Meniere's Disease: Diagnosis and Management

Introduction

Meniere's disease is a chronic disorder of the inner ear causing recurrent attacks of vertigo alongside fluctuating auditory symptoms. It typically presents between 40 and 60 years of age and is unilateral at onset, though a significant proportion of patients go on to develop bilateral disease over time.¹

The condition is defined by its classic tetrad of episodic vertigo, fluctuating sensorineural hearing loss, tinnitus, and a sensation of aural fullness or pressure. Recognising the pattern is important both to guide treatment and to reassure patients about a condition that, while not curable, is very rarely dangerous.

Aetiology and pathophysiology

The underlying pathological finding is endolymphatic hydrops: an excess accumulation of endolymph within the membranous labyrinth, causing it to distend. This is thought to result from impaired resorption of endolymph by the endolymphatic sac, though the precise trigger for most cases remains unclear.^1,2

The great majority of cases are idiopathic (primary) Meniere's disease. A minority are secondary, following head trauma, autoimmune inner ear disease, or (rarely) otosyphilis, and there is a recognised association with migraine. As pressure within the endolymphatic system builds and periodically ruptures the membrane separating endolymph from perilymph, the resulting mixing of fluids with different ionic compositions is thought to transiently disable hair cell function, producing the acute attack of vertigo and hearing loss.²

Meniere's disease affects roughly 1 in 1,000 people, most often presenting between 40 and 60 years, with no strong sex predominance. Onset before adolescence or after 70 years is unusual and should prompt consideration of an alternative diagnosis.

Risk factors

• Age 40-60 years at onset
• Family history of Meniere's disease
• History of migraine
• Autoimmune disease
• Previous head or ear trauma
• History of allergic conditions (proposed but weaker association)

Clinical features

Attacks consist of vertigo lasting 20 minutes to several hours (up to around 12 hours), accompanied by nausea and vomiting, alongside fluctuating hearing loss, tinnitus, and a sensation of fullness or pressure in the affected ear.¹ Patients often describe the fullness or a change in tinnitus as a warning sign that an attack is about to begin.

Attacks tend to occur in clusters, with periods of remission lasting weeks to months in between, during which the patient may feel entirely well, though as the disease progresses some residual imbalance and hearing loss can persist between attacks. A rare but distinctive feature is the Tumarkin otolithic crisis ("drop attack") - a sudden fall to the ground caused by an abrupt otolithic dysfunction, without loss of consciousness.

Examination and diagnostic criteria

Between attacks, examination is often entirely normal. During an acute episode, there may be spontaneous horizontal or horizontal-torsional nystagmus and an unsteady gait. Otoscopy is normal, since the pathology lies within the inner ear.¹

Diagnosis is clinical, based on internationally agreed criteria (Barany Society/AAO-HNS): 2 or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours; audiometrically documented sensorineural hearing loss in the low-to-mid frequencies on at least one occasion; fluctuating aural symptoms (hearing, tinnitus or fullness) in the affected ear; and the presentation not better explained by another vestibular diagnosis.¹

Differential diagnosis

• Vestibular schwannoma: progressive, non-fluctuating asymmetric hearing loss and tinnitus rather than episodic attacks - needs MRI if suspected
• BPPV: much shorter, position-triggered episodes without hearing loss
• Vestibular migraine: vertigo with headache, photophobia or phonophobia, often without progressive hearing loss
• Vestibular neuritis/labyrinthitis: a single prolonged episode rather than recurrent attacks
• Autoimmune inner ear disease: rapidly progressive bilateral sensorineural hearing loss, sometimes with systemic autoimmune features

Investigations

Pure tone audiometry performed during or shortly after an attack classically shows a low-frequency sensorineural hearing loss, which fluctuates in the early stages but tends to become fixed and extend to all frequencies as the disease progresses.¹

MRI of the internal auditory meatus should be arranged in atypical presentations, particularly asymmetric or progressive hearing loss without the fluctuating pattern, to exclude a vestibular schwannoma. Caloric testing may show reduced vestibular response on the affected side but is not required to make the diagnosis, which remains primarily clinical.

Additional tests are occasionally used in specialist settings but are not required for routine diagnosis: electrocochleography can demonstrate an elevated summating potential to action potential ratio, reflecting raised endolymphatic pressure, and vestibular evoked myogenic potentials (VEMPs) assess otolith organ function. Blood tests (autoimmune screen, treponemal serology, thyroid function) are considered when secondary causes are suspected, particularly in bilateral or atypical disease.

Management

Acute attacks

Vestibular sedatives such as prochlorperazine or cyclizine (oral, buccal or intramuscular depending on severity) control nausea and vertigo during an acute attack, alongside general supportive measures.¹

Prophylaxis between attacks

Lifestyle measures - a low-salt diet, and reducing caffeine and alcohol intake - are commonly recommended to reduce fluid retention and attack frequency, although the evidence base is limited. Betahistine is widely prescribed to reduce the frequency and severity of attacks, thought to act by improving inner ear blood flow, though trial evidence for its effectiveness is mixed.³

For patients with frequent or disabling attacks despite these measures, options include intratympanic corticosteroid injection (aiming to reduce inflammation with lower risk to hearing) or intratympanic gentamicin (an ablative treatment that reduces vestibular function on the affected side to control vertigo, at the cost of a risk of further hearing loss).¹ Surgical options - endolymphatic sac decompression, vestibular nerve section, or labyrinthectomy - are reserved for severe, refractory disease, with the more destructive procedures used when useful hearing has already been lost.

Complications

• Progressive, eventually permanent sensorineural hearing loss
• Bilateral disease, developing in a significant minority of patients over time
• Falls and injury, particularly from Tumarkin drop attacks
• Chronic imbalance between attacks as the disease progresses
• Significant impact on employment, driving and quality of life
• Anxiety and depression related to the unpredictability of attacks

Red flags

Prognosis

The natural history is variable but the frequency and severity of vertigo attacks typically decrease ("burns out") over years, even without treatment, though this can take a decade or more. Hearing loss, by contrast, tends to be progressive and often becomes permanent, and imbalance can persist even once vertigo attacks have settled.¹ Around a third of patients eventually develop bilateral involvement, which has a greater impact on long-term balance and hearing than unilateral disease.