What are the diagnostic criteria and management options for Meniere's (Inner Ear) disease?

Diagnostic Criteria and Management of Meniere's Disease

Meniere's disease should be diagnosed based on specific clinical criteria including recurrent vertigo episodes lasting 20 minutes to 12 hours, documented low-to-midfrequency sensorineural hearing loss, and fluctuating aural symptoms, with management focused on vertigo control and hearing preservation through a stepwise approach from conservative to more invasive interventions. 1

Diagnostic Criteria

Definite Meniere's Disease

• Two or more spontaneous attacks of vertigo, each lasting 20 minutes to 12 hours 1
• Audiometrically documented fluctuating low- to mid-frequency sensorineural hearing loss in the affected ear on at least one occasion before, during, or after one of the episodes of vertigo 1
• Fluctuating aural symptoms (hearing loss, tinnitus, or fullness) in the affected ear 1
• Other causes excluded by appropriate tests 1

Probable Meniere's Disease

• At least 2 episodes of vertigo or dizziness lasting 20 minutes to 24 hours 1, 2
• Fluctuating aural symptoms (hearing loss, tinnitus, or fullness) in the affected ear 1, 3
• Other causes excluded by appropriate tests 1

Differential Diagnosis

When evaluating patients with suspected Meniere's disease, it's important to rule out other conditions that may present with similar symptoms:

• Vestibular migraine: Vertigo episodes lasting minutes to days, often with headache history 1
• Benign paroxysmal positional vertigo (BPPV): Brief vertigo episodes provoked by head position changes 1
• Labyrinthitis: Sudden severe vertigo with prolonged symptoms (>24 hours) and hearing loss 1
• Vestibular neuritis: Acute prolonged vertigo without hearing loss 1
• Cerebellopontine angle tumors: Progressive unilateral hearing loss with imbalance 1

Diagnostic Workup

• Audiometry: Essential for documenting the characteristic low- to mid-frequency sensorineural hearing loss 1, 4
• MRI with contrast of internal auditory canals: To exclude other causes like vestibular schwannoma 5
• Vestibular testing: May include caloric testing, vestibular-evoked myogenic potentials, and head impulse tests 6
• Electrocochleography: May show increased summating potential/action potential ratio 6

Management Algorithm

First-Line (Conservative) Treatments

• Low-sodium diet (1500-2300 mg daily) to reduce fluid retention 7
• Avoidance of dietary triggers: caffeine, alcohol 7
• Stress management and improved sleep hygiene 5
• Diuretics: To reduce excess body fluid and potentially stabilize inner ear fluid dynamics 7
• Limited course of vestibular suppressants during acute attacks 1

Second-Line Treatments (If First-Line Fails)

• Intratympanic steroid injections: For patients with active Meniere's disease not responsive to conservative measures 1, 7
• Betahistine: May improve inner ear circulation (commonly used in Europe) 7, 8

Third-Line Treatments

• Intratympanic gentamicin: For patients with active Meniere's disease not responsive to nonablative therapy 1
• Endolymphatic sac decompression: A hearing-preservation surgical option, though efficacy is controversial 7, 5

Fourth-Line (Ablative) Treatments

• Vestibular nerve section: For severe, refractory cases with usable hearing 7
• Labyrinthectomy: For patients with severe symptoms and non-usable hearing 7

Hearing Rehabilitation

• Conventional hearing aids for patients with usable hearing 7
• CROS (Contralateral Routing of Signal) hearing aids when speech discrimination is severely compromised 7
• Cochlear implants for patients with severe hearing loss where amplification provides limited benefit 7

Monitoring and Follow-up

• Regular audiometric testing to monitor hearing progression 4, 7
• Assessment of quality of life impact as part of ongoing management 7
• Documentation of changes in hearing loss, tinnitus, and vertigo after treatment 7

Important Clinical Considerations

• The hearing loss pattern in Meniere's disease initially fluctuates but may eventually involve all frequencies as the disease progresses 1, 7
• Bilateral involvement occurs in 25-40% of cases 8
• Vestibular rehabilitation/physical therapy should be offered for patients who have failed less definitive therapy and have non-usable hearing 1
• Positive pressure therapy is not recommended for patients with Meniere's disease 1
• Drop attacks (Tumarkin's otolithic crisis) may occur in later stages of the disease 1

By following this structured approach to diagnosis and management, clinicians can effectively identify and treat patients with Meniere's disease, focusing on controlling vertigo episodes and preserving hearing function to improve quality of life.