Meniere disease

Question 1

Rauch, Merchant, & Thedlinger (1989)

Answer 1

Meniere disease (MD) is a multifactorial disorder associated with the accumulation of endolymph in the cochlear duct and vestibular organs

Question 2

Merchant, Adams, & Nadol (2005)

Answer 2

Endolymphatic hydrops, however, do not explain all of the clinical features of the disease

Question 3

Nakashima et al. (2016) Histopathology

Answer 3

Histopathological evaluation of the diseased cochlea shows distention of the scala media with ballooning of Reissner’s membrane into the scala vestibuli

Question 4

Lopez-Escamez et al. (2016) Epidemiology

Answer 4

The age of onset ranges from the third to the seventh decade of life with a small female predominance

Question 5

Nakashima Epidemiology

Answer 5

Estimated prevalence of the disease in the U.S. is 0.19%

Question 6

Tyrrell et al. (2014)

Answer 6

The odds of MD 1.7 times greater for people of white ethnicity or people who with obesity

Question 7

Morrison et al. (2009)

Answer 7

Although most cases are sporadic, 5-15% of patients with MD have a family history of an autosomal dominant inheritance pattern with variable penetrance

Question 8

Pyykko et al. (2013)

Answer 8

The time delay between the onset of vertigo and hearing loss with or without tinnitus and aural fullness is long in many patients

Question 9

Nakshima Clinical Features

Answer 9

Episodes of vertigo are usually much more common in the first years of the disease
Symptoms start with vertigo in about 41% of patients with or without tinnitus and aural fullness
The overall frequency of bilateral is estimated to be 24% of patients with MD

Question 10

Lopez Escamez Clinical Features

Answer 10

Hearing loss and vestibular hypofunction show great variability among patients, which makes phenotyping MD very difficult

Question 11

Young, Wu, & Wu (2002)

Answer 11

Asymmetry ratio of VEMP amplitudes can correlates with the stage of MD

Question 12

Wen, Cheng, & Young (2012)

Answer 12

Augmented oVEMP results with an asymmetry ratio of >.40 might result from compensatory enlargement of the utricular hydrops as endolymph pressure in the utricle increases- this can reflect the status of the inner ear close to a vertigo attack but will be missed when testing is completed between subsequent attacks

Question 13

Levine, Margolis, & Daly (1998)

Answer 13

ECochG in MD patients may demonstrate a high average summating potential to action potential ratio

Question 14

Kim et al. (2005)

Answer 14

However, because of its lack of sensitivity, ECochG should not play a decisive role in determining the presence or absence of MD

Question 15

Lopez-Escamez et al. (2016) Diagnosis of MD

Answer 15

The recent development of 3 Tesla MRI with gadolinium chelate has allowed the visualization of endolymphatic hydrops in 93% of ears with symptoms attributable to MD, but also in 65% of asymptomatic contralateral ears

Question 16

Santos et al. (1993)

Answer 16

Combining diuretics with sodium restriction has been shown to slow the progression of hearing loss in MD

Question 17

Nakashima et al. (2016) Interventions/Treatments

Answer 17

When standard first-line treatments are unable to suppress vertigo attacks, intratympanic gentamicin is a widely used form of treatment

Question 18

Lopez-Escamez et al. (2016) Differential Dx AIED

Answer 18

AIED begins as sudden SNHL in one ear progressing rapidly to the second ear with tinnitus and balance symptoms- may resemble MD since hearing loss can fluctuate and occur suddenly or come on over days to months- While some patients with MD have bilateral hearing loss, this is less common, and in these cases, the clinician should consider the possibility of AIED

Question 19

Lopez-Escamez et al. (2016) Differential Dx Vestibular Migraine

Answer 19

Patients with features of both MD and vestibular migraine have been repeatedly reported
o Some research has suggested a common pathophysiology between symptoms of vestibular migraine and MD, perhaps linked to an ion channel dysfunction and endolymphatic hydrops, which have been reported in patients with vestibular migraine and auditory symptoms
o Fluctuating hearing loss, tinnitus and aural pressure may occur in vestibular migraine, but SNHL does not typically progress to severe hearing loss over the years in migraine
o When hearing loss develops in vestibular migraine, it is often bilateral, whereas involvement of both ears from the onset is rare in MD

Question 20

Main talking points for epidemiology

Answer 20

1. disease of middle age/onset 3rd-7th decade of life
2. prevalence - 0.19% in US
3. 1.7x odds for white and obese
4. genetic predisposition with 5-15% autosomal dominant inheritance

Question 21

Clinical Features talking points

Answer 21

1. clinical syndrome with episodes of spontaneous vertigo usually associated with unilateral fluctuating hearing loss, aural fullness, and tinnitus
2. Episodes of vertigo are more common in the early years- 41% of patients develop vertigo symptoms first with or without tinnitus/fullness
3. time delay is long between vertigo and HL
4. intense tinnitus later on
5. variability phenotyping
6. triggers

Question 22

Histopathology

Answer 22

” Meniere disease (MD) is a multifactorial disorder associated with the accumulation of endolymph in the cochlear duct and vestibular organs (Rauch, Merchant, & Thedlinger, 1989)
“ Endolymphatic hydrops, however, do not explain all of the clinical features of the disease (Merchant, Adams, & Nadol, 2005)
“ Histopathological evaluation of the diseased cochlea shows distention of the scala media with ballooning of Reissner’s membrane into the scala vestibuli (Nakashima et al., 2016)

Question 23

Vestibular Evoked Myogenic Potential (VEMP) testing

Answer 23

o Vestibular Evoked Myogenic Potential (VEMP) testing
“ The asymmetry ratio of VEMP amplitudes can correlates with the stage of MD (Young, Wu, & Wu, 2002)
“ Augmented oVEMP results with an asymmetry ratio of >.40 might result from compensatory enlargement of the utricular hydrops as endolymph pressure in the utricle increases
“ Limitation of cVEMP for MD
“ Patients with MD show varying effects of stimulus parameters, such as test frequency, which may impact the results
“ This can reflect the status of the inner ear close to a vertigo attack but will be missed when testing is completed between subsequent attacks (Wen, Cheng, & Young, 2012)

Question 24

Calorics

Answer 24

” May help the clinician identify which ear is causing episodic vertigo in bilateral Meniere disease when one ear has weak or absent responses and the other ear has residual function
“ In this case, the ear with residual function can be presumed to be the source of the episodic vertigo

Question 25

ECochG

Answer 25

” ECochG in MD patients may demonstrate a high average summating potential to action potential ratio (Levine, Margolis, & Daly, 1998)
“ However, because of its lack of sensitivity, ECochG should not play a decisive role in determining the presence or absence of MD (Kim et al., 2005)

Question 26

Blood and Imaging tests

Answer 26

” Blood tests and other imaging tests may be used to rule out disorders that can cause problems similar to those of Meniere’s disease.
o Recent developments in 3 Tesla MRI has allowed the visualization of endolymphatic hydrops in 93% of ears with symptoms attributable to MD, but also in 65% of asymptomatic contralateral ears (Lopez-Escamez et al., 2016)

Question 27

definite MD

Answer 27

” Two or more spontaneous episodic vertigo attacks lasing between 20 minutes and 12 hours
“ Low-mid frequency SNHL documented by audiometry on at least one occasion before, during, or after a vertigo attack
“ Increased bone conduction sensitivity in the effected ear by at least 30 dB HL at two contiguous frequencies below 2000 Hz
“ Fluctuating aural symptoms (hearing, tinnitus or aural fullness) in the affected ear
“ Symptoms cannot be better accounted for by another vestibular diagnosis

Question 28

probable MD

Answer 28

o Broader category defined by
“ Two or more episodes of vertigo or dizziness each lasting 20 minutes to 24 hours
“ Fluctuating aural symptoms including hearing loss, tinnitus, aural fullness in the affected ear
“ Symptoms are not better accounted for by another vestibular diagnosis

Question 29

Management of MD

Answer 29

” A common first line approach to management of symptoms is the use of diuretics and sodium restriction
o Combining diuretics with sodium restriction has been shown to slow the progression of hearing loss in MD (Santos et al., 1993)
o Restriction of sodium and use of diuretics is believed to relieve pressure in the hydropic ear
“ When standard first-line treatments are unable to suppress vertigo attacks, intratympanic gentamicin is a widely used form of treatment (Nakashima et al., 2016)
o Gentamicin results in the generation of reactive oxygen species and triggers the caspase-dependent apoptosis of hair cells
o After compensation for the vestibular loss caused by the drug induced vestibular hypofunction, further classic attacks of vertigo should not occur

Question 30

Differential Diagnosis - AIED

Answer 30

” Autoimmune Inner Ear Disease
o AIED begins as sudden SNHL in one ear progressing rapidly to the second ear with tinnitus and balance symptoms
o May resemble MD since hearing loss can fluctuate and occur suddenly or come on over days to months (Lopez-Escamez et al., 2016)
o While some patients with MD have bilateral hearing loss, this is less common, and in these cases, the clinician should consider the possibility of AIED (Lopez-Escamez, 2016)

Question 31

Differential Dx - Vestibular Migraine

Answer 31

o Patients with features of both MD and vestibular migraine have been repeatedly reported
o Some research has suggested a common pathophysiology between symptoms of vestibular migraine and MD, perhaps linked to an ion channel dysfunction and endolymphatic hydrops, which have been reported in patients with vestibular migraine and auditory symptoms
o Fluctuating hearing loss, tinnitus and aural pressure may occur in vestibular migraine, but SNHL does not typically progress to severe hearing loss over the years in migraine
o When hearing loss develops in vestibular migraine, it is often bilateral, whereas involvement of both ears from the onset is rare in MD