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Dizziness and Balance Disorders-Meniere's Disease

Meniere's Disease

Ménière’s disease
A brief history
What causes Ménière’s disease?
Prognosis
Diagnosis of Ménière’s disease or hydrops
AudioMetric Testing
Medical management/treatment
Vestibular rehabilitation therapy
Surgical treatment

Ménière’s disease

One of the most common problems of the ear is Ménière’s disease.  In order to be diagnosed with Ménière’s, a triad of symptoms must accompany vertigo or dizziness.  These three symptoms include fullness or pressure in the ear, tinnitus or ringing in the ear, and a fluctuating sensorineural hearing loss.  There can be variants of Meniere’s disease, which lack the features listed above.  These variants are usually referred to as Cochlear Hydrops or Vestibular Hydrops.  Pressure or fullness in the ear without the other symptoms may be related to other ear problems, as may dizziness alone, or a hearing loss without accompanying tinnitus, fullness, or vertigo.  Back to Top

A brief history

Prosper Ménière was a French Physician who described a new disease process in the mid-1800’s.  The disease he identified consisted of four symptoms: attacks of vertigo, ear fullness or pressure, low-pitch tinnitus (ringing/roaring), and fluctuations in hearing.  At the time, the cause of the ailment was unknown, but Dr. Ménière believed that it originated within the ear.  Since that time much has been learned about the disease and its treatment; interestingly, however, we still do not know the cause of the disease.  There are now known to be multiple variants of this disease process, with similar pathologic consequences (abnormal tissue changes).  The variants may affect only hearing, or balance, while another variant may have a few, but not all, of the classic symptoms (i.e. fluctuating hearing loss with tinnitus, but no fullness or dizziness).  Back to Top

What causes Ménière’s disease?

The inner ear contains an area called the labyrinth.  It has a bony portion, and inside that bony portion is a membranous portion.  Inside the membranous area are two different fluids, endolymph and perilymph, which are separated by a thin membrane.  The fluids help transmit sounds and head movement information to the brain through the inner ear.  All forms of Ménière’s disease involve an increase in endolymph fluid pressure.  The endolymph pushes against the membrane, and eventually, the membrane ruptures and the two different inner ear fluids mix.  This results in damage to the specialized cells (hair cells) that send information to the brain via the hearing nerve.  This process is called “endolymphatic hydrops”, meaning too much endolymph.  Only the classic four-symptom complex described by Ménière himself should be referred to as Ménière’s disease, whereas all the other forms are more properly termed “hydrops” or “endolymphatic hydrops.”  All patients with Meniere’s disease have hydrops, but not all patients with hydrops develop Meniere’s disease.  The precise cause of the excessive fluid is unknown and whether it is due to an overproduction of endolymph or an under-resorption is debated.  There are specific cells that produce fluid and certain areas in the inner ear that absorb the fluid.  Anatomic development of the inner ear may have some influence on how the fluid is absorbed.  Genetically, an individual may be more inclined to develop Meniere’s disease.  The inner ear also appears to be immunosensitive and factors affecting the individual’s immune system may trigger increased production of the fluid because of the adverse effects on certain cells within the inner ear.  Either way, the net result is too much endolymph with distressing symptoms.  Although the etiology (cause) of “Ménière’s disease” is unknown, there are many known triggers of endolymphatic hydrops.  These include allergies, immune system problems, metabolic disorders, infections (syphilis), congenital malformations of the ear, and trauma. Back to Top

Prognosis

Hydrops is a chronic disease, like diabetes or hypertension and needs to be treated medically (low sodium diet and diuretic) for many years.  Similar to other chronic diseases, it can often be well controlled with medical therapy and lifestyle changes.  This fact needs to be taken into account when surgical options are considered.  Certain individuals with hydrops can develop the disease in the opposite ear, which occurs in 5-30% of patients with hydrops.  If an individual has had Ménière’s disease in one ear for three or more years, without symptoms in the other ear, the likelihood of getting Ménière’s in the other ear is quite rare.  As a general rule, younger individuals are more likely to have bilateral disease than older patients.  The vertigo associated with hydrops can burn out overtime (usually over several years or in the mid 50s).  Back to Top

Diagnosis of Ménière’s disease or hydrops

The discussion you have with Dr. Krueger, including the history of the symptoms and neurotologic exam is the most important aspect of making the diagnosis.  Additionally, advanced audiometric and vestibular testing will be necessary to make the proper diagnosis.  Often imaging studies will also be necessary to rule out tumors or other intracranial pathology.

Vertigo/dizzy component

The vertigo spells are classically episodic, not usually brought on by movement, but often occur abruptly.  Vertigo (sensation of rotation or motion) in Meniere’s attacks is often preceded by other ear symptoms, such as ear fullness, an increase in ringing (tinnitus), or the sudden drop in hearing.  Rarely, an improvement in hearing precedes the attack (Lermoyez syndrome).  The vertigo attack typically lasts anywhere from 20 minutes to an entire day.  The vertigo is typically described as a spinning sensation (either the environment or the individual is perceived as spinning), which is quite disabling, and often associated with severe nausea and vomiting, sweating, and occasionally diarrhea.  When having an attack, the patient is better when lying down and motionless, as further movement will aggravate the symptoms.  After an attack of vertigo, one may experience a period of unsteadiness lasting for hours to days, because of the persistent irritability of the inner ear and inability of the brain to compensate for the labyrinthine abnormality.  The Tumarkin variant of Meniere’s has sudden loss of postural control with "drop attacks" and is thought to be due to involvement of the otolithic component of the inner ear.  These can be rather violent episodes where patients are seemingly pushed or thrown to the ground. Back to Top

Hearing loss component

Hearing loss in Meniere’s disease is quite variable, ranging from a sudden, severe loss without return of function, to a gradual decline over months to years as the episodic fluctuation of hearing continues to occur with the dizzy spells.  The most typical pattern is that of hearing fluctuations, with periods of good and poor hearing, associated with a gradual decline in word recognition.  The classic audiometric feature of Ménière’s disease consists of a low frequency sensorineural hearing loss during an attack that may (or may not) return to normal after a spell.  Essentially, any pattern of hearing loss may be associated with hydrops; therefore, the audiometric pattern can not be solely relied upon to make the diagnosis.  A demonstrable fluctuation in hearing in one ear or the other assists in the diagnosis of hydrops. Back to Top

Tinnitus/ringing in the ear component

Classically, a low-pitched roaring sound is heard in the affected ear prior to, or during an attack of vertigo.  This too is highly variable, as some individuals experience constant ringing (tinnitus) in the ear, which increases in loudness during the vertiginous spells.  After the dizziness resolves, the tinnitus either goes away or decreases to the previous intensity level. 

Pressure/aural fullness component

A sensation of fullness or pressure in the ear usually accompanies the vertigo attack.  Not unusually, patients with Meniere’s disease or hydrops are quite sensitive to changes in barometric pressure, or high allergens in the atmosphere, and may experience more attacks in the spring and fall.  The fullness or pressure sensation is thought to arise from over-distension of the endolymphatic compartment.  Hormonal changes that occur during menses and pregnancy may also trigger an attack.  Episodes can more often occur at times of increased tension, stress, anxiety, or exhaustion. Back to Top

Audiometric testing

A long list of disease processes must be excluded prior to making the diagnosis of hydrops or Ménière’s disease.  Tests of hearing, balance, and occasionally imaging are often performed.  Pure tone and speech audiometry, tympanometry, acoustic reflex testing, as well as electrocochleography (ECoG), are routinely performed at Ears of Texas, PA.  Advanced balance and vestibular function testing are also completed.  When the diagnosis of Meniere’s disease or hydrops is considered, an ABR, or imaging studies using an MRI or CT scan with contrast often will be required to ensure that the symptoms are not caused by a tumor.  It has been shown that nearly 1 patient in 10 with a tumor on the balance nerve will demonstrate “classic Ménière’s symptoms.”  Although these tumors are uncommon, imaging remains an integral part of the diagnostic workup. Back to Top

Medical management/treatment

Once a diagnosis of Ménière’s disease is established, non-surgical treatments are attempted as first-line therapy to control the vertigo and other symptoms.  Lifestyle modifications are recommended, including a low salt diet, and controlling known triggers of the disease.  Regular exercise may help control some of the stress and can help the central nervous system compensate for the labyrinthine abnormality.  Medications such as a diuretic (water pill) often are prescribed to eliminate excess fluid in the inner ear.  Since Ménière’s disease can be precipitated by environmental and food allergies, allergy testing and treatment are often recommended and performed at Ears of Texas, PA.  While medications (e.g. Meclizine/Antivert, Phenergan, Diphenidol, Transderm Scopolamine, and Valium) can be given during times of disabling vertigo episodes, it is best to otherwise avoid the long term use of vertigo suppressant medications since they can delay overall recovery by preventing central compensation.  For those patients who suffer from the disease, the symptoms can be life altering and dramatically affect ones ability to function in normal daily living.  Vestibular rehabilitation, by a specially trained therapist, has been proven to be extremely useful in speeding recovery and improving balance function when the patient has persistent unsteadiness due to the labyrinthine abnormality.  It is particularly helpful in the post-operative care after having a labyrinthectomy (removal of balance system) or vestibular nerve section (cutting the balance nerve to the brain) to remove the physical components related to dizziness and allows the brain to compensate.  Back to Top

Vestibular rehabilitation therapy

Often, the balance system has been permanently damaged by the vertigo attacks from Ménière’s.  This creates a chronic sensation of imbalance or lightheadedness.  Vestibular rehabilitation therapy has been proven to be highly effective in relieving these symptoms by improving the central balance system (within the brain).  A personalized program is designed to improve the weaknesses of the balance system, which may include problems with vision, proprioception, and vestibular input.  Vestibular rehabilitation therapy is often combined with other therapeutic modalities such as medications or allergy immunotherapy in controlling or improving the symptoms of dizziness.  It is often an integral part of the post-operative care if a patient has undergone a procedure such as a vestibular nerve section or labyrinthectomy.

Surgical treatment

When the symptoms of Meniere’s disease are not controlled and the patient is incapacitated, there are several options.  They include:

  • Gentamycin injections – This is an injection of an antibiotic in the middle ear, which diffuses across the round window membrane to chemically denervate the inner ear balance function on the affected side.  This is done in the office setting, using a topical anesthetic.  It does have a risk of an associated sensorineural hearing loss with each single injection, and although it can stop vertiginous episodes, it can result in a patient having chronic ataxia or unsteadiness.  It usually is best utilized if a prior vestibular neurectomy has not removed all of the functioning vestibular nerve fibers, or in older patients, who are not good candidates for general surgery.
  • Labyrinthectomy – This is a surgical procedure which removes the semi-circular canals, utricle, and saccule (e.g. all parts of the balance system within the inner ear).  The cochlea is not removed.  This procedure is done under general anesthesia and is very effective in eliminating the vertiginous episodes, but removes all hearing on the operated side, because it removes a portion of the inner ear.  Generally, it will control the dizziness in 95% of instances.
  • Vestibular nerve section – This procedure is quite effective is eliminating the vertiginous episodes and can preserve the hearing in the operated ear in most instances.  Generally, it will control the dizziness in 95% of those patients with intractable vertigo caused by Meniere’s disease.

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