Sensorineural Hearing Loss in the Setting of Systemic Autoimmune Disease: A Temporal Bone Study

Abstract

Objective: Determine if temporal bone specimens from patients with different systemic autoimmune diseases and sensorineural hearing loss will show similar histologic findings, including endolymphatic hydrops, strial atrophy, loss of hair cells, neuronal loss, and/or evidence of vasculitis. Method: This is a histopathologic study of 34 temporal bones from 21 patients with autoimmune disorders and sensorineural hearing loss. Specimens were studied under light microscopy, and pathologic changes were graded, including degree of endolymphatic hydrops, strial atrophy, hair cell loss, neuronal loss, and presence of vasculitis. Results: Strial atrophy was the most common finding and correlated with the degree of hearing loss. Only patients with Cogan syndrome or primary autoimmune sensorineural hearing loss had endolymphatic hydrops. Cochlear hair cell loss and neuronal loss were seen in some specimens but not universally. Vasculitis was only seen in polyarteritis nodosa. Conclusion: Strial atrophy may represent the underlying etiology of sensorineural hearing loss in patients with systemic autoimmune diseases. However, the age of these patients makes presbycusis a possible confounding factor in these specimens.