Ménière’s Disease

January 09, 2019 - by Preview - in About Dizziness, Ear Problems

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Ménière’s disease (pronounced “many-ears”) is an infrequent problem of the inner ear.

It is a repeating problem of the inner ear in which there is a build-up of fluid in the membranes of the inner ear giving a feeling of pressure or fullness in the ears. These membranes then suddenly burst making you feel very dizzy.  

It is characterized by 2 or more attacks of vertigo* lasing 20-min to 12 hours with fluctuating hearing loss and a feeling of fullness in the affected ear. The classical “sequential” presentation occurs in about a 1/3rd of patients.

A Meniere’s attack often starts with a sensation of ‘fullness’ in the ear with a low frequency buzzing. This is followed by hearing loss and then vertigo with nausea and vomiting. Gradually the hearing recovers.

However, in about 2/3rd of patient it is in a more simultaneous pattern where there is no warning the attack is about to happen with all the symptoms of vertigo etc. occurring at the same time. Again, the hearing gradually recovers.

The vertigo can occur at any time of day and night and be triggered by the head being in various positions. Patients may also complain that loud sounds can induce vertigo (the Tullio Phenomenon).

The clinical picture is spells of multiple irregular attacks and then spells without attacks. The disease may be controlled by medication, resolve or progresses with fluctuating but diminishing hearing loss.  Initially 1/7th of cases involve both ears, but by 10 years 1/3rd of patients have both ears involved and by 10 years it increases to ½ of patients.

In the long run the balance system can so damaged where the spinning vertigo is replaced by a constant feeling of unsteadiness** because the inner ear balance system is failing (vestibular failure).

The disease has well described variants: –

i)Tumarkin’s Otolithic Crises (Vestibular Drop Attacks): This occurs in 7% of patients in which the patient suddenly falls to the ground without loss of consciousness. This is a dangerous condition because the falls can result in injuries.

  1. ii) Vestibular Meniere’s where there is dizziness without hearing complaints or evidence of hearing problems

iii Cochlear Meniere’s where there is hearing problems without balance complaints or evidence of balance problems

  1. iv) Delayed Meniere’s where it starts with hearing loss with a later development of Meniere’s symptoms.
  2. vi) Lermoyez syndrome. In this form the hearing loss very rapidly recovers after the attack of vertigo so that the patient may describe the vertigo as “restoring the hearing”.

 

In some patients’ medical history there is a history of vestibular migraine and benign paroxysmal positional vertigo.

 

Examination

In an acute attack there are 2 phases: –

In the initial excitatory phase patients have abnormal eye movements: unidirectional horizontal jerk nystagmus towards the affected ear, and a tendency to fall to the side of the unaffected ear

In the subsequent inhibitory phase patients, the abnormal eye movements change to unidirectional horizontal jerk nystagmus towards the unaffected ear and a tendency to fall to the side of the affected ear.

The patient’s response to tuning fork test are Rinne positive in both ears with the Weber to the unaffected ear consistent with a damaged inner ear hearing damage. Patients may also have a condition called “diplacusis” where they describe the  pitch of the tuning fork as being  higher in the affected ear. In addition, patients may exhibit a positive “Henneberts Sign” where pressure on the ear canal induces eye movement. 

Most importantly of all the patient does not have abnormal brain function.

 

Hearing test abnormalities

There is a low tone neural deafness which fluctuates but declines over time.

The accepted definition of is a reduction of the hearing levels at two contiguous frequencies on the pure tone audiogram at the time of spell of ³30dB at 250Hz/500Hz/1KHz.

 

Imaging

MRI of the inner ear shows black cochlear endolymphatic lacunae (also seen in vestibular migraine)

 

For the symptomatic relief of the acute vertigo and nausea.

 

Buccastem (prochlorperazine maleate) 3mg buccal tablet for no than 2 days 3mg bd for 2 days only.

OR

Cinnarizine 30mg tds for 2 days only.

 

During an acute attack, lay down on a firm surface. Stay as motionless as possible, with your eyes open and fixed on a stationary object. Do not try to drink or sip water immediately, as you’d be very likely to vomit. Stay like this until the severe vertigo (spinning) passes, then get up SLOWLY. After the attack subsides, you’ll probably feel very tired and need to sleep for several hours.

 

For the specific preventative treatment of the Ménière’s disease

This is used when there is a significant reduction in the quality of life of the patient. It is clearly needed in patients with severe frequent attacks and not in patients with mild infrequent attacks.

 

What preventative treatment?

This is controversial – there are 2 schools of thought.

  1. a) Intratympanic injections of steroids

This has been popularized in the UK and North America. There is a body of medical evidence which supports its use e.g. Lancet. 2016 Dec 3;388(10061):2753-2762.

The indications for its use in this paper were 2-3 attacks a month for at least 6 months.

 

  1. b) Betahistine

This has been popular for many years. There is a body of medical evidence which supports its use https://www.ncbi.nlm.nih.gov/pubmed/29282702

 

The recommended dose :

48 mg betahistine dihydrochloride orally three times daily should be given for:-

3-6 months if the patient has had 2 attacks in the last 6 months

6-12 months if the patient has had 1 attack or more a month in the last 6 months

In individual cases, this dose can be increased up to 480 mg daily if no sufficient symptom alleviation is achieved after 3 months of treatment.

Recent work from Strupp M et al from the Department of Neurology and German Centre for Vertigo and Balance Disorders, University of Munich Hospital, Germany, has documented the use of approximately 1000mg betahistine per day in a group of patients which was subsequently reduced to 200mg per day with the concomitant use of MAO-B inhibitor selegiline (5mg/day) – https://www.ncbi.nlm.nih.gov/pubmed/29532287

Prescribing issues

Betahistine is contraindicated in patients with phaeochromocytoma. 

Caution is advised in the treatment of patients with peptic ulcer or a history of peptic ulceration, because of the occasional dyspepsia encountered in patients on betahistine.

Clinical intolerance to Betahistine may occur in bronchial asthma patients. These patients should therefore be monitored carefully during the treatment with betahistine.

Caution is advised in prescribing betahistine to patients with either urticaria, rashes or allergic rhinitis, because of the possibility of aggravating these symptoms.

Caution is advised in patients with severe hypotension.

Patients with rare hereditary problems of galactose intolerance, the Lapp lactase deficiency or glucose-galactose malabsorption should not take this medicine.

In vitro data indicate an inhibition of betahistine metabolism by drugs that inhibit monoamino-oxidase (MAO) including MAO subtype B (e.g. selegiline). Caution is recommended when using betahistine and MAO inhibitors (including MAO-B selective) concomitantly.

It is preferable to avoid the use of Betahistine during pregnancy.

Breast feeding: The importance of taking the medicine by the mother must be weighed against the benefits of breastfeeding and the potential risk for the child.

Fertility issues: Animal studies show no influence on fertility in rats.

The following undesirable effects have been experienced with the below indicated frequencies in betahistine-treated patients in placebo-controlled clinical trials and in post-marketing reports: very common ( 1/10); common ( 1/100 to <1/10); uncommon ( 1/1,000 to <1/100); rare ( 1/10,000 to <1/1,000); very rare (<1/10,000); and not known (frequency cannot be estimated from the available data).

Immune system disorders:

Not known: hypersensitivity reactions, e.g. anaphylaxis.

Nervous system disorders:

Common: headache, occasional drowsiness

Cardiac disorders

Not known: palpitations

Respiratory disorders

Not known: Bronchospasms may occur in patients with bronchial asthma (see section 4.4)

Gastrointestinal disorders:

Common: dyspepsia *, nausea 

Skin and subcutaneous tissue disorders

Not known: cutaneous and subcutaneous hypersensitivity reactions, in particular angioneurotic oedema, urticarial, rash, and pruritus 

*Mild gastric complaints (e.g. vomiting, gastrointestinal pain, abdominal distension and bloating) have been observed. These can normally be dealt with by taking the dose during meals or by lowering the dose.”

A few overdose cases have been reported. Some patients experienced mild to moderate symptoms with doses up to 640 mg (e.g. nausea, somnolence, abdominal pain).Other symptoms of betahistine overdose are vomiting, dyspepsia, ataxia and seizures. More serious complications (convulsion, pulmonary or cardiac complications) were observed in cases of intentional overdose of betahistine especially in combination with other overdosed drugs. No specific antidote. Gastric lavage and symptomatic treatment are recommended within one hour after intake.

 

My own preference

My own preference is the use of betahistine over intratympanic steroids.

 

Other issues

If you have Ménière’s disease you may need to report yourself to your country’s driving regulator.

The use of a Meniett device, diuretics, salt free diet and  operations are all ineffective.

*vertigo is the sensation of spinning or the world moving: I am spinning” the room is spinning” or “I feel like I’m falling backward” “the room is rocking like on a boat”

**non-spinning imbalance: “Unsteady on walking”, “Unsteady on feet”, “cannot feel the ground properly” “Like being drunk”

If you are experiencing symptoms which could be linked with Meniere’s Disease, please contact me to arrange a consultation.

Harley Street Dizzy Doctor are a certified Dizziness and Meniere’s specialist in London. 

 

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