International Journal of Pediatric Otorhinolaryngology 79 (2015) 428431

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International Journal of Pediatric Otorhinolaryngology

journal homepage: www.elsevier.com/locate/ijporl

Case Report

Atypical Cogans syndrome: A case report and summary of current

treatment options
Debora Jancatova a, Karol Zelenk a,b,*, Pavel Komnek a,b, Petr Matousek a,b
a
Department of Otorhinolaryngology, University Hospital Ostrava, 17 listopadu 1790, 708 52 Ostrava, Czech Republic
b
Faculty of Medicine, University of Ostrava, Syllabova 19, 703 00 Ostrava, Czech Republic

A R T I C L E I N F O A B S T R A C T

Article history: Cogans syndrome is a rare chronic vasculitis, characterized in its typical form by progressive
Received 25 October 2014 sensorineural bilateral hearing loss, vestibular symptoms and non-syphilitic interstitial keratitis. Only a
Received in revised form 21 December 2014 few cases have been reported in children, most of whom have been diagnosed with the typical form.
Accepted 22 December 2014
Early diagnosis and treatment are crucial to ensure a favorable prognosis. Systemic treatment usually
Available online 3 January 2015
begins with high dosage corticosteroids. In case the initial treatment fails, other immunosuppressive
drugs are used (cyclophosphamide, methotrexate, cyclosporine A and azathioprine). Additional
Keywords:
treatment possibilities, such as plasmapheresis, TNF-alpha blockers (etanercept and iniximab),
Cogans syndrome
Interstitial keratitis
rituximab, tocilizumab and mycophenolate mofetil have been described over the past few years.
Sensorineural hearing loss 2014 Elsevier Ireland Ltd. All rights reserved.
Vertigo
Corticosteroids
Immunosuppressive therapy

1. Introduction 2. Case report

Cogans syndrome (CS) was rst described by the American
ophthalmologist David C. Cogan in 1945. CS is a rare autoimmune
vasculitis characterized by non-syphylitic interstitial keratitis and
bilateral cochlear and vestibular dysfunction [14]. In 1980,
Haynes et al. dened the atypical form of Cogans syndrome as
including audiovestibular symptoms and accompanying intraocu-
lar lesions, such as episcleritis, scleritis, iritis, conjunctivities,
vitritis, retinitis, choroiditis and optical neuritis [3]. CS is a rare
condition and its incidence remains unknown. It is extremely rare
in children, with only a few cases of the atypical form described
in the literature [2]. Without treatment, profound hearing loss
develops in a matter of weeks or months in most patients [1,3].
In children, slightly better outcomes have been observed [2].
The case of a 16 year old female with the atypical form of
the disease is presented and new therapeutic approaches are
discussed.
* Corresponding author at: Department of Otorhinolaryngology, University
Hospital Ostrava, 17 listopadu 1790, 708 52 Ostrava, Czech Republic.
Tel.: +420 597375812.
E-mail address: karol.zelenik@fno.cz (K. Zelenk).
A sixteen year old female presenting with rotational vertigo
lasting for 1 h without nausea sought treatment at the ENT clinic.
Clinical examination of the audiovestibular system showed no
pathology, and no hearing loss was found. The patient was
examined by a neurologist the same day, with a negative result.
Dehydration and stress were considered to be the causes of vertigo.
One week previously, the patient had been treated for episcleritis
with steroid eye drops.
A week after this treatment, rotational vertigo with nausea,
vomiting and bilateral hearing loss appeared. There was no sign of
nystagmus during the clinical examination and audiological tests
revealed bilateral sensorineural hearing loss, mild on the left
side and severe on the right side (Fig. 1). Systemic corticosteroids
(methylprednisolon 250 mg/day), with vasodilatant drugs (vinpo-
cetinum 20 mg/day, pentoxifylin 100 mg/day and betahistin
48 mg/day) were administered, and a 10 day course of hyperbaric
oxygen therapy was completed as well. After 10 days of receiving
this therapy, there was signicant improvement. Vertigo and
episcleritis disappeared completely, but mild sensorineural hearing
loss at high frequencies in both ears remained.
One day after completing the therapy, deterioration of hearing
loss to the pretreatment level was observed and the likelihood of
systemic or autoimmune disease, including Cogans syndrome,

http://dx.doi.org/10.1016/j.ijporl.2014.12.028
0165-5876/ 2014 Elsevier Ireland Ltd. All rights reserved.
 D. Jancatova et al. / International Journal of Pediatric Otorhinolaryngology 79 (2015) 428431 429

Fig. 1. Initial audiological test. Bilateral sensorineural hearing loss, mild on the left side and severe on the right side.

was considered. Detailed examination (chest X-ray, abdominal
ultrasound, brain MRI, PET/CT, serology and immunology tests)
revealed no pathology. The diagnosis of atypical Cogans syndrome
was arrived at the basis of clinical presentation. The treatment
with systemic corticosteroids (methylprednisolon 250 mg/day)
was re-administered for 3 days, with consequent progressive
decrease of the dosage to 8 mg of methylprednisolon per day.
Despite continuation of this treatment (8 mg of methylprednisolon
per day), after initial improvement, deterioration of hearing
occurred again after 3 months. Therefore azathioprine (50 mg)
was added to the treatment regimen. Despite this combined
immunosuppressive therapy the patient developed bilaterally
severe sensorineural hearing loss (Fig. 2) 6 months after the initial
presentation.
3. Discussion
The diagnosis of CS is often delayed or missed because of
extensive differential diagnosis of ocular and audiovestibular
manifestations. Moreover, there are still no specic tests and no
diagnostic criteria. Lately, anti-Hsp70 antibodies have been
suggested as potential markers of typical CS [5]. Nevertheless,
their relevance remains to be conrmed and they seem to be of no
signicance in the diagnosis of the atypical form of CS. Thus, the

Fig. 2. Audiological test after 6 months. Bilaterally severe sensorineural hearing loss.
430 D. Jancatova et al. / International Journal of Pediatric Otorhinolaryngology 79 (2015) 428431
Table 1
Treatment options used in the treatment of patients with Cogans syndrome. Effect, contraindications and the most common side effects are summarized.
Active substance Effect
Cyclophosphamide Immunosuppressant, alkylating agent
Methotrexate Immunosuppressant, antimetabolite
Cyclosporine A Immunosuppressant, interferring
with growth of T-cells
Azathioprine Immunosuppressant, purine analogs
Etanercept TNF-alpha blocker
Iniximab TNF-alpha blocker
Rituximab Anti-CD20 receptor
Tocilizumab Anti-IL-6 receptor
diagnosis of CS is still clinical, based on the combination of
audiovestibular and ocular symptomatology [2,3,7].
Audiovestibular symptoms usually start with an isolated
vestibular syndrome. At the beginning the clinical presentation
resembles Meniers disease (tinnitus and vertigo accompanied by
nausea and vomiting). But unlike Meniers disease, audiovestibular
symptoms are usually bilateral, and in most cases the vestibular
syndrome regresses when the auditory decit appears. Hearing
loss in CS is usually sensorineural, bilateral and progressive [2,3,6].
If untreated or not treated in a timely fashion, the result in most
patients is complete and irreversible deafness in a matter of weeks
or years [2]. This is why clinical suspicion is the basis for proper
diagnosis and early treatment.
The ocular symptoms are pain, redness, photophobia, tearing or
transitory diminution of visual acuity. Unlike the audiovestibular
symptoms, the ocular manifestations typically respond to local
corticosteroids [1,2]. The interval between involvement of the
audiovestibular and ocular symptomatology is usually 16 months
[6].
CS is a kind of chronic vasculitis, therefore systemic clinical
manifestations can be present fever, weight loss, anorexia,
lymphadenopathy, skin rash and cardiovascular signs are the most
frequent [7]. Cardiovascular involvement is considered to be a
serious complication. Aortic insufciency affects 15% of patients
and in some cases requires valve replacement to prevent possible
fatal ventrical insufciency [4,7]. Moreover, same cases with mitral
insufciency, aneurysm of the abdominal aorta and coronary
arteries have been reported [4].
Differential diagnosis of CS includes autoimmune and infec-
tious diseases like systemic lupus erythematosus, Wegeners
granulomatosis, rheumatoid arthritis, polychondritis, Sjogrens
syndrome, polyarteritis nodosa, lyme disease and others [2,7].
Timely treatment is critical for a favorable prognosis and should
begin as early as possible [2]. Systemic treatment starts with high
dosage corticosteroids, usually prednisolone at 1 mg/kg/daily or a
higher dosage [2,7]. Signicant improvement with high dosage
corticosteroid treatment can be expected in Cogans syndrome,
where only limited vasculitis resulting in ischemia of labyrinths
occurs. For ocular inammation, topical corticosteroid treatment is
recommended. In the case of treatment failure, other immunosup-
pressive drugs are used, such as cyclophosphamide (12 mg/kg/
day), methotrexate (7.510 mg/kg/day), cyclosporine A (12 mg/
kg/day) and azathioprine [6,7]. The aim of the treatment is to
prevent irreversible sensorineural hearing loss, deafness, vestibu-
lar dysfunction and irreversible ocular or cardiovascular complica-
tions.
Other treatment possibilities, such as plasmapheresis, TNF-
alpha blockers (etanercept and iniximab), rituximab, tocilizumab
and mycophenolate mofetil have been reported over the past few
Contraindications The most common side effects
Infection, anemia, leukopenia Nausea, alopecia, neutropenia,
leukopenia, SIADH, cardiac failure
Infection, hepatopathy, Hepatopathy, atulency, nausea
renal dysfunction, anemia
Infection, psoriasis, atopic Headache, hypertension, renal
dermatitis, renal dyfunction dysfunction
Infection Infection, leukopenia, nausea
Infection Infection, fever
Infection, cardiac failure Infection, leukopenia, anemia,
headache, nausea
Infection, cardiac failure Infection, cardiac failure, neutropenia,
leukopenia, nausea, headache
Infection Infection, hepatopathy
years (Table 1) [811]. Iniximab and rituximab appear to be
the most effective at an early stage of the disease. This is why
some authors suggest starting treatment with these drugs before
attempting any other immunosuppressive treatment [7,9]. Ritux-
imab, an anti CD20 surface antigen, can not only prevent deafness,
but it may also be used to reduce the number of immunosup-
pressants needed. Etanercept binds TNF-alpha blockers and is
effective in improving word recognition [7]. Tocilizumab therapy, a
humanized anti-interleukin-6 receptor antibody, was prescribed
with good effect in one patient with Cogans syndrome complicat-
ed with aortitis, who was resistant to other immunosuppressive
therapy [10]. Mycophenolate mofetil produces a targeted inhibi-
tion of the proliferation of B and T lymphocytes and has low long
term toxicity and overall good tolerance. Good results were
reported in the treatment of an 11-year-old girl with CS [11].
For patients who progressed to deafness, cochlear implantation
is indicated as soon as possible, because after a period of time
brosis of organ of corti may appear and it can be difcult or
impossible to perform cochlear implantation [7,1215].
4. Conclusion
There should always be suspicion of Cogans syndrome when
a combination of bilateral audiovestibular symptoms and ocular
symptoms occur. Early diagnosis and treatment is crucial for a
favorable prognosis. In addition to corticosteroids and formerly
used immunosuppressive drugs, new treatment modalities such as
plasmapheresis, TNF-alpha blockers, tocilizumab and mycopheno-
late mofetil have been described over the past few years.
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