Ménière’s Disease

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Ménière’s Disease

Otology/Audiology
Author Credentials 

Jeffrey T Vrabec, MD
Professor
Otolaryngology - Head and Neck Surgery
Baylor College of Medicine
Houston, TX

Module Summary

Meniere’s disease remains a challenge for otolaryngologists as the diagnosis hinges upon careful history taking and thorough knowledge of defining criteria.  Diagnostic testing may provide supportive evidence but there is no definitive test.  Conservative treatment is advised as there is expectation of decreasing symptoms over time.  Patients that require more significant intervention are managed with intratympanic therapies, reserving ablation for those with the most severe vertigo. 

Module Learning Objectives 
  1. Develop familiarity of clinical criteria for diagnosis of Meniere’s disease.
  2. Discuss theories of pathogenesis including strengths and weaknesses of each.
  3. Formulate treatment plans with best available evidence from clinical studies. 

Pathogenesis

Learning Objectives 

Understand that the etiology of Meniere’s disease is unconfirmed. Multiple theories have been proposed:

  1. Autoimmunity
  2. Allergy
  3. Viral reactivation
  4. Vascular 
  5. Electrolyte imbalance
References 
  1. Paparella MM, Djalilian HR. Etiology, pathophysiology of symptoms, and pathogenesis of Meniere’s disease. Otolaryngol Clin North Am. 2002;35:529-45
  2. Nakashima T, Pyykkö I, Arroll MA, Casselbrant ML, Foster CA, Manzoor NF, Megerian CA, Naganawa S, Young YH. Meniere's disease. Nat Rev Dis Primers. 2016 May 12;2:16028
  3. Derebery MJ, Berliner KI. Allergy and its relation to Meniere's disease. Otolaryngol Clin North Am. 2010 Oct;43(5):1047-58
  4. Vrabec JT. Herpes simplex virus and Meniere's disease. Laryngoscope. 2003 Sep;113(9):1431-8

Basic Science

Learning Objectives 
  1. Discuss the significance of temporal bone histopathological finding of endolymphatic hydrops. Nearly all temporal bone from patients with MD display endolymphatic hydrops.  However, there are also many temporal bones with hydrops that do not have any history of clinical symptoms of Meniere’s disease. 
  2. Understand the value and limitations of animal models of endolymphatic hydrops. Surgical obstruction of the endolymphatic duct will produce hydrops in many animal species, but not in primates.  Animals with hydrops do not display recurrent vertigo or fluctuating hearing loss. The presence of hydrops likely reflects local ion transport dysfunction.  Early theories of endolymphatic hypertension and impaired endolymph secretion or resorption are not supported. 
References 
  1. Schuknecht HF: Pathology of the Ear. Philadelphia: Lea & Febiger 1993 2nd ed.
  2. Swart JG, Schuknecht HF. Long-term effects of destruction of the endolymphatic sac in a primate species.  Laryngoscope. 1988;98:1183-9 
  3. Merchant SN, Adams JC, Nadol JB Jr. Pathophysiology of Meniere's syndrome: are symptoms caused by endolymphatic hydrops? Otol Neurotol. 2005;26:74-81
  4. Salt AN, Plontke SK. Endolymphatic hydrops: pathophysiology and experimental models. Otolaryngol Clin North Am. 2010;43:971-83

Incidence

Learning Objectives 
  1. Discuss the epidemiology of Meniere’s disease. Population based studies find a prevalence of 1-2 /1000 persons, with increased prevalence in Caucasians and a slight female preponderance. 
References 
  1. Alexander TH, Harris JP. Current epidemiology of Meniere's syndrome.  Otolaryngol Clin North Am. 2010 Oct;43(5):965-70 
  2. Wladislavosky-Waserman P, Facer GW, Mokri B, Kurland LT. Meniere’s disease: a 30-year epidemiologic and clinical study in Rochester, Mn, 1951-1980.  Laryngoscope. 1984;94:1098-102
  3. Kotimaki J, Sorri M, Aantaa E, Nuutinen J.  Prevalence of Meniere disease in Finland. Laryngoscope. 1999;109:748-53

Genetics

Learning Objectives 
  1. Discuss current understanding of genetic aspects of Meniere’s disease. About 10% of Meniere’s disease cases are familial. Meniere’s disease is genetically complex and not due to a single gene defect. Sporadic and familial variants show genetic heterogeneity. 

 

References 
  1. Morrison AW, Johnson KJ. Genetics (molecular biology) and Meniere’s disease. Otolaryngol Clin North Am. 2002;35:497-516 
  2. Vrabec JT. Genetic investigations of Meniere's disease. Otolaryngol Clin North Am. 2010 Oct;43(5):1121-32
  3. Arweiler-Harbeck D, Horsthemke B, Jahnke K, Hennies HC. Genetic aspects of familial Meniere’s disease. Otol Neurotol. 2011;32(4):695–700

Patient Evaluation

Learning Objectives 
  1. Recite the clinical criteria for diagnosis of Meniere’s disease In order to be designated “definite” Meniere’s disease, a case must display two or more episodes of characteristic vertigo, documented hearing loss, presence of aural pressure or tinnitus in the affected ear and other causes of vertigo have been excluded.  Vertigo must be continuous for 20 minutes or longer. Hearing loss should be predominantly low frequency or asymmetric.  The average of 0.25, 0.5, and 1 kHz should be >15 dB more than the average of 1, 2, and 3 kHz; OR the average of 0.5, 1, 2, and 3 kHz is >20 dB more than the contralateral ear. 
  2. Understand that many patients will not have all the features of the disease. Approximately 20% of patients display a cochlear variant.  They only have hearing loss and no vertigo.

 

References 
  1. Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Meniere’s disease. Otolaryngol Head Neck Surg 1995;113:181-185
  2. Goebel JA. 2015 Equilibrium Committee Amendment to the 1995 AAO-HNS Guidelines for the Definition of Meniere’s Disease. Otolaryngol Head Neck Surg. 2016 Mar;154(3):403-4 
  3. Lopez-Escamez JA, Carey J, Chung WH, Goebel JA, Magnusson M, Mandalà M,Newman-Toker DE, Strupp M, Suzuki M, Trabalzini F, Bisdorff A; Classification Committee of the Barany Society; Japan Society for Equilibrium Research; European Academy of Otology and Neurotology (EAONO); Equilibrium Committee of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS); Korean Balance Society. Diagnostic criteria for Meniere’s disease. J Vestib Res. 2015;25(1):1-7
  4. House JW, Doherty JK, Fisher LM, Derebery MJ, Berliner KI. Meniere's disease: prevalence of contralateral ear involvement. Otol Neurotol. 2006 Apr;27(3):355-61

Measurement of Functional Status

Learning Objectives 
  1. To understand to role of audio vestibular testing in Meniere’s disease. Audiometry can support the diagnosis of Meniere’s disease when a characteristic pattern of hearing loss is noted. However, the hearing loss is never definitive for the diagnosis.  Vestibular testing will show variable results, though typically vestibular hypofunction is seen in advanced disease. Results of ECoG and VEMP are frequently abnormal in Meniere’s disease, though sensitivity and specificity are highly variable.  
References 
  1. Zhu Y, McPherson J, Beatty C, Driscoll C, Neff B, Eggers S, Shepard NT. Cervical VEMP threshold response curve in the identification of Meniere’s disease. J Am Acad Audiol. 2014 Mar;25(3):278-88
  2. Egami N, Ushio M, Yamasoba T, Yamaguchi T, Murofushi T, Iwasaki S. The diagnostic value of vestibular evoked myogenic potentials in patients with Meniere's disease. J Vestib Res. 2013;23(4-5):249-57
  3. Nguyen LT, Harris JP, Nguyen QT. Clinical utility of electrocochleography in the diagnosis and management of Meniere’s disease: AOS and ANS membership survey data. Otol Neurotol. 2010 Apr;31(3):455-9 

Imaging

Learning Objectives 
  1. Discuss the evolving role of imaging in Meniere’s disease.  Application of gadolinium to the middle ear allows enhanced visualization of the endolymph - perilymph border on MRI.   
References 
  1. Pyykkö I, Zou J, Poe D, Nakashima T, Naganawa S. Magnetic resonance imaging of the inner ear in Meniere's disease. Otolaryngol Clin North Am. 2010 Oct;43(5):1059-80.
  2. Nakashima T, Naganawa S, Sugiura M, Teranishi M, Sone M, Hayashi H, Nakata S, Katayama N, Ishida IM. Visualization of endolymphatic hydrops in patients with Meniere's disease. Laryngoscope. 2007 Mar;117(3):415-20.

Treatment

Learning Objectives 
  1. Understand the natural history of symptoms in Meniere’s disease.  Favorable response of many therapies for MD may reflect the tendency for decreasing symptoms over time. 
References 
  1. Grant IL, Welling DB  The treatment of hearing loss in Meniere’s disease. Otolaryngol Clin North Am. 1997;30:1123-44
  2. Ruckenstein MJ, Rutka JA, Hawke M.  The treatment of Meniere’s disease: Torok revisited. Laryngoscope. 1991;101:211-8 
  3. Silverstein H, Smouha E, Jones R. Natural history vs. surgery for Meniere’s disease. Otolaryngol Head Neck Surg. 1989;100:6-16
  4. Green JD Jr, Blum DJ, Harner SG. Longitudinal follow-up of patients with Meniere’s disease. Otolaryngol Head Neck Surg. 1991;104:783-8
  5. Friberg U, Stahle J, Svedberg A. The natural course of Meniere’s disease. Acta Otolaryngol Suppl 1984;406:72-77 

Medical Therapies

Learning Objectives 
  1. Discuss the utility of medications for acute symptoms of vertigo.  Vestibular suppressants provide relief from acute vertigo attacks.  Oral steroids are beneficial for acute symptoms
  2. Discuss the evidence for common medical therapies for Meniere’s disease. Frequently employed therapies include:
  • Low salt diet and diuretic
  • Betahistine
References 
  1. Adrion C, Fischer CS, Wagner J, Gürkov R, Mansmann U, Strupp M; BEMED Study Group. Efficacy and safety of betahistine treatment in patients with Meniere's disease: primary results of a long term, multicentre, double blind, randomised,placebo controlled, dose defining trial (BEMED trial). BMJ. 2016 Jan21;352:h6816
  2. Crowson MG, Patki A, Tucci DL. A Systematic Review of Diuretics in the Medical Management of Meniere’s Disease. Otolaryngol Head Neck Surg. 2016 May;154(5):824-34.
  3. Claes J, Van de Heyning PH. A review of medical treatment for Meniere’s disease. Acta Otolaryngol Suppl. 2000;544:34-9.

Surgical Therapies

Learning Objectives 
  1. Understand the different types of procedures that may be offered for vertigo control.  Surgical management may include minor in office procedures or more invasive temporal bone or intracranial procedures.  
  2. Discuss the advantages and limitations of each procedure.  Non ablative techniques include intratympanic steroid injections and sac surgery.  Ablative techniques include gentamicin injection, vestibular neurectomy and labyrinthectomy.  Efficacy of office based procedures has significantly reduced application of more invasive procedures.  
References 
  1. Crowson MG, Schulz K, Parham K, Vambutas A, Witsell D, Lee WT, Shin JJ, Pynnonen MA, Nguyen-Huynh A, Ryan SE, Langman A. Meniere’s Disease: A CHEER Database Study of Local and Regional Patient Encounter and Procedure Patterns. Otolaryngol Head Neck Surg. 2016 Jul;155(1):15-21.
  2. Syed MI, Ilan O, Nassar J, Rutka JA. Intratympanic therapy in Meniere's syndrome or disease: up to date evidence for clinical practice. Clin Otolaryngol. 2015 Dec;40(6):682-90.
  3. Thomsen J, Bonding P, Becker B, et al. The non-specific effect of endolymphatic sac surgery in treatment of Meniere’s disease: a prospective, randomized controlled study comparing “classic” endolymphatic sac surgery with the insertion of a ventilating tube in the tympanic membrane. Acta Otolaryngol (Stockh) 1998;118:769-73.
  4. Thomsen J, Bretlau P, Tos M, et al. Placebo effect in surgery for Meniere’s disease: three-year follow-up. Otolaryngol Head Neck Surg 1983;91:183-86.
  5. Welling DB, Nagaraja HN. Endolymphatic mastoid shunt: a reevaluation of efficacy. Otolaryngol Head Neck Surg 2000;122:340-45.
  6. Garduño-Anaya MA, Couthino De Toledo H, Hinojosa-González R, Pane-Pianese C,Ríos-Castañeda LC. Dexamethasone inner ear perfusion by intratympanic injection in unilateral Meniere’s disease: a two-year prospective, placebo-controlled, double-blind, randomized trial. Otolaryngol Head Neck Surg. 2005 Aug;133(2):285-94.
  7. Cohen-Kerem R, Kisilevsky V, Einarson TR, Kozer E, Koren G, Rutka JA. Intratympanic gentamicin for Menière's disease: a meta-analysis. Laryngoscope. 2004 Dec;114(12):2085-91.
  8. Boleas-Aguirre MS, Lin FR, Della Santina CC, Minor LB, Carey JP. Longitudinal results with intratympanic dexamethasone in the treatment of Meniere’s disease. Otol Neurotol. 2008;29:33-8.

Complications

Learning Objectives 
  1. Understand that vestibular disorders including Meniere’s disease are frequently associated with anxiety disorders and depression.  Amelioration or rehabilitation of the hearing loss and vertigo is beneficial in reducing concurrent anxiety. 
References 
  1. Anderson JP, Harris JP. Impact of Meniere’s disease on quality of life. Otol Neurotol 2001;22:888-94.
  2. van Esch BF, van Benthem PP, van der Zaag-Loonen HJ, Bruintjes TD. Two Common Second Causes of Dizziness in Patients With Meniere’s Disease. Otol Neurotol.  2016 Dec;37(10):1620-1624. 
  3. Best C, Eckhardt-Henn A, Tschan R, Dieterich M. Psychiatric morbidity and comorbidity in different vestibular vertigo syndromes. Results of a prospective longitudinal study over one year. J Neurol. 2009 Jan;256(1):58-65.

Review

Review Questions 
  1. Discuss the criteria for diagnosis of definite Meniere’s disease.
  2. Discuss the expected outcomes for intratympanic medications, emphasizing the differences between steroids and gentamicin.

 

Resources 

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