Testing for Meniere's disease

Timothy C. Hain, MD • Page last modified: February 12, 2022

Main Meniere's page is here:

Whats new: VHIT plus caloric testing is a recent addition to the diagnostic repertoire. Serial audiometry is still the best method.

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

In our clinical otoneurology practice in Chicago, we usually see at least one new patient with Meniere's disease every week. As of 2022, we had more than 1000 patients in our clinical database.

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

Some practices (not ours) also do "dehydration tests" using the glycerol, isorbide or furosemide to rule in Meniere's with a variety of outcome variables. (Lee et al, 2016)

Seo et al (2017) reported using the "furosemide loading test" with VEMP, and reported "Normalized amplitude of VEMP, using a tone burst sound at 250, 500, 700, 1000, 1500 and 2000 Hz, was measured before and after furosemide administration in the two groups. Improvement ratio (IR) of amplitude was calculated at each frequency." This to us seems like a large # of VEMP tests 6 per ear, before and after a strong diuretic (which might have consequences as well).

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.

MRI testing is very low yield in patients with "definate or probable" Meniere's disease (Robinette et al, 2018). It would seem to us that if someone starts with a "classic" low-tone hearing loss, it would be extremely unlikely to find an acoustic. The author of this page did, however, once find an acoustic on the opposite side of the Meniere's ear. This was probably just serendipity.

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 the disorder. Here is the detail:

Bedside testing

At the bedside one can get a few good clues that the patient has Meniere's disease. These involve hearing and testing for nystagmus (which requires video frenzel goggles).

Below is a short movie of nystagmus (recorded with video frenzels) during an attack in the office.

Hearing -- Almost all Meniere's patients will have some hearing loss. Usually it is enough just to screen with "rubbed fingers", and patients will indicate that one ear is louder, or that the sound vanishes first as the fingers are moved further away from the ear.

Nystagmus -- Most patients with Meniere's will have weak spontaneous nystagmus between attacks, and strong nystagmus if you are see them in an attack in the office or Emergency room.

An example of strong nystagmus in in the video above, which was taken in a patient who had an attack in the office (actually while going down in the Elevator). Typically the acute nystagmus is an "irritative" nystagmus, meaning that the eyes jump towards the bad ear. There nearly always is a small torsional (twisting) component as well, as the entire labyrinth is affected and the up/down components of the vertical canals subtract from each other

Most of the time, patients will be between attacks. Nearly always they will have a weak nystagmus (seen only on video frenzel goggles), which can be in either direction (with respect to the bad ear).

On head-shaking testing, often they will have a "wrong way" nystagmus, similar in direction to an irritative nystagmus.

 

 

Combined VHIT and caloric testing in Meniere's disease.

See also: conflicts between VHIT and rotatory chair testing.

Many have recently pointed out that there is a pattern in Meniere's where the VHIT is normal and caloric is reduced. McGarvie et al (2015) discussed that calorics are more sensitive than VHITs to Meniere's. In particular, they said "There was a clear dissociation: patients with MD had a small or absent response to caloric stimulation of their affected ear, whilst their response to vHIT was in the normal range. " Choi et al (2017) also pointed out the discrepency. Fukushima et al (2018) compared VHIT to caloric tests in patients with MRI documented endolymphatic hydrops (EH). They reported "The difference in the vestibular EH between the presence and absence of CP was not significant if assessed using the vHIT (P = .5591), but it was statistically different if assessed using the caloric test (P = .0467)." Or in other words, calorics were sensitive to endolymphatic hydrops, but VHIT was not. Limviriyakul et al (2019) reported similar findings in patients with definite Meniere's disease. Similar findings are reported in EVA, another hydropic inner ear disease. One would think that this technique might also work for perilymph fistula, but no reports are available, and lacking a gold standard for diagnosis of PLF, they seem to be likely to continue to be unavailable

In aggregate, these data are convincing. This suggests that the pattern of a normal VHIT and abnormal caloric, in a patient with hearing symptoms typical of Meniere's, raises the odds substantially of this diagnosis. This may be better than doing an ECOG.

One would think that this relationship would also hold for Rotatory chair testing and VHIT testing, as the main difference between caloric and VHIT testing is the frequency spectrum tested, and the rotatory chair covers both ends-- but this has not been examined.

Goals of testing for Menieres's disease

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 ?)

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.

References