Download Cogan`s Syndrome: Auditory and Medical Management

J Am Acad Audiol 3 : 225-229 (1992)
Cogan's Syndrome : Auditory and Medical
Management
Diane C. Osborne*
John T. Jacobson*
Mary L. Olsen'
Abstract
Cogan's syndrome is a rare autoimmune disease characterized by the presence of interstitial
keratitis and audiovestibular symptoms . The audiovestibular symptoms include fluctuating
sensory hearing impairment, tinnitus, vertigo, and reduced vestibular response . Immediate
diagnosis and medical intervention provides optimum auditory recovery . Frequent audiologic
assessments are necessary to monitor the disease activity and to aid in the therapeutic levels
of steroidal medications . Amplification is often required on a temporary or permanent basis.
Two case studies are presented to illustrate the audiologist's role in the identification and
management of patients with Cogan's syndrome .
Key Words: Cogan's syndrome, interstitial keratitis, autoimmune disease, vasculitis,
hearing loss, amplification, corticosteroid therapy
ogan's syndrome (Cogan, 1945) is considered a nonsyphilitic, autoimmune disease characterized by the presence of
interstitial keratitis in conjunction with vertigo, tinnitus, and hearing loss (Smith, 1970 ;
Peeters et al, 1986 ; Barna and Hughes, 1988 ;
Hughes et al, 1988 ; Jahrsdoerfer et al, 1988).
Other symptoms may include vasculitis and
general systemic complaints such as fever,
weight loss, and fatigue . Histopathologic evidence indicates infiltration of lymphocytes and
plasma cells in the cornea and the spiral ligament (Hughes et a1,1983; McDonald et a1,1985;
Barna and Hughes, 1988).
Cogan's syndrome affects both men and
women with onset ranging from 20 to 50 years
of age (median age 25 years) . The interstitial
keratitis component presents clinically as
photophobia, decreased visual acuity, ocular
pain and redness (Peeters et al, 1986), and to a
C
*Department of Otolaryngology Head and Neck Surgery, Division of Audiology, The University of Texas Medical
School at Houston, Houston, Texas
t Department of Internal Medicine, Division of
Rheumatology and Clinical Immunogenetics, The University
of Texas Medical School at Houston, Houston, Texas
Reprint requests : John Jacobson, The University of
Texas Medical School at Houston, Department of
Otolaryngology Head and Neck Surgery, 6431 FanninMSB 6 .132, Houston, TX 77030
lesser degree, conjunctivitis, uveitis, and
scleritis have also been observed (Hughes et al,
1983) . Other possible laboratory findings may
include medium and small vessel vasculitis and
nonspecific inflammatory signs such as
leukocytosis, thrombocytosis, anemia, and elevated erythrocyte sedimentation rate . Differential diagnosis involves ruling out other causes
of interstitial keratitis (e .g ., congenital syphilis), other forms of autoimmune inner ear disease, audiovestibular symptoms with eye involvement (e .g., viral labyrinthitis), or systemic
diseases with eye, and ear complaints (e .g .,
relapsing polychrondritis) .
AUDITORY OR VESTIBULAR DEFICITS
he audiovestibular component presents
Tclinically with a fluctuating hearing loss,
vertigo, nystagmus, nausea, and vomiting
(Bachynski and Wise, 1984 ; Morgan et a1,1984;
Jahrsdoerfer et al, 1988). The hearing loss is
sensory in nature and may be either unilateral
or bilateral. Auditory sensitivity fluctuates between the range of normal limits and a severe
hearing auditory deficit. Interestingly, puretone thresholds may remain stable from one
evaluation to the next, yet word recognition
ability may show significant deterioration or
improvement. Typically, an initial evaluation
Journal of the American Academy of Audiology/Volume 3, Number 3, May 1992
shows sensory hearing loss with abnormal
electronystagmography .
The clinical course may begin with either
ocular or audiovestibular complaints, but both
are usually present within 1 month of onset. If
untreated, severe to profound auditory deficits
generally occur within 3 months (Vollertsen et al,
1986). Immediate recognition and intervention
with high-dose corticosteroids (1 .0-1 .5 mg/kg/
day) is essential to provide optimum auditory
recovery . Studies suggest the administration of
corticosteroids must be initiated within 2 weeks
of initial presentation of hearing loss for adequate auditory restoration (McDonald et al,
1985 ; Laffin et al, 1987 ; Barna and Hughes,
1988).
CASE REPORTS
he following case reports describe the audiTtory and clinical findings of two young adult
females who presented with Cogan's syndrome .
Both patients demonstrated typical interstitial
keratitis and associated fluctuating auditory
deficits of varying degree and amelioration .
Both patients received corticosteroids in the
management of their disease and audiometric
work-ups that demonstrated improvements in
hearing sensitivity as a result . Although ampli-
fication was required during therapy for both
patients, only one patient required long-term
auditory rehabilitative management .
Case 1
CR is a 25-year-old Hispanic female who
initially presented with left ear tinnitus . One
week later she experienced sudden nausea,
vomiting, and an ataxic gait . By the second
week, there was evidence of significant left ear
auditory deficits . The complaint of photophobia
and "foggy vision" prompted the suspicion of
Cogan's syndrome . Figure 1 illustrates the results of the first baseline audiologic evaluation .
The patient was immediately placed on highdose corticosteroids (Prednisone, 60 mg daily)
while differential diagnosis was completed.
Formal evaluation involved complete rheumatologic, ophthalmologic, and otolaryngologic
examinations . Laboratory findings included
nonspecific inflammatory signs of thrombocytosis, leukocytosis, elevated erythrocyte sedimentation rate, and hyperglobulinemia .
Initially, hearing sensitivity and visual acuity showed a rapid and dramatic improvement
to the high-dose anti-inflammatory drug regimen. Figure 1 shows the audiometric improvements in left ear scores . Vestibular abnormaliFigure 1 Audiometric records of patient CR. Serial
audiograms cover an 8-month
period showing fluctuation of
PURE TONE AUDIOMETRY
LEFT EAR
RIGHT EAR
dB .
FREQUENCY (Hz)
dSo
FREQUENCY (Hz)
4K
pure-tone sensitivity.
SK
00
IN
Date of Assessment
0 030590 baseline
SPEECH AUDIOMETRY
a
A
(PB word recogni[(on scores)
HL in d8
226
04-o9.901sttreaunent
06.29-90 treatment tapered
0 11-12.90 current status
HL in dB
00
IY~lGllil~l MUat4ut~l
INioI lIIlrllldi
lti 1111 lta ' f : . .
IAINC11w i ~OA I I,
+
, . mew
Cogan's Syndrome/Osborne et al
ties were somewhat slower to improve however,
and a residual ataxic gait remained . After 1
month of treatment, a cautious steroid taper
was begun. Three months into the steroid taper,
the patient experienced a significant flare-up
with new right ear pain, tinnitus, and further
hearing loss that is presented in Figure 1.
Amplification was then initiated to support
communication demands . Steroid treatments
were immediately increased to previous levels .
Ocular steroids and atropine were begun to
address ocular inflammation . After development of upper extremity weakness, immunosuppressants were further increased by the
addition of IV-pulse methylprednisolone and a
cytotoxic agent-azathioprine .
With steroid administration, CR had a subsequent improvement of all symptomatology
and although a mild hearing loss remained,
amplification was no longer required . CR remained audiologically stable throughout the
second steroid taper as demonstrated in Figure
1 . Audiologic management continues routinely
and although a mild decrease in pure-tone sensitivity has been observed above 3000 Hz in the
right ear, speech recognition has remained stable with no subjective change in right ear sensitivity . Currently, the patient does not use amplification but recognizes the potential need
given possible fluctuations in decreased auditory sensitivity .
Case 2
LC, a 27-year-old white female, initially
presented clinically with ocular symptoms of
erythema, pain, and photophobia. This was
interpreted as an allergic reaction and discontinuation of contact lens use was recommended .
In the following 2 weeks, the diagnosis of interstitial keratitis was made and treated locally.
Following, she experienced sudden onset of
vertigo, nausea, vomiting, and left ear sensory
hearing loss . Initial baseline audiometrics are
seen in Figure 2. Symptoms worsened over the
next 6 weeks and a moderate-to-severe hearing
loss was recorded bilaterally . The eventual diagnosis of Cogan's syndrome finally initiated
corticosteroid therapy. Although left ear symptoms improved with a subsequent dramatic
improvement in word recognition ability, right
ear abnormality continued to deteriorate . Figure 2 reflects the decrease in right ear sensitivity with essentially stable left ear results .
As the disease entered a remission state,
hearing sensitivity began to stabilize and steroid treatments were tapered off to cessation.
Figure 3 shows the results of testing performed
PURE TONE AUDIOMETRY
RIGHT EAR
Figure 2 Initial series of audiometric records of patient LC
with original baseline .
LEFT EAR
FREQUENCY (Hz)
so
dB,
FREQUENCY (Hz)
4i
BK
AK
L
L
100
Date of Assessment
SPEECH AUDIOMETRY
(PB word recognition scores)
1
n
4
80
HL in dB
100
0
02-12-86 baseline
El
04-16-86 remission
Z~, 10-22-86 marked RE loss
%
20
"
60
HL in dB
100
Journal of the American Academy of Audiology/volume 3, Number 3, May 1992
during remission of the disease. Of particular
interest are the unexplained fluctuations of
speech recognition scores with little change in
pure-tone thresholds . This phenomenon reflects
the often unrecognized existing differences between the physiologic complexity of speech
processing and the comparative rudimentary
requirements of pure-tone measurement.
One year following remission, symptomatology recurred with associated sudden left
ear decreased auditory sensitivity (see Fig. 3) .
The reinstitution of steroid treatments was
successful in re-establishing the previous baseline audiometric in the left ear, however, little
overall auditory recovery was observed . Immunosuppressive therapy has been discontinued and no further recovery is expected . The
most recent audiometric evaluation is shown in
Figure 3. LC was initially given an FM system
as she pursued speech reading classes, psychological counseling, and coping strategies through
various resources. She presently wears binaural amplification for daily communicative needs.
LC is now accustomed to monitoring her auditory acuity and speech recognition abilities and
returns for periodic re-evaluation and hearing
aid monitoring. This case clearly demonstrates
the need for longitudinal audiologic management in Cogan's syndrome .
COMMENTS
A
Ithough Cogan's syndrome is rare, it should
be considered a possibility in patients
presenting with audiovestibular symptoms
similar to Meniere's disease. This suspicion
should be heightened by the concurrent presence of ocular symptoms . As demonstrated in
the two case reports, prompt corticosteroid
treatment should be initiated to prevent permanent severe sensory hearing impairment
while formal diagnostic evaluation is conducted . It becomes clear that audiologic documentation of fluctuating hearing loss is a primary
criterion in the diagnosis and management of
Cogan's syndrome . Frequently, treatment decisions are based on audiologic evaluation, and
therefore, open dialogue between collaborators
is necessary during the course of the disease
process .
The optimum use of amplification in the
rehabilitative management of Cogan's syndrome
is challenging. Due to the fluctuating auditory
nature of the syndrome, a versatile hearing aid
and thorough patient counseling is recommended . Amplification may be necessary at any
time and therefore continuous audiologic monitoring is a requirement. Hearing sensitivity
PURE TONE AUDIOMETRY
LEFT EAR
RIGHT EAR
SW
FREQUENCY (Hz)
Figure 3 Final series of audiometric records of patient
LC indicating remission and
exacerbation ofhearing loss in
the left ear.
1m
Date of Aaseaamant
SPEECH AUDIOMETRY
(PB word recognftlon scores)
tao
20
80
HL in dB
100
HL in dB
!1M !IW ~i~ll 111
l1~ Iihf14l~I l~itlt i!If! 1!.k1611t114 &~
Cogan's Syndrome/Osborne et al
may fluctuate such that very different styles
and models of hearing aids may be required .
The patient must be aware of the flexibility and
limitations of each hearing aid in order to utilize appropriate fitting. Because auditory deficits may be the first indication of disease exacerbation, long-term audiologic follow-up is necessary to monitor the disease activity . Finally,
these case reports unquestionably demonstrate
the required working relationship between patient, physician, and audiologist in the diagnosis and medical and audiologic management of
Cogan's syndrome .
Hughes GB, Barna BP, Kinney SE, Calabrese LH, Nalepa
NJ . (1988). Clinical diagnosis of immune inner-ear disease . Laryngoscope 98 :251-253 .
Hughes GB, Kinney SE, Barna BP, Tomsak RL, Calabrese
LH . (1983) . Autoimmune reactivity in Cogan's syndrome : a
preliminary report . Otolaryngol Head Neck Surg 91 :24-32 .
Jahrsdoerfer RA, Hall JW 111, Gray L. (1988) . A review of
syndromes that deafen . In : Bess FH, ed . Hearing Impairment in Children, Parkton, MD : York Press, 57-64.
Lain MA, Winkelaar R, Diduch LT . (1987) . Vestibuloauditory impairment in Cogan's syndrome : a case
report . J Otolaryngol 16 :137-139 .
McDonald TJ, Vollertsen RS, Younge BR . (1985) . Cogan's
syndrome : audiovestibular involvement and prognosis in
18 patients . Laryngoscope 95 :650-654 .
Acknowledgment . Portions of this article were presented at the 3rd Annual Convention of the American
Academy of Audiology, Denver, Colorado, April, 1991 .
Morgan GJ, Hochman R, Weider DJ . (1984) . Cogan's
syndrome: acute vestibular and auditory dysfunction
with interstitial keratitis . Am J Otolaryngol 5:258-261 .
REFERENCES
Peeters GJ, Cremers CW, Pinckers AJ, Hoefnagels WH .
(1986) . Atypical Cogan's syndrome : an autoimmune disease? Ann Otol Rhinol Laryngol 95 :173-175 .
Barna BP, Hughes GB . (1988) . Autoimmunity and otologic disease: clinical and experimental aspects. Clin Lab
Med 8:385-398 .
Bachynski B, Wise J. (1984) . Cogan's syndrome : a treatable cause of neurosensory deafness . Can J Ophthalmol
19:145-147 .
Cogan DG . (1945) . Syndrome of non-syphilitic interstitial
keratitis and vestibuloauditory symptoms . Arch Ophthalmol 33:144-149 .
Smith JL . (1970) . Cogan's syndrome . Laryngoscope
80:121-132 .
Vollertsen RS, McDonald TJ, Younge BR, Banks TM,
Stanson AW, Ilstrup DM . (1986) . Cogan's syndrome : eighteen cases and a review of the literature . Mayo Clin Proc
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