- Clinical science
Ménière disease is an idiopathic condition affecting the inner ear, in which impaired resorption of endolymphatic fluid causes it to accumulate in the membranous labyrinth (endolymphatic hydrops). The disease usually manifests episodically with a triad of symptoms: vertigo, hearing loss, and tinnitus. Episodes may last from minutes to hours and decrease in frequency as patients age. Hearing loss tends to worsen with every episode. Diagnostic tests show low-frequency hearing loss, typically with horizontal nystagmus. Acute episodes are treated with bed rest and vestibular suppressants, while long-term prevention therapy focuses on lifestyle changes such as adopting a low-sodium diet and reducing both alcohol consumption and stress. For patients who do not respond to these measures, interventional and/or surgical treatment may be necessary to shut down the vestibular organ.
All patients with Ménière disease have impaired endolymph resorption that results in endolymph hydrops; however, not all patients with endolymphatic hydrops have symptoms of Ménière disease. The cause of impaired resorption is unknown. There are currently two main theories about why some patients develop symptoms:
- Endolymph hydrops: accumulation of fluid in the endolymphatic sac.
- Rupture theory: fluid accumulation in the endolymphatic sac → tear in the Reissner membrane → increased perilymphatic potassium → depolarization of the afferent acoustic nerve fibers → symptom onset
- Compression theory: impaired endolymph resorption → compression of the semicircular canals → symptom onset
- Recurrent episodes of acute, unilateral symptoms that last from minutes to hours.
- Frequency of these episodes decreases as patients age.
- In 30% of patients, the disease eventually affects both sides.
- Ménière triad
- Nausea and vomiting
- Ear fullness
- Horizontal nystagmus
- Diagnostic criteria (American Academy of Otolaryngology) 
- Evaluation of hearing loss
- Vestibular evaluation: declining peripheral vestibular function in the affected ear
- Imaging: MRI or CT can rule out CNS lesions (e.g., tumors, aneurysms or stenosis of the posterior circulation, Arnold-Chiari malformations, multiple sclerosis).
- Consider serological screening for neurosyphilis.
|Central vertigo||Peripheral vertigo|
|Site of lesion|
|Ataxia|| || |
|Vertigo|| || |
|Nystagmus|| || |
|Hearing loss and/or tinnitus|| || |
|Other neurological findings|| || |
The differential diagnoses listed here are not exhaustive.
- Lifestyle adjustments
- Avoid dietary and environmental triggers (caffeine, alcohol, and stress).
- Low-sodium diet
- Vestibular rehabilitation therapy in patients with persistent disequilibrium symptoms between attacks
- Bed rest
- Medical therapy
- Lifestyle adjustments
- Intratympanic gentamicin injection: repeated application of gentamicin to the middle ear cavity through a small incision in the eardrum (paracentesis)
- Sacculotomy: The endolymphatic sac and duct (part of the vestibular organ) are surgically exposed in order to promote drainage of endolymph.
- Vestibular neurectomy: Surgical lysis of the vestibular bundle entering the internal auditory canal. In some cases, the procedure is associated with hearing loss.