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European Archives of Oto-Rhino-Laryngology

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01-11-2018 | Otology

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

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

Published in: European Archives of Oto-Rhino-Laryngology | Issue 11/2018

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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Title: Impact of cochlear implantation on the management strategy of patients with neurofibromatosis type 2
Authors: Haoyue Tan
Huan Jia
Yun Li
Zhihua Zhang
Weidong Zhu
Yun Cai
Zhaoyan Wang
Hao WU
Publication date: 01-11-2018
Publisher: Springer Berlin Heidelberg
Published in: European Archives of Oto-Rhino-Laryngology / Issue 11/2018
Print ISSN: 0937-4477
Electronic ISSN: 1434-4726
DOI: https://doi.org/10.1007/s00405-018-5127-9

At a glance: The STEP trials

Springer Medicine

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

Abstract

Purpose

Methods

Results

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

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