fMRI, Genes and Outcome for Cochlear Implants in Infants

描述(申请人提供)：人工耳蜗植入(CI)彻底改变了先天性耳聋儿童的治疗方法。[1]越来越多的证据表明，在先天性耳聋的婴幼儿和学步儿童中尽可能早地实施CI效果最佳。[2，3]然而，婴儿的听觉和语言技能很难准确评估。[4]目前在38个州实施的新生儿听力筛查计划，预计每天在全国范围内识别30-40名先天性耳聋儿童，[6][7]迫切需要制定评估和管理战略，以便在12-18个月龄前利用早期干预机会窗口(如CI)进行干预。 功能磁共振成像(FMRI)为评估婴儿的中枢听觉过程提供了一种新的工具。[8-10]功能磁共振成像是非侵入性的，不存在已知的生物学风险，即使对婴儿也是如此。[11]功能磁共振成像可以在正常和听力受损的婴儿中区分对应于听觉检测、言语感知和语言处理的大脑反应。[12]功能磁共振成像能够显示婴幼儿不同水平的听觉和语言处理，这可以为临床医生提供任何其他方法在语前婴儿中无法获得的重要数据。 目前的建议旨在确定(H2)植入前皮层激活模式在婴儿期是否会与人工耳蜗术后两年的听觉表现相关，以及(H1)fMRI是否可以揭示婴儿时期中枢听觉系统的临床相关细节。我们设计了三个具体的目标来检验这些假说。我们计划(S1)在临床3T MRI系统上优化无声背景、听觉刺激的fMRI方法，(S2)获取正常听力(n=30)和听力受损婴儿(n=60)的fMRI和听力测试数据，(S3)1岁听力受损婴儿fMRI显示的相关中枢听觉激活模式，并以人工耳蜗植入2年后的结果为指标。我们希望这项拟议研究的成功完成将为临床医生提供一种新的工具来指导听力受损婴儿的最佳干预策略。

DESCRIPTION (provided by applicant): Cochlear implantation (CI) has revolutionized the management of congenitally deaf children.[1] A growing body of evidence suggests that that performing CI as early as possible in congenitally deaf infants and toddlers results in the best outcome.[2, 3] However, auditory perception and language skills are difficult to assess accurately in infants.[4] Newborn hearing screening programs now in place in 38 states, are expected to identify 30-40 children per day nationally with congenital hearing loss, [6][7] precipitating the need for assessment and management strategies that utilize early windows of opportunity for interventions (such as a CI) by 12-18 months of age. Functional magnetic resonance imaging (fMRI), offers a novel tool for evaluation of central auditory processes in infants.[8-10] fMRI is non-invasive and presents no known biological risks, even for infants.[11] fMRI may permit differentiation of brain responses corresponding to auditory detection, speech perception and language processing in normal and hearing-impaired infants.[12] The ability of fMRI to demonstrate various levels of auditory and language processing in infants and toddlers can provide important data to clinicians that is not available by any other means in prelingual babies. The current proposal is designed to ascertain whether (H2) pre-implant cortical activation patterns revealed by fMRI during infancy will correlate with auditory performance two years after cochlear implantation and whether (H1)fMRI can reveal clinically relevant details about the central auditory system in infancy. Three specific aims are designed to test these hypotheses. We plan to (S1) optimize a silent background, auditory stimulation fMRI method on a clinical 3T MRI system, (S2) acquire fMRI and hearing test data on normal hearing (n=30) and hearing impaired infants (n=60) and (S3) correlate central auditory activation pattems revealed by fMRI in hearing impaired infants at 1 year of age, with outcome measures 2 years after cochlear implantation. Our hope is that successful completion of the proposed study will provide clinicians with a new tool to guide optimal intervention strategies for hearing-impaired infants.