Ménière’s Disease

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

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Ménière’s disease (pronounced “many-ears”) is 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 affected ear.

The classical “sequential” presentation occurs in about a 1/3rd of patients.

The attack starts with a “fullness” sensation in the ear with a low frequency buzzing followed by hearing loss and then vertigo with nausea, vomiting and sweating. Gradually the hearing recovers. The patient often has a period of imbalance lasting for days.

A measure of severity is to record how many of the eepisodes were associated with vomiting and those without vomiting

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 can 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 such that 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.
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
iv) Delayed Meniere’s where it starts with hearing loss with a later development of Meniere’s symptoms.
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. Meniere’s and migraine is frequently an overlapping diagnosis.
Salt
Labyrinth is a sodium osmolality change detecting organ so any spikes in salt intake and plasma concentration are detected ( not the absolute level) by the membranous labyrinth hense patients who eat a salty meal may get a return of their symptoms.

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 (256Hz). In addition, patients may exhibit a positive “Henneberts Sign” where pressure on the ear canal induces eye movement.

It is worth considering four-hour delayed contrast MRI to predict contralateral Meniere’s before clinical signs become apparent.
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 30dB at 250Hz/500Hz/1KHz.

High tone loss does occur and should not be dismissed

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.
See – Advice and Medication for Acute Vertigo and Nausea for all conditions other than BPPV

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?

Treatment should be 3 times as long as typical interval between attacks to see an effect.

Patients should be initially recommended

i) low salt diet,
ii) dietary avoidance
iii) Dyazide® which is co-triamterzide (triamterene 37.5mg with hydrochlorothiazide 25mg) o.d. Triamterene is a potassium-sparing diuretic often used in combination with a thiazide diuretic e.g.hydrochlorothiazide ( See Risks of medication below)

If more control is needed add:

Betahistine
This has been popular for many years and is more conventional* and is my next preference ( see below – Intratympanic injections of steroids).

*The medical evidence which supports its use https://www.ncbi.nlm.nih.gov/pubmed/29282702

The recommended dose :
Initially start on 8mg orally three times daily increasing to 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

Intratympanic injections of steroids
This is a relatively new treatment which I do not offer and is somewhat controversial.

It 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.

Status Hydrops
Prednisolone 40 mg EC for 5 days reducing (with PPI and no contra-indications and side-effects)

Prescribing issues
Dyazide®: There is a risk of increased skin cancer* with dyazide is related to the length of drug use, amount of sun exposure and especially a risk in susceptible populations (i.e. fair-haired, blue-eyed Scandinavians). Essentially the evidence suggests a significant increased risk of non-melanoma skin cance if the medication has been used for 6 years+. I don’t think this warrants discontinuation of the drug for Meniere’s disease if it seems to be working but I would advise patients to use adequate sunscreen when exposed.
*Hydrochlorothiazide use and risk of nonmelanoma skin cancer: A nationwide case-control study from Denmark. J Am Acad Dermatol 2018;78:673-81. and https://www.gov.uk/drug-safety-update/hydrochlorothiazide-risk-of-non-melanoma-skin-cancer-particularly-in-long-term-use

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.

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”

 

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