THE EAR NOSE AND THROAT INSTITUTE
OF
JOHANNESBURG
------------------------
Menière Syndrome
A Short Description
by
Herman Hamersma, M.D.
Otology and Neuro-otology
Flora Clinic, Roodepoort, South Africa.
January 2012
Prosper Menière in 1833
1
CONTENTS
History………………………………………………..........3
The Clinical Picture of the disease……………………….7
The natural course of the disease……………………….11
Classification of Recurrent (viral) Vestibulopathies ….. 15
The Cause of the disease ………………………………..17
Pathophysiology of Herpes Virus reacxtivation …………20
Treatment …………………………………………………35
Surgical treatment (non destructive) ……………………42
(destructive …………………………48 - 66
Anatomy & Physiology ……………………………to follow
January 2012
Synonyms: Morbus Menière (M.M.), Menière’s disease.
P.O.Box 5973, Weltevreden Park 1715, South Africa.
hamersmah@surgeon.co.za
or
hamersma@global.co.za
2
HISTORY
Basic research in the laboratory remains a cornerstone of scientific progress. However, countless
success stories originated from the astute observations made by clinicians when they carefully listened
to, and also examined their patients thoroughly. It is with pride that we recall the events which led to the
historic presentation of Prosper Menière at the Académie impériale de médicine de Paris on January 8,
1861, when, with trepidation, he asked his colleagues whether any of them had seen similar cases in their
practices:
“A healthy middle aged person notices a sudden buzzing and hissing in one ear. The noise
increases in intensity and eventually becomes unbearable. Then a severe turning sensation
occurs which disturbs the balance. The patient has to hold on to something or has to lie down.
Nausea and vomiting then follow, similar to a severe case of seasickness. The patient never
loses consciousness. The attack usually lasts for only a few hours. The patient is exhausted
and will then often fall asleep. When the patient wakes up, the dizziness and nausea have
disappeared but the noise in the ear and a hearing loss remain. The attacks recur at regular
intervals of days, weeks or even years, and the hearing loss increases as the attacks recur.
After a number of years the attacks become less frequent and eventually cease. The ear is by
then practically deaf, as if the disease has burnt itself out. The disease never causes death.”
Prosper Menière in 1861
At the time of Menière’s presentation it was thought that dizziness (the hallucination of movement of
either the patient or the surroundings) was caused by intracranial disease, and that nausea was caused
by abdominal conditions. Menière had previously worked with Flourens, the anatomist and physiologist,
who experimented with pigeons. Flourens had caused violent rotational head movements on sectioning of
the semicircular canals of the inner ear or the cerebellum of pigeons. Thus, when Menière attended to a
girl with severe imbalance in 1837, he must have been reminded of the experiments of Flourens. This girl
had travelled in an open coach and contracted a cold. On admission to the hospital she was off balance,
3
totally deaf in both ears but had no earache. Any head movement caused severe nausea and vomiting.
She died 5 days later. At the post mortem no pathology of the brain was found but a red exudate was
present in the semicircular canals and vestibule of the inner ear. No pathology was observed in the
cochlea.
An anecdotal story often told was that Menière, while travelling in an open tram, saw a
severely unbalanced woman on the sidewalk. People were laughing at her because
they regarded her as being drunk. Menière, however, observed that her falling tendency
was towards one side and concluded that she may be ill. He got off the tram, went to
the girl and immediately saw that she was very pale and totally off balance (did he
possibly observe nystagmus?). He had her admitted to a hospital, where she died.
Today we are sure that she probably suffered from acute leukaemia with a hemorrhagic
infiltrate in her inner ear.
After having been alerted to the inner ear as a probable cause of vertigo, deafness and vomiting, Menière
took an active interest whenever patients suffered from symptoms of imbalance plus dizziness, because
they reminded him of the experiments of Flourens. He eventually became totally convinced that the inner
ear was the cause of the symptoms, and fortunately made the presentation 13 months before his own
death from pneumonia.
The presentation was followed by a lively discussion, and Menière had patients referred to him.
Within one year Politzer in Vienna (Austria) and Voltolini in Breslau (Poland) reported similar
observations. Voltolini was the first scientist to start using the term Morbus Menière (Menière’s disease).
Thirteen months after his presentation Menière died from pneumonia.
What a pity that not all the colleagues in France took note of the syndrome, because then Van Gogh, the
painter, would not have been regarded as psychotic, but as suffering from episodic cochleovestibulopathy, i.e. Menière syndrome.
4
This painting, of a fellow patient, was made by Van
Gogh when he was a voluntary patient in the clinic in
St. Rémy in southern France.
5
Other famous people who suffered from this condition were:
 Martin Luther (1483 – 1546), theologian, who had severe vertigo attacks and a unilateral hearing loss and
tinnitus. Legend has it that Luther threw an ink pot at a shadow which he thought to be the devil himself.
Could it be that this happened during an attack of vertigo and roaring tinnitus, when nystagmus made the
shadows produced by candle light move? – see next page.
 Bedrich Smettana, the Czech composer (1824-1888), who became totally deaf in 1874 and because of
depression had himself admitted to an asylum and stayed there until his death;
 General Lee (1807 – 1870) of American civil war fame;
 Alan Shepard, the first astronaut to travel in space (a successful endolymphatic sac operation had
previously been performed on him by Dr William House of Los Angeles).
 If Shakespeare’s writings are taken seriously, the possibility that Julius Caesar suffered from Menière
syndrome can be considered, because he spoke of a deaf ear, and he got seizures which could have
been severe vertigo attacks.
The past 146 years saw a tremendous accumulation of knowledge about Menière syndrome, but the end
is still not in sight. The rest of this paper deals with exciting new and perhaps revolutionary view points
concerning the etiology and mechanism of Menière syndrome, especially the fact that more nerves than
the eighth cranial nerve may be involved during a vertigo attack. The author has taken this knowledge
into consideration during the assessment of hundreds of patients with Menière syndrome seen during the
last eleven years, and is convinced of the value of this new approach.
Prof Harald Feldmann from Münster University published research on Martin Luther’s affliction.
Quote (translated from German by the author):
"Luther had his first major attack in 1527 at the age of 43. As had often been the case, he
had many guests at his house. He did not feel well and lay down for a while, but later
joined his guests at supper. He became unwell again and his friend Jonas recorded:
He complained of an uncommon and annoying humming and ringing in his left ear. This
ringing and hissing noise became more severe and he reclused himself. When he entered
his bedroom he felt weak and said to me: ‘Oh Doctor Jonas, I feel unwell. Give me water
please, or whatever you have, or I die!’ Luther became deadly pale but not unconscious.
He was convinced that he was going to die and he prayed aloud and took leave of his
family. Dr Schurff, his doctor, was called and could not help but prescribed heat
treatment. Luther then fell asleep. The next morning the nausea was gone but he
complained of a horrible hissing in his left ear. That evening he could join his guests at
supper.
These attacks then recurred at irregular intervals during the following months and years.
The tinnitus in the left ear became Luther’s constant companion until his death. However,
in later years the attacks became less frequent and less severe. Luther died from a heart
infarct 19 years after the first attack of vertigo. Luther regarded his suffering as an
unnatural disease caused by Satan. In 1530, after having suffered for ore than two years,
Luther wrote to his friend Hans Honold: “I regard it that the loathsome fellow from hell
does not like my presence in his kingdom on earth.”
6
The incidence of Menière syndrome
The common age of onset is between 30 and 50 years with a slight preponderance of females. Children
are seldom affected, but it has been reported at the age of 9 years. Elderly people also get the condition,
even at age eighty.
It has been stated that annually about 100,000 cases of classic Menière syndrome are diagnosed in the
United States (440 per million inhabitants), and in Japan it is estimated that 40 persons per million
inhabitants suffer from the classic syndrome. In the United Kingdom one person per 1000 inhabitants
have the condition, and in Sweden one person per 2613 inhabitants.
If one takes into account that these figures do not include the vestibular form of Menière syndrome, an
additional 30% can be added to the figures.
Genetic predisposing factors are suggested by frequently positive family histories, but this has not been
proven conclusively.
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The Clinical Picture of Menière Syndrome
Attacks of
dizziness and imbalance without hering loss =
(vestibular Menière or recurrent vestibular neuritis –frequent
or
fluctuating hearing loss only
(cochlear Menière – rare);
or
dizziness plus ear symptoms
(classic Menière – frequent
plus
Symptoms between attacks
----------------------------------------------------
7
VARIATIONS IN THE SYMPTOMATOLOGY
The syndrome complex which Meniére described is currently known as Classic Menière's and presents
no problems with diagnosis. However, in the majority of patients suffering from Menière syndrome, the
first couple of attacks of vertigo need not be accompanied by any ear symptoms, thereby confusing the
doctors who deal with the patient in the acute stage. It is now known that this can be the vestibular form
of Menière syndrome. To confuse the issue even more, in a small percentage of classic Menière's
cases the first attacks can have episodes of fluctuating hearing los and noise in the ear (tinnitus) without
any dizziness or rotational dizziness (vertigo), and this is then called the Cochlear form of Menière
disease.
.
By and large a considerable number of cases can eventually convert into classic Menière's. Fortunately a
percentage of cases who suffer from the vestibular form of Menière’s do not change to classic Menière’s,
and can then be regarded as episodic vestibular neuritis (see Gacek & Gacek’s explanation of recurrent
viral vestibulopathies at the end of this article).
A very important aspect of the disease is the fact that, after one single attack, or after a few attacks over
some months, the condition can go into remission, either for many years or even permanently. Not only
do these present problems as far as the etiology is concerned, but also leads to erroneous conclusions
about the efficacy of treatments.
The variation in symptoms support the proposal that a virus infection is the cause of the
disorder, and that endolymphatic hydrops only develops later on as a complication due to
damage to the fluid system. Microscopic examinations of inner ears of patients who died from
other diseases have shown that virus infections of the inner ear do not always follow set
patterns. The involvement tends to be patchy, similar to loose thunder storms.
1..Vestibular Menière (attacks of dizziness only)
This can also be called:
Recurrent attacks of vestibular neuritis of the whole nerve, or of the superior
branch only, or of the inferior branch only, with involvement of the central
connections also..
The cardinal symptom of Menière syndrome is repeated attacks (episodes) of sudden rotational
(spinning) dizziness - real vertigo), i.e. an illusion that the patient is spinning or that the surroundings are
spinning), plus loss of balance. The patient feels as if he is falling to both sides, but later on the falling
tendency is to one side only. The attack reaches a maximum intensity within a few minutes, usually lasts
for minutes to hours, and then the intensity decreases. Attacks most often last 30 minutes to 6 hours, but
can even last up to 48 hours. In exceptional cases an episode of vertigo may only last 5 – 10 minutes,
but never less than 5 minutes.
The patient gets little or no warning of the approaching attack, and it is not precipitated by positional
changes. However, once the attack has started, the slightest head motion accentuates the symptoms.
Some patients find that visual stimuli can trigger an attack, but this probably occurs when an attack is
latent and then manifests itself. Attacks can occur both during the day and even during sleep. In a great
number of patients the first attack (and many subsequent ones) is experienced when waking up in the
morning and when the patient makes the first movement.
NB. Occasionally a patient is so overwhelmed by the symptoms of the first attack(s) that they state that
they do not recall any spnning sensations – but usually they do observe them during subsequent attacks.
8
Varying degrees of nausea and vomiting, sweating, and pallor (drop in blood pressure) make the attacks
very unpleasant. The drop in blood pressure is part of the autonomic (vegetative) symptoms, and this
can mislead the attending doctor to make a diagnosis of an attack of low blood pressure.
Rhythmic jerking eye movements (nystagmus) occur and this may blur vision to a certain extent. The
nystagmus consists of a horizontal-rotatory slow movement to one side, followed by a rapid horizontal
(correcting) phase back to the midline. Occasionally the nystagmus may be more vertical than horizontal.
These eye movements are often visible to bystanders, especially if the patient is asked to look straight
ahead. If recorded on a tracing, the eye movements will have the appearance of saw teeth. The slow
phases represent the peripheral vestibular stimulations, and the fast phases are correcting phases by the
brain because the anatomical situation of the eye muscles do not allow the eyeball to rotate 360 degrees
in the eye socket
The custom is to describe the direction of the nystagmus according to the direction of the fast phase. This
is actually incorrect, because the slow phase represents the vestibular component, and can vary in its
speed – thereby representing the magnitude of the stimulation. The speed of the fast phase is always the
same. However, because the fast phase is the easier to observe, this phase is used to describe the
direction of the nystagmus. Very often the patient prefers to lie on one side, and this is usually the side to
which the direction of the fast phase is. The reason for this is because, if the patient opens the eyes and
looks in the direction of the slow phase, the involuntary eye movements will be far less.
These eye movements are more distinct if visual suppression is reduced by means of Frenzel’s glasses
(eyes open and +20 dioptre lenses) or eliminated by means of infrared videonystagmoscopy (eyes open
in the dark and the movements are observed or recorded with infrared videocameras) or eye movements
recorded with electronystagmography (= ENG, eyes closed and electrodes applied around the eyes).
During the attack the patient is completely orientated as to his surroundings and has no neurological
deficit such as paresthesia, diplopia, loss of consciousness, weakness or paralysis, or dysarthria.
However, subjective visual illusions can occur:
in the frontal plane the patient can experience
that the vertical axis (subjective visual vertical – SVV) is tilted a few degrees towards the
affected ear, or
(very seldom) the vertical axis can be rotated 90 or 180 degrees (upside-down inversion
of vision, or room tilt illusion – RTT), i.e. the patient observes that objects are tilted, and
in extreme cases inverted, e.g., sees somebody approaching walking totally upside
down!
It can also occur in the saggital plane and then the patient experiences falling forwards or
backwards.
These illusions last for seconds to hours. In the past these illusions were frequently reported in central
(brain) disorders, but recently they have also been reported in peripheral lesions. The explanation for
these occurrences is that a viral infection temporarily disturbs the peripheral balance organs (utricle and
saccule) as well as the central pathways of the vestibulo-ocular system. Directly after one balance organ
has been eliminated the skew deviation often occurs but usually disappears after a few days, but it may
take the brain months to accommodate to this change.
The patient can suppress the nystagmus by trying to focus on a distant object. The clinician can observe
the nystagmus much better if fixation is reduced by putting a goggle with +20 dioptre lenses over the eyes
(Frenzel glasses), or an infrared videonystagmoscope (eyes open in total darkness), or electrical
recording of eye movements while the eyes are closed (electronystagmography).
When the vertigo has stopped, the patient can feel normal within minutes. In most cases however, the
patient feels weak for hours because of the vomiting that had taken place, and also experiences slight
unsteadiness for a day or two, especially following rapid head movements. Inspection for nystagmus can
detect a slight nystagmus for a few days. A new name
“Episodic Vestibulopathy”
9
is proposed for these attacks of vertigo without ear symptoms (also called the vestibular form of Menière’s
disease). In approximately one third of patients who get these attacks, ear symptoms never develop.
These attacks can also be called recurrent vestibular neuritis (Gacek).
In the other two thirds of patients, ear symptoms will eventually accompany the attacks of vertigo (even
after months or years), i.e. the disorder progresses to the classic form of Menière syndrome.
The original description of vestibular neuritis stated that the symptoms of the attack lasted for
many days, and recovery took weeks. Caloric testing revealed weakening of one vestibular
labyrinth (called canal paresis or vestibular paresis). Nevertheless, many cases are now seen
where caloric tests do not detect weakening on one side, and the patients still get subsequent
attacks of vertigo originating from the sam side.- the original definition of vestibular neuritis can
then not apply. Sequential neuritis in the opposite side have also been reported (Schuknecht &
Witt, 1985). The fact that the caloric test only tests the superior branch of the vestibular nerve
must be kept in mind. Recently the VEMP test (vestibular evoked myogenic potential test) has
become available. With this test weakening of the inferior vestibular nerve can be detected. It is
then possible to determine which ear is the culprit . In 2002 Halmagyi, Aw, Karlberg, Curthoys &
Todd (Sydney) suggested that this be called “inferior vestibular neuritis”. In 2006 Monstad,
Økstad & Mygland (Norway) published three cases with inferior vestibular neuritis.
2. Cochlear Menière syndrome (episodic cochleopathy)
This is a very rare form of Menière syndrome. Fluctuating hearing loss, with or without tinnitus, occurs
but no vertigo develops. Most of these patients eventually progress to the classic form of Menière
syndrome.
3. Classic Menière syndrome (episodic cochleo-vestibulopathy)
In approximately one third of first attacks of vertigo, ear symptoms already accompany the first attack of
vertigo, i.e. the “triad” of “Menière’s disease” is present and this makes the diagnosis easy:
Vertigo, tinnitus, and fullness (hearing loss) in one ear.
The hearing loss and tinnitus may recover completely after the first attack, but sometimes a small residual
loss of hearing will remain. The remaining third of patients will only develop ear symptoms after having
had many attacks of vertigo alone.
The patient may get a warning of an imminent attack: low tone or high tone tinnitus suddenly develops in
one ear (sometimes even more than one type of noise at the same time), as well as a
blocked feeling (as if there is water in the ear). The tinnitus may disappear some time after the attack, but
the tendency is later on for tinnitus to remain permanently.
In the beginning the hearing loss is confined to the low tones, but eventually all frequencies are involved.
The hearing loss can also disappear after a few days, i.e. fluctuating hearing loss, but again the tendency
is for some hearing loss to remain permanently. Therefore each attack carries the threat of further
hearing deterioration.
Lermoyez syndrome is a rare feature of classic Menière syndrome: The attack announces itself by a
blocked feeling in the ear, and after a few minutes the patient experiences an exceptionally good feeling
(salubrious feeling), followed by a vertigo attack – but as soon as the vertigo starts the hearing improves!
In about two third of cases the first episode of vertigo occurs without any ear symptoms, and
the diagnosis of classic Menière syndrome cannot be made because the attack only
represents a vestibulopathy (diseased balance organ only). The ear symptoms of classic
Menière’s will then appear after the patient has had a third or fifth attack of vertigo (sometimes
even more). The clinical picture of an episodic cochleo-vestibulopathy is then established
which is identical to Menière’s original description in 1861.
10
During the early attacks of vertigo the fast phase of the nystagmus tends to be directed TOWARDS the
diseased ear, but can change to the opposite side later on in the attack. In the far advanced cases the
nystagmus tends to beat away from the diseased ear. The direction of the nystagmus can therefore assist
to determine the side of the lesion in cases where no ear symptoms have appeared, as is the case in
episodic vestibulopathy (the vestibular form of Menière’s). However, this is not a totally reliable method,
but, in the absence of other localising signs, and in case of a negative electrocochleography (Ecog)
examination, the nystagmus will serve as a guideline.
4. Symptoms and signs in the vertigo free interval of classic
Menière syndrome
The symptoms of classic Menière’s are divided between
definitive symptoms (fluctuating hearing loss and episodes of vertigo),
and
adjunctive symptoms (persistent, non-episodic and often progressive hearing loss and
dysequilibrium).
In the early stages of the disease patients are symptom-free in the vertigo-free interval. The hearing
improves to the previous level, but, depending on the number of attacks, the hearing may not recover fully
and lead to a significant percentage of hearing loss and permanent tinnitus. To make matter worse, the
impaired ear becomes hyper sensitive to loud sounds, i.e. the recruitment phenomenon, and sounds may
be distorted, which can reduce the effectiveness of a hearing aid. The term phonophobia is suggested to
replace recruitment.
Occasionally interesting phenomena can occur:
Hennebert’s sign (1911): Excessive air pressure in the external ear canal (e.g. finger tip in
the ear canal) causes momentary vertigo and nystagmus in an ear with no signs of middle ear or
mastoid disease. This occurs in 70% of ears with congenital syphilis and in 30% of ears with
classic Menière’s disease (Nadol – 1977). Explanation: the movement of eardrum is transmitted
to the very sensitive sensors inside the vestibule of the inner ear. Hydrops of the saccule often
occurs in advanced classic Menière disease, and can touch the inner surface of the stapes
footplate.
Hennebert sign must be distinguished from the fistula sign, which can be present when the bony
capsule of the inner ear is deficient – see illustration lower down.
Tullio phenomenon (1929: A loud sound, especially with a low frequency, elicits vertigo,
nystagmus and a body sway. Explanation: same as for the Hennebert sign – see the illustration
from Schuknecht’s “Pathology of the Ear” (p. 543).
-
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The natural course of the disease.
The disease has a protracted course, a serious impact on the patient’s lifestyle, and requires many doctor
hours.
The syndrome often starts with attacks of vertigo, nausea and vomiting, and loss of balance, without any
ear symptoms, i.e. an episodic vestibulopathy. The full picture of classic Menière syndrome, i.e. episodic
cochleo-vestibulopathy, often only presents after the patient has endured a few vertigo attacks without
ear symptoms.
Fortunately remissions of up to 10 years, or even permanent remissions can occur. However, in more
than 50% of cases the patients suffers from a few attacks every month or two before a remission occurs.
Some patients are less fortunate and have to endure many years of attacks before a remission occurs. In
a small percentage of cases the disease is progressive and no remissions occur.
11
The chances of the other ear also being involved are approximately 10%, although some reports in the
literature quote a 30 - 50% chance for bilateral involvement.
VERTIGO AND LOSS OF BALANCE:
The pattern of occurrence of the dizzy spells varies, but commonly there is a gradual increase in the
frequency of the spells until a maximum is reached over a period of years. During the active phase of the
disease one third of patients have more than 20 attacks of vertigo per year. Then the frequency
decreases as the disease begins to run its course and destroys the inner ear. Some patients exhibit long
remissions between attacks with or without therapy; others show a clustering of attacks with long
symptom-free periods between. It has been reported that after 5 – 10 years, there is an 80% chance of
spontaneous remission, which indicates that the disease has a relatively benign course, except for the
hearing loss which need not recover at all.
In some patients there is a tendency for the vertigo attacks to become less frequent and less severe as
the years progress, but the hearing symptoms still affects them a lot. However, the majority of patients
find that the attacks of vertigo plus loss of balance are the most incapacitating and interfere with quality of
life, and they fear losing their work. On the other hand the occasional patient may get to the stage where
the sever vertigo attacks are replaced by something worse:
Without warning, without experiencing any dizziness or loss of consciousness, the patient falls to the
ground, as if pushed by an external force. This phenomenon is called the otolithic crisis (catastrophe)
of Tumarkin. This only occurs in long standing cases, where hearing is also bad. The inner ear fluid
system is in a decompensated state (according to William House), which results in instability of the utricle
and saccule. Surgical removal of the utricle and saccule (labyrinthectomy) solves the problem. Although
these patients will have no hearing after the operation, they are very grateful and can be rehabilitated,
even in the elderly.
HEARING:
Reduced hearing can occur immediately, before or during vertigo attack, and can return to normal after
the attack, or it can only recover partially – this varies considerably. Early in the disease the hearing
fluctuates, and the low tones are mostly affected. Later on the high tones are also affected. The hearing
loss is of the sensorineural type and is characterised by reduced speech discrimination, distortion of
sound, and phonophobia (loud sounds hurt the ear). Eventually the hearing may not fluctuate at all. Total
hearing loss seldom occurs. Occasionally the disorder is very progressive, and then the hearing loss and
unpleasant tinnitus plus phonophobia can render the patient disabled for work – even if the vertigo
attacks are infrequent.
TINNITUS can be very annoying (Van Gogh complained bitterly)..
VISUAL STIMULI can be very bothersome, especially when walking in long passages (in a
supermarket). Looking at moving objects (passenger in a car, television), and looking at stripes on the
wall or on a shirt are frequent complaints from patients.
DRIVING A CAR may be dangerous. Usually the patient gets enough time to pull off the road.
Patients should be careful when driving and should stay in the slow traffic lane of a highway.
SLURRED SPEECH during the acute phase of a Menière attack are reported from time to time, and is
probably due to the virus affecting neural pathways central to the vestibular ganglion.
It is advisable not to work in hazardous situations, e.g., on a ladder, or on a roof, or with machinery like a
lathe.
12
Summary of the natural course of Menière syndrome
It is impossible to predict how the future will be because the course of the disease can vary so much.
Remissions with or without treatment often occur and the tendency is to ascribe it to the treatment given.
During the past four decades, the author has observed the following:
The first attack of vertigo without ear symptoms:
Approximately 50% of first attacks occur early in the morning after waking up and when the patient makes
a movement to get out of bed. The severe part of the vertigo lasts from 30 minutes to a few hours, and
the patient usually has to crawl to the bathroom to vomit (sometimes diarrhoea also). The next day the
patient is normal. In a small percentage of cases this is the only attack of vertigo.
The second attack of vertigo:
Within weeks or months, 50% of patients will have another attack of vertigo, usually without ear
symptoms.
Subsequent attacks of vertigo:
In about one third of cases no further attacks occur, i.e. a total remission.
In about one third of cases further attacks occur at intervals of a few months, and then the disease goes
into remission for 2 – 10 years. Many of these patients experience no ear symptoms and are usually not
diagnosed as Menière syndrome. However, some of these patients may have fluctuating hearing loss
and tinnitus, but only a small percentage have a slight to moderate permanent hearing loss.
In about one third of cases attacks recur, and these patients develop the typical ear symptoms on one
side. Of these patients some will report earache, or a warm feeling in the cheek, or pain in the throat, and
a small number will present with actual herpetic blisters on the cheek or in the throat. This indicates that
the disease is more aggressive,
and the patients are desperate to have something done to stop the attacks from recurring. Treatment to
halt the attacks can be successful, but unfortunately progression of hearing loss can occur despite
treatment.
Microscopic examination of temporal bones have revealed that viral infections of the temporal bone can
involve various sections of the temporal bone in a haphazard way (like loose thunderstorms), and this
explains the great variety of symptoms which can occur.
Please note:
Occasionally patients are seen who have attacks which last longer than the customary few days, i.e. it is
a protracted attack. These attacks are identical to vestibular neuritis attacks. In the light of the findings of
Drs Richard and Mark Gacek these protracted attacks will be manifestations of a more severe viral
infection.
It also happens that patients may get more than one attack a week, and this can continue for weeks on
end. These patients have severe stress, and it is not clear which comes first, the stress or the attack. The
result is that the patient becomes very anxious, and the term “status menièriensis may be used.
Remission:
It is known that the disease can go into remission (‘heal itself’), and this is the best the patient can
hope for - it is better than any medicine or surgery During remission the antibodies to the HSV-1,
which are in the patient’s immune system ever since the original first infection in childhood, become
more effective and allows the remission to occur.
If further attacks occur after a remission of many years:
In a small percentage the patient gets occasional attacks only, and the hearing is not severely affected.
However, many patients in this group will again have regular attacks of vertigo, and the hearing becomes
severely affected. If medical treatment does not benefit the patient, surgery is indicated.
13
Stress affects the immune system adversely, and the patient must therefore try and reduce stress as
much as possible.
Bilateral Menière disease
The chances of getting the disease in both ears are a real concern. Reports from the literaure quote the
chances of getting it on both sides from 5% to 40%. In the author’s experience not more than 10%
develop disease in the other ear at some later stage, and when this happens the second ear usually
starts within a few years already.
-
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14
Classification of Recurrent (Viral) Vestibulopathies
(Gacek & Gacek 2002)
The syndromes of Vestibular neuronitis, Benign Positional Vertigo (BPV), and Menière’s
disease are clinical expressions of vestibular ganglionitis probably caused by the alpha Herpes
virinae family. Several factors may determine the “face” presented in individual patients. These
are
the amount of virus present (viral load),
the virus type and strain,
the location and number of affected vestibular ganglion cells, and host resistance.
It is possible that other forms of recurrent vertigo are expressions of vestibular ganglionitis.
Anterograde virus strain (hearing preserved)
Superior vestibular ganglionitis (vestibular neuritis, vestibular Menière disease)
Inferior vestibular ganglionitis (BPV = benign positional vertigo)
Superior and inferior vestibular ganglionitis (vestibular neuritis and BPV)
15
Retrograde virus strain (hearing affected)
Superior vestibular ganglionitis (Menière disease, neurolabyrinthitis)
Subtype, utricular ganglionitis (Tumarkin’s otolithic crisis)
Superior and inferior vestibular ganglionitis (Menière disease and BPV)
16