Meniere’s Disease. Causes, Diagnosing and Treatment

Meniere’s Disease is also called Idiopathic Endolymphatic Hydrops. It is a disorder of the inner ear that causes vertigo (usually a spinning sensation that can be violent), ringing in the ears (tinnitus or low tone roaring), a feeling of fullness in the ear (aural fullness), and fluctuating hearing loss. It is almost always only in one ear, and it is typically episodic.

Endolymphatic hydrops is a technical term that means there is increased hydraulic pressure within the inner ear fluids. It is important to understand because Meniere’s disease occurs secondary to various processes interfering with the inner ear fluid or endolymph.

What is the difference between vertigo and Meniere’s Disease, or is there?

The distinction is nomenclature or naming. It is analogous to Bell palsy. When the cause of facial paralysis is known, Bell palsy is not the diagnosis.

If the cause of vertigo is known, Meniere’s disease is not the diagnosis. Furthermore, Meniere’s disease can be caused by a specific reason or be of unknown etiology.

Either way, no matter how you name a disease, the evaluation and management of vertigo and dizziness is challenging, with many nuances. If there is any good news by being diagnosed with Meniere’s disease, there are not only ways to treat it, but also to prevent it.

What causes Meniere’s Disease?

The short answer is that the cause is by definition, idiopathic. In other words, no one really knows. The elevated inner ear fluid pressure can be caused by several things

• Metabolic disturbances.
• Hormonal imbalances.
• Trauma.
• Infections (e.g., a viral infection or something rarer such otosyphilis and Cogan’s syndrome or interstitial keratitis).
• Autoimmune diseases (systemic lupus or rheumatoid arthritis).
• Allergy, particularly food triggers (alleged in many patients).

What defines Meniere’s Disease?

The American Academy of Otolaryngology–Head and Neck Surgery Foundation (AAO-HNS) Committee on Hearing and Equilibrium published guidelines on the clinical diagnosis of Meniere’s disease in 1972, 1985, and 1995. According to these guidelines, Meniere’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.”1

Some key features of patients who truly suffer from Meniere’s disease include:

• The sensation of motion while motionless.
• Eye movements call nystagmus which are always present during the vertiginous attacks.
• Acute attacks of vertigo may be accompanied by sudden falls without loss of consciousness. These are called drop attacks or crisis of Tumarkin, and they are real and frightening. Some people can predict these crises according to dietary triggers, the menstrual cycle, or psychological stress.
• Post vertiginous attack fatigue, unsteadiness, and nausea.
• Hearing loss must be documented in the affected ear for the diagnosis of Meniere’s disease.
• Tinnitus can be described as whistling or a low-tone roar of an ocean which usually corresponds to the vertiginous attack.

What will my doctor look for to determine a diagnosis of Meniere’s Disease?

Often, when the patient is asymptomatic, the physical examination is normal. During a vertiginous attack, the patient can present under significant stress. In some cases, there is nausea, vomiting, rapid eye movements, sweating, pale skin, and even their blood pressure, heart rate and respiration can be elevated. These patients sometimes need hospital admission.

When the doctor looks in the patient’s ear and there is a suspicion of Meniere’s disease, the eardrum can look normal. However, by using a pneumo-otoscope, or instilling a slight puff of air, the vertigo and eye movements can worsen.

There are tests the doctor can perform to assess the patient's ability to walk or maintain balance called the Romberg and Fukuda marching tests that offer clues. A hearing test is always necessary to document the episodic, temporary hearing loss.

A complete neurological examination is necessary to rule out other potential causes of the vertigo. One important test is the Dix-Hallpike positional test (also known as the Nylen-Barany maneuver). This test confirms benign positional vertigo (BPPV) which can coexist with Meniere’s disease. It is done by a physician using head movements and sitting the patient up and down.

What can be done to treat Meniere’s Disease?

First, medical treatment is aimed at symptomatic relief, particularly managing the acute vertigo. Some patients require intravenous or intramuscular medications such as diazepam (valium) to suppress the vestibular system and provide anti nausea effects. Steroids and intravenous fluids are also helpful.

Usually, in between vertiginous episodes, patients are given vestibular suppressants and anti nausea medications to be used as needed, many of which are over the counter. Of note, use of these medications long term is not recommended, however, since they can hinder compensatory mechanisms after a patient has a vertiginous episode. Also, the medications tend to not work after a while due to a phenomenon called tachyphylaxis or reaching a tolerance.

Most Meniere’s patients adjust to lifestyle and dietary changes which can be remarkably effective in preventing vertiginous attacks. These recommendations include:

• Low salt, chocolate, and caffeine diets.
• Stopping smoking.
• A trial of a diuretic (hydrochlorothiazide/triamterene) for periods of 3 months (thought to maintain the inner ear fluid equilibrium).
• Vestibular rehabilitation exercises (there is no activity restriction required).

What about surgery?

Even after decades of various treatments for Meniere’s disease, surgical options remain controversial. There are two main approaches: destructive and nondestructive surgical procedures.

Destructive procedures are a last resort. Success depends on the adequacy of the opposite ear function. The procedure is called a labyrinthectomy and both hearing and vestibular function are typically irreversibly lost.

Nondestructive procedures aim to improve the state of functioning of the vestibular apparatus. They include:

• Endolymphatic sac decompression or shunt placement.
• Vestibular nerve section.
• Intratympanic injection of medications such as gentamicin antibiotic or steroids.

There is a new innovation in the treatment of Meniere’s Disease called the Meniett device. It offers hope to patients who are long term sufferers. It requires insertion of an ear tube by an ENT doctor which can usually be done in an office setting.

The Meniett device delivers pulses of pressure to the inner ear through the ear tube. There is no medication required. No one knows why this device is effective, but it can be if used on a daily basis. It offers hope for a complex and frustrating disease.