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Summary Table

Feature	Detail
Risk Factors	Age 40-60, family history, autoimmune conditions, viral infections (HSV), allergies, head trauma
Etiology	Endolymphatic hydrops (excess endolymph in the membranous labyrinth)
Patient Presentation	Episodic vertigo lasting 20 minutes to 12 hours, fluctuating sensorineural hearing loss, tinnitus, aural fullness
Classic Physical Exam	Horizontal or rotatory nystagmus during acute attacks, positive Rinne (AC > BC), Weber lateralizing to the unaffected ear, Romberg falling toward the affected side
Key Diagnostic Results	Low-frequency sensorineural hearing loss on audiometry, electrocochleography showing elevated SP/AP ratio
Management	Low-sodium diet, thiazide diuretics, acute episodes treated with vestibular suppressants (meclizine, diazepam), antiemetics; refractory cases may require intratympanic gentamicin or endolymphatic sac surgery
Keywords	"Episodic vertigo," "fluctuating hearing loss," "tinnitus with aural fullness," "low-frequency hearing loss," "endolymphatic hydrops"

1. Pathophysiology

Meniere's disease results from endolymphatic hydrops, a condition where excess endolymph accumulates within the membranous labyrinth of the inner ear. Under normal conditions, endolymph is produced by the stria vascularis in the cochlea and reabsorbed by the endolymphatic sac. When reabsorption fails or production is excessive, the membranous labyrinth distends.

This distension has two consequences. First, the swollen endolymphatic compartment mechanically distorts the basilar membrane and organ of Corti, impairing cochlear transduction. Because the apical turn of the cochlea (responsible for low-frequency sound) is most compliant and therefore most susceptible to pressure changes, low-frequency hearing loss appears first. This is a hallmark finding and the reason audiometry in early Meniere's disease shows a characteristic rising pattern on the audiogram.

Second, periodic rupture of the distended Reissner's membrane allows potassium-rich endolymph to mix with perilymph. This sudden ionic shift bathes the vestibular hair cells and nerve fibers in high-potassium fluid, causing depolarization blockade. The result is a sudden, intense episode of vertigo with nystagmus as the brain receives asymmetric vestibular input. The affected labyrinth initially fires excessively (irritative phase), then becomes temporarily paralyzed (paralytic phase). This explains why the nystagmus may initially beat toward the affected ear and then reverse direction.

The tinnitus and sensation of aural fullness correlate directly with the degree of endolymphatic pressure. As the membrane heals and ionic gradients normalize, symptoms resolve, which is why the disease is episodic and self-limiting per attack but progressive over years. Repeated membrane ruptures cause cumulative damage to both cochlear and vestibular hair cells, leading to permanent hearing loss and eventual burnout of the vestibular organ in late-stage disease.

2. Classification and Clinical Manifestation

Staging by Hearing Loss

Stage	Pure Tone Average at 0.5, 1, 2, 3 kHz	Clinical Features
Stage 1	PTA $\leq$ 25 dB	Episodic vertigo, fluctuating low-frequency hearing loss that fully recovers between attacks, intermittent tinnitus
Stage 2	PTA 26-40 dB	More frequent attacks, hearing loss begins to persist between episodes, tinnitus becomes more constant
Stage 3	PTA 41-70 dB	Hearing loss now involves mid and high frequencies, episodes may become less violent but imbalance persists
Stage 4	PTA > 70 dB	Severe-to-profound sensorineural hearing loss, vertigo episodes may decrease ("burnt-out" Meniere's), chronic disequilibrium replaces episodic vertigo

Clinical Manifestation by Symptom

Symptom	Characteristics	Mechanism
Vertigo	Spontaneous, rotational, lasting 20 min to 12 hours; accompanied by nausea and vomiting; never lasts only seconds	Acute endolymph-perilymph mixing causing vestibular hair cell depolarization blockade
Hearing loss	Fluctuating, initially low-frequency, sensorineural; recovers early but becomes permanent over time	Distension of cochlear duct distorting the basilar membrane, preferentially affecting the apical turn
Tinnitus	Low-pitched roaring or buzzing, ipsilateral, worsens before or during attacks	Abnormal stimulation of cochlear hair cells from increased endolymphatic pressure
Aural fullness	Sensation of pressure or blockage in the affected ear, often precedes vertigo	Direct effect of endolymphatic distension on the tympanic membrane and middle ear structures

Definite vs. Probable Meniere's Disease (2015 Classification Criteria)

Category	Criteria
Definite	Two or more spontaneous episodes of vertigo each lasting 20 min to 12 hours AND audiometrically documented low-to-mid frequency sensorineural hearing loss in the affected ear on at least one occasion before, during, or after an episode AND fluctuating aural symptoms (hearing, tinnitus, fullness) in the affected ear AND not better accounted for by another vestibular diagnosis
Probable	Two or more episodes of vertigo or dizziness each lasting 20 min to 24 hours AND fluctuating aural symptoms in the affected ear AND not better accounted for by another vestibular diagnosis

3. Diagnostic Workup

Test	Role	Expected Finding
Pure tone audiometry	Best initial test	Low-frequency sensorineural hearing loss ($\geq$ 30 dB difference at 250, 500, 1000 Hz compared to 2000, 4000 Hz); fluctuates between episodes
Speech discrimination testing	Supportive	Disproportionately poor speech discrimination relative to the degree of hearing loss
Electrocochleography (ECoG)	Confirmatory / supportive	Elevated SP/AP ratio > 0.4 (summating potential to action potential), indicating endolymphatic hydrops
MRI with gadolinium (inner ear protocol)	Most accurate for visualizing hydrops	Direct visualization of endolymphatic hydrops on delayed gadolinium-enhanced MRI; also rules out vestibular schwannoma
Caloric testing / VNG	Assess vestibular function	Reduced vestibular response (canal paresis) on the affected side
Glycerol or furosemide test	Provocative / adjunctive	Temporary improvement in hearing after osmotic challenge supports the diagnosis of hydrops

Pure tone audiometry is the cornerstone of the workup and should be the first test ordered when Meniere's disease is suspected. The classic finding is a low-frequency sensorineural hearing loss pattern, often described as a rising audiogram. Serial audiometry is valuable because the hallmark of early disease is fluctuation: hearing may be normal between attacks. During or immediately after an episode, audiometry will capture the characteristic dip.

Electrocochleography measures the ratio of the summating potential (SP) to the action potential (AP) at the cochlear nerve. An SP/AP ratio exceeding 0.4 is considered abnormal and suggests endolymphatic hydrops. This test is particularly useful when audiometry alone is inconclusive.

Gadolinium-enhanced MRI of the inner ear (using intratympanic or intravenous gadolinium with delayed imaging) has become the gold standard for directly visualizing endolymphatic hydrops. It also serves a critical role in excluding vestibular schwannoma (acoustic neuroma), which can mimic Meniere's disease with unilateral hearing loss and tinnitus. Any patient with asymmetric sensorineural hearing loss should receive MRI to rule this out.

Videonystagmography (VNG) with caloric testing assesses the function of each horizontal semicircular canal independently. In Meniere's disease, the affected ear typically shows a reduced caloric response (unilateral weakness). This test does not confirm the diagnosis on its own but quantifies the degree of vestibular damage.

The glycerol dehydration test (oral glycerol followed by repeat audiometry) can provide supportive evidence. If hearing improves by at least 10 dB after the osmotic challenge, it suggests that reversible hydrops is present.

4. Management and Treatment

Phase	Intervention	Details
Lifestyle modification	Low-sodium diet	Target $<$ 1500-2000 mg/day; reduces endolymph volume
Lifestyle modification	Avoid triggers	Caffeine, alcohol, tobacco, stress
Acute attack	Vestibular suppressants	Meclizine 25 mg PO every 8 hours PRN, or diazepam 5 mg PO/IV PRN
Acute attack	Antiemetics	Ondansetron 4 mg IV/PO or promethazine 25 mg IM/PR
Prophylaxis (first-line)	Thiazide diuretics	Hydrochlorothiazide 25 mg PO daily or combination with triamterene
Prophylaxis (first-line)	Betahistine	16-48 mg PO daily in divided doses (widely used outside the US)
Refractory (second-line)	Intratympanic dexamethasone	4 mg/mL injected through tympanic membrane; preserves hearing
Refractory (third-line, ablative)	Intratympanic gentamicin	Selectively destroys vestibular hair cells; controls vertigo in ~80-90% but carries risk of hearing loss
Surgical (last resort)	Endolymphatic sac decompression	Hearing-preserving surgical option
	Vestibular nerve section	Highly effective for vertigo control, preserves hearing
	Labyrinthectomy	Definitive but causes complete hearing loss in the operated ear; reserved for patients with no serviceable hearing

Acute management focuses on symptom control. During an episode, the patient should be placed in a quiet, dark room. Meclizine (an antihistamine with anticholinergic properties) is the most commonly used vestibular suppressant. Benzodiazepines such as diazepam suppress the vestibular nucleus directly and are effective for severe episodes. These medications should not be used long-term because they impair central vestibular compensation.

Long-term prophylaxis begins with dietary sodium restriction, which is the most consistently recommended intervention. Reducing sodium intake lowers the osmotic gradient driving fluid into the endolymphatic space. Thiazide diuretics (hydrochlorothiazide 25 mg daily, often combined with triamterene to prevent hypokalemia) are added when lifestyle changes alone are insufficient. Patients on thiazides require periodic monitoring of electrolytes and renal function. Betahistine, a histamine H1 agonist and H3 antagonist, improves microcirculation in the stria vascularis and is widely prescribed in Europe and Asia, though it is not FDA-approved in the United States.

For patients who fail medical therapy, intratympanic dexamethasone injections are the preferred next step because they offer anti-inflammatory benefit without risk to hearing. If vertigo remains uncontrolled, intratympanic gentamicin is the ablative option of choice. Gentamicin is preferentially vestibulotoxic (more toxic to vestibular than cochlear hair cells), so it can eliminate vertigo episodes while partially preserving hearing. However, there is a 15-30% risk of further hearing loss, so this must be discussed with the patient.

Surgical options are reserved for truly refractory cases. Endolymphatic sac decompression is the least invasive surgical approach and preserves both hearing and vestibular function, though its efficacy is debated. Vestibular nerve section (translabyrinthine or retrosigmoid approach) has the highest vertigo cure rate among hearing-preserving surgeries. Labyrinthectomy is definitive but sacrifices all hearing in the operated ear and is only appropriate when the ear has no serviceable hearing remaining.

Contraindications and cautions: Avoid meclizine and anticholinergics in elderly patients due to sedation and fall risk. Benzodiazepines carry dependence risk. Gentamicin is contraindicated if the contralateral ear also has hearing loss (risk of bilateral deafness). Diuretics require caution in patients with renal insufficiency or electrolyte abnormalities.

5. Differential Diagnosis and Distractors

Differential Diagnosis	Why It's Similar	Key Discriminator
Vestibular schwannoma (acoustic neuroma)	Unilateral sensorineural hearing loss, tinnitus	Hearing loss is progressive and non-fluctuating, vertigo is uncommon (imbalance is more typical), and MRI shows a CP angle mass enhancing with gadolinium
Benign paroxysmal positional vertigo (BPPV)	Episodic vertigo with nystagmus	Episodes last seconds to one minute (not 20 min to hours), triggered by head position changes, no hearing loss, positive Dix-Hallpike maneuver
Vestibular neuritis	Acute severe vertigo with nystagmus	Single prolonged episode lasting days (not recurrent short episodes), no hearing loss, no tinnitus, no aural fullness
Labyrinthitis	Vertigo with hearing loss	Typically a single acute episode (often post-viral), hearing loss is sudden and non-fluctuating rather than episodic
Superior semicircular canal dehiscence (SSCD)	Vertigo, hearing changes, aural fullness	Vertigo triggered by loud sounds (Tullio phenomenon) or Valsalva maneuver, conductive hearing loss with intact reflexes, CT temporal bone shows bony dehiscence
Migraine-associated vertigo (vestibular migraine)	Recurrent episodic vertigo, aural fullness, can have auditory symptoms	Episodes often associated with headache, photophobia, phonophobia; hearing loss, if present, is not progressive; audiometry between attacks is normal
Perilymphatic fistula	Episodic vertigo with fluctuating hearing loss	History of barotrauma, straining, or surgery; positive fistula test (vertigo/nystagmus with pneumatic otoscopy)

6. Traps and High-Yield Pearls

The most common way students miss Meniere's disease questions is by confusing it with BPPV or vestibular migraine. The discriminator is duration: BPPV episodes last seconds, Meniere's episodes last 20 minutes to 12 hours, and vestibular migraine episodes can overlap in duration but lack progressive audiometric hearing loss. When a vignette describes episodic vertigo, the first reflex should be to check the duration and whether hearing loss is documented.

Another frequent trap involves the diagnostic workup. Students often select "MRI" as the first test when they see unilateral hearing loss and tinnitus, thinking of vestibular schwannoma. While MRI is indeed necessary to rule out a schwannoma, the best initial test for a patient presenting with the classic Meniere's triad is audiometry. MRI comes into the workup to exclude structural pathology, not as the first-line diagnostic step for Meniere's itself.

A third pitfall involves management sequencing. Test-writers will present a Meniere's patient who has failed dietary modification and diuretics, then ask for the next step. The answer is intratympanic dexamethasone (non-ablative, hearing-preserving), not intratympanic gentamicin (ablative). Gentamicin is reserved for patients who have failed steroid injections. Jumping to gentamicin too early is a common error, as is jumping directly to surgery.

Finally, remember the "burnt-out" Meniere's concept. A late-stage patient may present with chronic disequilibrium and severe hearing loss but few vertigo attacks. This occurs because the vestibular end organ has been destroyed by repeated injury. Students may not recognize this as Meniere's because the classic episodic vertigo is absent, but the history of prior recurrent episodes and the audiometric pattern will point to the diagnosis.

The core competency being tested is the ability to recognize the full clinical tetrad (episodic vertigo, fluctuating sensorineural hearing loss, tinnitus, aural fullness), correctly sequence the diagnostic workup, and apply a stepwise escalation of therapy from conservative to ablative to surgical.

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