HOME Meniere's disease affects a part of the inner ear known as the labyrinth. The labyrinth is a system of tiny fluid filled channels which send signals of sound and balance to the brain. Meniere's disease causes the fluid in the labyrinth to build up, disrupting both your balance and hearing. Attacks of Meniere's disease may be frequent, or occur only every few months. Sometimes, they can last for several minutes, while at other times they can last for as long as 24 hours. The length and severity of attacks cannot be predicted. As M��'s disease is an unpredictable and sometimes difficult condition to manage, it can leave you feeling stressed, anxious and depressed. M��'s disease can have a significant impact on your day-to-day lifestyle. For example, the condition can affect your work, and it can be particularly dangerous for those with M��'s disease to use ladders, scaffolding and to operate machinery.

Meniere's disease, also called idiopathic endolymphatic hydrops, is a disorder of the inner ear and is one of the most common causes of dizziness originating in the inner ear. In most cases only one ear is involved, but both ears may be affected in about 15% of patients. Meniere's disease typically starts between the ages of 20 and 50 years. Men and women are affected in equal numbers.

Meniere's disease is a progressive condition, which means it will gradually get worse the longer you have it. The disease will normally begin by affecting one ear but, in 30% of cases, symptoms will progress to both ears. Although hearing usually returns to normal after an attack, repeated pressure increases can cause serious damage to the hearing cells which is why, in late stage Meniere's disease, hearing loss is often permanent.


The symptoms of Meniere's disease vary from person to person and can occur without warning, and at irregular intervals.

Vertigo is usually the most troublesome symptom of Meniere's disease. It is defined as a sensation of movement when no movement is occurring. Vertigo is commonly produced by disorders of the inner ear, but may also occur in central nervous system disorders. The vertigo of Meniere's disease occurs in attacks of a spinning sensation and is accompanied by disequilibrium (an off- balance sensation), nausea, and sometimes vomiting. The vertigo lasts for 20 minutes to two hours or longer. During attacks, patients are usually unable to perform activities normal to their work or home life. Sleepiness may follow for several hours, and the off-balance sensation may last for days. As it is difficult to predict when an attack will occur, keep any vertigo medication that you have to hand so that it is easily accessible. As vertigo can leave you disorientated and unbalanced, you should avoid activities such as driving, operating dangerous machinery, using ladders or scaffolding, or going swimming.

Tinnitus is the perception of noise, in your ear or head, which is generated from inside your body, rather than coming from outside. It is usually more noticeable when you are tired and at quiet times when there is less background noise to distract you from the sounds tinnitus causes. Tinnitus can also sometimes make it difficult to sleep, leaving you tired and more susceptible to stress.

There may be an intermittent hearing loss early in the disease, especially in the low pitches, but a fixed hearing loss involving tones of all pitches commonly develops in time. Loud sounds may be uncomfortable and appear distorted in the affected ear.

The tinnitus and fullness of the ear in Meniere's disease may come and go with changes in hearing, occur during or just before attacks, or be constant. The symptoms of Meniere's disease may be only a minor nuisance, or can become disabling, especially if the attacks of vertigo are severe, frequent, and occur without warning.

1. Follow a strict low-salt diet - excess accumulation of salt is known to cause Meniere's disease, so reduction of salt in the diet is the obvious first part of the treatment.
2. Increase blood flow and circulation - the reduction of blood flow to the inner ear is known to aggravate Meniere's disease, so prevention and treatment of poor circulation in the inner ear is the obvious second part of the treatment. All forms of tobacco cause reduction in the blood supply to the ear and other parts of the body, and must be stopped completely if treatment has any hope of success. All forms of allergy can result in the reduction in blood supply to the ear, and should be eliminated, as far as possible, and who cannot be eliminated should be treated with an antihistamine and/or steroid. The foods most likely to produce allergic reactions are milk, corn, wheat, eggs, peanuts and chocolate, and should be avoided. Many diseases cause a reduction in blood flow in the body. Tests must be done for unsuspected diabetes, excesses of cholesterol and triglycerides, etc.
3. Excessive nervousness, stress and fatigue exert harmful effect on the inner ear, and can be relieved by a mild tranquilizer, such as alprazolam, etc.

Positional Vertigo, also known as "Benign Positional Vertigo", "Benign Paroxysmal Positional Vertigo" or "BPPV", is caused by tiny calcium particles that have broken free from the balance detectors in the inner ear and are floating in the inner ear fluid. These "floaters" become trapped in the balance canals of the inner ear and cause them to become overly sensitive to head movements. They cause a spinning sensation (vertigo) that is brought on by head movements or changes in position and typically lasts for several seconds. The spinning dizziness is especially noticeable when the head is turned in a particular direction, such as when getting out of bed in the morning or rolling over in bed. The vertigo usually subsides as the crystals settle to the bottom of the inner ear from gravity but the feeling of being off balance or light headed may persist. The dizzy spells of Positional Vertigo may be debilitating and are sometimes accompanied by blurred vision, nausea and vomiting.

Positional Vertigo may be treated with repositioning maneuvers and exercises, such as Semont Exercises, in which the head is held in a series of different positions to allow the floaters to fall out of the balance canal to another part of the inner ear to lessen their effect. Additionally, Balance Exercises and Vestibular Rehabilitation (physical therapy for the balance system) may be helpful in reducing the dizziness and effects of Positional Vertigo. If medical treatment and balance exercises do not provide relief from the vertigo, a procedure called Inner Ear Perfusion can help reduce or eliminate the dizziness.

Perfusion of the Inner Ear with Streptomycin/Dexamethasone reduces the sensitivity of the balance receptors in the inner ear so that the patient's dizzy spells are greatly reduced even though the "floaters" may still be there. Inner Ear Perfusion is easily performed as a simple outpatient procedure in which a small amount of medication is injected through a tiny hole in the ear drum into the middle ear space once daily for three consecutive days. Dizziness is a symptom which can be used to describe many different sensations depending on the patient who is experiencing it. These symptoms can be brought on by a number of different disorders. Patients often describe their dizziness as being lightheaded, feeling unbalanced, and/or experiencing a spinning sensation. These are all commonly referred to as dizziness but each can arise from completely different causes. Metabolic, neuromuscular, and cerebrovascular disorders as well as tumors or trauma are common causes for dizziness but each is experienced in a different way and has its own different method of treatment. This is why it is imperative when seeking help from a physician about dizziness, you must see someone who is specialized enough to discern between the different types of dizziness and identify the primary cause before treatment can be rendered. Tinnitus is the perception of noise in the ear or head, which is generated inside the body rather than coming from outside. Tinnitus is usually only heard by the person with the condition, but in a very few rare cases it can also be heard by other people. Temporary tinnitus is common if you have a cold, after exposure to loud noise such as at a music concert, or following a blow to the head. About 1 in 100 people experience serious problems with long-term, established tinnitus. The sounds can be heard in one ear, both ears, or can appear to come from the middle of the head. The condition is more common in older people, but it can occur at any age, even in quite young children. It often gets worse at quiet times, such as when you are trying to get to sleep. This is because there is less background noise to mask or distract you from the sounds associated with tinnitus. Tinnitus may also be more noticeable when you are tired.

It is estimated that around 10% of the population are affected by some degree of tinnitus. For most people it is an irritation they learn to live with, but for others it can lead to poor concentration, difficulty in sleeping and depression.

The most common cause of tinnitus is damage to the hearing nerves in the ear (cochlea or inner ear). We hear things through a stream of nerve impulses going from the cochlea to the auditory system in the brain. If the tiny nerves in the ear are damaged or destroyed, this produces an abnormal stream of impulses, which the brain interprets as a sound. This causes the noise associated with tinnitus.

Tinnitus in older people is usually caused by natural hearing loss (presbyacusis), which lessens the sensitivity of hearing nerves. In younger people, it is most often caused by damage to hearing as a result of excessive noise.

However, there are a number of other causes of tinnitus. It is not always possible to identify the exact cause even after examination by a specialist.

Other common causes include:

• excessive wax in the ear causing it to become blocked
• middle ear infection (otitis media) or glue ear (serous otitis media)
• otosclerosis (stiffening of the tiny bones which transmit sound from the eardrum to the sound-detecting organ, the cochlea)
• Meniere's disease
• anaemia (in which the thinner blood circulates so rapidly it produces sound)
• pierced eardrum
• exposure to sudden or very loud noise (i.e. gunfire, explosion, loud music)

Otosclerosis is an excessive growth in the bones of the middle ear which interferes with the transmission of sound.

The middle ear consists of the eardrum and a chamber which contains three bones called the hammer, the anvil, and the stirrup (or stapes). Sound waves passing through the ear cause the ear drum to vibrate. This vibration is transmitted to the inner ear by the three bones. In the inner ear, the vibrations are changed into impulses which are carried by the nerves, to the brain. If excessive bone growth interferes with the stapes ability to vibrate and transmit sound waves, hearing loss will result.

Otosclerosis is classified as a conductive disorder because it involves the bones of the ear, which conduct the sound to the nerve. If a person has hearing loss classified as neural, the nerve conducting the impulses to the brain is involved.

Otosclerosis is a common hereditary condition. About 10% of the caucasion population has some form of otosclerosis, however, it is rare among other ethnic backgrounds. Women are more likely than men to suffer from otosclerosis. It is the most common cause of conductive hearing loss between the ages of 15-50, but if the bony growth affects only the hammer or anvil, there are no symptoms and the condition goes undetected. Disease affecting the stapes is also associated with progressive hearing loss.

Autophony is a life-spoiling problem usually relieved by Eustachian tuboplasty.

Autophony is a rare but annoying complaint caused by a continuously open Eustachian tube, the narrow passageway between the back of the nose and the middle ear. Most of the time the Eustachian tube is closed, except when you swallow, during which air passes up the tube into the middle ear. When the Eustachian tube is open most of the time, as in autophony, air goes up the tube into the middle ear with each breath, causing an annoying roaring noise in the ear. In addition, when you speak, your own voice reverberates in your middle ear in such an annoying way you don't want to speak above a whisper, so as not to cause this reverberating noise in your ear. Patients with autophony, which literally means "hearing your voice in your own ear", begin to speak out in a normal way, but when the annoying noise of their own voice appears in their ear, they drop their voice to a whisper. The condition can become so annoying as to make you reclusive, not wanting to be around others and required to speak. Some patients have it so bad outside sounds are also annoying to the affected ear, but less so than the patient's own voice. Singing is especially annoying to those with autophony.

The cause of autophony is not known since there have been no ear bones examined after death with this condition. Careful CAT scans of the Eustachian tube in patients with autophony show atrophy of the soft tissues of and surrounding the tube, so it remains open most of the time. One of the peculiar diagnostic features of autophony is it is always relieved by lying on the back, or bending forward at the waist, both of which close the tube. Some patients with severe autophony will hold the tip of the finger against the side of the neck on the affected side, which closes the end of the tube and gives relief. Other patients will "sniff" continuously to close the end of the tube in the nose to get relief.

The typical history preceding the onset of autophony is weight loss, which presumably causes the loss of fat and connective tissue in and surrounding the tube, leaving it open most of the time.

Another important associated condition with autophony is autoimmune disease of other parts of the body, the most important being of the urinary bladder (Interstitial Cystitis), facial nerve (Bell's Palsy), lungs (Wegener's Granuloma), inner ear (Meniere's Disease), central nervous system (Guillain-Barre Syndrome), the intestines (Crohn's Disease), and the joints (Rheumatoid Arthritis). Just how autoimmune disease, in which you become allergic to your own tissues and your immune system attacks them, relates to autophony is not known.

The typical history preceding the onset of autophony is weight loss, which presumably causes the loss of fat and connective tissue in and surrounding the tube, leaving it open most of the time.

Another important associated condition with autophony is autoimmune disease of other parts of the body, the most important being of the urinary bladder (Interstitial Cystitis), facial nerve (Bell's Palsy), lungs (Wegener's Granuloma), inner ear (Meniere's Disease), central nervous system (Guillain-Barre Syndrome), the intestines (Crohn's Disease), and the joints (Rheumatoid Arthritis). Just how autoimmune disease, in which you become allergic to your own tissues and your immune system attacks them, relates to autophony is not known.

Whether it causes or contributes to the atrophy of the walls of the Eustachian tube, so that it remains open most of the time, is not known, but presumably it must. Only about one-third of patients with autophony have autoimmune disease.

Most patients have autophony on one side only, but rarely is it in both, and it is more common in women then men, middle age or older.

Through the years, there have been many treatments for this condition. As the pathology of autophony as become better understood, reducing the size of the Eustachian tube, which gives immediate relief to most patients, has become the treatment of choice. Since the Eustachian tube is too large, too wide open, and does not close between swallows, it is possible to reduce the size of the Eustachian tube by inserting a length of silicone tubing, 1mm in diameter, into the middle ear end of the tube, past the narrow place (isthmus). For those with a very large Eustachian tube, it may be necessary to partially block the tube by inserting a rod of silicone sponge, as used in scleral buckling operations, into the tube. The patient awakens from anesthesia with complete relief, often crying for joy. Some patients need a temporary ventilation tube in the drum to prevent fluid accumulation in the middle ear.

For those patients with associated autoimmune disease of the urinary bladder (Interstitial Cystitis), facial nerve (Bell's Palsy), lungs (Wegener's Granuloma), inner ear (Meniere’s Disease), central nervous system (Guillain-Barre Syndrome), the intestines (Crohn's Disease) or the joints (Rheumatoid Arthritis), etc., these conditions should be treated in the usual way, usually with steroids and other anti-inflammatory drugs. Strangely enough, large doses of steroid and anti-inflammatory drugs, usually beneficial to the associated autoimmune disease, do not help autophony, apparently because these drugs cannot reverse the atrophy of the Eustachian tube, which is present in autophony.

As we have learned more about the normal function and pathology of the middle ear and Eustachian tube, annoying diseases like autophony lend themselves to reasonable treatment, such as narrowing the Eustachian tube with silicone tubing or sponge, known as Eustachian tuboplasty.

Two typical case reports will explain the loss of quality of life-style from autophony, and the relief gained by successful operation.

One of my first patients with autophony successfully treated was a 73-year old man with heart failure, accompanied by weight loss and autophony. He was so sick his own doctor gave him no more than a 50% chance to survive the anesthesia for the operation, but he was so miserable with the autophony, he accepted the risk gladly. Fortunately, he survived the operation and got a good result from Eustachian tuboplasty, and lived many more years with heart disease, but no more autophony.

Another younger patient, a mother with two children, has severe Interstitial Cystitis (inflammation of the bladder), an autoimmune disease, and then developed autophony. She was so miserable with the autophony she held her hand to the side of her neck all the time to close the Eustachian tube, to get relief from her autophony. After Eustachian tuboplasty, she got complete relief from her autophony and has been able to deal with her Interstitial Cystitis without removal of her bladder, which had been offered to her as an alternate treatment. An ear that functions normally is one in which air goes up the Eustachian tube into the middle ear with each swallow(far left drawing). If the Eustatian tube gets blocked then air cannot get into the middle ear, and fluid accumulates behind the drum (center left drawing). In some patients, MIDDLE EAR FLUID can be relieved by inflating the ears (holding the nostrils and forcing air into the ears). If this does not get air into the ears, MIDDLE EAR FLUID can be relieved by the use of Mathes inflation bulb by swallowing. During swallowing, the mouth of the Eustachian tube is opened, and the passageway into the lungs (larynx) is closed. If you put the small inflation bulb in either nostril and close the other and squeeze the bulb exactly at the same time you swallow, you can usually get air into each middle ear. This relieves the vacuum in the middle ear, displace the fluid, and relieves the hearing loss (center right drawing). After getting air up the Eustachian tube into the middle ear, fluid drains down the tube out of the middle ear, and hearing is improved. This may need to be repeated from time to time to prevent its recurrence, especially during the winter cold or summer allergy season. Sometimes an antihistamine, such as Chlortrimeton 4 mg, can be taken 30 minutes before inflation to facilitate opening the Eustachian tube and getting air into the ears (far right drawing).

 

Acoustic neuromas are noncancerous growths. These growths are located deep inside the skull and are adjacent to vital brain centers. The first symptoms that a patient notices are related to the ear and include ear noise, hearing loss, and dizziness. As the acoustic neuroma enlarges, it involves other surrounding nerves and brain areas which control more vital functions. Headaches may develop as a result of increased pressure on the brain. If allowed to continue over a long period of time, this pressure on the brain is ultimately fatal. In most patients, these tumors grow slowly over a period of years. In others, the rate of growth is more rapid. In some patients, the symptoms are few and mild, while in others, many severe symptoms develop quickly.

Great care is exerted before, during, and after operation in these patients in order to preserve life. The preservation of life is the most important objective of operation in these most difficult patients. A secondary objective of operation is to preserve as many vital structures as possible. For many, a completely normal life results following operation. Some patients experience minimum physical disabilities. For a few patients, maximum degrees of physical disability may persist. To accomplish the preservation of life with a minimum of disability, removal of the acoustic neuroma is performed by a team, including an internist, an anesthesiologist, a specially trained surgical nurse, a neurosurgeon and a neuro-otologist. The neurosurgeon operates with the neuro-otologist.

Risks and complications of acoustic neuroma surgery vary with the size and rate of growth of the tumor. In general, the larger the tumor, the more difficult to remove and the more serious the complications. The removal of an acoustic neuroma, whether large or small, is a major surgical procedure, with possibilities of serious complications, including death. Acoustic neuromas are classified as small, medium, or large

• A small acoustic neuroma is still confined within the bony canal that extends from the inner ear to the brain. The hearing, balance, and facial nerves, and the blood vessels which supply the inner ear pass through this canal.
• A medium-sized acoustic neuroma extends from the bony canal into the brain cavity, but it has not yet produced pressure on the brain itself.
• A large acoustic neuroma has extended out of the bony canal into the brain cavity and is large enough to produce pressure on the brain

The choice of surgical approaches depends upon the size of the tumor and the amount of residual hearing. It is usually not possible to save the hearing. If hearing is saved, it is no better than the preoperative level and may be worse. The larger the tumor, the less the chances for hearing preservation. With poor preoperative hearing or a large tumor, it is better to sacrifice the hearing in order to remove the entire tumor. All procedures are performed under general anesthesia.

• Translabyrinthine Approach - This involves an incision behind the ear. The mastoid and inner ear structures are removed to expose the tumor. The tumor is totally removed. Only rarely is partial removal performed. The mastoid defect is closed with fat taken from the abdomen. The translabyrinthine approach sacrifices the hearing and balance mechanism of the inner ear. As a result, the ear is permanently deaf. Even though the balance mechanism has been removed on the operated ear, the balance mechanism from the other ear takes over and provides stabilization for the patient in one to four months.
• Middle Fossa Approach - An incision is made above the ear, and the brain is elevated to expose the tumor. The tumor is totally removed in most patients. Every effort is made to preserve hearing and still remove the tumor. In about 50% of patients, the tumor involves the hearing nerve or the artery leading to the inner ear and total loss of hearing occurs in the operated ear.
• Retrosigmoid Approach - An incision is made behind the ear, and the brain is elevated to expose the tumor. The tumor is totally removed in most patients. Every effort is made to preserve hearing and still remove the tumor. In some patients, it is necessary to sacrifice the hearing to remove the entire tumor. In about 50% of patients, the tumor involves the hearing nerve or the artery leading to the inner ear and total loss of hearing occurs in the operated ear. Following this approach, some patients may experience persistent headaches.
• Radiation Therapy - Since acoustic neuromas are benign growths and are not cancerous, we do not routinely advise radiation treatment. Radiation therapy is not risk-free and does not result in disappearance of the tumor. Hearing loss, facial paralysis, and serious complications have also occurred after radiation therapy. After this treatment, some patients have experienced continued tumor growth and have required surgical removal, which is much more difficult due to the effects of the radiation.

Rupture of a delicate membrane in your inner ear, in a "weak" place from birth that bursts and allows the potassium-rich endolymph to escape into the perilymph space and come into contact with the "hair cells" that do the hearing and damage them. This causes sudden hearing loss, the feeling of fullness, etc., and noise in your ear, plus the loss of balance, and dizzy spells. Some of these ruptures heal, and all or part of the hearing returns. Most do not heal, and little or no healing returns. The loss of balance and dizzy spells disappear more slowly, together with the feeling of fullness and noise.

Loss of the energy enzyme system in the inner ear, the cause of which is not known. Most such losses are not so sudden and not complete, with no loss of balance. Most such sudden hearing losses improve after dexamethasone perfusion.

Virus infections, such as cold, flu, sinusitis, etc., which spread to the inner ear. There is no direct treatment, although 4 mg of dexamethasone daily and dexamethasone perfusion may be of some benefit.

Loss of circulation to the inner ear from blockage of an artery, or hemorrhage from an artery, or an aneurysm. Most patients with sudden hearing loss due to loss of circulation have evidence of poor circulation in other parts of the body, especially the brain, such as a prior stroke. There may be some recovery of hearing after such a sudden hearing loss with inhalation of 5% carbon dioxide and 95% oxygen for thirty minutes, four to eight times a day, if done soon enough after the loss.

There are about 25,000 such "spontaneous" sudden hearing losses in the United States each year, most of which do not recover, with or without treatment. The chance of having a sudden hearing loss is approximately 1 in 10,000 each year. After you have had one such sudden hearing loss in one ear, the chance of another sudden hearing loss in the other ear is much greater, perhaps as much as 1 in 100. Special tests must be done, including brain-stem audiometry, or MRI with gadolinium, to eliminate the possibility of a tumor on the balance and/or hearing nerve (acoustic neuroma) as a cause of the sudden hearing loss.

Such a "spontaneous" sudden hearing loss, without obvious cause, must be distinguished from a "traumatic" sudden hearing loss due to a known cause, such as a loud noise, sudden pressure change, head injury, drugs, Meniere's Disease, autoimmune sensorineural hearing loss, etc., for which the treatment is very different.

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