Ménière Disease

Ménière disease is a condition characterized by episodes of vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo, tinnitus, and hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss, likely caused by endolymphatic hydrops of the labyrinthine system in the inner ear. The risk factors include a family history of Ménière disease, preexisting autoimmune disorders, allergies, and trauma to the head or ear. A diagnosis is made clinically, by audiometry, by vestibular testing, and occasionally by imaging. Management can be by diet and lifestyle modification, vasodilators, diuretics, antihistamines Antihistamines Antihistamines are drugs that target histamine receptors, particularly H1 and H2 receptors. H1 antagonists are competitive and reversible inhibitors of H1 receptors. First-generation antihistamines cross the blood-brain barrier and can cause sedation. Antihistamines, benzodiazepines Benzodiazepines Benzodiazepines work on the gamma-aminobutyric acid type A (GABAA) receptor to produce inhibitory effects on the CNS. Benzodiazepines do not mimic GABA, the main inhibitory neurotransmitter in humans, but instead potentiate GABA activity. Benzodiazepines, antiemetics Antiemetics Antiemetics are medications used to treat and/or prevent nausea and vomiting. These drugs act on different target receptors. The main classes include benzodiazepines, corticosteroids, atypical antipsychotics, cannabinoids, and antagonists of the following receptors: serotonin, dopamine, and muscarinic and neurokinin receptors. Antiemetics, glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids, surgical intervention, or hearing aids.

Last update:

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Table of Contents

Share this concept:

Share on facebook
Share on twitter
Share on linkedin
Share on reddit
Share on email
Share on whatsapp

Overview

Definition

Ménière disease is a triad of episodic vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo, tinnitus, and hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss likely caused by endolymphatic hydrops of the labyrinthine system of the inner ear.

Ménière syndrome is Ménière disease occurring secondary to other inner ear infections.

Epidemiology

  • 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

  • Family history of Ménière disease
  • Preexisting autoimmune disorders
  • Congenital inner ear malformations
  • Allergies
  • Trauma to the head or ear
  • Syphilis Syphilis Syphilis is a bacterial infection caused by the spirochete Treponema pallidum pallidum (T. p. pallidum), which is usually spread through sexual contact. Syphilis has 4 clinical stages: primary, secondary, latent, and tertiary. Syphilis

Pathophysiology

  • Endolymph and perilymph are separated by thin membranes that contain the neural apparatus of hearing and balance.
  • Pressure fluctuations cause stress on the nerve-rich membranes → hearing disturbances, tinnitus, vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo, imbalance, and a pressure sensation in the inner ear
  • Increase in endolymphatic pressure → breaks in the membrane separating perilymph from endolymph
  • The resultant chemical mixture bathes the vestibular nerve receptors → depolarization blockade and transient loss of function
  • Sudden change in the rate of vestibular nerve firing creates an acute vestibular imbalance.
  • Increased endolymphatic pressure → physical distension → mechanical disruption of auditory and otolithic organs
  • The utricle and saccule are responsible for linear and translational motion detection.
  • Irritation produces nonrotational vestibular symptoms.
  • Also physical distension → mechanical disturbance of organ of Corti → distortion of the basilar membrane and the inner and outer hair cells → hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss and/or tinnitus 
  • The apex of the cochlea is wound up tighter than the base → apex more sensitive to pressure changes than the base
The internal ear

Anatomy of the inner ear

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

Clinical Presentation

  • Vertigo:
    • Perceived rotatory spinning or rocking motion while remaining still
    • Sometimes associated with nausea and vomiting lasting 20 minutes to 24 hours
  • Hearing loss: initially intermittent, then progresses to permanent hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss
  • Tinnitus:
    • Perceived noises (usually high-pitched ringing) that are not actually present
    • Fluctuating or constant with variable Variable Variables represent information about something that can change. The design of the measurement scales, or of the methods for obtaining information, will determine the data gathered and the characteristics of that data. As a result, a variable can be qualitative or quantitative, and may be further classified into subgroups. Types of Variables pitch and intensity

Diagnosis

Diagnostic criteria

Clinical diagnosis made by the presence of:

  • 2 or more spontaneous episodes of vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo, each lasting 20 minutes to 12 hours
  • Audiometrically documented low- to mid-frequency sensorineural hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss in the affected ear
  • Fluctuating aural symptoms (reduced or distorted hearing, tinnitus, or fullness) in the affected ear
  • Symptoms not better accounted for by another vestibular diagnosis

Testing

  • Audiometry
    • For all patients
    • Most common pattern:
      • Low-frequency sensorineural hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss 
      • Combined low- and high-frequency sensorineural hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss
      • Normal hearing in the middle frequencies
    • With time, hearing “flattens out” with hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss across all frequency ranges. 
  • Vestibular testing
    • May be normal early in the course of the disease
    • Abnormal as disease advances on the affected side
    • Include electronystagmography (ENG), rotary chair testing, and computerized dynamic posturography 
  • Imaging
    • MRI of the temporal bone Bone Bone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy. Structure of Bones: can identify supporting features (e.g., less prominent endolymphatic duct)
    • MRI/CT brain: CNS lesions

Management

Treatment improves and relieves symptoms but does not correct the underlying pathophysiology.

Goals of treatment

  • Reducing frequency and severity of vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo attacks
  • Reducing or eliminating the tinnitus and hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss associated with attacks
  • Alleviating chronic symptoms of tinnitus and disequilibrium
  • Minimizing disability associated with Ménière disease
  • Preventing progressive hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss

Initial diet and lifestyle management

  • Salt restriction
  • Limiting caffeine and alcohol to 1 drink daily
    • Caffeine and nicotine are vasoconstrictors reducing microvascular flow in the labyrinthine system.
    • Alcohol causes fluid and electrolyte shifts, stressing a vulnerable ear.

Vestibular rehabilitation for residual disequilibrium between attacks

  • Used in:
    • Patients with response to surgical or medical treatment with residual disequilibrium
    • Patients with significant balance and disequilibrium symptoms between acute vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo attacks
  • Does not reduce the frequency or severity of vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo attacks

Pharmacotherapy for patients with refractory symptoms

  • Goal: Reduce the severity and intensity of attacks.
  • Chronic management:
    • Betahistine: vasodilator (preferred because it is well tolerated)
    • Hydrochlorothiazide: diuretic
    • Furosemide: diuretic
    • Acetazolamide: diuretic
  • Acute management:
    • Antihistamines: reduce swelling and improve blood flow to inner ear
      • Dimenhydrinate
      • Diphenhydramine
      • Meclizine
    • Benzodiazepines: vestibular suppression
      • Alprazolam
      • Clonazepam
      • Diazepam
      • Lorazepam
    • Antiemetics: suppress associated nausea
      • Domperidone
      • Metoclopramide
      • Ondansetron
      • Prochlorperazine
      • Promethazine
  • Refractory, disabling symptoms despite initial pharmacotherapy:
    • Continue betahistine or diuretics, if beneficial.
    • Glucocorticoid therapy for all patients
      • Systemic glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids: prednisone
      • Intratympanic glucocorticoids Glucocorticoids Glucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs. Glucocorticoids: dexamethasone
  • Refractory, disabling symptoms despite glucocorticoid therapy:
    • 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 for hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss:
    • Patients with significant binaural hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss
    • In early disease, hearing fluctuation leads to frustration with amplification devices and poor patient compliance.

Differential Diagnosis

  • Migraine Migraine Migraine headache is a primary headache disorder and is among the most prevalent disorders in the world. Migraine is characterized by episodic, moderate to severe headaches that may be associated with increased sensitivity to light and sound, as well as nausea and/or vomiting. Migraine Headache (migrainous vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo or vestibular migraine): condition associated with headache and sometimes vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. 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 Migraine Headache Migraine headache is a primary headache disorder and is among the most prevalent disorders in the world. Migraine is characterized by episodic, moderate to severe headaches that may be associated with increased sensitivity to light and sound, as well as nausea and/or vomiting. Migraine Headache, photophobia, phonophobia, or visual or other aura) occurring during at least 2 vertiginous episodes should be present. 
  • Vestibular schwannoma Vestibular schwannoma Acoustic neuroma, also referred to as vestibular schwannoma, is a benign tumor arising from Schwann cells of the vestibular component of the cranial nerve VIII. Acoustic neuroma forms within the internal auditory meatus and extends into the cerebellopontine angle. Acoustic Neuroma: presents with progressive asymmetric hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss with occasional fluctuating hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss. Patients rarely have true vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo but often complain of imbalance and disequilibrium. Patients may occasionally have tinnitus and imbalance but no hearing loss Hearing loss Hearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point. Hearing Loss. Auditory brainstem response testing and MRI show abnormalities indicating nerve compression of the 8th cranial nerve.  
  • Multiple sclerosis Multiple Sclerosis Multiple sclerosis (MS) is a chronic inflammatory autoimmune disease that leads to demyelination of the nerves in the CNS. Young women are more predominantly affected by this most common demyelinating condition. Multiple Sclerosis (MS): characterized by disseminated patches of demyelination in the brain and spinal cord Spinal cord The spinal cord is the major conduction pathway connecting the brain to the body; it is part of the CNS. In cross section, the spinal cord is divided into an H-shaped area of gray matter (consisting of synapsing neuronal cell bodies) and a surrounding area of white matter (consisting of ascending and descending tracts of myelinated axons). Spinal Cord. Patients can present with symptoms identical to those of Ménière disease. However, during an attack of MS with vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. 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 Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo (BPPV): condition characterized by perceived rotatory motion. The condition is associated with vertigo Vertigo Vertigo is defined as the perceived sensation of rotational motion while remaining still. A very common complaint in primary care and the ER, vertigo is more frequently experienced by women and its prevalence increases with age. Vertigo is classified into peripheral or central based on its etiology. Vertigo related to head movements, lasting seconds to minutes with no aural symptoms. Hearing symptoms are absent, unlike in Ménière disease.

References

  1. Meyerhoff, WL, Paparella, MM, & 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. Otolaryngol Head Neck Surg. 162(4), 415-434. https://pubmed.ncbi.nlm.nih.gov/32267820/ 
  3. Coelho, DH, & Lalwani, AK. (2008). Medical management of Ménière’s disease. Laryngoscope. https://pubmed.ncbi.nlm.nih.gov/18418279/ 
  4. Lustig, LR. (2021). Meniere disease. [Online] 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, JC. (2020). Meniere disease (Idiopathic endolymphatic hydrops). In Lorenzo, N, et al. (Eds.), Medscape. Retrieved September 14, 2021, from https://emedicine.medscape.com/article/1159069
  6. Koenen, L, & Andaloro, C. Meniere Disease. [Updated 2021 Jul 19]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Retrieved October 5, 2021, from https://www.ncbi.nlm.nih.gov/books/NBK536955/

Study on the Go

Lecturio Medical complements your studies with evidence-based learning strategies, video lectures, quiz questions, and more – all combined in one easy-to-use resource.

Learn even more with Lecturio:

Complement your med school studies with Lecturio’s all-in-one study companion, delivered with evidence-based learning strategies.

User Reviews

0.0

()

¡Hola!

Esta página está disponible en Español.

🍪 Lecturio is using cookies to improve your user experience. By continuing use of our service you agree upon our Data Privacy Statement.

Details