Audio Vestibular Involvement in Behcet's Disease.

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Paolo

Vannucchi

and

Rudi

Pecci

The audio-vestibular system is often involved

in

many auioimmune syndromes as

Cogan's syndrome and Granulomatosis

with

polyangiitis (Wegener's granuloma-tosis) but an involvement

in

BehEet's disease is reported more frequently only in the last years. Behqet's disease was first deflned by Hulusi Behget

in

1937.

It

is a

refractory multisystem disorder mainly presenting

with

recument oral aphthae and genital ulcerations, skin lesions, and uveitis. The disease is a chronic inflammatory disorder

involving the small

vessels,

which

is of

unknown

etiology.

It

has a worldwide distribution

with a

prevalence ranging

from l:1,000

to

1:10,000 in Japan and Turkey

to

1:500,000

in North

America and Europe. The aetiopatho-genesis is obscure but BehEet's disease is considered to be immune-mediated.

Audio-vestibular disturbance, including heanng impairment. tinnitus, arld diz-ziness, is one

of

the multisystemic characteristics

of

BehEet's disease.

There are

studies

and

case reports

in

the literature about the inner

ear involvement, sudden cochlear sensorineural hearing loss (SNHL) and incidence

of

SNHL in Behqet's disease. In these studies, the incidence of

HL

has been reported as 12*80 7o.

Auditory

and vestibular lesions, among the

clinical

manifestations

of

central nervous system (CNS) involvement, were f,rst described by Alajouaine et

al.

[1]; the authors described SNHL and gaze paretic nystagmus in a patient with BehEet's disease suffering from meningoencephalitis. Since then, other authors have shown that SNHL and dizziness are frequent symptoms

in

patients affected by BehEet's syndrome

with

or without the involvement

of

the CNS. ErdinE

et

al.

l2l

suggest

P. Vannucchi

(X)

.R. Pecci

Deparlment of Surgical Sciences and Translational Medicine, Unit of Audiology,

Careggi Hospital, University of Florence, Largo Giovanni Alessandro Brambilla 3, 50134 Florence, Italy

e-mail: paoiovannucchi @ libero.it

L. Emmi (ed.), BehEet's Syndrome, Rare Diseases of the Immune System, DOI: I 0. I 007/91 8-88 - 47 0 - 541 1 -6- 1 3, O Springer-Veria g ltalia 20 7 4

l,

\iannuc9.!j and R. Pec:

152

that presence

of

SNHL in

Behqet's disease

is

prevalent and represents a fbr.rr'

clinical

symptom after ora[, genital ulcers, and skin lesions: a statistically sigm'-icant SNHL at high liequencies when comparecl

with

the speech frequencies

hr'

been reported and this high incidence

of

SNHL can be attributed

to

srnall venoli: disease that

is

seen

in

the n-rajority

of

neuro-Behqet patients.

Kulahli et

al

[-: ctinied out a stucly

to

determine the characteristics and incidence

of

SNHL an; vestibular disturbance

in

BehEet's syndrome

with

tr large number (62) of patient! ancl they found that (1) the hearing and vestibular disturbances

in

BehEet's sr.n-drome are more prevalent than previously recognized'

(2) SNHL

in

high

tì'e quencies in BehEet's patients is an jndicator of cochlear involvement, (3) there is

_'

t igt-r.. prevalenie

of

central vestibular synclrorne

in

Behqet's patients than

it

wa: thoughi tefore, ancl (4) HLA-35 1 antigen may be able to be a prognostic factor

fc:

SNHL

in

Behqet's Patients.

BehEet,s disease is typically characterized by a lack of correlation betu'een othe: orgalls ancl audio-vestibuiar involvement, which is

in

keeping

with

the multifbca' nature

of

the djsease process. Also, a lack

of

correlation was found hetw.een

thi

auditory and vestibular lesions, but this may be explained by an understanding of the ,ascr,lar supply: the common cochlear artery anci anterior vestibular artery are the

main branches of the labyrinthine artery and can be selectively involved by immu-nologicaliy mediated inflammation. Furthermore, the incidence of dizziness is ofien

*o.".o--on

than audiologic symptoms, probably due to the more diverse causes o; dizziness, inclucling peripheral and central verti-co'

In

an interesting study, comparing BehEet's patients

with

sex and age matched healthy subjects, Sùslù et

al'

['1] showed that

in

younger patients there cor-rld be a lesser ratio of SNHL comparecl to older ones. However, although hearing thresholds were

within

the normal

limits

accorcling

to

pure-tone

avelage-PTA- (at

500' 1,000,2,000, and 4,000 Hz), hearing levels

of

the patients

with

BehEet,s disease were found

to

be higher: than the controls at most

of

the single liequencies and difièrences

were

seen

to

be

signiflcant. Furthermore,

in

the high

frequencies

(9,000-16,000H2)63.4olcofthepatientshadhearinglossandthedifferences

between the hearing levels of patient and control group tend to increase de{ìnitely in high frequencies. This fìnding points a cochlear involvement

in

Behqet's disease ueginning and prominent in basal turn of the cochiea. ln these patients ìt also could

be fbuncl decreased responses w:ith otoacoustic emission (OAE) and lower values

oi

signal to noise rario (SNR); this meiins that the physiological

motility

of the outer hair cells

of

the patients

with

Behget's disease is lesser than the normal subjects Meanwhile, normal hearing thresholds to-eether u'ith the decreased OAE responses could be accepted as the early findings

of

sub-clinical cochlear involvement in Behget,s disease.

Finally,

not

oniy

the duration

of

the disease,

but

also clinical tèatures or other system involvements

gslally

<1o not conelate with hearing leveLs

These clata show that cochlear involvement can be regarded as an early sign and takes place even

in

patients without significant organ involvements.

Erbek

et

til. [5]

suggest

eÌl

association between <lelayed vestibular evoked myogenic potentials responses

(VEMP)

and BehEet's disease' Prolonged VEMP

..rporrr",

in

patients

with

BehEet's disease

maybe erplained

by

chronic

:3

Audio Vestibul

rnflammation inr, VEMPs are not a

inferior and supe

Jisease. Therefor tibular evaluation Even Cinar et .igni{ìcantly hi-ul-Behget's disease erperience headr common clinical

.kin

lesions, ar.rd

:he routine follor lndeed, Behqe

of

symptoms. Ra region

will

hope rerious condition

References

1. Alqouanine T. encephalite de 1a

l.

Erdinc AK, Harp Auris Nasus Larl -:. Kulahli

t.

Balci

disturbances in E

-i. Siislù AE. Polat

l

in Behqet's diseir

:.

Erbek S. ErbeK potentials ilì Beh

Éj. Cinar S. Cinar- F Behqet disease Ì I

nnucchi and R. Pecci

epresents a fburth

-itati stically

signif-:h

frequencies has :d to small venous

Kulahli et al.

L3l nce

of SNHL

and er (62) of patients, ,s

in

Behqet's

syn-\HL

in

high

fre-ment, (3) there is a

atients than

it

was 'ognostic factor fbr Ltìon between other r

ith

the multifbcal found between the nderstanding of the rular artery are the

Lr.rvolved by

immu-rI dizziness is ofien re diverse causes

of

r

and age matched ts there could be a

L hearing thresholds

:e-PTA-

(at

500, h Behget's disease :1e frequencies and

:

hi.gh frequencies

rurd the differences rcrease deflnitely in

n Behget's disease arients

it

also could and lower values

of

rotility

of the outer re nonnal subjects. sed OAE responses ear involvement in e. but also clinical

nith

hearing levels. s an early sign and lents.

vestibular evoked . Prolonged VEMP

tained

by

chronic

'l

3

_{Audio Vestibular lnvolvement in BehEet's Disease}

inflarnmation involving the pathways of the sacculo-col1ic reflex. Since abnormal VEMPs are not always associated with caloric weakness,

it

can be speculated that

inferior

and superior vestibular nerves might be damaged separately

in

BehEet's disease. Therefore, the VEMP response may be a useful diagnostic

tool fbr

ves-tibular evaluation

in

this disease.

Even Cinar et al.

_[6]

more recently revealed that the prevaÌence

of

SNHL was signiflcantly

highel in

the BehEet patients than

in

the controls. The duration

of

Behqet's disease had

no

signiflcant impact

on

whether patients

did or did

not experience hearing loss. The authors reported hearing loss was the

lburth

most common clinical finding

in

the BehEet group, after oral ulcers, genitiil ulcers, and skin lesions, and they suggest the need

for

an adequate investigation of hearing in the routine follow-up

of

these patients.

lndeed, BehEet's disease may present

with

fèatures other than the classic triad

of

symptoms. Raised awareness

of

the

clinical

features

within

the head and neck

region

will

hopefully

enable

early

diagnosis and treatment

of

this

potentially serious condition.

References

[. Alajouanine

T,

Castaigne

V.

Lhermitte

F,

Cambier J, Gautier Jc (1961) La meningo-encephalite de la naladie de Behqet. Presse Med 69 2519 2582

2. Erdinc AK. Harputluoglu U. Oghan F, Baykal B (2004) BehEet's disease and hearìng loss.

Auris Nasus Larynx 3l:29-33

3. Kulahli

I,

Balci

K.

Koseoglu E, Yuce

I,

Cagli S, Senturk

M

(2005) Audio-vestibular disturbances in BehEet's patients: report of 62 cases. Hear Res 203(1-2):28 31

4. Sùslii AE. Polat M, Kòyba;i S, BiEer YO. Funda YO. Parlak AH (2010) Inner ear involvement

in Behqet's disease. Auris Nasus Larynx 37(3):286-290

5. Erbek S, ErbeK SS. Yilmaz S. YuceL E. Ozluoglu LN (2008) Vestibular evoked myogenic potentials in Behqet's disease. Eur Arch Otorhinolaryngol 265:1315-1320

6. Cinar S, Cinar F. Kiran S (2012) Is there a need tbr audiologic evaluation in patients with

Behqet disease? Ear Nose Throat J 91(3):E15-9