ABSTRACT
<p><b>OBJECTIVE</b>To investigate the etiologies and clinical features for bilateral acute sensorineural hearing loss (bi-ASNHL).</p><p><b>METHODS</b>The clinical data of 19 cases presenting with bi-ASNHL were retrospectively analyzed, including the clinical features, systemic examinations, laboratory examinations, audiology and radiology results, as well as the prognosis.</p><p><b>RESULTS</b>There were 15 non-otologic diseases in 19 patients, accounting for 78.9% of the total cases, most of which were disorders with multisystem and multi-organ disorder. The central nervous system diseases including fungal meningitis, tuberculous meningitis, and viral encephalitis in 3 patients. The clinical features of deafness were bilateral, progressive, accompanied with fever, headache, dizziness, nausea, vomiting and change of mental status. There was a decrease in speech recognition score (SRS), and speech recognition threshold (SRT) was obviously inferior to pure tone average (PTA) disproportionally. Diseases of immune system including antineural cytoplasmic antibody (ANCA)-associated systemic vasculitis (AASV), relapsing polychondritis (RP), rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE) in 5 patients. They showed the characteristics of bilateral, progressive and simultaneous autoimmune disease. Hematological and endocrine system diseases including diabetes mellitus, leukemia, and thyroid hypofunction in 5 patients. The deafness had the characteristics of symmetry and progressivity. Otologic diseases including large vestibular aqueduct syndrome (LVAS) and sudden sensorineural hearing loss (SSNHL) in 4 patients; Drug-induced sensorineural deafness happened in 2 patients. After the treatment aimed at the causes, 1 case was cured, 3 patients were markedly effective, 7 patients were effective, and 8 patients were ineffective(including dead and refusal cases), with a total effective rate of 57.9%.</p><p><b>CONCLUSIONS</b>The most of bi-ASNHL cases are often associated with systemic diseases. Clinicians should analyze the history and clinical characteristics in detail, and complete specific laboratory examinations, audiology and imaging examinations in order to reveal the causative diseases. It should be treated aimed at the etiology.</p>