Malignant Cerebellopontine Angle Peripheral Nerve Sheath Tumor with Divergent Mesenchymal (Cartilaginous) Differentiation Presenting with Catastrophic Hemorrhage: Case Report and Review.

Malignant peripheral nerve sheath tumors of the cerebellopontine angle are rare, especially even outside of the context of neurofibromatosis or malignant transformation of previously radiated vestibular schwannomas. This case report describes a case of a presumed vestibular schwannoma without previous radiation or history of neurofibromatosis presenting with progressive hearing loss, facial weakness, growth, and ultimately catastrophic hemorrhage requiring urgent surgery. Histopathology revealed an exceptionally rare malignant peripheral nerve sheath tumor with divergent mesenchymal (chondrosarcomatous) differentiation with few rigorously interrogated cases in the literature. In retrospect, facial weakness, growth, and early intratumoral hemorrhage were harbingers of atypical malignant pathology. We advocate for a heightened index of suspicion, shorter interval follow-up, and consideration of early surgery in such cases in hopes of preventing potentially catastrophic outcomes.

Long-Term Outcome of Cochlear Implantation in Post-meningitic Deafnes.

BACKGROUND: This study was planned (1) to evaluate long-term outcome after cochlear implantation in patients with post-meningitic deafness and (2) to compare the outcome measures with patients implanted for deafness due to other causes. METHODS: Records of 54 patients deafened as a sequel of bacterial meningitis and implanted at the largest university-based cochlear implant program in Turkey were retrospectively reviewed. Fifty-four age- and sex-matched patients with a similar interval of implant use were selected for controls. Surgical and long-term audiological outcome (in terms of categories of auditory performance-II scores) was assessed and compared. RESULTS: Twenty-seven (52%) patients had some degree of labyrinthitis ossificans and 19 of them had full electrode insertion via basal turn cochleostomy. Patients with and without labyrinthitis ossificans in the post-meningitic group had no difference in final categories of auditory performance-II score (P=.559). Median categories of auditory performance-II scores were 6 for post-meningitic group and 7 for controls, with a significant statistical difference (P < .001). Partial or full insertions did not differ in outcome (P=.938). Mean time to implantation was not correlated with the final categories of auditory performance-II score for the post-meningitic group (P=.695). CONCLUSION: Cochlear implant recipients deafened due to meningitis have a worse long-term hearing and speech performance as measured by categories of auditory performance-II than patients implanted for congenital deafness. The presence of labyrinthitis ossificans or the limited extent of electrode insertion produced overall results that were comparable with other cases.