Mnire's disease

Febryanti P Sari

Pembimbing : Sally Mahdiani,dr., M.Kes., SpTHTKL(K)

Literature Reading

Department of Otorhinolaryngology Head & Neck Surgery

Faculty of Medicine Universitas Padjadjaran
RS Hasan Sadikin
Bandung
1
2016

Definition
Mnire's disease or Mnire's
syndrome is an inner ear disorder marked
by spontaneous attacks of vertigo,
fluctuating sensorineural hearing loss,
aural fullness and tinnitus.

2
Lee, KJ, Essential Otolaryngology Head & Neck Surgery, 9 th ed, McGraw-Hill, 2008

Introduction
French physician

Published in 1861

vertigo was caused by

inner ear disorders
3

decitre.fr

Introduction
- First describe by Prosper Meniere in 1861
Vertigo may linked to inner ear rather than only
to central sources
- In 1870, Friedrich Goltz clarified the function of
the vestibular system by concluding that the
semicircular canals were responsible for
mediating equilibrium only and were not involved
with hearing

Johnson J, Lalwani AK. Menieres . Ballengers Otorhinolaryngology Chapter 20. 2003 BC Decker Inc.

Introduction
- Knapp (1871)
Menieres disease was caused by elevated
endolymphatic pressure
- Hallpike and Cairns (1938)
Gross dilation of the saccule and scala media
with obliteration of the perilymph spaces of the
vestibule and scala vestibuli and confirmed the
concept of endolymphatic hydrops

Johnson J, Lalwani AK. Menieres . Ballengers Otorhinolaryngology Chapter 20. 2003 BC Decker Inc.

ANATOMY - PERIPHERAL VESTIBULAR SYSTEM

6
Probst-Grevers-Iro, Basic Otorhinolaryngology, 2006

Modul Ilmu Kesehatan THT-KL, 2008

ANATOMY - PERIPHERAL VESTIBULAR SYSTEM

Types of hair cell bodies: type I (chalice) & type II (cylindrical)

Type I & type II are not evenly distributed throughout the neuroepithelium
of either the semicircular canal ampullae/the utricular maculae
7
Modul Ilmu Kesehatan THT-KL, 2008

Introduction

9
Lee, KJ, Essential Otolaryngology Head & Neck Surgery, 9 th ed, McGraw-Hill, 2008

ETIOLOGY

10
Li, JC, et al Meniere Disease (Idiopathic Endolymphatic Hydrops),Emedicine Medscape,,com

EPIDEMIOLOGY

11

Hain TC. Epidemilogy of Menieres disease [online]. 2008

EPIDEMIOLOGY
Peak incidence of Mnires disease is in
the 40- to 60-year-old age group
Children as young as 4 years and in
elderly persons older than 90 years
females : males = 1.3:1

12

Minor LB, Schessel DA, Carey JP. Mnire's disease. Curr Opin Neurol. Feb 2004

Pathophysiology
Exact pathophysiology of Mnire disease
is controversial

Distortion of the membranous labyrinth

resulting from overaccumulation of
endolymph

Merchant SN, Adams JC, Nadol JB Jr. Pathophysiology of Meniere's syndrome: are symptoms caused by endolymphatic
hydrops?.Otol Neurotol. Jan 2005

13

Pathophysiology

Normal membranous labyrinth

Dilated membranous labyrinth in Meniere's

14
disease (Endolymphatic Hydrops

Pathophysiology
Hydrops
endolymphatic duct or sac is blocked
obstructed by scar tissue, or narrow from birth

Immune Disease
Migraine
Underlying cause of Meniere's disease is
unknown
15

Hain TC. Menieres disease [online] 2008

Pathophysiology
Attacks of hydrops probably are caused by
an increase in endolymphatic pressure,
which, in turn, causes a break in the
membrane that separates the perilymph
(potassium-poor extracellular fluid) from
the endolymph (potassium-rich
intracellular fluid).

16

Hain TC. Menieres disease [online] 2008

Pathophysiology
The resultant chemical mixture bathes the
vestibular nerve receptors, leading to a
depolarization blockade and transient loss
of function. The sudden change in the rate
of vestibular nerve firing creates an acute
vestibular imbalance (ie, vertigo).

17

Hain TC. Menieres disease [online] 2008

Pathophysiology
Physical distention caused by increased
endolymphatic pressure also leads to a
mechanical disturbance of the auditory
and otolithic organs
Utricle and saccule irritation
nonrotational vestibular symptom

18

Hain TC. Menieres disease [online] 2008

Pathophysiology
This physical distention mechanical
disturbance of the organ of Corti
Distortion of the basilar membrane and
the inner and outer hair cells hearing
loss and/or tinnitus

19

Hain TC. Menieres disease [online] 2008

Pathophysiology
Since the apex of the cochlea is wound
much tighter than the base, the apex is
more sensitive to pressure changes than
the base.

20

Hain TC. Menieres disease [online] 2008

21

Etiology
Disorders that may give rise to elevated
endolymphatic pressure include metabolic
disturbances, hormonal imbalance,
trauma, and various infections (eg,
otosyphilis and Cogans syndrome
[interstitial keratitis]

22

Paparella MM, Djalilian HR. Etiology, pathophysiology of symptoms, and pathogenesis of Meniere's disease. Otolaryngol Clin North
Am. Jun 2002;35(3):529-45

Symptoms & Signs

Diagnosis of Menieres disease
according to AAO-HNS (1995)

23
Johnson J, Lalwani AK. Menieres . Ballengers Otorhinolaryngology Chapter 20. 2003 BC Decker Inc.

Symptoms & Signs

Diagnosis of Menieres disease according to JSER
(Mizukoshi et al.1995)

Johnson J, Lalwani AK. Menieres . Ballengers Otorhinolaryngology Chapter 20. 2003 BC Decker Inc.

Staging
Staging of definite and certain Menieres disease according
to AAO-HNS (1995)

25
Johnson J, Lalwani AK. Menieres . Ballengers Otorhinolaryngology Chapter 20. 2003 BC Decker Inc.

Physical Examination
Depending upon the phase of disease
During remission, physical examination
findings may be completely normal
During an acute attack, the patient has severe
vertigo
Significant distress
Elevated blood pressure, pulse & respiration
Significant nystagmus may be present
26

Hain TC. Menieres disease [online] 2008

Evaluation of vertigo
The Dix-Hallpike positional test
Nystagmus
The Romberg test generally shows
significant instability and worsening during
acute attacks when the eyes are closed

27

Hain TC. Menieres disease [online] 2008

Evaluation of hearing loss

Audiologic testing is more accurate

28
Probst-Grevers-Iro, Basic Otorhinolaryngology, 2006

Complications

Injury due to falls

Anxiety regarding symptoms
Accidents due to vertigo spells
Disability due to unpredictable vertigo
Progressive imbalance and deafness
Intractable tinnitus

29

Other problems to be
considered include the following

Trauma
Endocrine abnormalities
Hyperlipidemia
Diabetes
Congenital anomalies
Autoimmune
problems/inner ear
inflammation
Otosclerosis
Perilymphatic fistula
Electrolyte imbalance

Basilar meningitis
Brainstem tumors
Neoplasms (eg, acoustic
neuroma)
Toxic or pharmaceutical
injury to the vestibular
apparatus
Vascular infarction of the
labyrinth (usually
associated with unilateral
hearing loss)
30

Hain TC. Menieres disease [online] 2008

Differentials Diagnosis

Anterior Circulation Stroke

Arteriovenous Malformations
Basilar Artery Thrombosis
Benign Positional Vertigo in Emergenc
y
Medicine
Brainstem Gliomas
Cerumen Impaction Removal
Ear Foreign Body Removal in Emergen
cy Medicine
HIV-1 Associated CNS Conditions:
Meningitis
Hypothyroidism and Myxedema Coma i
n Emergency Medicine
I
ntracranial Hemorrhage
Labyrinthitis And Related Conditions

Migraine Headache
Neurosyphilis
Otitis Media in Emergency Medicine
Polyarteritis Nodosa
Posterior Cerebral Artery Stroke
Skull Tumors
Rheumatoid Arthritis
Temporal Lobe Epilepsy
Transient Ischemic Attack
Vestibular Neuronitis
Viral Encephalitis
Viral Meningitis

31

Hain TC. Menieres disease [online] 2008

Differentials Diagnosis

32
Bailey, Byron et al: Head & Neck Surgery - Otolaryngology, 5th Edition, Lippincott Williams & Wilkins2014

Work Up
Should be
directed at
differentiating the
disease from
other causes on
the basis of
associated
symptoms

Audiometry
Brainstem auditory evoked
potentials
Transtympanic
electrocochleography
(ECOG)
Electronystagmography
(ENG)
Otoscopy
Caloric testing/ENG
33

Hain TC. Menieres disease [online] 2008

Treatment
In the emergency department (ED) is
based on symptomatic relief of the clinical
findings
Surgical therapy for Mnire disease is
reserved for medical treatment failures

34

Pharmacologic Therapy
Vestibulosuppressants
meclizine, droperidol, prochlorperazine,
diazepam, lorazepam, alprazolam
Diuretics and diureticlike medications
hydrochlorothiazide and triamterene,
hydrochlorothiazide, acetazolamide,
methazolamide)

35

Hain TC. Menieres disease [online] 2008

Pharmacologic Therapy
Steroids
probably by reducing endolymphatic
pressure orally, intramuscularly, or even
transtympanically
Aminoglycosides
toxic to the vestibular (balance) end organ
36
Sajjadi H. Medical management of Meniere's disease. Otolaryngol Clin North Am. Jun 2002

Pharmacologic Therapy
Histamine agonists
betahistine (Serc) increasing circulatory
flow to the cochlear stria vascularis?
has not been approved by the US Food
and Drug Administration (FDA)

37
Phillips JS, Prinsley PR. Prescribing practices for Betahistine. Br J Clin Pharmacol. Apr 2008

Surgical therapy
Endolymphatic sac decompression or
shunt placement
Vestibular nerve section
Labyrinthectomy
Intratympanic injection of medications
such as gentamicin or steroids

38

Wetmore SJ. Endolymphatic sac surgery for Mnire's disease: long-term results after primary and revision surgery. Arch
Otolaryngol Head Neck Surg. Nov 2008

Diet and Activity

Dietary measures
Avoiding foods with high sodium content
(pizza, preserved foods, smoked fish)
Activity restriction
dangerous tasks (eg, especially climbing
ladders) should be avoided

39

Prevention
Caffeine
Nicotine
Chocolate, which has
shown to be a potent
trigger substance
Tobacco
Alcohol, particularly
red wine and beer

Foods with high

cholesterol or
triglyceride content
Foods with high
carbohydrate content
Excessive sweets and
candy

40

Prognosis
Patient presentation and progression of
Mnire disease vary widely
In general, the patients condition tends to
spontaneously stabilize over time
However: many patients are left with poor
balance and poor hearing

41
Minor LB, Schessel DA, Carey JP. Mnire's disease. Curr Opin Neurol. Feb 2004