Pediatrics

Meniere's disease

OVERVIEW: What every practitioner needs to know

Are you sure your patient has Meniere's disease? What are the typical findings for this disease?

Meniere's disease is a condition of the inner ear that leads to episodic vertigo and hearing loss.

  1. Short episodes from 15 minutes to a few hours

  2. Rotatory vertigo (sensation of world rotating around the head)

  3. Hearing loss in the affected ear, usually low frequency

  4. Tinnitus, or change in tinnitus

  5. Sensation of fullness or pressure in the affected ear

Characteristics of Vertigo:

1. Rotatory vertigo is most common; rare variants may present with "drop attacks ", known as crisis of Tumarkin

2. Ambulation may be difficult or impossible

3. Horizontal nystagmus accompanies the episode

4. Nausea and vomiting are common

5. Other neurological symptoms are absent, consciousness is not lost

Characteristics of Hearing Loss:

1. Low frequency hearing loss is the most common

2. Hearing loss fluctuates between episodes

3. Hearing loss is sensorineural, and is usually progressive

Characteristics of Tinnitus and Aural Fullness:

1. Tinnitus fluctuates but is loud during the episode

2. Changes from baseline tinnitus if present

3. Fullness or feeling of pressure pressure in the ear is common, "like a balloon inflating" in ear

4. Aural symptoms lateralize to the affected ear

What other disease/condition shares some of these symptoms?

Other conditions that may share symptoms of Meniere's disease include the following:

1. Migraine and migraine variant without headache mimic many symptoms of Meniere's disease.

2. Benign paroxsymal postural vertigo (BPPV).

3. Rarely, tumors of the internal audiotory canal (vestibular schwannoma, also called acoustic neuroma).

What caused this disease to develop at this time?

The cause of Meniere's disease is unknown. It may be triggered by an increase in salt intake, and it may be affected by nicotine, caffeine, alcohol, sleep quality, or stress.

What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results?

An audiogram is performed to evaluate for hearing loss, including tympanometry, and otoacoustic emissions (OAE). A low frequency, sensorineural hearing loss with normal or near normal speech discrimination is common.

Balance function is assessed with electronystagmogram (ENG) or rotatory chair. Decreased function (unilateral vestibular hypofunction) of the affected ear is common.

Some centers perform electrocochleography (ECoG), increased SP/AP ratios may support the diagnosis of Meniere's disease.

Would imaging studies be helpful? If so, which ones?

Magnetic resonance imaging (MRI) of the brain and internal auditory canal/cerebellopontine angle (IAC/CPA protocol) can help identify retrocochlear causes of asymmetric hearing loss such as vestibular schwannoma and meningioma.

Confirming the diagnosis

  • American Academy of Otolaryngology, Head and Neck Surgery Diagnosic Guidelines:

    • Certain Meniere's disease

      • Definite Meniere's disease, plus confirmation by pathology

    • Definite Meniere's disease

      • 2 or more definitive episodes of spontaneous vertigo lasting at least 20 minutes

      • Audiologic confirmation of hearing loss on at least one occasion

      • Tinnitus or aural fullness in the affected ear

      • Other cases excluded

    • Probable Meniere's disease

      • 1 definitive episode of spontaneous vertigo lasting at least 20 minutes

      • Audiologic confirmation of hearing loss on at least one occasion

      • Tinnitus or aural fullness in the affected ear

      • Other cases excluded

    • Possible Meniere's disease

      • Episodic vertigo consistent with Meniere's disease without documented hearing loss, or

      • Fluctuating or fixed hearing loss with dysequilibrium, but without definitive episodes of vertigo

      • Other causes excluded

If you are able to confirm that the patient has Meniere's disease, what treatment should be initiated?

  • Low salt diet: less than the dietary reference values of 1000-1500 mg (ages 2-3), 1200-1900 (ages 4-8), 1500-2300 (ages 9 and above).

  • Diuretic therapy, may benefit some individuals (very limited evidence).

  • Suppressive therapy for vertiginous symptoms: phenergan, meclizine, ondansetron.

  • Suppressive therapy for vestibular excitation: benzodiazepines, such as clonazepam.

  • Supportive therapy with intratympanic injection of corticosteroid, such as dexamethosone (good evidence).

  • Ablative therapy with intratympanic injection of gentamicin (excellent evidence).

  • Ablative therapy with surgical intervention (very limited evidence).

What are the adverse effects associated with each treatment option?

  • Low salt diet: There are minimal adverse effects, but there is likely to be low compliance.

  • Diuretic therapy: Electrolyte abnormalities, symptomatic hypotension, risk of renal lithiasis with acetazolamide.

  • Suppressive therapy for vertiginous symptoms: Sedation, dry mouth, urinary retention, diarrhea.

  • Suppressive therapy for vestibular excitation: Sedation, dependence.

  • Supportive therapy with intratympanic injection of corticosteroid: Results are temporary, it requires invasive procedure, and it carries a risk of injury to tympanic membrane, hearing.

  • Ablative therapy with intratympanic injection of gentamicin: Permanent, but requires invasive procedure and carries a risk of injury to the tympanic membrane. Hearing loss is more likely.

  • Ablative therapy with surgical intervention: Depends on the procedure. This approach requires an invasive procedure and hearing loss is more likely, or planned (labyrinthectomy).

What are the possible outcomes of Meniere's disease?

Episodic vertigo with progressive sensorineural hearing loss. Most patients have asymptomatic periods between attacks, sometimes lasting years. Progressive loss of vestibular function leads to a decrease in vertiginous symptoms as the population of vestibular afferents decreases (the Meniere's "burnt out" ear).

Classically the condition affects one ear, but some patients develop symptoms in the other ear, perhaps as often as 15% over their lifetime.

What causes this disease and how frequent is it?

  • Lifetime incidence proposed as about 1:50,000

    • Incidence in children thought to be 1-2% of all diagnoses of Meniere's disease.

    • Usual age of onset 40-60 years.

    • Affects men and women equally.

    • Some international studies propose differing incidence, but there is no demonstrated ethnic difference.

  • High incidence of family history of Meniere's disease support theories that inherited factors such as autoimmunity, inner ear anatomy, and ion-channel physiology contribute to Meniere's disease.

How do these pathogens/genes/exposures cause the disease?

  • Herpes simplex virus-1 (HSV) antibody titer are increased in some patients with Meniere's disease.

What complications might you expect from the disease or treatment of the disease?

See sections above

Are additional laboratory studies available; even some that are not widely available?

Vestibular evoked myogenic potentials (VEMP) can help assess loss of vestibular function.

Laboratory testing for other causes of hearing loss: RPR for syphilis, Lyme disease, autoimmune markers such as ANA, CRP, ESR.

How can Meniere's disease be prevented?

The cause of Meniere's disease is unknown, and no preventative measures are known.

What is the evidence?

Recent Cochrane Reviews have been very helpful in identifying and assessing the publications related to medical therapy, injection therapy, or surgical intervention for Meniere's disease. There have been limited prospective, blinded studies. Due to the high rate of spontaneous, symptom-free periods, sometimes lasting years, it can be difficult to assess the efficacy of different treatments.

The following are helpful Cochrane Reviews for Meniere's disease:

Ongoing controversies regarding etiology, diagnosis, treatment

Diagnosis of Meniere's disease can be controversial due to the cost and time of comprehensive testing. Some treatments are expensive and not covered by insurance, with no clear evidence to justify their use.

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