Meniere disease
- Overview
- Theory
- Diagnosis
- Management
- Follow up
- Resources
Treatment algorithm
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
all patients
dietary changes and lifestyle modification
All patients should be educated on dietary changes and lifestyle modification. Patients should be advised to restrict salt intake to 1500 to 2300 mg/day, as this is thought to prevent sodium-related water retention and redistribution into the endolymphatic system.[21]Basura GJ, Adams ME, Monfared A, et al. Clinical practice guideline: Ménière's disease. Otolaryngol Head Neck Surg. 2020 Apr;162(suppl 2):S1-55. https://aao-hnsfjournals.onlinelibrary.wiley.com/doi/10.1177/0194599820909438 http://www.ncbi.nlm.nih.gov/pubmed/32267799?tool=bestpractice.com [52]Colletti V. Medical treatment in Meniere's disease: avoiding vestibular neurectomy and facilitating postoperative compensation. Acta Otolaryngol Suppl. 2000;120(suppl 544):27-33. http://www.ncbi.nlm.nih.gov/pubmed/10904798?tool=bestpractice.com Although there are no randomized controlled trials to document the benefits of low-salt diet on the treatment of meniere disease (MD), patients often report exacerbation of their symptoms or even precipitation of an attack after a salty meal.[52]Colletti V. Medical treatment in Meniere's disease: avoiding vestibular neurectomy and facilitating postoperative compensation. Acta Otolaryngol Suppl. 2000;120(suppl 544):27-33. http://www.ncbi.nlm.nih.gov/pubmed/10904798?tool=bestpractice.com [53]Hussain K, Murdin L, Schilder AG. Restriction of salt, caffeine and alcohol intake for the treatment of Ménière's disease or syndrome. Cochrane Database Syst Rev. 2018 Dec 31;(12):CD012173. https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD012173.pub2/full http://www.ncbi.nlm.nih.gov/pubmed/30596397?tool=bestpractice.com
Limiting caffeine intake, reducing alcohol consumption, ceasing smoking, and managing stress are also advisable, as these may trigger an attack. However, there is no evidence from randomized controlled trials to support or refute the restriction of salt, caffeine, or alcohol intake in patients with MD.[53]Hussain K, Murdin L, Schilder AG. Restriction of salt, caffeine and alcohol intake for the treatment of Ménière's disease or syndrome. Cochrane Database Syst Rev. 2018 Dec 31;(12):CD012173. https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD012173.pub2/full http://www.ncbi.nlm.nih.gov/pubmed/30596397?tool=bestpractice.com [54]Webster KE, George B, Lee A, et al. Lifestyle and dietary interventions for Ménière's disease. Cochrane Database Syst Rev. 2023 Feb 27;2(2):CD015244. https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD015244.pub2/full http://www.ncbi.nlm.nih.gov/pubmed/36848645?tool=bestpractice.com
Such dietary changes may be the only necessary treatment required in the early stages of the disease.
diuretic
Treatment recommended for SOME patients in selected patient group
Diuretics are believed to reduce the volume of the endolymph and may be offered for maintenance therapy.[21]Basura GJ, Adams ME, Monfared A, et al. Clinical practice guideline: Ménière's disease. Otolaryngol Head Neck Surg. 2020 Apr;162(suppl 2):S1-55. https://aao-hnsfjournals.onlinelibrary.wiley.com/doi/10.1177/0194599820909438 http://www.ncbi.nlm.nih.gov/pubmed/32267799?tool=bestpractice.com [55]Webster KE, Galbraith K, Harrington-Benton NA, et al. Systemic pharmacological interventions for Ménière's disease. Cochrane Database Syst Rev. 2023 Feb 23;2(2):CD015171. https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD015171.pub2/full http://www.ncbi.nlm.nih.gov/pubmed/36827524?tool=bestpractice.com
The most commonly used diuretics in the treatment of MD are thiazides with or without potassium-sparing diuretics (e.g., hydrochlorothiazide/triamterene) and acetazolamide.[21]Basura GJ, Adams ME, Monfared A, et al. Clinical practice guideline: Ménière's disease. Otolaryngol Head Neck Surg. 2020 Apr;162(suppl 2):S1-55. https://aao-hnsfjournals.onlinelibrary.wiley.com/doi/10.1177/0194599820909438 http://www.ncbi.nlm.nih.gov/pubmed/32267799?tool=bestpractice.com
If the patient remains symptom-free for 6 months, diuretics may be slowly tapered and restarted if required. If there is no response, the patient should be changed to the alternative diuretic. These medications should not be used in patients with a known or suspected reaction to sulfonamides.
The evidence of the efficacy of diuretics on MD is controversial, and direct evidence of its efficacy on disease progression is lacking in the literature.[2]Thirlwall AS, Kundu S. Diuretics for Ménière's disease or syndrome. Cochrane Database Syst Rev. 2006 Jul 19;(3):CD003599. https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD003599.pub2/full http://www.ncbi.nlm.nih.gov/pubmed/16856015?tool=bestpractice.com However, diuretics are still considered by many physicians to be first-line treatment in all patients with MD.
Primary options
triamterene/hydrochlorothiazide: 37.5 mg (triamterene)/25 mg (hydrochlorothiazide) orally once daily
-- OR --
acetazolamide: 250 mg orally (regular-release) twice daily
vestibular suppressant, antiemetic, or corticosteroid
Treatment recommended for ALL patients in selected patient group
Symptoms of individual and acute vertigo spells can be treated with vestibular suppressants and antiemetics. However, much of the effect is from the sedative action of these drugs.
The literature lacks randomized controlled trials assessing the effects of these medications for acute attacks of MD.
Commonly used treatments include antihistamines (e.g., meclizine, dimenhydrinate, promethazine), benzodiazepines (e.g., diazepam), and phenothiazines (e.g., prochlorperazine).[57]Soto E, Vega R. Neuropharmacology of vestibular system disorders. Curr Neuropharmacol. 2010 Mar;8(1):26-40. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2866460 http://www.ncbi.nlm.nih.gov/pubmed/20808544?tool=bestpractice.com Diazepam should only be used in acute attacks.[21]Basura GJ, Adams ME, Monfared A, et al. Clinical practice guideline: Ménière's disease. Otolaryngol Head Neck Surg. 2020 Apr;162(suppl 2):S1-55. https://aao-hnsfjournals.onlinelibrary.wiley.com/doi/10.1177/0194599820909438 http://www.ncbi.nlm.nih.gov/pubmed/32267799?tool=bestpractice.com It should be prescribed at low doses where possible, and long-term prescription avoided due to the risk of dependency. Prochlorperazine is a second-line treatment for patients with refractory nausea.
Oral corticosteroids may be used to treat acute attacks of vertigo, especially when accompanied by acute hearing loss and tinnitus. They are widely used because of their anti-inflammatory properties, although no randomized controlled trials are available to assess their efficacy in MD.[60]Silverstein H, Isaacson JE, Olds MJ, et al. Dexamethasone inner ear perfusion for the treatment of Meniere's disease: a prospective, randomized, double-blind, crossover trial. Am J Otol. 1998 Mar;19(2):196-201. http://www.ncbi.nlm.nih.gov/pubmed/9520056?tool=bestpractice.com The dose, indications, and duration of corticosteroids used vary in the literature. Oral corticosteroids have very well-known adverse effects but are often tolerated at such a dose and for short durations.
Primary options
meclizine: 12.5 to 25 mg orally every 6 hours when required
OR
dimenhydrinate: 50 mg orally every 4-6 hours when required
OR
promethazine: 12.5 to 25 mg orally/rectally every 4-6 hours when required
Secondary options
diazepam: 2-10 mg orally every 4-6 hours when required
OR
prochlorperazine maleate: 5-10 mg orally every 6-8 hours when required
OR
prochlorperazine rectal: 25 mg rectally twice daily when required
Tertiary options
prednisone: 20 mg orally three times daily for 2-3 weeks, then gradually taper
intratympanic injection
Treatment recommended for SOME patients in selected patient group
Intratympanic corticosteroids: there is wide variation in the dose used, frequency of administration, and method of application in the literature. Tympanic membrane perforation and infection can occur.
Intratympanic gentamicin: gentamicin (an aminoglycoside antibiotic) preferentially destroys the vestibular labyrinth when injected into the middle ear, resulting in chemical labyrinthectomy. In one prospective, double-blind, randomized, placebo-controlled clinical trial, intratympanic gentamicin treatment was found to reduce the score of vertigo severity and perceived aural fullness in the treatment group.[77]Postema RJ, Kingma CM, Wit HP, et al. Intratympanic gentamicin therapy for control of vertigo in unilateral Menière's disease: a prospective, double-blind, randomized, placebo-controlled trial. Acta Otolaryngol. 2008 Aug;128(8):876-80. http://www.ncbi.nlm.nih.gov/pubmed/18607963?tool=bestpractice.com Evidence suggests that intratympanic gentamicin injections improve vertigo symptoms, are well tolerated, and have a low incidence of severe hearing loss.[21]Basura GJ, Adams ME, Monfared A, et al. Clinical practice guideline: Ménière's disease. Otolaryngol Head Neck Surg. 2020 Apr;162(suppl 2):S1-55. https://aao-hnsfjournals.onlinelibrary.wiley.com/doi/10.1177/0194599820909438 http://www.ncbi.nlm.nih.gov/pubmed/32267799?tool=bestpractice.com However, two systematic reviews in 2023 found that the efficacy of intratympanic gentamicin and corticosteroid injections in the treatment of patients with MD is unclear due to the uncertainty of available evidence.[78]Webster KE, Lee A, Galbraith K, et al. Intratympanic corticosteroids for Ménière's disease. Cochrane Database Syst Rev. 2023 Feb 27;2(2):CD015245. https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD015245.pub2/full http://www.ncbi.nlm.nih.gov/pubmed/36847608?tool=bestpractice.com [79]Webster KE, Galbraith K, Lee A, et al. Intratympanic gentamicin for Ménière's disease. Cochrane Database Syst Rev. 2023 Feb 27;2(2):CD015246. https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD015246.pub2/full http://www.ncbi.nlm.nih.gov/pubmed/36847592?tool=bestpractice.com
Primary options
dexamethasone sodium phosphate: consult specialist for guidance on intratympanic dose
OR
methylprednisolone sodium succinate: consult specialist for guidance on intratympanic dose
OR
gentamicin: consult specialist for guidance on intratympanic dose
nonpharmaceutical therapy
Treatment recommended for ALL patients in selected patient group
Patients with severe, intractable tinnitus can receive relief with a number of modalities, such as tinnitus maskers, tinnitus retraining therapy (TRT), amplifications, medication, biofeedback, and neuromonics.[67]Hanley PJ, Davis PB, Paki B, et al. Treatment of tinnitus with a customized, dynamic acoustic neural stimulus: clinical outcomes in general private practice. Ann Otol Rhinol Laryngol. 2008 Nov;117(11):791-9. http://www.ncbi.nlm.nih.gov/pubmed/19102123?tool=bestpractice.com
Tinnitus maskers (white noise generators) are devices similar to hearing aids that fit behind the ear. They produce a quiet external sound that distracts the patient from the internal tinnitus noise. In one form of tinnitus masking, the level of the masker is increased until the patient's own tinnitus is rendered inaudible. In TRT the masker remains audible along with the patient's tinnitus, and the patient learns to adjust to the audible masking level along with his or her own tinnitus. Thus, both remain audible in a graduated situation where the patient learns to tolerate his or her own tinnitus while accepting the audible tinnitus masker.
TRT is counseling accompanied by white noise generators. TRT is a favored treatment, but it may take up to 18 months before full benefits are achieved.[64]Jastreboff PJ, Hazell JW. A neurophysiological approach to tinnitus: clinical implications. Br J Audiol. 1993 Feb;27(1):7-17. http://www.ncbi.nlm.nih.gov/pubmed/8339063?tool=bestpractice.com [65]Jastreboff PJ, Hazell JW. Treatment of tinnitus based on a neurophysiological model. In: Vernon J, ed. Tinnitus: treatment and relief. Boston, MA: Allyn & Bacon; 1998:201-16.
Neuromonics uses a customized neural stimulus combined with specific music, delivered through a coordinated program to interact with, interrupt, and desensitize tinnitus disturbance for long-term benefit.[66]Davis PB, Paki B, Hanley PJ. Neuromonics Tinnitus Treatment: third clinical trial. Ear Hear. 2007 Apr;28(2):242-59. http://www.ncbi.nlm.nih.gov/pubmed/17496674?tool=bestpractice.com [67]Hanley PJ, Davis PB, Paki B, et al. Treatment of tinnitus with a customized, dynamic acoustic neural stimulus: clinical outcomes in general private practice. Ann Otol Rhinol Laryngol. 2008 Nov;117(11):791-9. http://www.ncbi.nlm.nih.gov/pubmed/19102123?tool=bestpractice.com [68]Davis PB, Wilde RA, Steed LG, et al. Treatment of tinnitus with a customized acoustic neural stimulus: a controlled clinical study. Ear Nose Throat J. 2008 Jun;87(6):330-9. http://www.ncbi.nlm.nih.gov/pubmed/18561116?tool=bestpractice.com
Amplifications (hearing aids) may help in masking the tinnitus.
Biofeedback techniques attempt to decrease the anxiety that is associated with the tinnitus. This can be achieved through relaxation techniques, hypnosis, and cognitive behavioral therapy.
antidepressant or benzodiazepine
Treatment recommended for SOME patients in selected patient group
Medications such as antidepressants (e.g., amitriptyline) and benzodiazepines (e.g., alprazolam) may help patients with intractable tinnitus, but are associated with adverse effects.[69]Johnson RM, Brummett R, Schleuning A. Use of alprazolam for relief of tinnitus. A double-blind study. Arch Otolaryngol Head Neck Surg. 1993 Aug;119(8):842-5. http://www.ncbi.nlm.nih.gov/pubmed/8343245?tool=bestpractice.com They should only be used if nonpharmaceutical treatments are unsuccessful and debilitating tinnitus persists.
Primary options
amitriptyline: 25-75 mg/day orally given in 1-3 divided doses
OR
alprazolam: 0.25 to 0.5 mg orally (immediate-release) three times daily
corticosteroid
Treatment recommended for ALL patients in selected patient group
The dose, indications, and duration of oral corticosteroids used vary in the literature. Oral corticosteroids have very well known adverse effects but are often tolerated at such a dose and for short durations.
Intratympanic corticosteroids are used in patients with MD where systemic corticosteroids are contraindicated, or in patients who do not respond to oral corticosteroids. There is a wide variation in the dose used, frequency of administration, and method of application in the literature. Tympanic membrane perforation and infection can occur.
Primary options
prednisone: 20 mg orally three times daily for 2-3 weeks, then gradually taper
Secondary options
dexamethasone sodium phosphate: consult specialist for guidance on intratympanic dose
OR
methylprednisolone sodium succinate: consult specialist for guidance on intratympanic dose
persistent hearing loss
amplification (hearing aid) or assistive listening device
Because hearing varies dramatically in these patients, access to expert audiologic personnel for reprogramming the hearing aid(s) is essential. Use of instruments incorporating algorithms to improve word recognition in noisy listening environments is also essential.
Amplification using fully digital hearing aids with variable digitally adjustable circuitry should be evaluated. The traditional view that amplification does not work for patients with MD is not based on experience with modern amplification.
New forms of directional microphones, digital signal processing circuitry, and wireless technology can provide significant benefits in helping MD patients to hear better in environments with competing noise.[70]Valente M, Mispagel K, Valente LM, et al. Problems and solutions for fitting amplification to patients with Meniere's disease. J Am Acad Audiol. 2006 Jan;17(1):6-15. http://www.ncbi.nlm.nih.gov/pubmed/16640056?tool=bestpractice.com
Assistive listening devices are a form of amplification for those with situational difficulties in hearing, and who are not yet ready or willing to use personal hearing aids.
intensive high-quality audiologic counseling
Treatment recommended for ALL patients in selected patient group
Intensive high-quality audiologic counseling is needed for patients with MD in the adjustment to, and acceptance of, amplification.
failure of medical and intratympanic therapies; hearing adequate
endolymphatic sac surgery
A nondestructive procedure that consists of decompression of the endolymphatic sac from the overlying bone and drainage of its endolymph. This maintains the vestibular neuroepithelium and its innervation.
Decreases endolymphatic pressure and addresses both cochlear and vestibular dysfunctions.
Its role in MD is controversial, with studies that show 90% resolution of vertigo, and others that demonstrate that it is no more effective than placebo, or that there is insufficient evidence of the beneficial effect of endolymphatic sac surgery in MD.[7]Paparella MM. Pathogenesis and pathophysiology of Meniere's disease. Acta Otolaryngol Suppl. 1991;111(suppl 485):26-35. http://www.ncbi.nlm.nih.gov/pubmed/1843169?tool=bestpractice.com [84]Bretlau P, Thomsen J, Tos M, et al. Placebo effect in surgery for Meniere's disease: nine-year follow-up. Am J Otol. 1989 Jul;10(4):259-61. http://www.ncbi.nlm.nih.gov/pubmed/2679115?tool=bestpractice.com [85]Pillsbury HC, Arenberg IK, Ferraro J, et al. Endolymphatic sac surgery. The Danish sham surgery study: an alternative analysis. Otolaryngol Clin North Am. 1983 Feb;16(1):123-7.[86]Thomsen J, Bretlau P, Tos M, et al. Ménière's disease: endolymphatic sac decompression compared with sham (placebo) decompression. Ann N Y Acad Sci. 1981 Nov;374(1):820-30. http://www.ncbi.nlm.nih.gov/pubmed/7041752?tool=bestpractice.com [87]Thomsen J, Bonding P, Becker B, et al. The non-specific effect of endolymphatic sac surgery in treatment of Meniere's disease: a prospective, randomized controlled study comparing "classic" endolymphatic sac surgery with the insertion of a ventilating tube in the tympanic membrane. Acta Otolaryngol. 1998 Nov;118(6):769-73. http://www.ncbi.nlm.nih.gov/pubmed/9870617?tool=bestpractice.com [88]Pullens B, Verschuur HP, van Benthem PP. Surgery for Ménière's disease. Cochrane Database Syst Rev. 2013 Feb 28;(2):CD005395. https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD005395.pub3/full http://www.ncbi.nlm.nih.gov/pubmed/23450562?tool=bestpractice.com [89]Szott FA, Westhofen M, Hackenberg S. Is endolymphatic sac surgery an efficient treatment of Menière's disease patients? A systematic literature search and meta-analysis. Eur Arch Otorhinolaryngol. 2023 Mar;280(3):1119-28. https://link.springer.com/article/10.1007/s00405-022-07580-8 http://www.ncbi.nlm.nih.gov/pubmed/36208333?tool=bestpractice.com A 2023 systematic literature search and meta-analysis revealed a paucity of studies on this surgical procedure, indicating ELS may be a beneficial treatment for patients with MD.[89]Szott FA, Westhofen M, Hackenberg S. Is endolymphatic sac surgery an efficient treatment of Menière's disease patients? A systematic literature search and meta-analysis. Eur Arch Otorhinolaryngol. 2023 Mar;280(3):1119-28. https://link.springer.com/article/10.1007/s00405-022-07580-8 http://www.ncbi.nlm.nih.gov/pubmed/36208333?tool=bestpractice.com However, further studies are needed to attain a better understanding of the efficacy of ELS for treating MD.
Endolymphatic sac surgery carries a risk of hearing loss in up to 2% of patients.[93]Paparella MM, Sajjadi H. Endolymphatic sac enhancement. Principles of diagnosis and treatment. Am J Otol. 1987 Jul;8(4):294-300. http://www.ncbi.nlm.nih.gov/pubmed/3631235?tool=bestpractice.com Other potential complications of this procedure include bleeding from the sigmoid sinus and cerebrospinal fluid leak.
vestibular nerve section
In this procedure, the vestibular portion of the eighth cranial nerve (CN VIII) is selectively cut and its cochlear portion is left intact; thus, this is potentially a hearing conservation approach. This prevents the vestibular afferent stimuli from reaching the brain.
This does not alter the pathophysiology of MD, but provides relief from vertigo, its most disturbing symptom. It should be avoided in bilateral MD, otherwise oscillopsia (perception of bouncing of the visual field with walking) and permanent imbalance may occur.
Central compensation after vestibular nerve section is crucial for postoperative recovery of balance. Central nervous system disease such as cerebellar dysfunction, multiple sclerosis, physiologic aging, and poor medical condition are relative contraindications for vestibular nerve section.
Vertigo control rates are up to 90% with vestibular nerve section.[94]Silverstein H, Jackson LE. Vestibular nerve section. Otolaryngol Clin North Am. 2002 Jun;35(3):655-73. http://www.ncbi.nlm.nih.gov/pubmed/12486846?tool=bestpractice.com [95]Møller MN, Cayé-Tomasen P, Thomsen JH. Vestibular nerve section in the treatment of morbus Ménière [in Danish]. Ugeskr Laeger. 2009 Mar 16;171(12):1000-3. http://www.ncbi.nlm.nih.gov/pubmed/19284921?tool=bestpractice.com Persistent or recurrent vertigo after vestibular nerve section can be treated by intratympanic gentamicin.
Potential complications are uncommon and include hearing loss, facial nerve paralysis, cerebrospinal fluid leak, and headache.[94]Silverstein H, Jackson LE. Vestibular nerve section. Otolaryngol Clin North Am. 2002 Jun;35(3):655-73. http://www.ncbi.nlm.nih.gov/pubmed/12486846?tool=bestpractice.com
failure of medical and intratympanic therapies; hearing severely impaired
labyrinthectomy
Involves surgical removal of the inner ear's neuroepithelium in an attempt to eliminate vertigo.
Hearing loss is inevitable with this procedure and it should only be used in patients with no serviceable hearing. Avoided in patients with bilateral disease, as bilateral loss of vestibular input to the brain may result in oscillopsia (perception of bouncing of the visual field with walking) and permanent imbalance.
Central compensation after labyrinthectomy is important for balance recovery, and vestibular rehabilitation therapy after surgery may help speed the recovery. Central nervous system disease, advanced age, and a variety of significant medical conditions can prevent central compensation after surgery and these patients are, therefore, not good candidates for labyrinthectomy.
Vertigo control rates up to 97% after have been reported.[96]Berryhill WE, Graham MD. Chemical and physical labyrinthectomy for Meniere's disease. Otolaryngol Clin North Am. 2002 Jun;35(3):675-82. http://www.ncbi.nlm.nih.gov/pubmed/12486847?tool=bestpractice.com
Complications from labyrinthectomy include facial nerve injury (2%) and cerebrospinal fluid leak in 3%.[97]Graham MD, Colton JJ. Transmastoid labyrinthectomy indications. Technique and early postoperative results. Laryngoscope. 1980 Aug;90(8 Pt 1):1253-62. http://www.ncbi.nlm.nih.gov/pubmed/7401826?tool=bestpractice.com
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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer
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