ArticleChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2016
Pediatric neurofibromatosis type 2: clinical and molecular presentation, management of vestibular schwannomas, and hearing rehabilitation.
Article in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed.
- The clinical spectrum of paediatric NF2- related schwannomatosis.Familial cancer · 2025Review
- Clinical and Genetic Overview of Neurofibromatosis Type 2 (NF2).Journal of Korean Neurosurgical Society · 2025Article
- Commentary to 'Incidence of vestibular schwannoma in Finland, 1990-2017' Pediatric vestibular schwannomas: an overlooked epidemiological aspect?Acta oncologica (Stockholm, Sweden) · 2024Article
- Review
- Audiologic Natural History of Small Volume Cochleovestibular Schwannomas in Neurofibromatosis Type 2.Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology · 2018Article
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Authors and funding
9 authors.
Funding
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Abstract
objectiveThis study aims to describe the clinical and molecular presentation of pediatric neurofibromatosis type 2 (NF2) and the subsequent management of vestibular schwannomas (VS) and hearing rehabilitation.
methodsThis is a single-center retrospective study of neurofibromatosis type 2 diagnosed before the age of 18 years old from 1997. Natural history of vestibular schwannomas and surgical outcomes were evaluated using volumetric MRI, hearing, and facial nerve assessment. Patients included in chemotherapy protocols were excluded.
resultsFrom a database of 80 patients followed up for NF2 on a regular basis, 25 patients were eligible (11 sporadic cases, 14 inherited in five families). The mean age at diagnosis was 11.6 years old. The average clinical follow-up was 6.5 years. NF2 mutation was identified in 81 % of the probands. The average growth rate based on the maximum linear diameter (DGR) was 1.68 mm/year (n = 33, average follow-up 4.22 years) and 545 mm
conclusionEarly diagnosis and treatment of small growing VS should be carefully discussed considering familial history and possible rehabilitation with a CI.
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