Inner Ear, Meniere Disease, Medical Treatment 

  • Author: John C Li, MD; Chief Editor: Arlen D Meyers, MD, MBA   more...
 
Updated: May 18, 2011
 

Background

Evaluation and management of dizziness and vertigo can be one of the most difficult medical tasks. Sources of imbalance can range from simple conditions (eg, dehydration) to serious conditions (eg, brain tumors). CNS problems must be distinguished from circulation anomalies, chemical and hormonal imbalances, and peripheral inner ear disorders. Often, this distinction is not clear.

This article covers a form of peripheral inner ear disorders, specifically, endolymphatic hydrops and Ménière syndrome.

Endolymphatic hydrops refers to a condition of increased hydraulic pressure within the inner ear endolymphatic system. Excess pressure accumulation in the endolymph can cause a tetrad of symptoms: (1) fluctuating hearing loss, (2) occasional episodic vertigo (usually a spinning sensation, sometimes violent), (3) tinnitus or ringing in the ears (usually low-tone roaring), and (4) aural fullness (eg, pressure, discomfort, fullness sensation in the ears).

Tinnitus model. Two phenomena in the auditory cortTinnitus model. Two phenomena in the auditory cortex are associated with peripheral deafferentation: 1) hyperactivity in the lesion projection zone and 2) increased cortical representation of the lesion-edge frequencies (here, C6) in the lesion projection zone. These two phenomena are presumed to be the neurophysiological correlates of tinnitus. The red letters correspond to octave intervals of a fundamental frequency.

The term endolymphatic hydrops is often used synonymously with Ménière disease and Ménière syndrome. Ménière disease and Ménière syndrome are both believed to result from increased pressure within the endolymphatic system. However, Ménière disease is idiopathic, whereas Ménière syndrome can occur secondary to various processes interfering with normal production or resorption of endolymph (eg, endocrine abnormalities, trauma, electrolyte imbalance, autoimmune dysfunction, medications, parasitic infections, hyperlipidemia).

The distinction in nomenclature is similar to that of Bell palsy. When the source of facial paralysis is known, Bell palsy is not the diagnosis. Similarly, when the cause of vertigo is known, Ménière disease is not the diagnosis; the diagnosis is Ménière syndrome.

In other words, Ménière syndrome refers to endolymphatic hydrops caused by a specific condition (eg, thyroid hormone disease, inner ear inflammation due to syphilis). Ménière disease refers to endolymphatic hydrops with unknown etiology; it is the catch term for idiopathic endolymphatic hydrops.

Next

Pathophysiology

Two fluids fill the chambers of the inner ear: endolymph and perilymph. These fluids are separated by thin membranes, which house the nervous tissue of hearing and balance. Fluctuations in pressure of these fluids stress these nerve-rich membranes and can cause hearing disturbance, ringing in the ears, vertigo, imbalance, and a pressure sensation in the ear.

More specifically, episodes of hydrops are probably caused by an increase in endolymphatic pressure that causes a break in the membrane separating the perilymph (a potassium-poor extracellular fluid) and the endolymph (a potassium-rich intracellular fluid). The resultant chemical mixture bathes the vestibular nerve receptors, leading to depolarization blockade and transient function loss. Sudden change in the vestibular nerve firing rate creates an acute vestibular imbalance, giving the sense of vertigo.

Physical distention caused by the increase in endolymphatic pressure leads to mechanical disturbance of the otolithic organs. Because the utricle and saccule are responsible for linear and translational motion detection, rather than angular and rotational acceleration, irritation of these organs may produce nonrotational vestibular symptoms.

This physical distention causes mechanical disturbance of the organ of Corti as well. Distortion of the basilar membrane and the inner and outer hair cells may cause hearing loss and tinnitus. Because the apex of the cochlea is wound much tighter than the base, the apex is more sensitive to pressure changes than is the base. This explains why hydrops affects low frequencies (located at the apex) more than high frequencies (located at the relatively wider base of the cochlea).

Symptoms improve after the membrane is repaired and normal sodium and potassium concentrations are restored.

Previous
Next

Epidemiology

Frequency

United States

Although probably underestimated, approximately 1,000 cases per 100,000 people occur. A familial predisposition seems to exist; approximately half of the patients have a notable family history of this disease.

Mortality/Morbidity

Although the disease itself is not fatal, significant morbidity can arise from various manifestations of the disease. Vertigo can cause devastating accidents and falls. Hearing loss is often progressive over time. Many patients are unable to continue working and are forced to claim disability.

Sex

Although sex-related rates are nearly equal, a slight female preponderance may exist, in the range of 50-65%.

Age

  • In some studies, the mean age among treatment groups was 49-67 years.
  • Ménière disease has been seen at almost all ages.
  • Typical onset begins at early-to-middle adulthood.
Previous
 
 
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.

Specialty Editor Board

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

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

Disclosure: eMedicine Salary Employment

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

Christopher L Slack, MD  Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders

Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association

Disclosure: Nothing to disclose.

Chief Editor

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

References
  1. Paparella MM. Benign paroxysmal positional vertigo and other vestibular symptoms in Ménière disease. Ear Nose Throat J. Oct 2008;87(10):562. [Medline].

  2. Pullens B, van Benthem PP. Intratympanic gentamicin for Ménière's disease or syndrome. Cochrane Database Syst Rev. Mar 16 2011;3:CD008234. [Medline].

  3. Monsell EM. New and revised reporting guidelines from the Committee on Hearing and Equilibrium. American Academy of Otolaryngology-Head and Neck Surgery Foundation, Inc. Otolaryngol Head Neck Surg. Sep 1995;113(3):176-8. [Medline].

  4. Bretlau P, Thomsen J, Tos M, et al. Placebo effect in surgery for Menière's disease: nine-year follow-up. Am J Otol. Jul 1989;10(4):259-61. [Medline].

  5. Densert B, Sass K. Control of symptoms in patients with Meniere's disease using middle ear pressure applications: two years follow-up. Acta Otolaryngol. Jul 2001;121(5):616-21. [Medline].

  6. Glasscock ME 3rd, Jackson CG, Poe DS, et al. What I think of sac surgery in 1989. Am J Otol. May 1989;10(3):230-3. [Medline].

  7. Gottshall KR, Hoffer ME, Moore RJ, et al. The role of vestibular rehabilitation in the treatment of Meniere's disease. Otolaryngol Head Neck Surg. Sep 2005;133(3):326-8. [Medline].

  8. Kato BM, LaRouere MJ, Bojrab DI, et al. Evaluating quality of life after endolymphatic sac surgery: The Ménière's Disease Outcomes Questionnaire. Otol Neurotol. May 2004;25(3):339-44. [Medline].

  9. Kitahara T, Kondoh K, Morihana T, et al. Surgical management of special cases of intractable Meniere's disease: unilateral cases with intact canals and bilateral cases. Ann Otol Rhinol Laryngol. May 2004;113(5):399-403. [Medline].

  10. Monsell EM, Wiet RJ. Endolymphatic sac surgery: methods of study and results. Am J Otol. Sep 1988;9(5):396-402. [Medline].

  11. Odkvist LM, Arlinger S, Billermark E, et al. Effects of middle ear pressure changes on clinical symptoms in patients with Ménière's disease--a clinical multicentre placebo-controlled study. Acta Otolaryngol Suppl. 2000;543:99-101. [Medline].

  12. Pyykkö I, Ishizaki H, Kaasinen S, et al. Intratympanic gentamicin in bilateral Menière's disease. Otolaryngol Head Neck Surg. Feb 1994;110(2):162-7. [Medline].

  13. Shea JJ Jr. Classification of Menière's disease. Am J Otol. May 1993;14(3):224-9. [Medline].

  14. Shea JJ Jr, Ge X. Streptomycin perfusion of the labyrinth through the round window plus intravenous streptomycin. Otolaryngol Clin North Am. Apr 1994;27(2):317-24. [Medline].

  15. Silverstein H, Lewis WB, Jackson LE, et al. Changing trends in the surgical treatment of Ménière's disease: results of a 10-year survey. Ear Nose Throat J. Mar 2003;82(3):185-7, 191-4. [Medline].

  16. Silverstein H, Smouha E, Jones R. Natural history vs. surgery for Menière's disease. Otolaryngol Head Neck Surg. Jan 1989;100(1):6-16. [Medline].

Previous
Next
 
Tinnitus model. Two phenomena in the auditory cortex are associated with peripheral deafferentation: 1) hyperactivity in the lesion projection zone and 2) increased cortical representation of the lesion-edge frequencies (here, C6) in the lesion projection zone. These two phenomena are presumed to be the neurophysiological correlates of tinnitus. The red letters correspond to octave intervals of a fundamental frequency.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.