NF2-related schwannomatosis is a rare tumor-predisposition syndrome characterized by bilateral vestibular schwannomas (VS) that frequently lead to progressive hearing loss and neurological morbidity. Multi-modality strategies-microsurgery, radiosurgery, radiotherapy, systemic therapy, observation-are employed, but optimal treatment selection remains challenging due to disease heterogeneity, evolving practice...
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Discussion
Signed responses from readers of the wire.
No actionable change for general audiology practice — management of NF2-related vestibular schwannomas is a highly specialised neurotological decision; this review informs specialist teams rather than frontline audiologists.
A synthesis of management strategies for this rare but devastating condition helps neurotology and audiology teams weigh surgery, radiation, observation, and emerging medical therapies for a patient population with very limited options.
- 01NF2-related schwannomatosis causes bilateral vestibular schwannomas and progressive hearing loss.
- 02Management options include surgical resection, stereotactic radiosurgery, watchful waiting, and emerging drug therapies.
- 03The condition is rare, making high-quality comparative evidence difficult to generate.
- 04Systematic review design consolidates scattered evidence to guide specialist decision-making.
- 05Published in European Archives of Otorhinolaryngology.
NF2-related schwannomatosis is characterised by bilateral vestibular schwannomas and progressive hearing loss.
guidelinesupported- PMID
- 42625018
- DOI
- 10.1007/s00405-026-10536-x.
- Journal
- European Archives of Otorhinolaryngology
- Publication type
- systematic_review
- Evidence level
- 1a
- Population
- Patients with NF2-related schwannomatosis and vestibular schwannomas
- Intervention
- Various management strategies (surgery, radiosurgery, observation, pharmacotherapy)
Primary outcomes
Tumour control rates; Hearing preservation outcomes; Treatment-related adverse events