Nivoloni Karin de A B, da Silva-Costa Sueli M, Pomílio Mariza C A, Pereira Tânia, Lopes Karen de C, de Moraes Vanessa C S, Alexandrino Fabiana, de Oliveira Camila A, Sartorato Edi L
Associação Terapêutica de Estimulação Auditiva e Linguagem-ATEAL, Av Antonio Frederico Ozanan 6561, Jundiai SP 13201-125, Brazil.
Int J Pediatr Otorhinolaryngol. 2010 Aug;74(8):926-9. doi: 10.1016/j.ijporl.2010.05.015. Epub 2010 Jun 9.
An early diagnosis has been a priority in the audiological practice. Identifying hearing loss until 3 months old through Universal Newborn Hearing Screening and intervention before 6 months old, minimize the impact of auditory loss in the health and communication development of these children. However, in the clinical practice, despite the help of the risk indicators in the audiological and etiological diagnosis, the integrated services have come up against the challenge of determining the causes of auditory loss, bearing in mind that approximately 50% of the subjects who have congenital loss do not show risk factors in their clinical history. The current research aims introduce together etiologic and audiological diagnosis of newborns.
We eluted dried blood spots from paper and performed genetic testing for 35delG mutation in 8974 newborns that were also screened for transient otoacoustic emissions (TOAE). In addition, the A1555G and A827G mutations in the MTRNR1 mitochondrial gene were screened in all newborns.
We have found 17 individuals who failed in TOAE. Among them, we detected 4 homozygous newborns for 35delG mutation and 3 individuals with A827G mutation in the MTRNR1 mitochondrial gene. The frequency of 35delG carriers was 0.94% [84/8974]. In all 17 individuals who failed in OAE no other mutation besides those mentioned above was found.
The results greatly contribute to the public health area indicating the etiologic diagnosis, allowing family counseling as well as the early rehabilitation treatment or surgical intervention. Over time that will help to reduce the costs of rehabilitation considerably.
早期诊断一直是听力学实践中的首要任务。通过新生儿听力普遍筛查在3个月大之前识别听力损失,并在6个月大之前进行干预,可将听力损失对这些儿童健康和沟通发展的影响降至最低。然而,在临床实践中,尽管有听力学和病因诊断中的风险指标的帮助,但综合服务在确定听力损失原因方面仍面临挑战,因为大约50%的先天性听力损失患者在其临床病史中未显示出风险因素。当前的研究旨在综合介绍新生儿的病因诊断和听力学诊断。
我们从滤纸上洗脱干血斑,并对8974名同时接受瞬态耳声发射(TOAE)筛查的新生儿进行了35delG突变的基因检测。此外,还对所有新生儿的线粒体基因MTRNR1中的A1555G和A827G突变进行了筛查。
我们发现17名个体的TOAE检测未通过。其中,我们检测到4名35delG突变的纯合新生儿和3名线粒体基因MTRNR1中存在A827G突变的个体。35delG携带者的频率为0.94%[84/8974]。在所有17名OAE检测未通过的个体中,除上述突变外未发现其他突变。
这些结果对公共卫生领域有很大贡献,表明了病因诊断,有助于进行家庭咨询以及早期康复治疗或手术干预。随着时间的推移,这将有助于大幅降低康复成本。