CAJ | 학술논문

目的：探讨CHARGE综合征的临床特点、听力学特征及人工耳蜗植入疗效。方法通过对我科收治的CHARGE综合征的患儿临床资料收集，结合相关文献分析其临床特征及耳部解剖特点、听力学特点，探讨其人工耳蜗植入的价值及要点。结果两例患儿临床上诊断CHARGE综合征基本明确，均有不同程度的耳部畸形及听力障碍，病例一因其他一些严重的功能障碍于生后5个月时死亡；病例二经过后期的充分及时的治疗，恢复良好，随访听力1年，言语发育良好。结论CHARGE综合征的诊断基于临床表现，对于合并重度耳聋的患儿，在全身状况允许的情况下进行人工耳蜗植入是听力康复的最佳选择。
목적：탐토CHARGE종합정적림상특점、은역학특정급인공이와식입료효。방법통과대아과수치적CHARGE종합정적환인림상자료수집，결합상관문헌분석기림상특정급이부해부특점、은역학특점，탐토기인공이와식입적개치급요점。결과량례환인림상상진단CHARGE종합정기본명학，균유불동정도적이부기형급은력장애，병례일인기타일사엄중적공능장애우생후5개월시사망；병례이경과후기적충분급시적치료，회복량호，수방은력1년，언어발육량호。결론CHARGE종합정적진단기우림상표현，대우합병중도이롱적환인，재전신상황윤허적정황하진행인공이와식입시은력강복적최가선택。
Objective To investigate clinical and audiological features of CHARGE syndrome and effica-cy of cochlear implant. Methods Clinical data of 2 children with CHARGE syndrome seen in our department, including clinical and audiological features, were analyzed in reference to relevant literatures. The value of co-chlear implant as treatment and related key points were discussed. Results Both children showed varying de-grees of ear malformations and hearing disorders. One child died five months after birth from unrelated seri-ous conditions, while the other recovered well after full and timely treatment, with good speech development in the following year. Conclusion Diagnosis of CHARGE syndrome is primarily based on clinical manifesta-tions. Cochlear implants is the best option for patients with severe sensorineural hearing loss.