European Archives of Oto-Rhino-Laryngology

, Volume 275, Issue 11, pp 2667–2674 | Cite as

Impact of cochlear implantation on the management strategy of patients with neurofibromatosis type 2

  • Haoyue Tan
  • Huan JiaEmail author
  • Yun Li
  • Zhihua Zhang
  • Weidong Zhu
  • Yun Cai
  • Zhaoyan WangEmail author
  • Hao WUEmail author



To investigate the outcomes of cochlear implantation in patients with neurofibromatosis type 2 (NF2), and to discuss the current management strategy for NF2 patients.


The medical records of NF2 patients who received cochlear implants (CI) at our center between 2012 and 2016 were retrospectively reviewed. Pre-operative hearing status, tumor status, treatment of tumors, and auditory outcomes post-implantation were evaluated.


Twelve patients were included in the study. Five were implanted with the tumor in situ; two of them received radiotherapy pre-implantation, and three were implanted without any previous treatment. Four patients were implanted simultaneously with tumor removal. Three patients were implanted as second-stage after failed hearing preservation surgery. The mean pure tone audiometry with the implant was 44 dB (range 25–80 dB) and the mean sentence recognition score (SRS) in a quiet environment without lip reading was 63% (range 0–97%). A poorer objective auditory outcome was identified in one patient who showed no response to electrical promontory stimulation (EPS), but the sound perception was still helpful. In total, 11 of 12 (91%) patients were daily users, and the other patient used the implant as a “sleeper” device due to its interference with contralateral hearing provided by a hearing aid.


CI is an effective option in auditory rehabilitation and should be considered primarily for NF2 patients with intact cochlear nerve. EPS might be a predictor for cochlear implant performance. Good contralateral hearing may present a barrier to daily use.


Cochlear implants Neurofibromatosis type 2 Audiologic rehabilitation Vestibular schwannoma 



We thank Colin Woodham for language editing.


This work was supported by the National Natural Science Foundation of China (Grant no. 81700900 to Weidong ZHU, Grant no. 81200742 to Huan JIA, Grant no. 81371086 and Grant no. 81670919 to Zhaoyan WANG and Grant no. 81570906 to Hao WU), the National Key Technology Research and Development Program of the Ministry of Science and Technology of China (Grant no. 2014BAI04B01 to Hao WU), Shanghai Key Laboratory of Translational Medicine on Ear and Nose Diseases (Project Number: 14DZ2260300 to Hao WU) and Natural Science Foundation of Shanghai (Grant no. 17ZR1416100 to Huan JIA).

Compliance with ethical standards

Conflict of interest

The authors have no conflict of interest.


  1. 1.
    Carlson ML, Breen JT, Driscoll CL, Link MJ, Neff BA, Gifford RH, Beatty CW (2012) Cochlear implantation in patients with neurofibromatosis type 2: variables affecting auditory performance. Otol Neurotol 33:853–862. CrossRefPubMedGoogle Scholar
  2. 2.
    Roehm PC, Mallen-St Clair J, Jethanamest D, Golfinos JG, Shapiro W, Waltzman S, Roland JT (2011) Auditory rehabilitation of patients with neurofibromatosis Type 2 by using cochlear implants. J Neurosurg 115:827–834. CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Edgerton BJ, House WF, Hitselberger W (1982) Hearing by cochlear nucleus stimulation in humans. Ann Otol Rhinol Laryngol Suppl 91:117–124PubMedGoogle Scholar
  4. 4.
    Hitselberger WE, House WF, Edgerton BJ, Whitaker S (1984) Cochlear nucleus implants. Otolaryngol Head Neck Surg 92:52–54. CrossRefPubMedGoogle Scholar
  5. 5.
    Colletti V, Shannon RV (2005) Open set speech perception with auditory brainstem implant? Laryngoscope 115:1974–1978. CrossRefGoogle Scholar
  6. 6.
    Lloyd SKW, King AT, Rutherford SA, Hammerbeck-Ward CL, Freeman SRM, Mawman DJ, O’Driscoll M, Evans DG (2017) Hearing optimisation in neurofibromatosis type 2: a systematic review. Clin Otolaryngol 42:1329–1337. CrossRefPubMedGoogle Scholar
  7. 7.
    Bouccara D, Kalamarides M, Bozorg Grayeli A, Ambert-Dahan E, Rey A, Sterkers O (2007) Auditory brainstem implant: indications and results. Ann Otolaryngol Chir Cervicofac 124:148–154. CrossRefPubMedGoogle Scholar
  8. 8.
    Hoffman RA, Kohan D, Cohen NL (1992) Cochlear implants in the management of bilateral acoustic neuromas. Am J Otol 13:525–528PubMedGoogle Scholar
  9. 9.
    North HJ, Mawman D, O’Driscoll M, Freeman SR, Rutherford SA, King AT, Hammerbeck-Ward C, Evans DG, Lloyd SK (2016) Outcomes of cochlear implantation in patients with neurofibromatosis type 2. Cochlear Implants Int 17:172–177. CrossRefPubMedGoogle Scholar
  10. 10.
    Zhang H, Chen J, Wang S, Wang L, Guo LS, Zhao XY, Ji C, Chen XQ (2005) Edit and evaluation of mandarin sentence materials for Chinese speech audiometry. Chin J Otorhinolaryngol Head Neck Surg 40:774–778. CrossRefGoogle Scholar
  11. 11.
    Lustig LR, Yeagle J, Driscoll CL, Blevins N, Francis H, Niparko JK (2006) Cochlear implantation in patients with neurofibromatosis type 2 and bilateral vestibular schwannoma. Otol Neurotol 27:512–518. CrossRefPubMedGoogle Scholar
  12. 12.
    Peng KA, Lorenz MB, Otto SR, Brackmann DE, Wilkinson EP (2018) Cochlear implantation and auditory brainstem implantation in neurofibromatosis type 2. Laryngoscope. CrossRefPubMedGoogle Scholar
  13. 13.
    Vincenti V, Pasanisi E, Guida M, Di Trapani G, Sanna M (2008) Hearing rehabilitation in neurofibromatosis type 2 patients: cochlear versus auditory brainstem implantation. Audiol Neurootol 13:273–280. CrossRefPubMedGoogle Scholar
  14. 14.
    Graham J, Lynch C, Weber B, Stollwerck L, Wei J, Brookes G (1999) The magnetless Clarion cochlear implant in a patient with neurofibromatosis 2. J Laryngol Otol 113:458–463. CrossRefPubMedGoogle Scholar
  15. 15.
    Ahsan S, Telischi F, Hodges A, Balkany T (2003) Cochlear implantation concurrent with translabyrinthine acoustic neuroma resection. Laryngoscope 113:472–474. CrossRefPubMedGoogle Scholar
  16. 16.
    Tran Ba Huy P, Kania R, Frachet B, Poncet C, Legac MS (2009) Auditory rehabilitation with cochlear implantation in patients with neurofibromatosis type 2. Acta Otolaryngol 129:971–975. CrossRefPubMedGoogle Scholar
  17. 17.
    Lloyd SK, Glynn FJ, Rutherford SA, King AT, Mawman DJ, O’Driscoll MP, Evans DG, Ramsden RT, Freeman SR (2014) Ipsilateral cochlear implantation after cochlear nerve preserving vestibular schwannoma surgery in patients with neurofibromatosis type 2. Otol Neurotol 35:43–51. CrossRefPubMedGoogle Scholar
  18. 18.
    Li Y, Wu H, Chen X, Wang W, Jia H, Xiang M, Shen M (2006) Study on the effect of internal auditory artery vasospasm on hearing and its protection topically using vasodilator. J Audiol Speech Pathol 6:436–438. CrossRefGoogle Scholar
  19. 19.
    Mukherjee P, Ramsden JD, Donnelly N, Axon P, Saeed S, Fagan P, Irving RM (2013) Cochlear implants to treat deafness caused by vestibular schwannomas. Otol Neurotol 34:1291–1298. CrossRefPubMedGoogle Scholar
  20. 20.
    UK Cochlear Implant Study Group (2004) Criteria of candidacy for unilateral cochlear implantation in postlingually deafened adults I: theory and measures of effectiveness. Ear Hear 25:310–335. CrossRefGoogle Scholar
  21. 21.
    Trotter MI, Briggs RJ (2010) Cochlear implantation in neurofibromatosis type 2 after radiation therapy. Otol Neurotol 31:216–219. CrossRefPubMedGoogle Scholar
  22. 22.
    Wagner J, Welzel T, Habermehl D, Debus J, Combs SE (2014) Radiotherapy in patients with vestibular schwannoma and neurofibromatosis type 2: clinical results and review of the literature. Tumori 100:189–194. CrossRefPubMedGoogle Scholar
  23. 23.
    Frischer JM, Gruber E, Schöffmann V, Ertl A, Höftberger R, Mallouhi A, Wolfsberger S, Arnoldner C, Eisner W, Knosp E, Kitz K, Gatterbauer B (2018) Long-term outcome after Gamma Knife radiosurgery for acoustic neuroma of all Koos grades: a single-center study. J Neurosurg 2:1–10. CrossRefGoogle Scholar
  24. 24.
    Neff BA, Wiet RM, Lasak JM, Cohen NL, Pillsbury HC, Ramsden RT, Welling DB (2007) Cochlear implantation in the neurofibromatosis type 2 patient: long-term follow-up. Laryngoscope 117:1069–1072. CrossRefPubMedGoogle Scholar
  25. 25.
    Harris F, Tysome JR, Donnelly N, Durie-Gair J, Crundwell G, Tam YC, Knight RD, Vanat ZH, Folland N, Axon P (2017) Cochlear implants in the management of hearing loss in Neurofibromatosis Type 2. Cochlear Implants Int 18:171–179. CrossRefPubMedGoogle Scholar
  26. 26.
    Lloyd SK, Evans DG (2016) Neurofibromatosis type 2 service delivery in England. Neurochirurgie. CrossRefPubMedGoogle Scholar
  27. 27.
    Aboukais R, Bonne NX, Baroncini M, Zairi F, Schapira S, Vincent C, Lejeune JP (2015) Management of multiple tumors in neurofibromatosis type 2 patients. Neurochirurgie. CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Otolaryngology Head and Neck Surgery, Shanghai Ninth People’s Hospital, School of MedicineShanghai Jiaotong UniversityShanghaiChina
  2. 2.Ear InstituteShanghai Jiaotong University School of MedicineShanghaiChina
  3. 3.Shanghai Key Laboratory of Translational Medicine on Ear and Nose DiseasesShanghaiChina

Personalised recommendations