Impact of Surgery on Long-Term Results of Hearing in Neurofibromatosis Type-2 Associated Vestibular Schwannomas

Abstract

Hearing preservation is a major goal in the treatment of neurofibromatosis type 2 (NF2) associated vestibular schwannoma (VS), particularly in children and adolescents. In this study, we retrospectively reviewed hearing and volumetry data sets of 39 operated tumors (ears) in 23 patients under the age of 25 and in a follow-up period of 21 to 167 months. Hearing data over a compatible period on 20 other tumors, which did not receive surgery due to their less aggressive nature, were included for comparison. Surgery was carried out via a retrosigmoid approach with the brainstem auditory evoked potential (BAEP) guide. Immediately after surgery, functional hearing was maintained in 82% of ears. Average hearing scores were better in the non-surgery ears. However, the hearing scores in both groups worsened gradually with a similar dynamic during the 42-month postoperative follow-up period. No accelerated impairment of hearing was evident for the operated cases. Rather, the gap between the two hearing deterioration lines tended to close at the end of the follow-up period. Our result suggested that the BAEP-guided surgery did not cause additional hearing deterioration in the long-term and seemed to slow down hearing deterioration of those tumors that were initially more aggressive.

Minimal Effect of Bevacizumab Treatment on Residual Vestibular Schwannomas after Partial Resection in Young Neurofibromatosis Type 2 Patients

“…Due to known adverse effects and potential long-term risks of bevacizumab, postponing this therapy as long as possible is a desirable approach, especially for young NF2 patients. We, therefore, investigated the effect of bevacizumab in a combined-treatment approach of primary hearing-preserving surgery [7] and subsequent bevacizumab treatment.…”

“…Radical microsurgery with total tumor removal carries a high risk of hearing loss and facial palsy. By contrast, partial resection aiming at the decompression of the internal auditory canals in young NF2 patients provides a more conservative option with improved growth control and hearing stabilization [6,7]. However, the risk of continuing growth of the residual tumor and hearing deterioration remains.…”

Contribution of mTOR and PTEN to Radioresistance in Sporadic and NF2-Associated Vestibular Schwannomas: A Microarray and Pathway Analysis

“…Established treatment options are microsurgery [6,7], radiation treatment [8,9], or, for patients with NF2-associated VS, systemic therapy (e.g., with bevacizumab) [10,11]. Particularly in young patients with NF2, good short-to-medium-term hearing results and growth control can be achieved by electrophysiological-guided microsurgery [7,12].…”

Managing NF2-associated vestibular schwannomas in children and young adults: review of an institutional series regarding effects of surgery and bevacizumab on growth rates, tumor volume, and hearing quality

“…This review paper summarizes our institutional experience with adolescents and young adults with NF2 regarding the VS management, aiming at preservation of a functional hearing as long as possible. We related to and condense the results of three original publications on the topics of growth rate [24], effects of surgery on hearing [25], and effects of second line bevacizumab [26] into one review, to create a comprehensive overview on the topic for the reader. We discuss the results in the light of the recent literature.…”