Meniere's Disease
Summary
Meniere's disease is a chronic inner ear disorder caused by abnormal endolymphatic fluid accumulation (endolymphatic hydrops). It is characterised by the classic triad of episodic vertigo, fluctuating sensorineural hearing loss, and tinnitus, often accompanied by a sensation of aural fullness. Episodes typically last 20 minutes to several hours and are followed by symptom-free intervals. Over time, progressive hearing loss develops. The diagnosis is clinical with audiometric confirmation. Treatment is aimed at symptom control during acute attacks (antiemetics) and prevention (betahistine, low-salt diet). Refractory cases may require intratympanic injections or surgery.
Key Facts
- Triad: Vertigo, Tinnitus, Sensorineural Hearing Loss (+ Aural fullness)
- Episode Duration: 20 minutes to 24 hours
- Pathophysiology: Endolymphatic hydrops (excess endolymph)
- Course: Progressive hearing loss over years
- Acute Treatment: Prochlorperazine, Cyclizine
- Prevention: Betahistine, Low-salt diet
Clinical Pearls
"Triad + Time": Meniere's is diagnosed by the triad of symptoms AND episodes lasting 20 minutes to hours. Seconds = BPPV. Constant = labyrinthitis.
"Low-Frequency Loss First": Early Meniere's causes low-frequency sensorineural hearing loss, which is fluctuating. High frequencies affected later.
"Betahistine for Prevention": Betahistine (a histamine analogue) is used for prophylaxis, not acute attacks.
"Exclude Acoustic Neuroma": Any unilateral sensorineural hearing loss should have an MRI of the IAMs to rule out vestibular schwannoma.
Incidence
- 20-200 per 100,000 per year
- Peak age: 40-60 years
Demographics
- Equal M:F (or slight female predominance)
- Usually unilateral at presentation
- Becomes bilateral in 30-50% over time
Risk Factors
- Family history (in some)
- Autoimmune conditions
- Previous ear infections/trauma
- Allergies
Endolymphatic Hydrops
- Abnormal accumulation of endolymph in the membranous labyrinth
- Causes distension of endolymphatic compartment
- Periodic rupture of membranes → Mixing of endolymph and perilymph → Symptoms
Why Episodic?
- Episodes occur with membrane rupture
- Symptoms resolve when membranes heal
- Progressive damage to hair cells → Permanent hearing loss
Classic Episode
| Feature | Description |
|---|---|
| Vertigo | Rotational, severe, prostrating |
| Duration | 20 minutes to 24 hours |
| Tinnitus | Usually low-pitched roaring/rushing |
| Hearing loss | Fluctuating initially; low frequencies first |
| Aural fullness | Sensation of pressure in ear |
| N&V | Common during attacks |
Between Episodes
Natural History
During Attack
- Nystagmus (horizontal-rotatory, towards affected ear initially then away)
- Romberg positive
- Unable to walk
- Pallor, sweating (autonomic)
Between Attacks
- May be normal
- Sensorineural hearing loss on tuning fork tests (Rinne positive, Weber lateralises to normal ear)
Red Flag Examination
- Cerebellar signs (ataxia, intention tremor) → Suggests central cause
First-Line
| Test | Findings |
|---|---|
| Audiometry | Low-frequency sensorineural hearing loss (may fluctuate) |
| Tympanometry | Normal (middle ear function) |
| Vestibular testing | Caloric testing shows reduced vestibular response |
Exclude Acoustic Neuroma
- MRI IAMs (internal auditory meatus) — Essential if unilateral SNHL to rule out vestibular schwannoma
Diagnostic Criteria (AAO-HNS 2020)
- ≥2 spontaneous episodes of vertigo (20 min to 12 hours)
- Audiometrically documented low-to-medium frequency SNHL in affected ear
- Fluctuating aural symptoms (hearing, tinnitus, fullness)
- Other causes excluded
Treatment Approach
┌──────────────────────────────────────────────────────────┐
│ MENIERE'S DISEASE MANAGEMENT │
├──────────────────────────────────────────────────────────┤
│ │
│ ACUTE ATTACK: │
│ • Prochlorperazine (buccal/IM) or Cyclizine │
│ • Rest in quiet, dark room │
│ • Avoid head movement │
│ • Short course only (avoid vestibular sedation) │
│ │
│ PROPHYLAXIS: │
│ • Betahistine 16mg TDS (may reduce attack frequency) │
│ • Low-salt diet (<2g sodium/day) │
│ • Caffeine and alcohol reduction │
│ • Stress management │
│ │
│ SECOND-LINE (Specialist): │
│ • Intratympanic steroids (may preserve hearing) │
│ • Intratympanic gentamicin (ablates vestibular function)│
│ • Meniett device (positive pressure therapy) │
│ │
│ SURGERY (Refractory): │
│ • Endolymphatic sac decompression │
│ • Vestibular neurectomy │
│ • Labyrinthectomy (destroys hearing - last resort) │
│ │
│ REHABILITATION: │
│ • Vestibular rehabilitation therapy (VRT) │
│ • Hearing aids for progressive loss │
│ │
└──────────────────────────────────────────────────────────┘
Of Disease
- Progressive sensorineural hearing loss
- Chronic tinnitus
- Falls and injury during attacks
- Anxiety and depression
- Social isolation
- Bilateral disease (30-50%)
Of Treatment
- Intratympanic gentamicin: Permanent vestibular loss, hearing loss risk
- Surgery: Hearing loss, CSF leak, facial nerve injury
Natural History
- Vertigo attacks typically reduce over years ("burn out")
- Hearing loss is progressive
- Quality of life significantly affected
With Treatment
- Most can be managed with medical therapy
- 5-10% require surgical intervention
Key Guidelines
- AAO-HNS: Clinical Practice Guideline on Meniere's Disease (2020)
- NICE CKS: Meniere's Disease
Key Evidence
Betahistine
- Cochrane review: Limited evidence but widely used; safe
Intratympanic Gentamicin
- Effective for vertigo control
- Risk of hearing loss
What is Meniere's Disease?
Meniere's disease is a disorder of the inner ear that causes episodes of spinning dizziness (vertigo), ringing in the ear (tinnitus), hearing loss, and a feeling of fullness in the ear.
What Causes It?
It's thought to be caused by too much fluid in the inner ear, but the exact reason isn't fully understood.
What Are the Symptoms?
- Vertigo: Sudden, severe spinning sensation lasting minutes to hours
- Tinnitus: Ringing, roaring, or buzzing in the ear
- Hearing loss: Comes and goes at first, then becomes permanent
- Aural fullness: Pressure/blocked feeling in the ear
How is it Treated?
- During an attack: Anti-sickness tablets
- To prevent attacks: Betahistine tablets, low-salt diet
- Hearing aids: For hearing loss
- Specialist treatments: Injections or surgery for severe cases
What's the Outlook?
Most people can manage the condition with medication. Attacks often become less frequent over time, but hearing loss usually gets gradually worse.
Primary Guidelines
- NICE Clinical Knowledge Summaries. Meniere's Disease. cks.nice.org.uk
- AAO-HNS. Clinical Practice Guideline: Meniere's Disease. Otolaryngol Head Neck Surg. 2020.
Key Studies
- James A, Burton MJ. Betahistine for Ménière's disease or syndrome. Cochrane Database Syst Rev. 2001. PMID: 11279752