[Perioperative management of cochlear implantation for CHARGE syndrome].

Objective:To explore the perioperative period characteristics of paediatric cochlear implant recipients of CHARGE syndrome with complex deformities. Methods:Retrospective case series of CHARGE syndrome were included. Radiological results, intraoperative findings, surgical planning and post-operative complications were analyzed. Routine audiometric measurements, speech perception categories and speech intelligibility ratings were performed pre and post-operatively to measure auditory speech rehabilitation outcomes. Results:Five prelingual profoundly deaf children were identified, aged from 14 months to 60 months. All patients had congenital heart disease and underwent surgery before cochlear implantation. Upper airway abnormalities were detected as choanal atresia, laryngomalacia and tracheal stenosis. All ten ears showed cochlear abnormalities（Incomplete partition Ⅱ）, eight of them combined with secretory otitis media and/or middle ear deformity. All patients underwent single side surgery using standard transmastoid facial recess approach. Full insertion of the electrode was achieved in two cochleas, while partial insertion was done in three cochleas. Three ears with absent auditory nerves in MRI showed no response in the neural remote test. All patients had improved audio-speech performance with CAP scores 3.0±0.7 and 3.6±0.9, SIR scores 1.2±0.4 and 1.8±0.8, IT-MAIS scores 18.8±9.1 and 26.2±10.0, MUSS scores 2.2±2.4 and 7.2±8.3 after twelve months and twenty-four months follow up. Conclusion:Cochlear implantation in patients with CHARGE syndrome is a challenge in both its surgical and rehabilitation aspects due to multiple abnormalities. Adequate treatment planning is necessary for safe and effective surgery, including airway structures and intricate temporal bone landmarks.目的：探讨复杂畸形CHARGE综合征患儿人工耳蜗植入围手术期特点。 方法：回顾分析于空军军医大学第一附属医院耳鼻咽喉头颈外科行人工耳蜗植入手术的CHARGE综合征患儿资料，包括影像学、听力学和术中所见以及术后随访听觉言语量表评估结果。 结果：5例患儿接受手术时年龄14～60月龄。所有患儿均合并心脏畸形，在人工耳蜗植入前行心脏手术。术前及术中呼吸道评估发现患儿合并后鼻孔闭锁、气管狭窄、会厌软化等疾病。10耳均为耳蜗畸形（分隔不全2型），8耳合并分泌性中耳炎和/或听骨链发育畸形等中耳疾病，1耳合并面神经垂直段前移畸形。所有患儿接受单侧经乳突-面隐窝途径植入，3例电极部分植入耳蜗；MRI蜗神经显示不良的3耳植入后未检测到神经电反应。所有患儿听觉言语能力评估结果术后12、24个月CAP平均值分别为3.0±0.7和3.6±0.9，SIR平均值分别为1.2±0.4和1.8±0.8，IT-MAIS平均值分别为18.8±9.1和26.2±10.0，MUSS平均值分别为2.2±2.4和7.2±8.3，随时间增加康复效果均有不同程度改善。 结论：CHARGE综合征患儿均存在多器官发育畸形，因极重度聋接受人工耳蜗植入术前多学科全面评估有助于安全有效的手术，尤其需要关注呼吸道结构和颞骨精细解剖标志。.