Article
Neurosurgical management in childrens and adolescents with NF2-associated vestibular schwannomas regarding growth control and hearing preservation.
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Authors
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Isabel Gugel
- Klinik für Neurochirurgie, Universitätsklinikum Tübingen, Eberhard Karls Universität Tübingen, Tübingen, Deutschland
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Victor-Felix Mautner
- Klinik für Neurologie, Universitätsklinikum Hamburg-Eppendorf, Hamburg, Deutschland
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Marcos Tatagiba
- Universitätsklinikum Tübingen, Klinik für Neurochirurgie, Tübingen, Deutschland
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Martin Schuhmann
- Universitätsklinikum Tübingen, Klinik für Neurochirurgie, Bereich Pädiatrische Neurochirurgie, Tübingen, Deutschland
| Published: | June 9, 2017 |
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Outline
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Objective: To evaluate tumor growth rate and hearing outcome after decompression of the internal auditory canal (IAC) with or without tumor resection in NF2-associated vestibular schwannomas
Methods: In this retrospective study we reviewed twenty children and adolescent (9-18 years) with Neurofibromatosis Type 2 who had undergone decompression of the IAC with or without subtotal or total tumor resection between 2004 and 2016. The minimum follow-up was 1 year. Hearing outcome was assessed using pure-tone average, speech discrimination test, acoustic evoked potentials and was classified with known classification systems. Tumor volumetry was performed before and after surgery to control tumor growth rate using the Brainlab software. Pre- and postoperative facial grade were also recorded. 7 patients received postoperative chemotherapy.
Results: In total 30 surgeries (10 patients bilaterally and 10 unilaterally) were performed under continuous electrophysiological monitoring. All cases received decompression of the IAC. Respecting functional and anatomical preservation of the facial and acoustic nerve, total tumor removal was achieved in 18,75 % and subtotal tumor removal in 65,63% of the operated tumors. Facial palsy was postoperatively observed in 3 (30) cases. Following surgery, the majority of patients showed functional hearing preservation over several years. In all patients acoustic evoked potentials deteriorated before conventional hearing test.
Conclusion: Decompression of the IAC seems to be a useful procedure for hearing maintenance in NF2 patients, with very low morbidity. Ideal timing and association with chemotherapy can stabilize or even maintain tumor growth rate and hearing over the time.
