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