Human otopathology in scleroderma

Objective: Scleroderma is a complex chronic progressive immune-mediated disease that causes fibrosis of the skin and internal organs, and vasculopathy. Ear involvement has been poorly studied in patients with scleroderma. Vasculitic and autoimmune mechanisms are considered as possible etiologies on hearing impairment, however, this etiology still unclear. Herein, we reviewed three cases of scleroderma from a temporal bone repository. Methods: The national temporal bone database was reviewed for cases with scleroderma. Clinical case review and correlative otopathologic analysis. Middle and inner ear otopathologic analysis was performed following hematoxylin and eosin staining under light microscopy. Findings were compared to three age-matched controls. Results: Two patients (three cases) with a history of serologically confirmed scleroderma were identified. Both individuals reported tinnitus and demonstrated bilateral moderate to severe down-sloping sensorineural hearing loss on audiometry. Histologically, the incudomallear joint space was diminished and ossicles appeared demineralized. A loss of hyaline cartilage, and obliteration of the incudomallear and incudostapedial joint synovial spaces was observed. Decreased caliber and intimal hyperplasia of arteries adjacent to ossicles was also identified. Mild diffuse atrophy of stria vascularis in the middle and apical turns of cochlea were found. Hair cell populations were normal. Total spiral ganglion neurons were lower in cases of scleroderma (range 29%–51%) compared to age-matched controls. Conclusion: Fibrosis, inflammation, and vascular changes were observed in the middle and inner ear in patients with scleroderma. Findings have implications for understanding hearing and vestibular dysfunction in this patient population. Level of evidence: Retrospective study.