Testing for Meniere's disease

Timothy C. Hain, MD Page last modified: December 3, 2009

Patients sometimes ask why they need to have inner ear testing with Meniere's disease.

In our practice in Chicago, we usually see at least one new patient with Meniere's disease every day.

A patient who fits clinical criteria for Meniere's, but who is without any previous testing, is usually asked to undergo the following procedures

• Hearing testing (audiometry), with tympanometry and OAE. (hearing should be down)
• ECochG (electrocochleography)
• VENG, rotatory chair and VEMP testing (to document amount of damage and rule out SCD).
• Posturography
• Blood tests -- (should be normal)
  • RPR or FTA (for syphilis). Lyme if exposed.
  • CRP and ANA (for autoimmune)
  • anti-thyroglobulin and anti-TPO antibodies (for Hashimoto's thyroiditis)
• MRI of brain and IAC

They sometimes say -- I am already dizzy - -why should I have tests done that could make me dizzier (briefly of course). Patients are also often concerned about expense of the tests, and of course, don't want to waste their time either.

There are good reasons for this process, and in this page I attempt to explain the logic. In brief, the reasons are: 1). Confirm the diagnosis 2). Rule out common alternatives 3). Follow progress of disorder. Here is the detail:

Goals of testing for Menieres's disease

• Confirm diagnosis --
  • Hearing loss (Audiogram), OAE and tympanometry
    • Is hearing loss present ? Meniere's disease cannot be diagnosed without hearing loss.
    • Is the hearing loss of the correct type (sensorineural, not conductive)
    • Does the hearing loss fluctuate ? Fluctuation of hearing is strongly supportive of the diagnosis
  • Pressure in inner ear
    • Is it present (ECOG test). A well done ECochG test that shows high pressure supports diagnosis
• Rule out common alternatives (differential diagnosis)
  • Eustachian tube malfunction (diagnosed with combination of abnormal tympanometry and normal ECochG)
  • Labyrinthitis, vestibular neuritis (tests should show stable damage to one ear)
  • Migraine (hearing and balance tests should be normal)
  • Noise induced hearing loss (tests should show noise notch, absent OAE)
  • Perilymph Fistula (imitates Menieres)
  • Otosyphilis (positive blood test)
  • Superior canal dehiscence syndrome (asymmetrical VEMP)
  • Stroke and TIA (imaging studies)
  • Tumors (progressively downhill testing, imaging studies positive).
• Measure functional status
  • Hearing testing deteriorates over time
  • Balance testing deteriorates over time

Confirming the diagnosis:

The diagnosis of Meniere's is a serious one, which has long term implications for deterioration in hearing and balance. Why confirm it (or rule it out ?)

• Knowing that you have Meniere's is important over the long term because Meniere's is an uncurable, chronic illness.
• Some of the alternative diagnoses are life-threatening (i.e. tumors, strokes)
• Unfortunately, the diagnosis of Menieres is viewed negatively by health insurance and disability plans. We have encountered situations where patients are refused life or disability insurance, or when applying for new insurance, coverage for Meniere's is excluded as a "preexisting condition".
• Meniere's is often overdiagnosed -- the Framingham study suggested that the condition is overdiagnosed by a factor of 10 !  If the diagnosis is wrong, it is best to get it "off the table" quickly.

Differential diagnosis

The differential diagnosis of Menieres is broad and includes perilymph fistula, recurrent labyrinthitis, migraine, congenital ear malformations of many kinds, syphilis, Lyme disease, tumors such as acoustic neuroma, multiple sclerosis, posterior fossa arachnoid cysts, and other rare entities. Symptoms similar to Meniere's (fluctuating hearing, tinnitus, vertigo) can also be caused by impending strokes in the distribution of the anterior inferior cerebellar artery (Lee and Cho, 2003). Bilaterality of hearing fluctuation suggests a vascular cause such as migraine.

Measure functional status

In Meniere's disease, hearing and balance generally progressively worsen over decades. This may lead to disability from hearing loss or imbalance or both. Monitoring hearing loss guides the intensity of treatment. Monitoring balance is less helpful, but may also be useful in situations where persons are working in dangerous environments where good balance is required.