Auditory rehabilitation of patients with neurofibromatosis Type 2 by using cochlear implants

Clinical article

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Object

The aim of this study was to determine whether patients with neurofibromatosis Type 2 (NF2) who have intact ipsilateral cochlear nerves can have open-set speech discrimination following cochlear implantation.

Methods

Records of 7 patients with documented NF2 were reviewed to determine speech discrimination outcomes following cochlear implantation. Outcomes were measured using consonant-nucleus-consonant words and phonemes; Hearing in Noise Test sentences in quiet; and City University of New York sentences in quiet and in noise.

Results

Preoperatively, none of the patients had open-set speech discrimination. Five of the 7 patients had previously undergone excision of ipsilateral vestibular schwannoma (VS). One of the patients who received a cochlear implant had received radiation therapy for ipsilateral VS, and another was undergoing observation for a small ipsilateral VS. Following cochlear implantation, 4 of 7 patients with NF2 had open-set speech discrimination following cochlear implantation during extended follow-up (15–120 months). Two of the 3 patients without open-set speech understanding had a prolonged period between ipsilateral VS resection and cochlear implantation (120 and 132 months), and had cochlear ossification at the time of implantation. The other patient without open-set speech understanding had good contralateral hearing at the time of cochlear implantation. Despite these findings, 6 of the 7 patients were daily users of their cochlear implants, and the seventh is an occasional user, indicating that all of the patients subjectively gained some benefit from their implants.

Conclusions

Cochlear implantation can provide long-term auditory rehabilitation, with open-set speech discrimination for patients with NF2 who have intact ipsilateral cochlear nerves. Factors that can affect implant performance include the following: 1) a prolonged time between VS resection and implantation; and 2) cochlear ossification.

Abbreviations used in this paper: ABI = auditory brainstem implant; CNC = consonant-nucleus-consonant; CNCp, CNCw = CNC phonemes and words; CROS = contralateral routing of sound devices; CUNY = City University of New York; CUNYn, CUNYq = CUNY sentences in noise and in quiet; HINT = Hearing in Noise Test; HINTn, HINTq = HINT performed in noise and in quiet; MCF = middle cranial fossa; NF2 = neurofibromatosis Type 2; RS = retrosigmoid; SDT = speech detection threshold; SNHL = sensorineural hearing loss; SRT = speech reception threshold; VS = vestibular schwannoma.

Article Information

Address correspondence to: Pamela Roehm, M.D., Ph.D., Department of Otolaryngology, New York University School of Medicine, 530 First Avenue, Suite 7Q, New York, New York 10016. email: pamela.roehm@nyumc.org.

Please include this information when citing this paper: published online July 15, 2011; DOI: 10.3171/2011.5.JNS101929.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Illustration of an ABI. The device consists of an external microphone and processor, which connects to an internalized receiver and electrode array through the scalp. The electrode paddle, shown in greater detail in the inset, is placed within the lateral recess by the cochlear nucleus. Illustration provided courtesy of CochlearTM Americas, ©2009 Cochlear Americas.

  • View in gallery

    Illustration of the cochlear implant. The device consists of an external microphone and processor, which connects to an internalized receiver and electrode array through the scalp. The linear electrode is placed within the cochlea. Illustration provided courtesy of CochlearTM Americas, ©2009 Cochlear Americas.

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