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PostScript Funding: None. Competing interests: None. Ethics approval: Ethics approval was obtained. Accepted 19 May 2007 Br J Ophthalmol 2008;92:423–425. doi:10.1136/bjo.2007.121475
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Sensorineural hearing loss in a patient with HLA-B27 sclerouveitis Audiovestibular disease exists in conjunction with several autoimmune disorders. Ankylosing spondylitis, Cogan disease, rheumatoid arthritis, systemic lupus erythematosus, Sjo ¨gren syndrome, Wegener granulomatosis, ulcerative colitis, relapsing polychondritis, Behc¸et disease and scleroderma are associated with autoimmune inner ear disease. We present a patient with HLA-B27-associated sclerouveitis and sensorineural hearing loss, and postulate an association between atypical Cogan syndrome and HLA-B27 autoimmunity.
CASE HISTORY A 52-year-old HLA-B27 positive Caucasian woman with a 3-year history of intermittent sclerouveitis, presented with a 2-month history of bilateral redness and discomfort. She reported a concurrent 3-month history of left-sided hearing deficit and tinnitus. The corticosteroids prescribed for her ocular symptoms also improved her auditory deficit. Past medical history was significant for vitiligo and Raynaud phenomenon. Family history was non-contributory. Review of systems revealed a prodrome of fever, chills, myalgia, arthralgia and night sweats. Intraocular inflammation was quiescent. An audiogram demonstrated a leftsided sensorineural deficit. Brainstem auditory evoked potentials were also normal. Br J Ophthalmol March 2008 Vol 92 No 3
Given that she responded to oral prednisone, and her disease process was in remission, we commenced methotrexate 10 mg weekly. The patient responded well to the immunomodulatory therapy with a further improvement in her auditory deficit.
DISCUSSION In 1945, David Cogan described an association between non-syphilitic interstitial keratitis and vertigo, tinnitus, and deafness. Classically, typical Cogan disease is described as interstitial keratitis associated with audiovestibular impairment similar to Me´nie`re disease, with progressive hearing loss within two years.1 In 1980, Haynes and colleagues described atypical Cogan disease.2 This syndrome includes forms of ocular inflammation other than interstitial keratitis. Many patients with Cogan disease experience constitutional symptoms and systemic organ involvement, the most important of which involves the cardiovascular system. During the course of Cogan disease, aortic insufficiency can present in 15% of the patients, with the possible development of aortitis and aortic insufficiency. In our patient, the use of corticosteroids was instrumental in the prevention of deafness. This phenomenon is well supported by the literature: systemic corticosteroids improve ocular, vascular, visceral and audiovestibular symptoms, if therapy is promptly instituted.2 While there are several gene associations with Cogan disease (HLA-B17, A19, B14, Bw35 and Cw4), the underlying aetiology remains unclear, but it is hypothesised to be autoimmune or vasculitic in nature. Interestingly, however, 30%1 to 40%2 of patients report upper-respiratory-tract infection in the weeks preceding presentation. Ankylosing spondylitis has additionally been associated with sensorineural hearing loss. In one study, Wang and colleagues, report an incidence of more than 50% of patients with ankylosing spondylitis who suffered hearing loss, which was most striking at the high frequency range.3 It is hypothesised that the underlying mechanism involved is autoimmunity. While such a high association has not been reported elsewhere, Alatas and Yazgan demonstrated that ankylosing spondylitis was associated with sensorineural hearing loss in 28.6% of patients and in only 4.35% of controls.4 This study concluded that ankylosing spondylitis primarily affects the inner ear. Associations between ankylosing spondylitis, as well as other autoimmune diseases (systemic lupus eryththematosus, Sjo ¨gren syndrome, inflammatory bowel disease, Wegener granulomatosis, rheumatoid arthritis and scleroderma) and autoimmune inner ear disease have additionally been identified. The molecular basis of autoimmune sensorineural hearing loss is multifaceted and has been postulated to include several autoantibodies, some which may even be in response to chronic infection. Antibodies to heat-shock protein
70 (a 68 kDa inner ear antigen), previously related to ulcerative colitis and Lyme disease, may additionally play a causative role in autoimmune sensorineural hearing loss.5 Lunardi and colleagues screened random peptides from pooled IgG immunoglobulins derived from eight patients with Cogan disease. One protein in particular, an immunodominant peptide, demonstrated similarity with autoantigens such as SSA/Ro and with Reovirus III major core protein, lambda 1. The peptide shows similarity with a celldensity-enhanced protein tyrosine phosphatase-1 [DEP-1/CD148] which is expressed on the sensory epithelia of the inner ear and on the endothelial cells.6 We believe that the sensorineural hearing loss in our patient is most likely autoimmune in origin. The association between infection and autoimmunity is well established. Whether infection triggers a genetic predisposition to an immunological reaction resulting in the manifestations of Cogan syndrome, as it does in HLA-B27 associated disease, remains to be proven. The association between HLA-B27 and Cogan syndrome has profound clinical implications. Untreated autoimmune disease results in significant morbidity, if not mortality. Physicians must be alerted to the possibility of coexisting significant small-vessel autoimmune disease which may affect the cerebral, gastrointestinal, myocardial or renal vasculatures. Such vessel involvement requires prompt immunomodulation, which may also prevent permanent hearing loss. V Rao,1 M Gallagher,2 P Bhat,2 C S Foster2 1 Michigan State University College of Human Medicine, East Lansing, MI, USA; 2 Massachusetts Eye Research and Surgery Institute, Cambridge, MA, USA
Correspondence to: C S Foster, Massachusetts Eye Research and Surgery Institute, 5 Cambridge Center, 8th Floor, Cambridge, MA 02142, USA;
[email protected] Competing interests: None. Accepted 19 May 2007 Published Online First 30 May 2007 Br J Ophthalmol 2008;92:425. doi:10.1136/bjo.2007.122077
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Miserocchi E, Baltatzis S, Foster CS. A case of atypical Cogan’s syndrome with uncommon corneal findings. Cornea 2001;20:540–1. Vinceneux P. Cogan’s syndrome. Orphanet Encyclopedia. February 2005. http://www.orpha.net/ data/patho/Pro/en/Cogan-FRenPro3333.pdf (accessed 26 Apr 2007). Wang G, Liu Y, Bao C, et al. Relationships between ankylosing spondylitis and ear disease. Zhonghua Er Bi Yan Hou Ke Za Zhi 2000;35:117–9. Alatas N, Yazgan P. Audiological findings in patients with ankylosing spondylitis. J Laryngol Otol 2005;119:534–9. Garcia Berrocal JR, Ramirez-Camacho R, Arellano B, et al. Validity of the western blot immunoassay for heat shock protein-70 in associated and isolated immunorelated inner ear disease. The Laryngoscope 2002;112:304–9. Lunardi C, Bason C, Leandri M, et al. Autoantibodies to inner ear and endothelial antigens in Cogan’s syndrome. Lancet 2002:360:915–21.
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Sensorineural hearing loss in a patient with HLA-B27 sclero-uveitis V Rao, M Gallagher, P Bhat and C S Foster Br J Ophthalmol 2008 92: 425 originally published online May 30, 2007
doi: 10.1136/bjo.2007.122077 Updated information and services can be found at: http://bjo.bmj.com/content/92/3/425
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