Neurofibromatosis type 2-related schwannomatosis (NF2-SWN) is a rare genetic tumor syndrome causing progressive neurological morbidity. This systematic review evaluates current evidence on the effectiveness, safety, and patient-reported outcomes of these treatments, primarily in vestibular schwannomas.
Audiologists managing patients with NF2-related schwannomatosis should be aware that targeted therapies (e.g., bevacizumab, MEK inhibitors) may stabilize or improve hearing in some patients, but evidence remains heterogeneous; coordinate with neuro-oncology for individualized management.
Targeted therapies represent a growing alternative to surgery or radiosurgery for NF2-related vestibular schwannomas, with direct implications for hearing preservation strategies in a population audiologists may encounter.
- 01Systematic review evaluates targeted drug therapies for NF2-related schwannomatosis with vestibular schwannomas.
- 02NF2-related schwannomatosis is a rare genetic condition causing progressive tumors on hearing and balance nerves.
- 03Targeted therapies (e.g., anti-VEGF agents, MEK inhibitors) show variable tumor control and hearing outcomes.
- 04Evidence base is heterogeneous, limiting definitive clinical recommendations.
- 05Published in Neuro-Oncology Practice.
Targeted therapies are effective in treating vestibular schwannomas in patients with NF2-related schwannomatosis.
studypartially supported- PMID
- 42453176
- DOI
- 10.1093/nop/npag017.
- Journal
- Neuro-Oncology Practice
- Publication type
- meta_analysis
- Evidence level
- 1a
- Population
- Patients with neurofibromatosis type 2-related schwannomatosis and vestibular schwannomas
- Intervention
- Targeted drug therapies (e.g., bevacizumab, MEK inhibitors) for NF2-related schwannomatosis
Primary outcomes
Tumor response (volume change) to targeted therapy; Hearing preservation or improvement outcomes; Treatment tolerability and adverse effects