R Gentry Wilkerson, MD,
Clinical Assistant Professor, Department of
Emergency Medicine, State University of New York-Downstate;
Attending Physician, Department of Emergency Medicine, Kings
County Hospital
Nothing to disclose.
Coauthor(s)
Christopher I Doty, MD, FACEP,
FAAEM,
Assistant Professor of Emergency Medicine,
Residency Program Director, Department of Emergency Medicine,
Kings County Hospital Center, State University of New York
Downstate Medical Center
Nothing to disclose.
Specialty Editor(s)
Francisco Talavera, PharmD, PhD,
Senior Pharmacy Editor,
eMedicine
eMedicine Salary Employment
J Stephen Huff, MD,
Associate Professor, Emergency Medicine and
Neurology, Department of Emergency Medicine, University of
Virginia Health Sciences Center
Nothing to disclose.
John D Halamka, MD, MS,
Associate Professor of Medicine, Harvard
Medical School, Beth Israel Deaconess Medical Center; Chief
Information Officer, CareGroup Healthcare System and Harvard
Medical School; Attending Physician, Division of Emergency
Medicine, Beth Israel Deaconess Medical
Center
Nothing to disclose.
Mark S Slabinski, MD, FACEP, FAAEM,
Vice President, EMP Medical
Group
Nothing to disclose.
Chief Editor
Rick Kulkarni, MD,
Assistant Professor of Surgery, Section of
Emergency Medicine, Yale-New Haven Hospital
WebMD Salary Employment
Background
Ménière's disease, also known as idiopathic endolymphatic hydrops, is a disorder of the inner ear resulting in the clinical triad of vertigo, tinnitus and hearing loss. Prosper Ménière first proposed the disorder in 1861 in a series of papers in which he rejected the term apoplectic cerebral congestion, commonly used for vertigo, as this suggested that the pathology lay in the brain. He initially proposed the term inner ear glaucoma.[1, 2, 3, 4] Patients report an intermittent and progressive nature of the disease with a significant number having spontaneous resolution.
The exact cause of Ménière's disease is controversial. The underlying mechanism is believed to be distortion of the membranous labyrinth due to overaccumulation of endolymph.
Endolymph is produced primarily by the stria vascularis in the cochlea and also by the planum semilunatum and the dark cells in the vestibular labyrinth.[5]
Endolymphatic flow has been previously described as a "lake-river-pond" model. The endolymph flows from the endolymphatic fluid space (lake) through the vestibular aqueduct (river) to the endolymphatic sac (pond).[6] If there is obstruction, then endolymphatic hydrops will occur.
Various extrinsic mechanisms are thought to contribute to the development of endolymphatic hydrops including infection, trauma, and allergens.[7]
Recent studies have questioned whether endolymphatic hydrops is a marker of disease rather than a cause. One study looking at temporal bones found all cases of patients with Ménière’s disease had hydrops in at least one ear. Hydrops was also found in patients who exhibited no signs of the disease.[8]
Reported prevalence of Ménière's disease varies widely, from 15 per 100,000 in the United States to 157 per 100,000 in the United Kingdom.[3] This difference in prevalence based on geographic area is likely due to reporting biases and not geographic patterns of disease.
Bilateral disease is found in 10% of patients with Ménière's disease at initial diagnosis but, with disease progression, it may increase to over 40%.[9]
A genetic predisposition appears to exist.[10]
Mortality/Morbidity
Mortality is not directly associated with Ménière's disease; however, the disease is associated with drop attacks, which could lead to accidental trauma resulting in morbidity or mortality.
The main morbidity associated with Ménière's disease is the debilitating nature of vertigo and the progressive and possibly permanent loss of hearing. In a Finnish study using a questionnaire, 22% of respondents listed problems with mobility and 19% listed mental effects of their illness.[11]
Race
Ménière's disease primarily affects Caucasians,[12] although this may be due to a reporting bias.[13]
Sex
A slight increase may be noted in females compared to males (1.3:1).[3] This may be biased due to more females seeking treatment.
Age
The peak incidence of Ménière's disease is in the 40- to 60-year-old age group.[3]
Ménière's disease has been described in children as young as 4 years and in elderly persons older than 90 years.[6]
The American Academy of Otolaryngology–Head and Neck Surgery Foundation (AAO-HNS) Committee on Hearing and Equilibrium published guidelines on the clinical diagnosis of Ménière's disease in 1972, 1985, and, most recently, in 1995. According to these guidelines, Ménière's disease is defined as "recurrent, spontaneous episodic vertigo; hearing loss; aural fullness; and tinnitus. Either tinnitus or aural fullness (or both) must be present on the affected side to make the diagnosis."[14]
Vertigo
Vertigo is a subjective sensation of motion while motionless.
At least 2 definitive episodes of vertigo of at least 20 minutes duration must have occurred to make the diagnosis.[14]
Duration is usually several hours long.
Horizontal or rotatory nystagmus is always present during attacks of vertigo.[14]
Symptoms are often accompanied with nausea, vomiting, and anxiety.
Acute attacks may be accompanied with sudden falls without loss of consciousness. These are termed crises of Tumarkin or drop attacks.[15] Most studies find the incidence of drop attacks to be less than 10%. In one case series, self-reporting of drop attacks was 72% among patients with diagnosis of Ménière's disease.[16]
In patients with the symptom of vertigo, Ménière's disease is the cause in 10%.[17]
Hearing loss
Sensorineural hearing loss must be documented audiometrically in the affected ear at least once during the course of the disease.
There may be fluctuation in the degree of hearing loss superimposed on a gradual decrement in function.
Hearing loss affects low frequencies primarily.
Tinnitus and aural fullness
Tinnitus is often nonpulsatile and may be described as whistling or roaring.
It may be continuous or intermittent.
Less than one third of patients present with all components of the triad at onset. Vertigo is the most common initial component with additional components appearing after a delay of months to years.[13]
A complete neurological examination is necessary to differentiate Ménière's disease from other conditions.
Evaluation of vertigo
Dix-Hallpike positional test (also known as Nylen-Bárány maneuver) is performed.[18]
The patient is positioned in the middle of the table so that the patient's head will extend past the head of the bed when supine.
The patient is rapidly moved backward so that the head hangs over the edge and the eyes are observed for evidence of nystagmus. If there is no nystagmus observed for 20 seconds, the patient is returned to the upright position. The next step is to bring the patient rapidly to head-right supine position again looking for nystagmus. This is then repeated in the head-left supine position.
Any nystagmus or symptoms and the position of the head on eliciting these symptoms or signs should be noted.
Nystagmus typically has a latency of 2-5 seconds.
Nystagmus and vertigo associated with peripheral causes such as Ménière's disease should be fatigable and exhibit habituation.
Central lesions should have no latency and do not fatigue or habituate.
The Romberg test reveals significant instability during acute attacks.
Hennebert's sign is nystagmus owing to applied pressure in the external auditory canal.[3]
Tullio phenomenon is sound-induced vertigo, nystagmus, or both.[19] It is historically associated with syphilis but has been described in Ménière's disease.
Evaluation of hearing loss
Gross evaluation can be performed by gently rubbing the examiner's fingers near the patient's ears. The farthest distance the sound can be heard should be noted for each ear.
Rinne test performed with a 256-MHz tuning fork indicates that air conduction is greater than bone conduction.
Weber test is also performed with a 256-MHz tuning fork. Normally, sound should be heard equally on both sides. The sound is more pronounced on the affected side in middle ear disease or blockage of the external auditory canal. The sound is more pronounced on the unaffected side if there is cochlear nerve dysfunction.
Multiple causes of dysfunction of the vestibular system are known.
No single entity is known to be responsible for Ménière's disease. It is currently thought to be due to overaccumulation of endolymph in the cochlear duct.
Ménière's disease must be distinguished from other causes of endolymphatic hydrops such as post-traumatic, post-infectious, otosyphilis, and Cogan's syndrome (interstitial keratitis).[20]
An autoimmune etiology has been postulated after it was found that there was an association with presence of thyroid autoantibodies in patients with Ménière's disease.[21, 22]
A patient with a history classic for Ménière's disease does not normally need imaging studies performed. If there is concern for other processes, then MRI or CT can be obtained based on the differential diagnosis.
MRI of the brain is used to detect abnormal inner ear anatomy, masses, and lesions such as multiple sclerosis and Arnold-Chiari malformations.[23]
CT scans are used to detect dehiscence of the superior semicircular canals, widened cochlear and vestibular aqueducts, and subarachnoid hemorrhage. While CT scans are useful at imaging the anatomy of temporal bone structures, specific findings and their association with Ménière's disease is a matter of debate.[24]
Most procedures are reserved for patients who have undergone extensive workup by an otolaryngologist and in whom conservative medical management has failed.
Surgical therapy for Ménière's disease as a last resort includes the following:
Intratympanic injection of medications such as gentamicin[25, 26, 27] or steroids[28]
A Cochrane review article, recently, concluded that there is insufficient evidence to support endolymphatic sac surgery for the treatment of Ménière's disease.[30]
The diagnosis of Ménière's disease is based on history and clinical findings. Most care in the emergency department is based on symptomatic relief of the clinical findings.
Control of vertiginous sensation using the medications discussed below
IV hydration and antiemetics for nausea and vomiting
If classic findings of benign paroxysmal positional vertigo are found during Dix-Hallpike testing, then canalith-repositioning procedures may be performed.[31]
Medical treatment of Ménière's disease is aimed at symptomatic relief. The primary target is relief of vertiginous symptoms.[32] Antiemetics may be used for nausea and vomiting. A trial of intramuscular steroid injection followed by a tapering dose of oral steroids has been recommended.[31] Diuretics are often used, but their efficacy has not been established with appropriate clinical trials. Loop diuretics should be used with caution due to the potential for ototoxicity. Vasodilators, such as betahistine, have been used for the treatment of vertigo but are not FDA approved for this indication. They are thought to act by increasing circulatory flow to the cochlear stria vascularis[33] or through inhibition of vestibular nuclei activity.[34] Transtympanic steroid injection has been shown to be beneficial in controlling loss of hearing and the number of vertigo attacks.[35]
Clinical Context:
Decreases the excitability of the middle ear labyrinth and blocks conduction in the middle ear vestibular-cerebellar pathways. These effects are associated with its therapeutic effects in vertigo.
Clinical Context:
Used for treatment and prophylaxis of vestibular disorders that may cause nausea and vomiting. Through its central anticholinergic activity, it diminishes vestibular stimulation and depresses labyrinthine function.
Clinical Context:
Blocks action of acetylcholine at parasympathetic sites and antagonizes histamine and serotonin action. Transdermal scopolamine may be the most effective agent for motion sickness. Use in the treatment of vestibular neuronitis is limited by its slow onset of action.
By binding to specific receptor sites, these agents appear to potentiate the effects of gamma-aminobutyric acid (GABA) and to facilitate inhibitory GABA neurotransmission and other inhibitory transmitters. These effects may offer benefits in the treatment of vertigo and emesis.
Clinical Context:
Depresses all levels of the CNS, including limbic and reticular formation, possibly by increasing GABA activity, which is a major inhibitory neurotransmitter.
Clinical Context:
Antidopaminergic agent effective in the treatment of emesis. Blocks postsynaptic mesolimbic dopaminergic receptors in the brain and reduces stimuli to the brainstem reticular system.
Clinical Context:
Antidopaminergic drug that blocks the postsynaptic mesolimbic dopaminergic receptors. Has an anticholinergic effect and can depress the reticular activating system.
IV administration is not recommended for children.
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. No trials evaluating the efficacy of systemic steroids for the treatment of Ménière's disease have been done.[36]
Clinical Context:
Useful in the treatment of inflammatory and allergic reactions. By reversing increased capillary permeability and suppressing PMN activity, it may decrease inflammation.
Clinical Context:
Dopamine antagonist that stimulates acetylcholine release in the myenteric plexus. Acts centrally on chemoreceptor triggers in the floor of the fourth ventricle, which provides important antiemetic activity.
All patients with Ménière's disease should have rapid follow up with their primary care provider.
Some patients may require referral to an ENT specialist or neurologist.
Salt-restricted diet and the use of diuretics are often first-line therapies.[3]
A relatively new device (Meniett, Medtronic Inc; Minneapolis, Minnesota) is a positive pressure pulse generator that may increase longitudinal flow of the endolymph.[37] Use of this machine requires placement of a tympanostomy tube to allow transmission of pressures to the inner ear.
Quality evidence is lacking regarding deterrence and prevention of acute attacks of Ménière's disease; however, the following suggestions are often made:
Patient presentation and progression of disease vary widely. Periods of remission punctuated by exacerbations of symptoms are typical for the disease.[38] Some have minimal symptoms, whereas others have severe attacks. Episodes may occur as infrequently as once or twice a year or they may occur on a regular basis.
The spontaneous remission rate is high at over 50% within 2 years and over 70% after 8 years.[3]
Most of the remainder are well managed with medications.
Surgical treatment is required for 5-10% of patients.
Meniere P. Nouveaux documents relatifs aux lesions de l'oreille interne caracterisee pardes symptomes de congestion cerebrale apoplectiforme. Gaz Med de Paris. 1861;16:239.
