Cogan syndrome (CS) is a rare disease that involves a wide range of audiovestibular, ophthalmological and unusual systemic manifestations. There are very few cases reported in Spain. We described a typical CS with permanent deafness.
Methods
A 40-year-old man presented to ophthalmology department complaining of blurred vision and painful red left eye. Visual acuity was 20/25. On examination with slit lamp, Tyndall’s phenomenon was observed. 48 hours later, keratic precipitates and corneal stromal infiltrates were found. Two months before, he was diagnosed with bilateral idiopathic labyrinthitis and secondary cophosis. After carrying out complementary tests, a diagnosis of CS was established. Topical dexamethasone and systemic methylprednisolone and rituximab were started. After a week, the ophthalmological symptoms improved, however cophosis did not mitigated.
Results
CS is a rare disease (approximately 300 cases described in the literature), whose etiology is not clearly elucidated. Multiple ophthalmological manifestations have been reported, including interstitial keratitis and uveitis, as in this case, in addition to scleritis, optical neuritis and even retinal vasculitis. The vestibulo-auditory manifestations are similar to Ménière syndrome, as well as hearing loss up to 90% of the cases. Diagnosis is based on clinical manifestations, after exclusion of inflammatory or infectious causes. Management is not well established, and it has been observed that even though corticosteroids are the most used, they are not proven effective. The visual prognosis is good; however, audiovestibular affectation is usually irreversible.
Conclusion
CS, continues to be a diagnostic and therapeutic challenge, which can lead to deafness and blindness.
Conflict of interest
No
Authors 1
Last name
MEJIA-FREIRE
Initials of first name(s)
S
Department
Ophthalmology, Hospital Clinico Universitario de Valladolid
City
Valladolid
Country
Spain
Authors 2
Last name
PRIETO DOMINGUEZ
Initials of first name(s)
L
Department
Ophthalmology, Hospital Clinico Universitario de Valladolid
City
Valladolid
Country
Spain
Authors 3
Last name
COCHO ARCHILES
Initials of first name(s)
L
Department
Ophthalmology, Hospital Clinico Universitario de Valladolid
City
Valladolid
Country
Spain
Authors 4
Last name
HERRERAS CANTALAPIEDRA
Initials of first name(s)
JM
Department
Ophthalmology, Hospital Clinico Universitario de Valladolid
City
Valladolid
Country
Spain
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