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

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.

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.

• Authors: Sarah F Poissant, Cliff A Megerian, Dayna Hume
• Journal: Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology (Otol Neurotol) Vol. 24 Issue 5 Pg. 751-6 (Sep 2003) ISSN: 1531-7129 [Print] United States
• PMID: 14501452 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Brain Stem (physiopathology)
• Cochlear Implantation
• Cochlear Nerve (physiopathology)
• Deafness (physiopathology, rehabilitation)
• Evoked Potentials, Auditory, Brain Stem (physiology)
• Follow-Up Studies
• Humans
• Male
• Neurofibromatosis 1 (surgery)
• Postoperative Complications (etiology, physiopathology)
• Reaction Time (physiology)
• Speech Reception Threshold Test
• Treatment Outcome