Costello Mark S, Golub Justin S, Barrord John V, Pater Luke, Pensak Myles L, Samy Ravi N
Department of Otolaryngology - Head and Neck Surgery, University of Cincinnati (UC) College of Medicine, Cincinnati, OH 45267-0528, USA.
Neurosensory Disorders Center at UC Neuroscience Institute, Cincinnati, OH 45219, USA.
J Radiosurg SBRT. 2016;4(1):69-74.
Neurofibromatosis type 2 (NF-2) represents the complex issue of hearing restoration after treatment for a patient with bilateral acoustic neuromas. This scenario is difficult for skull base teams considering that all treatment options (including observation of tumors) pose a risk to the patient for further or total hearing loss. In this case of a patient with bilateral deafness, restoration options were auditory brainstem or cochlear implantation (CI). The deciding factor for CI was based on the presence of a functioning cochlear nerve and blood supply. Ultimately, treatment with radiation therapy and subsequent CI proved effective as evidenced by dramatic improvement in communication (with lip reading cues) and speech perception on 1-year audiologic testing. Radiosurgery followed by CI may represent a potential emerging option for patients with NF-2.
2型神经纤维瘤病（NF-2）代表了双侧听神经瘤患者治疗后听力恢复的复杂问题。对于颅底治疗团队来说，这种情况很棘手，因为所有治疗方案（包括观察肿瘤）都有导致患者进一步听力丧失或完全失聪的风险。对于这位双侧耳聋患者，恢复听力的选择是听觉脑干植入或人工耳蜗植入（CI）。决定是否进行人工耳蜗植入的因素是耳蜗神经功能及血供情况。最终，放射治疗及后续的人工耳蜗植入治疗被证明是有效的，1年的听力测试显示，患者在唇读提示下的沟通能力和言语感知能力有显著改善。对于NF-2患者，放射外科手术联合人工耳蜗植入可能是一种潜在的新选择。
