Section 1: Epidemiology and aetiology
Ménière's disease is a disorder of the inner ear that is linked to endolymphatic hydrops, an excess of fluid in the inner ear. The condition affects people differently; it can range in intensity from being a mild annoyance to a chronic, lifelong disability.
Endolymphatic hydrops refers to a condition of increased hydraulic pressure within the inner ear endolymphatic system. Excess pressure accumulation in the endolymph can cause the following symptoms: fluctuating hearing loss, occasional episodic vertigo, tinnitus and aural fullness.
The term endolymphatic hydrops is often used synonymously with Ménière's disease and Ménière's syndrome, both of which are believed to result from increased pressure within the endolymphatic system.
However, Ménière's disease is idiopathic by definition, whereas Ménière's syndrome can occur secondary to various processes interfering with normal production or resorption of endolymph (for example, endocrine abnormalities, trauma, medications, parasitic infections and hyperlipidaemia).
Other possible conditions that may lead to similar symptoms include syphilis, Cogan's syndrome, autoimmune disease of the inner ear, dysautonomia, perilymph fistula, MS, acoustic neuroma, and both hypo and hyperthyroidism.
Pathophysiology
The exact pathophysiology of Ménière's disease is controversial. Ménière's disease is idiopathic, but it is believed to be linked to endolymphatic hydrops.
The inner ear consists of the membranous labyrinth and the bony labyrinth. The membranous labyrinth, a system of membranes in the ear, contains a fluid called endolymph.
Endolymph is formed at the stria vascularis within the cochlear duct. The membranes of the labyrinth can become dilated like a balloon when pressure increases and drainage is blocked.
It is thought that endolymphatic fluid bursts from its normal channels in the ear and flows into other areas, causing damage. This is called 'hydrops'. This may be related to swelling of the endolymphatic sac or other tissues in the vestibular system of the inner ear, which is responsible for the body's sense of balance.
In some cases, the endolymphatic duct may be obstructed by scar tissue or may be narrow from birth. In other cases there may be too much fluid secreted by the stria vascularis. The symptoms may occur in the presence of a middle ear infection, head trauma or URTI, or by using aspirin, smoking or drinking alcohol. They may be further exacerbated by excessive consumption of salt in some patients.
It has also been proposed that Ménière's symptoms in many patients are caused by the deleterious effects of a herpes virus. Herpes viridae are present in most of the population in a dormant state. It is suggested that the virus is reactivated when the immune system is depressed due to a stressor, such as trauma or infection.
Section 2: Making the diagnosis
Vertigo is a sensation of motion while motionless. Attacks of rotational vertigo can be incapacitating and unpredictable. It lasts from minutes to hours, but generally no longer than 24 hours.
For some patients, however, prolonged attacks can occur. This combines with an increase in volume of tinnitus and temporary hearing loss. Hearing may improve after an attack, but often becomes progressively worse. There may be fluctuation in the degree of hearing loss and low frequencies may be primarily affected.
In addition to hearing loss, sounds can appear distorted, and patients can experience hyperacusis. Tinnitus is often nonpulsatile and may be described as whistling or roaring. It may be continuous or intermittent. Nausea, vomiting and sweating sometimes occur.
Some patients experience 'drop attacks' - a sudden attack of vertigo that causes the patient to fall. There is no loss of consciousness and patients may also experience the feeling of being pushed or pulled.
Some patients find it impossible to get up until the attack passes or medication takes effect.
After an acute attack, patients generally feel tired and nauseous. The timing and frequency of attacks are variable. Some patients can predict an attack while others note a random pattern. Attacks may be linked to dietary triggers, the menstrual cycle or psychosocial stresses. Between episodes, some patients are usually asymptomatic.
Clinical examination varies depending on the phase of disease. A targeted neurological examination is necessary to differentiate Ménière's disease from other conditions. New-onset vertigo might be an early sign of stroke, migraine, or brainstem compression that may require urgent referral if other signs are present, such as limb weakness, dysarthria or reduced level of consciousness.
Romberg's test generally shows significant instability and worsening during acute attacks.
Patients with Ménière’s disease may be offered a hearing aid (Photograph: SPL)
Investigations
There are no specific tests for Ménière's disease. However, blood tests may exclude metabolic disturbances (renal function, glucose), infections (FBC, ESR, CRP), or hormonal imbalances (TFT).
CT and MRI are not required but may be used if a central cause of vertigo is suspected. Patients should be referred to ENT for an audiological assessment, such as the vestibular function test.
Typically, the lower frequencies are affected more severely. This is due to preferential sensitivity of the apex of the cochlea to the hydrops. Electronystagmography is a test of inner ear function, which determines inner ear responsiveness to movement and caloric stimulation. It tests central and peripheral function and can help localise the site of the lesion.
Diagnosis
Ménière's symptoms can begin at any age, but typically begin between 30 and 60 years. Hearing loss can affect both ears either simultaneously or separately. Classic symptoms include:
- Periodic episodes of rotary vertigo or dizziness.
- Fluctuating, progressive, unilateral or bilateral hearing loss.
- Tinnitus.
- A sensation of fullness or pressure in one or both ears.
Ménière's often begins with one symptom, and gradually progresses. However, not all symptoms must be present for a doctor to make a diagnosis.
Section 3: Managing the condition
A holistic approach should be taken in the management of Ménière's disease. This should include pharmacological treatment, vestibular rehabilitation and adjunctive therapy.
Pharmacological treatment aims to suppress the symptoms of acute vestibular attacks and to specifically treat the conditions that cause these vestibular symptoms (see table right).
Drugs used to suppress the symptoms of acute vestibular attacks (vestibular sedatives), include hyoscine, prochlor-perazine, promethazine, cyclizine, dimenhydrinate, metoclopramide, cinnarizine and diazepam.
In addition, dietary salt restriction has been shown to control vertigo in some studies.
Vestibular rehabilitation
The rationale for vestibular rehabilitation is based on the concept that the vestibular system has the capacity for adaptation and recalibration of vestibular reflexes by substitution of sensory input, motor responses and strategies in order to achieve symptomatic recovery.
Vestibular rehabilitation may include Cawthorne-Cooksey exercises (see online resources, at the bottom of the page) and low-impact exercise, such as cycling or walking.
Adjunctive treatment may include psychological or psychiatric intervention, for example cognitive behavioural therapy, and techniques such as relaxation and breathing exercises.
Vision or proprioception issues, including environmental modifications and safety measures, may require assessment.
Patients who do not respond to these conservative treatments will require referral to an ENT specialist for further investigation and management. This may include destructive treatment of the vestibular system such as chemical labyrinthectomy by the use of local amino- glycosides (such as gentamicin), endolymphatic sac surgery, vestibular nerve sectioning and labyrinthectomy.
Safety advice
Patients with a diagnosis of Ménière's disease should cease driving, especially during acute attacks. The patient should inform the DVLA. Driving will be permitted when satisfactory control of symptoms is achieved. If the patient remains asymptomatic, the licence will be restored.
Patients may be reminded that if they ignore medical advice to cease driving, there may be consequences with respect to their insurance. Doctors are advised to document formally and clearly in the notes the advice that has been given.
| COMMON DRUGS FOR Ménière'S DISEASE | |||
|---|---|---|---|
| Drug | Dose | Side-effects | Contraindications/precautions |
| Bendroflumethiazide | 2.5mg daily | Hypokalaemia |
Hypersensitivity to thiazides
|
| Betahistine |
16mg three times
|
GI upset, headache, skin rash, pruritus |
Phaeochromocytoma, hypersensitivity
|
Section 4: Prognosis
The pattern of exacerbation and remission of Ménière's disease makes the evaluation of treatment and prognosis difficult. As such the prognosis of patients with Ménière's disease is highly variable.
Acute attacks are normally replaced with constant imbalance problems and progressive hearing loss. The patient's condition tends to stabilise when the disease is regarded as 'burnt out'. However, many are left with poor hearing and balance.
Follow up
Patients should be reviewed to ensure control of acute symptoms and treatment compliance. Vestibular rehabilitation should be commenced after the acute attack. Vestibular sedatives should be reduced after resolution of an acute attack. Prolonged use of these medications may mitigate against vestibular rehabilitation.
Some patients will require referral for a hearing aid while others will benefit from tinnitus retraining therapy. Targeted physiotherapy may be a useful adjunct to vestibular rehabilitation exercises.
Ménière's disease is a distressing illness and can be regarded as a devastating condition which severely affects lifestyle and employment. Early referral to a psychologist is recommended in such cases.
Section 5: Case study
A 49-year-old woman with a main complaint of dizziness has had five attacks of vertigo over the past four months. The episodes last for one to two hours and are associated with nausea and vomiting.
She also complains of left aural fullness, hearing loss and tinnitus which worsens before the onset of vertigo.
No historical features
There is no previous history of ear surgery or family history of ear disease. She has no history of noise exposure, use of oto-toxic medication, or history of head trauma. She takes medication for hypertension and has allergic rhinitis.
Physical examination reveals Weber lateralising to the right and positive Rinne bilaterally. There is no evidence of effusion and fistula test is negative. The remainder of her head and neck, and neurological examination is normal.
She was seen by the ENT specialist who confirmed the diagnosis of Ménière's disease. Her pure tone audiogram revealed asymmetrical sensorineural hearing loss in the left.
She was commenced on vestibular rehabilitation exercises and offered a hearing aid.
There has been no further recurrence of dizziness and she was discharged from further follow up.
Section 6: Evidence base
Clinical trials
Several Cochrane reviews have been published on various treatments for Ménière's disease.
The drug betahistine hydrochloride has been used to reduce the frequency and severity of the attacks. While this drug is very acceptable to patients who use it, the review of clinical trials did not find enough evidence to show whether it is helpful.1
The authors of another systematic review on the use of diuretics carried out an extensive search but could not identify any RCTs of sufficient quality and concluded that there was no good evidence about the effect of diuretics on the symptoms of Ménière's disease.2
Conversely, chemical labyrinthectomy with gentamicin was regarded as an effective treatment for vertigo complaints in Ménière's disease, but carried a risk of increasing hearing loss. 3
Despite an extensive search the review authors only found two RCTs studying surgical interventions for Ménière's disease.4 The two trials included in the review on surgical treatments for Ménière's disease concluded that there was insufficient evidence of the beneficial effect of endolymphatic sac surgery.
Guidelines
There is no uniformly accepted evidence-based treatment protocol given the pathophysiology of Ménière's disease.
Other factors responsible for the lack of a uniform treatment protocol include the fluctuating nature of this disease, with spontaneous, potentially long-term remissions, the frequent but poorly understood overlap with conditions such as migraine, and the high placebo effect of treatment. As such, no guidelines on Ménière's disease are available from NICE or SIGN.
Curriculum
This topic is covered in the GP curriculum in statement 15.4: ENT and facial problems
More online
This summary helps patients understand Ménière's disease.
Surgical procedures for selected patients with Ménière's disease.
- Ménière's Society www.menieres.org.uk/
- Detailed description of Cawthorne-Cooksey vestibular rehabilitation exercises. www.entkent.com/Cawthorne-Cooksey.html
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References
1. James A, Burton MJ. Betahistine for Ménière's disease or syndrome. Cochrane Database Syst Rev 2001, Issue 1. Art No CD001873. DOI: 10.1002/14651858.CD001873.
2. Burgess A, Kundu S. Diuretics for Ménière's disease or syndrome. Cochrane Database Syst Rev 2006, Issue 3. Art No CD003599. DOI: 10.1002/14651858.CD003599.pub2.
3. Pullens B, van Benthem PP. Intratympanic gentamicin for Ménière's disease or syndrome. Cochrane Database Syst Rev 2011, Issue 3. Art No CD008234. DOI: 10.1002/14651858.CD008234.pub2.
4. Pullens B, Giard JL, Verschuur HP et al. Surgery for Ménière's disease. Cochrane Database Syst Rev 2010, Issue 1. Art No CD005395. DOI: 10.1002/14651858.CD005395.pub2.
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