A review of targeted therapies for NF2-related vestibular schwannoma: molecular pathogenesis, emerging therapeutics, and future clinical horizons

Neurofibromatosis type 2 (NF2) is a hereditary tumor syndrome driven by mutations in the NF2 gene. The mutation leads to aberrant proliferation of Schwann cells along the vestibular division of cranial nerve VIII, resulting in bilateral vestibular schwannomas (VS) that cause progressive hearing loss and neurological dysfunction....

No actionable change at this time — this is a review of emerging and investigational therapies not yet in routine clinical use for NF2-related vestibular schwannoma.

As drug-based alternatives to surgery and radiation are explored for NF2-related vestibular schwannoma, audiologists need to understand the therapeutic landscape to counsel patients on evolving management options.

1. 01Reviews molecular mechanisms by which NF2 gene mutations drive Schwann cell proliferation and vestibular schwannoma growth.
2. 02Summarises the pipeline of targeted therapies (e.g., Bevacizumab, mTOR and MEK inhibitors) currently under investigation.
3. 03Discusses future clinical directions including combination approaches and biomarker-guided treatment selection.
4. 04Published in Journal of Neuro-Oncology (2026), indicating growing interdisciplinary interest in non-surgical management.
5. 05Targeted therapies aim to preserve hearing and reduce tumour burden without the risks of surgery or radiation.

PMID

42142209

DOI

10.1007/s11060-026-05599-z.

Journal

Journal of Neuro-Oncology

Publication type

review

Evidence level

5

Population

Patients with NF2-related vestibular schwannoma (literature review, no direct patient cohort)

Intervention

Targeted molecular therapies for NF2-related vestibular schwannoma