J Int Adv Otol 2015; 11(2): 176-8
•
DOI: 10.5152/iao.2015.1039
Case Report
Ankylosing spondylitis is a chronic systemic inflammatory disease of unknown origin affecting up to 1% of the population. Audiovestibular im-
pairment has been observed in ankylosing spondylitis and sensorineural hearing loss (SNHL) is the most common form. The cause of SNHL is still
unknown but the possible causes are as follows: vascular inflammation (obliterative vasculitis) of small vessels, ossification of the articular tissue of
the middle ear, and use of non-steroid anti-inflammatory drugs. This is a case report of a patient with ankylosing spondylitis and SNHL along with a
discussion of the literature regarding cochleovestibular impairment in ankylosing spondylitis.
KEYWORDS: Ankylosing spondylitis, sensorineural hearing loss, inflammatory disease
INTRODUCTION
Ankylosing spondylitis (AS) is a chronic systemic inammatory disease. The disease primarily aects the axial skeleton and its cause
is unknown; it is known to aect up to 1% of the population. The onset is usually in the 30s and aects men three times more often
than women. Autoimmune etiology characterized by inammation is the suspected etiology. AS typically aects the spine and sac-
roiliac joints. Peripheral joints are the other sides of the skeleton involvement. Inammation of the joints leads to synovitis, fusion,
and a tendency to ankylosis. Osteoporosis and vertebral fractures can also be seen. This rheumatic disease also involves extraskel-
etal organs, such as the eyes, heart, lungs, and gut, along with manifestations such as anterior uveitis, cardiac conduction defect,
aortic valve incompetence, inammatory bowel disease, and renal and lung abnormalities. The diagnosis depends on the following
New York criteria: the presence of back pain (because of the inammation of the spine and sacroiliac joints), the restriction of lum-
bar exion and chest expansion, and radiological ndings of sacroiliitis and the human leukocyte antigen (HLA) B27 positivity. The
disease is strongly associated with the HLA B27. Sensorineural hearing loss (SNHL) is the most common form of cochleovestibular
impairment of AS; however, the cause of SNHL still remains unknown.
In this report, a patient who has AS with SNHL was presented and the literature of cochleovestibular impairment in AS was dis-
cussed.
CASE PRESENTATION
A 52-year-old man who was a bank clerk visited our clinic as an outpatient; he had a 2-year history of slowly progressing hearing
impairment. He was diagnosed with AS at the age of 34 years. With bilateral sacroiliitis on plain radiograph and magnetic resonance
imaging (MRI) as well as positivity for B27 on HLA typing according to the modied New York criteria, he was diagnosed with anky-
losing spondylitis. Previous episodes of peripheral inammatory arthritis had been treated with sulphasalazine and some non-ste-
roid anti-inammatory drugs (NSAID). He has been using naproxen since the last 2 years. The respiratory, cardiovascular, and other
systems as well as the eyes were normal. In his laboratory investigations, hemoglobin was 13.2 g/dL with normal white cell and
platelet counts. The urea, electrolyte, and calcium levels as well as the liver and thyroid function tests were normal. The erythrocyte
sedimentation rate (ESR) was 20 mm in the rst hour. The patient had a normal complete ear, nose, and throat (ENT) examination;
normal facial nerve function; no complaints of tinnitus and vertigo; and no family history for hearing loss or no history of acoustic
or head trauma as well as otologic or neurotologic operation. Pure tone audiometry revealed bilateral SNHL and 50-dB hearing level
in both ears, particularly increasing after 6000 Hz. With impedance audiometry (tympanogram), there was a type A curve for each
ear. Speech discrimination scores were 92% for each ear. With videonystagmography, he had no pathologic nystagmus. Written
informed consent was obtained from the patient.
Corresponding Address:
Ahmet Koç, ahmetkoc2010@yahoo.com
Submitted: 09.04.2015 Revision received: 19.08.2015 Accepted: 21.08.2015
Copyright 2015 © The Mediterranean Society of Otology and Audiology
176
Audiovestibular Manifestations in Patients with
Ankylosing Spondylitis - A Case Report and Review of the
Literature
Ahmet Koç, İsmet Emrah Emre
Department of Ear Nose and Throat Diseases, Acıbadem University Faculty of Medicine, İstanbul, Turkey
DISCUSSION
Audiovestibular manifestations believed to be depended on vascu-
litis or autoimmunity have been observed in autoimmune-mediat-
ed diseases or rheumatoid connective tissue diseases. SNHL is the
most prominent audiovestibular symptom in these diseases. Despite
the unknown underlying pathophysiological mechanism, there are
some theories for explaining the emergence of SNHL such as im-
mune complex-dependent vasculitis in the inner ear, antibody for-
mation against the inner ear, and neuritic as well as ototoxic eects
of drugs. The most accepted theory is the autoimmune theory (auto-
immune inner ear disease)
[1]
.
Sensorineural hearing loss, conductive hearing loss, and vestibular
symptoms were described in patients with AS. A discussion on the
characteristic ear manifestation of AS is still ongoing. Some authors
advocate that middle ear involvement leading to conductive hearing
loss is the typical form but others think that SNHL because of inner
ear involvement
[2, 3]
. The following three mechanisms may be respon-
sible for otologic involvement: 1) pathology of inner ear epithelium
and vascular tissue because of autoimmune reaction, 2) impaired
mobility of middle ear ossicles, and 3) NSAID ototoxicity
[4]
.
Amor-Dorado et al.
[5]
found SNHL in 58% of the 50 patients with AS.
Casellini et al.
[6]
determined higher SNHL rates in 22 patients with AS
than those in the control group. Alatas et al.
[4]
reported the ratio of
SNHL to be 28.6% in 28 patients with AS, whereas it was reported to
be 4.3% in the control group. Dagli et al.
[7]
studied 28 patients with
AS and 25 controls and found SNHL in 10 patients (35%). Eryılmaz et
al.
[1]
reported the ratio of SNHL to be 28.6%.
Sensorineural hearing loss can be observed in all frequencies but
high frequencies are most aected. Eryılmaz et al.
[1]
observed signi-
cant deterioration at higher frequencies in 59 patients with AS. In the
study by Adam et al.
[2]
, there was no statistically signicant dierence
between the AS group (45 patients) and the control group (31 pa-
tients) with respect to conventional frequency thresholds. However,
in this study, there was a statistically signicant dierence at a fre-
quency of 14–16000 Hz in 32 patients with AS (71%) as compared
with 12 controls (40%)
[2]
. Alatas et al.
[4]
observed SNHL both at low
and high frequencies in patients with AS. Erbek et al.
[8]
found statisti-
cally signicant dierence at high frequencies in 32 patients with AS.
Acoustic brainstem responses (ABR) and otoacoustic emission test-
ing results were also reported in patients with AS having SNHL. Ala-
tas et al.
[4]
, did not nd any statistically signicant dierence in the
ABR results between patients and the control group. Erbek et al.
[8]
found that the rates of reproducibility in transient evoked otoacous-
tic emission testing (TEOAE) were signicantly lower in patients with
AS than those in controls. Daglı et al.
[7]
determined the same result
with distortion product otoacoustic emission testing (DPOAE). Ac-
cording to Daglı et al.
[7]
, high frequencies are organized in the basal
part of the cochlea; therefore, the damage is in the outer hairy cells
of basal and intermediate parts of the cochlea in patients with AS.
Nevertheless, Kahveci et al.
[9]
did not nd any cochlear dysfunction in
patients with AS with regard to the audiological and DPOAE results.
They explained this with the multifactority of SNHL in AS (age, drugs,
duration of the disease, duration of medication, acoustic trauma, and
previous middle and inner ear infections). They added that another
possible reason for obtaining normal DPOAE results might be owing
to SNHL because of hairy cell dysfunction in the inner ear and synap-
tic or retro cochlear pathology
[9]
.
During the course of AS, immunologic vasculitis, autoantibodies
against the inner ear, and ototoxic eects of drugs may cause SNHL.
Vasculitis, which was observed in autoimmune diseases, can cause
degenerative changes in tissues. Because of autoimmune and vascu-
lar causes, degeneration or loss of hairy cells can be observed in the
patients with AS. Stria vascularis may be the aected area in AS. Stria
vascularis is an epithelial tissue that is rich in vascularization, and its
main function is to arrange the electrical potentials of endocochlea
and to supply the hairy cells
[4, 10]
.
The most common cause of renal involvement in patients with AS is
secondary amyloidosis (abnormal accumulation of amyloid proteins
in various tissues of the body). Amyloidosis can cause polyneuropa-
thies. According to Kahveci et al.
[9]
, the involvement of cochlear and
vestibular nerves as well as the central pathways of cochleovestibular
system with amyloidosis may cause SNHL. Drugs used for the med-
ical treatment of AS were discussed as a possible etiological factor
for SNHL. The most used medications in AS are as follows: NSAID and
sulfasalazine (an antibacterial from sulfonamides). Active metabo-
lites of sulfasalazine are 5-aminosalisilic acid and sulfapiridine. These
drugs are immune suppressants. Ototoxic eects of these immuno-
suppressant drugs are well discussed in the literature. Savastano et
al.
[10]
argued that the use of salicylates may cause SNHL but cochlear
improvement can be seen after the cessation of treatment. Besides,
SNHL was observed to be greater in patients treated with anti-TN-
F∝+methotrexate than in patients treated with anti-TNF∝ alone
[10]
.
There was no correlation determined between hearing levels and the
clinical or laboratory parameters of patients with AS. Amor-Dorado et
al.
[5]
, considered that the risk of SNHL was higher in patients with the
involvement of the hip, anterior uveitis, and presence of the HLA B27.
Conductive hearing loss because of the damage of ossicular chain
was also dened besides SNHL in patients with AS. Amor-Dorado et
al.
[5]
reported abnormal tympanometry results in 8% of the patients
despite normal otomicroscopic examination and absence of any
middle ear eusion. Amor-Dorado et al.
[5]
explained this statement
with the stiness of the ossicular chain.
Vestibular dysfunction is also seen in patients with AS. In the study by
Amor-Dorado et al.
[5]
, the incidence of abnormal results in vestibular
tests was high. Erbek et al.
[8]
determined vestibular pathology in 34%
of the patients and statistically signicant abnormal results in caloric
tests.
Similar to other rheumatologic diseases, inner ear involvement must
be kept in mind for patients with AS; these patients must be followed
up with audiological and vestibular examinations.
Informed Consent: Written informed consent was obtained from the patient
who participated in this study.
Peer-review: Externally peer-reviewed.
177
Koç and Emre. Audiovestibular Signs in Ankylosing Spondylitis
Author Contributions: Concept - A.K., I.E.E.; Design - A.K.; Data Collection
and/or Processing - A.K., I.E.E.; Analysis and/or Interpretation - A.K., I.E.E.; Liter-
ature Review - A.K., I.E.E.; Writing - A.K.; Critical Review - A.K., I.E.E.
Conict of Interest: No conict of interest was declared by the authors.
Financial Disclosure: The authors declared that this study has received no
nancial support.
REFERENCES
1. Eryılmaz A, Daglı M, Karabulut H, Acar FS, Inal EE, Gocer C. Evaluation
of hearing loss in patients with ankylosing spondylitis. J Laryngol Otol
2007; 121: 845-9. [CrossRef]
2. Adam M, Erkan AN, Arslan D, Leblebici B, Ozluoglu L, Akman MN.
High-frequency sensorineural hearing loss in patients with ankylosing
spondylitis: is it an extraarticular feature of disease?. Rheumatol Int 2008;
28: 413-7. [CrossRef]
3. Corapcı I, Armagan O, Tascioglu F, Oner C. Sensorineural hearing loss in a pa-
tient with ankylosing spondylitis. Rheumatol Int 2004; 24: 252-3. [CrossRef]
4. Alatas N, Yazgan P, Ozturk A, San I, Iynen I. Audiological findings in patients
with ankylosing spondylitis. J Larygol Otol 2005; 119: 534-9. [CrossRef]
5. Amor-Dorado JC, Barreira-Fernandez MP, Vazquez-Rodriguez TR, Go-
mez-Acebo I, Miranda-Filloy J, de Teran T et al. Audiovestibular manifes-
tations in patients with ankylosing spondylitis. Medicine 2011; 90: 99-
109. [CrossRef]
6. Casellini C, Citera G, Rosemffet M, Ruggeri S, Saviotti A, Cocco AM. Audio-
vestibular disorders in patients with ankylosing spondylitis. J Clin Rheu-
matol 2005; 11: 81-5. [CrossRef]
7. Daglı M, Acar FS, Karabulut H, Eryılmaz A, Inal EE. Evaluation of hearing
and cochlear function by DPOAE and audiometric tests in patients with
ankylosing spondylitis. Rheumatol Int 2007; 27: 511-6. [CrossRef]
8. Erbek SS, Erbek HS, Yılmaz S, Topal O, Yucel E, Ozluoglu LN. Cochleoves-
tibular dysfunction in ankylosing spondylitis. Audiol Neurotol 2006; 11:
294-300. [CrossRef]
9. Kahveci OK, Demirdal US, Duran A, Altuntas A, Kavuncu V, Okur E. Hear-
ing and cochlear function of patients with ankylosing spondylitis. Clin
Rheumatol 2012; 31: 1103-8. [CrossRef]
10. Savastano M, Marioni G, Bernini G, Punzi L. Tinnitus and bilateral sensori-
neural hearing loss: ankylosing spondylitis or a side-effect of sulphasala-
zine treatment? Acta Otolaryngol 2005; 125: 671-3. [CrossRef]
178
J Int Adv Otol 2015; 11(2): 176-8
