A Giant Silence - An atypical association of sensorineural hearing loss with Giant Cell Arteritis

Abstract

Giant cell arteritis (GCA) is a chronic vasculitic disorder predominantly affecting medium to large sized arteries, prevalent in the 50 plus age group. This case illustrates an atypical presentation of this disease in the form of bilateral sensorineural hearing loss (SNHL). Apart from the presence of constitutional and vertiginous symptoms, there were essentially no classical features of GCA. Differentials were broad including infection, malignancy and medication toxicity as well as brain, eye and ear syndromes such as Cogan's syndrome, all of which were eventually excluded. Her diagnosis was ultimately confirmed on positron emission tomography, which highlights the diagnostic importance of this modality. She was managed with corticosteroids then tocilizumab and is making a gradual recovery. Literature review demonstrates that SNHL is more prevalent than previously suggested in GCA, although this does not have widespread recognition. Mechanisms of SNHL in GCA include vascular occlusion, immunological mechanisms including cross reactivity with viral antigens and direct viral infection. SNHL does appear to improve with corticosteroids. This case emphasizes the importance of considering GCA as an important differential in SNHL.

Keywords: clinical aspects; disease etiology and pathogenesis; giant cell arteritis; sensorineural hearing loss; vasculitides.

FIGURE 1

A. Whole‐body positron emission tomography (PET) findings. B. PET image demonstrating increased metabolism in thoracic ascending aorta and both internal carotid arteries suggestive of large vessel vasculitis. C. PET image demonstrating increased metabolism in aortic arch, also suggestive of large vessel vasculitis