POU3F4基因突变致内耳畸形人工耳蜗术后脑脊液漏的观察及处理

Observation and Management of Cerebrospinal Fluid Leakage after Cochlear Implant in Patients with Inner Ear Malformation Caused by POU3F4 Gene Mutation

目的探讨POU3F4基因突变导致的IP-Ⅲ型内耳畸形患者人工耳蜗植入术后脑脊液漏并发其他并发症的护理观察与处理。方法收集解放军总医院第一医学中心耳鼻咽喉头颈外科收治的8例IP-Ⅲ内耳畸形患儿。入院后行基因检测明确分子病因,经颞骨CT及颅脑核磁检查后均提示内耳结构异常,发育畸形,按Sennarolu内耳畸形分类符合IP-Ⅲ型,排除各项绝对禁忌症后在全麻下行人工耳蜗植入+编程术,术后根据患者病情变化给予相对应处置措施。结果8例患者除一例未行基因检查外,7例均在POU3F4基因上明确了致病突变。6例患者术中均发生镫井喷,1例患者术后出现脑脊液漏、肺部感染、脑膜炎等严重并发症。结论POU3F4基因突变导致的内耳畸形IP-Ⅲ患者接受人工耳蜗植入术时脑脊液漏的发生率高,术中修补及术后早期发现都是避免严重并发症发生的要素。针对此类患者,临床护理中需对患者术后的体温变化、隐匿性脑脊液漏及脑脊液漏并发脑膜炎进行更严密的观察,降低脑脊液漏及其他相关并发症的发生率。

Objective To report nursing experiences with observation and treatment of post-cochlear implantationcerebrospinal fluid leakage and other complications in patients with IP-III inner ear malformation caused by POU3F4gene mutation.Methods The data from 8 patients with IP-III inner ear malformation treated at the Department of Otolaryngology,Head and Neck Surgery,Chinese PLA General Hospital First Medical Center,were collected.After admission,gene detection was performed on all the patients to identify the molecular etiology.Both temporal bone CT scanand craniocerebral magnetic resonance imaging showed abnormal structures and developmental deformity of the innerear.According to Sennaroglu’s classification of inner ear malformation,IP-III type was confirmed in all the patients.Cochlear implantation combined and programming were performed under general anesthesia after absolute contraindicationswere eliminated.After operation,corresponding treatment measures were taken based on the patient conditions.Results POU3F4 gene mutations were identified in all but one patient,who did not undergo genetic testing.Stapedialgusher was found during the operation in 6 patients.One of the patients had severe postoperative complications,includingcerebrospinal fluid leakage,pulmonary infection and meningitis.Conclusion The incidence of cerebrospinal fluidleakage is relatively high in patients with IP-III inner ear malformation caused by POU3F4 mutations who undergo cochlearimplantation.Intraoperative repair and early detection after cochlear implantation are crucial to avoid severe complications.For these patients,rigorous postoperative observation of clinical signs must be performed in nursing practice,so as to reduce the incidence of cerebrospinal fluid leakage and other related complications.