Etiology of 26 Cases with Progressive Bilateral Sensorineural Hearing Loss

Abstract

Objective: To reveal the causes of rapidly progressive bilateral sensorineural hearing loss (SNHL), too fast to be age-related degeneration, and too slow to be sudden sensorineural hearing loss. Method: We retrospectively evaluated 26 patients with bilateral progressive HL who visited our hospital from 2007 to 2011. We excluded the patients with bilateral Ménière’ss disease and acute low-tone SNHL from our study. Results: We diagnosed 5 cases with immune-mediated inner ear disease (IMIED) such as vasculitis, 5 with functional HL, 4 with intracranial diseases including nonbacterial meningitis, lymphoma, and siderosis, and 1 with drug-induced HL. We could not identify causes in the remaining 11 cases. HL was one of the first symptoms in 10 of 11 cases with IMIED and intracranial diseases. Treatments including steroid administration achieved significant hearing recovery in 4 cases, including 3 with IMIED and 1 with lymphoma. Conclusion: We revealed that the bilateral progressive HL was often the first symptom of systemic or intracranial diseases. Screening for systemic and intracranial diseases is, thus, important for adequate treatment at early stage of disease.