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Bilateral facial nerve schwannomas.
J E Fenton1, M M Morrin, M Smail
1Department of Otology, Clinique Bachaumont, Paris, France.
Summary
Bilateral facial nerve schwannomas are rare tumors. This case suggests these tumors may be a variant of neurofibromatosis 2, warranting inclusion in diagnostic criteria.
Area of Science:
- Neuro-oncology
- Genetics
- Otolaryngology
Background:
- Facial nerve schwannomas are uncommon, with bilateral involvement being exceptionally rare.
- This report details a unique case of bilateral facial nerve neuromas affecting critical intra-canalicular and labyrinthine segments.
Observation:
- Radiologic imaging confirmed the origin of the bilateral tumors.
- Progressive tumor growth led to involvement of the only functioning cochlea, resulting in complete hearing loss.
- Auditory rehabilitation via cochlear implantation yielded unsatisfactory outcomes.
Findings:
- Genetic analysis revealed a mutation in the NF2 gene.
- The patient's presentation is consistent with a variant of neurofibromatosis 2.
Implications:
- Bilateral facial neuromas should be considered for inclusion in the clinical diagnostic criteria for neurofibromatosis 2.
- This case expands the understanding of NF2-related manifestations and the challenges in managing bilateral facial nerve tumors.