Cochlear implantation in a patient with neurofibromatosis type 1 and profound hearing loss: evidence to support a cochlear site of lesion.

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.

Study Design: Postoperative assessment of an adult male with neurofibromatosis type 1.

Setting: The study was completed in the Departments of Otolaryngology and Audiology, University of Massachusetts Medical Center, Massachusetts.

Patients: One adult male with neurofibromatosis type 1 and bilateral, chiefly profound, sensorineural hearing loss.

Interventions: The subject underwent cochlear implantation as treatment of bilateral, chiefly profound sensorineural hearing loss.

Main Outcome Measures: Outcome measures include results of electrical auditory brainstem response testing, electrical compound action potential measures, and speech recognition scores.

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.

Conclusions: This is the first case report describing the outcome of cochlear implantation in a patient with neurofibromatosis type 1. 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. 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 with neurofibromatosis type 1 and normal, VIIIth nerve radiographic findings.