Meniere’s Disease versus Audiovestibuar Loss of a Vascular Cause이 형

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Meniere’s Disease versus Audiovestibuar Loss of a Vascular Cause

이 형

계명대학교 의과대학 신경과

Meniere’s disease versus audiovestibuar loss of a vascular cause

이 형

계명대학교 의과대학 신경과

Positive HIT result in central lesion

 common neurotological syndrome characterized by sudden onset of prolonged (lasting days) vertigo and hearing loss.

 canal paresis to caloric stimulation and sensorineural hearing loss (SNHL) on pure tone audiogram

 Acute cause - AICA territory infarction

Chronic cause – Meniere’s disease and acoustic neuroma involving CP angle

Acute Audiovestibuar loss

Meniere’s disease

√ Chronic inner-ear disorder

√ Episodic attacks of vertigo, fluctuating hearing loss, tinnitus, aural pressure, and a progressive loss of audiovestibular function

√ Prosper Menie`re

√ Endolymphatic hydrops

√ Prevalence of 34-190per 100,000

명확한 메니에르병(Definite Ménierè’s disease)

1) 자발성 현훈이 2회 이상 발생하고, 발생 시간이 20분에서 12시간 까지 지속

2) 한쪽 귀에서 청력검사로 확인된 저주파에서 중주파수 대역의 감각 신경성 난청(2000 Hz 이하의 두 개의 연속된 주파수에서 최소 30 dB 이상), 이러한 감각신경성 난청이 어지럼 발작 전, 발작 시 또는 발작 후에 이환된 귀에서 최소 1회 이상 보이는 경우

3) 이환된 귀의 변동성이 있는 청각 증상들(청력, 이명 또는 이충만감) 4) 다른 전정질환의 진단으로 설명이 되지 않음

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Differential diagnosis of Menière’s disease

Autosomal dominant sensorineural hearing loss type 9 (DFNA9) caused by COCH geneAutosomal dominant sensorineural hearing loss type 6/14 (DFNA6/14) caused by WSF1 gene

Autoimmune inner ear disease Stroke/TIA in the VBS

Cogan‘s syndrome. Some cases may have recurrences.

Endolymphatic sac tumor

Meningiomas and other masses of the cerebellopontine angle Neuroborreliosis

Otosyphilis Susac syndrome

Third window syndromes (Perilymph fistula, canal dehiscence, enlarged vestibular aqueduct)

Vestibular migraine

Vestibular paroxysmia (neurovascular compression syndrome) Vestibular schwannoma

Vogt-Koyanagi-Harada syndrome

Internal auditory artery infarction.

Kim JS et al. Neurology 1999

A: horizontal SCC B: posterior SCC

MRI and audiologic findings in patient with AICA infarction and cochlear-type hearing loss

PTA and stapedial reflex test BAEP

MRI and audiovestibular findings in a patient with recurrent vertigo and

fluctuating hearing loss with tinnitus – a prodromal sign of AICA territory

infarction.

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Vestibular labyrinth & cochlear: Which one is more sensitive to ischemia ?

 transient occlusion of IAA using guinea pig - cochlear (apical part) > vestibular end organ

 isolated episodic vertigo can be the only manifestation of VBI, especially superior vestibular labyrinth

 In rats. the vestibular nucleus is more vulnerable to ischemia than the cochlear nucleus

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Why hearing symptoms are much less frequent than vestibular symptoms in VB ischemia?



Auditory pathway is less ubiquitous than vestibular pathways



Central auditory pathway have multiple sources of blood supply



Redundancy and bilateral representation of central auditory pathways



Vertigo is more distracted symptom than hearing loss

Sudden bilateral simultaneous deafness with vertigo as a a sole manifestation of VB insufficiency

- Lee H et al. JNNP2003 - -Lee et al. JNNP2003-

Lee, H et al. J Neurol Neurosurg Psychiatry 2003;74:1644-1648

Patient with episodic prodromal unilateral hearing loss and tinnitus

Copyright ?003 BMJ Publishing Group Ltd.

Acute auditory disturbance as a prodrome of anterior inferior cerebellar artery infarction

Five (31%) of 16 patients with AICA infarction had an acute auditory syndrome as a prodrome of AICA infarction Two types of acute auditory syndrome

- recurrent episodic hearing loss with or without tinnitus (3) - a single episode of prolonged hearing loss with tinnitus (2) Acute auditory syndrome may be a warning sign of impending ponto-cerebellar infarction in the distribution of

the AICA.

- Lee H et al. JNNP 2003 -

-Lee et al. JNNP 2003 -

Labyrinthine infarction Red light of health ? 8–30% of patients with posterior circulation stroke (mainly AICA territory) had acute audiovestibular prodromal symptoms before more widespread infarction and this symptom may come to be viewed as an opportunity to prevent an impending posterior circulation stroke. – Lee H et al (2005), Kim JS et al (2009), Lee H et al (2009) -

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Lee and Cho¹⁵Kim et al²⁶ Lee et al¹⁰ Number of studied patients

Pattern of AICA territory infarction - Isolated AICA - AICA plus

1612 4

5436 18

8255 27 Percentage of acute audiovestibular disturbance before VBIS

Identification of normal MRI at the stage of isolated audiovestibular dis turbance

Identification of CP at the stage of isolated audiovestibular disturbance Identification of SNHL on PTA at the stage of isolated audiovestibular disturbance

31% (5/16) NoneNone None

8% (4/54) Complete Complete Complete

16% (13/82) Incomplete Incomplete Incomplete Pattern of audiovestibular disturbance before VBIS

- Vertigo/hearing loss/tinnitus - Vertigo/tinnitus - Vertigo/hearing loss

31 1

30 1

31 9 Interval from audiovestibular disturbance to VBIS 10 days 4 days 30 days Kinetics of audiovestibular disturbance

- plus other symptoms

- isolated 0

5 0

4 0

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What factor(s) suggests impending stroke at the stage of isolated audiovestibular disturbance?

√ Vascular risk factors in elderly patients

√ Combined audiovestibular disturbance

√ Focal or diffuse stenosis of the basilar artery close to

the origin of the AICA

-Stroke. 2009;40:3745-3751-

and hearing level)

- Lee et al, 2009: Lee et al, 2012:, Lee et al, 2014 -

- Lee H at al. Stroke. 2009;40:3745-3751-

What a high risk factor(s) suggesting (predicting) impending stroke at the stage of isolated audiovestibular disturbance?

MRI findings in a patient with acute audiovestibular loss

as a prodromal sign of AICA territory infarction.

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MD Audiovestibular loss (TIA/infarct) Age/course/site/pathology 20-60 years/chronic inner ear disorder/EH elderly/acute inner ear disorder

/ischemia

Risk factor for stroke Uncommon Common

Duration of vertigo 20min -12hours Several minutes (TIA) , days(stroke)

Isolated vertigo during several

years Uncommon, most patients develop

the full picture within the first year. Uncommon, most strokes after TIA occur within 90 days Fluctuating hearing threshold Yes, typically fluctuates in the

first few years Yes, appear as a manifestation of

TIA

Pattern of hearing loss Low to medium (below 2000Hz) Prefer low tone (TIA/infarct) Profound HL during early period Never, rare even in late stage Possible, may occurs at initial state

(TIA/infarct) Non-simultaneous onset of vertigo

and hearing loss Common (vertigo may precede hearing loss by several months), begin with classic triad in only 40% 0f MD

Common (vertigo> hearing loss) in TIA, combined symptoms in infarct (60% in AICA)

Canal paresis Uncommon during early period, but observed

in up to 75% of MD Common (42% in TIA, 65% in

AICA infarct) Combined audiovestibular loss Uncommon during early period Common (60% in AICA infarct) Specific Inner ear MRI protocol Diagnosis of EH and earl y detection of

contralateral ear involvement Can detect labyrinthine infarct?

Vascular compromise on MRA None Stenosis/occlusion of VBS

EH, Endolymphatic hydrops

Take Home messages

√ TIA/VB ischemic stroke may mimic Meniere’s

disease

√ Clinical evidences when attempting to determine the

etiology of acute audio-vestibular syndromes

√ Combined audiovestibular loss

√ MRI and/or MRA

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