Abstract
Cogan’s syndrome is a rare presumed autoimmune disorder characterized by nonsyphilitic interstitial keratitis and progressive audiovestibular symptoms. The initial report by David G. Cogan in 1945 was modified by Haynes et al in 1980 who proposed diagnostic criteria for patients with other ocular or vestibular symptoms and suggested this to be atypical Cogan’s syndrome. In a more typical presentation of Cogan’s syndrome, ocular and audiovestibular signs and symptoms usually appear alone and are bilateral. We report a case of 50-year-old woman with an atypical Cogan’s syndrome manifested by unusual relatively rapid clinical deterioration.
1
Introduction
Cogan’s syndrome is a rare autoimmune entity with fewer than 250 cases reported in the literature. It primarily affects young adults of either sex. The classic form of Cogan’s syndrome, characterized by bilateral ocular involvement, primarily nonsyphilitic interstitial keratitis (IK), and bilateral audiovestibular involvement resembling Meniere’s disease, was first recognized as a separate clinical entity by David Cogan in 1945. Most of the patients present with bilateral eye redness and photophobia or eye pain. Similar to Meniere’s syndrome, the audiovestibular manifestations of Cogan’s syndrome are abrupt onset of vertigo, instability, nausea, vomiting, and tinnitus. Often, there is associated severe and rapidly progressive bilateral hearing loss, commonly leading to deafness. The interval between the onset of ocular and audiovestibular manifestations is usually less than 2 years. In addition to ocular and audiovestibular symptoms, various systemic manifestations, including aortitis and necrotizing vasculitis, have been reported in patients with this syndrome.
In 1980, Haynes et al broadened the definition of this condition to include patients with ocular symptoms other than IK or audiovestibular symptoms different from Meniere-like episodes and proposed diagnostic criteria for atypical Cogan’s syndrome.
In atypical Cogan’s syndrome, many inflammatory ocular manifestations have been reported to be either isolated or associated with IK, including episcleritis, scleritis, retinitis, choroiditis, optical neuritis, glaucoma, papillary edema, cataracts, ocular motor palsy, exophthalmia, central retinal artery occlusion, xerophthalmia, ptosis, and tendonitis . Usually, there is a delay of more than 2 years between the onset of typical ocular and audiovestibular manifestations.
In this article, we report a case of atypical Cogan’s syndrome highlighting its unusual presentation and rapid course of clinical deterioration.
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Case report
A 51-year-old woman without any significant medical history developed acute onset of vestibular symptoms including vertigo and imbalance associated with nausea and vomiting. She was also experiencing general upper respiratory infection symptoms including sore throat, nasal obstruction, and aural fullness. After speaking to her primary care physician, a diagnosis of viral labyrinthitis was made. Meclizine (Antivert) and diazepam (Valium) alleviated her symptoms. However, a week later, the patient complained of diplopia and developed acute onset of left profound sensorineural hearing loss with tinnitus. She was evaluated in the emergency department and admitted overnight to rule out any cerebral vascular accident. All study results were negative except for magnetic resonance imaging ( Fig. 1 ), which showed prominent enhancement within the left cochlea and semicircular canals, consistent with a diagnosis of labyrinthitis. Otolaryngology consultation was obtained identifying a normal otoscopic examination. Her audiogram ( Fig. 2 ) showed complete profound left sensorineural hearing loss with no word recognition. The patient was treated with high dose of dexamethasone (80 mg) and discharged with instruction for tapering the medication for 2 weeks. She experienced recurrent vestibular symptoms as she tapered off of the steroid. Her left hearing loss persisted, resulting in subsequent consultation with our service 1 month after onset of her initial symptoms. Her audiogram did not show any change. She underwent 2 injections of intratympanic dexamethasone, and the oral steroids were reinstated. She was subsequently tapered off prednisone 4 months after the onset of her initial symptoms. During this time, the patient was also evaluated with vestibular testing and underwent physical therapy. The testing showed a left peripheral vestibular lesion (reduced left caloric response and right directional preponderance) with an ongoing vestibuloocular asymmetry.
Seven months after presentation of her initial symptoms, the patient developed acute right orbital pain with photophobia. She was evaluated by an ophthalmologist and diagnosed with right anterior scleritis. At the same time, she was referred to a rheumatologist for additional autoimmune workup. Blood test results were negative for antinuclear antibody, antinuclear cytoplasmic antibody, rheumatoid factor, rapid plasma reagin, and HLA-B27. Erythrocytes sedimentation rate was elevated at 48, and C-reactive protein was mildly elevated at 1.6. The hepatitis serology finding was negative. The chest computed tomography finding was negative for any lymphadenopathy or other lesions.
Given the patient’s clinical history of acute left labyrinthitis causing complete unilateral sensorineural hearing loss and acute right anterior scleritis with negative laboratory and imaging test results, a diagnosis of atypical Cogan’s syndrome was determined by exclusion. She was started on a maintenance prednisone dose of 30 mg once a day and methotrexate 15 mg a week. Although her ocular symptoms improved, the patient developed left ankle fasciitis when her tapered dose of prednisone was lowered to 12.5 mg a day. Therefore, cyclosporine 100 mg twice a day was added and prednisone was increased to 25 mg once a day. While on this drug regimen, she developed left auricular cellulitis secondary to Staphylococcus aureus infection. The infection responded to ampicillin and sulbactam (Unasyn IV) and amoxicillin clavulanate (Augmentin). Because of the immunosuppressive effects of cyclosporine and methotrexate, these medications were stopped. She was subsequently started on another immunosuppressive agent, mycophenolate mofetil (CellCept) 500 mg twice a day, an inosine monophosphate dehydrogenase, which she tolerated better.
The patient’s current condition of atypical Cogan’s syndrome is clinically stable on the combination of CellCept (1 g twice a day) and prednisone (5 mg once a day). She uses prednisolone acetate (Pred Forte) eye drops for occasional ocular redness and discomfort. She also takes raloxifene (Evista) and calcium with vitamin D for prevention of steroid-induced osteoporosis.
She currently uses a contralateral routing of sound hearing aid for persistent left profound sensorineural hearing loss. A bone-anchored implantable device has been offered to the patient for the anacoustic left side. Hearing in her right ear remains normal at 1 year after the onset of her symptoms. There has been no recurrent vestibular or arthritic symptom. Her rheumatologic laboratory work has remained normal. An echocardiogram was performed and showed no evidence of aortic insufficiency. Follow-up magnetic resonance imaging studies have also been within normal limits, showing absence of enhancement of the left cochlea and semicircular canals ( Fig. 3 ).
