MÉNIÈRE’S

DISEASE
PRESENTER: BONKO NEVILLE

FEBRUARY 2023
 CASE PRESENTATION
• L M is a 34-year-old female
• vertigo 11 months prior to
consultation, accompanied by N/V
• remitted spontaneously.
• 8 months later left-sided, low-
pitched, "buzzing" tinnitus.
• In the next 4 months, severe
rotational vertigo, accompanied by
N/V, diaphoresis, and pallor.
• frequently associated with left aural
fullness
• Her hearing fluctuated from day to
day.
• She had no dysarthria, dysphagia,
visual change, numbness and tingling
in the extremities, or focal motor
weakness.
• There was no history of syncope or
headache.
• MRI scan was obtained to rule out
retrocochlear pathology, she was
started on diuretics and a low-salt diet
QUESTIONS

• What further enquiry will

you do?
• What is your diagnosis?
• Was the management
adequate?
 1. Definition
2. Anatomy and physiology review
3. History
4. Epidemiology
5. Pathogenesis/etiology
OUTLINE 6. Natural history
7. Symptoms & signs
8. Diagnosis
9. Treatment
10.Future & controversies
 • Meniere's disease is a chronic disorder
of inner ear that affects hearing and
balance and presents with episodes of
vertigo, tinnitus,(fluctuant) hearing
loss and fullness in the ear.
DEFINITION
• This disease was first described by
Prosper Meniere in 1861 (1).

• Meniere’s disease vs Meniere’s

syndrome???
1.Seo YJ et al. Auris, nasus, larynx. 2013
 RELEVANT ANATOMY
AND PHYSIOLOGY
•Endolymphatic sac-proximal, intermediate
and distal portions

•Endolymph-stria vascularis, dark cells of

the vestibular organ.

•Radial flow theory

•Longitudinal flow theory

Nakashima T et al. Meniere's disease. Nature reviews Disease primers. 2016

 PATHOPHYSIOLOGY
• Over-accumulation of endolymph-hydrops(2,3),
cause or effect?

• Breakage of delicate membranes( rupture and

non-rupture theory-LERMOYEZ SYNDROME)

• Mixing of endolymph and perilymph

• Irritation of utricle and saccule

• Distortion of organ of corti

2.Okumura T et al official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) 2017
3.Merchant SN: official publication of the American Otological Society. 2005
 EPIDEMIOLOGY
• Similar prevalence among Africans and
Caucasians(4)
• F>M
• Increases with age(5,6)
• Increases with population size
• Inceases with household income(5)

4. Ibekwe TS et al:official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) 2007
5. Simo H et al. American journal of otolaryngology. 2015
6. Paparella MM et al Otolaryngologic clinics of North America. 2002;
 GENETICS
• Sporadic MD vs Familial MD(7,8,)
• No genetic model yet developed
Phenotypic diversity
Adult onset of disease
Ethnic group differences and
differences in presentation even within
families(11)
7. Eppsteiner RW et al : Genetic disorders of the vestibular system. Current opinion in otolaryngology & head and neck surgery. 2011

8. Vrabec JT et al. Genetic investigations of Meniere's disease. Otolaryngologic clinics of North America. 2010

11. Chiarella G et al: The genetics of Ménière’s disease. The Application of Clinical Genetics. 2015
 four tone average
of the pure-tone thresholds
at 0.5, 1,2, and 3 kHz of the
worst audiogram during the
interval 6 months before
treatment
four tone average
of the pure-tone thresholds
STAGE 2 at 0.5, 1,2, and 3 kHz of the STAGE 4
worst audiogram during the
interval 6 months before
treatment

≤25dB 41-70dB
STAGES

26-40dB >70dB
four tone average four tone average
of the pure-tone thresholds of the pure-tone thresholds
at 0.5, 1,2, and 3 kHz of the
STAGE 1 worst audiogram during the
STAGE 3 at 0.5, 1,2, and 3 kHz of the
worst audiogram during the
interval 6 months before interval 6 months before
treatment treatment
Mizukoshi K et al. Acta oto-laryngologica Supplementum. 1995
 • Vertigo-BPPV!!!(13)
• Tinnitus
CLINICAL • Aural fullness
PRESENTATION • Hearing loss
• AAO-HNS-not very
specific but sensitive(14)
13. Psillas G et al: Benign paroxysmal positional vertigo in the first acute attack of Meniere's
disease. B-ent. 2011

14. Stapleton E et alThe Journal of laryngology and otology. 2008

 AAO-HNS 1995 vs 2015 (16,17)
   1995 2015
CERTAIN Definite MD plus histopathological confirmation of DEFINITE Two or more spontaneous episodes of vertigo lasting
hydrops 20mins to 12hours.
DEFINITE Two or more definite spontaneous episodes of vertigo, 20min Audiometrically documented low to mid-frequency
or longer. SNHL in one ear, defining the affected ear on at least
Audiometrically documented hearing loss on at least one
one occasion before, during or after one of the episodes
occasion.
of vertigo.
Tinnitus or aural fullness in treated ear.
Other causes excluded. Fluctuating aural symptoms (hearing, tinnitus or
PROBABLE One definitive episode of vertigo. fullness) in the affected ear.
Audiometrically documented hearing loss on at least one Not better accounted for by other vestibular diagnosis
occasion.
Tinnitus or aural fullness in the treated ear PROBABLE Two or more episodes of vertigo each lasting 20mins to

Other causes excluded. 24hours

POSSIBLE Episodic vertigo of the Meniere’s type without documented HL Fluctuating aural symptoms (hearing, tinnitus or fullness) in

or SNHL fluctuating or fixed, with disequilibrium but without the affected ear.

definitive episodes Not better accounted for by other vestibular diagnosis

16. Lopez-Escamez JA et al. Journal of vestibular research : equilibrium & orientation. 2015
17. Lopez-Escamez JA et al. [Diagnostic criteria for Meniere's disease according to the Classification Committee of the Barany Society]. Hno. 2017
 Tumarkin drop
TUMARKIN DROP attacks VS vestibular
syncope
ATTACKS 18. Pyykko I et al: Vestibular syncope:
Auris, nasus, larynx. 2018
 PHYSICAL
EXAMINATION
• General
• Otoscopy vs pneumatic otoscopy
• Tuning fork
• Rhomberg test
• Fukuda test
• Dix-Hallpike
 INVESTIGATIONS
• TFT to rule out hyperthyroidism
and hypothyroidism
• Glucose level to rule out diabetes
• ESR and ANA test to rule out
autoimmune disorders
• Urinalysis to rule out proteinuria
and hematuria and indicators of
otorenal syndrome
Greco A, et al. Meniere's disease might be an autoimmune condition? Autoimmunity Reviews. 2012
• CBC to rule out anemia and leukemia
• UECs to rule out salt/water imbalance
• VDRLand fluorescent treponemal
antibody (FTA-ABS) to rule out
neurosyphilis and Lyme disease
• Allergy testing for allergy-mediated
Ménière syndrome
• CRP
INVESTIGATIONS
CONT’D
• PTA
• MRI vs CT(19)
• VEMP
• ECoG
• ENG

Sepahdari AR et al: Delayed intravenous contrast-enhanced 3D FLAIR MRI in Meniere's disease: correlation of
quantitative measures of endolymphatic hydrops with hearing. Clinical imaging. 2015
 TREATMENT-
OVERVIEW

The aim of treatment is to prevent

attacks of MD; to reduce the
severity of vertigo in acute attacks;
to relieve chronic symptoms of
hearing loss and tinnitus; to improve
quality of life, with minimum
adverse effects of treatment.

• MD vs MS
 •Supportive-disability act!
•Medical
TREATMENT • Surgical
CLASSIFICATION Conservative
Destructive
 Lifestyle modification-vestibular
rehabilitation
Psychotherapy
Conservative first-line medical therapy-
diuretics and betahistine or local
2018 pressure therapy
INTERNATIONAL Second line-ITS
CONSENSUS
Thrid-line ELSS or ITG

4th line labyrinthectomy+/- cochlear

implant or vestibular nerve section
30. Nevoux et al: International consensus (ICON) on treatment of Meniere's disease. European
annals of otorhinolaryngology, head and neck diseases. 2018
 • Low salt diet- aldosterone mechanism
• Diuretic
• Vestibulosupressants
• Anti-nausea + antiemetics
• Lifestyle modification
SUPPORTIVE
TREATMENT Reduce chocolate
(20,21,22) Caffeine
Smoking cessation
alcohol
20. Wright T. Meniere's disease. BMJ clinical evidence. 2015

21. Mori N, et al. Ion transport its regulation in the endolymphatic sac: official journal of the
European Federation of Oto-Rhino-Laryngological Societies (EUFOS) 2017

22. Miyashita T, et al. Hormonal changes following a low-salt diet in patients with Meniere's
disease. Auris, nasus, larynx. 2017
 • ITS vs ITG(24)

• Intratympanic dexamethasone
VS intratympanic
MEDICAL methylprednisolone(23)

• ITG vs vestibulotoxicity(25)
23. Masoumi E, et al. Methylprednisolone versus Dexamethasone for Control of Vertigo in Patients
with Definite Meniere's disease. Iranian journal of otorhinolaryngology. 2017

24. Ardic FN, et al: Selective Window Application of Gentamicin+ Dexamethasone in Meniere's
Disease. The journal of international advanced otology. 2017

25. Guneri EA, et al. Cochlear and Vestibular Effects of Combined Intratympanic Gentamicin and
Dexamethasone. The journal of international advanced otology. 2017
 SURGERY
• Endolymphatic sac surgery (29)
• Vestibular nerve section
• Labyrinthectomy
• Cochlear implant
Pullens B et al: Surgery for Meniere's disease. The Cochrane database of
systematic reviews. 2010
 MINIETT
DEVICE

• low pressure
pulse system
• Effectiveness??
(26,27,28)

26. Gates GA et al. The Laryngoscope. 2002

27. Watanabe Y, et al. Acta oto-laryngologica. 2011

28. Russo FY, et al. Meniett device in meniere disease: Randomized, double-
blind, placebo-controlled multicenter trial. The Laryngoscope. 2017
 FUTURE
• To elucidate the genetic
contribution to MD and
possible gene therapy in the
future
• Genetic counselling geared
towards prevention
• Stem cells transplant?
TAKE-HOME MESSAGE

• Menieres disease is a
rare entity
• Diagnosis is clinical
• Management is mainly
supportive
 REFERENCES
• 1. Seo YJ, Kim J, Choi JY, Lee WS. Visualization of endolymphatic hydrops and correlation with audio-vestibular functional testing in patients with definite Meniere's disease. Auris, nasus,
larynx. 2013;40(2):167-72.

• 2. Okumura T, Imai T, Takimoto Y, Takeda N, Kitahara T, Uno A, et al. Assessment of endolymphatic hydrops and otolith function in patients with Meniere's disease. European archives of oto-
rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck
Surgery. 2017;274(3):1413-21.

• 3. Merchant SN, Adams JC, Nadol JB, Jr. Pathophysiology of Meniere's syndrome: are symptoms caused by endolymphatic hydrops? Otology & neurotology : official publication of the
American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology. 2005;26(1):74-81.

• 4. Ibekwe TS, Ijaduola GT. Meniere's disease: rare or underdiagnosed among Africans. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-
Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery. 2007;264(12):1399-403.

• 5. Simo H, Yang S, Qu W, Preis M, Nazzal M, Baugh R. Meniere's disease: importance of socioeconomic and environmental factors. American journal of otolaryngology. 2015;36(3):393-8.

• 6. Paparella MM, Djalilian HR. Etiology, pathophysiology of symptoms, and pathogenesis of Meniere's disease. Otolaryngologic clinics of North America. 2002;35(3):529-45, vi.

• 7. Eppsteiner RW, Smith RJ. Genetic disorders of the vestibular system. Current opinion in otolaryngology & head and neck surgery. 2011;19(5):397-402.

• 8. Vrabec JT. Genetic investigations of Meniere's disease. Otolaryngologic clinics of North America. 2010;43(5):1121-32.

• 9. Morrison AW, Bailey ME, Morrison GA. Familial Meniere's disease: clinical and genetic aspects. The Journal of laryngology and otology. 2009;123(1):29-37.

• 10. Hietikko E, Sorri M, Mannikko M, Kotimaki J. Higher prevalence of autoimmune diseases and longer spells of vertigo in patients affected with familial Meniere's disease: A clinical
comparison of familial and sporadic Meniere's disease. American journal of audiology. 2014;23(2):232-7.
 REFERENCES CONT’D
• 11. Chiarella G, Petrolo C, Cassandro E. The genetics of Ménière’s disease. The Application of Clinical Genetics. 2015;8:9-17.
• 12. Silverstein H, Smouha E, Jones R. Natural history vs. surgery for Meniere's disease. Otolaryngology--head and neck surgery : official journal of American Academy of
Otolaryngology-Head and Neck Surgery. 1989;100(1):6-16.
• 13. Psillas G, Triaridis S, Markou K, Tsalighopoulos M, Vital V. Benign paroxysmal positional vertigo in the first acute attack of Meniere's disease. B-ent. 2011;7(2):131-5.
• 14. Stapleton E, Mills R. Clinical diagnosis of Meniere's disease: how useful are the American Academy of Otolaryngology Head and Neck Surgery Committee on Hearing and
Equilibrium guidelines? The Journal of laryngology and otology. 2008;122(8):773-9.
• 15. Goebel JA. 2015 Equilibrium Committee Amendment to the 1995 AAO-HNS Guidelines for the Definition of Meniere's Disease. Otolaryngology--head and neck surgery :
official journal of American Academy of Otolaryngology-Head and Neck Surgery. 2016;154(3):403-4.
• 16. Lopez-Escamez JA, Carey J, Chung WH, Goebel JA, Magnusson M, Mandala M, et al. Diagnostic criteria for Meniere's disease. Journal of vestibular research : equilibrium
& orientation. 2015;25(1):1-7.
• 17. Lopez-Escamez JA, Carey J, Chung WH, Goebel JA, Magnusson M, Mandala M, et al. [Diagnostic criteria for Meniere's disease according to the Classification Committee of
the Barany Society]. Hno. 2017;65(11):887-93.
• 18. Pyykko I, Manchaiah V, Zou J, Levo H, Kentala E. Vestibular syncope: A disorder associated with drop attack in Meniere's disease. Auris, nasus, larynx. 2018;45(2):234-41.
• 19. Sepahdari AR, Ishiyama G, Vorasubin N, Peng KA, Linetsky M, Ishiyama A. Delayed intravenous contrast-enhanced 3D FLAIR MRI in Meniere's disease: correlation of
quantitative measures of endolymphatic hydrops with hearing. Clinical imaging. 2015;39(1):26-31.
• 20. Wright T. Meniere's disease. BM
 REFERENCES CONT’D
• 21. Mori N, Miyashita T, Inamoto R, Matsubara A, Mori T, Akiyama K, et al. Ion transport its regulation in the endolymphatic sac: suggestions for clinical aspects of Meniere's disease. European
archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head
and Neck Surgery. 2017;274(4):1813-20.

• 22. Miyashita T, Inamoto R, Fukuda S, Hoshikawa H, Hitomi H, Kiyomoto H, et al. Hormonal changes following a low-salt diet in patients with Meniere's disease. Auris, nasus, larynx.
2017;44(1):52-7.

• 23. Masoumi E, Dabiri S, Khorsandi Ashtiani MT, Erfanian R, Sohrabpour S, Yazdani N, et al. Methylprednisolone versus Dexamethasone for Control of Vertigo in Patients with Definite
Meniere's disease. Iranian journal of otorhinolaryngology. 2017;29(95):341-6.

• 24. Ardic FN, Tumkaya F, Aykal K, Cabuk B. Selective Window Application of Gentamicin+ Dexamethasone in Meniere's Disease. The journal of international advanced otology. 2017;13(2):243-
6.

• 25. Guneri EA, Olgun Y, Aslier M, Nuti D, Kirkim G, Mungan S, et al. Cochlear and Vestibular Effects of Combined Intratympanic Gentamicin and Dexamethasone. The journal of international
advanced otology. 2017;13(1):47-52.

• 26. Gates GA, Green JD, Jr. Intermittent pressure therapy of intractable Meniere's disease using the Meniett device: a preliminary report. The Laryngoscope. 2002;112(8 Pt 1):1489-93.

• 27. Watanabe Y, Shojaku H, Junicho M, Asai M, Fujisaka M, Takakura H, et al. Intermittent pressure therapy of intractable Meniere's disease and delayed endolymphatic hydrops using the
transtympanic membrane massage device: a preliminary report. Acta oto-laryngologica. 2011;131(11):1178-86.

• 28. Russo FY, Nguyen Y, De Seta D, Bouccara D, Sterkers O, Ferrary E, et al. Meniett device in meniere disease: Randomized, double-blind, placebo-controlled multicenter trial. The
Laryngoscope. 2017;127(2):470-5.

• 29. Pullens B, Giard JL, Verschuur HP, van Benthem PP. Surgery for Meniere's disease. The Cochrane database of systematic reviews. 2010(1):Cd005395.

• 30. Nevoux J, Barbara M, Dornhoffer J, Gibson W, Kitahara T, Darrouzet V. International consensus (ICON) on treatment of Meniere's disease. European annals of otorhinolaryngology, head and
neck diseases. 2018;135(1s):S29-s32