OBJECTIVE: This study will demonstrate an individualized approach to hearing preservation and tumor management in pediatric patients with neurofibromatosis type 2 (NF2). The unique nature of each case discussed will provide valuable guiding principles for the treating surgeon. PATIENTS: Pediatric patients under the care of the senior authors presenting with bilateral vestibular schwannoma and NF2. INTERVENTIONS: Interventions used for hearing optimization, in conjunction with surgical interventions, include use of hearing aids, cochlear implants, auditory brainstem implants, radiation therapy, and chemotherapeutic agents such as bevacizumab. RESULTS: Pediatric patients with NF2 present a unique and difficult challenge to the neurotologist. These children are still developing physically, mentally, and socially. They generally have a poorer prognosis and diminished life expectancy. Considerations of utmost importance include management of the tumor with the goal of minimizing outcomes such as significant bilateral hearing loss, facial paralysis, and injury to other cranial nerves, which would be detrimental to the quality of life. These patients are best served by a team of physicians who can provide individualized patient care. The team can proactively develop the treatment strategy before initial tumor resection. MAIN OUTCOME MEASURES: Hearing results, tumor control. CONCLUSION: Each patient with NF2 is unique; methods to preserve hearing must be individually tailored to preserve optimal hearing and avoid disruption of the development of the pediatric patient.
OBJECTIVE: This study will demonstrate an individualized approach to hearing preservation and tumor management in pediatric patients with neurofibromatosis type 2 (NF2). The unique nature of each case discussed will provide valuable guiding principles for the treating surgeon. PATIENTS: Pediatric patients under the care of the senior authors presenting with bilateral vestibular schwannoma and NF2. INTERVENTIONS: Interventions used for hearing optimization, in conjunction with surgical interventions, include use of hearing aids, cochlear implants, auditory brainstem implants, radiation therapy, and chemotherapeutic agents such as bevacizumab. RESULTS: Pediatric patients with NF2 present a unique and difficult challenge to the neurotologist. These children are still developing physically, mentally, and socially. They generally have a poorer prognosis and diminished life expectancy. Considerations of utmost importance include management of the tumor with the goal of minimizing outcomes such as significant bilateral hearing loss, facial paralysis, and injury to other cranial nerves, which would be detrimental to the quality of life. These patients are best served by a team of physicians who can provide individualized patient care. The team can proactively develop the treatment strategy before initial tumor resection. MAIN OUTCOME MEASURES: Hearing results, tumor control. CONCLUSION: Each patient with NF2 is unique; methods to preserve hearing must be individually tailored to preserve optimal hearing and avoid disruption of the development of the pediatric patient.
Authors: Katrina A Morris; John F Golding; Patrick R Axon; Shazia Afridi; Claire Blesing; Rosalie E Ferner; Dorothy Halliday; Raj Jena; Pieter M Pretorius; D Gareth Evans; Martin G McCabe; Allyson Parry Journal: Neurooncol Pract Date: 2016-01-07