CAJ | 학술논문

目的评价Waardenburg综合征(Waardenburg syndrome,WS)患者人工耳蜗植入术后的听觉言语康复效果。方法用纯音听阈、听觉行为分级标准（Categories of Auditory Performance,CAP）、言语可懂度分级标准（Speech Intelligibility Rating,SIR)、儿童听觉言语能力家长评估问卷(Parents， Evaluation of Aural/Oral Performance of Children，PEACH)，对行人工耳蜗植入术的4例WS1型患者、17例WS2型患者进行术后效果调查，并与非综合征且无明显病因的人工耳蜗植入患者比较，用SPSS 13.0进行统计学分析。结果9例WS患者与9例对照组患者术后平均助听听阈（250 Hz、500 Hz、1000 Hz、2000 Hz、4000 Hz）比较，差别无统计学意义（P>0.05）。WS患者组CAP评分、SIR评分、安静环境下得分比、噪声环境下得分比、电话交流得分与对照组相比，差异均无统计学意义（P>0.05）；WS1型组患者CAP评分、SIR评分、安静环境下得分比、噪声环境下得分比、电话交流得分与WS2型组患者相比，差异均无统计学意义（P>0.05）；所有患者安静环境下得分比明显高于噪声环境下得分比（P<0.05）。结论伴有重度或极重度感音神经性聋的Waardenburg综合征患者人工耳蜗植入术后的听觉言语能力与耳蜗形态正常的重度或极重度感音神经性聋患者无显著差别，安静环境下听觉言语能力好于噪声环境下听觉言语能力；且WS1型与WS2型患者人工耳蜗植入术后的听觉言语能力无显著差别。
목적평개Waardenburg종합정(Waardenburg syndrome,WS)환자인공이와식입술후적은각언어강복효과。방법용순음은역、은각행위분급표준（Categories of Auditory Performance,CAP）、언어가동도분급표준（Speech Intelligibility Rating,SIR)、인동은각언어능력가장평고문권(Parents， Evaluation of Aural/Oral Performance of Children，PEACH)，대행인공이와식입술적4례WS1형환자、17례WS2형환자진행술후효과조사，병여비종합정차무명현병인적인공이와식입환자비교，용SPSS 13.0진행통계학분석。결과9례WS환자여9례대조조환자술후평균조은은역（250 Hz、500 Hz、1000 Hz、2000 Hz、4000 Hz）비교，차별무통계학의의（P>0.05）。WS환자조CAP평분、SIR평분、안정배경하득분비、조성배경하득분비、전화교류득분여대조조상비，차이균무통계학의의（P>0.05）；WS1형조환자CAP평분、SIR평분、안정배경하득분비、조성배경하득분비、전화교류득분여WS2형조환자상비，차이균무통계학의의（P>0.05）；소유환자안정배경하득분비명현고우조성배경하득분비（P<0.05）。결론반유중도혹겁중도감음신경성롱적Waardenburg종합정환자인공이와식입술후적은각언어능력여이와형태정상적중도혹겁중도감음신경성롱환자무현저차별，안정배경하은각언어능력호우조성배경하은각언어능력；차WS1형여WS2형환자인공이와식입술후적은각언어능력무현저차별。
Objection To evaluate the postoperative outcomes of cochlear implant recipients with Waardenburg syndrome(WS). Methods The pure-tone audiometry, Categories of Auditory Performance (CAP), Speech Intelligibility Rating(SIR), Parents, Evaluation of Aural/Oral Performance of Children (PEACH) were performed in 4 children with WS type1 and 17 children with WS type2 after cochlear implantation. The outcomes were compared between WS patients and patients with non-syndromic congenital deafness. Results There was no significant difference in average aided hearing thresholds between 9 WS patients and 9 controls(P>0.05).There was no significant difference in CAP, SIR, scores in quiet,scores in noise and telephone communication scores between WS and non-syndromic patients, as well as WS type1 and type2(P>0.05).The scores in quiet were significantly higher than those in noise in both WS and non-syndromic hearing-impaired patients(P<0.05).Conclusion The WS patients with severe or profound hearing impairment and non-syndromic hearing-impaired patients show no significant difference in auditory and speech abilities and the scores in quiet are higher than those in noise. There is no significant difference in auditory and speech abilities between WS type1 and WS type 2 patients.