1277例0～6岁永久性听力损失患儿确诊年龄与发现途径
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摘要
目的分析0～6岁永久性听力损失儿童的确诊年龄及发现途径。方法回顾性分析经北京同仁医院儿童听力诊断中心确诊的1 277例0～6岁永久性听力损失儿童的临床资料,根据是否接受新生儿听力筛查及筛查结果分为三组:筛查未通过组、筛查通过组及未筛查组,分析患儿的确诊年龄及发现途径。结果 1 277例听力损失患儿中,筛查未通过组1 005例(78.70%),确诊的中位年龄为4个月;筛查通过组96例(7.52%),确诊的中位年龄为21.5个月;未筛查组176例(13.78%),确诊的中位年龄为24.5个月。筛查未通过组的听力损失确诊年龄明显早于筛查通过组和未筛查组(P<0.01);筛查通过组与未筛查组之间差异无统计学意义(P>0.05)。1 277例中,发现途径主要为新生儿听力筛查(78.70%,1 005/1277);筛查通过组96例中,主要通过家人对其听觉和言语发育观察发现(66.67%,64/96),其次为耳聋基因筛查阳性转诊(11.46%,11/96)和入园体检发现(7.29%,7/96);未筛查组176例中,主要通过家人对其听觉和言语发育观察发现(98.29%,173/176)。结论新生儿听力筛查是早期发现永久性听力损失患儿的主要途径,使患儿确诊年龄明显提前,而筛查通过组和未筛查组确诊年龄仍然较晚。
Objective To study the diagnosis age and the approaches to identifying permanent hearing loss(PHL) in children aged 0～6 years, and their relationship with universal newborn hearing screening(UNHS).Methods The medical histories of 1 277 0～6-year-old children diagnosed with permanent hearing loss(PHL) were reviewed. According to their acceptance of the UNHS and screening results, children were divided into three groups: the failed group, the passed group and the unscreened group. The diagnosis age and the methods to identify PHL were analyzed.Results Of the 1 277 cases, 1 005 cases(78.70%) failed the UNHS, with a median diagnosis age of 4 months; 96 cases(7.52%) passed the UNHS, with a median diagnosis age of 21.5 months; and 176 cases(13.78%) were not screened, with a median diagnosis age of 24.5 months. The diagnosis of hearing loss in the failed group was significantly earlier than that in the passed group(P<0.01) and the unscreened group(P<0.01). UNHS was the main way of identifying PHL with 1 005 cases(78.70%). In the passed group, the identification of hearing loss was most often from parental observation on hearing and speech development(n=64, 66.67%), followed by deafness gene screening(n=11, 11.46%) and school hearing screening(n=7,7.29%).In the unscreened group, the identification of hearing loss was mainly from parental observation on hearing and speech development(n=173, 98.29%).Conclusion UNHS significantly improved the early diagnosis age of PHL; however, for the passed group it remained late. This study highlights the importance of UNHS. Furthermore, the parental observation on the hearing and speech development of the children who passed the UNHS and hearing health care programs can promote the early identification for children with hearing loss.
Objective To study the diagnosis age and the approaches to identifying permanent hearing loss(PHL) in children aged 0～6 years, and their relationship with universal newborn hearing screening(UNHS).Methods The medical histories of 1 277 0～6-year-old children diagnosed with permanent hearing loss(PHL) were reviewed. According to their acceptance of the UNHS and screening results, children were divided into three groups: the failed group, the passed group and the unscreened group. The diagnosis age and the methods to identify PHL were analyzed.Results Of the 1 277 cases, 1 005 cases(78.70%) failed the UNHS, with a median diagnosis age of 4 months; 96 cases(7.52%) passed the UNHS, with a median diagnosis age of 21.5 months; and 176 cases(13.78%) were not screened, with a median diagnosis age of 24.5 months. The diagnosis of hearing loss in the failed group was significantly earlier than that in the passed group(P<0.01) and the unscreened group(P<0.01). UNHS was the main way of identifying PHL with 1 005 cases(78.70%). In the passed group, the identification of hearing loss was most often from parental observation on hearing and speech development(n=64, 66.67%), followed by deafness gene screening(n=11, 11.46%) and school hearing screening(n=7,7.29%).In the unscreened group, the identification of hearing loss was mainly from parental observation on hearing and speech development(n=173, 98.29%).Conclusion UNHS significantly improved the early diagnosis age of PHL; however, for the passed group it remained late. This study highlights the importance of UNHS. Furthermore, the parental observation on the hearing and speech development of the children who passed the UNHS and hearing health care programs can promote the early identification for children with hearing loss.
引文
1 American Academy of Pediatrics, Joint Committee on Infant Hearing. Year 2007 position statement: principles and guidelines for early hearing detection and intervention[J]. Pediatrics,2007,120:898.
2 袁涛, 曾祥丽. 新生儿耳聋防控体系建设的历程与现状(1)——新生儿听力筛查[J]. 听力学及言语疾病杂志, 2018, 26:209.
3 张圆, 李刚, 郑芸. 0～9月龄弱听婴儿早期诊断及干预状况研究[J]. 临床耳鼻咽喉头颈外科杂志, 2014,28:1748.
4 卜行宽, 刘鋋.世界卫生组织预防聋和听力减退工作情况介绍[J]. 中华耳鼻咽喉科杂志, 2000, 35:237.
5 Kim SY, Park G, Han KH, et al. Prevalence of p.V37I variant of GJB2 in mild or moderate hearing loss in a pediatric population and the interpretation of its pathogenicity[J]. PLoS One, 2013, 8:e61592.
6 黄丽辉,韩德民,张蕾,等. 0～6岁听力损失儿童的发现年龄及发现途径[J]. 中华耳鼻咽喉头颈外科杂志,2006,41:331.
7 Dalzell L, Orlando M, Macdonald M, et al. The New York State universal newborn hearing screening demonstration project: ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention[J]. Ear Hear, 2000, 21:118.
8 Kai U, Bamford J. Effectiveness of population-based newborn hearing screening in England: ages of interventions and profile of cases[J]. Pediatrics, 2006, 117:887.
9 张亚梅, 张天宇.实用小儿耳鼻咽喉科学[M].北京: 人民卫生出版社,2011.4～166.
10 Weichbold V, Nekahm-Heis D, Welzl-Mueller K. Universal newborn hearing screening and postnatal hearing loss[J]. Pediatrics,2006,117:e631.
11 Young NM, Reilly BK, Burke L. Limitations of universal newborn hearing screening in early identification of pediatric cochlear implant candidates[J]. Arch Otolaryngol Head Neck Surg,2011,137:230.
12 Dedhia K, Kitsko D, Sabo D, et al. Children with sensorineural hearing loss after passing the newborn hearing screen[J]. JAMA Otolaryngol Head Neck Surg,2013,139:119.
13 黄丽辉, 王雪瑶.儿童迟发性听力损失:值得重视[J]. 中华耳鼻咽喉头颈外科杂志,2018,53:172.
14 Wu CC, Tsai CH, Hung C, et al. Newborn genetic screening for hearing impairment: a population-based longitudinal study[J]. Genet Med,2017,19:6.
15 Chen G, Fu S, Luo S, et al.Screening of delayed-onset hearing loss in preschool children in the mid-south of China[J]. Int J Audiol,2013, 52:568.
2 袁涛, 曾祥丽. 新生儿耳聋防控体系建设的历程与现状(1)——新生儿听力筛查[J]. 听力学及言语疾病杂志, 2018, 26:209.
3 张圆, 李刚, 郑芸. 0～9月龄弱听婴儿早期诊断及干预状况研究[J]. 临床耳鼻咽喉头颈外科杂志, 2014,28:1748.
4 卜行宽, 刘鋋.世界卫生组织预防聋和听力减退工作情况介绍[J]. 中华耳鼻咽喉科杂志, 2000, 35:237.
5 Kim SY, Park G, Han KH, et al. Prevalence of p.V37I variant of GJB2 in mild or moderate hearing loss in a pediatric population and the interpretation of its pathogenicity[J]. PLoS One, 2013, 8:e61592.
6 黄丽辉,韩德民,张蕾,等. 0～6岁听力损失儿童的发现年龄及发现途径[J]. 中华耳鼻咽喉头颈外科杂志,2006,41:331.
7 Dalzell L, Orlando M, Macdonald M, et al. The New York State universal newborn hearing screening demonstration project: ages of hearing loss identification, hearing aid fitting, and enrollment in early intervention[J]. Ear Hear, 2000, 21:118.
8 Kai U, Bamford J. Effectiveness of population-based newborn hearing screening in England: ages of interventions and profile of cases[J]. Pediatrics, 2006, 117:887.
9 张亚梅, 张天宇.实用小儿耳鼻咽喉科学[M].北京: 人民卫生出版社,2011.4～166.
10 Weichbold V, Nekahm-Heis D, Welzl-Mueller K. Universal newborn hearing screening and postnatal hearing loss[J]. Pediatrics,2006,117:e631.
11 Young NM, Reilly BK, Burke L. Limitations of universal newborn hearing screening in early identification of pediatric cochlear implant candidates[J]. Arch Otolaryngol Head Neck Surg,2011,137:230.
12 Dedhia K, Kitsko D, Sabo D, et al. Children with sensorineural hearing loss after passing the newborn hearing screen[J]. JAMA Otolaryngol Head Neck Surg,2013,139:119.
13 黄丽辉, 王雪瑶.儿童迟发性听力损失:值得重视[J]. 中华耳鼻咽喉头颈外科杂志,2018,53:172.
14 Wu CC, Tsai CH, Hung C, et al. Newborn genetic screening for hearing impairment: a population-based longitudinal study[J]. Genet Med,2017,19:6.
15 Chen G, Fu S, Luo S, et al.Screening of delayed-onset hearing loss in preschool children in the mid-south of China[J]. Int J Audiol,2013, 52:568.