Date of Degree


Document Type

Capstone Project

Degree Name





Carol Silverman

Subject Categories

Speech Pathology and Audiology


Neurofibromatosis Type II (NF2), Vestibular Schwannoma (VS), Cochlear implant (CI), Duration of Deafness (DoD), open-set speech recognition


The objective of the current study was to examine, in a retrospective case series, outcome in terms of word-recognition performance in patients with neurofibromatosis Type 2 (NF2) who have received a cochlear implant (CI) The primary independent variables were duration of deafness (DoD) and age at cochlear implantation. The secondary independent variables were status of hearing sensitivity in the ear contralateral to the one that received cochlear implantation and the type of tumor treatment.

The retrospective case series comprised eight patients who were implanted at New York University Medical Center, or who were receiving follow-up care there. All NF2 patients in this study had an anatomically intact auditory nerve in the ear that was implanted, and were implanted unilaterally. The results post implantation revealed that all eight patients achieved an auditory percept and were daily users of a CI. Of the 8 patients, 87.5% (7 of 8) gained openset speech-recognition ability. Additionally, 75% (6 of 8) of the patients were high performers, achieving open-set speech recognition greater than 66%. One patient was an intermediate performer (33% - 66%).

These findings were incorporated into data collected from other investigators, yielding a total of 36 patients with NF2 and an intact auditory nerve in the affected ear, and unilateral cochlear implantation. The findings based on this larger set of patients revealed that 92% were vii daily users of the implant, and 67% (24 of the 36) achieved some degree of open-set speech recognition.

In conclusion, the results show that cochlear implantation can be a promising option for individuals with NF 2 who have an anatomically intact auditory nerve. No statistically significant relation was found between DoD or age at implantation and word-recognition scores post implantation. Therefore, it is difficult to predict, based on DoD or age at implantation, how well an individual with NF2 will do with his/her CI with regard to word-recognition performance; nonetheless, the probability of an auditory percept post implantation is high. Furthermore, the probability of gaining some degree of open-set speech recognition post implantation is somewhat greater than chance (about 60%).

Keywords: Neurofibromatosis Type II (NF2), Vestibular Schwannoma (VS), Cochlear implant (CI), Duration of Deafness (DoD), open-set speech recognition