Neurofibromatosis 2 (Neurofibromatosis Type II)

An autosomal dominant disorder characterized by a high incidence of bilateral acoustic neuromas as well as schwannomas (NEURILEMMOMA) of other cranial and peripheral nerves, and other benign intracranial tumors including meningiomas, ependymomas, spinal neurofibromas, and gliomas. The disease has been linked to mutations of the NF2 gene (GENES, NEUROFIBROMATOSIS 2) on chromosome 22 (22q12) and usually presents clinically in the first or second decade of life.
Also Known As:
Neurofibromatosis Type II; Bilateral Acoustic Neurofibromatosis; Neurofibromatosis Type 2; Neurofibromatosis II; Familial Acoustic Neuroma; Type II Neurofibromatosis; Schwannoma, Acoustic, Bilateral; Acoustic Neurinoma, Bilateral; Acoustic Schwannomas, Bilateral; Central Neurofibromatosis; Familial Acoustic Neuromas; NF2 (Neurofibromatosis 2); Neurofibromatosis, Central NF2; Neurofibromatosis, Central, NF 2; Neurofibromatosis, Type 2; Neurofibromatosis, Type II; Neurofibromatosis, central type; Acoustic Neurinomas, Bilateral; Acoustic Neurofibromatoses, Bilateral; Acoustic Neurofibromatosis, Bilateral; Acoustic Neuroma, Familial; Acoustic Neuromas, Familial; Acoustic Schwannoma, Bilateral; Bilateral Acoustic Neurinoma; Bilateral Acoustic Neurinomas; Bilateral Acoustic Neurofibromatoses; Bilateral Acoustic Schwannoma; Bilateral Acoustic Schwannomas; Central NF2 Neurofibromatoses; Central NF2 Neurofibromatosis; Central Neurofibromatoses; NF2s (Neurofibromatosis 2); Neurinoma, Bilateral Acoustic; Neurinomas, Bilateral Acoustic; Neurofibromatoses, Bilateral Acoustic; Neurofibromatoses, Central; Neurofibromatoses, Central NF2; Neurofibromatoses, Type 2; Neurofibromatoses, Type II; Neurofibromatosis IIs; Neurofibromatosis, Bilateral Acoustic; Neurofibromatosis, Central; Neuroma, Familial Acoustic; Neuromas, Familial Acoustic; Schwannoma, Bilateral Acoustic; Schwannomas, Bilateral Acoustic; Type 2 Neurofibromatoses; Type 2 Neurofibromatosis; Type II Neurofibromatoses; Neurofibromatosis, Acoustic, Bilateral; Neurofibromatosis, Central, NF2; Neuroma, Acoustic, Bilateral
Networked: 853 relevant articles (13 outcomes, 60 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Acoustic Neuroma (Acoustic Neurinoma)
2. Neoplasms (Cancer)
3. Neurofibromatosis 1 (Neurofibromatosis Type I)
4. Tuberous Sclerosis (Bourneville's Disease)
5. Deafness (Deaf Mutism)


1. Plotkin, Scott R: 33 articles (01/2022 - 01/2009)
2. Giovannini, Marco: 23 articles (01/2022 - 08/2002)
3. Evans, D Gareth: 19 articles (01/2022 - 01/2012)
4. Gutmann, David H: 17 articles (01/2022 - 09/2002)
5. Hanemann, C Oliver: 16 articles (01/2022 - 07/2003)
6. Mautner, Victor-Felix: 16 articles (01/2022 - 07/2007)
7. Kalamarides, Michel: 12 articles (01/2022 - 12/2008)
8. Morrison, Helen: 12 articles (01/2019 - 12/2002)
9. Blakeley, Jaishri O: 11 articles (01/2022 - 05/2009)
10. Brackmann, Derald E: 10 articles (01/2021 - 01/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Neurofibromatosis 2:
1. Bevacizumab (Avastin)FDA Link
2. Vascular Endothelial Growth Factor A (Vascular Endothelial Growth Factor)IBA
3. Neurofibromin 2 (Merlin)IBA
4. Proteins (Proteins, Gene)FDA Link
5. Biological ProductsIBA
6. Legius syndromeIBA
7. Angiogenic ProteinsIBA
8. SchwannomatosisIBA
9. DNA (Deoxyribonucleic Acid)IBA
10. Biomarkers (Surrogate Marker)IBA

Therapies and Procedures

1. Auditory Brain Stem Implants (Auditory Brainstem Implants)
2. Cochlear Implantation
3. Radiotherapy
12/28/2022 - "In the adjusted model, male gender (hazard ratio [HR] =1.39, 95%CI[1.27-1.54], p< .001), age at surgery (HR=0.97, 95%CI[0.97-0.97], p < .001), type 2 neurofibromatosis (HR=2.95, 95%CI[1.95-4.46], p < .001), comorbidities HR=1.39, 95%CI[1.36-1.42], p < .001), location (HR=0.8, 95%CI[0.67-0.95], p= .0111), pre-operative embolization, (HR=1.3, 95%CI[1.08-1.56], p= .00507), cerebro-spinal fluid shunt, (HR=2.48, 95%CI[2.04-3.01], p < .001), atypical (HR=1.3, 95%CI [1.09-1.54], p= .00307) or malignant histology (HR=1.86, 95%CI[1.56-2.22], p< .001), redo surgery (HR=1.19, 95%CI[1.04-1.36], p= .0122) and radiotherapy (HR=1.43, 95%CI[1.26-1.62], p < .001) were established as independent predictors of RS. "
09/01/2021 - "Additionally, results of CI after stereotactic radiotherapy in a 54-year-old male with neurofibromatosis type 2 are presented. "
01/01/2020 - "The aim of this systematic review is to assess hearing rehabilitation outcomes for CI recipients with VS, either sporadic or associated with neurofibromatosis type 2, whose tumours have been managed with either observation or radiotherapy.Methods"
09/01/2007 - "There were five neurofibromatosis type 2 patients treated, two of whom had useful hearing before radiotherapy. "
12/01/2005 - "In addition to the WHO classification, the following potential prognostic factors were analyzed: age, sex, extent of resection, history of radiotherapy, diagnosis of neurofibromatosis Type 2 (NF2) or other inherited syndromes, and the presence of a comorbidity. "
4. Aftercare (After-Treatment)
5. Therapeutics