Advertisement

Journal of Neuro-Oncology

, Volume 138, Issue 2, pp 417–424 | Cite as

Surgical treatment of large vestibular schwannomas in patients with neurofibromatosis type 2: outcomes on facial nerve function and hearing preservation

  • Fu ZhaoEmail author
  • Bo Wang
  • Zhijun Yang
  • Qiangyi Zhou
  • Peng Li
  • Xingchao Wang
  • Jing Zhang
  • Junting Zhang
  • Pinan LiuEmail author
Clinical Study

Abstract

Surgical treatment of vestibular schwannoma (VS) in patients with neurofibromatosis type 2 (NF2) along with functional preservation of cranial nerves is challenging. The aim of this study was to analyze the outcomes of hearing and facial nerve function in patients with NF2 who underwent large-size VS (> 2 cm) surgery. From 2006 to 2016, one hundred and forty NF2 patients were included with 149 large-size VS resections using retrosigmoid approach. Hearing function was classified according to the American Academy of Otolaryngology–Head and Neck Surgery (AAO–HNS) criteria. Preoperative and one-year postoperative facial nerve function were both assessed using the House–Brackmann (H–B) grading scale. A multivariate logistic regression was performed to identify preoperative predictors for facial function outcomes. No operative death we noted. Total tumor removal was achieved in 82.6% of the operated VSs. The anatomical integrity of the facial nerve was preserved in 67.8% of surgeries. Good facial nerve function (H–B Grades I–III) was maintained in 49.6% of patients at 12 months after surgery. Tumor size larger than 3 cm and preoperative facial weakness related with worse outcome of facial nerve function (P < 0.001; for both). Hearing preservation surgeries were attempted in 31 ears. Class B or C hearing according to the AAO–HNS criteria was maintained in 7 ears (22.5%), and measurable hearing was maintained 11 ears (35.5%). It is challenging to maintain hearing and facial nerve function in NF2 patients with large VSs. Early surgical intervention is an appropriate choice to decrease the risk of neurological functions deficit.

Keywords

Neurofibromatosis type 2 Vestibular schwannoma Hearing preservation Facial nerve Microsurgery 

Notes

Author contributions

Conception and design—FZ, PL, JZ; Provision of study materials and/or patients—BW, ZY; Collection and assembly of data—QZ, PL; Data analysis and interpretation—ZF, XW, JZ; Manuscript writing—All authors; Final approval of manuscript—All authors.

Funding

This work was supported by the National Natural Science Foundation of China (Grant Numbers: 81372715, 8150110473, 61671187), Beijing Natural Science Foundation of China (Grant Number: 7162057).

Compliance with ethical standards

Conflict of interest

The authors indicated no potential conflicts of interest.

References

  1. 1.
    Asthagiri AR, Parry DM, Butman JA et al (2009) Neurofibromatosis type 2. Lancet 373:1974–1986.  https://doi.org/10.1016/S0140-6736(09)60259-2 CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Baser ME, Friedman JM, Joe H et al (2011) Empirical development of improved diagnostic criteria for neurofibromatosis 2. Genet Med 13:576–581.  https://doi.org/10.1097/GIM.0b013e318211faa9 CrossRefPubMedGoogle Scholar
  3. 3.
    Baser ME, Kuramoto L, Joe H et al (2004) Genotype-phenotype correlations for nervous system tumors in neurofibromatosis 2: a population-based study. Am J Hum Genet 75:231–239.  https://doi.org/10.1086/422700 CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Baser ME, Makariou EV, Parry DM (2002) Predictors of vestibular schwannoma growth in patients with neurofibromatosis type 2. J Neurosurg 96:217–222.  https://doi.org/10.3171/jns.2002.96.2.0217 CrossRefPubMedGoogle Scholar
  5. 5.
    Blakeley JO, Plotkin SR (2016) Therapeutic advances for the tumors associated with neurofibromatosis type 1, type 2, and schwannomatosis. Neuro Oncol 18:624–638.  https://doi.org/10.1093/neuonc/nov200 CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Brackmann DE, Fayad JN, Slattery WH 3rd, et al. (2001) Early proactive management of vestibular schwannomas in neurofibromatosis type 2. Neurosurgery 49:274–280 (discussion 280–273)PubMedGoogle Scholar
  7. 7.
    Dewan R, Pemov A, Kim HJ et al (2015) Evidence of polyclonality in neurofibromatosis type 2-associated multilobulated vestibular schwannomas. Neuro Oncol 17:566–573.  https://doi.org/10.1093/neuonc/nou317 CrossRefPubMedGoogle Scholar
  8. 8.
    Dirks MS, Butman JA, Kim HJ et al (2012) Long-term natural history of neurofibromatosis type 2-associated intracranial tumors. J Neurosurg 117:109–117.  https://doi.org/10.3171/2012.3.JNS111649 CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Evans DG, Baser ME, O’Reilly B et al (2005) Management of the patient and family with neurofibromatosis 2: a consensus conference statement. Br J Neurosurg 19:5–12.  https://doi.org/10.1080/02688690500081206 CrossRefPubMedGoogle Scholar
  10. 10.
    Evans DG, Howard E, Giblin C et al (2010) Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service. Am J Med Genet A 152A:327–332.  https://doi.org/10.1002/ajmg.a.33139 CrossRefPubMedGoogle Scholar
  11. 11.
    Jaaskelainen J, Paetau A, Pyykko I et al (1994) Interface between the facial nerve and large acoustic neurinomas. Immunohistochemical study of the cleavage plane in NF2 and non-NF2 cases. J Neurosurg 80:541–547.  https://doi.org/10.3171/jns.1994.80.3.0541 CrossRefPubMedGoogle Scholar
  12. 12.
    Jung S, Kang SS, Kim TS et al (2000) Current surgical results of retrosigmoid approach in extralarge vestibular schwannomas. Surg Neurol 53:370–377 (discussion 377–378)CrossRefPubMedGoogle Scholar
  13. 13.
    Kanzaki J, Tos M, Sanna M et al (2003) New and modified reporting systems from the consensus meeting on systems for reporting results in vestibular schwannoma. Otol Neurotol 24:642–648 (discussion 648–649)CrossRefPubMedGoogle Scholar
  14. 14.
    MacNally SP, Rutherford SA, King AT et al (2009) Outcome from surgery for vestibular schwannomas in children. Br J Neurosurg 23:226–231.  https://doi.org/10.1080/02688690902968844 CrossRefPubMedGoogle Scholar
  15. 15.
    Mallory GW, Pollock BE, Foote RL et al (2014) Stereotactic radiosurgery for neurofibromatosis 2-associated vestibular schwannomas: toward dose optimization for tumor control and functional outcomes. Neurosurgery 74:292–300.  https://doi.org/10.1227/NEU.0000000000000264 (discussion 300–291)CrossRefPubMedGoogle Scholar
  16. 16.
    Mathieu D, Kondziolka D, Flickinger JC et al (2007) Stereotactic radiosurgery for vestibular schwannomas in patients with neurofibromatosis type 2: an analysis of tumor control, complications, and hearing preservation rates. Neurosurgery 60:460–468.  https://doi.org/10.1227/01.NEU.0000255340.26027.53 (discussion 468–470)CrossRefPubMedGoogle Scholar
  17. 17.
    Mautner VF, Baser ME, Thakkar SD et al (2002) Vestibular schwannoma growth in patients with neurofibromatosis type 2: a longitudinal study. J Neurosurg 96:223–228.  https://doi.org/10.3171/jns.2002.96.2.0223 CrossRefPubMedGoogle Scholar
  18. 18.
    Mautner VF, Lindenau M, Baser ME et al (1996) The neuroimaging and clinical spectrum of neurofibromatosis 2. Neurosurgery 38:880–885 (discussion 885–886)CrossRefPubMedGoogle Scholar
  19. 19.
    Nowak A, Dziedzic T, Czernicki T et al (2015) Strategy for the surgical treatment of vestibular schwannomas in patients with neurofibromatosis type 2. Neurol Neurochir Pol 49:295–301.  https://doi.org/10.1016/j.pjnns.2015.06.008 PubMedGoogle Scholar
  20. 20.
    Odat HA, Piccirillo E, Sequino G et al (2011) Management strategy of vestibular schwannoma in neurofibromatosis type 2. Otol Neurotol 32:1163–1170.  https://doi.org/10.1097/MAO.0b013e3182267f17 CrossRefPubMedGoogle Scholar
  21. 21.
    Parry DM, Eldridge R, Kaiser-Kupfer MI et al (1994) Neurofibromatosis 2 (NF2): clinical characteristics of 63 affected individuals and clinical evidence for heterogeneity. Am J Med Genet 52:450–461.  https://doi.org/10.1002/ajmg.1320520411 CrossRefPubMedGoogle Scholar
  22. 22.
    Peyre M, Goutagny S, Bah A et al (2013) Conservative management of bilateral vestibular schwannomas in neurofibromatosis type 2 patients: hearing and tumor growth results. Neurosurgery 72:907–913.  https://doi.org/10.1227/NEU.0b013e31828bae28 (discussion 914; quiz 914)CrossRefPubMedGoogle Scholar
  23. 23.
    Roehm PC, Mallen-St Clair J, Jethanamest D et al (2011) Auditory rehabilitation of patients with neurofibromatosis type 2 by using cochlear implants. J Neurosurg 115:827–834.  https://doi.org/10.3171/2011.5.JNS101929 CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Rouleau GA, Merel P, Lutchman M et al (1993) Alteration in a new gene encoding a putative membrane-organizing protein causes neurofibromatosis type 2. Nature 363:515–521.  https://doi.org/10.1038/363515a0 CrossRefPubMedGoogle Scholar
  25. 25.
    Samii M, Gerganov V, Samii A (2006) Improved preservation of hearing and facial nerve function in vestibular schwannoma surgery via the retrosigmoid approach in a series of 200 patients. J Neurosurg 105:527–535.  https://doi.org/10.3171/jns.2006.105.4.527 CrossRefPubMedGoogle Scholar
  26. 26.
    Samii M, Gerganov V, Samii A (2008) Microsurgery management of vestibular schwannomas in neurofibromatosis type 2: indications and results. Prog Neurol Surg 21:169–175.  https://doi.org/10.1159/000156905 PubMedGoogle Scholar
  27. 27.
    Samii M, Matthies C (1997) Management of 1000 vestibular schwannomas (acoustic neuromas): the facial nerve-preservation and restitution of function. Neurosurgery 40:684–694 (discussion 694–685)CrossRefPubMedGoogle Scholar
  28. 28.
    Samii M, Matthies C, Tatagiba M (1997) Management of vestibular schwannomas (acoustic neuromas): auditory and facial nerve function after resection of 120 vestibular schwannomas in patients with neurofibromatosis 2. Neurosurgery 40:696–705 (discussion 705–696)CrossRefPubMedGoogle Scholar
  29. 29.
    Sepehrnia A, Borghei-Razavi H (2015) Vestibular schwannoma between 1 and 3 cm: importance of the tumor size in surgical and functional outcome. Clin Neurol Neurosurg 129:21–26.  https://doi.org/10.1016/j.clineuro.2014.11.020 CrossRefPubMedGoogle Scholar
  30. 30.
    Slattery WH 3rd, Brackmann DE, Hitselberger W (1998) Hearing preservation in neurofibromatosis type 2. Am J Otol 19:638–643PubMedGoogle Scholar
  31. 31.
    Slattery WH 3rd, Fisher LM, Hitselberger W et al (2007) Hearing preservation surgery for neurofibromatosis type 2-related vestibular schwannoma in pediatric patients. J Neurosurg 106:255–260.  https://doi.org/10.3171/ped.2007.106.4.255 CrossRefPubMedGoogle Scholar
  32. 32.
    Sughrue ME, Yang I, Aranda D et al (2010) Hearing preservation rates after microsurgical resection of vestibular schwannoma. J Clin Neurosci 17:1126–1129.  https://doi.org/10.1016/j.jocn.2010.01.018 CrossRefPubMedGoogle Scholar
  33. 33.
    Sun S, Liu A (2014) Long-term follow-up studies of Gamma Knife surgery for patients with neurofibromatosis type 2. J Neurosurg 121(Suppl):143–149.  https://doi.org/10.3171/2014.8.GKS141503 PubMedGoogle Scholar
  34. 34.
    Tysome JR, Macfarlane R, Durie-Gair J et al (2012) Surgical management of vestibular schwannomas and hearing rehabilitation in neurofibromatosis type 2. Otol Neurotol 33:466–472.  https://doi.org/10.1097/MAO.0b013e318248eaaa CrossRefPubMedGoogle Scholar
  35. 35.
    Wanibuchi M, Fukushima T, Friedman AH et al (2014) Hearing preservation surgery for vestibular schwannomas via the retrosigmoid transmeatal approach: surgical tips. Neurosurg Rev 37:431–444.  https://doi.org/10.1007/s10143-014-0543-9 (discussion 444)CrossRefPubMedGoogle Scholar
  36. 36.
    Wanibuchi M, Fukushima T, McElveen JT Jr et al (2009) Hearing preservation in surgery for large vestibular schwannomas. J Neurosurg 111:845–854.  https://doi.org/10.3171/2008.12.JNS08620 CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Neural Reconstruction Department, Beijing Neurosurgical InstituteCapital Medical UniversityBeijingChina
  2. 2.Department of Neurosurgery, Beijing Tian Tan HospitalCapital Medical UniversityBeijingChina
  3. 3.Beijing Neurosurgical Institute, Beijing Tian Tan HospitalCapital Medical UniversityBeijingChina

Personalised recommendations