DESCRIPTION

• Disease of the inner ear
• Classically unilateral ear involvement (may be bilateral in up to 40% of cases)
• Characterized by recurrent spontaneous and episodic vertigo, sensorineural hearing loss, "roaring" tinnitus, and aural fullness
• Estimated incidence about 15/100,000 in US
• Slight female > male (1.3:1)
• Positive family history up to 20%
• May develop at any age
  • Peak incidence is age 40–60 yr.
• Affects more Whites of Northern European descent than Africans or Blacks
• A benign disease without cure
• Can be associated with significant morbidity

ETIOLOGY

• Idiopathic
• Endolymphatic hydrops: Blockage of the endolymphatic sac and duct leading to endolymphatic outflow obstruction and increased hydraulic pressure within the endolymphatic system:
  • Increased pressure causes a break in the membrane that separates the perilymph (potassium [K]-poor extracellular fluid) and the endolymph (K-rich intracellular fluid)
  • The resultant chemical mixture bathes the vestibular nerve receptors, leading to a depolarization blockade and transient loss of function
• May be associated with structural abnormalities such as atrophy of the sac, hypoplasia of the vestibular aqueduct, narrowing of the endolymphatic duct, forwardly located lateral sinus causing compression, and obstruction of the endolymphatic sac
• Autoimmune processes suggested as an etiology, with immune complex deposition in endolymphatic sac and autoantibodies directed against endolymphatic sac
• Thought to have a genetic predisposition, leading to earlier age of onset and more severe symptoms
• Other proposed mechanisms are subclinical viral infection causing hydrops many decades later, and ischemia of the endolymphatic sac and the inner ear
• Need to differentiate Ménière syndrome from other disease processes that interfere with normal production or resorption of endolymph (e.g., thyroid disease, inner ear inflammation due to syphilis, medication)

[Outline]

• DESCRIPTION
• ETIOLOGY

• Diagnosis based upon clinical symptoms and neurotologic evaluation
• Definitive diagnosis currently can only be made postmortem, though MRI holds potential for definitive diagnosis
• Diagnostic criteria (1995 American Academy of Otolaryngology guidelines):
  • At least 2 episodes of spontaneous and episodic vertigo, ≥20 min
  • At least 1 episode of hearing loss documented by audiogram
  • Tinnitus or aural fullness in the affected ear
  • Certain Ménière disease: Definite disease with histopathologic confirmation
  • Definite Ménière disease: 2 or more definitive episodes of vertigo with hearing loss, plus tinnitus, aural fullness, or both
  • Probable Ménière disease: Only 1 definitive episode of vertigo and the other symptoms and signs
  • Possible Ménière disease: Definitive vertigo with no associated hearing loss or hearing loss with nondefinitive disequilibrium

SIGNS AND SYMPTOMS

History

• Classical tetrad of symptoms:
  • Vertigo
  • Hearing loss
  • Tinnitus
  • Aural fullness
• Vertiginous attacks lasting minutes to hours, often associated with nausea and vomiting (96.2%)
• Sensorineural hearing loss is typically fluctuating and progressive
  • Low frequencies are affected more severely than high frequencies (87.7%)
  • Can result in permanent hearing loss at all frequencies
• Tinnitus is typically low pitch
• Aural fullness is described as pressure, discomfort, fullness sensation in unilateral ear
• Attacks reach maximum intensity within minutes, slowly subside over hours
• After the acute attack, patients generally feel tired, unsteady, and nauseated for hours to days
• Between episodes, some patients are completely symptom free
• Sudden, unexplained falls without loss of consciousness or associated vertigo may also occur
• Constellation of symptoms can vary from patient to patient
  • Auditory and vestibular symptoms may not be present simultaneously or in the same pattern, particularly in the early phases of disease
• Close clustering of attacks may occur

Physical Exam

• Exam results vary, depending upon the phase of disease
• During acute attack, patients are often in significant distress, diaphoretic, and pale
• Vital signs may show elevated blood pressure, pulse, and respiration
• Horizontal nystagmus
• Impaired hearing
• Pneumo-otoscopy may elicit symptoms or cause nystagmus
• Weber tuning fork test usually lateralizes away from the affected ear
• Rinne test usually indicates better air than bone conduction
• Positive Romberg test, with instability, especially when eyes are closed
• Must exclude central CNS lesion, peripheral pathology in ear (ruptured tympanic membrane, cholesteatoma, cerumen impaction, etc.)

ESSENTIAL WORKUP

• Complete history and neurologic exam
• Patients with central vertigo or focal neurologic findings require neuroimaging
• Focal findings include new unilateral hearing loss, usually with tinnitus

DIAGNOSIS TESTS & INTERPRETATION

Lab

When indicated:

• CBC
• Sedimentation rate
• Thyroid function
• Fasting lipid profiles
• Fasting blood glucose, hemoglobin A1c
• Treponemal antibody-absorption test
• Chemistry panel
• Urinalysis for proteinuria or hematuria

Imaging

• MRI with intratympanic gadolinium and views of internal auditory canal (typically outpatient)
• CT scan of temporal bone
• Standard lateral mastoid radiographs

Diagnostic Procedures/Surgery

• Audiometric assessment
• Bithermal caloric testing
• Transtympanic electrocochleography
• Electronystagmography

DIFFERENTIAL DIAGNOSIS

• Otologic:
  • Chronic suppurative otitis media
  • Benign positional vertigo
  • Acoustic neuroma
  • Vestibular neuronitis
  • Vestibular paroxysmia
  • Otosclerosis
  • Otic capsule dysplasia
  • Perilymphatic fistula
  • Labyrinthitis
• Systemic:
  • Vertebrobasilar insufficiency
  • Stroke and transient ischemic attack
  • Basilar artery thrombosis
  • Intracranial hemorrhage
  • Head trauma
  • CNS lesions (tumors)
  • Epilepsy
  • Multiple sclerosis
  • Paget disease
  • Diabetes
  • Concussive syndrome
  • Pseudotumor cerebri
  • Complex or vestibular migraine
  • Thyroid dysfunction
  • Drugs/medications
  • Autoimmune disorders (e.g., systemic lupus erythematosus)
  • Viral meningitis/encephalitis
  • Lyme disease
  • Neurosyphilis
  • Electrolyte imbalance
  • Panic attacks

[Outline]

• INFORMATION
• SIGNS AND SYMPTOMS
• ESSENTIAL WORKUP
• DIAGNOSIS TESTS & INTERPRETATION
• DIFFERENTIAL DIAGNOSIS

PRE-HOSPITAL

• Vertigo and neurologic symptoms can represent a stroke
• Rapid transport to ED
• Protect patient from falling
• Maintain patient in comfortable position
• IV isotonic fluids for patients with vomiting
• Monitor for dysrhythmia

INITIAL STABILIZATION/THERAPY

• IV hydration with isotonic fluids
• IV benzodiazepines
• IV antiemetics

ED TREATMENT/PROCEDURES

Supportive therapy

MEDICATION

• Symptomatic:
  • Meclizine: 12.5–25 mg PO q8h
  • Diazepam: 5–10 mg PO/PR/IV
  • Lorazepam: 0.5–2 mg PO/IV/IM
  • Dimenhydrinate: 12.5–50 mg PO
  • Ondansetron: 4–8 mg IV/IM/PO
  • Metoclopramide: 10 mg IV/IM
  • Promethazine: 10–25 mg PO/PR/IV/IM
  • Prochlorperazine: 10 mg IV
• Therapeutic:
  • Hydrochlorothiazide: 25–50 mg PO daily
  • Triamterene: 100 mg PO daily
  • Acetazolamide: 250 mg PO daily
  • Furosemide: 20 mg PO daily
  • Prednisone: 1 mg/kg PO daily with taper over 7–14 days
  • Dexamethasone: 4 g/L transtympanic injection
  • Gentamicin transtympanic perfusion
  • Pressure pulse treatment
  • Surgery (surgical labyrinthectomy, vestibular neurectomy, sacculotomy)

First Line

• Diazepam or lorazepam
• Ondansetron for nausea, vomiting
• IV fluid

Second Line

• Meclizine
• Prochlorperazine

[Outline]

• PRE-HOSPITAL
• INITIAL STABILIZATION/THERAPY
• ED TREATMENT/PROCEDURES
• MEDICATION

DISPOSITION

Admission Criteria

Patient refractory to acute control of vertigo and associated effects (e.g., dehydration from protracted vomiting)

Discharge Criteria

• Tolerate oral fluids
• Steady gait
• Normal neurologic exam
• Fall precautions
• Recurrent attacks are typical
• Dietary restrictions: Sodium, caffeine, chocolate, tobacco, and alcohol intake
• Patient needs to avoid driving, operating dangerous equipment, and working at heights until attacks have resolved and sedating medications have been withdrawn

Issues for Referral

• Persistent/intractable symptoms and medical treatment failures
• Presence of ear pathology

FOLLOW-UP RECOMMENDATIONS

• Proper education in terms of dietary control and avoidance techniques is helpful
• Vestibular rehabilitation can be helpful in teaching patients to cope with vertigo and imbalance
• Counsel regarding fall risks and avoiding dangerous tasks due to the unpredictable nature of the disease
• Refer to neurologist, otologist, and otolaryngologist for outpatient audiometry and electronystagmography testing

[Outline]

• DISPOSITION
• FOLLOW-UP RECOMMENDATIONS

• Ménière disease typically presents with the classic tetrad of vertigo, hearing loss, tinnitus, and aural fullness
• Treatment focus is symptom relief, not cure
• Discharged patients should be referred to an outpatient neurologist, otologist, and otolaryngologist
• Inpatient care is generally unnecessary and is reserved for patients refractory to acute control of their symptoms or associated effects such as dehydration and vomiting
• Surgery is reserved for patients who fail medical therapy with intractable symptoms

ICD9

386.00 Meniere's disease, unspecified

ICD10

• H81.01 Meniere's disease, right ear
• H81.02 Meniere's disease, left ear
• H81.09 Meniere's disease, unspecified ear

[Outline]

• ICD9
• ICD10

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• James A. Ménière's disease. Clin Evid. 2004;11:664–672.
• Kerber KA. Vertigo and dizziness in the emergency department. Emerg Med Clin North Am. 2009;27:39–50.
• Kim HH, Wiet RJ, Battista RA. Trends in the diagnosis and management of Ménière's disease: Results of a survey. Otolaryngol Head Neck Surg. 2005;132:722–726.
• Lempert T. Recurrent spontaneous attacks of dizziness. Continuum (Minneap Minn). 2012;18:1086–1101.
• Li JC. Meniere Disease (Idiopathic Endolymphatic Hydrops). Emedicine. Updated Sept 15, 2011. Available at http://emedicine.medscape.com/article/1159069-overview.
• Pierce NE, Antonelli PJ. Endolymphatic hydrops perspectives 2012. Curr Opin Otolaryngol Head Neck Surg. 2012;20:416–419.
• Sajjadi H, Paparella M. Ménière's disease. Lancet. 2008;372:406–414.
• Semaan MT, Alagramam KN, Megerian CA. The basic science of Meniere's disease and endolymphatic hydrops. Curr Opin Otolaryngol Head Neck Surg. 2005;13:301–307.
• Syed I, Aldren C. Meniere's disease: An evidence based approach to assessment and management. Int J Clin Pract. 2012;66:166–170.

See Also (Topic, Algorithm, Electronic Media Element)

• Dizziness
• Labyrinthitis

Amira M. Bass

Charles V. Pollack, Jr.