Ménière's disease is characterized by an increased amount of endolympha in the internal ear. The aetiology is unknown.
The diagnosis is based on three symptoms:
tinnitus or feeling of pressure in the ear
recurrent rotational vertigo
cochlear-type impaired hearing, initially unilateral with emphasis on low (< 2 kHz) frequencies.
The diagnosis can often be made only after follow-up of several months. A consultation by an ENT specialist is indicated at this stage at the latest.
Other disorders of the vestibular apparatus are often labelled as Ménière's disease. The most important differential diagnostic alternatives include vestibular migraine, sudden deafness, infarction of the cerebellum/brain stem, vestibular neuronitis and vestibular schwannoma (acoustic neuroma).
Symptoms and signs
The disease onset is usually at the age of 20-60 years.
The attacks (lasting from 20 minutes to 12 hours) include rotational type vertigo, and they are associated with nausea and often vomiting. Nystagmus is often observed during the attack irrespective of the body position. The patient's level of consciousness during the attack remains normal and there are no paralytic symptoms.
The symptoms varyingly include hearing impairment, tinnitus or a feeling of pressure in the affected ear.
Between bouts, postural balance may be normal.
At the initial stage the unilateral hearing impairment may temporarily subside.
In some patients (15-50%) the hearing impairment later turns bilateral.
Levelling of hearing and unpleasant sensations caused by sounds (distortion of sounds, hyperacusis) are associated with the hearing impairment.
The examination of the eardrums shows normal findings.
A cochlear-type hearing impairment is detected on the audiogram, usually at low frequencies.
In unilateral cases, the average auditory threshold of frequencies 0.5, 1, 2, 3 kHz is at least 20 dB worse than in the other ear.
As the disease advances the impairment becomes permanent and affects all frequencies. The disease does not, however, make the ear completely deaf.
During an attack a nystagmus directed towards the unaffected ear can be observed. The caloric response of the affected ear is continuously diminished.
Unterberger's stepping test (30 seconds of stationary walking with the eyes closed) may reveal a pathological rotation of over 45° towards the side of the affected ear.
In 5-23% of patients with Ménière's disease, the disease is hereditary. Hereditary, i.e. familial Ménière's disease begins earlier than the non-hereditary disease form.
Differential diagnosis
Vestibular migraine
The patient usually has an already known migraine with or without aura.
Migraine-like clinical picture (pulsating, unilateral moderate or severe headache) at least in 50% of the attacks
The duration of vestibular symptoms is between 5 minutes and 72 hours.
Hearing impairment may be bilateral also at the early stage, but does not lead to a more severe hearing impairment over the years.
Sudden deafness
Unilateral deterioration of hearing usually develops within 24 hours.
The symptoms may include vertigo at the early stage.
Tinnitus is initially intensive.
In most cases hearing returns to normal and the disease does not usually recur.
Vestibular neuronitis
No hearing impairment
Horizontal nystagmus with slight rotation directed away from the affected ear
One or just a few severe attacks of vertigo with violent initial symptoms, but usually resolves within 4-8 weeks with no residual symptoms
Infarction of the cerebellum or brain stem
Vertigo may be of rotational type, but nausea is usually milder than in an attack of Ménière's disease.
Atypical nystagmus (often vertical)
The patient often has other concomitant neurological symptoms.
Not necessarily detected on CT scan at the primary stage
Vestibular schwannoma (acoustic neuroma)
Vertigo may be paroxysmal at first.
Unilateral hearing impairment that usually does not subside
Discrimination of words is particularly poor on the affected side.
Treatment
The initial treatment is conservative.
BetahistineBetahistine for Ménière's Disease and Vertigo, a diuretic or a combination of these has been used as basic pharmacotherapy. Betahistine is a safe and affordable drug and it is one of the most used drugs for the treatment of Ménierè's disease in Europe. It has not, however, been possible to prove that betahistine would be more effective than placebo in preventing vertigo attacks.
The starting dose of betahistine is 24 mg twice daily, and the maintenance dose after the condition has settled is 12-24 mg twice daily. Once the patient feels well, the treatment can be gradually tapered off.
Low-dose hydrochlorothiazide-amiloride is a suitable diuretic, if the patient is not particularly hypotensive. Due to the risk of hypokalaemia, the potassium levels should be followed up from time to time.
The patient should also have an antiemetic as rectal suppositories at home in reserve for an acute attack.
Patient information and counselling, avoidance of stress and a regular lifestyle are as important as drug treatment. The patients need a personal doctor.
Reduced salt intake can be recommended, although firm scientific evidence of its effect is lacking.
Patient organisations can provide counselling and help in coping with the disease.
In severe cases with impaired hearing and incapacitating attacks the treatment options include low-pressure pulse treatment (Meniett® device), intratympanic gentamicin injections Intratympanic Gentamicin for Ménière's Disease, or transsection of the vestibular nerve. Another surgical option is decompression of the endolymphatic sac but the evidence on the benefits of the operation is scarce Surgery for Ménière's Disease.
Driving licence
As the attacks are nearly alway preceded by warning symptoms the disease does not prevent driving. Drugs used for the treatment of nausea (but not those for circulation) may impair driving performance.
Working capacity
Ménière's disease may cause temporary need of sick leaves when in active symptomatic phase.
Most patients are able to continue their working life despite the disease. This can be promoted if the job description can be adapted to suit the patient's symptoms.
In severe disease forms, permanent pension arrangements due to disability may be required.
Rehabilitation and adjustment training courses are arranged by governmental and non-governmental organizations.
References
Adrion C, Fischer CS, Wagner J et al. Efficacy and safety of betahistine treatment in patients with Meniere's disease: primary results of a long term, multicentre, double blind, randomised, placebo controlled, dose defining trial (BEMED trial). BMJ 2016;352():h6816. [PubMed]
Lopez-Escamez JA, Carey J, Chung WH et al. Diagnostic criteria for Menière's disease. J Vestib Res 2015;25(1):1-7. [PubMed]
Ghavami Y, Mahboubi H, Yau AY et al. Migraine features in patients with Meniere's disease. Laryngoscope 2016;126(1):163-8. [PubMed]