What is Ménière’s disease and how should it be diagnosed and managed in a 54‑year‑old woman with left‑sided unilateral tinnitus and dull headache?

What is Ménière's Disease

Ménière's disease is a chronic inner ear disorder characterized by recurrent spontaneous vertigo attacks lasting 20 minutes to 12 hours, fluctuating low- to mid-frequency sensorineural hearing loss, tinnitus, and aural fullness in the affected ear. 1

Pathophysiology

The underlying pathologic process involves distortion of the membranous labyrinth with formation of endolymphatic hydrops (excess fluid accumulation in the inner ear). 2 This results from a deficiency in endolymph absorption, though the exact mechanism by which hydrops produces symptoms remains debated—theories include membranous ruptures, mechanical displacement of sensory organs, or intermittent obstruction of the endolymphatic duct. 3

Diagnostic Criteria

Definite Ménière's Disease

The American Academy of Otolaryngology-Head and Neck Surgery established specific criteria that require ALL of the following: 1

• Two or more spontaneous vertigo attacks, each lasting 20 minutes to 12 hours
• Audiometrically documented low- to mid-frequency sensorineural hearing loss in the affected ear on at least one occasion before, during, or after a vertigo episode
• Fluctuating aural symptoms (hearing loss, tinnitus, or ear fullness) in the affected ear
• Other causes excluded by appropriate testing

Probable Ménière's Disease

For patients who don't yet meet definite criteria: 1

• At least 2 episodes of vertigo or dizziness lasting 20 minutes to 24 hours
• Fluctuating aural symptoms in the affected ear
• Other causes excluded

Clinical Presentation in Your 54-Year-Old Patient

For this patient with left-sided unilateral tinnitus and dull headache, you must first determine if she has experienced the hallmark symptom: true rotatory vertigo (a false sensation of spinning or self-motion), not just vague dizziness or lightheadedness. 1 Many patients incorrectly describe presyncope as "dizziness," which is inconsistent with Ménière's disease. 1

Key questions to ask:

• Has she had discrete episodes of spinning sensation lasting 20 minutes to 12 hours? 1
• Does she experience fluctuating hearing loss, particularly noticing difficulty with lower-pitched sounds? 1
• Does she feel ear fullness or pressure in the left ear? 1
• Do her symptoms fluctuate over time rather than being constant? 1

Important caveat: Elderly patients with long-standing Ménière's disease may present with "vague dizziness" rather than frank vertigo, making diagnosis more challenging. 1

Essential Diagnostic Workup

Required Testing

Audiometry is mandatory to document the characteristic low- to mid-frequency sensorineural hearing loss pattern. 4 The Weber tuning fork test will lateralize to the unaffected (right) ear if she has unilateral sensorineural hearing loss. 5

MRI with contrast of the internal auditory canals is essential to exclude cerebellopontine angle tumors (particularly vestibular schwannoma), which can mimic Ménière's disease but typically present with chronic imbalance and non-fluctuating hearing loss. 1, 6

Critical Differential Diagnoses to Exclude

The following conditions must be ruled out, as they can present similarly: 1

Condition	Key Differentiating Features
Vestibular migraine	Vertigo episodes may be shorter (<20 min) or longer (>24 hours); hearing loss less common; migraine history; photophobia [1]
Vestibular schwannoma	Chronic imbalance rather than episodic vertigo; hearing loss doesn't fluctuate; progressive unilateral hearing loss [1]
BPPV	Brief vertigo (<1 minute) triggered by position changes; no hearing loss or tinnitus [1]
Labyrinthitis	Sudden severe vertigo lasting >24 hours; hearing loss doesn't fluctuate; not episodic [1]
Vestibular neuritis	Acute prolonged vertigo (12-36 hours) WITHOUT hearing loss, tinnitus, or fullness [1]
Stroke/ischemia	Symptoms don't fluctuate; may have dysphagia, dysphonia, or other neurologic signs; usually no hearing loss [1]

Additional testing to consider: Exclude otosyphilis, autoimmune inner ear disease, Lyme disease, and multiple sclerosis, particularly if atypical features are present. 6

Management Algorithm

First-Line Conservative Measures

Begin with dietary and lifestyle modifications: 4, 6

• Low-sodium diet (1500-2300 mg daily) to reduce fluid retention 7
• Avoid caffeine, alcohol, and nicotine to prevent symptom exacerbation 7, 8
• Stress management and sleep hygiene 6
• Diuretics (typically thiazide-type) as first-line medical therapy to reduce endolymphatic pressure 4, 7

Second-Line Medical Therapy

If conservative measures fail after several months: 4

• Betahistine (histamine analogue) to increase inner ear vasodilation and improve circulation 7
• Antihistamines if allergies are suspected as contributing factors 7
• Intratympanic steroid injections for active disease not responsive to conservative measures 4, 7

Third-Line Interventions

For persistent vertigo despite optimal medical management: 6

• Low-dose intratympanic gentamicin to reduce vestibular function in the affected ear (does NOT require complete ablation to control vertigo) 2, 6
• Vestibular rehabilitation/physical therapy for patients with residual imbalance 4

Surgical Options

Reserved for severe, refractory cases: 6, 3

• Endolymphatic sac decompression (hearing-sparing procedure) 7
• Vestibular nerve section (hearing-sparing, controls vertigo) 7
• Labyrinthectomy (ablative, sacrifices remaining hearing but controls vertigo) 7

Hearing Rehabilitation

As hearing deteriorates over time: 7

• Conventional hearing aids for usable hearing (Class A, B, or C) 4, 7
• CROS hearing aids when speech discrimination is severely compromised 4, 7
• Cochlear implants for severe hearing loss where amplification provides limited benefit 4, 7

Disease Progression and Prognosis

Critical points to discuss with your patient: 4, 9

• Hearing loss typically worsens over time despite treatment, initially fluctuating in low-to-mid frequencies but eventually involving all frequencies 7
• Complete hearing restoration is not currently possible 7
• 10-25% of cases eventually become bilateral, which impacts surgical decision-making 4
• Drop attacks (Tumarkin's otolithic crisis) may occur in later stages without warning 4, 7
• Vertigo episodes often decrease over 10+ years as hearing deteriorates 8
• Headache is common during attacks and bilateral involvement occurs in 25-40% of cases 9

Monitoring Requirements

Regular follow-up must include: 4, 7

• Serial audiometry to track hearing progression
• Documentation of vertigo frequency and severity
• Assessment of quality of life impact
• Monitoring for bilateral involvement

Common Pitfalls to Avoid

Do not diagnose Ménière's disease if: 1

• The patient describes lightheadedness or presyncope rather than true rotatory vertigo
• Vertigo episodes last less than 20 minutes or more than 12 hours (consider vestibular migraine or other diagnoses)
• Hearing loss is not documented audiometrically
• Symptoms are constant rather than fluctuating
• Other causes have not been adequately excluded

Remember: Up to 60% of patients show improvement with placebo in clinical trials, making treatment efficacy assessment challenging. 4 This underscores the importance of objective audiometric monitoring rather than relying solely on subjective symptom reports.