A disorder affecting inner ear homeostasis, classically manifested by episodic vertigo lasting hours, fluctuating hearing loss, tinnitus and aural fullness. Rarely sudden drop attacks can develop, but when present are debilitating. It is however heterogenous in both presentation and cause, and thus a single treatment will not be effective for everyone. 1 2The 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. 3. 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, .4 necessitating parallel treatment.
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. 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. The use of serial audiograms to confirm fluctuating hearing is essential. Doing this at home using an app such as Mimi Hearing Test can be very helpful.
An important early goal 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. The recent International Consensus on the management of Meniere’s Disease has reinforced the evidence based approach to management outlined below. 5
Management of acute vertigo
Usually this involves the use of vestibular suppressants. Commonly used are Stemetil, Phenergen, Lorazepam and cinnarizine. Usually ondansetron is used sublingually 10 minutes prior to the oral vestibular suppressant to ensure absorption. An injection or suppository is occasionally required if the ondansetron cannot control severe vomiting. Sometimes patients get a premonitory sensation and can abort an attack with the use of one of the vestibular suppressants, a diuretic or anti-inflammatory steroids.
During a prolonged attack or a series of attacks, these medications can be taken regularly or the use of intra-tympanic steroids can be used. (This involves an injection through the ear drum under a local anaesthetic in the clinic.) 6,7
As the pattern and severity of the disease varies greatly from patient to patient so does their management. Approximately 75-80% of patients can achieve good control with non-interventional treatment. 4 The first step is to control the two major triggers of an attack, stress and salt. 8 When we use the term stress this includes physiologic, emotional, endocrinologic, metabolic, muscular.
Management of stress and ensuring adequate sleep is essential.9 Alternative treatments such as massage therapy and acupuncture can be helpful, as can formal psychological treatment in the form of cognitive behavioral therapy, especially in severe cases where consideration of surgical intervention is being made. 10 The induced anxiety related to the severity of the disease and the unpredictability of the vertigo episodes is often enough to trigger further episodes, inducing a horrible feed back loop.
A low salt diet from a Meniere’s perspective requires a significant change in lifestyle, even in those who generally live a healthy life and do not add salt to their food, or eat much in the way of processed foods. There is significant hidden salt in many foods, especially salt and breakfast cereals. Obtaining salt contents in all foods is essential and the book ‘Salt matters’ is a good resource. Consulting a dietician can also be very helpful. The only way to monitor salt intake is to take a 24 hour urine collection. In general terms the first goal is to achieve less than 1200mg/day. If vertigo continues then further tightening to less than 600mg/day is targeted.
Other considerations such as withdrawing caffeine, chocolate, smoking and alcohol are important for some patients. It is important to note that salt is not a trigger for many patients.
Management of inhalant and food allergies is important in a subset of patients. Allergies are up to three times as prevalent in Meniere’s Disease. Interestingly this is especially the case if also suffering from Migraine. The symptoms of MD are generally better controlled, with fewer vertigo attacks and more stable hearing, in those patients with allergy and MD whose underlying allergic disorder is down-regulated with immunotherapy and/or dietary avoidance of reactive food allergens. 11 (Another form of ‘stress’ on the body)
When there is inadequate control medications and surgical interventions must then be considered.
The commonest drug used is Betahistine (Serc). It is thought to act by increasing the blood supply to the inner ear, acting as a H3-antagonist. Anecdotally betahistine is helpful in some patients, but again there is no overwhelming evidence to support it’s use. 12 A recent paper has suggested that it acts on the endolymphatic sac. 13 It has a very low side effect profile, so is worth trying prior to interventional management. The dose can be elevated to 32mg three times a day.
Diuretic therapy, (fluid tablets) have been used for many years in Meniere’s. In theory they may alter in the electrolyte balance within the inner ear. There has been controversy over their use, with no evidence to support their use. 14 Side effects at the low doses used for Meniere’s Disease however are minimal, so can their use can be considered. A typical drug is Moduretic: Amiloride HCl 5 mg, hydrochlorothiazide 50 mg; 1-2 tablets per day.
Migraine Prophylaxis: As mentioned it is not uncommon to have co-existing migrainous vertigo, or it is difficult to differentiate. Many of the same triggers exist such as any physiologic or emotional stress and certain foods. There is a low threshold to use migraine preventors, initially non-pharmacologic supplements containing magnesium, riboflavin and feverfew. Pharmacologic options include amitriptyline, dothep, pizotifen, propranolol, topomax, cinnarizine and flunarizine. 15-17
Intervention in Meniere’s disease is divided into non-destructive and destructive categories. Again the principal of any intervention is to stop vertigo, prevent ongoing hearing loss and minimize tinnitus and fullness, while minimizing damage to the vestibular system. Unfortunately the procedures with the highest rates of success stopping the vertigo attacks carry the highest rates of permanent disequilibrium and often place the hearing at risk. Therefore minimal impact interventions are often tried first, even if there is no guarantee of success. The caveat here is in those situations where definite control of vertigo is needed, such as in those patients carrying a heavy vehicle license where their livelihood depends on cure from vertigo. The development of drop attacks is another circumstance where a more aggressive approach is needed. Drops attacks are a fortunately rare development in Meniere’s disease where a sudden loss of postural support results in a sudden collapse with no loss of consciousness.
Another important factor in avoiding destructive procedures if possible, is in the presence of or chance of developing bilateral disease. Bilateral severe vestibular loss is an extremely debilitating condition.
Intratympanic steroid therapy,has now become the initial interventional non-ablative treatment option. It has been shown (usually dexamethasone or methylpredisone) to have good levels of success in reducing vertigo but with no risk to hearing, as apposed to gentamicin.22 23,24 We currently compound dexamethasone to 24mg/ml (as opposed to the standard available concentration of 4mg/ml. Therefore it is almost always used before any potentially destructive procedure is considered. There is no defined regime at this point, but one protocol is to repeat an injection each week or fortnight for 4 injections. A ventilation tube at the beginning of the course to make sequential injections/infusions easier, and can also be continued at home, with an appropriate concentration of steroid.
Tympanostomy tube insertion (grommets) can be placed and are thought to influence inner ear pressure by stabilizing middle ear pressure, and perhaps the degree of inner ear instability. They are predominantly helpful in reducing the sensation of fullness, but can also (rarely) reduce vertigo. The most important fact is there is no risk to hearing nor of worsening overall balance function. The only precaution is that the ear needs to be kept dry. The placement of a tube also allows the use of the Meniett device. This device emits repeated pressure pulses with amplitude of 12 cm of water and consist of a complex pressure wave composed of static pressure and a 6-Hz sinus modulation. It is an essentially minimally invasive device which in some studies has reasonable rates of control.18-20 In Australia this is currently not funded by insurance companies. 21Personally I do not feel it plays a great role in Meniere’s Disease.
The main role for tympanostomy tubes is as a portal for the self application of intratympanic steroids.
The following interventions range from partially to completely destructive. The concept here is that we whilst we are controlling vertigo episodes, this often comes at the cost of overall reduction in balance and hearing. This can usually but not always adequately rehabilitated.
Endolymphatic sac surgery. This involves a mastoidectomy and opening of the endolymphatic sac which in theory stabilizes inner ear homeostasis. There is varying evidence to support its use, although in some hands is reported to have good control rates with acceptable rates of hearing loss. 25-29
Intratympanic gentamicin 30-34 35has significantly changed interventional management in Meniere’s disease over the last 20 years. Most studies show around 80% good control of vertigo, but with a 10-20% chance of worsening hearing and about 2% chance of complete hearing loss.36 Essentially this is a partial chemical labyrinthectomy, and can make overall balance worse. Balance training in the form of vestibular rehabilitation after this intervention is essential. If the initial treatment fails, normally a repeated injections are performed if there are ongoing vertigo episodes, prior to moving on to more significant interventions. The procedure itself can be either performed under local anaesthetic in the clinic or in the operating theatre. The latter allows visualization of the middle ear to ensure that there is not a thin membrane covering the round window which can impair absorption of the drug, but is really only done if inital injections are not effective.
Vestibular nerve section is a highly effective procedure to control vertigo, with only a 2% chance of hearing loss (in the short term) It does however involve an intracranial procedure, albeit with a low rate of complications. Essentially this is considered in young patients with good residual hearing, especially if definitive control of vertigo is needed. 37 The comment about short term hearing preservation is that the cochlear (component) of the disease may well progress.
Surgical Labyrinthectomy is a highly effective procedure to control vertigo, but results in sacrifice of any residual hearing and vestibular function on that side. 37 In most cases now a concurrent cochlear implant is placed. 38 which does give a good degree of hearing rehabilitation but obviously the concern as for gentamicin and vestibular nerve section is reliance on the contralateral ear and other components of the balance system to compensate.
Hearing and Vestibular Rehabilitation
From a hearing rehabilitation and often control of tinnitus, a hearing aid is strongly recommended. Hearing aid technology is constantly improving with easy self adjustment to manage fluctuating hearing levels. When an ear has no remaining functional hearing, a cross over hearing aid is an option. This can either utilise Bluetooth or bone conducting technology, ie a bone anchored hearing aid [BAHA, Bonebridge] The other option is cochlear implantation, both in unilateral hearing loss, but even more importantly in bilateral disease. In select cases concomitant surgical labyrinthectomy and cochlear implantation can be considered.38
There is some evidence that boosting the hearing can also help to some degree with balance.
Gentle to moderate exercise is very important physically and psychologically. Formal vestibular rehabilitation is essential, especially when the disease has caused permanent vestibular impairment and or an ablative procedure is required. It is often much more difficult to compensate if the level of vestibular function is fluctuating.
This is a very important (probably the most important) and much underestimated aspect in the management of Menieres disease. This can help minimize stressors which act as a trigger to acute attacks, and can also help in the management of underlying tinnitus, dizziness and imbalance. A syndrome labeled psychophysiologic dizziness plays a large role in many patients with Meniere’s Disease. This essentially where an insult to the vestibular system leaves a degree of nerve damage. The brain needs to compensate for this loss and anxiety, especially anxiety centred on the fear of further attacks or dizziness can further amplify the symptoms of instability.
Recommended Treatment Regime
Confirm probable diagnosis, and exclude central pathology. MRI scan, hearing and vestibular function tests.
Institute low salt diet, and monitor levels using 24 hour urine collections
Lifestyle modifications, stress reduction with consideration of psychologic therapy if required.
Consider trial of betahistine (Serc), up to a dose of 32mg three times a day
Consider dietary supplements
Hearing monitoring, hearing and vestibular rehabilitation
Consider trial of diuretic, especially if an appropriately low salt level is not reached.
Insertion of ventilation tube, with dexamethasone (steroid infusion) into middle ear, or transtympanic dexamethasone injection. These treatments can be repeated indefinitely if controlling symptoms.
Consider trial of Meniett Device
Consider Gentamicin Infusion, Endolymphatic sac surgery or Vestibular Nerve Section in young patients with good residual hearing.
Consider repeat Gentamicin Infusion
Consider surgical labyrinthectomy +/- cochlear implantation
Important Contacts and Links
Psychological Management for Cognitive behavioral therapy and Tinnitus Retraining Therapy (Clinical psychology list)
(Vestibular Physiotherapist list)
Helpful internet resources
- Phillips JS, Murdin L, Rea P, Sutton L. Clinical Subtyping of Meniere’s Disease. Otolaryngol Head Neck Surg 2018:194599818773077.
- al BGe. Clinical Practice Guidline: Meniere’s Disease Executive Summary. Otolaryngol Head Neck Surg 2020;162:415-34.
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- Sbeih F, Christov F, Gluth MB. Newly Diagnosed Meniere’s Disease: Clinical Course With Initiation of Noninvasive Treatment Including an Accounting of Vestibular Migraine. Ann Otol Rhinol Laryngol 2018;127:331-7.
- Nevoux J, Barbara M, Dornhoffer J, Gibson W, Kitahara T, Darrouzet V. International consensus (ICON) on treatment of Meniere’s disease. Eur Ann Otorhinolaryngol Head Neck Dis 2018;135:S29-S32.
- Shea JJ, Jr. The role of dexamethasone or streptomycin perfusion in the treatment of Meniere’s disease. Otolaryngol Clin North Am 1997;30:1051-9.
- Garduno-Anaya MA, Couthino De Toledo H, Hinojosa-Gonzalez R, Pane-Pianese C, Rios-Castaneda LC. Dexamethasone inner ear perfusion by intratympanic injection in unilateral Meniere’s disease: a two-year prospective, placebo-controlled, double-blind, randomized trial. Otolaryngol Head Neck Surg 2005;133:285-94.
- Yeo NL, White MP, Ronan N, Whinney DJ, Curnow A, Tyrrell J. Stress and Unusual Events Exacerbate Symptoms in Meniere’s Disease: A Longitudinal Study. Otol Neurotol 2018;39:73-81.
- Yeo NL, White MP, Ronan N, Whinney DJ, Curnow A, Tyrrell J. Stress and Unusual Events Exacerbate Symptoms in Meniere’s Disease: A Longitudinal Study. Otol Neurotol 2017.
- Sajjadi H. Medical management of Meniere’s disease. Otolaryngologic Clinics of North America;35:581-9.
- Derebery MJ, Berliner KI. Allergy and its relation to Meniere’s disease. Otolaryngologic Clinics of North America;43:1047-58.
- James AL, Burton MJ. Betahistine for Meniere’s disease or syndrome. Cochrane Database of Systematic Reviews 2001:CD001873.
- Moller MN, Kirkeby S, Vikesa J, Nielsen FC, Caye-Thomasen P. Expression of histamine receptors in the human endolymphatic sac: the molecular rationale for betahistine use in Menieres disease. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies 2015.
- Thirlwall AS, Kundu S. Diuretics for Meniere’s disease or syndrome. Cochrane Database of Systematic Reviews 2006;3:CD003599.
- von Brevern M, Lempert T. Vestibular migraine. Handbook of clinical neurology 2016;137:301-16.
- Sohn JH. Recent Advances in the Understanding of Vestibular Migraine. Behavioural neurology 2016;2016:1801845.
- Kang WS, Lee SH, Yang CJ, Ahn JH, Chung JW, Park HJ. Vestibular Function Tests for Vestibular Migraine: Clinical Implication of Video Head Impulse and Caloric Tests. Frontiers in neurology 2016;7:166.
- Zhang SL, Leng Y, Liu B, Shi H, Lu M, Kong WJ. Meniett Therapy for Meniere’s Disease: An Updated Meta-analysis. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 2016;37:290-8.
- Syed MI, Rutka JA, Hendry J, Browning GG. Positive pressure therapy for Meniere’s syndrome/disease with a Meniett device: a systematic review of randomised controlled trials. Clinical otolaryngology : official journal of ENT-UK ; official journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery 2015;40:197-207.
- Ahsan SF, Standring R, Wang Y. Systematic review and meta-analysis of Meniett therapy for Meniere’s disease. Laryngoscope 2015;125:203-8.
- Mattox DE, Reichert M. Meniett device for Meniere’s disease: use and compliance at 3 to 5 years. Otology & Neurotology 2008;29:29-32.
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- Boleas-Aguirre MS, Lin FR, Della Santina CC, Minor LB, Carey JP. Longitudinal results with intratympanic dexamethasone in the treatment of Meniere’s disease. Otology & Neurotology 2008;29:33-8.
- Patel M, Agarwal K, Arshad Q, et al. Intratympanic methylprednisolone versus gentamicin in patients with unilateral Meniere’s disease: a randomised, double-blind, comparative effectiveness trial. Lancet 2016.
- Pullens B, Giard JL, Verschuur HP, van Benthem PP. Surgery for Meniere’s disease. Cochrane Database of Systematic Reviews:CD005395.
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- Lim MY, Zhang M, Yuen HW, Leong JL. Current evidence for endolymphatic sac surgery in the treatment of Meniere’s disease: a systematic review. Singapore Med J 2015;56:593-8.
- Kitahara T, Horii A, Imai T, et al. Effects of endolymphatic sac decompression surgery on vertigo and hearing in patients with bilateral Meniere’s disease. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 2014;35:1852-7.
- Bento RF, Cisneros JC, De Oliveira Fonseca AC. Endolymphatic sac drainage for the treatment of Meniere’s disease. J Laryngol Otol 2017;131:144-9.
- Flanagan S, Mukherjee P, Tonkin J. Outcomes in the use of intra-tympanic gentamicin in the treatment of Meniere’s disease. J Laryngol Otol 2006;120:98-102.
- Postema RJ, Kingma CM, Wit HP, Albers FWJ, Van Der Laan BFAM. Intratympanic gentamicin therapy for control of vertigo in unilateral Menire’s disease: a prospective, double-blind, randomized, placebo-controlled trial. Acta Oto-Laryngologica 2008;128:876-80.
- Chia SH, Gamst AC, Anderson JP, Harris JP. Intratympanic gentamicin therapy for Meniere’s disease: a meta-analysis. Otology & Neurotology 2004;25:544-52.
- Paradis J, Hu A, Parnes LS. Endolymphatic sac surgery versus intratympanic gentamicin for the treatment of intractable Meniere’s disease: a retrospective review with survey. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 2013;34:1434-7.
- Yetiser S. Intratympanic Gentamicin for Intractable Meniere’s Disease – A Review and Analysis of Audiovestibular Impact. Int Arch Otorhinolaryngol 2018;22:190-4.
- Naples JG, Henry L, Brant JA, Eliades SJ, Ruckenstein MJ. Intratympanic Therapies in Meniere Disease: Evaluation of Outcomes and Early Vertigo Control. Laryngoscope 2019;129:216-21.
- Pullens B, van Benthem PP. Intratympanic gentamicin for Meniere’s disease or syndrome. Cochrane database of systematic reviews (Online);3:CD008234.
- Teufert KB, Doherty J. Endolymphatic sac shunt, labyrinthectomy, and vestibular nerve section in Meniere’s disease. Otolaryngologic Clinics of North America;43:1091-111.
- Mukherjee P, Eykamp K, Brown D, et al. Cochlear Implantation in Meniere’s Disease With and Without Labyrinthectomy. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 2016.