Options and strategies for hearing restoration in pediatric neurofibromatosis type 2.
Auditory brainstem implant
Cochlear implant
Deafness
Hearing loss
Neurofibromatosis type 2
Vestibular schwannoma
Journal
Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
ISSN: 1433-0350
Titre abrégé: Childs Nerv Syst
Pays: Germany
ID NLM: 8503227
Informations de publication
Date de publication:
10 2020
10 2020
Historique:
received:
22
04
2020
accepted:
02
06
2020
pubmed:
22
6
2020
medline:
22
6
2021
entrez:
22
6
2020
Statut:
ppublish
Résumé
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. Review of the published literature. 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. 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.
Identifiants
pubmed: 32564156
doi: 10.1007/s00381-020-04721-4
pii: 10.1007/s00381-020-04721-4
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
2481-2487Références
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