Ménière's disease

Notes

Introduction

Ménière's disease is a disorder of the inner ear that manifests itself with attacks of vertigo, tinnitus and hearing loss.

The majority of cases of Ménière’s are idiopathic but the condition is thought to be related to abnormal production and absorption of endolymph. Where an underlying cause is identified it is referred to as Ménière’s syndrome.

The disease is characterised by episodes of vertigo, tinnitus and fluctuating hearing loss. As the condition develops symptoms, in particular tinnitus and hearing loss may become permanent and progressively worse.

Management involves supportive care during acute attacks and treatment to reduce the frequency and severity of episodes.

Epidemiology

The incidence of Ménière’s disease is estimated to be 13.1 per 100,000 person years in the UK.

It is thought to affect more women than men and is most commonly diagnosed in those aged 30-60.

Figures from NICE CKS.

Pathogenesis

Ménière's disease is classically characterised by the finding of endolymphatic hydrops.

Endolymphatic hydrops is a disorder of the vestibular system that is thought to result from abnormal fluctuations in endolymph (the fluid that fills portions of the labyrinthine system). The term describes distention and distortion of the membranous endolymph system.

The exact pathophysiology is not understood and it should be noted that people may have endolymphatic hydrops without symptoms of Ménière's disease.

There are a number of risk factors that are associated with an increased risk of Ménière's disease. These include:

  • Genetic susceptibility
  • Autoimmune diseases
  • Viral infections
  • Migraines
  • Trauma
  • Syphilis

Clinical features

Ménière's disease is characterised by episodes of vertigo, tinnitus and fluctuating hearing loss.

Attacks are frequently accompanied by nausea and vomiting resulting from the vertigo. Patients often complain of a feeling of aural pressure or fullness. Tinnitus and hearing loss may precede and herald the onset of vertigo.

These episodes vary in their length but are often present for minutes or hours. Attacks may occur in clusters (several over a few weeks) with periods of remission in between.

As the disease progresses tinnitus may become permanent and significantly affect quality of life and mental wellbeing. Hearing loss can become permanent and progressive.

Less common features include ‘Otholitic crises of Tumarkin’ - drop attacks that occur without loss of consciousness.

Investigations

Investigations are used to identify features supportive of a diagnosis of Ménière's disease and to exclude alternative pathologies.

Bloods

  • FBC
  • U&E
  • Thyroid function
  • Lipid profile
  • Syphilis screen

Audiogram

Audiograms aid the diagnosis of Ménière's disease. Transient sensorineural unilateral low-frequency hearing loss is typical. It may however be difficult to arrange in the window of an acute attack.

Imaging

MRI is used to exclude alternative diagnoses like vestibular schwannoma. With modern, high-resolution MRI scanners, endolymphatic hydrops may be visible. Changes visible on MRI (and indeed high-resolution CT) are still being characterised and are not currently used for diagnosis.

Specialist tests

  • Bithermal caloric test: a complex test completed in stages using warm and cold water placed in the ear canal and monitoring eye movements.
  • Electrocochleography: allows for the direct measure of evoked potentials of the inner ear.
  • Vestibular-evoked myogenic potential: utilises sound stimulus and measures the cervical and ocular muscle potentials created in response.

Diagnosis

Ménière's disease is a clinical diagnosis based upon the presence of key clinical features and the exclusion of alternative causes.

NICE CKS (2017 revision) advise that the diagnosis be based on the presence of all of the following features:

  • Vertigo: at least two spontaneous episodes lasting 20 minutes to 12 hours.
  • Fluctuating hearing, tinnitus, and/or perception of aural fullness in the affected ear.
  • Hearing loss confirmed by audiometry to be sensorineural, low-to-mid frequency, and defining the affected ear on one or more occasions before, during, or after an episode of vertigo.
  • Not better accounted for by an alternative vestibular diagnosis.

These criteria are taken from the Equilibrium Committee amendment to the American Academy of Otolaryngology - Head and Neck Surgery guidelines for the definition of Ménière's disease.

The diagnosis of Ménière’s disease is often challenging. Referral to ENT should be offered to all patients with suspected Ménière's disease to allow for a formal diagnosis to be made.

Management

Management involves symptomatic relief during acute attacks and measures to reduce the frequency and severity of further attacks.

Acute attacks

Vertigo, nausea and vomiting may be treated with phenothiazines (prochlorperazine) used as vestibular suppressants or an antihistamine like cinnarizine, cyclizine, or promethazine.

Buccal, IM or IV medication may be used in acute attacks where vomiting/nausea prevents effective oral administration. Rarely severe attacks may require admission for labyrinthine sedatives and supportive measures (e.g. IVF).

Prevention

Lifestyle measures: possible triggers should be avoided, these include high salt diets, caffeine, excessive fatigue, stress, allergies, chocolate and alcohol.

Medical prophylaxis: betahistine may be given (as a trial initially) to reduce the frequency and severity of attacks. The evidence for their use is relatively weak. Diuretics have been used but the evidence of beneficial effect is scant.

Additional measures

Medical ablation: aminoglycosides have been used for a number of decades to treat Ménière's disease. Taking advantage of its ototoxicity and administered via the intratympanic route it causes damage to the vestibular system preventing vertigo. It has been shown to reduce or stop vertigo in many patients but does risk causing or worsening hearing loss.

Surgery: surgical intervention is relatively rare and used as a last resort. Labyrinthectomy is used in unilateral cases and involves the opening of the semicircular canals and vestibules with excision of the neurosensory epithelia. It results in deafness in the affected ear but prevents vestibular input with the hope of eliminating vertigo. Vestibular nerve section requires intracranial surgery where the vestibular nerve is identified and cut leaving the cochlear nerve unaffected.

Driving

The Driver and Vehicle Licensing Agency (DVLA) advise all people with a 'liability to sudden and unprovoked or unprecipitated episodes of disabling dizziness' to stop driving and inform them. They will review each case individually.

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