John G. Oas, MD
DESCRIPTION 
Ménière syndrome (MS) is a peripheral vestibular disorder characterized by symptoms and signs without a defined pathogenesis. Symptoms of otogenic distress include unilateral hearing loss with tinnitus and attacks of objective vertigo. Signs include documented fluctuation (loss – recovery – loss) of low-frequency sensorineural (SN) hearing with greatly diminished speech recognition and eventually a unilateral loss of peripheral vestibular function ipsilateral to the hearing loss.
EPIDEMIOLOGY
- Age: Mean age of onset is 42–46 with a wide distribution rarely in children or elderly
- Sex: No preponderance
Incidence
15 cases per 100,000
Prevalence
200 cases per 100,000
RISK FACTORS
- Migraine
- Trauma (labyrinthine concussion, acoustic trauma, temporal bone fracture)
- Systemic illness (autoimmune inner ear disease, Cogan's syndrome, polyarteritis nodosa)
- Infection (syphilis, alpha-human herpesviridae)
- Neoplasia (macroglobulinemia, leukemia, von Hippel–Lindau disease)
Pregnancy Considerations
Pregnancy is neither protective nor preventive. The use of daily diuretics in pregnancy is not advised (Category C) unless the disorder is poorly controlled and use of the medication has provided benefit in the past.
Genetics
Familial clusters have been described yet exclusion from migraine was not performed.
PATHOPHYSIOLOGY
No definitive pathophysiology
ETIOLOGY
Described by Prosper Ménière in 1861, no defined etiology has emerged1; thus, its designation as a syndrome rather than a disease. The syndrome may have several separate etiologies, with symptoms and signs merely the expression of labyrinthine distress and destruction. Leading candidates for pathogenesis include alpha-human herpesviridae infection of the temporal bone, labyrinthine trauma, and autoimmune disorders of the endolymphatic sac. Syphilis has been implicated as a possible cause. Rarely an endolymphatic sac tumor (papillotubular neoplasia) causes Ménière syndrome.
[Outline]
HISTORY
According to the 1995 guidelines4, the three major symptoms are vertigo, hearing loss, and tinnitus. Vertigo criteria include recurrent, well-defined episodes of spinning or rotation, lasting from 20 minutes to 24 hours, accompanied by nystagmus during the vertigo attacks, commonly accompanied by nausea and vomiting but no neurologic symptoms. Hearing loss criteria include audiometrically documented SN hearing deficits; must fluctuate; progress over time. Tinnitus criteria include variable, low pitched, and louder during attacks, unilateral on the affected side, subjective type (examiner cannot hear the sound). Diagnosis can be specified from possible to certain: Possible – vertigo without hearing loss; or SN hearing loss, fluctuating or fixed, with disequilibrium without vertigo. Probable – one definitive episode of vertigo with hearing loss on at least one occasion with tinnitus or aural fullness in the designated ear. Definite – two or more episodes of vertigo with hearing loss on at least one occasion with tinnitus or aural fullness in the designated ear. Certain – meeting definite specification with histopathology confirmation. Proper diagnosis requires careful exclusion of common competing etiologies, especially vestibular migraine (migraine-associated vertigo, migrainous vertigo, migraine vestibulopathy) and recurrent vestibular ganglionitis (viral labyrinthitis, vestibular neuronitis). The diagnosis of Ménière syndrome becomes more defined as the disorder progresses to produce losses of hearing and peripheral vestibular function. Early, when destruction to the labyrinth is minor, spontaneous remission is common. The simultaneous appearance of bilateral signs and symptoms with rapid deterioration in bilateral hearing loss and tinnitus strongly implicates a diagnosis of autoimmune inner ear disease rather than Ménière syndrome. Lack of hearing loss despite multiple recurrent attacks of vertigo suggests recurrent vestibular ganglionitis, unless temporally associated with migraine headache, meeting International Headache Society classification, where a diagnosis of vestibular migraine is more likely. Tumarkin's otolithic catastrophes (rare in definite Ménière syndrome) are extremely brief attacks that throw the patient downward without any loss of consciousness and without the perception of falling. Lermoyez's phenomenon is a transient recovery of hearing loss after a vertigo event.
PHYSICAL EXAM
Generally normal except during an attack; later in course may have hearing loss.
DIAGNOSTIC TESTS AND INTERPRETATION
Lab
Initial Lab Tests
None. Exclusionary tests for autoimmune disease and syphilis are advised prior to considering vestibular ablative treatment.
Imaging
Initial Approach
None. Both high-resolution (3T or higher) MRI of brain and internal auditory canals with or without contrast and high-resolution thin-slice CT imaging of the temporal bone are advised to exclude structural pathology.
Diagnostic Procedures/Other
- Audiogram (pure tone threshold audiometry, speech recognition or word discrimination score): When repeated many times during the course of the disease, this test provides the most diagnostic certainty. A fluctuating SN hearing loss (pure tone threshold reduction of more than 15 dB) occurs in the lowest frequencies (250–2,000 Hz) early in the course of the disorder. Also reductions in speech or word recognition scores (% words correctly heard) are worse than predicted by the degree of tone threshold reduction. This loss stops fluctuating with progression to profound SN hearing loss across all frequencies late in the course of the disorder.
- Electrocochleography (ECochG): An audiometric electrophysiological test that is a poor screening procedure because of less than optimal sensitivity and selectivity. An elevated ratio of the summating potential to action potential of the auditory evoked response is considered positive. This is helpful in situations where other tests have failed to clarify the diagnosis.
- Vestibular testing (caloric tests, videonystagmography, rotational chair tests): During a vertigo event, videonystagmography can document the nystagmus necessary for the diagnosis, but it is difficult to arrange such testing on demand in a sickened patient. Interictally, videonystagmography can identify the nystagmus of either a recovering peripheral vestibular loss (fast component directed toward the ear involved) or an uncompensated peripheral loss (fast component directed away from the ear involved). A peripheral loss can be documented by caloric testing (alternating bithermal water irrigations) later in the course. Rotational chair testing must support the videonystagmography findings or caloric testing to confirm a unilateral loss of peripheral vestibular function. It is most helpful to identify bilateral peripheral losses (relative contraindication) prior to vestibular ablative treatment measures.
Pathological Findings
Endolymphatic hydrops should be considered only as a histologic marker rather than a cause.
DIFFERENTIAL DIAGNOSIS
Early in the course of Ménière syndrome, when episodic vertigo may be the only symptom, many inner ear and neurologic disorders can also cause episodic vertigo, including vestibular migraine2, recurrent vestibular ganglionitis3, benign paroxysmal positional vertigo, sporadic and familial episodic ataxia, vertebrobasilar ischemic disease, eighth nerve root entry zone neurovascular compression, symptomatic Arnold–Chiari malformation, hyperviscosity syndromes, endolymphatic sac tumor, brainstem neoplasia, epilepsy, and otosyphilis. Tumarkin's otolithic catastrophes can be confused with myoclonic-astatic epilepsy (rare in adults).
[Outline]
MEDICATION
First Line
- All medications are used off-label (FDA specifications) and are either empirical or symptomatic. None are curative.
- Symptomatic control of the vertigo event: Sublingual Ativan (generic formulations of lorazepam are not useful for SL usage) at the start of each episode allows for the rapid parenteral absorption of a benzodiazepine and reduction in the intensity of the vertigo event. The dose should be titrated to the lowest effective dose.
- Symptomatic control of nausea and vomiting: Rectal administration of an antiemetic medication (promethazine, prochlorperazine) should be used to prevent the risks of esophageal rupture, dehydration, and electrolyte depletion from protracted vomiting. The dose should be titrated until the lowest effective dose is established.
- Diuretics: This approach as medical treatment is universal. Avoid loop diuretics (furosemide, etc.) due to ototoxicity risk to the opposite ear.
- Antivirals: Treatment with daily doses of antiviral medication with proven efficacy against the alpha-human herpesviridae, implicated in the pathogenesis of some cases with Ménière syndrome, is not universal. Acyclovir 400 mg twice daily, famciclovir 250 mg twice daily, or valacyclovir (prodrug of acyclovir) 1,000 mg once daily are taken for 1 year in the same manner as used in genital herpes simplex virus suppression.
Second Line
Betahistine: Of varying efficacy with conflicting results from multiple trials conducted worldwide. It is not a FDA-approved pharmaceutical and must be provided by a compounding pharmacy in the US. Dosing should be titrated over several days from 16 to 48 mg every 6 hours. If no effect is seen within a couple of weeks, benefit is unlikely.
ADDITIONAL TREATMENT
General Measures
Vestibular rehabilitation physical therapy is advised whenever an uncompensated peripheral vestibular loss develops or after vestibular ablative treatment.
Additional Therapies
See Complementary and Alternative Additional Therapies.
COMPLEMENTARY AND ALTERNATIVE THERAPIES
A 1,000 mg daily maximum sodium-restricted diet prior to considering vestibular ablative treatment measures. Allergy desensitization and dietary restriction from allergens in cases with comorbid allergies.
SURGERY/OTHER PROCEDURES
- Endolymphatic sac decompression/shunting: This surgical approach is often considered in medically refractive cases where vestibular ablative treatment measures are contraindicated or not contemplated. There is no consensus among neurotological surgeons regarding its use in the control of episodic vertigo.
- Intratympanic gentamicin: This intervention requires a myringotomy with local anesthesia. Several protocols have shown good (80–95%) efficacy in vertigo cessation. Hearing loss is a complication and increases significantly with the number and frequency of the dosage, especially in older patients. This is the most popular among the vestibular ablative treatment measures.
- Selective vestibular nerve section: Used in cases where intratympanic gentamicin has failed to control the episodic vertigo and hearing preservation is desired. This procedure has the highest efficacy of all vestibular ablative treatments with the lowest risk to hearing loss as a near term result of the intervention. However, it cannot control the process of eventual hearing destruction and chronic tinnitus. The procedure results in a permanent peripheral vestibular loss.
- Transmastoid labyrinthectomy: Used when intratympanic gentamicin has failed to control episodic vertigo. Craniotomy is not required, and there are fewer perioperative complications. Both hearing and vestibular function are irreparably lost, and tinnitus does not resolve with this procedure.
IN-PATIENT CONSIDERATIONS
Admission Criteria
Hospital admission is advised when the course suggests alternative diagnoses, for control of autonomic symptoms, for rehydration or electrolyte repletion, or when surgical vestibular ablative treatment requires medical stabilization.
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FOLLOW-UP RECOMMENDATIONS
Patient Monitoring
Serial audiograms document fluctuations in hearing essential to establish the diagnosis. Vertigo diaries to assess symptomatic management and determine the timing of vestibular ablative treatment. Regular office visits to monitor the effects of medical management and to assess the development of uncompensated vestibular peripheral loss.
DIET
Nutritional counseling for a low-sodium diet.
PATIENT EDUCATION
Emphasize lack of pathophysiology to confirm the cause and the absence of a cure, and provide hope that remissions are common.
PROGNOSIS
Remission of varying duration is common in most cases. Atypical presentations can evolve into more typical symptoms and signs over time. Cure is not possible, and treatments do not alter the highly variable progression to varying degrees of permanent hearing loss, constant tinnitus, and peripheral vestibular loss. The disease may progress to involve both ears. Vestibular ablative treatments can achieve up to 95% cessation of vertigo events but usually result in a permanent peripheral vestibular loss and do not prevent progression of hearing loss nor resolve the tinnitus.
COMPLICATIONS
Hearing loss
[Outline]
ICD9
386.00 Ménière's disease, unspecified
Treatment is symptomatic, not curative, and mostly empirical. Ultimately vestibular ablative treatment measures may be needed to control the vertigo.