Cogan’s syndrome is a chronic inflammatory disease of unknown origin and an autoimmune disease, featuring bilateral sensorineural hearing loss, vestibular symptoms, and inflammatory ocular manifestations with variable risk of developing into a systemic disease. 

A report describes a case of a 31-year-old white female who presented with vertigo, hearing loss and tinnitus referred in the left ear, alongwith a one-week history of bilateral conjunctivitis treated with topical corticosteroids, which occurred after pharyngeal inflammation that initiated before 4-5 days. 

First otorhinolaryngologic (ORL) evaluation revealed acute left vestibular paresis without cutaneous herpes signs. 

Despite the description of symptoms, audiometry revealed normal bilateral and symmetric hearing function. White blood count was found to be 13640/mm3 and erythrocyte sedimentation rate was found to be elevated (70 mm/h) with all other laboratory tests occuring within normal limits. Ophthalmologic evaluation confirmed a diagnosis of bilateral conjunctivitis. 

Three days after initiating the treatment, worsening of symptoms was described, accompanying with headache, mild left sensorineural hearing loss and relevant dizziness. Systemic corticosteroid treatment (prednisone 60 mg i.v/day) was initiated. The left vestibular paresis was confirmed by Caloric testing. Auditory Brainstem Responses (ABR) utilizing 120 dB SPL click stimulus revealed a delay of I, III, V waves and a normal I-V latency interval on the left side. 

Following 10 days of treatment further worsening of ocular inflammation, headache, left hemifacial pain and periauricular paraesthesia was described. Neurologic evaluation and laboratory tests (antibodies IgG and IgM against Epstein Barr virus, Cytomegalovirus, Hepatitis virus B and C, HIV, Toxoplasma gondii) came out to be negative. Cerebral high-resolution magnetic resonance imaging (HR-MRI) revealed abnormal MRI findings (compatible with inflammatory signals), in the vestibule, semicircular canals, cochlea, vestibular nerve and first part of the facial nerve on the left side of the head. Considering a hypothetic sine herpes vestibuloauditory deficit caused by Herpes Zoster virus, the patient was given valacyclovir and discharged with a mild improvement in general conditions but with moderate to severe left sensorineural hearing loss on high frequencies and tinnitus.

Ten days later, she described even more severe left hearing loss and mild right sensorineural hearing loss, right paracusia, bilateral aural fullness, dizziness, ataxia, bilateral photophobia, lacrimation, persistent headache and an acute right vestibular paralysis. A few days later caloric testing revealed bilateral vestibular paresis. Vestibular evocated myogenic potentials (VEMPs) were missing bilaterally. Treatment remained same as before. Viral meningitis was ruled-out by a neurological evaluation and normal electroencephalogram (EEG), and Cogan’s syndrome was suspected. Audiometry showed mild to severe right sensorineural hearing loss at high frequencies while cerebral HR-MRI was normal. Ophthalmologic evaluation for the 4th time showed the presence of a bilateral IK, an element crucial for diagnosing Cogan’s syndrome. 

Laboratory tests and cerebro-spinal fluid (CSF) analysis came out to be negative for an acute viral infection (Herpes Simplex 1 and 2, Herpes Zoster), syphilis, Lyme disease, and for auto-antibodies (ANA, AMA, ENA, APCA, ANDNA, ASMA, ACARD, ANCA, LAC). Chest X-rays, echocardiogram, abdomen echography were all normal. 

She was continued on systemic corticosteroid treatment and topical corticosteroids for the inflammatory eye disorder. Considering all the manifestations, examinations, treatments, a diagnosis of Cogan’s syndrome was made. She was then given a therapeutic protocol based on methotrexate, cyclophosphamide, cyclosporine, folin, acetylsalicylic acid, and a low dose of corticosteroids considering iatrogenic Cushing’s syndrome, for a minimum of first 6 months of treatment, for achieving improvement or stabilization of the disease. 

She was also given an ORL follow-up with audiometric tests after 1 month of treatment and thereafter about every 2 months. After 1 month, the patient reported mild right sensorineural hearing loss limited to high frequencies, severe to profound left hearing loss, oscillopsia and ataxia. After 4 months’ treatment, she presented unchanged left hearing loss, improvement of right hearing function, reduction of oscillopsia and ataxia, persistent bilateral vestibular paresis and disappearance of ophthalmologic symptoms.

SOURCE- Migliori G, Battisti E, Pari M, Vitelli N, Cingolani C. A shifty diagnosis: Cogans syndrome. A case report and review of the literature. Acta Otorhinolaryngol Ital. 2009;29(2):108-113.