Continuing Education Activity

Meniere disease is an inner ear disorder characterized by tinnitus, vertigo and hearing loss. This is thought to occur due to the accumulation of endolymphatic fluid in the cochlea and the vestibular organ. This activity reviews the evaluation and management of Meniere disease and explains the role of the interprofessional team in improving care for patients with this condition.

Objectives:

• Outline the epidemiology of Meniere disease.
• Identify the use of Rinne and Weber test in the history and physical of patients with Meniere disease.
• Describe the audiometric tests used in the evaluation of Meniere disease.
• Review the importance of improving care coordination among the interprofessional team to enhance delivery of care for patients affected by Meniere disease.

Introduction

Meniere disease is a disorder of the inner ear characterized by hearing loss, tinnitus, and vertigo. In most cases, it is slowly progressive and has a significant impact on the social functioning of the individual affected.[1]

The current diagnostic criteria defined by the Barany society by Lopez-Escamez et al. can help differentiate between a probable and a definite Meniere's disease. 

Patients with a definite Meniere disease according to the Barany Society have:

1. Two or more spontaneous episodes of vertigo with each lasting 20 minutes to 12 hours

2. Audiometrically documented low- to medium- frequency sensorineural hearing loss in one ear, defining and locating to the affected ear on in at least one instance prior, during or after one of the episodes of vertigo

3. Fluctuating aural symptoms (fullness, hearing, tinnitus) located in the affected ear

4. Not better accounted for by any other vestibular diagnosis

Probable Meniere disease can include the following clinical findings:

1. Two or more episodes of dizziness or vertigo, each lasting 20 minutes to 24 hours

2. Fluctuating aural symptoms (fullness, hearing, or tinnitus) in the affected ear

3. The condition is better explained by another vestibular diagnosis[2]

Etiology

Studies of the temporal bone revealed endolymphatic accumulation in the cochlea and the vestibular organ in patients with Meniere disease. Current research links endolymphatic hydrops to a hearing loss of >40dB. Vertigo may or may not be associated.[3] Therefore endolymphatic hydrops is not entirely specific for Meniere disease and can be found in cases of idiopathic sensorineural hearing loss. 

The exact etiology of Meniere disease remains unclear. Different theories exist, but genetic and environmental factors play a role. The relation to common comorbidities remains elusive. 

Epidemiology

The prevalence of Meniere disease varies between 3.5 per 100.000 and 513 per 100.000[4][5] and occurs more often in older, white and female patients.[4][5][6]

The identification of several comorbidities which occur in an increased fashion in patients with Meniere disease gave rise to new theories about the origins of the disease.

1) Migraine: Migraine occurs more often in patients diagnosed with Meniere disease although there might be an overlap between basilar migraine wrongly diagnosed as Meniere disease.[7]

2) Autoimmune Diseases: Several autoimmune diseases are associated with Meniere disease namely rheumatoid arthritis, systemic lupus erythematosus and ankylosing spondylitis.[8]

History and Physical

At the emergency room or in the general practice the physician will differentiate between vertigo of central, peripheral and cardiovascular cause. Red flags for a central origin of vertigo, according to Harcourt et al., are neurological symptoms or signs, acute deafness, new type or onset of headache, or vertical/torsional/rotatory nystagmus.[9]

If Meniere disease is suspected, the patient should be questioned about the character of vertigo, hearing loss and earlier episodes. A full otologic history is part of the clinical investigation. 

If Meniere disease is suspected, one should perform a full otologic examination, facial nerve testing and assessment of nystagmus with Frenzel goggles, Rinne, and Weber tests. 

Rinne and Weber: Will show sensorineural hearing loss in acute Meniere disease or advanced disease.

Frenzel goggles: May show horizontal nystagmus with a fast beating component away from the affected vestibular organ in the acute setting.

Head impulse testing (HIT): In contrast to other peripheral vestibular disorders, this test has a low sensitivity in Meniere disease.[10]

Evaluation

Audiometric evaluation is mandatory in all patients with Meniere disease. Fluctuating low frequency unilateral sensorineural hearing loss is characteristic for the disease. The hearing loss can progress to all frequencies. Tinnitus is common and ipsilateral.[11]

All patients with one-sided hearing loss should undergo magnetic resonance imaging (MRI) to rule out retrocochlear pathology. In some countries a BERA (brainstem evoked response audiometry) is sufficient. There is no need to perform imaging in the acute setting but may be done within a few weeks after onset of symptoms. High resolution MRI imaging may directly show endolymphatic hydrops in the affected organs. More research is under way to show if this is of clinical use.[12][13]

Vestibular (caloric) function testing may show a significantly under-functioning affected organ in 42% to 74% and a full loss of function in 6% to 11%.[14]

Treatment / Management

Different treatment options for Meniere disease exist with substantial variability between countries. None of the treatment options cure the disease. As many treatments have a significant impact on the functioning of surrounding structures, one should start with non-invasive approaches with the fewest possible side effects and proceed to more invasive steps.

1. Sodium restriction diet: Low-level evidence suggests that restricting the sodium intake may help to prevent Meniere attacks.[9]
2. Betahistine: Substantial disagreement in the medical community about the use of betahistine exist. A Cochrane review found low-level evidence to support the use of betahistin with substantial variability between studies.[15] Medical therapy in many medical centers often starts with betahistine orally.
3. Intratympanic steroid injections may reduce the number of vertigo attacks in patients with Meniere disease.[16]
4. Intratympanic gentamycin injections: Gentamycin has strong ablative properties towards vestibular cells. Side effects are a sensorineural hearing loss because of a certain amount of toxicity towards cochlear cells.[17] 
5. Surgery with vestibular nerve section or labyrinthectomy: Nerve section is a therapeutic option in patients who failed the conservative treatment options and labyrinthectomy when surgical options failed. Labyrinthectomy leads to a complete hearing loss in the affected side.[14]

Differential Diagnosis

1. Basilar migraine: Associated with vertigo but without aural symptoms
2. Vestibular neuronitis: Associated with vertigo lasting for several days, no aural symptoms
3. Benign paroxysmal positional vertigo: Associated with vertigo related to head movements, lasting seconds to minutes, no aural symptoms
4. Medications (e.g., aminoglycosides and loop diuretics)

Prognosis

According to Perrez-Garrigues et al. the number of episodes of vertigo is higher in the first years of the disease and decrease in later years regardless whether patients receive treatment; most patients reach a "steady-state phase free of vertigo."[18]

As with vertigo, loss of hearing is highest in the early years of the disease and stabilizes in later years. Usually, there is no recovery from the hearing loss.[19]

Complications

In later stages of the pathology, patients may experience sudden unexpected drops without loss of consciousness (Tumarkin attacks).[20]

One systematic review reports bilateral involvement of the vestibular organ in up to 47% of patients within 20 years.[21][22]

Patients with Meniere disease report significantly impaired quality of life compared to healthy individuals.[23]

Consultations

Refer patients with signs suggestive for Meniere disease for otolaryngologic consultation.

Deterrence and Patient Education

Suspect Meniere disease if the patient experiences loss of hearing on one ear with attacks of vertigo which last from several minutes to several hours, and tinnitus.

Patients who experience the above seek consultation with their general practitioner or the emergency room.

The emergency room doctor will exclude vertigo secondary to disease of the heart or your vessels, or of neurologic origin, and refer the patient to an otolaryngologist for further testing and treatment.

Enhancing Healthcare Team Outcomes

The evaluation of patients with vertigo is complex, and patients often require medical attention from neurologists, otolaryngologists, and internal medicine. The Bárány Society published the current classification of Meniere disease. It is important to base the diagnosis of Meniere disease on the criteria published and mentioned in this article to warrant a uniform diagnosis especially in the presence of different international approaches to the diagnosis of patients with vertigo. (Level II)