Menière Syndrome
A Short Description
by
H. Hamersma, M.D.
Otology and Neuro-otology
Florida, Roodepoort, South Africa.
August 2011
TREATMENT - a
Prosper Menière in 1833
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TREATMENT of Menière Syndrome
The best result is spontaneous remission.
The primary aim of the treatment must be to enhance a
remission, and for this anti-stress treatment is essential.
Medicinal treatments against the HSV-1 are prescribed but
they must be improved.
The side effects of the disease require symptomatic
treatment.
If the disability and quality of life are severely affected,
surgical treatments should be considered because they can be
very successful.
----------------------------------------------------Until two decades ago it was customary to tell patients that
1. The cause of Menière’s disease was unknown;
2. Definitive treatment did not exist - except for destructive surgery of the inner ear/balance
nerve if suffering was unbearable, but that only helped for the attacks of balance
disturbance and did not help to stop any hearing deterioration;
3. Symptomatic treatment would help somewhat;
4. Fortunately the disease could go into remission for many years, or even “burn itself out”.
Patients therefore heard “there is no treatment” and “you must learn to live with this
disease”.
Progress in respect of 1–2 has now occurred, i.e. a likely cause has been found, i.e. replication
of the Herpes Simplex-1 Virus (the ordinary cold sore virus).
The discovery that a virus infection causes vestibular neuritis (dizziness without hearing loss)
and Bell’s palsy (paralysis of the facial muscles), and can similarly affect the hearing nerve
resulting in fluctuating hearing loss with or without dizziness (pages 37 – 51), makes it
necessary to reconsider the approach to “Menière syndrome”. Also, the original description of
vestibular neuritis or neuronitis as being a once off infection and very seldom recurrent, is not
true. Many clinicians have already noticed that a purely “vestibular” form of Menière’s disease
probably does exist, just as the “cochlear form” of Menière’s disease is seen from time to time.
Similarly, Bell’s palsy recurs in approximately 12% of patients, either on the same side or on the
other side (Hamersma-2004).
Therefore, a viral Polyganglionitis Episodica being the mechanism of Menière syndrome
has to be entertained because that mechanism is the only one which explains all the
symptoms and signs of Menière syndrome.
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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. Stress affects the immune
system adversely, and the patient must therefore try and reduce stress as much as possible.
To help the patient
Supply the patient with information about the disease:
Vestibular Menière syndrome patients are very anxious because they usually have not been
diagnosed. They are very relieved when they are diagnosed at last.
Establish a line of contact, i.e. advising the patient to make immediate direct contact with the
doctor when attacks recur, and request the patient to keep the doctor informed as to the course
of the disease (this also help to get data for research).
The patient should study http://www.timeurgency.com
What patients fear the most are the unexpected vertigo attacks. It is very helpful if these
patients are instructed to contact the treating otologist telephonically, and if possible, be seen by
the otologist as an emergency (only 10 minutes required). When the otologist sees the patient,
important information can be gained by examining the nystagmus and hearing acuity. If the
patient is seen by the personal physician (house doctor) or at an emergency care facility,
symptomatic treatment can be given, and the type of dizziness (rotatory?) should be enquired
about, the nystagmus examined and recorded to which side the fast phase is (in teaching
facilities an infrared videonystagmoscopy should be done and also recorded for scrutiny later
on). Gastro-intestinal and circulatory conditions do not cause true vertigo, and unnecessary
gastroscopies, MRI’s, ECG’s, etc. can be avoided.
Antivirals:
At present no medicines to kill the virus (like an antibiotic kills bacteria) are available, but
‘antivirals’ which suppress replication of the viruses (not 100% effective at this stage) are
available:
Acyclovir (Zelitrex and Famvir) can be taken orally at the full dose for a week as soon as an
attack occurs, followed by a reduced dose dose for a few weeks, and then once daily for an
extended period in order to try and prevent recurrences (caused by the virus escaping from the
ganglion).;
Gancyclovir solution introduced into the middle ear cavity is being tried out on an experimental
basis;
L-lysine, an essential amino-acid, is prescribed because it has been discovered that this food
substance, if taken in high doses, can inhibit replication of the herpes virus (see
http://www/smartbodyz.com/cold-sores-lysine-1.htm).
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Steroids:
Once the virus has escaped from its hiding place, the antigen-antibody-complement reaction
takes place (“Krieg im Innenohr = War in the inner ear). The effect of this war in the vestibular
and cochlear nerves can be reduced by giving steroids for a short while.
Treat symptomatically:
In all diseases the symptoms and side-effects have to be treated, and if the cause of the
disease is known, that should also be addressed. This is the case with Menière syndrome also.
The perception amongst patients and doctors is that there is nothing that can be done for these
patients. This is not the case at all. The patient will already be less stressed if the symptomatic
treatment is given, the condition is properly explained to the patient, and dangerous conditions
like intracranial tumours are excluded.
Aim at spontaneous remission by encouraging the patient not to lose hope. This is the most
effective treatment, and there is a chance that it may happen.
All reports of successful treatment must be weighed against the possibility that this
could have been because of a natural remission!
Following the discovery of apparent endolymphatic hydrops in 1938, treatments aimed at
reducing the pressure of the fluid inside the endolymphatic tube were developed. Diuretic
tablets, decreased salt in the diet, reducing fluid intake, and also surgery to the endolymphatic
sac and the saccule did not give the final answer. The discovery of Gacek et al indicates that
the appearance of ‘hydrops’, always attributed to increased pressure in the endolymphatic
system, could also be a result of damage to the membranous labyrinth caused by fibrosis in the
perilymphatic spaces.
Attempts to increase the blood supply to the inner ear by means of carbogen inhalation (95%
oxygen mixed with 5% carbon dioxide), nicotinic acid tablets, histamine intravenous infusions,
and Serc tablets (betahistine) are still being advocated. These treatments do not influence the
vertigo attacks, but may be of some value for the hearing loss and the tinnitus.
Management Strategy
A.
Treatment of the acute episode;
B.
Treatment between episodes.
----------------------
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A -TREATMENT OF THE ACUTE ATTACK
(1) - For the dizziness, loss of balance, nausea and vomiting:

Vestibular suppressants by injection, suppository or tablets, e.g., cyclizine lactate (Valoid),
cinnarizine (Stugeron), or prochlorperazine (Stemetil).

If the vomiting is severe, intravenous fluid may be needed. Some patients may become
dehydrated, and admission to hospital for a day or two will then be indicated.
(2) - Antiviral treatment for the Polyganglionitis Episodica (PGE):

Valacyclovir (Zelitrex), or famciclovir (Famvir) orally, are given three times daily for a week
because the virus escapes from the ganglion for one week only. After one week a twice daily dose is
advised for weeks until the attacks stop, followed by a once daily dose for an extended period may be
given to prevent the virus to escape from its hiding place.

Gancyclovir instillations into a special wick placed into the round window niche have been
used in 200 patients by Gacek et al (USA) and Guyot & Kos et al (Genève, Switzerland). The hearing was
not damaged and the preliminary results have been encouraging (for details see under surgery). H.H.
used this method in 16 patients, but the long term results are not encouraging.

NEW: L-lysine capsules 1000 mg per day (capsules contain 500 mg) may make herpes
flare-ups milder and less frequent and this therapy is worthwhile trying.
(3) - For the Antigen-Antibody-Complement reaction:
High doses steroids, e.g., prednisone or methyl prednisolone (if necessary give this intramuscularly or
intravenously), followed by oral tablets for 4 days (b.i.d.), and then taper over another 6 days. Very often
dramatic improvement can be expected within 24 hours.
(4) - Antistress treatment:
Supply information about the disease – this will help to reduce stress, and encourage the patient.
Sedate the patient by prescribing a tranquiliser, e.g., alprazolam, diazepamn, lorazepam, etc.
Stress affects the patient’s immunity adversely, and reduction of the stress should be attempted, e.g.,
try and eliminate stress at work and at home. A tranquilizer is recommended during the acute stage as
well as between attacks.
B. TREATMENT BETWEEN ATTACKS
(1)
Antiviral treatment
Daily Antiviral tablets, e.g., Zelitrex 500mg, or Famvir 125 mg twice daily, or Acyclovir (generic) 400-mg
tablets given twice daily for 3 weeks, followed by a daily dose for many weeks is recommended in order
to try and prevent the HSV-1 virus from escaping form its hiding place in the ganglia, and thus prevent
further attacks (see recent presentation of Prof Gacek). This treatment has been taken by scores of
patients for the past five years, and preliminary findings are encouraging. Absolute proof of the efficacy of
these medicines against HSV-1 is not possible via a double blind study due to the variability in the
recurrence of attacks of vertigo. However, a recent editorial in the New England Journal of Medicine (Vol.
350:67-68, January 1, 2004) on the findings with HSV-2 gives credence to using it for HSV-1 infections;
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“One of the main lessons of antiviral-drug therapy is that the drugs that inhibit viral replication are
frequently more effective at preventing viral disease than they are at treating established disease.
Acyclovir, for example, when taken on a daily basis, will prevent outbreaks of recurrent genital herpes
infection, but when taken early to treat an episode of recurrent genital herpes, the result is a shortening of
the course of the disease by only one day – a marginal clinical benefit.
The report by Corey et al (N Engl J Med 2004;350-:11-20) records another important advance in the
use of antiviral drugs in cases of, i.e. the incidence of clinically symptomatic HSV-2 infection was reduced
by 75 percent.
These results have enormous clinical implications and have led the Food and Drug Administration to
approve a new indication for valacyclovir: the prevention of sexual transmission of HSV infection.
Based on the above, it is reasonable to prescribe the antiviral treatment for HSV-1 infection.
L-lysine capsules, an essential amino-acid) 1000 mg per day (capsules contain 500 mg) may
make herpes flare-ups milder and less frequent, and this therapy is cheap and worthwhile trying.
(2)
Vestibular suppressants
Cinnarizine (Stugeron), or cyclizine lactate (Valoid) may be taken for a few days, but not for longer than 1
– 2 weeks at a time.
(3)
Other medicines:
Betahistine (Serc) is supposed to improve the bloodflow to the inner ear. It can be tried for one month
to influence the hearing symptoms, but if it does not prove beneficial within one month it has no sense
it using it on a long-term basis.
(4)
Rule out other diseases as a cause of the symptoms
e.g., systemic diseases, and do an MRI (+ contrast) investigation of the brain and inner ear canals to
rule out acoustic neuroma, MS, and other intracranial pathologies.
(5)
Anti-stress treatment:
When a patient gets frequent attacks, advise sick leave, prescribe a tranquilizer, and recommend less
stress at work and a less hectic life style. The dishes need not be done immediately after a Sunday lunch
– go and rest for a while before attending to the chores!
NB NB http://www.timeurgencyc.om
(6) Other measures:
Surgery, to reduce the endolymphatic hydrops, i.e., draining the endolymphatic sac: The long term
results of this surgery have shown that this is not the final answer. However, encouraging short term
results frequently appear in the literature, and therefore this surgery has a place to try and interrupt
frequent attacks of vertigo, and thereby getting the condition into a remission. Because this is not
destructible surgery, it can be done in early cases also, because there is a chance of preventing further
hearing loss. The chances of damaging the inner ear are small, and in refractory cases destructive
surgery can always be done later on.
.
Vestibular rehabilitation exercises, or Tai Chi, or Yoga exercises can be of value if the patient’s
balance does not recover after an acute episode (http://www.smartaichi.com/
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Medicines introduced into the middle ear space:
Antiviral solution into the round window niche via a special wick has been used by Gacek et al (USA 100 patients) and Guyot, Kos & Montandon (Genève, Switzerland – 100 patients) with favourable results.
This method can now be tried before gentamicin or streptomycin, because this treatment does not
damage the hearing. For further details see under 'surgical procedures'.
If the attacks occur frequently, instillation of steroids into the middle ear (recommended by Shea) as
well as systemic steroids for a few weeks help the inner ear, and do not damage the hearing. Animal
experiments performed by Parnes have confirmed that instillation of a steroid into the middle ear results
in a higher concentration of steroid in the inner ear fluid is than is the case when only systemic steroids
(per mouth or intramuscular injection) are given. This treatment is now advised for patients who get
frequent attacks (in preference to gentamicin instillations) - see under surgical treatment.
Instillations of gentamicin or streptomycin into the middle ear have now been in use for many years,
and reasonable results have been reported. The aim of the treatment is to reduce the sensitivity of the
balance organ (see under surgical treatment). Good results are supposedly possible even without
reducing the vestibular organ’s activity to zero, i.e. creating a ‘functional labyrinthectomy’. The author has
used this treatment in 270 patients over 10 years, and has abandoned it because the long term results
of intratympanic medicines are not good, and this is probably due to the fact that the medicine does
not penetrate deep enough to reach the viruses (which are inside the balance and hearing nerve).
Homeopathy and Acupuncture
To the author’s knowledge no definite proof of the benefit achieved by homeopathy and acupuncture has
been presented. At the moment these treatments will have to be classified as placebo treatments
Placebo treatment:
Placebo:
 An inert substance given as a medicine for its suggestive effect.
 An inactive substance administered to a patient usually to compare its effects with those of a real
drug but sometimes for psychological benefit to the patient through his believing he is receiving
treatment.
 An inert compound identical in appearance with material being tested in
o Experimental research, which may or may not be known to the physician and/or patient,
administered to distinguish between drug action and suggestive effect of the material
under study.
Numerous placebo treatments are available. They vary from herbs to increased air pressure (with a
machine) via a grommet in the eardrum. As long as these placebo treatments are not too expensive they
probably do no harm (except to the patient’s bank account). However, they do tend to support myths
about the disease, and, most important, contribute to the bank accounts of unscrupulous businessmen.
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.
A course of daily intramuscular injections of streptomycin sulphate can be used in cases of incapacitating
bilateral classic Menière syndrome. The balance organ on both sides are then made less sensitive
(titration method of Silverstein – stop injections as soon as the patient is better), or can be knocked out
totally by consecutive injections until the caloric tests are negative (Schuknecht). If given correctly, no
hearing loss occurs due to the injections. The titration method is favoured because if attacks recur, the
treatment can be given again. The results of these treatments are good as far as the balance attacks are
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concerned. However, patients may suffer from chronic ataxia (loss of balance) for quite a while, as well
as oscillopsia (the horizon moves when walking or in a car – due to impairment of the vestibulo-ocular
reflex – the eyes do not automatically focus on the horizon when the head moves). The ataxia and
oscillopsia usually stops after 6 – 12 months thanks to central compensation by the brain.
In order to reduce the chances of oscillopsia, intratympanic gentamicin instead of intramuscular
streptomycin injections were used for the first time in a few patients with bilateral Menière disease by
Schmidt & Beck in Germany in 1980 (the second ear was treated six months after the first ear). Pyykkö
et al (Finland 1994), also treated patients with bilateral disease when they had reason to believe one
ear’s disease was active (and causing attacks of vertigo) while the other ear was in remission: the active
ear was then treated – and they reported good results.
The big problem with bilateral disease is the fact that a small number of patients do not recover
adequately from the ataxia, and we do not have final methods to assess the capacity for adaptation
beforehand. Therefore these patients must be treated very slowly- see bilateral neurectomy on page 48.
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Surgical Treatments: non-destructive or destructive
Non-destructive:
If the attacks of vertigo do not stop on its own, surgical treatment of the diseased ear can sometimes
stop these attacks by means of medication directly into the middle ear space (antiviral or steroids), or
weakening of the balance organ on that side (with an ototoxic antibiotic). Please note that no effective
treatments for the hearing symptoms exist at present, although some good results have been reported
following intratympanic plus systemic steroids (Shea).
In 1926 Georges Portmann developed an operation to decompress the endolymphatic sac, which
should then help for the vertigo attacks. This operation, and its many modifications, e.g., of draining the
fluid inside the sac, was used frequently for many decades, but the long term results were not
encouraging. Many other operations were developed to relieve the pressure of the endolymphatic system,
e.g., sacculotomy (Fick), the ‘tack operation’ (Cody), an internal endolymphatic shunt by means of
cochleo-sacculotomy (Schuknecht), but all of them have been abandoned. Recently modifications of the
technique of the sac operation were published. Reports from China about the results in 3000 patients
indicate that this operation has a place to try and interrupt frequent attacks of vertigo, i.e. to get the
condition into remission. There is also a chance of possibly preventing further hearing loss, and therefore
the operation may be done in the early stages of the disease. If the condition resumes after a while,
destructive surgery can still be done.
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43
44
Medicine into the middle ear space:
Treatment of the inner ear by means of steroids instilled into the middle ear space via an incision into
the ear drum (lignocaine can be added for possible help with tinnitus);
Treatment of the inner ear by means of antiviral solution instilled into the middle ear space. The
author has used this method in 16 patients, but the series is too small to express an opinion.
Reducing the sensitivity of the balance organ by instilling an ototoxic antibiotic (gentamicin or
streptomycin) into the middle ear.
Intratympanic Steroid treatment:
Good results were reported by Shea et al when this treatment was given early in the disease.
Lignocaine, a local anaesthetic, can be added to the steroid in order to influence the tinnitus. Daily
instillations for one hour are given for three consecutive days, together with high doses of systemic
dexamethasone (which are then continued orally for at least another week). According to the Shea Clinic
in Memphis, USA, favourable results were obtained in respect of the fullness in the ear, low frequency
tinnitus, fluctuating hearing loss, as well as the frequency of the vertigo attacks. As the steroid treatment
does not stop the vertigo attacks, streptomycin instillations are given. Parnes et al (Canada) use this
treatment for sudden sensorineural hearing loss.
The author has applied this method in 40 patients. The series is too small to express a final opinion.
However, it is worthwhile to give this method a good trial, especially since the laboratory and clinical
studies of Parnes and co-workers (1999) reported favourably about this method.
Intratympanic Antiviral Treatment:
Treatment of the inner ear by means of antiviral solution (Gacek, Guyot, Kos and Montandon) is applied
to the round window niche by means of a special wick introduced into the middle ear via either a
myringotomy or after lifting a posterior tympanomeatal flap. The antiviral solution is very irritating to the
mucosa and cannot be instilled into the middle ear as is done with steroids or antibiotics. The wick is
placed via a minor surgical procedure, and the wick is moistened with the antiviral solution. The patient is
then discharged from hospital and the wick is moistened once daily for 5 – 7 days in the doctor’s
consulting rooms by injecting the solution into the wick. The author has used this method in 16 patients,
but the series is too small to express an opinion.
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Decreasing the sensitivity of the balance organ:
Without performing actual surgery on the inner ear, this method can achieve good results. The aim of the
method is to reduce the sensitivity of the balance organ so that the vertigo attacks are not so severe.
However, it has been found that this method also influences the repetition of the vertigo attacks (possibly
by helping to get the condition into remission?).
The balance organ is weakened by means of gentamicin or streptomycin which is introduced into the
middle ear space through an incision into the eardrum. From the middle ear the medicine reaches the
inner ear by absorption through the very thin round window membrane. These antibiotics are toxic to
both the balance and the hearing organ, but first affect the balance organ. The method has now been
developed so that the balance organ can be damaged first, and the hearing not affected at all.
The method was developed in Germany by Lange (1977), and also used by Beck and Schmidt (1978). At
first it was intended for patients whose hearing was bad already. Nedzelski et al (1992, Toronto, Canada)
used this “shotgun method” in a large series of patients. The patients were admitted to hospital and a thin
tube inserted through the eardrum. Gentamicin was then introduced three times a day for three days.
Within a few days the patients felt like walking on a ship. Depending on how active the balance organ was
before the treatment was started, the patient could then be severely off balance for many weeks.
Because of the good experiences gained with the titration method of administering streptomycin
intramuscularly, the same titration principle was applied to gentamicin treatment, also with success.
Parnes et al (London, Ontario, Canada) introduced the antibiotic through an incision in the ear drum
(myringotomy only), or introduced a grommet into the ear drum (local anaesthetic in the office), and left
the antibiotic in the middle ear space for 20-30 minutes only, with repeat instillations scheduled once
weekly for 3-4 weeks. None of the patients experienced any hearing losses due to the antibiotic.
If the author decides to use gentamicin treatment, a modification of the method of Parnes is used. After
an MRI of the brain and internal ear canals has excluded any other pathology, a caloric test is done to
determine the capacity of the balance organ before treatment is started. The first dose is for 10 minutes
only and is given via a myringotomy. If necessary, subsequent doses (of 10 – 20 –30 minutes) are only
given once the side effects of the previous instillations have disappeared totally (usually after 4 weeks or
more), and after a caloric test and rotation test has been done to monitor the effect of the treatment (the
myringotomy incision will have healed after 2-3 weeks). The side effects can be monitored by means of
looking for spontaneous nystagmus with Frenzel’s glasses, or infrared videonystagmoscopy, or ENG. In
case of doubt, the balance capacity can also be determined quantitatively by means of computerised
static posturography: the patient stands on a piezo electric platform with eyes open, and then closed. A
foam cushion is then placed on the platform, and again the eyes are kept open and closed. Hopefully the
dynamic computerised posturograph (Equitest) will also be available in due course, but with the
weakening of our local currency this will probably not be realized in the near future.
Usually the treatments have to be given at monthly intervals. However, if the patient is lucky, no attack
occurs after the first treatment, and then one can wait and see, because it is possible that the disorder is
going into a remission on its own or due to the treatment. The fact that the patient receives active
treatment helps to reduce stress, and in that way influences the disorder favourably.
The author’s experience gained with 270 patients during the past six years was favourable in that no
hearing losses were caused by the antibiotic (gentamicin and sometimes streptomycin). However, the
gentamicin could not eliminate attacks in all patients, even after reduction in the vestibular responses to
caloric testing had been achieved. Vestibular neurectomies via the middle fossa approach (with removal
of the vestibular ganglia) were still needed in some patients who had serviceable hearing, while patients
with unserviceable hearing had to undergo labyrinthectomies.
Prof Gacek has the following explanation (personal communication):
“Regarding the gentamicin, I am quite sure that gentamicin, even thought it relieves symptoms,
incompletely ablates the vestibular labyrinth, particularly the utricle and saccule, which can both
be responsible for ataxia. This would be so even though the caloric response is absent.”
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The advantage of the minimal method is that it can be used early in the disease, and also in the
vestibular form of Menière (where the patient has normal hearing), provided of course that the
diseased ear is identified. Electrocochleography can help to identify the diseased ear. If the patient is
seen early during an attack of vestibular Menière’s, the author found that the nystagmus often has the
fast phase towards the diseased ear, and later on the direction changes to the other ear..
Prof Thomas Brandt (München) concludes as follows about intratympanic gentamicin therapy: There is
no general agreement on the optimal concentration, temporal sequence, and total dosage of
intratympanic gentamicin instillations. All the reported experience with this kind of treatment indicates
that one injection per week on an outpatient basis could be recommended in order to better monitor the
delayed ototoxic effects. Low-dose treatment - which does not even diminish or abolish caloric responses
of the treated ear – has been demonstrated to be effective, and is therefore recommended as the standard
procedure.
No method of treatment is perfect, however. Halmagyi (Australia - 1994) reported that in Menière's
disease, despite reducing the activity of the diseased balance organ, 15% of patients nevertheless
develop symptoms of chronic vestibular insufficiency, such as oscillopsia with head motion and
unsteadiness. This can be partly attributed to the impaired vestibular function of this (treated) labyrinth,
and to incomplete central compensation for loss of peripheral function during rapid head movements (Aw
et al, 1994).
It must also be kept in mind that progression of the Menière disease can continue despite treatment.
Concomitant disease of the brain or cerebellum is also possible (especially in older persons), and these
can be very difficult to assess.
Conclusion by the author:
The partial or total ablation of the function of the vestibular system caused
by the treatment, has the major disadvantage of making monitoring of the
disease process inaccurate – because the disease can already reduce the
vestibular function.
Remember, the disease may even become a bilateral condition (Schuknecht
& Witt – 1985 ). Monitoring the progress will then be totally impossible.
The value of intratympanic gentamicin treatment should be reviewed.
This treatment is not beneficial in the long run.
Destructive Surgery See Treatment - b
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