- Clinical science
Meniere disease (endolymph hydrops) is a disorder of the inner ear caused by impaired endolymph resorption. The exact etiology of endolymph malabsorption is unknown but viral infections, autoimmunity, and allergies are thought to play a role. Meniere disease most commonly manifests in adults between 40–50 years of age. Clinical manifestations include recurrent episodes of peripheral vertigo, fluctuating unilateral sensorineural hearing loss (SNHL), and unilateral tinnitus (referred to as the Meniere triad); or horizontal rotatory nystagmus may also be present. The episodes fluctuate in severity, typically lasting from 20 minutes to 12 hours. The diagnosis is based on characteristic clinical features and low to mid-frequency SNHL on audiometry. Treatment is symptomatic. Vestibular suppressants (e.g., benzodiazepines and first-generation antihistamines) may be used during acute vertigo attacks. Lifestyle modifications (e.g., avoidance of allergens, low-sodium diet) and vestibular rehabilitation therapy can help minimize the risk of recurrence. Diuretics should be considered in patients with frequent attacks. Interventional therapy (e.g., chemical vestibular ablation with intratympanic gentamicin, intratympanic steroids) or surgical vestibular ablation (e.g., labyrinthectomy, vestibular neurectomy) is reserved for patients with intractable symptoms that significantly impact their quality of life.
All patients with Meniere disease have impaired endolymph resorption that results in endolymph hydrops; however, not all patients with endolymphatic hydrops have symptoms of Meniere 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
Meniere disease characteristically manifests as recurrent episodes of acute, unilateral symptoms that last from minutes to hours. 
Meniere triad 
- Peripheral vertigo
- Asymmetric fluctuating sensorineural hearing loss (SNHL)
- Additional symptoms: may be present in some patients
- Triggers: Definitive triggers of Meniere disease are not known to exist 
- Episodes fluctuate in severity and typically last from 20 minutes to 12 hours
- Periods of remission between attacks vary from months to years.
- In 10–25% of patients, the disease becomes bilateral. 
Lermoyez syndrome 
- A variant of Meniere disease in which hearing improves during or immediately after the episode of vertigo
- Thought to be caused by the movement of endolymph from the cochlea towards the semicircular canals, which results in:
Drop attacks (Tumarkin otolithic crisis) 
- An uncommon feature that may occur in advanced Meniere disease, characterized by suddenly falling to the ground without warning
- There is no loss of consciousness.
- Falls may have severe to life-threatening adverse consequences (e.g., TBI, hip fractures).
- Difficult to treat but may resolve spontaneously
Meniere disease is diagnosed based on the characteristic clinical features and demonstrable low- to mid-frequency SNHL on audiometry. Specialized tests (e.g., vestibular function testing, electrocochleography) are reserved for patients with atypical symptoms or before attempting ablative therapies. should be considered if is suspected. 
- Definite Meniere disease: must include all of the following criteria
- Probable Meniere disease: Patients that meet all of the above criteria but do not demonstrable hearing loss on audiometry.
- All patients with suspected Meniere disease
- Before and after ablative therapy
- Modalities and characteristic findings: Subjective audiometry should always be performed in both ears.
Vestibular function tests and electrophysiologic testing 
Not routinely recommended
- Atypical symptoms
- Identifying the affected ear
- Before vestibular ablative procedures.
Modalities and supportive findings
-  : decreased vestibular function in the affected ear
Electrocochleography: measures the summating potential (SP) generated by cochlear hair cells and the action potential (AP) of the cochlear nerve in response to acoustic stimulation
- Supportive findings: elevated SP/AP ratio
- Normal vestibular function testing or electrocochleography does not rule out Meniere disease.
Imaging studies are not routinely indicated in patients with suspected Meniere disease.
- Preferred modality: MRI internal auditory canal and posterior fossa (without and with IV contrast)
- Supportive findings: endolymphatic space distention (endolymphatic hydrops) in the cochlea and vestibule 
- Otological disorders (see also “ ”)
- Neurologic disorders (see “”)
- Otosyphilis 
Always consider vestibular migraine as a differential diagnosis of Meniere disease.
The differential diagnoses listed here are not exhaustive.
There is currently no definitive cure for Meniere disease. Treatment is directed toward symptomatic management and prevention of recurrence. Interventional therapy or surgery is reserved for patients with intractable symptoms that significantly hinder their quality of life. 
Acute therapy 
- Vestibular suppressants: drugs that suppress the effects of vestibular dysfunction, such as vertigo, nystagmus, and nausea
- Adjunctive therapy
Recurrence prevention 
- Lifestyle modifications 
- Vestibular rehabilitation and physical therapy
Maintenance therapy 
Patients with frequently recurring episodes of Meniere disease may be considered for chronic pharmacotherapy.
- Diuretics 
- Other agents: : systemic steroids, antivirals, benzodiazepines, betahistine 
Monitoring and duration
- Patients should be monitored frequently for:
- Symptomatic improvement
- Drug intolerance and side effects (e.g., measurement of BMP in patients on diuretics)
- Medications can be tapered off once symptoms subside.
- Duration of therapy has not been established.
- Patients should be monitored frequently for:
Interventional therapy 
- Chemical ablation with intratympanic gentamicin (injection of gentamicin into the middle ear through the tympanic membrane)
- Intratympanic steroid therapy (injection of methylprednisolone or dexamethasone into the middle ear through the tympanic membrane)
Positive pressure pulse generator devices (e.g., Meniett device) are no longer recommended for Meniere disease.
Surgical intervention 
- Labyrinthectomy (Hearing-sacrificing surgery): destruction and removal of the labyrinth through the mastoid
- Vestibular neurectomy (Hearing preservation surgery): selective transection of the vestibular nerve within the middle cranial fossa via a craniotomy
- Endolymph drainage procedures (e.g., sacculotomy , cochleosacculotomy , endolymph sac decompression ): No longer recommended as they are of doubtful clinical benefit