Meniere Disease


Article Author:
Lukas Koenen


Article Editor:
Claudio Andaloro


Editors In Chief:
Laurie Graham
Andrew Wilt
Mary Cataletto


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Hussain Sajjad
Steve Bhimji
Muhammad Hashmi
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Beenish Sohail
Nazia Sadiq
Hajira Basit
Phillip Hynes


Updated:
1/9/2019 5:18:28 PM

Introduction

Meniere disease is a disorder of the inner ear characterized by hearing loss, tinnitus, and vertigo. In most cases, it is slowly progressive and has a significant impact on the social functioning of the individual affected.[1]

The current diagnostic criteria defined by the Barany society by Lopez-Escamez et al. can help differentiate between a probable and a definite Meniere's disease. 

Patients with a definite Meniere disease according to the Barany Society have:

  1. Two or more spontaneous episodes of vertigo with each lasting 20 minutes to 12 hours

  2. Audiometrically documented low- to medium- frequency sensorineural hearing loss in one ear, defining and locating to the affected ear on in at least one instance prior, during or after one of the episodes of vertigo

  3. Fluctuating aural symptoms (fullness, hearing, tinnitus) located in the affected ear

  4. Not better accounted for by any other vestibular diagnosis

Probable Meniere disease can include the following clinical findings:

  1. Two or more episodes of dizziness or vertigo, each lasting 20 minutes to 24 hours

  2. Fluctuating aural symptoms (fullness, hearing, or tinnitus) in the affected ear

  3. The condition is better explained by another vestibular diagnosis[2]

Etiology

Studies of the temporal bone revealed endolymphatic accumulation in the cochlea and the vestibular organ in patients with Meniere disease. Current research links endolymphatic hydrops to a hearing loss of >40dB. Vertigo may or may not be associated.[3] Therefore endolymphatic hydrops is not entirely specific for Meniere disease and can be found in cases of idiopathic sensorineural hearing loss. 

The exact etiology of Meniere disease remains unclear. Different theories exist, but genetic and environmental factors play a role. The relation to common comorbidities remains elusive. 

Epidemiology

The prevalence of Meniere disease varies between 3.5 per 100.000 and 513 per 100.000[4][5] and occurs more often in older, white and female patients.[4][5][6]

The identification of several comorbidities which occur in an increased fashion in patients with Meniere disease gave rise to new theories about the origins of the disease.

1) Migraine: Migraine occurs more often in patients diagnosed with Meniere disease although there might be an overlap between basilar migraine wrongly diagnosed as Meniere disease.[7]

2) Autoimmune Diseases: Several autoimmune diseases are associated with Meniere disease namely rheumatoid arthritis, systemic lupus erythematosus and ankylosing spondylitis.[8]

History and Physical

At the emergency room or in the general practice the physician will differentiate between vertigo of central, peripheral and cardiovascular cause. Red flags for a central origin of vertigo, according to Harcourt et al., are neurological symptoms or signs, acute deafness, new type or onset of headache, or vertical/torsional/rotatory nystagmus.[9]

If Meniere disease is suspected, the patient should be questioned about the character of vertigo, hearing loss and earlier episodes. A full otologic history is part of the clinical investigation. 

If Meniere disease is suspected, one should perform a full otologic examination, facial nerve testing and assessment of nystagmus with Frenzel goggles, Rinne, and Weber tests. 

Rinne and Weber: Will show sensorineural hearing loss in acute Meniere disease or advanced disease.

Frenzel goggles: May show horizontal nystagmus with a fast beating component away from the affected vestibular organ in the acute setting.

Head impulse testing (HIT): In contrast to other peripheral vestibular disorders, this test has a low sensitivity in Meniere disease.[10]

Evaluation

Audiometric evaluation is mandatory in all patients with Meniere disease. Fluctuating low frequency unilateral sensorineural hearing loss is characteristic for the disease. The hearing loss can progress to all frequencies. Tinnitus is common and ipsilateral.[11]

All patients with one-sided hearing loss should undergo magnetic resonance imaging (MRI) to rule out retrocochlear pathology. In some countries a BERA (brainstem evoked response audiometry) is sufficient. There is no need to perform imaging in the acute setting but may be done within a few weeks after onset of symptoms. High resolution MRI imaging may directly show endolymphatic hydrops in the affected organs. More research is under way to show if this is of clinical use.[12][13]

Vestibular (caloric) function testing may show a significantly under-functioning affected organ in 42% to 74% and a full loss of function in 6% to 11%.[14]

Treatment / Management

Different treatment options for Meniere disease exist with substantial variability between countries. None of the treatment options cure the disease. As many treatments have a significant impact on the functioning of surrounding structures, one should start with non-invasive approaches with the fewest possible side effects and proceed to more invasive steps.

  1. Sodium restriction diet: Low-level evidence suggests that restricting the sodium intake may help to prevent Meniere attacks.[9]
  2. Betahistine: Substantial disagreement in the medical community about the use of betahistine exist. A Cochrane review found low-level evidence to support the use of betahistin with substantial variability between studies.[15] Medical therapy in many medical centers often starts with betahistine orally.
  3. Intratympanic steroid injections may reduce the number of vertigo attacks in patients with Meniere disease.[16]
  4. Intratympanic gentamycin injections: Gentamycin has strong ablative properties towards vestibular cells. Side effects are a sensorineural hearing loss because of a certain amount of toxicity towards cochlear cells.[17] 
  5. Surgery with 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 a complete hearing loss in the affected side.[14]

Differential Diagnosis

  1. Basilar migraine: Associated with vertigo but without aural symptoms
  2. Vestibular neuronitis: Associated with vertigo lasting for several days, no aural symptoms
  3. Benign paroxysmal positional vertigo: Associated with vertigo related to head movements, lasting seconds to minutes, no aural symptoms
  4. Medications (e.g., aminoglycosides and loop diuretics)

Prognosis

According to Perrez-Garrigues et al. the number of episodes of vertigo is higher in the first years of the disease and decrease in later years regardless whether patients receive treatment; most patients reach a "steady-state phase free of vertigo."[18]

As with vertigo, loss of hearing is highest in the early years of the disease and stabilizes in later years. Usually, there is no recovery from the hearing loss.[19]

Complications

In later stages of the pathology, patients may experience sudden unexpected drops without loss of consciousness (Tumarkin attacks).[20]

One systematic review reports bilateral involvement of the vestibular organ in up to 47% of patients within 20 years.[21][22]

Patients with Meniere disease report significantly impaired quality of life compared to healthy individuals.[23]

Consultations

Refer patients with signs suggestive for Meniere disease for otolaryngologic consultation.

Deterrence and Patient Education

Suspect Meniere disease if the patient experiences loss of hearing on one ear with attacks of vertigo which last from several minutes to several hours, and tinnitus.

Patients who experience the above seek consultation with their general practitioner or the emergency room.

The emergency room doctor will exclude vertigo secondary to disease of the heart or your vessels, or of neurologic origin, and refer the patient to an otolaryngologist for further testing and treatment.

Enhancing Healthcare Team Outcomes

The evaluation of patients with vertigo is complex, and patients often require medical attention from neurologists, otolaryngologists, and internal medicine. The Bárány Society published the current classification of Meniere disease. It is important to base the diagnosis of Meniere disease on the criteria published and mentioned in this article to warrant a uniform diagnosis especially in the presence of different international approaches to the diagnosis of patients with vertigo. (Level II)


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Meniere Disease - Questions

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Fluctuating tinnitus accompanied with dizziness and hearing loss is commonly seen in patients with which of the following conditions?



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Which drug is used for the treatment of Meniere disease?



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Which drug is sometimes used to treat severe bilateral Meniere disease?



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In which of the following conditions is recruitment phenomenon often seen?



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Which of the following is not a component of Meniere disease?



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What is the most common cause of fluctuating hearing loss and episodic vertigo of peripheral origin?



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Which of the following is NOT a treatment of Meniere disease?



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Which of the following is a useful treatment for tinnitus associated with Meniere disease?



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What is the definitive cure for Meniere disease in a 35 year old female?



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During an episode of Meniere disease, what is the best advice?



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Which medication is not recommended for use in Meniere disease?



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What is the most troubling symptom of Meniere disease?



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What type of hearing loss occurs most often in patients with Menieres Disease at the beginning of the disease?



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A 23 year old has been having recurrent episodes of rotational vertigo lasting from 6-10 hours. After the first couple of attacks, he also developed unilateral tinnitus, decreased hearing, hyperacusis, and sensation of fullness which waxes and wanes. What is the most likely diagnosis?



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The etiology of Meniere disease is idiopathic. Which of the following is not considered a possible etiology of this condition?



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How does Meniere disease generally present?



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A female presents with vertigo lasting several hours associated with tinnitus and fullness in the right ear. She has rotary (torsional) nystagmus that is worse with left gaze and high-tone hearing loss of the right ear. What is the most likely diagnosis?



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A man complains of tinnitus and dizziness. He had 3 episodes of this but no other significant past medical history. These bouts prevent him from working and are always unilateral. Meclizine has not improved his symptoms. The symptoms are not aggravated by ambulation or the Dix-Hallpike maneuvers. Select the best treatment option for long-term therapy.



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The classic triad of Meniere disease includes all of the following except:



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Which of the following is a true statement about hearing loss in Meniere disease?



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Which is not seen in Meniere disease?



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Vertigo is a common sensation in patients with meniere disease. To make the diagnosis, the episodes should?



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Which of the following is not an approved treatment for the symptoms associated with Meniere disease?



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Meniere Disease - References

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. The journal of international advanced otology. 2018 Aug;     [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, Diagnostic criteria for Menière's disease. Journal of vestibular research : equilibrium     [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. European radiology. 2018 Jul;     [PubMed]
Alexander TH,Harris JP, Current epidemiology of Meniere's syndrome. Otolaryngologic clinics of North America. 2010 Oct;     [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. The Laryngoscope. 1984 Aug;     [PubMed]
Tyrrell JS,Whinney DJ,Ukoumunne OC,Fleming LE,Osborne NJ, Prevalence, associated factors, and comorbid conditions for Ménière's disease. Ear and hearing. 2014 Jul-Aug;     [PubMed]
Ray J,Carr SD,Popli G,Gibson WP, An epidemiological study to investigate the relationship between Meniere's disease and migraine. Clinical otolaryngology : official journal of ENT-UK ; official journal of Netherlands Society for Oto-Rhino-Laryngology     [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;     [PubMed]
Syed I,Aldren C, Meniere's disease: an evidence based approach to assessment and management. International journal of clinical practice. 2012 Feb;     [PubMed]
Shi S,Guo P,Wang W, Magnetic Resonance Imaging of Ménière's Disease After Intravenous Administration of Gadolinium. The Annals of otology, rhinology, and laryngology. 2018 Nov;     [PubMed]
Patel VA,Oberman BS,Zacharia TT,Isildak H, Magnetic resonance imaging findings in Ménière's disease. The Journal of laryngology and otology. 2017 Jul;     [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. Frontiers in neurology. 2018;     [PubMed]
Murdin L,Hussain K,Schilder AG, Betahistine for symptoms of vertigo. The Cochrane database of systematic reviews. 2016 Jun 21;     [PubMed]
Phillips JS,Westerberg B, Intratympanic steroids for Ménière's disease or syndrome. The Cochrane database of systematic reviews. 2011 Jul 6;     [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 oto-laryngologica. 2008 Aug;     [PubMed]
Pyykkö I,Manchaiah V,Zou J,Levo H,Kentala E, Do patients with Ménière's disease have attacks of syncope? Journal of neurology. 2017 Oct;     [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. Archives of otolaryngology--head     [PubMed]
Stahle J, Advanced Meniere's disease. A study of 356 severely disabled patients. Acta oto-laryngologica. 1976 Jan-Feb;     [PubMed]
Huppert D,Strupp M,Brandt T, Long-term course of Menière's disease revisited. Acta oto-laryngologica. 2010 Jun;     [PubMed]
Green JD Jr,Blum DJ,Harner SG, Longitudinal followup of patients with Menière's disease. Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery. 1991 Jun;     [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. Otology     [PubMed]
Harcourt J,Barraclough K,Bronstein AM, Meniere's disease. BMJ (Clinical research ed.). 2014 Nov 12;     [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. The Laryngoscope. 2018 Dec 4;     [PubMed]

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