MECHANISMS AND ANTIVIRAL THERAPY OF HEARING LOSS IN CONGENITAL CMV INFECTION

本子项目是利用由NIH/NCRR资助的中心赠款提供的资源的众多研究子项目之一。子项目和研究者（PI）可能已经从另一个NIH来源获得了主要资金，因此可以在其他CRISP条目中表示。列出的机构是中心的，不一定是研究者的机构。该项目为在UAB医院和（自1999年起）Cooper Green医院通过新生儿筛查发现的先天性巨细胞病毒（CMV）感染的婴儿和儿童提供多学科随访。本研究的目的是确定宫内感染引起神经损伤的机制。该研究对先天性巨细胞病毒感染患者进行随访，提供听力、视力和认知功能的一系列评估，从而确定先天性巨细胞病毒感染的结局。听力损失为先天性巨细胞病毒感染儿童提供了一个方便、容易获得和重要的指标，可以客观地测量。大多数先天性巨细胞病毒感染导致听力损失的儿童表现出出生后听力缺陷的进展，这表明影响听力的致病性事件发生在儿童早期。我们将调查先天性巨细胞病毒感染儿童的全身病毒负担与预后之间的关系。还将分析抗病毒抗体和细胞免疫反应的系统性措施在不良后果发展中的作用。纳入的受试者将被随访3年，定期进行听力学评估并收集样本（尿液和外周血），以检测病毒载量和cmv特异性免疫反应。GCRC的支持将促进先天性巨细胞病毒感染儿童的登记和随访以及标本的收集。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. This project provides multi-disciplinary follow-up of infants and children with congenital cytomegalovirus (CMV) infection who are identified through screening of newborns at UAB Hospital and (since 1999) Cooper Green Hospital. The objective of this study is to define the mechanisms of neurologic damage from this intrauterine infection. The study follows patients with congenital CMV infection providing serial assessments of hearing, vision, and cognitive function, allowing definition of outcome of congenital CMV infection. Hearing loss offers a convenient, readily available and important endpoint that can be objectively measured in children with congenital CMV infection. The majority of children with hearing loss from congenital CMV infection exhibit postnatal progression of the deficit and this suggests that pathogenic events affecting hearing are occurring during early childhood. We will investigate the association between measures of systemic virus burden and outcome in children with congenital CMV infection. An analysis of the role of systemic measures of antiviral antibody and cellular immune responses in the development of an adverse outcome will also be undertaken. The enrolled subjects will be followed for a period of 3 years with regular audiologic assessments and samples collected (urine and peripheral blood) for viral load and CMV-specific immune response. The GCRC support will facilitate in the enrollment and follow-up of children with congenital CMV infection and in the collection of specimens.