What is Meniere's Disease?
Meniere's disease is an inner ear disorder where the patient has episodes of vertigo, i.e. it feels like the patient is spinning around. There is also fluctuating hearing loss along with progressive and eventual permanent loss of hearing, tinnitus (ringing in the ear), and a feeling of pressure or fullness in the ear. In majority of the patients only one ear is affected by Meniere's disease. Individuals of any age can be affected by Meniere's disease; however, it more commonly starts between the age of 20 and 50 years. Meniere's disease is a chronic disorder, however, there are different types of treatment available to relieve symptoms and reduce the long-term impact of this disease on the patient’s quality of life.
Causes of Meniere's Disease
The exact cause of Meniere's disease is not clear. One probable cause, which has not been proved is the abnormal amount of endolymph, the fluid present in the inner ear, that can cause Meniere's disease. This finding is commonly seen on autopsies, but it is not definite whether this causes the episodes of Meniere's disease.
Factors affecting the endolymph which can contribute to Meniere's disease are:
- Abnormal immune response.
- Improper drainage of fluid due to anatomic abnormality or blockage.
- Viral infection.
- Genetic predisposition.
- Head trauma.
There has been no single cause, which has been proven to cause Meniere's disease. So, it is thought to occur as a result of a combination of the above factors.
Signs & Symptoms of Meniere's Disease
- Patient has persistent or recurring episodes of vertigo where he/she experiences a spinning sensation that spontaneously starts and stops. These attacks of vertigo occur without any prior warning and often last around 20 minutes to many hours, however, do not exceed 24 hours. Patient can also experience nausea and vomiting from severe vertigo.
- Hearing loss occurs in Meniere's disease, which has a tendency to come and go, especially in the initial stages. In the end, majority of the patients suffer from some permanent type of hearing loss.
- Tinnitus or ringing in the ear also occurs in the patient where he/she perceives ringing, roaring, buzzing, hissing, or whistling sound in the ear.
- There is a feeling of fullness or stuffiness in the ear, as patients having Meniere's disease commonly feel aural fullness, i.e. pressure in the involved ear or pressure on the side of their heads.
- After a vertigo episode, there may be improvement in the signs and symptoms of the patient or the symptoms can also disappear completely. Episodes can be occur from a few weeks to few years apart.
Diagnosis of Meniere's Disease
Physical exam and medical history is taken and to confirm the diagnosis of Meniere's disease, the patient should have experienced the following:
- There should be at least two episodes of vertigo, lasting about 20 minutes or longer, but not exceeding 24 hours.
- A feeling of fullness/stuffiness in the ear.
- Loss of hearing, which is verified by doing a hearing test.
- Excluding other known causes for the above problems.
Hearing Assessment: Audiometry is a hearing test which assesses how well the patient detects sounds at different volumes and pitches and also how accurately the patient is able to distinguish between words which sound similar. Patients having Meniere's disease commonly have problems with hearing low frequencies or a combination of high and low frequencies with normal ability to hear in mid frequencies.
Balance Assessment: Between the attacks of vertigo, most of the patient’s sense of balance returns back to normal. However, patient may have some continuous problems with balance.
Videonystagmography (VNG) is a test which assesses the balance function of the inner ear by evaluating the movement of the eyes. Sensors which are related to balance are present in the inner ear and these are connected to the muscles, which control the movement of the eyes. Due to this connection, one can move their head while keeping their eyes focused on a point. In the VNG test, warm and cool air or warm and cool water are introduced into the ear canal. Involuntary eye movements, which occur in response to this stimulation, are measured using a special pair of video goggles.
Rotary-chair testing also measures the function of the inner ear based on the eye movement. The patient is made to sit in a rotating chair that is controlled by a computer and which stimulates the patient’s inner ear.
Posturography is a computerized test which shows the part of the balance system on which the patient relies the most and the part that is causing problems. It could be any part of the balance system, such as inner ear function, vision, or sensations from the skin, tendons, muscles and joints etc. During the posturography test, the patient is made to wear a safety harness and stand in bare feet on a platform and tries to maintain his/her balance under different conditions.
Vestibular Evoked Myogenic Potentials (VEMP) testing is a new test which helps in not only diagnosing Meniere's disease, but also in monitoring it. VEMP test reveals the characteristic changes that occur in the affected ears of patients suffering from Meniere's disease.
Video Head Impulse Test (vHIT) is also a new test that utilizes video to measure the reactions of the eye to sudden movement. When the patient focuses on a point, his/her head is quickly and unpredictably turned. If the patient’s eyes shift from the target when the head is turned, then it means that the patient has an abnormal reflex.
Electrocochleography (ECoG) test is used to visualize the inner ear in response to sounds. This test helps in determining if there is an abnormal accumulation of fluid in the inner ear. However, this is not specific for Meniere's disease.
Blood tests and imaging tests, such as MRI scan can be done to rule out those diseases which can produce symptoms which are similar to Meniere's disease. These other medical conditions include brain tumor or multiple sclerosis.
How is Meniere's Disease Treated?
There is no cure for Meniere's disease; however, there are various treatments which can help alleviate the frequency and severity of the vertigo episodes in the patient. There is no treatment for hearing loss that is experienced with Meniere's disease. All the treatments for Meniere's disease aim at stopping the vertigo spells. Some treatment measures are conservative, whereas some are aggressive.
Medications for vertigo can be prescribed to be taken during a vertigo episode to reduce the severity of the attack and they include:
- Medications for nausea, such as promethazine, help in controlling the nausea and vomiting which can occur during a vertigo episode.
- Motion sickness medications, such as diazepam or meclizine helps in reducing the spinning sensation along with helping in alleviating nausea and vomiting.
- Long-Term Medication Use where the doctor will prescribe diuretics, i.e. medications which help in decreasing the fluid retention. In some patients, the frequency and severity of Meniere's disease is controlled with a diuretic. Use of diuretic medication is often combined with restricted dietary salt intake.
Noninvasive Therapies for Meniere's Disease
There are patients who can benefit from noninvasive procedures, which include:
- Hearing aid placed in the affected ear helps in improving the patient’s hearing. The patient can be referred to audiologist to decide which hearing aid options are best for the patient.
- Rehabilitation, such as vestibular rehabilitation therapy, is beneficial in patients who have balance problems between spells of vertigo and this therapy can help in improving the patient’s balance.
- Meniett device is used in patients who have vertigo which is difficult to treat. Meniett device applies pressure to the middle ear which helps in improving the fluid exchange. Meniett pulse generator is a device which applies pulses of pressure to the ear canal via a ventilation tube. This treatment is done at home, about three times daily for five minutes. Meniett device helps in improvement of symptoms such as tinnitus, vertigo, and aural pressure, however, its long-term effectiveness is yet to be determined.
Aggressive Therapies for Meniere's Disease
If the above conservative treatments are not successful, then aggressive treatments are used that include the following:
Middle ear injections where the medications are injected into the middle ear, which then get absorbed into the inner ear help in improving the symptoms of vertigo:
- Gentamicin is an antibiotic which is toxic to the inner ear and will decrease the balancing function of the ear and the other ear takes up the responsibility for balance. This procedure can be performed under local anesthesia and commonly helps in reducing the severity and frequency of the vertigo attacks. However, this procedure also carries the risk of further hearing loss.
- Steroids, such as dexamethasone, can also help in controlling the episodes of vertigo in some patients. This procedure is also done under local anesthesia. However, examethasone can be not as effective as gentamicin, but dexamethasone is also less likely to cause further hearing loss than gentamicin.
Surgical Procedures for Meniere's Disease
Surgery is done if the other treatment modalities do not help the patient and if the vertigo attacks from Meniere's disease are so severe that they are debilitating to the patient. Surgical procedures include:
- Endolymphatic sac procedure helps in alleviating the vertigo by decreasing the production of fluid or increasing the absorption of the fluid. The function of the endolymphatic sac is regulating the levels of inner ear fluid.
- In endolymphatic sac decompression, a small part of the bone is removed which is present over the endolymphatic sac. Sometimes, placement of a shunt is also done with this procedure, which helps in draining the excess fluid from the inner ear.
- Labyrinthectomy is a procedure where the surgeon removes the part that is responsible for balance in the inner ear, hence removing both the hearing and balance function from the ear affected by Meniere's disease. Labyrinthectomy is only done when the patient has had near-total or total hearing loss in the affected ear.
- Vestibular nerve section is a procedure where the vestibular nerve, which connects balance and movement sensors from the inner ear to the brain, is severed. Vestibular nerve section helps in correcting problems with vertigo along with trying to preserve the hearing in the affected ear. This procedure requires general anesthesia and the patient will need an overnight hospital stay.
Lifestyle Modifications for Meniere's Disease
- Patient should immediately sit or lie down when the dizziness or vertigo attack comes. During the vertigo episode, patient should also avoid things that worsen the symptoms, such as bright lights, sudden movement, reading or watching television.
- Patient should be aware that they might lose their balance and fall which could lead to serious injury. Good lighting should always be used if the patient needs to get up during the night. A cane can also be used for walking to provide stability if the patient is suffering from chronic balance problems.
- Always rest during and after the vertigo attacks. Never immediately rush back to the normal activities.
- Patient should also avoid operating heavy machinery or driving a car if he/she suffers from episodes of vertigo.
- Patient should restrict their salt intake, as beverages and foods high in salt, increases the risk of fluid retention and thus increases the risk of vertigo.
- Stress also can trigger vertigo episodes, so it is important that the patient manage stress, which will also help in reducing the severity of the symptoms and help the patient in coping with Meniere's disease.
Complications from Meniere's Disease
- The most difficult complication is the unpredictable episodes of vertigo and the likelihood of permanent loss of hearing from Meniere's disease. This disorder interferes with the patient’s daily life and causes emotional stress, fatigue, anxiety and depression.
- Vertigo from Meniere's disease causes loss of balance thus increasing the risk of falls and accidents when operating heavy machinery or driving.