To report findings of a cohort of patients with new onset subacute/acute sensorineural hearing loss (SNHL) who were evaluated in a neuro-rheumatology clinic.

Subacute/acute SNHL can be infectious, autoimmune, or vascular in origin.  Optimal evaluation and management of SNHL in neurology and rheumatology practice is uncertain.

All patients evaluated for subacute/acute SNHL in an informal neuro-rheumatology clinic from Jan 2014- August 2018 were evaluated in a retrospective chart review, where data including gender, age of onset, unilateral vs bilateral onset, audiogram findings, delay to diagnosis, treatment, comorbidities, disease course and final diagnosis were collected. With serial audiograms, patients were categorized as having treatment-responsive vs treatment-refractory SNHL. SNHL etiology was classified as either idiopathic or associated with other autoimmune disease including demyelinating disease.

A total of 20 patients with SNHL were evaluated throughout the study period, 80% of which were female.  Age of onset showed a mean of 47.7±17.0 years.  SNHL onset and/or effect was bilateral in 75% of patients.  Audiograms showed moderate-severe SNHL in 45% of cases, and severe-profound SNHL in 30% of cases. Response to corticosteroids occurred in 50% of patients, including five cases which had been treated with high-dose IV methylprednisolone.  Recurrence occurred in 75% of cases. Other immune modulating agents such as cyclophosphamide, methotrexate, or rituximab were used in 65% of cases. Following evaluation, idiopathic SNHL accounted for 30% of cases. Autoimmune diseases associated with presentation of SNHL included Susac Syndrome (3), demyelinating disease (5), small vessel vasculitis (2), and antiphospholipid antibody syndrome (3).

In a neuro-rheumatology clinic, subacute/acute SNHL can be associated with diagnoses that predict SNHL recurrence and other morbidity.  Patients with SNHL should be rapidly evaluated for autoimmune disease and treated with appropriate immunotherapy to prevent disabling hearing loss or systemic involvement.