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Mechanisms of Hearing Loss in Neurofibromatosis Type 2
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3458837/
Neurofibromatosis type 2 (NF2) is an autosomal dominant heritable neoplasia syndrome. It results from a germline mutation of the NF2 tumor suppressor gene located on the long arm of chromosome 22 . NF2 has an incidence of 1 in 25,000 persons and a penetrance of nearly 100% by age 60 years , .
Neurofibromatosis 2 | USC Acoustic Neuroma Center
https://acousticneuroma.keckmedicine.org/educating-patients/neurofibromatosis-2/
Neurofibromatosis type 2 (NF2) is a genetic disorder that causes largely non-cancerous (benign) tumors of the nervous system including the brain, spinal cord and nerves. It is estimated that approximately 1 in every 25,000 people worldwide is affected by this disorder regardless of sex, race or ethnic background.
Neurofibromatosis Type 2 (NF2) | Johns Hopkins Medicine
https://www.hopkinsmedicine.org/health/conditions-and-diseases/neurofibromatosis/neurofibromatosis-type-2
Neurofibromatosis type 2, or NF2, is a condition that causes tumors to arise on nerves, particularly those in the skull and spine, though other nerves can be affected. Vestibular schwannomas (also called acoustic neuromas) are tumors that occur in most people with NF2; they affect the nerves that help with balance and hearing.
NF Type 2 | Texas Neurofibromatosis Foundation
https://www.texasnf.org/nf-type-2
NEUROFIBROMATOSIS - TYPE 2. NF2 is less common than NF1. NF2 was formerly called central neurofibromatosis. Patients with NF2 may have only a few café au lait spots, light patches of skin and skin tumors. There will be tumors that affect hearing and balance called vestibular schwannoma, or acoustic neuroma.
Auditory brainstem implants in NF2 patients: results and ...
https://pubmed.ncbi.nlm.nih.gov/22246383/
Objective: Neurofibromatosis Type 2 (NF2) patients have multiple central nervous system tumors and, specifically, bilateral vestibular schwannomas (VSs) causing bilateral deafness. If the cochlear nerve is not preserved during tumor removal, the only hearing rehabilitation in these patients could be via an auditory brainstem implant (ABI).
Hearing and facial function outcomes for …
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3908336/
Objectives: Vestibular schwannomas are the hallmark of neurofibromatosis 2 (NF2), occurring in >95% of patients. These tumors develop on the vestibulocochlear nerve and are associated with significant morbidity due to hearing loss, tinnitus, imbalance, facial weakness, and risk of early mortality from brainstem compression.
Neurofibromatosis type 2 | Genetic and Rare Diseases ...
https://rarediseases.info.nih.gov/diseases/7193/neurofibromatosis-type-2
NF2 | Children's Tumor Foundation
https://www.ctf.org/understanding-nf/nf2
There are three types of NF: NF1, NF2, and schwannomatosis . Neurofibromatosis type 2 (NF2) is much less common than NF1, affecting about 1 in 25,000 people worldwide. The disorder is characterized by the development of benign tumors, called vestibular schwannomas (formerly called acoustic neuromas), on the nerve that carries sound and balance information from the …
Hearing loss in neurofibromatosis type 1: report of two …
https://pubmed.ncbi.nlm.nih.gov/10442137/
We report two cases of sixteen year old female patients with NF1 and hearing loss. One patient had only cafe-au-lait spots, while the other patient had severe manifestations of NF1 in form of kyphoscoliosis, requiring multiple laminectomies, vertiginous …
Surveillance for Neurofibromatosis | NYU Langone Health
https://nyulangone.org/conditions/neurofibromatosis/treatments/surveillance-for-neurofibromatosis
Surveillance of Neurofibromatosis Type 2 For people with neurofibromatosis type 2, NYU Langone doctors often recommend audiograms, or hearing tests, typically every six months to a year. These tests can help physicians determine whether a vestibular schwannoma, or tumor that develops on the nerves leading from the ears to the brain, has developed.
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