Inner-ear disorder of recurrent vertigo episodes, fluctuating low-frequency sensorineural hearing loss, tinnitus, and aural fullness.
Also known as: Meniere disease, endolymphatic hydrops, Ménière's disease
Overview
An idiopathic inner-ear disorder characterized by recurrent spontaneous episodes of vertigo lasting 20 minutes to 12 hours, accompanied by fluctuating low- to mid-frequency sensorineural hearing loss, tinnitus, and aural fullness in the affected ear (per Bárány Society / AAO-HNS criteria).
Epidemiology
Prevalence ~200 per 100,000. Onset typically between ages 40 and 60. Female predominance ~1.3:1. Bilateral involvement develops in 25-50% over years. Genetic predisposition (HLA associations, familial clusters).
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Question 1NeurologyMedium
A 49-year-old woman has episodic vertigo lasting hours, fluctuating hearing loss, tinnitus, and aural fullness. Which of the following best explains this disorder?
AExcess endolymphatic fluid pressure
BOtoliths displaced into the posterior canal
CVestibular schwannoma compressing CN VIII
DCerebellar infarction from an embolus
EReactivation of vestibular nerve herpesvirus
Reveal answer & full explanation
Correct answer: A — Excess endolymphatic fluid pressure
AExcess endolymphatic fluid pressure✓
BOtoliths displaced into the posterior canal
CVestibular schwannoma compressing CN VIII
DCerebellar infarction from an embolus
EReactivation of vestibular nerve herpesvirus
Why Excess endolymphatic fluid pressure is correct
Ménière disease reflects endolymphatic hydrops, an overaccumulation of endolymph that distends the membranous labyrinth.
This produces the classic tetrad of episodic vertigo lasting 20 minutes to hours, fluctuating sensorineural hearing loss, tinnitus, and aural fullness.
The cochlear plus vestibular involvement together distinguish it from purely positional or purely vestibular causes.
Why the others are wrong
Otoliths displaced into the posterior canal — Canalithiasis causes BPPV, which is brief seconds-long positional vertigo WITHOUT hearing loss or tinnitus (vertigo-subtype trap).
Reactivation of vestibular nerve herpesvirus — Vestibular neuritis causes a single days-long vertiginous spell after a viral illness and spares hearing, unlike this fluctuating cochlear picture (right-organ-wrong-pattern).
Cerebellar infarction from an embolus — A cerebellar stroke gives continuous vertigo with ataxia and other neurologic deficits, not recurrent attacks with aural fullness (dangerous-mimic trap).
Vestibular schwannoma compressing CN VIII — An acoustic neuroma also causes sensorineural hearing loss and tinnitus, but the hearing loss is typically slowly progressive rather than fluctuating, and the slow growth allows central compensation, so patients usually have mild chronic imbalance rather than discrete hours-long vertigo attacks (hearing-loss-overlap trap).
Question 2NeurologyMedium
A 48-year-old woman presents with a 6-month history of recurrent spontaneous episodes of spinning vertigo, each lasting 2 to 3 hours and accompanied by nausea and vomiting. During the episodes she notices a roaring, low-pitched ringing and a sense of pressure in her right ear, along with muffled hearing on that side that improves between attacks. She denies positional triggers. An audiogram performed after a recent attack shows fluctuating low-frequency sensorineural hearing loss in the right ear, and MRI of the internal auditory canals is normal. Which of the following is the most likely diagnosis?
AMénière disease
BVestibular migraine
CVestibular neuritis
DViral labyrinthitis
EVertebrobasilar insufficiency
Reveal answer & full explanation
Correct answer: A — Ménière disease
AMénière disease✓
BVestibular migraine
CVestibular neuritis
DViral labyrinthitis
EVertebrobasilar insufficiency
Why Ménière disease is correct
The Bárány Society / AAO-HNS 2015 criteria define definite Ménière disease as ≥2 episodes of spontaneous vertigo lasting 20 minutes to 12 hours, plus audiometrically documented low- to mid-frequency sensorineural hearing loss in the affected ear, plus fluctuating aural symptoms (hearing loss, tinnitus, fullness) in that ear, with no better explanation.
This patient hits every element: recurrent 2-3 hour spontaneous vertigo, unilateral fluctuating low-frequency SNHL on audiogram, roaring low-pitched tinnitus, and aural fullness on the right.
A normal MRI of the internal auditory canals appropriately excludes vestibular schwannoma, supporting the clinical diagnosis.
Why the others are wrong
Vertebrobasilar insufficiency — posterior circulation ischemia produces vertigo accompanied by brainstem or cerebellar signs such as diplopia, dysarthria, ataxia, or visual field loss, and spells usually last minutes; it does not cause 6 months of recurrent attacks with fluctuating low-frequency sensorineural hearing loss, roaring tinnitus, and aural fullness confined to one ear.
Vestibular migraine — the most common Ménière mimic, and it can produce recurrent spontaneous vertigo of similar duration, but hearing is characteristically preserved; audiometrically documented fluctuating low-frequency sensorineural hearing loss with aural fullness in one ear points to endolymphatic hydrops instead.
Vestibular neuritis — a single prolonged attack of vertigo lasting days after a viral illness, without hearing loss; it does not recur episodically and spares cochlear function.
Viral labyrinthitis — vertigo accompanied by unilateral hearing loss and tinnitus is tempting, but labyrinthitis is a single acute attack of continuous vertigo lasting days, often after a viral illness, with hearing loss that does not fluctuate with recurrent attacks; 6 months of recurrent 2-3 hour spells with fluctuating low-frequency loss points to endolymphatic hydrops.
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Endolymphatic hydrops — overaccumulation of endolymph distends the scala media and may rupture Reissner's membrane, producing acute potassium-induced injury to vestibular and cochlear hair cells. Etiology of hydrops is multifactorial: impaired endolymph absorption at the endolymphatic sac, autoimmune injury, ionic dysregulation, viral.
Clinical presentation
Symptoms
Episodic spontaneous vertigo lasting 20 min to 12 h (rarely up to 24 h)
Fluctuating low- to mid-frequency sensorineural hearing loss, typically unilateral
Roaring or low-pitched tinnitus that may worsen during episodes
Aural fullness or pressure in the affected ear
Nausea, vomiting, diaphoresis during episodes
Tumarkin 'otolithic crisis' drop attacks (sudden falls without warning, no LOC) in advanced disease
Signs / physical exam
Between attacks: exam often normal; later, persistent unilateral sensorineural hearing loss on audiogram
Spontaneous horizontal-torsional nystagmus during attacks (initially toward the affected ear in irritative phase, then away as the labyrinth fatigues)
Positive head-impulse test toward the affected side once vestibular function is reduced
Tuning fork tests (Weber, Rinne) consistent with unilateral SNHL
Perilymph fistula — Vertigo triggered by Valsalva, sneezing, or pressure changes; recent barotrauma or head injury
Posterior circulation TIA/stroke (vertebrobasilar ischemia, cerebellar infarct) — Vertigo with brainstem/cerebellar signs (diplopia, dysarthria, ataxia, visual field loss); spells last minutes or the deficit persists; vascular risk factors; no fluctuating low-frequency SNHL or aural fullness
Diagnostic workup
Diagnostic criteria
Definite Ménière (Bárány/AAO-HNS 2015): ≥2 episodes of spontaneous vertigo lasting 20 min to 12 h + audiometrically documented low- to mid-frequency SNHL in the affected ear before, during, or after an episode + fluctuating aural symptoms (hearing loss, tinnitus, fullness) in the affected ear + no better explanation.
Pure-tone and speech audiometry — low/mid-frequency SNHL, often fluctuating; word recognition typically preserved early
MRI with gadolinium of internal auditory canals — exclude vestibular schwannoma and central pathology
Vestibular testing (videonystagmography, caloric, VEMPs) — not needed to establish the diagnosis (AAO-HNS 2020 recommends against ordering it for that purpose); reserved for unclear cases or before ablative therapy
Electrocochleography — elevated SP/AP ratio supports hydrops, but it is not recommended to establish the diagnosis (AAO-HNS 2020)
Delayed gadolinium-enhanced MRI inner ear (specialized centers) can visualize hydrops
Treatment
First-line
Low-sodium diet (<2 g/day), caffeine and alcohol reduction, smoking cessation
Thiazide diuretic (hydrochlorothiazide, often combined with triamterene) or acetazolamide — to reduce endolymph volume
Vestibular suppressants for acute attacks: meclizine, dimenhydrinate, promethazine, lorazepam, prochlorperazine — limit to acute use; chronic use impairs vestibular compensation
Antiemetics (ondansetron, prochlorperazine) for nausea
Patient education and reassurance about benign nature of attacks
Vestibular rehabilitation for interictal imbalance
Persistent or refractory disease
Intratympanic corticosteroid (dexamethasone) injections — vestibular-sparing, may help hearing and vertigo
Intratympanic gentamicin — selectively ablates vestibular hair cells; controls vertigo at the cost of vestibular function (and some hearing risk)
Betahistine — used widely outside the US; evidence mixed; not FDA-approved
Hearing aids for permanent SNHL; cochlear implant for severe bilateral hearing loss
Counseling and support groups for chronic disease impact
Complications
Progressive permanent sensorineural hearing loss
Bilateral involvement with cumulative hearing and balance disability
Drop attacks (Tumarkin crises) with fall-related injury
Chronic anxiety and depression
Loss of driving privileges and employment impact
Persistent disequilibrium between episodes
PANCE pearls
The combination of low-frequency SNHL with fluctuation is highly specific for Ménière — pure high-frequency loss argues for presbycusis or noise exposure.
Always image with MRI to exclude vestibular schwannoma before settling on Ménière, especially with unilateral hearing loss.
Vestibular migraine is the most common Ménière mimic — overlapping features include episodic vertigo, photophobia, and family history of migraine.
Intratympanic gentamicin trades vestibular function for vertigo control; counsel about persistent imbalance.
Avoid chronic vestibular suppressants — they prevent central compensation.
References
Bárány Society 2015 — Lopez-Escamez JA et al. Diagnostic criteria for Ménière's disease. J Vestib Res 2015;25:1-7.
AAO-HNS 2020 — Basura GJ et al. Clinical practice guideline: Ménière's disease. Otolaryngol Head Neck Surg 2020;162(2_suppl):S1-S55.
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