Inner-ear disorder of recurrent vertigo episodes, fluctuating low-frequency sensorineural hearing loss, tinnitus, and aural fullness.
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Risk factors
- Family history of Ménière disease
- Autoimmune inner ear disease
- Migraine (overlap with vestibular migraine)
- Allergic disorders
- Possible viral or immune triggers
Pathophysiology
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
Classic findings
Recurrent spontaneous vertigo + unilateral low-frequency hearing loss + tinnitus + aural fullness.
Differential diagnosis
- Vestibular migraine — Recurrent vertigo + migraine features (headache, photophobia, phonophobia, aura); hearing usually normal; responds to migraine prophylaxis
- BPPV — Brief (seconds), positional vertigo with Dix-Hallpike; no hearing loss; treated with Epley maneuver
- Vestibular neuritis — Single prolonged episode (days), no hearing loss, follows viral illness; unilateral caloric weakness
- Labyrinthitis — Single episode with hearing loss; viral or bacterial; does not recur in classic form
- Acoustic neuroma (vestibular schwannoma) — Progressive unilateral hearing loss + tinnitus; vertigo less prominent; MRI internal auditory canal diagnostic
- Autoimmune inner ear disease — Bilateral, rapidly progressive sensorineural hearing loss; steroid-responsive
- Perilymph fistula — Vertigo triggered by Valsalva, sneezing, or pressure changes; recent barotrauma or head injury
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.
Labs
- TSH, CBC, comprehensive metabolic panel, ANA, RPR, B12 (exclude metabolic/autoimmune mimics)
- Lyme serology in endemic areas
Imaging
- 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) as indicated
- Electrocochleography — elevated SP/AP ratio supports hydrops (not diagnostic alone)
- 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)
- Endolymphatic sac decompression — controversial benefit
- Selective vestibular nerve section — preserves hearing; effective for vertigo control in serviceable-hearing patients
- Labyrinthectomy — definitive vertigo control but destroys hearing; reserved for non-serviceable hearing
Migraine overlap
- Trial migraine prophylaxis (propranolol, topiramate, amitriptyline, venlafaxine, magnesium) if vestibular migraine features present
- Lifestyle measures: sleep, hydration, trigger avoidance
Second-line / adjunct
- 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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