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

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.


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).

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.

------

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;

“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/

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).