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Neurology
Meniere Disease Synonyms, Key Words, and Related Terms: Ménière's disease, Meniere’s disease, Ménière syndrome, Meniere syndrome, Ménière's syndrome, Meniere’s syndrome, endolymphatic hydrops, inner ear disorder, labyrinthine disorder, tinnitus, vertigo |
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| AUTHOR INFORMATION | Section 1 of 11 |
Authored by Nicholas Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Lorenzo, MD, is a member of the following medical societies: Alpha Omega Alpha, and American Academy of Neurology
Edited by Mark S Slabinski, MD, Director, Emergency Services, Southeastern Ohio Regional Medical Center; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; J Stephen Huff, MD, Associate Professor of Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia Health System; John Halamka, MD, Chief Information Officer, CareGroup Healthcare System, Assistant Professor of Medicine, Department of Emergency Medicine, Beth Israel Deaconess Medical Center; Assistant Professor of Medicine, Harvard Medical School; and Rick Kulkarni, MD, Medical Director, Assistant Professor of Surgery, Section of Emergency Medicine, Yale-New Haven Hospital
| Author's Email: | Nicholas Lorenzo, MD | |
|---|---|---|
| Editor's Email: | Mark S Slabinski, MD |
eMedicine Journal, July 12 2006, VOLUME 7,
Number 7
| INTRODUCTION | Section 2 of 11 |
Background: Ménière syndrome, also known as endolymphatic hydrops, is an inner ear (labyrinthine) disorder in which there is an increase in volume and pressure of the endolymph of the inner ear. It typically presents with waxing and waning hearing loss and tinnitus associated with vertigo. Patients may report an abnormal sensation of pressure or fullness in the ears. Multiple episodes may occur over a span of years, with remissions in between each acute episode. Hearing loss typically occurs.
Pathophysiology: Autopsy studies involving patients with Ménière syndrome have shown an increase in the volume of endolymph with distension of the entire endolymphatic system. This distension and increased fluid results in permanent damage to both the vestibular and cochlear apparatuses.
Frequency:
Mortality/Morbidity:
Sex: Generally, men and women are affected equally.
Age: Onset typically occurs in early- to mid-adulthood.
| CLINICAL | Section 3 of 11 |
History:
Physical: A complete neurological examination is necessary to discover other conditions. Particular attention should be given to the cranial nerve examination; any abnormalities in the neurological examination that are not attributable to the vestibular system point toward another diagnosis.
Causes:
| DIFFERENTIALS | Section 4 of 11 |
Headache, Migraine
Hypothyroidism and Myxedema Coma
Labyrinthitis
Multiple Sclerosis
Otitis Media
Stroke, Ischemic
Subarachnoid Hemorrhage
Toxicity, Salicylate
Transient Ischemic Attack
Vestibular Neuronitis
Other Problems to be Considered:
Aminoglycoside toxicity
Barre-Lieou syndrome (In addition to vertigo and unsteadiness, this syndrome is associated with cervical spondylosis. It presents with neck pain and a burning sensation in the face, tinnitus, dysphagia, and arm pain.)
Basilar meningitis
Brainstem tumors
Labyrinthine concussion
Labyrinthine otosclerosis
Neoplasms (especially those associated with cranial nerve VIII, eg, acoustic neuroma)
Posttraumatic (especially if associated with a skull fracture)
Quinine toxicity
Toxic or pharmaceutical injury to the vestibular apparatus (eg, alcohol, streptomycin, quinine, barbiturate, phenytoin, antihistamines)
Various infections (especially herpes zoster and CNS syphilis)
Vascular infarction of the labyrinth (usually associated with unilateral hearing loss)
Vasculitis (Cogan syndrome)
Wernicke encephalopathy
| WORKUP | Section 5 of 11 |
Lab Studies:
Imaging Studies:
Other Tests:
Procedures:
| TREATMENT | Section 6 of 11 |
Emergency Department Care:
Consultations:
| MEDICATION | Section 7 of 11 |
Medication use in the treatment of Ménière disease is primarily for symptomatic relief. Several medications (eg, meclizine, scopolamine, ephedrine, dimenhydrinate, diazepam) have antivertiginous properties, and other medications (eg, promethazine, prochlorperazine) are useful as antiemetics. The majority of acute episodes are short-lived and self-limited. A short tapering course of oral steroids occasionally may be helpful in treating an acute attack of Ménière disease. Diuretics, such as acetazolamide or hydrochlorothiazide, also may be helpful in specific patients when administered on a continuing basis. Innovar has been used successfully to treat patients failing first-line therapies, but its use should probably be considered only in consultation with the patient's primary care physician.
Drug Category: Antihistamines -- These agents are extremely useful in treating vertigo symptoms.
| Drug Name | Meclizine (Antivert) -- Decreases the excitability of the middle ear labyrinth and blocks conduction in the middle ear vestibular-cerebellar pathways. These effects are associated with its therapeutic effects in vertigo. |
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| Adult Dose | 25 mg PO q4-6h |
| Pediatric Dose | Children: Not established Adolescents: Administer as in adults |
| Contraindications | Documented hypersensitivity |
| Interactions | May increase toxicity of CNS depressants, neuroleptics, and anticholinergics |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| Precautions | Caution in angle-closure glaucoma, prostatic hypertrophy, pyloric or duodenal obstruction, and bladder neck obstruction |
| Drug Name | Dimenhydrinate (Dramamine) -- Used for the treatment and prophylaxis of vestibular disorders that may cause nausea and vomiting. Through its central anticholinergic activity, it diminishes vestibular stimulation and depresses labyrinthine function. |
|---|---|
| Adult Dose | 50 mg PO/IM q4-6h or a 100 mg suppository q8h |
| Pediatric Dose | Neonates: Do not administer 2-6 years: 12.5-25 mg q6-8h; not to exceed 75 mg/d 6-12 years: 25-50 mg PO q6-8h; not to exceed 150 mg/d |
| Contraindications | Documented hypersensitivity |
| Interactions | Alcohol or other CNS depressants may have additive effect on dimenhydrinate; caution when administering concurrently with antibiotics that may cause ototoxicity; may mask ototoxic symptoms, caused by certain antibiotics and irreversible damage may result |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| Precautions | IV products may contain benzyl alcohol, which has been associated with fatal gasping syndrome in premature infants and low birth weight infants; do not treat severe emesis with antiemetic drugs alone; may contain either sulfites or tartrazine, which may cause allergic-type reactions in susceptible persons; may impede diagnosis of conditions such as brain tumors, intestinal obstruction, and appendicitis; may obscure signs of toxicity from overdosage of other drugs |
| Drug Name | Scopolamine (Isopto) -- Blocks action of acetylcholine at parasympathetic sites and antagonizes histamine and serotonin action. Transdermal scopolamine may be the most effective agent for motion sickness. Use in the treatment of vestibular neuronitis is limited by its slow onset of action. |
|---|---|
| Adult Dose | 0.3-0.65 mg IM/SC/IV q4-6h Transdermal patch: Apply 2.5 cm2 patch to hairless area behind the ear q3d |
| Pediatric Dose | 6 mcg/kg/dose IV/IM/SC to a maximum of 0.3 mg/dose, or 0.2 mg/m2 q6-8h |
| Contraindications | Documented hypersensitivity; primary glaucoma (including initial stages), pyloric obstruction, toxic megacolon, hepatic disease, paralytic ileus, severe ulcerative colitis, renal disease, obstructive uropathy, and myasthenia gravis |
| Interactions | Antipsychotic effectiveness of phenothiazines may be decreased by coadministration with scopolamine; anticholinergic side effects may be increased by concurrent therapy and phenothiazine dosages should be adjusted prn; coadministration with tricyclic antidepressants may increase anticholinergic side effects (eg, dry mouth, constipation, urinary retention) due to additive effect (tricyclic antidepressants with less anticholinergic activity may be beneficial) |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Caution in the elderly because of increased incidence of glaucoma; large doses may suppress intestinal motility and precipitate or aggravate toxic megacolon; anticholinergics may aggravate hiatal hernia associated with reflux esophagitis; patients with prostatism can have dysuria and may require catheterization; use cautiously in patients with asthma or allergies; a reduction in bronchial secretions can lead to inspissation and formation of bronchial plugs |
| Drug Name | Diazepam (Valium) -- Depresses all levels of the CNS, including limbic and reticular formation, possibly by increasing GABA activity, which is a major inhibitory neurotransmitter. |
|---|---|
| Adult Dose | 5-10 mg PO/IV/IM q4-6h |
| Pediatric Dose | <6 months: Do not administer 0.05-0.3 mg/kg/dose IV/IM over 2-3 min; repeat in 2-4 h, prn 0.12-0.8 mg/kg/d PO divided q6-8h; not to exceed 10 mg/dose |
| Contraindications | Documented hypersensitivity; narrow-angle glaucoma |
| Interactions | Increases toxicity of benzodiazepines in CNS with coadministration of phenothiazines, barbiturates, alcohols, and MAOIs |
| Pregnancy | D - Unsafe in pregnancy |
| Precautions | Caution with other CNS depressants, low albumin levels, or hepatic disease (may increase toxicity) |
| Drug Name | Promethazine (Phenergan) -- Antidopaminergic agent effective in the treatment of emesis. Blocks postsynaptic mesolimbic dopaminergic receptors in the brain and reduces stimuli to the brainstem reticular system. |
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| Adult Dose | 25-50 mg PO/IM/PR q4-6h |
| Pediatric Dose | <2 years: Contraindicated >2 years: 0.5 mg/kg q4-6h |
| Contraindications | Documented hypersensitivity; children younger than 2 y (incidences of death due to respiratory depression) |
| Interactions | May have additive effects when used concurrently with other CNS depressants or anticonvulsants; coadministration with epinephrine may cause hypotension |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Can be associated with CNS depression, dry mouth, extrapyramidal symptoms, hypertension, hypotension, and skin rash; caution in patients with cardiovascular or hepatic disease May accentuate CNS depression when administered with other medications (that cause CNS depression), to include alcohol, narcotics, sedatives, and hypnotics |
| Drug Name | Prochlorperazine (Compazine) -- Antidopaminergic drug that blocks the postsynaptic mesolimbic dopaminergic receptors. Has an anticholinergic effect and can depress the reticular activating system. IV administration is not recommended for children. |
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| Adult Dose | 5–10 mg PO/IM q6h or 25 mg PR q12h |
| Pediatric Dose | 2.5 mg PO/PR q8h, or 5 mg q12h prn 0.1-0.15 mg/kg/dose IM; change to PO as soon as possible Total daily dosage is not to exceed 7.5 mg in children <30 lb, 10 mg in children <40 lb, and 15 mg in children <85 lb |
| Contraindications | Documented hypersensitivity; bone marrow suppression; narrow-angle glaucoma; severe liver or cardiac disease |
| Interactions | Coadministration with other CNS depressants or anticonvulsants may cause additive effects; with epinephrine may cause hypotension |
| Pregnancy | D - Unsafe in pregnancy |
| Precautions | Drug-induced Parkinson syndrome or pseudoparkinsonism occurs quite frequently; akathisia is most common extrapyramidal reaction in elderly; lowers seizure threshold; caution with history of seizures |
| Drug Name | Ephedrine (Pretz-D, Kondon's Nasal) -- Stimulates the release of epinephrine stores producing alpha- and beta-adrenergic effects. |
|---|---|
| Adult Dose | 25 mg PO q4-6h |
| Pediatric Dose | <2 years: Not recommended 2-5 years: 3 mg q6-8h >5 years: 6.25 mg q6-8h |
| Contraindications | Documented hypersensitivity; angle-closure glaucoma; cardiac arrhythmias |
| Interactions | Theophylline, atropine, or MAOIs may increase toxicity; alpha- and beta-blockers decrease vasopressor effects of ephedrine; cardiac glycosides and general anesthetics increase cardiac stimulation of ephedrine |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| Precautions | Caution in the elderly, diabetes mellitus, hyperthyroidism, hypertension, cardiovascular disease, prostatic hypertrophy, or cerebrovascular insufficiency |
| Drug Name | Prednisone (Deltasone) -- Useful in the treatment of inflammatory and allergic reactions. By reversing increased capillary permeability and suppressing PMN activity, it may decrease inflammation. |
|---|---|
| Adult Dose | A short (3-5 d) course of oral prednisone can be utilized; a starting dose of 40-60 mg PO qd should be considered |
| Pediatric Dose | A short course (3-5 d) can be used in children, with a starting dose of 5-10 mg PO |
| Contraindications | Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease |
| Interactions | Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use |
| FOLLOW-UP | Section 8 of 11 |
Further Inpatient Care:
Further Outpatient Care:
In/Out Patient Meds:
Deterrence/Prevention:
Complications:
Prognosis:
Patient Education:
| MISCELLANEOUS | Section 9 of 11 |
Medical/Legal Pitfalls:
| TEST QUESTIONS | Section 10 of 11 |
CME Question 1: Which of the following is not associated with Ménière syndrome?
A: Hearing loss
B: Vertigo
C: Diplopia
D: Endolymphatic hydrops
E: C and D
The correct answer is C: Diplopia, or any other neurological sign or symptom that is not directly attributable to the vestibulocochlear system, is worrisome for a more grave diagnosis (eg, a brainstem tumor), emphasizing the importance of a complete neurological history and examination in these patients.
CME Question 2: Which of the following is not associated with Ménière syndrome?
A: Congenital inner ear deformities
B: Labyrinthitis
C: Physical trauma to the head and ear
D: Penicillin family of antibiotics
E: Syphilis
The correct answer is D: Ménière syndrome can be associated with the aminoglycoside family (not the penicillin family) of antibiotics. Aminoglycosides are toxic to the vestibular and cochlear systems.
Pearl Question 1 (T/F): Surgery is almost never indicated for severe cases of Ménière syndrome.
The correct answer is False: Several surgical procedures including labyrinthectomy and vestibular nerve section can be considered in severe cases of Ménière syndrome.
Pearl Question 2 (T/F): Streptomycin can be used as an ablative therapy in the treatment of Ménière syndrome.
The correct answer is True: Daily injections of streptomycin are a reasonable therapeutic option.
Pearl Question 3 (T/F): The membranous labyrinth plays an important role in the pathophysiology of Ménière syndrome.
The correct answer is True: The membranous labyrinth progressively dilates until permanent damage is done to the vestibular and cochlear systems.
Pearl Question 4 (T/F): A high fiber diet can be helpful in some patients with Ménière syndrome.
The correct answer is False: Dietary fiber content has no known association with Ménière syndrome. However, a low-salt diet can be helpful. In addition, avoidance of foods with high sugar and such specific foods as alcohol, coffee, or chocolate also can also be helpful.
| BIBLIOGRAPHY | Section 11 of 11 |
| NOTE: |
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| Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment therapies daily. The authors, editors, and publisher of this journal have used their best efforts to provide information that is up-to-date and accurate and is generally accepted within medical standards at the time of publication. However, as medical science is constantly changing and human error is always possible, the authors, editors, and publisher or any other party involved with the publication of this article do not warrant the information in this article is accurate or complete, nor are they responsible for omissions or errors in the article or for the results of using this information. The reader should confirm the information in this article from other sources prior to use. In particular, all drug doses, indications, and contraindications should be confirmed in the package insert. FULL DISCLAIMER |
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