Meniere Disease (Idiopathic Endolymphatic Hydrops) Workup

  • Author: John C Li, MD; Chief Editor: Robert A Egan, MD   more...
 
Updated: Sep 15, 2011
 

Approach Considerations

Laboratory tests, though not specific for Ménière disease, should be directed at differentiating the disease from other causes on the basis of associated symptoms. More extensive testing is typically reserved for outpatient or inpatient workup and is not performed in the emergency department (ED), including the following otologic tests:

  • Audiometry
  • Brainstem auditory evoked potentials
  • Electrocochleography (ECOG)
  • Otoscopy
  • Caloric testing/electronystagmography (ENG)

A patient with a history classic for Ménière disease normally does not need imaging studies performed. If there is concern about the presence of other intracranial disease processes, then magnetic resonance imaging (MRI) or computed tomography (CT) can be obtained.

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Laboratory Studies

No blood test is specific for Ménière disease. However, the following studies may be ordered to exclude obvious metabolic disturbances, infections, or hormonal imbalances:

  • Thyroid-stimulating hormone (TSH), T4, and T3 to rule out hyperthyroidism and hypothyroidism
  • Glucose level to rule out diabetes
  • Erythrocyte sedimentation rate (ESR) and antinuclear antibody (ANA) test to rule out autoimmune disorders
  • Urinalysis to rule out proteinuria and hematuria and indicators of otorenal syndrome
  • Complete blood count (CBC) to rule out anemia and leukemia
  • Electrolyte levels to rule out salt/water imbalance
  • Venereal Disease Research Laboratory test (VDRL) and fluorescent treponemal antibody (FTA-ABS) to rule out neurosyphilis and Lyme disease
  • Allergy testing for allergy-mediated Ménière syndrome
  • C-reactive protein (CRP)
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MRI and CT Scanning

Although usually unnecessary when the patient has a classic history indicative of Ménière disease, MRI or CT may be useful when it is deemed important to identify or exclude other potential disease processes.

MRI of the brain should be done to rule out abnormal anatomy or mass lesions. Specifically, acoustic neuromas or other cerebellopontine angle lesions are sought. Other lesions, such as multiple sclerosis or Arnold-Chiari malformations, also can be ruled out.[23] Note that mass lesions rarely are found but are important to exclude.

CT scans should be normal. They are obtained to detect possible dehiscences of the semicircular canals, congenital abnormalities, widened cochlear and vestibular aqueducts, and subarachnoid hemorrhage. Whereas CT scans are useful at imaging the anatomy of temporal bone structures, specific findings and their association with Ménière disease remain subject to debate.[24]

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Audiometry

Audiometry is particularly helpful for documenting present hearing acuity and detecting future change. In any given patient, the audiogram may have a broad spectrum of results that ranges from normal hearing to profound hearing loss, reflecting the fluctuating nature of the impairment. From patient to patient, there is also a wide range of different types of hearing loss.

The patient may not notice a loss at specific frequencies. Low-frequency or mixed low- and high-frequency insufficiency may be observed. Typically, however, the lower frequencies are affected more severely. This is due to preferential sensitivity of the apex to the hydrops.

Multiple hearing tests, which document fluctuating hearing loss, are helpful in diagnosing Ménière.

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Electrocochleography

ECOG is an electrophysiological test that reflects elevation of inner ear pressure. Specifically, it detects distention of the basilar membrane of the inner ear. This distortion is presumably due to elevated endolymph pressure associated with hydrops. The pressure may cause the membranes to tear and the inner ear to misfire, causing vertigo.

ECOG measures the ratio of the summating potential (probably from the movement of the basilar membrane) and the nerve action potential in response to auditory stimuli. Hydrops (elevated pressure) is suggested when this ratio is greater than 35%. The test is most accurate when Ménière disease is active.

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Electronystagmography

ENG is a test of inner ear function (particularly the horizontal semicircular canals). The test determines inner ear responsiveness to movement and caloric stimulation. It tests central and peripheral function and can help localize the site of the lesion.

Administer the test when the patient has an empty stomach and after discontinuing meclizine (Antivert), antihistamines, and sedatives for 2 weeks. These drugs may alter test results.

The caloric portion of the test is performed by “irrigating” the ear with warm air and then cold air, with the patient in a supine position. The temperature differential causes the fluid within the horizontal semicircular canal to move, triggering a nystagmus response. The response on one side is compared with the opposite side. As with any reflex, one would expect equal reactions on each side. Usually, anything that would cause a weakened response would be considered pathological.

Typically, endolymphatic hydrops causes a reduced vestibular response in the affected ear, although response may be paradoxically increased secondary to an irritative lesion. The patient may feel dizzy or nauseated.

The direction of the spontaneous nystagmus during or after an attack of Ménière disease is not always a reliable indicator of the site of the lesion. In general, the nystagmus points away from the affected ear because Ménière's disease typically weakens the vestibular response in the affected ear. However, an irritative phase may occur during the attack (fast phases directed toward the involved ear), followed by a paretic phase (fast phases directed toward opposite ear). Since the examiner may not know exactly what phase the patient is in, it is difficult to definitively determine this side of lesion.

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Contributor Information and Disclosures
Author

John C Li, MD  Private Practice in Otology and Neurotology; Medical Director, Balance Center

John C Li, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Medical Association, American Neurotology Society, American Tinnitus Association, Florida Medical Association, and North American Skull Base Society

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher I Doty, MD, FACEP, FAAEM  Assistant Professor of Emergency Medicine, Residency Program Director, Department of Emergency Medicine, Kings County Hospital Center, State University of New York Downstate Medical Center

Christopher I Doty, MD, FACEP, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, Council of Emergency Medicine Residency Directors, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Gerard J Gianoli, MD  Clinical Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Tulane University School of Medicine; Vice President, The Ear and Balance Institute; Chief Executive Officer, Ponchartrain Surgery Center

Gerard J Gianoli, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society, American Otological Society, Society of University Otolaryngologists-Head and Neck Surgeons, and Triological Society

Disclosure: Vesticon, Inc. None Board membership

Michael E Hoffer, MD  Director, Spatial Orientation Center, Department of Otolaryngology, Naval Medical Center of San Diego

Michael E Hoffer, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery

Disclosure: American biloogical group Royalty Other

J Stephen Huff, MD  Associate Professor of Emergency Medicine and Neurology, Department of Emergency Medicine, University of Virginia School of Medicine

J Stephen Huff, MD is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Neurology, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Nicholas Lorenzo, MD  Consulting Staff, Neurology Specialists and Consultants

Nicholas Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and American College of Physician Executives

Disclosure: Nothing to disclose.

Arlen D Meyers, MD, MBA  Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine

Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society

Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation Unrestricted gift Unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Syndicom Ownership interest Consulting; Oxlo Consulting; Medvoy Ownership interest Management position; Cerescan Imaging Honoraria Consulting; GYRUS ACMI Honoraria Consulting

Mark S Slabinski, MD, FACEP, FAAEM  Vice President, EMP Medical Group

Mark S Slabinski, MD, FACEP, FAAEM is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, American Medical Association, and Ohio State Medical Association

Disclosure: Nothing to disclose.

R Gentry Wilkerson, MD  Assistant Professor, Director of Research, Emergency Medicine Residency Program, University of South Florida College of Medicine, Tampa General Hospital

R Gentry Wilkerson, MD is a member of the following medical societies: American College of Emergency Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Spiros Manolidis, MD  Associate Professor of Otolaryngology and Neurological Surgery, Columbia University

Spiros Manolidis, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American Head and Neck Society, American Medical Association, Canadian Society of Otolaryngology-Head & Neck Surgery, Society of University Otolaryngologists-Head and Neck Surgeons, and Texas Medical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Glenn Lopate, MD  Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Director of Neurology Clinic, St Louis ConnectCare; Consulting Staff, Department of Neurology, Barnes-Jewish Hospital

Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa

Disclosure: Baxter Grant/research funds Other; Amgen Grant/research funds None

Chief Editor

Robert A Egan, MD  Director of Neuro-Ophthalmology, St Helena Hospital

Robert A Egan, MD is a member of the following medical societies: American Academy of Neurology, American Heart Association, North American Neuro-Ophthalmology Society, and Oregon Medical Association

Disclosure: Nothing to disclose.

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Transtympanic instillation device is used to administer drugs to inner ear.
Intraoperative view of the left ear treated with labyrinthectomy; endolymphatic sac can be seen in this view.
Tinnitus model. Two phenomena in auditory cortex are associated with peripheral deafferentation: (1) hyperactivity in lesion projection zone and (2) increased cortical representation of lesion-edge frequencies (here, C6) in lesion projection zone. These 2 phenomena are presumed to be neurophysiologic correlates of tinnitus. Red letters correspond to octave intervals of fundamental frequency.
 
 
 
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