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. It is the most common systemic arteritis in European and American populations, with a prevalence of 0.2% in those aged over 50 years. 1 Aging is the biggest risk factor with individuals >50 years old predominantly affected, with peak incidence at age 70-80 years. 2 Other risk factors include Scandinavian ethnicity, female gender, family history and polymyalgia rheumatica. 3 The classic symptoms of presentation include acute to subacute onset of headache, jaw claudication, scalp tenderness and constitutional symptoms. 4 Serious complications of visual loss and extra-cranial arterial manifestations such as aortic aneurysms can also occur. 4,5 Sensorineural hearing loss (SNHL) represents an atypical presentation of GCA, with only a few documented case reports. This case illustrates a debilitating presentation of GCA and highlights GCA as an important differential in the consideration of SNHL.

Hearing loss in Takayasu's arteritis

“…In contrast to SLE, TAK could not be consid-ered as an autoimmune disorder, thus mechanism of disease leading to impaired hearing is expected to be different in TAK. Several theories have been postulated regarding the mechanism of deafness in GCA, including vascular occlusion, immune-mediated damage and viral infections, all of which leading to inflammation and damage of the posterior or terminal cochleovestibular circulation (34). In an earlier report, however, anti-endothelial cell antibodies could not be identified in biopsyproven giant cell arteritis patients (35)…”