menieres-300x300Wrapping up our Meniere’s Update series, we have a contribution from Philippa Thomson. Below, I have pasted her biography from the Amazon webpage. I have read her book A Hole in My Life, and we have become email buddies over the past few months. She is smart, passionate, and has a heck of a story to tell. This article “ Meniere’s Syndrome or Meniere’s disease”, recently won an award from the Meniere’s Society for Scientific Essay Competition.

 

From Amazon.com

“Philippa Thomson is a researcher, editor and writer. During a long and successful career in illustrated book publishing, she held the post of picture manager on Macmillan’s multi-volume Dictionary of Art, and was picture editor at Phaidon Press. She has worked on a freelance basis since 2000. She put full-time work on hold to raise her children, as the youngest was born severely disabled as a result of a rare chromosome disorder. She has edited the books of others, but the memoir A Hole in My life – Battling Chronic Dizziness is the first book of her own. The balance disorder SCDS has had a major impact on her life, and she has been researching the subject since 2006. Find her profile on LinkedIn, or connect with her on Twitter at @PhilippaThomson”

Here is Philippa’s award winning essay:

Ménières’s Syndrome or Ménière’s Disease?

“You are correct that Ménière’s disease is over diagnosed. Many physicians diagnose it when they see any vertigo they do not understand.” The words of Dr Carol Foster, Associate Professor in the department of Otolaryngology at the University of Colorado, written in an email to me this week. She has researched the condition in depth (Foster 2015).

The classic symptoms (Nakashima et al. 2016) of Ménière’s syndrome are fluctuating hearing loss, low pitch tinnitus, fullness in the ear, and episodic spinning vertigo which lasts at least twenty minutes, but typically two to three hours. When it is secondary to another disorder it is called Ménière’s syndrome and there is a long list of possible causes, including hypothyroidism, acoustic neuroma, superior semicircular canal dehiscence (SCDS), perilymphatic fistula, genetic mutations (Verstreken et al. 2001) and many, many more.

Ménière’s disease is when a person has Ménière’s syndrome and the cause cannot be identified – idiopathic in other words. If someone has Ménière’s syndrome and on MRI an acoustic neuroma is seen (Homann et al. 2014), the patient has Ménière’s syndrome secondary to an acoustic neuroma rather than Ménière’s disease. This is more than simply an academic point. I would suggest that the more aggressive the doctor is at finding an underlying cause, the less a diagnosis of Ménière’s disease will be made. Furthermore, treating the underlying cause often resolves the Ménière’s syndrome with much greater success than the non-specific treatments employed for Ménière’s disease. A drug that works for one person does not necessarily work for another because the underlying reasons for Ménière’s syndrome are so varied and the complex pharmacokinetics of the inner ear make drug delivery to it a tricky business. Within this context, the role played by SCDS needs to be examined in closer detail, partly because the prevalence of that condition is now estimated to be 0.1% and also because there is mounting evidence that a large number of people diagnosed with Ménière’s disease actually have Ménière’s syndrome secondary to SCDS. The majority of SCDS symptoms can usually be resolved by surgical intervention (Thomeer et al. 2016).

A study (Alemanji et al. 2016) of 521 patients with dizziness examined the experiences of the participants. A third of them were reported to be frustrated with “being misdiagnosed or misdirected”, and only 20% felt their diagnosis was “accurate” and “timely”. The “data suggest that the diagnosis of Ménière’s disease is over-utilized.” The objective of a separate study at Apeldoorn Dizziness Centre (van Leeuwen and van der Zaag-Loonen 2015) was to establish how often a new diagnosis or treatment was given to patients with dizziness when they were referred for a second opinion to a specialised centre, as “the incremental diagnostic and therapeutic yield of this approach has rarely been studied.” The results were revealing. Out of the group of 327, a new or amended diagnosis was given to 54%, and new treatment advice to 62%.   

The high rate of misdiagnosis among patients with vestibular problems, causing unnecessary suffering and a waste of healthcare resources, was highlighted again in another analysis undertaken (Jin et al. 2012). In the study of 287 patients, mostly middle-aged women, who had visited the Dizziness Clinic at Changzheng Hospital between October 2010 and January 2011, 48 diagnoses ranging from Ménière’s disease to cerebral circulation insufficiency were overturned. All 48 were then successfully treated for BPPV.

 An attempt was made (Syed et al. 2015) to evaluate the evidence and trends in published literature for the treatment of Ménière’s syndrome or disease over the last two decades. The authors concluded that there had been “an increase in emphasis on randomized controlled trials and quality of life studies”, but “a significant number of studies failed to follow AAOHNS-CHE criteria for reporting treatment outcome and the CONSORT criteria for reporting randomized controlled trials.”

A much wider review (van Esch et al. 2016) is currently underway to examine all the different treatment modalities for patients diagnosed with Ménière’s disease. Dr Babette van Esch, leading the review, informed me: “Naturally we are looking at the definitions of the diagnosis applied in the studies and whether or not the evaluated patients met the generally accepted criteria for Ménière’s disease. Unfortunately, several studies do not describe the methods of diagnosing the Ménière’s disease patients in great detail. The assessment of the certainty of that diagnosis is therefore complicated sometimes ….. We are aware that other diseases may be included in these populations, including SCDS.”     

Continued after the holiday…

 

 

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A Shotgun Approach to Treatment

As we have reviewed Meniere’s disease treatments over the last several weeks, it is clear that there is not consensus on the proper, most effective treatment. There is also no consensus on the cause of Meniere’s type symptoms. Various theories on etiology include:

 

 

  1. Overproduction of endolymph by the stria vascularis
  2. Blockage of the endolymphatic duct
  3. Blockage of the reuniting duct
  4. Rupture of Reissner’s membrane
  5. Auto-immune disorder
  6. Migraine variant

The first four on this list all involve hydrops (too much fluid), and techniques to attempt regulating fluid have been discussed. We have also discussed the overlap between Meniere’s and migraine. Dr. Carol Foster, at the University of Colorado has proposed a combined approach, where they treat early, suspected Meniere’s disease as a possible hydropic condition AND/OR a cerebro-vascular condition such as migraine. Similar to the treatment ladder proposed for hydrops, they have a step wise approach to migraine, beginning with avoidance of migraine triggers, and moving to prophylactic medications if symptoms persist or progress. Here is her description of these steps, which can take place simultaneously with conventional hydrops treatments:

Migraine trigger elimination

Common migraine food triggers include monosodium glutamate, chocolate, red wine, fermented dairy products including yogurt, and aged or pickled foods. Trigger elimination is advocated as treatment for migraine-associated vestibular disorders, but controlled studies demonstrating the efficacy of this treatment in Ménière’s disease are lacking.

Therapeutic rationale

The migraine group recommends avoidance of these foods with the belief that Ménière’s disease will fluctuate and worsen if migraines are uncontrolled. There is no expectation that these triggers modify hydrops, but they are often included in dietary regimens for hydrops control based on their empiric efficacy. Our protocol is to recommend avoidance of these food triggers in patients with migraine headaches, aura, or in Ménière’s patients under the age of 50 years because migraine is the most common vascular risk factor in that group.

Migraine prophylactic medications

Amitriptyline, beta blockers, calcium channel blockers, acetazolamide, and topiramate are commonly used in the prevention of migraine headaches and are also used for vestibular symptoms in migraineurs. Migraine prophylactic medications are used by some centers for Ménière’s disease but controlled studies are lacking.

Therapeutic rationale

Migraine and hydrops have not been shown to be causally related, so migraine prophylactic medications are generally used only by the migraine group with the belief that vasospasm and other migraine phenomena can be controlled by these drugs. Calcium channel blockers such as verapamil, and in Europe, flunarizine, are effective in migraineurs. The carbonic anhydrase inhibitors have some diuretic effects and so may impact hydrops directly in addition to treating migraine. The blockers are also used in the treatment of hypertension and so may be of use in Ménière’s patients who also have migraine and hypertension.”

Dr. Foster’s article makes it clear that this approach is largely theoretical and lacks controlled studies of efficacy. As I mentioned in a previous blog on Meniere’s/Migraine overlap, Ghavani and colleagues at the University of Califonia/Irvine have followed up with patient diagnosed with Meniere’s, yet treated as migraine, with impressive results. This is an area that deserves study, but it will be some time before any conclusions can be drawn.