Otolaryngology–Head and Neck Surgery (2007) 136, 82-86
ORIGINAL RESEARCH
Audiovestibular disturbance in patients with
systemic lupus erythematosus
Erkan Karatas, MD, Ahmet Mesut Onat, MD, Cengiz Durucu, MD,
Tekin Baglam, MD, Muzaffer Kanlikama, MD, Orcun Altunoren, MD,
and Hakan Buyukhatipoglu, MD, Gaziantep, Turkey
OBJECTIVE: The aim of this study was to evaluate the audio-
vestibular disturbance in patients with systemic lupus erythema-
tosus (SLE).
STUDY DESIGN AND SETTING: Twenty-eight patients with
SLE and 28 healthy control subjects were included. Pure-tone
audiometry, impedance audiometry, and electronystagmography
(ENG) were used for baseline evaluation. Laboratory tests were
carried out. Cranial and brain stem magnetic resonance imagings
(MRI) were undertaken.
RESULTS: Nineteen (67%) patients reported audiovestibular
symptoms. Sensorineural hearing loss was found in 6 (21%) pa-
tients. Abnormal results on ENG were significantly higher (50%)
(P ⬍ 0.01). Abnormal laboratory data were available from 26
patients (P ⬍ 0.05). MRI did not show any pathosis.
CONCLUSION: The audiovestibular disturbances in SLE are
more prevalent than previously recognized. Although no cause and
effect relationship can be established by this type of study, it
appears that a relationship exists.
SIGNIFICANCE: Audiologic research should be directed to-
ward routine, pure tone audiometry, and ENG assessment for
patients with SLE to enable crucial treatment.
© 2007 American Academy of Otolaryngology–Head and Neck
Surgery Foundation. All rights reserved.
S ystemic lupus erythematosus (SLE) is considered a pro-
totype of the autoimmune diseases because of its mul-
tiorgan involvement. Pathogenic immune complexes and
autoantibodies are the main causes of organ damage. The
clinical picture of SLE may be mild or severe. Nevertheless,
all organs are susceptible during the course of the disease,
which is typically characterized by alternating periods of
activity and quiescence.1
From the Department of Otolaryngology–Head and Neck Surgery (Drs
Karatas, Durucu, Baglam, and Kanlikama), and the Section of Rheumatology,
Department of Internal Medicine (Drs Onat, Altunoren, and Buyukhatipo-
glu), Faculty of Medicine, Gaziantep University, Gaziantep, Turkey.
0194-5998/$32.00 © 2007 American Academy of Otolaryngology–Head and Neck Surgery Foundation. All rights reserved.
doi:10.1016/j.otohns.2006.06.1255
Auditory symptoms in SLE patients have rarely been
reported in the literature and these are seldom the initial
manifestations of the disease.2 Sensorineural hearing loss
(SNHL) and aural symptoms in SLE patients were first
identified by Kastanioudakis et al3 and Sperling et al.4
Since the description of the first case series in 1979,
several designations have been given to the syndrome of
sensorineural hearing loss (SNHL) that is often accompa-
nied by vertigo and tinnitus. These names include autoim-
mune SNHL and idiopathic progressive bilateral SNHL.5
Since that time, numerous studies have documented the
presence of auditory symptoms in various autoimmune dis-
orders such as Cogan’s syndrome, Wegener’s granuloma-
tosis, polyarteritis nodosa, temporal arteritis, and SLE.
Vertigo and dizziness are not common symptoms in SLE
but are probably present more often than was formerly thought.
However, there are only a few articles that describe vertigo in
SLE; therefore, this subject is not a well-known issue for many
otolaryngologists.6 The objective of this study is to compare
the frequencies of audiovestibular abnormalities in SLE and
healthy controls with the use of pure tone audiometry, imped-
ance audiometry, and ENG (electronystagmography) and to
investigate predictors of ENG abnormalities in SLE patients.
STUDY DESIGN AND SETTING
The study was conducted as a prospective, randomized, and
controlled study. It is approved by the ethics committee of
our faculty, and written informed consent was obtained
Reprint requests: Erkan Karatas, MD, Gaziantep University, Medical
Faculty Sahinbey Medical Center, Otolaryngology–Head and Neck Sur-
gery Department (KBB) 27060 Gaziantep, Turkey.
E-mail address: erkaratas@yahoo.com.
Karatas et al Audiovestibular disturbance in patients with . . . 83

from each patient and healthy controls in accordance with Cranial brain stem magnetic resonance imagings (MRI)
the ethical principles for human investigations, as outlined of patients with vestibular disturbances were performed to
in the 2nd Helsinki Declaration. Twenty-eight healthy con- evaluate the central nervous system.
trols and 28 patients with SLE who were regularly followed The statistical analysis of the audiovestibular symptoms
by the Department of Rheumotology and who fullfilled the and pure tone audiometry, ENG and laboratory results were
American Rheumatism Association7 revised criteria for performed with the Chi-square test. Differences in the me-
SLE were included in this study. The control group did not dian of age between SLE and control group was performed
have any acute or chronic otologic disease or any otologic with the Mann-Whitney U test. All data were analyzed using
and acoustic history of trauma or surgery. SPSS (Version 13.0) for Windows (SPSS) with differences at
Patients and controls were asked and examined for any P ⬍ 0.05 interpreted as statistically significant.
audiovestibular symptoms and any history of audiovestibu-
lar disturbance, cranial trauma, and exposure to noise, ear
infection, metabolic disease, and ototoxic drug use. Patients
and controls who did not meet these criteria were excluded. RESULTS
All subjects underwent complete systemic examination,
There were three men and 25 women in the SLE group and
otolaryngologic examination, and also the following audio-
the mean age was 35.6 years (18 to 71 years). The mean age
logic evaluations: pure-tone audiometry (0.25, 0.5, 1, 2, 4,
of the control group was 34.6 years (18 to 66) (6 male and
and 6 kHz), impedance audiometry, ENG, and caloric test.
22 female). There was no statistically significant difference
Otologic assessments of patient and control groups were
in the median age between the groups.
performed using pure tone audiometry (Interacoustics AC
Nineteen (67%) of 28 patients with SLE reported to have
40, Clinical Audiometer, Assens, Denmark) and tympanom-
audiovestibular symptoms including tinnitus in nine (32%),
etry (Interacoustics AZ T, Impedance Audiometer, Den-
hearing loss in two (7%), vertigo in eight (28%) patients.
mark, calibrated to ANSI S3.39-1987 standards). The clas-
Nine (32%) patients had no audiovestibular symptoms.
sification of the audiovestibular assessments was done by Pure tone audiogram showed sensorineural hearing loss
one researcher who was blinded to groups. The otologic (ⱖ25 dB hearing level in pure tone averages of 0.5, 1, and
assessment was considered normal if the hearing threshold 2 kHz frequencies and ⱕ95 percent speech discrimination
was ⱕ25 dB hearing level in pure tone averages of 0.5, 1 scores) in 6 of 28 SLE patients (21.42%). Hearing loss was
and 2 kHz frequencies and ⱖ95 percent speech discrimina- unilateral in three and bilateral in three SLE patients. The
tion scores in pure tone audiometry, if there was no con- mean audiogram of patients was in the normal range in all
duction type hearing pathology, if peak of compliance was frequencies (Fig 1). The tympanogram did not show any
between 0.5-1.5 mL, and if middle ear pressure was be- abnormalities. None of the healthy controls had any kind of
tween ⫹50 and ⫺100 mm H2O in tympanometry. hearing loss, and their pure tone audiogram revealed bilat-
ENG with thermostatic otocalorimeter was used (Chart erally normal thresholds in all subjects.
Diagnostic systems, MCU-90, ICS Medical, Schaumburg, Abnormal results on ENG were significantly more fre-
IL, USA). The following tests applied in video ENG were, quent in SLE (50%) compared with the control group
in sequence: 1) saccadic test; 2) pursuit test; 3) gaze test; 4) (7.14%) (P ⬍ 0.01). The SLE group had significantly more
positional test; and 5) caloric test. The results were evalu- peripheral type vestibular pathology compared with the con-
ated according to criteria defined by Barber and Stockwell.8
The test results were classified as follows: 1) central
vestibulopathy in the presence of spontaneous central nys-
tagmus, dysmetric ocular movement, or abnormal pursuit
test results; 2) peripheral vestibulopathy in the presence of
classical paroxysmal positional nystagmus or an absence or
reduced caloric response with a 20 percent degree or more
in unilateral weakness or unilateral gaze nystagmus; 3)
nonspecific vestibulopathy in the presence of atypical posi-
tional nystagmus or directional preponderance; and 4)
mixed vestibulopathy in the presence of both central and
peripheral vestibulopathy features at the same time.8
The laboratory tests of erythrocyte sedimentation rate
(ESR), complete blood count, urine analysis, renal and liver
biochemistry, lung x-rays, C-reactive protein (CRP), C3-C4
components of complement, rheumatoid factor (RF), anti-
double-stranded DNA (dsDNA), antinuclear antibody (ANA), Figure 1 Mean audiograms of patients with SLE and control
anticardiolipin and antiphospholipid antibodies (both IgM group were in normal range in all frequencies. Control group had
and IgG) were analyzed. better results than SLE group. (A, SLE group; B, control group).
84 Otolaryngology–Head and Neck Surgery, Vol 136, No 1, January 2007

10 (35.71%) patients had positive anticardiolipin antibody

Table 1
Audiovestibular symptoms in SLE patients IgG and IgM. Cranial MRI did not show any pathosis in
patients with vestibular disturbances.
Audiovestibular Number of
symptoms patients Percentage

Tinnitus 2 7.14
Hearing loss — —
Vertigo 3 10.72
Tinnitus ⫹ hearing loss ⫹
vertigo 2⫹1⫹2 17.86
Tinnitus ⫹ hearing loss 3⫹1 14.28
Tinnitus ⫹ vertigo 2⫹3 17.85
No symptoms 9 32.15
Total 28 100
trol group (P ⬍ 0.05) (Tables 1 and 2). Prevalence of
vertigo in SLE was higher in patients with abnormal ENG
results (P ⬍ 0.01). There was no significant correlation
between the ENG abnormalities and hearing loss (P ⬎
0.05).
One patient had atypical positional nystagmus in sitting
and supine vision horizontal and 1 patient had reduced
caloric response in control group. Nineteen patients had
abnormal ENG findings in SLE group (Table 3). These
consisted of no caloric response (1 patient), reduced ca-
loric response (11 patients), unilateral left gaze nystagmus
(1 patient) in central and horizontal position, dysmetric
ocular movement (2 patients), abnormal pursuit (1 patient)
in central position, directional preponderance (2 patients),
atypical positional nystagmus (1 patient).
Abnormal laboratory data and ENG findings of SLE
group are summarized in Table 4. The control group had
normal laboratory data. Abnormal laboratory data were
available from 17 patients with ENG abnormalities and 9
without (P ⬍ 0.01) in the SLE group. All patients had a
normal urine analysis count, and lung x-rays. Twenty-one
(75%) patients had an elevated ESR, 50 percent of patients
tested positive for rheumatoid factor, and 82.14 percent of
patients had at least 1 abnormal low complement factor
level (C3 or C4). All patients tested positive for the ANA.
With respect to the antiphospholipid antibody IgG and IgM
screen, nine (32.14%) patients had positive test findings and
DISCUSSION
Immune-mediated audiovestibular abnormalities have been
widely studied in the past 20 years. However, despite great
efforts to outline the possible pathogenetic mechanisms, the
origins of these syndromes still remain mere hypotheses.8
Over the years, many clinical factors such as the active
phase of the autoimmune disease, the individual clinical
history, the varied types of therapeutic regimens have been
taken into consideration. None of these, however, has led to
significant results or clarifying conclusions.
SLE is the prototypical immune-mediated disease. Veld-
man9 has postulated that a vasculitis within the cochlea may
involve the stria vascularis, the spiral ligament, or the in-
ternal auditory artery. Caldarelli et al10 suggested that cir-
culating immune complexes, rather than vasculitis, are the
cause of microinfarctions of the capillaries or arterioles in
the temporal bone.10 Sone et al11 in a histopathologic study
of temporal bones of seven patients with SLE showed a loss
of spiral ganglion cells, various degrees of hair cell loss, and
atrophy of the stria vascularis. Cochlear hydrops was found
only in patients with positive anticardiolipin antibodies.
Bouman et al12 in an experimental study demonstrated that
the perisaccular deposition of immune complexes was a
cause of endolymphatic hydrops.
Auditory symptoms in SLE patients are frequently re-
ported in the literature and are sometimes present as the
initial manifestation of the disease. In one study, HL that
was unexplained by the patient’s otologic history was ob-
served in two (8%) of 30 SLE patients.1-4,13,14 In this study,
the patients were asymptomatic on an initial interview be-
fore audiometry. HL was unilateral in one patient and bi-
lateral in the other. Sperling et al4 reported unilateral hear-
ing loss in 13 (15%) of 84 and bilateral hearing loss in 14
(17%) of 84 patients with SLE. In our study, the presence of
tinnitus and vertigo was more common than among the
control group. Hearing loss was unilateral in 3 (10.71%) of

Table 2
Vestibular pathology frequencies detected according to ENG results in SLE groups and control groups

SLE group N ⫽ 28 Control group N ⫽ 28

N Percentage N Percentage

Hearing loss 6 21.42 — —

Central vestibulopathy 2 7.14 — —
Peripheral vestibulopathy 10 35.71 1 3.57
Nonspecific vestibulopathy 3 10.71 1 3.57
Mixed vestibulopathy 4 14.28 — —
N, number of patients.
Karatas et al Audiovestibular disturbance in patients with . . . 85

Table 3
Vestibular abnormalities in SLE patients

Central Peripheral Nonspecific Mixed

vestibulopathy vestibulopathy vestibulopathy vestibulopathy Total

Gaze nystagmus — — — 1 1
Saccade nystagmus — — —
Abnormal optokinetic nystagmus 1 — — 1 2
Abnormal pursuit 1 — — — 1
Spontaneous nystagmus — — — — —
Positional nystagmus — — 1 — 1
Directional preponderance — — 2 — 2
Absent caloric response — 1 — — 1
Reduced caloric response — 9 — 2 11
Total 2 10 3 4 N ⫽ 19
N, number of patients with vestibular abnormalities.

28, bilateral in three (10.71%) of 28, tinnitus was present in Ototoxic antibiotics, meningitis, tumors, bilateral en-
nine (32%) of 28, and vertigo in four (14%) of 28 SLE dolymphatic hydrops, and systemic autoimmune disorders
patients. (eg, lupus erythematosus, polychondritis, Cogan syndrome,
The inner ear may become vulnerable during the course rheumatoid arthritis) are among the many causes of periph-
of the disease. There are only a few articles describing eral vestibulopathy. Suggested immune mechanisms for this
vertigo in SLE.6,15 Liao et al6 presented a 11-year-old boy presentation have included a humoral-type antibody attack
with SLE with a fever, headache, and severe vertigo with on inner ear antigen,16 cell-mediated cytotoxicity to inner
bilateral vertical nystagmus. In our study, abnormal results ear antigen,17 and immune-complex disease in the mi-
on ENG were significantly frequent in SLE group (50%) crovessels of the inner ear.18 In our study, abnormal labo-
compared with the control group (7.14%). The SLE group ratory data (C3, C4, RF, ANA, dsDNA, ESR and CRP)
had significantly more peripheral type vestibular pathology were discerned in 17 patients with ENG abnormalities and
compared with the control group. Prevalence of vertigo in 9 without. There was a correlation between abnormal lab-
SLE was higher in patients with abnormal ENG results. oratory data and ENG abnormalities in SLE group.
Abnormal ENG findings and vestibular symptoms are prob- Systemic deposition of immune complexes typical of
ably present more often than were formerly thought in SLE SLE may act to incite a destructive process in the inner ear.
patients. In addition, a number of case reports have highlighted the
possible association of anticardiolipin antibodies.2 Mouadeb
and Ruckenstein19 proposed the hypothesis that antiphos-
Table 4 pholipid antibodies were involved in the pathogenesis of
ENG findings and abnormal laboratory data some forms of inner ear dysfunction, presumably by causing
ENG findings microthrombus formation in the labyrinthine vasculature.
Basic science studies are required to better understand the
Normal Abnormal mechanisms by which antibodies mediate inner ear dysfunc-
(%, N) (%, N)
tion. A statistically significant increase in the incidence of
ESR (ref: 0-20 mm/h) 25, 7 50, 14 antibodies and a decrease in the C3-C4 levels (P ⬍ 0.01) was
CRP (ref: ⫹ or ⫺) 14.28, 4 25, 7 found in this study population when compared with the inci-
C3 (ref: 0.9-1.8 gr/L) 10.71, 3 35.71, 10 dence in epidemiologically analogous normal subjects.
C4 (ref: 0,1-0.4 gr/L) 25, 7 28.57, 8 The identification of the pathogenetic mechanism that
RF (ref: ⫹ or ⫺) 21.42, 6 28.57, 8
determines audiovestibular symptoms is thus crucial to the
ANA (ref: ⫹ or ⫺) 32.14, 9 67.85, 19
dsDNA (ref: ⫹ or ⫺) 14.28, 4 14.28, 4 choice of a therapeutic treatment. SNHL or vertigo in SLE
IgG (ACL) 10.71, 3 14.28, 4 has been generically treated with many therapy regimens,
IgM (ACL) (ref: ⫹ or ⫺) 3.57, 1 7.14, 2 but we still do not know which of these treatments is most
IgG (APL) 10.71, 3 10.71, 3 appropriate and effective in these forms of hearing loss and
IgM (APL) (ref: ⫹ or ⫺) 7.14, 2 3.57, 1
vertigo.1 Multicenter studies are necessary in order to cat-
N, number of patients; ESR, erythrocyte sedimentation egorize and select significant sample groups of patients and
rate; CRP, C reactive protein; C3-C4, C3-C4 component of
apply the various therapeutic regimens under study.
complement; RF, rheumatoid factor; ANA, antinuclear anti-
body; DNA, antidouble-stranded DNA; ACL, anticardiolipin The evaluation of audiovestibular disturbances in SLE
antibody; APL, antiphospholipid antibody. patients requires a questionnaire for extensive and complete
history taking, complete laboratory analysis, audiograms,
86 Otolaryngology–Head and Neck Surgery, Vol 136, No 1, January 2007
and vestibular function tests for the treatment. A significant
number (19 of 28; 67%) of the SLE population in this study
reported subjective audiovestibular symptoms and objective
ENG findings.
Our study is not a large study group but it is the first
study to find the audiovestibular disturbances in SLE. How-
ever, it would be unfair for us to perform a prospective
study in a larger SLE group. SNHL and vestibulopathy are
common in SLE and are probably present more often than
were formerly thought. Although no cause and effect rela-
tionship can be established by this type of study, it appears
that a relationship exists.
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