What is Meniere’s Disease (MD)?
Meniere’s Disease (also called Idiopathic Endolymphatic Hydrops) is a condition characterized by episodes of vertigo associated with fluctuating hearing loss, aural fullness, and tinnitus (ringing in the ears) often described as a harsh roaring machinery-like sound. The vertigo often builds over minutes to hours and can be associated with nausea and vomiting. During these attacks, you may find yourself unable to perform daily activities and may find benefit from lying down until the attacks subside. MD is caused by a disruption of the fluid (endolymph) balance of the inner ear. An overproduction of endolymph, or an obstruction of outflow, increases the pressure in the vestibule and cochlea, your balance and hearing centers respectively, and is called endolymphatic hydrops. This swelling of the inner ear damages tiny sensory hairs, resulting in the typical symptoms of MD. Risk factors for MD include genetic and vascular abnormalities, infections, metabolic factors, allergies, and autoimmune disorders.1 However, in the vast majority of cases the underlying cause remains unknown. The condition most commonly affects a single ear, but can become bilateral with disease progression.
How is Meniere’s Disease Diagnosed?
How is Meniere’s Disease Treated?
There is currently no cure for MD, but long term management includes the following goals:
Reducing the frequency and duration of attacks.
Minimizing symptoms during an acute attack.
Preventing progression of the disease.
Your long term management may also include such lifestyle modifications as the avoidance of dietary triggers (i.e. caffeine, alcohol, nicotine, MSG) and a salt restricted diet. Lowered salt intake has been shown to reduce sodium fluctuations in the bloodstream, reducing the risk of developing endolymphatic hydrops. If your symptoms are not managed through lifestyle changes alone, your provider may recommend pharmacologic therapy. The first line of pharmacologic therapy will typically begin with a diuretic medication such as Hydrochlorothiazide, Dyazide, or Acetazolamide to reduce the fluid pressure in the inner ear. Betahistine is another medication widely used in Europe and the UK which is believed to act on histamine receptors in the central nervous system.
In our clinic, we treat Meniere’s disease patients concomitantly with migraine therapy. Prosper Meniere, whom the disease is named after proposed that Meniere’s disease was similar to migraine phenomena. There are studies that show a relationship between the two diseases. Radtke et al found that 45% of patients with Meniere’s disease have at least one component of migraine thus suggesting a relationship between the two conditions. In the appropriate patient, we use a migraine preventative medication such as Topamax. The goal of this medication is to decrease the frequency of Meniere’s attacks. Meniere’s disease triggers are very similar to migraine triggers and so a migraine may trigger Meniere’s disease or vice versa. During acute attacks, patients are treated with a migraine abortive medication, such as a triptan. These medication therapies in conjunction with low sodium diet and diuretic medication are ways to avoid surgical treatment.
An understanding of MD will help you to play an active role in the management of your condition, and we encourage you to contact our office in the case of an acute attack.
The following paper details the link between Meniere's disease and migraine:
1. Hamid, MA. Meniere’s Disease. Practical Neurology. 2009; 9:157-162.
2. Andrea Radtke, Hannelore Neuhauser, Michael von Brevern, Tilman Hottenrott and Thomas Lempert. Vestibular migraine - validity of clinical diagnostic criteria. Cephalalgia (2011) 31:906-913.
The diagnosis of MD is largely clinical, based on history and audiometry findings (hearing tests). Serial audiograms over time are crucial in tracking the type, degree and variation of hearing loss, providing useful information on disease progression. The clinician may also look for abnormal eye movements called nystagmus, which are recorded in our office with infrared video oculography during vertigo attacks. This relies upon the reflex pathway from the hair cells in the inner ear and the muscles around the eyes, which can cause nystagmus during an acute attack. The information from this study can also help to rule out other causes of vertigo such as benign paroxysmal positional vertigo. In addition, you may require vestibular tests, blood work, and imaging including MRI and CT scans to rule out other potential causes of vertigo and hearing loss.
Prebysacusis refers to age related hearing loss that gradually affects most individuals starting in their 60's. About 30-35% of people aged 65 or older have age related hearing loss. In this audiogram, the same individual was given a hearing test at age 66 and again at age 82. In prebycusis, it is common for hearing to decline most in the higher frequencies.
This audiogram depicts normal hearing function in both the right and left ear and all frequencies.
This is audiogram from a patient's suffering from a Meniere's attack on the left ear. During the Meniere's episode, low frequency hearing will be affected and the patient may also reports symptoms of tinnitus or aural fullness. An audiogram taken after an episode may show improvement in hearing function, however over time the patient's hearing would be expected to delcine.
In the late stages of Meniere's disease, the affected ear will likely have permanent sensorineural hearing loss due to repetitive damage.
This audiogram represents possible high frequent hearing loss that may be seen in a migraine variant syndrome. It is episodic and hearing should resume to normal after the migraine resolves.