To report the first Chinese case of complex mosaic neurofibromatosis type 2 (NF2)-recently reclassified as NF2-related schwannomatosis-in a patient presenting with concurrent severe visual impairment and an intracochlear schwannoma, and to evaluate the clinical efficacy and strategic priority of tumor-preserving in situ cochlear implantation (CI).
✦ The floor
Discussion
Signed responses from readers of the wire.
Cochlear implantation with intracochlear schwannoma left in situ may be a viable option for select NF2 patients with limited alternatives, but this single case report is insufficient to generalize; multidisciplinary team evaluation remains essential.
This case expands awareness of cochlear implantation as a rehabilitation option in complex NF2 patients, particularly those with concurrent severe visual impairment who face greater communication disability.
- 01First reported Chinese case of cochlear implantation with intracochlear schwannoma (tumor) left in place.
- 02Patient had mosaic NF2 (a milder genetic variant) and severe visual impairment, limiting other sensory options.
- 03Leaving the schwannoma in situ avoids additional surgical risk but raises questions about long-term tumor behavior.
- 04Includes a literature review of similar global cases to contextualize outcomes.
- 05Highlights the importance of individualized decision-making in complex NF2 cases.
Cochlear implantation with intracochlear schwannoma left in situ is feasible in mosaic NF2 patients.
studypartially supportedThis is the first reported such case in a Chinese patient.
studyunclear- PMID
- 42996911
- DOI
- 10.26599/JOTO.2026.9540075.
- Journal
- Journal of Otology
- Publication type
- case_report
- Evidence level
- 4
- Sample size
- 1
- Population
- Single patient with mosaic NF2, intracochlear schwannoma, and severe visual impairment
- Intervention
- Cochlear implantation with intracochlear schwannoma left in situ
Primary outcomes
Surgical feasibility; Post-implantation hearing and speech outcomes; Tumor behavior post-implantation