Meniere’s Disease (Endolymphatic Hydrops)

A disorder affecting inner ear homeostasis, manifested by episodic vertigo lasting hours, fluctuating hearing loss, tinnitus and aural fullness. Rarely sudden drop attacks can develop, but when present are debilitating. The reported prevalence varies widely but probably sits around 190 per 100,000. It is almost twice as common in women than men, and most commonly presents in the age range from 20-60 years. Whilst usually affecting only one ear, approximately 15% of cases will involve both ears. It usually arises de-novo, but can occasionally develop following severe infections or trauma

Diagnosis however is often not easy, as the classic symptoms are not always present and other conditions can manifest in similar ways. Episodes of vertigo can be triggered in many ways, most commonly stress (physical or emotional) and salt in the diet. Less commonly allergies, food triggers, menstrual fluctuations amongst others can play a role.

In addition to a careful history, documentation of sensorineural (nerve) hearing loss is essential to help confirm the presence of Meniere’s Disease. The commonest cause of episodic vertigo is BPPV (benign positional paroxysmal vertigo), but this is characterised by brief head positional vertigo. The major differential diagnosis is vestibular migraine (or MAV, migraine associated vertigo) which essentially cannot directly cause hearing loss. It is not uncommon, however to have coexisting Meniere’s Disease and migrainous vertigo.

There is no definitive test to confirm the diagnosis. An electrophysiologic test called the EChOG has been used in this regard, and if abnormal, can be suggestive but in general terms is not usually helpful. According to the AAO-HNS guidelines a diagnosis of definite Meniere’s Disease is only made at autopsy. Audiology and selective balance function tests can assist in assessing severity of disease and ability to compensate to the symptoms.

Initially one of the first steps to be taken is to rule out the presence of intracranial pathology such as an Acoustic Neuroma (Vestibular Schwannoma), which requires a MRI scan of the brain.

Traditionally different phases of the disease have been described, but rarely does the disease follow a predictable pattern. In general terms though over time many people suffer from a progressive hearing loss, and eventually recurrent vertiginous attacks fade. Like the permanent nerve damage to the hearing nerve, permanent damage to the vestibular system also occurs. This can lead to permanent imbalance, dizziness, and what is often described as a ‘cotton wool’ feeling in the head. These symptoms are more pronounced if other components of the balance system are impaired. These include eyesight, joint function (including the neck), and cardiovascular health.

The overwhelming goal of long term management is to minimise the number of vertigo attacks and maintain hearing and balance function, and in the acute phase to truncate the severity of an attack.

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