Abstract

There is a common misconception that improvement in sudden sensorineural hearing loss (SSNHL) after treatment with steroid therapy effectively excludes the diagnosis of a vestibular schwannoma (VS) and such cases do not warrant an MRI. Paralleling this, steroids are commonly withheld for SSNHL in patients with an existing diagnosis of VS, believing that this condition is not steroid-responsive. This study seeks to underscore that improvement or recovery of SSNHL with steroid therapy does not exclude the diagnosis of VS and does not preclude the need for magnetic resonance imaging. A retrospective chart review was performed (2002-2017) of patients with previously untreated sporadic VS who developed SSNHL that improved after steroid treatment. A clinically significant audiometric improvement was defined as an increase of more than or equal to 15% in word recognition score (WRS) and/or decrease of more than or equal to 15 dB in 4-frequency pure-tone average (PTA). To supplement these data, a separate population of patients with incomplete or missing audiometric data, who reported unequivocal subjective improvement in hearing after steroid treatment, were also described to reinforce the study objective. Patient demographics, tumor characteristics, steroid regimen, and data regarding treatment response were recorded. A total of 29 patients (55% women; median age of 47 yr) met inclusion criteria. Fourteen (48%) cases had objective audiometric documentation of SSNHL, while 15 (52%) had either subjective report only or incomplete audiometric data available. Eighteen (62%) had a single event, while 11 (38%) had more than one episode of SSNHL that was treated with steroids. For all patients, the median time between SSNHL and diagnosis of VS was 1.3 months (range, 0.13-148.4 mo). At the time of diagnosis, 15 tumors were purely intracanalicular, while 15 tumors had cerebellopontine angle extension. Of the latter, the median cisternal tumor size was 15.9 mm (range, 5.3-33). Twenty-six (90%) cases received oral steroid therapy alone, two (9%) had intratympanic steroid therapy alone, and one (3%) required combination therapy. The median PTA improvement with steroid therapy was 21 dB HL (range, -10-101.2) and the median WRS improvement was 40% (range, 4-100%). A therapeutic response to steroid therapy for SSNHL does not exclude the diagnosis of VS. All patients with SSNHL should undergo appropriate diagnostic imaging to prevent delays in diagnosis and potential treatment.

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