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

  • Otology
  • Published:
European Archives of Oto-Rhino-Laryngology Aims and scope Submit manuscript

Abstract

Purpose

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

Methods

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.

Results

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.

Conclusion

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.

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Acknowledgements

We thank Colin Woodham for language editing.

Funding

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).

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Author notes

  1. Haoyue Tan, Huan Jia and Yun Li contributed equally to this work.

Authors and Affiliations

  1. Department of Otolaryngology Head and Neck Surgery, Shanghai Ninth People’s Hospital, School of Medicine, Shanghai Jiaotong University, No 639 Zhizaoju Road, Shanghai, 200011, China

    Haoyue Tan, Huan Jia, Yun Li, Zhihua Zhang, Weidong Zhu, Yun Cai, Zhaoyan Wang & Hao WU

  2. Ear Institute, Shanghai Jiaotong University School of Medicine, Shanghai, China

    Haoyue Tan, Huan Jia, Yun Li, Zhihua Zhang, Weidong Zhu, Zhaoyan Wang & Hao WU

  3. Shanghai Key Laboratory of Translational Medicine on Ear and Nose Diseases, Shanghai, China

    Haoyue Tan, Huan Jia, Yun Li, Zhihua Zhang, Weidong Zhu, Zhaoyan Wang & Hao WU

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  1. Haoyue Tan
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  2. Huan Jia
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  4. Zhihua Zhang
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Correspondence to Huan Jia, Zhaoyan Wang or Hao WU.

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Tan, H., Jia, H., Li, Y. et al. Impact of cochlear implantation on the management strategy of patients with neurofibromatosis type 2. Eur Arch Otorhinolaryngol 275, 2667–2674 (2018). https://doi.org/10.1007/s00405-018-5127-9

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  • DOI: https://doi.org/10.1007/s00405-018-5127-9

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