A unilateral middle ear mass as an initial presentation of a probable IgG4-related disease: Case presentation

Over the past few years, multiple studies have shed light on the novel autoimmune inflammatory condition termed IgG4-related disorder (IgG4-RD). It predominantly affects middle-aged and older men, has an insidious presentation, and can affect multiple organs in the body. The presentation of this disease is highly variable, and many of its clinical manifestations have been described as "idiopathic". The exact pathogenesis of this disease is not fully understood; however, it is important to consider this diagnosis when encountering patients, as initiating management early in the course of the disease can prevent it from progressing to a late, irreversible stage. In this report, we present a case of a 70-year-old male who presented with a history of left aural fullness, vertigo pulsatile tinnitus, and progressive hearing loss. Radiological investigations revealed a soft tissue lesion in the middle ear cleft. The patient underwent exploration of the middle ear under general anesthesia; intraoperatively, a friable hemorrhagic lesion encapsulating the facial nerve and eroding the ossicles, the tegmen, and the labyrinth was found, and a complete excision was achieved. Histopathological evaluation of the lesion suggested a probable diagnosis of IgG4-RD. Early recognition of IgG4-RD is essential to provide the optimum patient care and prevent further progression of the disease. This report discusses an atypical presentation of IgG4-RD of the left temporal bone.