Welcome back to TGIF Dr Euan's blog-post :-)
If you have suffered from hearing loss and dizziness/giddiness, then you would probably have heard of Meniere's Disease (MD). It is somewhat akin to GLAUCOMA which affects the eye and vision, but unlike GLAUCOMA, MD affects the inner ear fluid pressure (in both the Cochlea and the Vestibular system).
Meniere’s disease (MD)
Ménière disease is a disorder caused by fluid build up in the inner ear chambers.
It causes symptoms such as vertigo, nausea, vomiting, loss of hearing, ringing in the ears, headache, loss of balance, and sweating.
Treatment choices may depend on the severity of the disease and you should discuss with your doctor about which choices are right for you.
Q: What is Ménière's disease?
Ménière disease is a balance disorder.
It is caused by an abnormality in a part of the inner ear called the labyrinth. Fluid build-up here can cause a severe spinning sensation (vertigo) and affect the hearing, which may fluctuate from time to time.
Q: What causes Ménière's disease?
The labyrinth holds the organs of balance and hearing. It’s made up of 2 parts:
Bony labyrinth
Membranous labyrinth
The membranous labyrinth is encased in bone and contains a fluid called endolymph.
When the head moves, the endolymph also moves. This causes nerve receptors in the membranous labyrinth to signal the brain about the body's motion.
Excess endolymph buildup in the labyrinth can interfere with the normal balance and hearing signals between the inner ear and the brain. This is what happens in Ménière disease.
Image of the inner ear anatomy
Fluid build-up in the inner ear may be caused by:
Allergies
Abnormal immune system response
Abnormal fluid drainage caused by a blockage
Post Head injury
Genetic risk
Migraine headaches
Viral infection
Most often, Ménière disease is caused by more than one factor.
Q: What are the risk factors for Ménière's disease?
Anyone can get Ménière disease.
It is more prevalent in people in their 40s and 50s. There is presently no cure for MD; treatment options are aimed at controlling the symptoms e.g. hearing loss and giddiness.
There is a correlation with migraine and MD is more common in ladies.
Q: What are the symptoms of Ménière disease?
These are the most common symptoms of Ménière disease.
Symptoms can happen suddenly and may occur daily or infrequently. The symptoms are most often in one ear but can affect both ears in about 20 per cent of cases.
Vertigo is the most debilitating symptom; this is a severe spinning sensation that is often associated with:
Severe nausea
Vomiting
Sweating
Image of a person suffering from dizziness and giddiness
Other symptoms may include:
Tinnitus (ringing in the ears)
Loss of hearing or muffled hearing
Loss of the ability to hear low frequencies
Pressure in the affected ear
Loss of balance
Headaches
The symptoms of Ménière disease may mimic other conditions or health problems. So it is important to get the correct diagnosis.
Q: How is Ménière disease diagnosed?
Your doctor will take a comprehensive medical history and perform a full physical exam,
The doctor may also request:
Hearing test. This will help find any changes in hearing related to disease in the middle ear or other causes.
Balance test eg VNG / vHIT / oVEMP / cVEMP
Magnetic resonance imaging (MRI) scans. An MRI is done to exclude any tumour e.g. Acoustic Neuroma
Electrocochleography (ECOG). This test measures the electrical activity of the inner ear.
Image of a patient doing a hearing test
You may see other specialists including:
Audiologist (for hearing evaluation)
Neurologist (for any concomitant conditions e.g. Migraine)
Q: How is Ménière disease treated?
Your doctor will discuss the various options and plan the best treatment based on:
Your age
Your overall health and medical history
How symptomatic you are
How well do you tolerate specific medicines, procedures, or therapies
How frequent and long the episodes are
Your opinion or preferences
Treatment options may include:
Medication. Medicines may be given to control allergies, reduce fluid build-up, reduce dizziness, or improve the blood circulation in the inner ear.
Change in diet. Eliminating caffeine, chocolate, alcohol, and salt may reduce the frequency and intensity of symptoms in MD patients.
Behavioural therapies. Reducing stress may lessen the severity of the disease symptoms.
Hearing Aids: to manage hearing loss and improve communication for patients.
Surgery. Several types of surgery are somewhat effective for treating the balance problems of Ménière disease eg vestibular nerve section or if the hearing is poor, a labyrinthectomy.
As of today, there is no cure for MD.
Different treatment options for Meniere's disease exist with great variability between countries and centers.
None of these treatment options cures the disease.
As many treatments significantly impact the functioning of surrounding structures, one should start with non-invasive approaches with the fewest possible side effects and proceed to more invasive steps.
Sodium restriction diet: Low-level evidence suggests that restricting sodium intake may help to prevent Meniere attacks.
Thiazide diuretics: Form the mainstay of medical treatment in many centres. Evidence suggests they reduce the frequency and severity of symptomatic episodes but do not appear to prevent hearing loss.
Betahistine: Substantial disagreement exists in the medical community about the use of betahistine. A Cochrane review found low-level evidence to support the use of betahistine with substantial variability between studies.
Intratympanic steroid injections: May reduce the number of vertigo attacks in patients with Meniere disease while preserving auditory function.
Image of Dr Euan performing the IT Dexa Injection procedure
5. Intratympanic gentamycin injections: Gentamycin has strong ablative properties towards vestibular cells. Side effects are sensorineural hearing loss because of a certain amount of toxicity toward cochlear cells.
6. Vestibular nerve section or labyrinthectomy: Nerve section is a therapeutic option in patients who failed the conservative treatment options, and labyrinthectomy when surgical options failed. Labyrinthectomy leads to complete hearing loss on the affected side.
Q: What are the complications of Ménière disease?
Vertigo is one of the main symptoms of Ménière disease. This can be quite disabling, depending on your occupation and/or lifestyle.
It can cause falls, trouble driving, or interfere with other normal activities of daily living.
Permanent hearing loss may also happen. Hearing aids can help rehabilitate hearing loss, for profound hearing loss, there is also the option of the Cochlear Implant or Bionic Ear.
All these problems can cause depression and anxiety. It may be hard to work or interact with your family and friends as MD is an invisible disease and patients may appear very well in between MD episodes. As such, work colleagues and even family members may not understand what the patient is going through, leaving the patient feeling lonely and helpless.
Your doctor needs to explain and communicate with your spouse/partner and family members about your MD so that you can get the support and help you need.
Well, I do hope this blog post has given you some useful information on MD; if you would like to delve deeper into the subject, here are some useful references.
Have a good weekend! Dr Euan :-)
REFERENCES:
Magnan J, Özgirgin ON, Trabalzini F, Lacour M, Escamez AL, Magnusson M, Güneri EA, Guyot JP, Nuti D, Mandalà M. European Position Statement on Diagnosis, and Treatment of Meniere's Disease. J Int Adv Otol. 2018 Aug;14(2):317-321. [PMC free article] [PubMed]
Lopez-Escamez JA, Carey J, Chung WH, Goebel JA, Magnusson M, Mandalà M, Newman-Toker DE, Strupp M, Suzuki M, Trabalzini F, Bisdorff A., Classification Committee of the Barany Society. Japan Society for Equilibrium Research. European Academy of Otology and Neurotology (EAONO). Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS). Korean Balance Society. Diagnostic criteria for Menière's disease. J Vestib Res. 2015;25(1):1-7. [PubMed]
Attyé A, Eliezer M, Medici M, Tropres I, Dumas G, Krainik A, Schmerber S. In vivo imaging of saccular hydrops in humans reflects sensorineural hearing loss rather than Meniere's disease symptoms. Eur Radiol. 2018 Jul;28(7):2916-2922. [PubMed]
Zou J, Zhao Z, Zhang G, Zhang Q, Pyykkö I. MEFV, IRF8, ADA, PEPD, and NBAS gene variants and elevated serum cytokines in a patient with unilateral sporadic Meniere's disease and vascular congestion over the endolymphatic sac. J Otol. 2022 Jul;17(3):175-181. [PMC free article] [PubMed]
Alexander TH, Harris JP. Current epidemiology of Meniere's syndrome. Otolaryngol Clin North Am. 2010 Oct;43(5):965-70. [PubMed]
Wladislavosky-Waserman P, Facer GW, Mokri B, Kurland LT. Meniere's disease: a 30-year epidemiologic and clinical study in Rochester, Mn, 1951-1980. Laryngoscope. 1984 Aug;94(8):1098-102. [PubMed]
Tyrrell JS, Whinney DJ, Ukoumunne OC, Fleming LE, Osborne NJ. Prevalence, associated factors, and comorbid conditions for Ménière's disease. Ear Hear. 2014 Jul-Aug;35(4):e162-9. [PubMed]
Ray J, Carr SD, Popli G, Gibson WP. An epidemiological study to investigate the relationship between Meniere's disease and migraine. Clin Otolaryngol. 2016 Dec;41(6):707-710. [PubMed]
Gazquez I, Soto-Varela A, Aran I, Santos S, Batuecas A, Trinidad G, Perez-Garrigues H, Gonzalez-Oller C, Acosta L, Lopez-Escamez JA. High prevalence of systemic autoimmune diseases in patients with Menière's disease. PLoS One. 2011;6(10):e26759. [PMC free article] [PubMed]
Zhang N, Lyu Y, Guo J, Liu J, Song Y, Fan Z, Li X, Li N, Zhang D, Wang H. Bidirectional Transport of IgE by CD23 in the Inner Ear of Patients with Meniere's Disease. J Immunol. 2022 Feb 15;208(4):827-838. [PMC free article] [PubMed]
Skarp S, Korvala J, Kotimäki J, Sorri M, Männikkö M, Hietikko E. New Genetic Variants in CYP2B6 and SLC6A Support the Role of Oxidative Stress in Familial Ménière's Disease. Genes (Basel). 2022 Jun 01;13(6) [PMC free article] [PubMed]
Jeng YJ, Young YH. Comparison of inner ear deficits in Meniere's variants and their significance. Acta Otolaryngol. 2021 Jul;141(7):684-688. [PubMed]
Harcourt J, Barraclough K, Bronstein AM. Meniere's disease. BMJ. 2014 Nov 12;349:g6544. [PubMed]
Fukushima M, Oya R, Nozaki K, Eguchi H, Akahani S, Inohara H, Takeda N. Vertical head impulse and caloric are complementary but react opposite to Meniere's disease hydrops. Laryngoscope. 2019 Jul;129(7):1660-1666. [PubMed]
Stölzel K, Droste J, Voß LJ, Olze H, Szczepek AJ. Comorbid Symptoms Occurring During Acute Low-Tone Hearing Loss (AHLH) as Potential Predictors of Menière's Disease. Front Neurol. 2018;9:884. [PMC free article] [PubMed]
Shi S, Guo P, Wang W. Magnetic Resonance Imaging of Ménière's Disease After Intravenous Administration of Gadolinium. Ann Otol Rhinol Laryngol. 2018 Nov;127(11):777-782. [PubMed]
Patel VA, Oberman BS, Zacharia TT, Isildak H. Magnetic resonance imaging findings in Ménière's disease. J Laryngol Otol. 2017 Jul;131(7):602-607. [PubMed]
Syed I, Aldren C. Meniere's disease: an evidence based approach to assessment and management. Int J Clin Pract. 2012 Feb;66(2):166-70. [PubMed]
Mori N, Miyashita T, Inamoto R, Matsubara A, Mori T, Akiyama K, Hoshikawa H. Ion transport its regulation in the endolymphatic sac: suggestions for clinical aspects of Meniere's disease. Eur Arch Otorhinolaryngol. 2017 Apr;274(4):1813-1820. [PMC free article] [PubMed]
Murdin L, Hussain K, Schilder AG. Betahistine for symptoms of vertigo. Cochrane Database Syst Rev. 2016 Jun 21;2016(6):CD010696. [PMC free article] [PubMed]
Phillips JS, Westerberg B. Intratympanic steroids for Ménière's disease or syndrome. Cochrane Database Syst Rev. 2011 Jul 06;(7):CD008514. [PubMed]
Postema RJ, Kingma CM, Wit HP, Albers FW, Van Der Laan BF. Intratympanic gentamicin therapy for control of vertigo in unilateral Menire's disease: a prospective, double-blind, randomized, placebo-controlled trial. Acta Otolaryngol. 2008 Aug;128(8):876-80. [PubMed]
Perez-Garrigues H, Lopez-Escamez JA, Perez P, Sanz R, Orts M, Marco J, Barona R, Tapia MC, Aran I, Cenjor C, Perez N, Morera C, Ramirez R. Time course of episodes of definitive vertigo in Meniere's disease. Arch Otolaryngol Head Neck Surg. 2008 Nov;134(11):1149-54. [PubMed]
Huppert D, Strupp M, Brandt T. Long-term course of Menière's disease revisited. Acta Otolaryngol. 2010 Jun;130(6):644-51. [PubMed]
Green JD, Blum DJ, Harner SG. Longitudinal followup of patients with Menière's disease. Otolaryngol Head Neck Surg. 1991 Jun;104(6):783-8. [PubMed]
Stahle J. Advanced Meniere's disease. A study of 356 severely disabled patients. Acta Otolaryngol. 1976 Jan-Feb;81(1-2):113-9. [PubMed]
Pyykkö I, Manchaiah V, Zou J, Levo H, Kentala E. Do patients with Ménière's disease have attacks of syncope? J Neurol. 2017 Oct;264(Suppl 1):48-54. [PubMed]
Söderman AC, Bagger-Sjöbäck D, Bergenius J, Langius A. Factors influencing quality of life in patients with Ménière's disease, identified by a multidimensional approach. Otol Neurotol. 2002 Nov;23(6):941-8. [PubMed]
Lahiji MR, Akbarpour M, Soleimani R, Asli RH, Leyli EK, Saberi A, Akbari M, Ramezani H, Nemati S. Prevalence of anxiety and depression in Meniere's disease; a comparative analytical study. Am J Otolaryngol. 2022 Sep-Oct;43(5):103565. [PubMed]
