AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

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

INTRODUCTION

Section 2 of 11  

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

Background

Ménière disease, also known as idiopathic endolymphatic hydrops, is a disorder of the inner ear resulting in the clinical triad of vertigo, tinnitus, and hearing loss. Prosper Ménière first proposed the disorder in 1861. Patients report an intermittent and progressive nature of the disease with a significant number having spontaneous resolution.

Pathophysiology

The exact cause of Ménière disease is controversial. The prevailing thought is that it is due to distortion of the membranous labyrinth due to overaccumulation of endolymph. The increased size of the cochlear duct pushes the Reissner membrane into the scala vestibuli. However, there remains the possibility that this is an epiphenomenon and not the actual cause.

Endolymph is produced by the stria vascularis in the cochlea and the dark cells in the vestibular labyrinth. Significant controversy exists regarding whether there is a circulatory nature to the flow of endolymph. Many feel that, in Ménière disease, absorption of endolymph in the endolymphatic sac is inadequate.

Frequency

• Reported prevalence of disease varies widely. It is likely between 50 and 200 per 100,000. This difference in prevalence based on geographic area is likely due to reporting biases and not geographic patterns of disease.
• Bilateral disease is found in 10-20% of patients with Ménière disease.
• A genetic predisposition appears to exist.

Mortality/Morbidity

• No direct mortality is associated with Ménière disease; however, it is associated with drop attacks, which could lead to accidents resulting in mortality.
• The main morbidity associated with Ménière disease is the debilitating nature of vertigo and the progressive and possibly permanent loss of hearing.

Sex

Distribution of symptoms is equal among men and women, although women may seek treatment more frequently.

Age

• The peak incidence is in the 40- to 60-year-old age group.
• It has been described in children as young as 4 years and in elderly persons older than 90 years.

CLINICAL

Section 3 of 11  

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

History

The American Academy of Otolaryngology - Head and Neck Surgery Foundation (AAO-HNS) Committee on Hearing and Equilibrium published guidelines in 1972, 1985, and, most recently, in 1995, on the clinical diagnosis of Ménière disease. 

According to these guidelines, Ménière disease is defined as recurrent, spontaneous episodic vertigo; hearing loss; aural fullness; and tinnitus. Either tinnitus or aural fullness (or both) must be present on the affected side to make the diagnosis.¹

• Vertigo
• • Vertigo is a subjective sensation of motion while motionless.
  • At least 2 definitive episodes of vertigo of at least 20 minutes duration must occur to make the diagnosis.
  • The duration is usually at least several hours.
  • Horizontal or rotatory nystagmus is always present during attacks of vertigo.
  • Symptoms are often accompanied with nausea, vomiting, and anxiety.
  • Acute attacks may be accompanied with sudden falls without loss of consciousness. These are termed crises of Tumarkin or drop attacks.
• Hearing loss
• • Sensorineural hearing loss must be documented audiometrically in the affected ear at least once during the course of the disease.
  • Fluctuation may occur in the degree of hearing loss superimposed on a gradual decrement in function.
  • Hearing loss primarily affects low frequencies.
• Tinnitus and aural fullness
• • Tinnitus is often nonpulsatile and may be described as whistling or roaring.
  • The symptoms may be continuous or intermittent.

Physical

• A complete neurologic examination is necessary to differentiate Ménière disease from other conditions.
• Evaluation of vertigo
• • Dix-Hallpike positional test (also known as Nylen-Barany maneuver)
  • • The patient is positioned in the middle of the table so that the patient's head extends past the head of the bed when supine.
    • The patient is rapidly moved backward so that the head hangs over the edge and the eyes are observed for evidence of nystagmus. If no nystagmus is observed for 20 seconds, the patient is returned to the upright position. The next step is to bring the patient rapidly to head-right supine position again looking for nystagmus. 
    • This is then repeated in the head-left supine position. 
    • Any nystagmus or symptoms and the position of the head on eliciting these symptoms or signs should be noted.
    • Nystagmus typically has a latency of 2-5 seconds.
    • Nystagmus and vertigo associated with peripheral causes such as Ménière disease should be fatigable and exhibit habituation. 
    • Central lesions should have no latency and do not fatigue or habituate.
  • The Romberg test reveals significant instability during acute attacks.
  • The Hennebert sign is nystagmus owing to applied pressure in the external auditory canal.
  • Tullio phenomenon is sound-induced vertigo or nystagmus or both. It is historically associated with syphilis but has been described in Ménière disease.
• Evaluation of hearing loss
• • Gross evaluation can be performed by gently rubbing the examiner's fingers near the patient's ears. The farthest distance the sound can be heard should be noted for each ear.
  • Rinne test performed with a 256-MHz tuning fork indicates that air conduction is greater than bone conduction.
  • Weber test is also performed with a 256-MHz tuning fork. Normally, sound should be heard equally on both sides. The sound is more pronounced on the affected side in middle ear disease or blockage of the external auditory canal. The sound is more pronounced on the unaffected side if cochlear nerve dysfunction is present.
  • Audiologic testing is more accurate.

Causes

• Multiple causes of dysfunction of the vestibular system exist.
• No single entity is known to be responsible for Ménière disease. It is currently thought to be due to overaccumulation of endolymph in the cochlear duct.
• Ménière disease must be distinguished from other causes of endolymphatic hydrops such as posttraumatic, postinfectious, otosyphilis, and Cogan syndrome.

DIFFERENTIALS

Section 4 of 11  

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

Other Problems to be Considered

Barre-Lieou syndrome
Basilar meningitis
Brainstem tumors
Cerumen impaction
Foreign body in ear canal 
Labyrinthine otosclerosis
Neoplasms (especially those associated with cranial nerve VIII, eg, acoustic neuroma) 
Perilymphatic fistula
Toxic or pharmaceutical injury to vestibular apparatus
Trauma (labyrinthine concussion)
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  

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

Lab Studies

• No laboratory test is specific for Ménière disease.
• Laboratory tests should be directed at differentiating Ménière disease from other causes based on associated symptoms.
• • Complete blood cell count
  • Electrolyte level
  • Sedimentation rate
  • C-reactive protein
  • Urinalysis
  • Thyroid panel
  • Fluorescent treponemal antibody

Imaging Studies

• A patient with a history classic for Ménière disease does not normally need imaging studies performed. If other processes are a concern, then MRI or CT can be obtained based on the differential diagnosis.
• • MRI of the brain is used to detect abnormal inner ear anatomy, masses, and lesions such as multiple sclerosis and Arnold-Chiari malformations.
  • CT scans are used to detect dehiscence of the superior semicircular canals, widened cochlear and vestibular aqueducts, and subarachnoid hemorrhage.

Other Tests

• More extensive testing is typically reserved for outpatient or inpatient workup and is not performed in the emergency department.
• • Audiometry
  • Brainstem auditory evoked potentials
  • Electronystagmography
  • Otoscopy
  • Caloric testing/electronystagmography (ENG)

Procedures

• Most procedures are reserved for patients that have undergone extensive workup by an otolaryngologist and failure of conservative medical management.
• Surgical therapy as a last resort includes the following:
• • Intratympanic injection of medication (gentamicin, streptomycin, steroids)
  • Endolymphatic sac decompression, shunt placement, or both
  • Labyrinthectomy
  • Vestibular nerve resection

TREATMENT

Section 6 of 11  

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

Emergency Department Care

• The diagnosis of Ménière disease is based on history and clinical findings. 
• Most care in the emergency department is based on symptomatic relief of the clinical symptoms.
• Control of vertiginous sensation using the medications discussed below (see Medication).
• Intravenous hydration and antiemetics for nausea and vomiting

Consultations

An ENT specialist or neurologist can be consulted for cases that are not definitive or if they are unremitting and severe.

MEDICATION

Section 7 of 11  

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

Medical treatment is aimed at symptomatic relief. The primary target is relief of vertiginous symptoms. Antiemetics may be used for nausea and vomiting. Diuretics are often used, but their efficacy has not been established with appropriate clinical trials.

Drug Category: Antihistamines

These agents are extremely useful in treating vertigo symptoms.

Drug Name	Dimenhydrinate (Dramamine)
Description	Used for 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 - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
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 Category: Anticholinergics

These agents are thought to work centrally by suppressing conduction in the vestibular cerebellar pathways.

Drug Category: Benzodiazepines

By binding to specific receptor sites, these agents appear to potentiate the effects of gamma-aminobutyric acid (GABA) and to facilitate inhibitory GABA neurotransmission and other inhibitory transmitters. These effects may offer benefits in the treatment of vertigo and emesis.

Drug Category: Phenothiazines

These agents are useful in treating emesis.

Drug Name	Prochlorperazine (Compazine)
Description	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.
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 - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions	Drug-induced Parkinson syndrome or pseudoparkinsonism occurs quite frequently; akathisia is most common extrapyramidal reaction in elderly persons; lowers seizure threshold; caution with history of seizures

Drug Category: Adrenergic Agonists

These agents are useful in treating vertigo, but their mechanism of action is unclear.

Drug Category: Corticosteroids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects.

FOLLOW-UP

Section 8 of 11  

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

Further Inpatient Care

• Patient's require admission only if the symptoms are severe and refractory to medical management.

Further Outpatient Care

• All patients with Ménière disease should have rapid follow-up with their primary care provider.
• Some patients may require referral to an ENT specialist or neurologist.

In/Out Patient Meds

• Medications that provide symptomatic relief in the Eemergency Ddepartment should be considered for use on an outpatient basis.

Deterrence/Prevention

• Quality evidence is lacking regarding deterrence and prevention of acute attacks of Ménière disease; however, the following suggestions are often made:
• • Salt-restricted diet
  • Loud noise avoidance
  • Stress-reduction techniques
  • Smoking cessation

Complications

• Progressive hearing loss
• Injury due to falls
• Anxiety regarding symptoms

Prognosis

• Wide variation exists in patient presentation and progression of disease. 
• Some patients have minimal symptoms, whereas others have severe attacks. 
• Episodes may occur as infrequently as once or twice a year or may occur on a regular basis.
• The spontaneous remission rate is high, at more than 50% of cases. Most of the remainder patients are well managed with medications.
• Surgical treatment is required in 5-10% of patients.

Patient Education

• Patients should be instructed that if their symptoms significantly worsen or if they develop any new symptoms suggestive of another disease process that they should return immediately to the emergency department for reevaluation.
• Patients should be warned of the possibility of falls.
• 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.

MISCELLANEOUS

Section 9 of 11  

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

Medical/Legal Pitfalls

• Performance of the Dix-Hallpike maneuver can result in spinal injury if performed improperly and in those with underlying spinal disease.
• Misdiagnosis as Ménière disease when the underlying disorder is more serious.
• Failure to warn patients of possibility of drop attacks, which could result in injury.
• Complications from medications are a pitfall.

ACKNOWLEDGMENTS

Section 10 of 11  

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

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Nicholas Y Lorenzo, MD, to the development and writing of this article.

REFERENCES

Section 11 of 11

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

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Article Last Updated: Jan 8, 2008