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Published on: February 29, 2020
[Neurofibromatosis type 2 and auditory brainstem implantation]
Torbjørn Elvsåshagen1, Volker Solyga, Søren Jacob Bakke
1Avdeling for nevropsykiatri og psykosomatisk medisin, Nevroklinikken, Oslo universitetssykehus, Rikshospitalet 0027 Oslo og Institutt for psykiatri Det medisinske fakultet, Universitetet i Oslo, Norway. torbjorn.elvsashagen@rikshospitalet.no
Summary
Neurofibromatosis type 2 (NF2) is a genetic disorder causing tumors and hearing loss. Auditory brainstem implants offer significant auditory benefits for NF2 patients, improving quality of life.
Area of Science:
- Genetics
- Oncology
- Neurology
Context:
- Neurofibromatosis type 2 (NF2) is a rare, severe autosomal dominant disorder.
- Caused by mutations in a tumor suppressor gene, leading to merlin protein dysfunction.
- Clinical manifestations include neurological symptoms from schwannomas and meningiomas.
Purpose:
- To review Neurofibromatosis type 2 (NF2).
- To discuss treatment options, focusing on auditory brainstem implantation.
- To highlight the importance of a multidisciplinary approach for patient management.
Summary:
- NF2 results from loss of the tumor suppressor protein merlin, impacting cell growth.
- Bilateral vestibular schwannomas and progressive hearing loss are hallmarks.
- Management aims to conserve function and enhance quality of life.
Impact:
- Auditory brainstem implants provide substantial auditory benefits for NF2 patients.
- Multidisciplinary care in specialty centers reduces mortality and improves surgical outcomes.
- Advances in understanding molecular mechanisms offer potential for new therapies.
