INTRODUCTION
Ménière’s disease is an idiopathic disorder of the inner ear causing the debilitating vestibular and auditory symptoms of vertigo, hearing loss, tinnitus, and aural fullness; vertigo attacks are the most problematic facet of the disease for sufferers.1 Diagnostic certainty is problematic given the absence of a pathognomonic test, making it a challenging and often incorrectly attributed diagnosis in both primary and secondary care.2 The lifetime prevalence has been measured as 34–190 cases per 100 000, tending to affect adults between the third and seventh decades of life3 with paediatric cases being rare. The effect on patients’ daily living can be significant. Eighty-six per cent of patients found that their job performance suffered as a result of their Ménière’s and 70% had to modify their work in some way to accommodate this.1
PATHOPHYSIOLOGY
While the exact pathophysiology remains unknown, there is believed to be a relationship to endolymphatic hydrops, where the potassium-rich extracellular fluid present within the inner ear is present at higher pressures than normal. This was shown in postmortem examinations of all Ménière’s sufferers.4 However, not all patients with endolymphatic hydrops exhibit signs and symptoms of Ménière’s disease. It has therefore been purported that there may be one or more other co-factors:4 autoimmune, viral, allergic, and vascular causes have all been suggested.2
CLINICAL ASSESSMENT
The first point of contact for patients presenting with Ménière’s disease is usually general practice. Patients may present during an acute attack or a quiescent period. They may also attend primary care with a pre-existing diagnosis for help with symptom control.
Like all patients presenting with dizziness, a thorough history is vital. Eliciting the time course and relationship of symptoms to each other is especially important. Vertigo attacks tend to last between 20 minutes and 12 hours, and are classically accompanied by a triad of aural symptoms (hearing loss, tinnitus, and aural fullness). It is typically unilateral and, although bilateral disease is reported, symptoms do not usually arise in both ears simultaneously.
Hearing loss is initially episodic but may progress to a persistent loss, classically a sensorineural loss in low frequencies.
Clinicians should elicit red flags that are of concern for central pathology (Box 1) that require medical consultation. In patients with acute vertigo, ruling out a cerebrovascular event is vital. Acute attacks of Ménière’s are, by definition, transient and self-limiting, with reassurance and bed rest sufficient treatment. Prochlorperazine can be considered for short-term symptomatic relief of nausea but should not be continued in the long term in management of vestibular problems because of systemic side effects and vestibular sedation.
New unilateral hearing loss. Focal neurological signs (facial weakness, diplopia, limb weakness). New-onset headache. Normal head thrust test.
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Box 1. Red flags in acute vertigo5
In 2015, diagnostic criteria for Ménière’s disease were redefined, with qualifications for definite and probable disease (Table 1). It should be noted that the key differences between the two are duration of vertigo and presence of aural symptoms.3
Table 1. Diagnostic criteria in Ménière’s disease3
There is considerable overlap of symptoms with vestibular migraine, with migraine also being a feature in 30% of Ménière’s sufferers. The key differences in terms of diagnosis are that the time course for diagnosing vestibular migraine is less strict (spells lasting 5 minutes to 72 hours) and there should be a history suggestive of migraine.2 In patients with vestibular migraine, symptoms range from unsteadiness or mild feeling of disorientation to true rotatory vertigo with (but often without) any headache symptoms.
Audiological testing can be useful in monitoring disease progression and guiding audiological rehabilitation in those with hearing loss and intrusive tinnitus.
As Ménière’s disease is a diagnosis of exclusion, patients are typically investigated with an MRI to rule out cerebellopontine angle or inner-ear pathology.2
Initial management can be commenced in primary care and is detailed below, but the majority of patients benefit from referral to otolaryngology services to confirm diagnosis and manage relapsing or refractory symptoms.
MANAGEMENT IN PRIMARY CARE
Lifestyle measures
Initial management is with dietary alterations. This is a long-established practice, although the evidence base is limited. Sodium restriction is proposed to help by reducing endolymphatic pressure and is defined as <2000 mg/day. Dietary restrictions to sodium have been shown to be efficacious in reducing the frequency and severity of vertigo attacks, and caffeine restriction is likely also beneficial.6 Increasing oral water intake (35 mL/kg/day) has also been shown to have some benefit, particularly in terms of vertigo and hearing.7 Dietary modifications can often be difficult for patients to manage alone, though input from a dietician improves outcomes and compliance.6 Because of the overlapping clinical symptoms between vestibular migraine and Ménière’s, some patients benefit from further lifestyle modifications such as healthy sleep patterns and reducing migraine precipitants such as dark chocolate and alcohol.
Betahistine
Betahistine has long been used for Ménière’s. Precisely why the histamine analogue helps is unknown, though it may improve labyrinthine circulation. A Cochrane review suggests benefit from betahistine in reducing vertigo and is generally well tolerated.8
Diuretics
Thiazides are frequently used as medical management of endolymphatic hydrops, with the same rationale as the above dietary modifications. A Cochrane review found no studies of sufficient quality to meet the inclusion criteria.9 Given the potential side effects of electrolyte disturbance and renal dysfunction, it is therefore not usually commenced in primary care.
Driving
The vertigo caused by Ménière’s disease can cause disruption to driving, and patients are therefore obliged to notify the Driver and Vehicle Licensing Agency (DVLA). Should a patient neglect to inform the DVLA, and the clinician responsible for their care has reason to believe they are still driving, then it is the clinician’s responsibility to report this.
Further management
In the ENT clinic, patients may undergo further management for their symptoms. This may include injection of steroid or gentamicin into the middle ear. In the most debilitating of cases, surgical options such as endolymphatic sac surgery or selective vestibular nerve section may be considered.
CONCLUSION
In summary, Ménière’s disease can be a source of considerable anguish for patients and its identification can prove problematic. However, first-line treatments such as dietary modifications and betahistine are safe measures that can provide clinical benefit when provided early in primary care before referral to secondary care.
Notes
Provenance
Freely submitted; externally peer reviewed.
Competing interests
The authors have declared no competing interests.
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- Received August 7, 2021.
- Revision requested September 14, 2021.
- Accepted October 15, 2021.
- © British Journal of General Practice 2021
