Options and strategies for hearing restoration in pediatric neurofibromatosis type 2.
Publication/Presentation Date
10-1-2020
Abstract
PURPOSE: In this article, we will review the mechanisms and natural history of hearing loss in neurofibromatosis type 2 (NF2) and discuss the hearing outcomes with different rehabilitation options.
METHODS: Review of the published literature.
RESULTS: NF2 is a rare autosomal dominant syndrome characterized by vestibular schwannomas and other intracranial and spinal tumors. Bilateral vestibular schwannomas are the hallmark of the disease which occur in 90 to 95% of the patients. As a result, hearing loss will eventually occur in almost all NF2 patients. Deafness can occur from tumor progression or from treatment of vestibular schwannomas and is among the most debilitating aspects of NF2. A number of surgical and non-surgical rehabilitation options are available for these patients including cochlear and auditory brainstem implants. The audiologic outcomes with surgical rehabilitation options have been variable but most patients are able to achieve sound awareness and benefit from auditory cues in lip reading.
CONCLUSION: Early identification and treatment of NF2 patients can help in achieving better hearing outcomes in the pediatric population. An increasing number of NF2 patients are receiving open set word understanding with refinement in surgical techniques.
Volume
36
Issue
10
First Page
2481
Last Page
2487
ISSN
1433-0350
Published In/Presented At
Mahboubi, H., Slattery, W. H., 3rd, Mehta, G. U., & Lekovic, G. P. (2020). Options and strategies for hearing restoration in pediatric neurofibromatosis type 2. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 36(10), 2481–2487. https://doi.org/10.1007/s00381-020-04721-4
Disciplines
Medicine and Health Sciences
PubMedID
32564156
Department(s)
Department of Surgery Faculty
Document Type
Article