Author(s)
Melissa Castillo-Bustamante
Prithwijit Roychowdhury
Dhrumi Gandhi
Elliott D. Kozin, MD
Aaron K. Remenschneider, MD, MPH
Affiliation(s)
Massachusetts Eye and Ear Infirmary- Harvard University; Department of Otolaryngology, UMass Memorial Medical Center; Eaton Peabody Laboratories; Department of Otolaryngology- Head and Neck Surgery, Harvard medical School; UMASS Memorial Medical Center;
Abstract:
Introduction: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects several organs in the body. Otologic symptoms have previously been described, but their etiology are unknown. Herein we describe an otopathology case of a young woman diagnosed with severe SLE.
Methods: Qualitative middle ear and quantitative assessment of cochlear neurons (SGN), hair cells (HC) and stria vascularis (SV) were performed.
Results: Review of the national temporal bone registry revealed 15 cases of patients with SLE. A 13-year-old female presented with fever, bitemporal headache, oral-genital ulcerations and found to have a positive ANA titer. She developed severe vertigo prior to a rapid health deterioration over the course of following 19 months. No vestibular or audiologic assessment was available. At age 15 she died of a cerebrovascular accident secondary to large vessel vasculopathy, confirmed radiographically. Her temporal bones were harvested at 2 hours post-mortem.
The right ear showed degenerative fibrillar changes with osteocytes in the cochlea, posterior semicircular canal and vestibule. Total occlusion of the lumen of the internal carotid artery as well as vasa nervorum of the facial nerve was present. Severe HC loss and moderate SV atrophy in the basal turn with wall thickening of the basal, middle, and apical turns due to occlusion of the vessels at the middle turn. The SGN number was decreased (48%) compared to controls.
The left ear was notable for inflammatory changes in the middle ear as well as well-organized granulomas in the facial neve. Similar HC, SV, and SGN findings as the right ear were observed.
Conclusions: Cochleovestibular dysfunction in SLE is likely due to SV and HC degeneration secondary to small vessel vasculitis.