not transmitted to the brain.
In either instance, vestibular physiotherapy is useful to help the brain compensate for the loss of inner ear function due to surgery. Examples of this type of surgery include transcanal (oval window) labyrinthectomy, translabyrinthine labyrinthectomy and section of the vestibular nerve, retrolabyrinthine section of the vestibular nerve, and middle fossa section of the vestibular nerve.
Dealing with Meniere’s on a day-to-day basis is challenging because attacks are unpredictable, the prognosis is difficult due to its erratic nature – symptoms can remain the same, disappear one day and never return or become more severe and disabling – and the fact that it is incurable. However, there are many things sufferers can do to alleviate symptoms and live life to the full.
Firstly, ensure you inform all your friends and colleagues that you have the condition and how it affects you. Because the symptoms of Meniere’s are invisible to others, it is important to make people aware of it. Explain what might happen before and during an attack and how they can help. Also, relate the importance of a low sodium diet.
As well as educating them about the effectiveness of this dietary regimen, it helps to have the support of others to stick to it. Ensure that you have appropriate medication and know what to do before, during and after an attack. Experts suggest lying down on a safe, firm surface and avoiding head movement. Focusing on a stationary object about 50cm away can help. Rest in bed or sleep afterwards for a short period but getting up and walking around as soon as possible is said to help the brain readjust quicker.
Most importantly, visit your doctor or a vestibular physiotherapist to learn about ‘reset’ exercise programs to regain your best equilibrium.
This article originally appeared in Hearing HQ Magazine Aug 15