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The information - Ménière’s disease

Consultant ENT surgeon Mr Nitesh Patel continues our series of evidence-based lowdowns on common presentations using PUNs and DENs

 

The patient’s unmet needs (PUN)

You are called out to see a 50-year-old woman with a severe attack of vertigo. You see from her notes that she has had previous episodes – which have been treated symptomatically - and that she has also described tinnitus and deafness. Examination is unremarkable, and you prescribe prochlorperazine. The patient is concerned when you explain you suspect Ménière’s disease. ‘My Mum had it and suffered terribly,’ she says. ‘What causes it? Will it get worse? And what can I do about it?’

The Doctor’s educational needs (DENs)

What causes Ménière’s disease?

In Ménière’s disease there is an overproduction or malabsorption of endolymph in the inner ear resulting in dilatation of the endolymphatic compartment, known as endolymphatic hydrops. Ménière’s attacks are thought to occur when the endolymphatic pressure becomes high enough to cause microtears in the membranous labyrinth, causing mixing of the potassium-rich endolymph with perilymph. The potassium-rich fluid bathes the basal surfaces of the inner ear hair cells causing sensory excitation. But, recent evidence suggests that this traditional model may not be the only cause of symptoms and Meniere’s disease may be the endpoint of a variety of causes including ischaemic and autoimmune injuries.

What is the main differential for Ménière’s disease, and how can GPs make a confident diagnosis?

When making a diagnosis, Ménière’s syndrome must be distinguished from Ménière’s disease.

Where there is an identifiable cause for the endolymphatic hydrops the term Ménière’s syndrome is applied. Secondary causes include mumps, syphilis, electrolyte imbalance, thyroid dysfunction, diabetes, dyslipidaemia, acoustic neuroma, ear surgery, trauma or an autoimmune disorder such as Cogan’s syndrome.

Ménière’s disease is idiopathic and a diagnosis of exclusion.  Known causes of endolymphatic hydrops must be excluded with appropriate tests. Investigations should include FBC, U&E, ESR, glucose, lipid profile, anti-neutrophil cytoplasmic antibodies, autoantibody screen, TFTs, syphilis serology and viral titres.

MRI scan enables exclusion of an acoustic neuroma.

A hearing test will often reveal a low frequency sensorineural hearing loss.

A diagnosis of ‘definite’ Ménière’s disease can be made if the patient has had two or more episodes of spontaneous vertigo lasting more than 20 minutes, documented hearing loss on one occasion, tinnitus or aural fullness on affected side, and exclusion of other causes.

What is the natural history of Ménière’s disease?

Attacks may occur days, months or years apart. Some patients have only a few attacks with little or no permanent hearing loss or imbalance, whereas others can progress with continuing episodic vertigo, no useful hearing and intrusive tinnitus in the affected ear.

Do all patients with likely Ménière’s require referral – if so, why?

Referral is advised for specialist investigations to exclude other causes of Ménière’s syndrome which may require specific treatment. Patients with known Ménière’s disease may need referral if symptoms are uncontrolled.

What treatment options are there?

During an acute episode a vestibular sedative - such as prochlorperazine, cinnarizine or cyclizine - is effective to reduce the nausea, vomiting and vertigo. But vestibular sedatives must not be continued after the episode has settled because they hinder balance adjustments made in the brain (central compensation).

Counselling or CBT may be necessary where there is a significant emotional impact.

Patients with significant hearing loss may need to have a hearing aid fitted. Troublesome tinnitus may require referral to an audiologist for tinnitus management. Vestibular rehabilitation exercises can help patients with imbalance that persists after episodes have settled, although are not always as effective in the presence of continuing Ménière’s attacks.

For prevention of Ménière’s attacks a stepwise treatment regime can be adopted depending on symptom control, starting with the least invasive and progressing to more invasive treatment modalities as necessary:

  1. Dietary modification – salt restriction (less than 2g per day), caffeine and alcohol avoidance.
  2. Betahistine 16mg tds – works as a labyrinthine vasodilator improving inner ear circulation.
  3. Diuretic – bendroflumethiazide 2.5mg daily aims to improve inner ear electrolyte imbalance. Note that a Cochrane review found that there is a lack of good evidence to support or refute this treatment modality. But, because there is no good evidence to refute its use it is still widely used for Meniere’s disease in the UK.
  4. Steroids – given either orally (prednisolone 1mg/kg daily up to a maximum of 60mg for one week) or intratympanic injection of steroid.  The mechanism by which this works is still unknown. In clinical practice, loading the inner ear with steroid has a beneficial effect in reducing the fragility of a system which is otherwise prone to wider variations in endolymphatic pressure. The dosing, frequency, and type of steroid is still widely debated and there is no current acceptable standard amongst otologists. Some 50% of the steroid delivered by injection intratympanically is cleared within 20-25 minutes, but a single injection can have a significant impact on improving symptoms. Relapse can occur many months or years later and a ‘top-up’ injection may be required.
  5. Grommet insertion – may help buffer changes in middle ear pressure which has an impact on inner ear pressure.
  6. The Meniett is a portable device which introduces low pressure pulses into the inner ear through the ear canal via a grommet. Three treatments daily for five minutes are usually required.
  7. Intratympanic gentamicin – gentamicin injected into the middle ear for inner ear absorption is highly effective in more than 90% cases but carries the risk of sensorineural hearing loss. Current regimes involve titrated doses to reduce this risk.

More invasive surgical procedures including endolymphatic sac decompression, vestibular nerve section or labyrinthectomy are rarely performed these days because intratympanic steroids or gentamicin give good results.

 Key points

 Causes

  • Ménière’s disease is an inner ear disorder of unknown cause

Epidemiology

  • The incidence is approximately 50-150 cases per 100,000 people per year.
  • It is more common in women and tends to present in the fourth or fifth decade of life.
  • Some 30-40% of patients develop bilateral disease

Clinical features

  • Ménière’s attacks are characterised by episodes of vertigo, fluctuating sensorineural hearing loss, tinnitus and aural fullness.
  • Episodes usually last hours and are associated with nausea and vomiting.
  • Hearing and balance can improve after an attack, but with repeated episodes and disease progression hearing loss and imbalance may persist between episodes.

Main differential diagnoses

  • For a single spontaneous attack of vertigo without deafness vestibular neuritis should be considered, whereas with deafness and tinnitus labyrinthitis should be considered.
  • Vestibular migraine should be considered in patients presenting with recurrent spontaneous vertigo, a past or present history of headaches, family history of migraine and with no significant hearing loss.
  • Autoimmune inner ear disease can have similar presentation and some authorities believe Ménière’s disease has an autoimmune basis.

Treatment

  • Most patients can be successfully managed with a combination of salt restriction and betahistine.
  • Some will require a diuretic or grommet insertion with intratympanic steroid or gentamicin for symptomatic control.
  • Hearing aids, tinnitus management, vestibular rehabilitation exercises and cognitive behavioural therapy may be necessary.

Prognosis

  • The disease tends to follow a relapsing and remitting course.
  • Some patients have few attacks and no permanent sequelae. Others are afflicted by progressive disease with frequent Ménière’s attacks and debilitating vestibular insufficiency, tinnitus and hearing loss

 

Mr Nitesh Patel is a consultant ENT surgeon at Barts Health NHS Trust, Whipps Cross University hospital. Mr Patel is a member of the Meniere’s Society.

The Meniere’s Society is a registered charity dedicated to providing support and information to those affected by Meniere’s disease and other vestibular disorders causing dizziness and balance problems. The Meniere’s Society is also a source of information for health professionals and funds vital research into all aspects of vestibular disorders. Visit www.menieres.org.uk for more information.

 

Further reading

  1. Berlinger NT. Ménière’s disease: new concepts, new treatments. Minn Med 2011; 94(11):33-6
  2. Semaan MT and Megerian CA. Ménière’s disease: a challenging and relentless disorder. Otolaryngol Clin North Am 2011: 44(2):383-403
  3. Syed I and Aldren C. Ménière’s disease: an evidence based approach to assessment and management. Int J Clin Pract 2012; 66(2):166-70

 

 

Readers' comments (3)

  • I stilll have balance episodes but neither the ENT specialist or an MRI scan seem to have determined what my problem is and what course of action needs to be followed. I was told to revisit my GP for referral again should the problem persist but I feel that they don't have a clue so I put up with it! any advice out there please? My problem seems to follow a cold or nasal disturbances or even a flight and occurs between 2 and 3 times a year for between days and weeks at any one time.

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  • Dear anonymous,
    There are so many causes of imbalance. The brain acts as a computer and receives signals from your sensory systems - predominently your eyes (visual), inner ears (vestibular) and proprioceptors (locomotor system - joints and muscles). Any disturbance to any of these systems, the connections to the brain, or the brain itself can give imbalance. Also the nutrition of these systems can be affected by systemic disorders such as diabetes, thyroid dysfunction, heart or heart rhythm problems and so on. So imbalance is not always easy to diagnose. It requires a very careful history and sometimes specialist investigations. In some cases the cause is multifactorial, or from a combination of dysfunction from many sensory inputs to balance.
    You will benefit from a thorough assessment by a specialist.

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  • We have also been using Arlevert (cinnarizine/dymenhydrinate) in Menieres and seeing good results.

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