AUTHOR AND EDITOR INFORMATION

Section 1 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

INTRODUCTION

Section 2 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Background

Evaluation and management of dizziness and vertigo can be one of the most difficult medical tasks. Sources of imbalance can range from simple conditions (eg, dehydration) to serious conditions (eg, brain tumors). CNS problems must be distinguished from circulation anomalies, chemical and hormonal imbalances, and peripheral inner ear disorders. Often, this distinction is not clear.

This article covers a form of peripheral inner ear disorders, specifically, endolymphatic hydrops and Ménière syndrome.

Endolymphatic hydrops refers to a condition of increased hydraulic pressure within the inner ear endolymphatic system. Excess pressure accumulation in the endolymph can cause a tetrad of symptoms: (1) fluctuating hearing loss, (2) occasional episodic vertigo (usually a spinning sensation, sometimes violent), (3) tinnitus or ringing in the ears (usually low-tone roaring), and (4) aural fullness (eg, pressure, discomfort, fullness sensation in the ears).

The term endolymphatic hydrops is often used synonymously with Ménière disease and Ménière syndrome. Ménière disease and Ménière syndrome are both believed to result from increased pressure within the endolymphatic system. However, Ménière disease is idiopathic, whereas Ménière syndrome can occur secondary to various processes interfering with normal production or resorption of endolymph (eg, endocrine abnormalities, trauma, electrolyte imbalance, autoimmune dysfunction, medications, parasitic infections, hyperlipidemia).

The distinction in nomenclature is similar to that of Bell palsy. When the source of facial paralysis is known, Bell palsy is not the diagnosis. Similarly, when the cause of vertigo is known, Ménière disease is not the diagnosis; the diagnosis is Ménière syndrome.

In other words, Ménière syndrome refers to endolymphatic hydrops caused by a specific condition (eg, thyroid hormone disease, inner ear inflammation due to syphilis). Ménière disease refers to endolymphatic hydrops with unknown etiology; it is the catch term for idiopathic endolymphatic hydrops.

Pathophysiology

Two fluids fill the chambers of the inner ear: endolymph and perilymph. These fluids are separated by thin membranes, which house the nervous tissue of hearing and balance. Fluctuations in pressure of these fluids stress these nerve-rich membranes and can cause hearing disturbance, ringing in the ears, vertigo, imbalance, and a pressure sensation in the ear.

More specifically, episodes of hydrops are probably caused by an increase in endolymphatic pressure that causes a break in the membrane separating the perilymph (a potassium-poor extracellular fluid) and the endolymph (a potassium-rich intracellular fluid). The resultant chemical mixture bathes the vestibular nerve receptors, leading to depolarization blockade and transient function loss. Sudden change in the vestibular nerve firing rate creates an acute vestibular imbalance, giving the sense of vertigo.

Physical distention caused by the increase in endolymphatic pressure leads to mechanical disturbance of the otolithic organs. Because the utricle and saccule are responsible for linear and translational motion detection, rather than angular and rotational acceleration, irritation of these organs may produce nonrotational vestibular symptoms.

This physical distention causes mechanical disturbance of the organ of Corti as well. Distortion of the basilar membrane and the inner and outer hair cells may cause hearing loss and tinnitus. Because the apex of the cochlea is wound much tighter than the base, the apex is more sensitive to pressure changes than is the base. This explains why hydrops affects low frequencies (located at the apex) more than high frequencies (located at the relatively wider base of the cochlea).

Symptoms improve after the membrane is repaired and normal sodium and potassium concentrations are restored.

Frequency

United States

Although probably underestimated, approximately 1,000 cases per 100,000 people occur. A familial predisposition seems to exist; approximately half of the patients have a notable family history of this disease.

Mortality/Morbidity

Although the disease itself is not fatal, significant morbidity can arise from various manifestations of the disease. Vertigo can cause devastating accidents and falls. Hearing loss is often progressive over time. Many patients are unable to continue working and are forced to claim disability.

Sex

Although sex-related rates are nearly equal, a slight female preponderance may exist, in the range of 50-65%.

Age

• In some studies, the mean age among treatment groups was 49-67 years.
• Ménière disease has been seen at almost all ages.
• Typical onset begins at early-to-middle adulthood.

CLINICAL

Section 3 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

History

The typical history involves episodic attacks of true whirling vertigo. These episodes are usually preceded by a variable sense of ear pressure and fullness, decreased hearing, and a low-tone roaring tinnitus.

• Episodes of vertigo usually last minutes to hours and are often associated with severe nausea and vomiting. In general, patients feel "run down," imbalanced, and nauseated for several hours to days after an acute attack.
• The timing and frequency of the attacks vary. Some patients can regularly predict when they will have an attack. Others note a completely random pattern. Attacks have been linked to triggers such as foods, menstrual cycles, and psychosocial stress.
• Some patients are completely asymptomatic between episodes. However, many notice progressive deterioration of hearing and balance function with each successive attack.

Physical

Results of the physical examination vary depending on the disease phase. Physical examination results may be completely normal during remission, particularly if the patient is asymptomatic.

• During an acute attack, patients generally have severe vertigo and are often in significant distress.
• • Many present to the physician's office with signs of recent vomiting.
  • Patients are occasionally diaphoretic and pale.
  • Vital signs may show elevated blood pressure, pulse, and respiration.
• Neurotologic examination may reveal significant nystagmus. Pneumo-otoscopy of the affected ear may elicit symptoms or cause nystagmus.
• • In general, the Romberg test (ie, observation of sway or loss of balance while the patient is standing with his or her eyes closed) reveals significant increase in instability and worsening with the eyes closed.
  • If the patient is able to stand with his or her eyes closed, the Fukuda marching step test (ie, observation of directional drift while the patient is marching with his or her eyes closed) may show significant deviation.
  • The Dix-Hallpike test (ie, observation of nystagmus while moving a patient from sitting to supine with the head angled 45° to 1 side and then the other) may be positive, indicating coexisting benign positional vertigo.
• Hearing is frequently affected.
• • The Weber tuning fork test usually shows results lateralized away from side of the affected ear.
  • In general, the Rinne test indicates that air conduction remains better than bone conduction.
• Perform a complete neurologic evaluation during the physical examination is important. New-onset vertigo may be an early sign of stroke, migraine, or brainstem compression, which require emergency care vastly different from those of Ménière disease.

Causes

A wide range of disorders (eg, metabolic imbalance, hormonal problems, infections) may cause increased endolymph pressure. Autoimmune diseases (eg, lupus, rheumatoid arthritis) may cause an inflammatory response in the labyrinth. Allergy has also been implicated in many cases of difficult-to-treat Ménière disease.

DIFFERENTIALS

Section 4 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Other Problems to be Considered

Acoustic neuroma and other brain tumors
Autoimmune processes (especially lupus, rheumatoid arthritis, sarcoidosis, periarteritis, giant cell arteritis, Susac and Cogan syndromes)
Congenital anomalies
Diabetes
High cholesterol or triglyceride levels
Inner ear inflammation
Labyrinthitis
Lyme disease
Microvascular compression syndromes
Multiple sclerosis
Neurosyphilis
Otosclerosis
Perilymphatic fistula
Salt or water imbalance
Thyroid disease
Thyroid hormone disease
Transient ischemic attacks and stroke
Trauma
Vestibular migraine
Viral infections

WORKUP

Section 5 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Lab Studies

• A panel of blood tests is used to rule out obvious metabolic imbalances, infections, and hormonal problems. Tests of the following should be performed:
• • Thyroid stimulating hormone (TSH), T4, and T3 to rule out hyperthyroidism and hypothyroidism
  • Glucose to rule out diabetes
  • Sedimentation rate and antinuclear antibody to rule out autoimmune disorders
  • Urine to rule out proteinuria and hematuria and indicators of otorenal syndrome
  • CBC count to rule out anemia and leukemia
  • Electrolyte levels to rule out salt/water imbalance
  • Fluorescent treponemal antibody (FTA-ABS) to rule out neurosyphilis and Lyme disease
• Allergy testing is needed for allergy-mediated Ménière disease.

Imaging Studies

• MRI of the brain is used to detect the following:
• • Abnormal masses or anatomy, specifically, acoustic neuromas or other cerebellopontine angle lesions
  • Other lesions (eg, multiple sclerosis, Arnold-Chiari malformations)
  • Tumors (rarely found but should be ruled out)
• CT scans are used to detect the following:
• • Dehiscent superior semicircular canals
  • Widened cochlear and vestibular aqueducts

Other Tests

• Audiometry is particularly helpful to document present hearing acuity and subsequent fluctuations. Occasionally, patients fail to notice loss at specific frequencies.
• • A low-frequency or mixed low- and high-frequency loss may develop. Typically, lower frequencies are affected more often than higher frequencies because of preferential sensitivity of the apex to hydrops.
  • Administering multiple hearing tests to document fluctuating hearing loss is helpful in diagnosing Ménière disease.
• Transtympanic electrocochleography (ECoG) is used to detect distortion of nerve-containing membranes of the inner ear (presumably due to pressure fluctuations of the perilymph pressure) and may show evidence of cochlear involvement.
• • ECoG is used to measure the ratio of summating potential (probably arising from movement of the basilar membrane) and action potential on the nerve in response to auditory stimuli. Hydrops is suggested when the ratio is greater than 35%.
  • ECoG is most accurate when Ménière disease is active.
• Electronystagmography (ENG) and videonystagmography (VNG) is performed to test vestibular function of the inner ear, particularly that of the semicircular canals.
• • Typically, endolymphatic hydrops causes reduced vestibular response in the affected ear, but increased vestibular response may develop secondary to an irritative lesion.
  • ENG is used to determine responsiveness of the inner ear to movement and caloric stimulation. It tests central and peripheral function and can help localize lesion sites.
  • Perform ENG when the patient has an empty stomach and when therapy with meclizine, antihistamines, and sedatives has been discontinued for 2 weeks, as these drugs may alter the test results.
  • ENG may cause dizziness and nausea.
• Direction of spontaneous nystagmus during or after an episode of Ménière syndrome is not a reliable indicator of the lesion site. An irritative phase may occur during the attack (fast phases directed toward involved ear), followed by a paretic phase (fast phases directed toward the opposite ear).

TREATMENT

Section 6 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Medical Care

Medical therapy is directed toward the mitigation of symptoms and/or their prevention.

In general, medications that decrease symptoms (eg, meclizine [Antivert], droperidol [Inapsine], prochlorperazine [Compazine], diazepam [Valium], lorazepam [Ativan], alprazolam [Xanax]) only mask the vertigo. These masking agents are vestibulosuppressants and work by dulling the brain's response to signals from the inner ear.

Some diuretics or medications with diuretic-like properties (eg, hydrochlorothiazide and triamterene [Dyazide], hydrochlorothiazide [Aquazide], acetazolamide [Diamox], methazolamide [Neptazane]) decrease fluid pressure in the inner ear. These medications help prevent attacks but do not help after the attack is triggered.

Steroids have also been helpful in treating endolymphatic hydrops because of their anti-inflammatory properties. Steroids can reverse vertigo, tinnitus, and hearing loss, probably by reducing endolymphatic pressure. Steroids can be given orally, intramuscularly, or even transtympanically. Although the transtympanic route is controversial, it is gaining wider acceptance throughout the otologic community.

Aminoglycosides are a class of antibiotics that were serendipitously discovered to be preferentially toxic to the vestibular (balance) end organ. Destruction of the vestibular end organ renders the brain insensitive to fluctuations in inner ear pressure brought on by Ménière disease. Given systemically, aminoglycosides affect both ears. Although aminoglycosides can be used to treat extremely severe bilateral Ménière disease, such treatment leaves the patient with little or no balance function. The resulting complete loss of inner ear function (ie, Dandy syndrome) can be debilitating.

One innovation in the treatment of Ménière disease is the Meniett device. Its use is not precisely a medical treatment, and the device itself does not require surgical installation. It does, however, require insertion of tympanostomy tube so that the device can work; therefore, its use may qualify as a surgical treatment. The Meniett device delivers pulses of pressure to the inner ear via the tympanostomy tube. Although no one knows exactly why this works, some patients have symptomatic relief when the device is used on a daily basis. Because it is new, long-term results have not been fully evaluated.

The histamine agonists that are used in countries outside of the United States must be mentioned. Medications such as betahistine (Serc) are widely used in Europe and South America. Although its mechanism of action is somewhat controversial, many have reported success with its use in mitigating symptoms of Ménière disease. Unfortunately, since betahistine (Serc) is not US Food and Drug Administration approved, it is not discussed much in the United States.

Surgical Care

Surgical treatment is discussed in detail in the article Inner Ear, Ménière Disease, Surgical Treatment. Briefly, the 4 most generally accepted management options are endolymphatic sac decompression or shunt, vestibular nerve section, labyrinthectomy, and transtympanic medication perfusion.

Consultations

Because many differential diagnoses exist, consultations with the following specialists are recommended:

• Neurologist
• Cardiologist
• Endocrinologist
• Internal medicine specialist

Diet

• Patients who are not severely affected may choose to manage their disease through diet.
• Dietary management of endolymphatic hydrops is based on the avoidance of substances that may trigger or exacerbate fluid pressure buildup in the inner ear. As with systemic hypertension, the goal of managing Ménière disease is to reduce the total volume of body fluid. This reduction, in turn, may reduce the fluid volume in the inner ear.
• Avoidance of salt is one of the mainstays of therapy because sodium seems to play a major role in fluid retention in the inner ear.
• • Patients should avoid foods with high sodium content (eg, pizza, preserved foods, smoked fish).
  • Sodium nitrate is used in many preserved and smoked foods; therefore, these foods are high in sodium content.
  • A rigid salt-restricted diet is recommended (ie, 1.5 g sodium), under the guidance of a nutritionist.
• Avoidance of other trigger substances has also been shown to help. The following should be avoided: caffeine; nicotine; chocolate, which has shown to be a potent trigger substance; tobacco; alcohol, particularly red wine and beer; foods with high cholesterol or triglyceride content; foods with high carbohydrate content; and excessive sweets and candy.

Activity

Endolymphatic hydrops does not preclude regular activity. Exercise is recommended in moderation. Because of the unpredictable nature of the disease, balance-intensive dangerous tasks (eg, ladder use, precarious activities) should be avoided.

MEDICATION

Section 7 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Medical therapy is directed toward the mitigation of symptoms and/or their prevention. Only a few representative drugs from each category are included below.

Drug Category: Vestibulosuppressants

These agents decrease symptoms and, in general, only mask vertigo. They work by dulling brain's response to signals from the inner ear.

Drug Category: Benzodiazepines

These drugs appear to potentiate effects of gamma-aminobutyric acid (GABA) and facilitate inhibitory GABA neurotransmission and other inhibitory transmitters by binding to specific receptor sites.

Drug Category: Corticosteroids

These agents are helpful in treating endolymphatic hydrops because of their anti-inflammatory properties; steroids can reverse vertigo, tinnitus, and hearing loss, probably by reducing endolymphatic pressure.

Drug Category: Diuretics

These drugs help prevent attacks by decreasing fluid pressure load in the inner ear but do not help after the attack is triggered.

FOLLOW-UP

Section 8 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Further Inpatient Care

• Further inpatient treatment is directed toward controlling nausea, vomiting, and dehydration in patients with acute vertigo.
• Intravenous or intramuscular diazepam can be used in addition to antinausea medication, which may provide excellent vestibular suppression and antinausea effects.
• Steroids can be used for their anti-inflammatory effects in the inner ear.
• Intravenous fluid support can help prevent dehydration and replace electrolytes.

Further Outpatient Care

Vestibular Rehabilitation: Because of its fluctuating nature, vestibular therapy is not particularly useful as a primary treatment of Ménière disease. However, it is useful in the rehabilitation of patients who have undergone vestibular ablation. In fact, vestibular rehabilitation is strongly recommended in those who have undergone aminoglycoside perfusion, labyrinthectomy, and vestibular nerve section.

Complications

• Accidents due to vertigo spells
• Disability due to unpredictable vertigo
• Progressive imbalance and deafness
• Intractable tinnitus
• Benign positional vertigo: More evident suggests that patients who have Ménière's disease manifest with benign positional vertigo.

Prognosis

• The prognosis of patients with Ménière disease varies.
• • The pattern of exacerbation and remission makes the evaluation of treatment and prognosis difficult. In general, the patient's condition tends to spontaneously stabilize over time. Ménière disease is said to "burn out" over time.
  • Studies show that about one half of all cases of vertigo spontaneously stabilize after several years.
  • However, this stabilization comes at a price. Patients are left with poor balance and poor hearing.
• Some authors classify Ménière disease into several stages of progression.
• • Early stages involve cochlear hydrops, which then proceed to affect the vestibular apparatus. Ménière syndrome is most bothersome to patients during these early stages.
  • Episodes of vertigo disappear in later stages when room for pressure fluctuation is eliminated by complete vestibule filling. Acute attacks are replaced with constant imbalance problems and progressive hearing loss.
• In order to introduce uniformity in the diagnosis and treatment of Ménière disease, the American Academy of Otolaryngology first issued guidelines in 1972. These guidelines were revised in 1985 and again updated in 1995.¹ The guidelines require analysis of symptoms, audiometric results, and functional impairment to allow staging. This staging tool is now widely used diagnostically and also as a measure of treatment outcome.

Patient Education

For excellent patient education resources, visit eMedicine's Brain and Nervous System Center and Ear, Nose, and Throat Center. Also, see eMedicine's patient education articles Ménière Disease and Tinnitus.

REFERENCES

Section 9 of 9

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

1. Monsell EM. New and revised reporting guidelines from the Committee on Hearing and Equilibrium. American Academy of Otolaryngology-Head and Neck Surgery Foundation, Inc. Otolaryngol Head Neck Surg. Sep 1995;113(3):176-8. [Medline].
2. Bretlau P, Thomsen J, Tos M, et al. Placebo effect in surgery for Menière's disease: nine-year follow-up. Am J Otol. Jul 1989;10(4):259-61. [Medline].
3. Densert B, Sass K. Control of symptoms in patients with Meniere's disease using middle ear pressure applications: two years follow-up. Acta Otolaryngol. Jul 2001;121(5):616-21. [Medline].
4. Glasscock ME 3rd, Jackson CG, Poe DS, et al. What I think of sac surgery in 1989. Am J Otol. May 1989;10(3):230-3. [Medline].
5. Gottshall KR, Hoffer ME, Moore RJ, et al. The role of vestibular rehabilitation in the treatment of Meniere's disease. Otolaryngol Head Neck Surg. Sep 2005;133(3):326-8. [Medline].
6. Kato BM, LaRouere MJ, Bojrab DI, et al. Evaluating quality of life after endolymphatic sac surgery: The Ménière's Disease Outcomes Questionnaire. Otol Neurotol. May 2004;25(3):339-44. [Medline].
7. Kitahara T, Kondoh K, Morihana T, et al. Surgical management of special cases of intractable Meniere's disease: unilateral cases with intact canals and bilateral cases. Ann Otol Rhinol Laryngol. May 2004;113(5):399-403. [Medline].
8. Monsell EM, Wiet RJ. Endolymphatic sac surgery: methods of study and results. Am J Otol. Sep 1988;9(5):396-402. [Medline].
9. Odkvist LM, Arlinger S, Billermark E, et al. Effects of middle ear pressure changes on clinical symptoms in patients with Ménière's disease--a clinical multicentre placebo-controlled study. Acta Otolaryngol Suppl. 2000;543:99-101. [Medline].
10. Pyykkö I, Ishizaki H, Kaasinen S, et al. Intratympanic gentamicin in bilateral Menière's disease. Otolaryngol Head Neck Surg. Feb 1994;110(2):162-7. [Medline].
11. Shea JJ Jr. Classification of Menière's disease. Am J Otol. May 1993;14(3):224-9. [Medline].
12. Shea JJ Jr, Ge X. Streptomycin perfusion of the labyrinth through the round window plus intravenous streptomycin. Otolaryngol Clin North Am. Apr 1994;27(2):317-24. [Medline].
13. Silverstein H, Lewis WB, Jackson LE, et al. Changing trends in the surgical treatment of Ménière's disease: results of a 10-year survey. Ear Nose Throat J. Mar 2003;82(3):185-7, 191-4. [Medline].
14. Silverstein H, Smouha E, Jones R. Natural history vs. surgery for Menière's disease. Otolaryngol Head Neck Surg. Jan 1989;100(1):6-16. [Medline].

Article Last Updated: Sep 3, 2008