• Lawrence R Lustig, Jennifer Yeagle, Colin L W Driscoll, Nikolas Blevins, Howard Francis, and John K Niparko.
• Department of Otolaryngology-Head and Neck Surgery, The Johns Hopkins University, Baltimore, Maryland, and Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota, USA. llustig@ohns.ucsf.edu
• Otol. Neurotol. 2006 Jun 1;27(4):512-8.

ObjectiveTo investigate the results of cochlear implantation in patients with neurofibromatosis Type 2 (NF2) and bilateral vestibular schwannoma.Study DesignRetrospective case review.SettingThree academic tertiary referral centers.PatientsSeven patients with NF2 and bilateral vestibular schwannoma who lost hearing in at least one ear after treatment of their tumor (surgery or radiation therapy).InterventionCochlear implantation after treatment of their vestibular schwannoma.Main Outcome MeasurePostimplantation audiometric scores (pure-tone average thresholds, consonant-nucleus-consonant (CNC) words/phonemes, Central Institute for the Deaf (CID) sentences, Hearing in Noise Test (HINT) quiet/noise, and Monosyllable, Trochee, Spondee (MTS) recognition/category tests), patient satisfaction, and device use patterns.ResultsThe average age at implantation was 40 years (range, 16-57 yr). Follow-up ranged from 6 to 88 months after implantation. Three patients were implanted with residual useful hearing in the contralateral ear, whereas four patients had no hearing in the contralateral ear. Hearing loss was due to surgical excision of tumor (n=5) or gamma-knife radiotherapy (n=2). Postactivation pure-tone average thresholds in the implanted ear ranged from 30 to 55 dB (average, 32.5 dB), although speech reception testing varied considerably among subjects. Despite this variability, all patients continue to use the device on a daily basis.ConclusionIn selected cases of deafness in patients with NF2 where there has been anatomic preservation of the auditory nerve after acoustic neuroma resection or radiation therapy, cochlear implantation may offer some improvement in communication skills, including the possibility of open-set speech communication in some patients. These results compare favorably to the auditory brainstem implant offering an alternative for hearing rehabilitation in patients with NF2.

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