Brian Kung, MD

What is Meniere’s disease?

Meniere’s disease, also known as endolymphatic hydrops, is a problem when there is a buildup of fluid in the inner ear. It was first characterized by a French ear doctor named Prosper Meniere in 1856. The main symptoms of this problems are a low frequency and sometimes fluctuating sensorineural hearing loss, tinnitus (ringing or roaring) in the ear, a feeling of fullness in the ear, and episodic spinning vertigo that usually lasts for anywhere between 15 minutes to hours at a time. It usually affects one ear, but can affect both ears in about 10-20% of cases.

Sometimes patients can just have the hearing loss and auditory symptoms (cochlear hydrops). However, classically, nearly all patients with the episodic vertigo associated with Meniere’s disease will also have the hearing loss and auditory symptoms preceding or during the episode. Most patients will recover from their episodes of vertigo but still continue to have some feelings of imbalance as well as fatigue for possibly several days after an episode. Sometimes patients do not perceive their episodes as spinning vertigo, but rather a feeling of the floor moving. Occasionally, in severe cases, patients can simply drop to the floor without loss of consciousness and without the warning signs of increasing tinnitus or fullness in the ear. These are known as “drop attacks” or “crisis of Tumarkin”.

Diagnosis of Meniere’s disease

A good patient history is very important in the diagnosis of Meniere’s disease. Classically, patients will have the symptoms of sometimes fluctuating hearing loss, fullness in the ear, tinnitus, and episodes of vertigo/dizziness.

Audiometry is important to characterize the type of hearing loss associated with Meniere’s disease. Most of the time, I am hesitant to diagnose Meniere’s disease unless I see a low frequency sensorineural hearing loss on audiometry. Sometimes, patients are between episodes in early stage Meniere’s disease and may not have any significant hearing loss. In these cases, it is often helpful to bring the patient back for an updated audiogram when the ear is feeling more fullness and/or the ear is having more tinnitus.

Electrophysiologic testing: VNG – videonystagmography, VEMP – vestibular evoked myogenic potentials, and ECOG – electrocochleography can all be used to help get more information in patients who have Meniere’s disease. I typically don’t get testing initially, but if patients are doing poorly with the treatments, and potentially need more invasive measures, I find these useful in helping to direct and choose the types of treatment.

MRI of the brain is usually recommended in patients who have sensorineural hearing loss worse in one ear, to make sure there is no other finding that can explain the hearing loss or other symptoms.

Migraine as a potential factor in Meniere’s disease

It has been observed that there is a high prevalence of classic migraine in patients with Meniere’s disease. It can be difficult to grasp as why migraine can be a big factor, but if you think of migraine not as necessarily a headache, but rather, a problem with a sensitive brain and sensitive nerves, it is easier to understand why. The inner ear is supplied by branches of cranial nerves that come from the brain. If these nerves are dysfunctional or easily irritated, the end organs of the inner ear can be affected. This can lead to extra fluid leakage in the inner ear, leading to an ear that can eventually be afflicted with Meniere’s disease. This realization has helped some of the treatments for Meniere’s disease evolve over the past 10-20 years.

https://briankungmd.com/migraine-associated-dizziness-migraine-in-otolaryngology/

Non-surgical treatment of Meniere’s disease

The first thing we usually do for patients who have Meniere’s disease is usually diet modification. Traditionally, we have placed patients on a low salt diet, but over the past 10 years or so, many ear specialists have changed their diet recommendations to more of a migraine diet, which is itself relatively low in salt. It may also help to try to reduce other known migraine triggers if possible. Sometimes we can also put patients on migraine preventative medications as well. (https://briankungmd.com/migraine-associated-dizziness-migraine-in-otolaryngology/)

Diuretics such as triamterene/hydrochlorothiazide or hydrochlorothiazide, are often prescribed in patients who have Meniere’s disease as well. The main goal of this and other treatments is to hopefully help prevent fluctuations in hearing and to prevent episodes of vertigo.

Betahistine is a non-FDA approved medication but is actually one of the most often prescribed medications for Meniere’s disease in the world. It can most often be obtained in the USA at compounding pharmacies and would usually be paid for out of pocket without insurance. As of 2022, the cost averages $50 for a 1 month supply.

Prednisone (oral steroids/glucocorticoids) – can be used at a high dose for short periods of time when patients are having attacks of vertigo or sudden decreases in the hearing. There are some side effects and some risk with taking prednisone, including but not limited to: insomnia, increased appetite, acid reflux, temporary increases in blood sugar and blood pressure, temporary immune system suppression, and extremely rarely, avascular necrosis of the hip (which would require hip replacement). Avascular necrosis of the hip is actually more common with long term steroid use. If steroids are effective, it is preferable to avoid use of oral steroids more than 3 courses per year.

Fludrocortisone – this is a mineralocorticoid. It has been shown to potentially be effective in Meniere’s patients even though it may cause the body to retain salt. It can be potentially prescribed long term.

Anti-secretory factor – there was a paper published in 2020 regarding the use of specially processed cereal that can induce the expression of anti-secretory factor. This is a protein that may help control ion and fluid balance across cell membranes. The paper found that there was a significant reduction in vertigo episodes in patients who tried this cereal vs intravenous glycerol and steroid treatments.

https://pubmed.ncbi.nlm.nih.gov/32829060/

https://orpharma.myshopify.com/collections/frontpage

Surgical treatment for Meniere’s disease

Intratympanic steroid injection – this is a well-tolerated in-office procedure during which a steroid called dexamethasone is injected into the middle ear. The steroid then diffuses into the inner ear via the round window membrane and/or the oval window and can help reduce Meniere’s attacks. It is not absorbed into the body nearly as much as oral steroids, so it can be repeated multiple times a year. The risk is very small – a miniscule risk of an eardrum perforation, and a risk that the treatment is not effective.

Intratympanic gentamicin injection – this procedure is also done in the office, but is reserved for refractory cases of Meniere’s disease that do not improve with medical therapy and non-destructive therapy such as steroid injections and even endolymphatic sac surgery. An antibiotic called gentamicin is injected into the middle ear and is meant to destroy the vestibular labyrinth. This can greatly help with patients who are having severe frequent attacks of vertigo. However, there is some risk to the hearing and it is meant to cause the labyrinth to not function well. So patients may get a slower vertigo that lasts a few days after an injection. The injection can be repeated usually a month later. If enough labyrinthine weakness results from this, there is an adjustment period for the patient to help fully regain balance function. Also, if the patient develops Meniere’s disease in the opposite ear, the options for treatment in the opposite ear are less.

Endolymphatic sac surgery – this procedure is done in the operating room. It is considered nondestructive of the labyrinth, but is not without some risk. The goal of surgery is to take the bone off of the endolymphatic sac, to place a silicone shunt into the sac, and sometimes even block the endolymphatic duct. It can be very effective in some patients to help decrease frequency and severity of episodes of vertigo. There is a small risk to the hearing and some risk to the balance organ itself, as well as other standard risks of ear surgery. There is also a small risk of a cerebrospinal fluid (CSF) leak as the endolymphatic sac and duct lie on the dura of the posterior fossa. If a CSF leak occurs, it should be able to be remedied during the surgery.

Transmastoid labyrinthectomy – this is also done in the operating room and reserved for refractory cases of Meniere’s disease, optimally in a patient with very poor hearing in the affected ear. The vestibular labyrinth is drilled away during surgery. This will typically result in postoperative vertigo which will require some recovery period (typically about 6 weeks with vestibular rehabilitation therapy), and the remaining hearing in the affected ear will be permanently lost. The surgery would virtually guarantee that episodic vertigo from the affected ear would no longer happen. However, as this procedure is definitively destructive, it would leave for fewer available treatment options should Meniere’s disease develop in the opposite ear later on. It also entails other standard risks of ear surgery. It is and should be pretty rarely performed.

Vestibular nerve section – this is also done in the operating room and reserved for refractory cases of Meniere’s disease. It can be done via a retrosigmoid craniotomy or transmastoid via a retrolabyrinthine approach. Both would involve opening the dura to access the vestibular nerves. The superior and inferior vestibular nerves are located and cut so that the diseased labyrinth can no longer send signals to the brain and cause vertigo. Optimally, hearing is preserved, although there is still risk to the hearing. This surgery should also virtually guarantee that episodic vertigo from the affected ear would no longer happen. There is an additional risk of meningitis and a higher risk of CSF leak with this procedure. This and other surgically destructive procedures are now very rarely performed mostly because of the availability and ease of gentamicin injections.

More information

Please see Dr. Teixido’s website for more helpful information regarding Meniere’s disease: https://entad.org/wp-content/uploads/2021/12/MENIERE_PDF.pdf

En espanol: https://entad.org/wp-content/uploads/2021/12/MENIERES-DISEASE-Espan%CC%83ol-1.pdf