ENT · Inner Ear Disorders
The facts most likely to be tested
Meniere disease is characterized by the classic triad of episodic vertigo, sensorineural hearing loss, and tinnitus.
The underlying pathophysiology involves endolymphatic hydrops, which is the distension of the endolymphatic space within the inner ear.
Patients typically experience aural fullness or a sensation of pressure in the affected ear during or preceding an attack.
The vertigo associated with Meniere disease is typically episodic, lasting from 20 minutes to several hours, and is often accompanied by nausea and vomiting.
Initial management for acute attacks includes vestibular suppressants such as meclizine or benzodiazepines.
Long-term prophylactic management focuses on low-sodium diet and diuretics (e.g., hydrochlorothiazide) to reduce endolymphatic volume.
Audiometry in Meniere disease typically demonstrates low-frequency sensorineural hearing loss.
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A 45-year-old female presents to the clinic complaining of recurrent episodes of severe spinning sensation that last approximately 2 hours. She reports that during these episodes, she feels a sense of aural fullness in her right ear accompanied by tinnitus and muffled hearing. She has experienced three such episodes in the past month. Physical examination reveals horizontal nystagmus during the attack, and audiometry shows low-frequency sensorineural hearing loss in the right ear.
What is the most likely diagnosis?
Meniere disease
The patient presents with the classic triad of episodic vertigo, tinnitus, and sensorineural hearing loss, which, combined with the finding of low-frequency hearing loss and aural fullness, is pathognomonic for Meniere disease (Bet 1 and Bet 7).
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Etiology / Epidemiology
Idiopathic endolymphatic hydrops typically presenting in ages 20-60.
Clinical Manifestations
Classic triad: episodic vertigo, sensorineural hearing loss, and tinnitus.
Diagnosis
Clinical diagnosis; audiometry confirms low-frequency sensorineural hearing loss.
Treatment
Salt restriction and diuretics (HCTZ/triamterene) are first-line; avoid caffeine/nicotine.
Prognosis
Chronic condition; permanent hearing loss occurs in 50% of patients.
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Epidemiology & Etiology
Meniere disease is an idiopathic disorder of the inner ear affecting adults aged 20-60. It is characterized by endolymphatic hydrops, or distension of the endolymphatic compartment. While often idiopathic, it may be triggered by autoimmune processes or prior viral infections.
Pertinent Anatomy
The pathology involves the membranous labyrinth of the inner ear. Excess fluid pressure disrupts the function of the cochlea (hearing) and the vestibular system (balance).
Pathophysiology
Increased volume of endolymph leads to rupture of the Reissner membrane. This allows potassium-rich endolymph to mix with perilymph, causing depolarization block of hair cells. This results in the classic episodic symptoms of vertigo and fluctuating hearing loss.
Clinical Manifestations
Patients present with the classic triad of episodic vertigo, fluctuating sensorineural hearing loss, and tinnitus or aural fullness. Vertigo attacks are sudden, severe, and last 20 minutes to 24 hours. Red flags include focal neurologic deficits or persistent vertigo, which suggest central causes like vestibular schwannoma.
Diagnosis
Diagnosis is primarily clinical based on the AAO-HNS criteria. Audiometry is the gold standard to document low-frequency sensorineural hearing loss. An electrocochleography may show an increased summating potential to action potential ratio.
Treatment
Initial management focuses on lifestyle modification: low-sodium diet (<2g/day) and diuretics (e.g., hydrochlorothiazide/triamterene). Avoid caffeine, nicotine, and alcohol as they exacerbate symptoms. Acute vertigo is managed with meclizine or benzodiazepines; refractory cases may require intratympanic gentamicin or surgical decompression.
Prognosis
The disease is chronic and progressive, often leading to permanent sensorineural hearing loss over time. Patients require serial audiometric monitoring to track disease progression and assess for bilateral involvement.
Differential Diagnosis
Vestibular schwannoma: unilateral hearing loss with absent corneal reflex
Labyrinthitis: continuous vertigo following a viral URI
BPPV: brief, positional vertigo lasting <1 minute
Vestibular migraine: vertigo associated with headache or photophobia
Multiple sclerosis: vertigo associated with internuclear ophthalmoplegia