26/4/24, 11:41 Ménière Disease (Clinical) - Lecturio

Ménière Disease (Clinical)

Ménière disease is a condition characterized by episodes of vertigo, tinnitus, and
hearing loss, likely caused by endolymphatic hydrops of the labyrinthine system in
the inner ear. Risk factors include a family history of Ménière disease, preexisting
autoimmune disorders, allergies, and trauma to the head or ear. The diagnosis is
made clinically by audiometry, vestibular testing, and, occasionally, imaging.
Management consists of diet and lifestyle modification, vasodilators, diuretics,
antihistamines, benzodiazepines, antiemetics, glucocorticoids, surgical intervention,
and/or hearing aids.

Last updated: 4 de marzo de 2024

CONTENTS

Overview
Pathophysiology
Clinical Presentation
Diagnosis
Management
Differential Diagnosis
References

Overview
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Definition
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Ménière disease is a triad of episodic vertigo, tinnitus,COMIENZA
and hearing
TUloss likelyGRATIS
PRUEBA
caused by endolymphatic hydrops of the labyrinthine system of the inner ear.
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Ménière syndrome is Ménière disease occurring secondary to other inner ear

infections.

Epidemiology[2,5,7]
Occurs at any age
Symptoms begin from age 20 to 40.
Incidence ranges from 10 to 150 per 100,000 persons.
Bilateral disease occurs in 10%–50% of patients.

Risk factors[1,4–6]
The etiology of Ménière disease is not fully known. However, risk factors for the
disease may include:
Family history of Ménière disease
Preexisting autoimmune disorders
Congenital inner ear malformations
Allergies
Trauma to the head or ear
Syphilis

Pathophysiology

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26/4/24, 11:41 Ménière Disease (Clinical) - Lecturio

Endolymph and perilymph:[2,5]

Fluid within the inner ear chambers
Have different concentrations of electrolytes (particularly K+)
Separated by thin membranes → contain the neural apparatus of hearing
and balance
Overall, pressure fluctuations → stress on the nerve-rich membranes results in:
Hearing disturbances
Tinnitus
Vertigo
Imbalance
Sensation of pressure in the inner ear
↑ Endolymphatic pressure can cause:
Breaks in the membranes:
Endolymph and perilymph mix → mixture has altered concentration
of K+ → affects vestibular nerve receptors → blocks depolarization
→ transient loss of function
Changed rate of vestibular nerve firing → vertigo
Physical distension → mechanical disruption of auditory and otolithic
organs
Utricle and saccule: responsible for linear and translational motion
detection
Irritation → nonrotational vestibular symptoms
Mechanical disturbance of the organ of Corti → distortion of the basilar
membrane and the inner and outer hair cells → hearing loss, tinnitus
Apex of the cochlea is more sensitive to pressure changes (coiled up
tighter than the base)

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26/4/24, 11:41 Ménière Disease (Clinical) - Lecturio

Anatomy of the inner ear

Image: “The internal ear” by BruceBlaus. License: CC BY 3.0

Clinical Presentation
Signs and symptoms[1,2,8,10]
The clinical course of Ménière disease can fluctuate, with varying frequency and
severity of acute vertigo episodes, as well as variable progression of hearing
derangement. The disease is typically unilateral, but 30% of cases may be
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4 bilateral.
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Vertigo:
Perceived rotatory spinning or rocking motion while remaining still
Sometimes associated with nausea and vomiting
Symptoms may persist from 20 minutes to 24 hours.
Imbalance:
Can be episodic or ongoing
Feeling of unsteadiness or instability (separate from vertigo)
Hearing loss:
Initially intermittent, then progresses to permanent hearing loss
Often involves low-frequencies (can involve all frequencies)
Tinnitus:
Perceived noises (usually high-pitched ringing) that are not actually
present
Fluctuating or constant, with variable pitch and intensity
Aural fullness

Physical examination[1]
During the evaluation of a patient with suspected Ménière disease, be sure to do
the following:
Examine the cranial nerve.
Observe balance (e.g., Romberg test).
Assess cerebellar function (test for dysdiadochokinesia).
Examine the ears.

Red flags[8,12]
Symptoms and findings that should prompt an urgent workup for an alternative
diagnosis include:
Central neurologic symptoms (e.g., facial weakness)
Sudden onset of severe deafness
New, severe headache
Vertical nystagmus

Diagnosis
Diagnostic criteria
A clinical diagnosis of definite or probable Ménière disease can be made with
freefollowing
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criteria:[2,7]
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Definite Ménière disease:

≥ 2 spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours
Audiometrically documented low- to mid-frequency
sensorineural hearing loss in the affected ear on ≥ 1 occasion (before,
during, or after episode of vertigo)
Fluctuating aural symptoms in the affected ear, including:
↓ or distorted hearing
Tinnitus
Fullness
Symptoms not better accounted for by another vestibular diagnosis (e.g.,
rule out vestibular migraine)
Probable Ménière disease:
≥ 2 spontaneous episodes of vertigo, each lasting 20 minutes to 24 hours
Fluctuating aural symptoms in the affected ear
Symptoms not better accounted for by another vestibular diagnosis

Testing

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26/4/24, 11:41 Ménière Disease (Clinical) - Lecturio

Audiometry:[2,8,9,12]
For all patients
Most common pattern:
Low-frequency sensorineural hearing loss
Combined low- and high-frequency sensorineural hearing loss
Normal hearing in the middle frequencies
With time, hearing “flattens out,” with hearing loss across all frequency
ranges.
Establishes baseline before treatment
Findings may also guide treatment
Imaging:[2,8,9]
Decision to order imaging should be made on an individual basis and in
conjunction with the patient's wishes.
Typically pursued in cases of asymmetric sensorineural hearing loss
confirmed by audiometry
MRI of the internal auditory canal:
May identify supporting features (e.g., less prominent endolymphatic
duct)
Can exclude causative lesions for symptoms, such as schwannoma
MRI/CT brain: CNS lesions
Vestibular testing:[2,8,9]
Should not routinely be ordered to establish the diagnosis of
Ménière disease
Results can vary:
May be normal early in the course of the disease
Abnormal as disease advances on the affected side
Possible tests:
Electronystagmography (ENG)
Rotary chair testing
Computerized dynamic posturography
Videonystagmography (VNG)
Vestibular evoked myogenic potentials (VEMPs)

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MRI of endolymphatic hydrops (EH) in patients with Ménière disease:

a: corresponding-to-mild EH
b: corresponding-to-significant EH
Image: “Fig2” by R. Gürkov et al. License: CC BY 4.0

Laboratory studies[1]
The following studies may be used to rule out other causes of vertigo or
hearing loss:
RPR, VDRL → syphilis
Hemoglobin A1c → diabetes
Cortisol and ACTH → adrenal insufficiency
TSH → hypothyroidism
CBC → anemia

Management
Management may vary based on practice location. The following information is
based on US, European, and UK guidelines and literature. Treatment improves
and relieves symptoms but does not correct the underlying pathophysiology.

Goals of treatment[2,3,8–10]
The goal of treatment is to improve a patient’s quality of life by:
Reducing frequency and severity of vertigo attacks
Reducing or eliminating the tinnitus and hearing loss associated with attacks
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and disequilibrium
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Minimizing disability associated with Ménière disease
Preventing progressive hearing loss
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General management[1–3,8–10]
Provide patient education on the course of the disease.
Diet:
Salt restriction to < 2 g/day
Limit nicotine, caffeine, and alcohol
Caffeine and nicotine are vasoconstrictors reducing microvascular
flow in the labyrinthine system.
Alcohol causes fluid and electrolyte shifts to stress a vulnerable ear.
Avoid any known triggers.
Consider allergy testing and treatment.
Reduce stress, when possible.
Psychological support may be beneficial.
Alternative therapies:
Acupuncture
Ginkgo
Ginger
Niacin
Tai chi

Pharmacotherapy
Medications can be provided for the management of symptoms.

Acute management:[1–3,10,11]

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Background:
Treatment involves suppressing central vestibular neural activity and
managing nausea.
Limited peer-reviewed evidence for pharmacotherapy in acute attacks, but
patients do tend to have symptom improvement
Insufficient evidence to recommend any particular drug class over another
Should only be used for acute events
Chronic use can perpetuate imbalance.
Antihistamines:
Potential options:
Dimenhydrinate
Diphenhydramine
Meclizine 12.5–20 mg orally every 8 hours, as needed
Potential side effects:
Sedation
Dry mouth
Urinary retention
Benzodiazepines:
Potential options:
Diazepam
Lorazepam
Clonazepam
Note: Alprazolam is not recommended owing to its potential to cause
tachyphylaxis.
Potential side effects:
Medication dependence
Sedation
Centrally acting anticholinergics:
Potential options:
Scopolamine
Atropine
Potential side effects:
Blurry vision
Dry mouth
Pupil dilation
Urinary retention
Sedation
Antiemetics (suppress associated nausea, but do not improve vertigo):
Metoclopramide
Ondansetron
Prochlorperazine
Promethazine

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Table: Medication options* and dosing for acute episodes of

Ménière disease[2]

Class Medication Typical dose (adult)

1st-generation Dimenhydrinate 25‒50 mg orally, IM, or

antihistamines IV every 6 hours, as
needed

Diphenhydramine 25‒50 mg orally, IM, or

IV every 6 hours, as
needed

Meclizine 12.5‒20 mg orally every

8 hours, as needed

Benzodiazepines Diazepam 2–10 mg every 8 hours,

as needed

Lorazepam 1–2 mg every 8 hours,

as needed

Clonazepam 0.5–1 mg every 8 hours,

as needed

*List of available medications is not exhaustive.

Chronic management:[1–3,8,9]

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Background:
Used to reduce symptoms and prevent attacks
Patients may have a varied response to treatment
Should not be used as an abortive treatment
Diuretics:
Potentially alter electrolyte balance of endolymph and reduce endolymph
volume
Paucity of supporting data
Potential options:
Hydrochlorothiazide-triamterene
Spironolactone
Furosemide
Acetazolamide
Betahistine:
Histamine analog:
H3 receptor antagonist
Weak H1 receptor agonist
Conflicting evidence
Dose: 8‒16 mg by mouth 2‒3 times daily[8–10]
Not available in the US

Management of refractory disease

Refractory, disabling symptoms despite initial pharmacotherapy:[2,8,9]
Continue betahistine or diuretics, if beneficial.
Consider glucocorticoid therapy:
Systemic glucocorticoids: prednisone
Intratympanic glucocorticoids:
Dexamethasone or methylprednisolone
Should be administered by an experienced practitioner

Refractory, disabling symptoms despite glucocorticoid therapy:[1,2,8,9]

Destructive therapies: reduce or eliminate signals from the affected labyrinthine
system
Intratympanic gentamicin
Surgical labyrinthectomy
Vestibular neurectomy
Nondestructive procedures: reduce fluid accumulation or alter fluid and
electrolyte physiology
Decompression and/or shunting of the endolymphatic sac
Sacculotomy

Hearing amplification (hearing aids) for hearing loss:[2,3,8,9]

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devices and poor patient compliance.
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Vestibular rehabilitation for residual disequilibrium

between attacks[2,3]
Used in:
Individuals with response to surgical or medical treatment with residual
disequilibrium
Individuals with significant balance and disequilibrium symptoms between
acute vertigo attacks
Not intended for acute vertigo episodes
Does not reduce the frequency or severity of vertigo attacks
Includes a range of exercises and maneuvers

Prognosis[8]
Acute attacks tend to cease after 5–15 years
Typically will still have constant symptoms of:
Dysequilibrium
Tinnitus
Moderate hearing loss

Differential Diagnosis

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Migraine (migrainous vertigo or vestibular migraine): condition associated with

headache and sometimes vertigo. Migraines are usually accompanied by
photophobia or phonophobia not seen in Ménière disease. For diagnosis,
episodic vestibular symptoms and at least 2 migraine symptoms (migraine
headache, photophobia, phonophobia, or visual or other aura) occurring during
at least 2 vertiginous episodes should be present.
Vestibular schwannoma: presents with progressive asymmetric hearing loss
with occasional fluctuating hearing loss. Patients rarely have true vertigo but
often complain of imbalance and disequilibrium. Patients may occasionally have
tinnitus and imbalance but no hearing loss. Auditory brainstem response testing
and MRI show abnormalities indicating nerve compression of the 8th cranial
nerve.
Multiple sclerosis (MS): characterized by disseminated patches of
demyelination in the brain and spinal cord. Patients can present with symptoms
identical to those of Ménière disease. However, during an attack of MS with
vertigo, the nystagmus is typically more severe and longer-lasting than in
patients with Ménière disease. Patients with MS also may have additional
neurologic complaints. On ENG testing, central abnormalities are often seen in
patients with MS but are typically absent in early Ménière disease. In MS, white-
matter lesions may be seen on brain MRI and CSF abnormalities may be
detected, but such findings are absent in patients with Ménière disease.
Benign paroxysmal positional vertigo (BPPV): condition characterized by
perceived rotatory motion. The condition is associated with vertigo related to
head movements, lasting seconds to minutes with no aural symptoms. Hearing
symptoms are absent, unlike in Ménière disease.

References

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26/4/24, 11:41 Ménière Disease (Clinical) - Lecturio

1. Meyerhoff, W. L., Paparella, M. M., Shea, D. (1978). Ménière's disease in children. Laryngoscope.
https://pubmed.ncbi.nlm.nih.gov/682806/
2. Basura, G., et al. (2020). Clinical practice guideline: Ménière's disease executive summary.
Otolaryngology—Head and Neck Surgery, 162(4), 415–434.
https://pubmed.ncbi.nlm.nih.gov/32267820/
3. Coelho, D. H., Lalwani, A. K. (2008). Medical management of Ménière's disease. Laryngoscope.
https://pubmed.ncbi.nlm.nih.gov/18418279/
4. Lustig, L. R. (2021). Meniere disease. MSD Manual Professional Version. Retrieved September 14,
2021, from https://www.msdmanuals.com/professional/ear,-nose,-and-throat-disorders/inner-ear-
disorders/meniere-disease
5. Li, J. C. (2020). Meniere disease (idiopathic endolymphatic hydrops). Medscape. Retrieved
September 14, 2021, from https://emedicine.medscape.com/article/1159069
6. Koenen, L., Andaloro, C. Meniere disease. (2021). StatPearls. Retrieved October 5, 2021, from
https://www.ncbi.nlm.nih.gov/books/NBK536955/
7. Lopez-Escamez, J. A., Carey, J., Chung, W.-H., Goebel, J. A., Magnusson, M., Mandalà, M.,
Newman-Toker, D. E., Strupp, M., Suzuki, M., Trabalzini, F., Bisdorff, A. (2015). Diagnostic criteria for
Menière’s disease. Journal of Vestibular Research, 25(1), 1–7. https://doi.org/10.3233/VES-150549
8. Harcourt, J., Barraclough, K., Bronstein, A. M. (2014). Meniere’s disease. BMJ, 349, g6544–g6544.
https://doi.org/10.1136/bmj.g6544
9. Magnan, J., Özgirgin, O. N., Trabalzini, F. ,et al. (2018). European position statement on diagnosis,
and treatment of Meniere's disease. Journal of International Advanced Otology, 14(2), 317–321.
https://doi.org/10.5152/iao.2018.140818
10. Swartz, R., Longwell, P. (2005). Treatment of vertigo. American Family Physician, 71(6), 1115–1122.
https://www.aafp.org/pubs/afp/issues/2005/0315/p1115.html
11. Ghossaini, S. N. (2022). Meniere’s disease: symptoms, diagnosis, and treatment. BMJ Best
Practice. https://bestpractice.bmj.com/topics/en-gb/155
12. National Institute for Health and Care Excellence. (2020). Tinnitus: assessment and management.
Retrieved June 6, 2023, from
https://www.nice.org.uk/guidance/ng155/chapter/Recommendations#support-and-information-for-
people-with-tinnitus

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