Meniere’s Disease

A disorder affecting inner ear homeostasis, manifested by

episodic vertigo lasting hours, fluctuating hearing loss,
tinnitus and aural fullness. Rarely sudden drop attacks can
develop, but when present are debilitating. The reported
prevalence varies widely but probably sits around 190 per
100,000. It is almost twice as common in women than men,
and most commonly presents in the age range from 20-60
years. Whilst usually affecting only one ear, approximately
15% of cases will involve both ears. 1. It usually arises de-
novo, but can occasionally develop following severe
infections or trauma
Diagnosis however is often not easy, as the classic
symptoms are not always present and other conditions can
manifest in similar ways. Episodes of vertigo can be
triggered in many ways, most commonly stress (physical or
emotional) and salt in the diet. Less commonly allergies,
food triggers, menstrual fluctuations amongst others can
play a role.
In addition to a careful history, documentation of
sensorineural (nerve) hearing loss is essential to help
confirm the presence of Meniere’s Disease. The commonest
cause of episodic vertigo is BPPV (benign positional
paroxysmal vertigo), but this is characterised by brief head
positional vertigo. The major differential diagnosis is
vestibular migraine (or MAV, migraine associated vertigo)
which essentially cannot directly cause hearing loss. It is not
uncommon, however to have coexisting Meniere’s Disease
and migrainous vertigo.
There is no definitive test to confirm the diagnosis. An
electrophysiologic test called the EChOG has been used in
this regard, and if abnormal, can be suggestive but in general
terms is not usually helpful. According to the AAO-HNS
guidelines a diagnosis of definite Meniere’s Disease is only
made at autopsy. Audiology and selective balance function
tests can assist in assessing severity of disease and ability to
compensate to the symptoms.
Initially one of the first steps to be taken is to rule out the
presence of intracranial pathology such as an acoustic
neuroma (vestibular schwannoma), which requires a MRI
scan of the brain.

Traditionally different phases of the disease have been

described, but rarely does the disease follow a predictable
pattern. In general terms though over time many people
suffer from a progressive hearing loss, and eventually
recurrent vertiginous attacks fade. Like the permanent nerve
damage to the hearing nerve, permanent damage to the
vestibular system also occurs. This can lead to permanent
imbalance, dizziness, and what is often described as a
‘cotton wool’ feeling in the head. These symptoms are more
pronounced if other components of the balance system are
impaired. These include eyesight, joint function (including
the neck), and cardiovascular health.

The overwhelming goal of long term management is to

minimise the number of vertigo attacks and maintain hearing
and balance function, and in the acute phase to truncate the
severity of an attack.

Management of acute vertigo

Usually this involves the use of vestibular suppressants.
Commonly used are Stemetil, Phenergen, Lorazepam. Often
due to severe accompanying nausea, taking an ondansetron
wafer under the tongue 10 minutes prior to administration of
a vestibular suppressant can help with absorption. Due to
severe vomiting occasionally an injection is required.
Sometimes patients get a premonitory sensation and can
abort an attack with the early use of one of the vestibular
suppressants. Ocassionally a diuretic or an oral steroid can
be helpful in this circumstance.
During a prolonged attack or a series of attacks a short
course of oral steroids or an intra-tympanic steroid can be
used. (This involves an injection through the ear drum under
a local anaesthetic in the clinic. 2,3

Prophylactic Management

As the pattern and severity of the disease varies greatly from

patient to patient so does their management. The first step is
to control the two major triggers of an attack; any
physiologic or emotional stress and salt in the diet.
A low salt diet from a Meniere’s perspective requires a
significant change in lifestyle, even in those who generally
live a healthy life and do not add salt to their food, or eat
much in the way of processed foods. There is significant
hidden salt in many foods, especially salt and breakfast
cereals. Obtaining salt contents in all foods is essential
(which we can supply), and the book ‘Salt matters’ is an
excellent resource. The only way to monitor salt intake is to
take a 24 hour urine collection. In general terms the first
goal is to achieve less than 1200mg/day. If vertigo
continues then further tightening to less than 600mg/day is
targeted.
Other considerations such as withdrawing caffeine,
chocolate, smoking and alcohol are important for some
patients.

Management of stress and ensuring adequate sleep is also

essential. Alternative treatments such as massage therapy
and acupuncture can be helpful, as can formal psychological
treatment in the form of cognitive behavioral therapy,
especially in severe cases where consideration of surgical
intervention is being made. 4

Management of inhalant and food allergies is important in a

subset of patients. Allergies are up to three times as
prevalent in Meniere’s Disease. Interestingly this is
especially the case if also suffering from Migraine. The
symptoms of MD are generally better controlled, with fewer
vertigo attacks and more stable hearing, in those patients
with allergy and MD whose underlying allergic disorder is
down-regulated with immunotherapy and/or dietary
avoidance of reactive food allergens. 5

When there is inadequate control medications and surgical

interventions must then be considered.

Drug Therapy
The commonest drug used is Betahistine (Serc). It is thought
to act by increasing the blood supply to the inner ear, acting
as a H3-antagonist. Anecdotally betahistine is helpful in
some patients, but again there is no overwhelming evidence
to support it’s use. 6 A recent paper has suggested that it acts
on endolymphatic sac. 7 It has a very low side effect profile,
so is worth trying prior to interventional management.
Diuretic therapy, (fluid tablets) have been used for many
years in Meniere’s. In theory they may alter in the
electrolyte balance within the inner ear. There has been
controversy over their use, with no evidence to support their
use. 8 Side effects at the low doses used for Meniere’s
Disease however are minimal, so can their use can be
considered. A typical drug is Moduretic:Amiloride HCl 5
mg, hydrochlorothiazide 50 mg; 1-2 tablets per day.
Migraine Prophylaxis: As mentioned it is not uncommon to
have co-existing migrainous vertigo, or it is difficult to
differentiate. Many of the same triggers exist such as any
physiologic or emotional stress and certain foods. There is a
low threshold to use migraine preventors, initially non-
pharmacologic supplements containing magnesium,
riboflavin and feverfew. Pharmacologic options include
amitriptyline, dothep, pizotifen, propranolol, topomax.9-11

Interventional management (Surgery)

Intervention in Meniere’s disease is divided into non-

destructive and destructive categories. Again the principal of
any intervention is to stop vertigo, prevent ongoing hearing
loss and minimize tinnitus and fullness, while minimizing
damage to the vestibular system. Unfortunately the
procedures with the highest rates of success carry the highest
rates of permanent disequilibrium and often place the
hearing at risk. Therefore minimal impact interventions are
often tried first, even if there is no guarantee of success. The
caveat here is in those situations where definite control of
vertigo is needed, such as in those patients carrying a heavy
vehicle license where their livelihood depends on cure from
vertigo. The development of drop attacks is another
circumstance where a more aggressive approach is needed.
Another important factor in avoiding destructive procedures
if possible, is in the presence of or chance of developing
bilateral disease. Bilateral severe vestibular loss is an
extremely debilitating condition.

Tympanostomy tube insertion (grommets) are placed and are

thought to influence inner ear pressure by stabilizing middle
ear pressure. They are predominantly helpful in reducing the
sensation of fullness, but can also reduce vertigo. The most
important fact is there is no risk to hearing nor of worsening
overall balance function. The only precaution is that the ear
needs to be kept dry. The placement of a tube also allows the
use of the Meniett device. This device emits repeated
pressure pulses with amplitude of 12 cm of water and
consist of a complex pressure wave composed of static
pressure and a 6-Hz sinus modulation. It is an essentially
minimally invasive device which in some studies has
reasonable rates of control.12-14 In Australia this is currently
not funded by insurance companies. 15
Intratympanic steroid injections, (usually dexamethasone or
methylpredisone ) have been shown some success in
reducing vertigo but with with no risk to hearing, as apposed
to gentamicin. 16,17 There is no defined regime at this point,
but my usual protocol is to repeat an injection each week or
fortnight for 4 injections. We often place a ventilation tube
at the beginning of the course to make sequential
injections/infusions easier.

Endolymphatic sac surgery. This involves a mastoidectomy

and opening of the endolymphatic sac which in theory
stabilizes inner ear homeostasis. There is no convincing
evidence to support its use, although in some hands is
reported to have good control rates with acceptable rates of
hearing loss. 18-21

Intratympanic gentamicin 22-25 has significantly changed

interventional management in Meniere’s disease over the
last 20 years. Most studies show around 80% good control
of vertigo, but with a 10-20% chance of worsening hearing
and about 2% chance of complete hearing loss.26 Essentially
this is a partial chemical labyrinthectomy, and can make
overall balance worse. Balance training in the form of
vestibular rehabilitation after this intervention is essential. If
the initial treatment fails, normally a repeat injection is
performed prior to moving on to more significant
interventions. The procedure itself can be either performed
under local anaesthetic in the clinic or in the operating
theatre. The latter allows visualization of the middle ear to
ensure that there is not a thin membrane covering the round
window which can impair absorption of the drug.
Vestibular nerve section is a highly effective procedure to
control vertigo, with only a 2% chance of hearing loss. It
does however involve an intracranial procedure, albeit with
a low rate of complications. Essentially this is considered in
young patients with good residual hearing, especially if
definitive control of vertigo is needed. 27

Surgical Labyrinthectomy is a highly effective procedure to

control of vertigo, but results in sacrifice of any residual
hearing and vestibular function on that side. 27In most cases
now a concurrent cochlear implant is placed. 28

Hearing Rehabilitation

From a hearing rehabilitation and often control of tinnitus, a

hearing aid is recommended. When an ear has no remaining
functional hearing, a cross over hearing aid is an option.
This can either utilise Bluetooth or bone conducting
technology, ie a bone anchored hearing aid [BAHA,
Bonebridge] The other option is cochlear implantation, both
in unilateral hearing loss, but even more importantly in
bilateral disease. In select cases concomitant surgical
labyrinthectomy and cochlear implantation can be
considered.28
There is some evidence that boosting the hearing can also
help to some degree with balance.
Vestibular Rehabilitation
Working with an experienced vestibular physiotherapist can
be very helpful especially before and after any destructive
procedure for Meniere’s Disease. Generally exercise is very
important and pursuits such as yoga, tai chi and plenty of
walking are beneficial.

Psychological Support
This is a very important and much underestimated aspect in
the management of Menieres disease. This can help
minimize stressors which act as a trigger to acute attacks,
and can also help in the management of underlying tinnitus,
dizziness and imbalance. A syndrome labeled
psychophysiologic dizziness plays a large role in many
patients with Meniere’s Disease. This essentially where an
insult to the vestibular system leaves a degree of nerve
damage. The brain needs to compensate for this loss and
anxiety, especially anxiety centred on the fear of further
attacks or dizziness can further amplify the symptoms of
instability.

Recommended Treatment Regime

Step 1
Confirm probable diagnosis, and exclude acoustic neuromas
Institute low salt diet, and monitor levels using 24 hour urine
collections
Lifestyle modifications, stress reduction with consideration
of psychologic therapy if required.
Consider trial of betahistine (Serc), up to a dose of 16mg
three times a day

Step 2
Consider trial of diuretic, especially if an appropriately low
salt level is not reached.
Insertion of ventilation tube, with steroid (steroid infusion)
into middle ear, or steroid injection. These treatments can be
repeated indefinitely if controlling symptoms.

Step 3
Consider trial of Meniett Device
Consider Gentamicin Infusion, or Vestibular Nerve Section
in young and with good residual hearing.

Step 4
Consider repeat Gentamicin Infusion
Consider surgical labyrinthectomy

Important Contacts and Links

Psychological Management
Cognitive behavioral therapy and Tinnitus Retraining
Therapy
Vestibular Rehabilitation
Audiology

Helpful internet resources

www.menieres.org.au
www.saltmatters.org
www.menieresnsw.org.au
www.tinnitus.asn.au

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Note a recent article outlining the link between flares in

Meniere’s Disease and stress
Stress and Unusual Events Exacerbate Symptoms in Menière’s Disease: A
Longitudinal Study

Yeo, Nicola L.; White, Mathew P.; Ronan, Natalie; More

Otology & Neurotology. 39(1):73-81, January 2018.