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    Abstract OBJECTIVE To determine the efficacy of cochlear implantation in an individual with neurofibromatosis type 1 and profound hearing loss and to determine, to the extent possible in a living subject, the site of lesion of the hearing loss in an individual with neurofibromatosis type 1.

    METHODS Outcome measures include results of electrical auditory brainstem neurofibromatosis dating site testing, electrical compound action potential measures, and speech recognition scores.

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    RESULTS Results of testing revealed expected latencies of waves II, III, and V of the electrical auditory brainstem response, measurable compound action potentials across virtually the entire length of the electrode array, and high levels of open-set, auditory-only speech understanding. Composite test results support the absence of VIIIth nerve degeneration or other anomalies, indicating a cochlear site of lesion for the hearing loss in this patient.

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    Based on the results of this investigation, standard cochlear implantation should be considered a viable option and the first line of therapy for the treatment of bilateral, severe to profound sensorineural hearing loss in patients neurofibromatosis dating site neurofibromatosis type 1 and normal, VIIIth nerve radiographic findings.