Outcomes in Children with Developmental Delay and Deafness: a Randomized Trial

描述(申请人提供)：有特殊需要的儿童需要复杂的、个性化的治疗，以最大限度地提高他们的长期生活质量。有特殊需要的儿童的一个子集包括那些既有智力障碍又有耳聋的儿童。目前，几乎没有令人信服的证据支持这样一种观点，即人工耳蜗植入对没有认知潜力发展正常言语和语言的儿童有利。此外，儿童人工耳蜗术确实存在一定程度的风险。因此，如果聋儿有严重的认知障碍，许多中心将不会植入他们。相反，治疗智力低下儿童听力损失的最受欢迎的方法是使用强大的助听器来实现对低频声音的振动触觉或声学敏感性，其方式类似于人工耳蜗开发之前对所有聋哑儿童所做的事情。典型的临床结果是声音感知，即检测环境声音和声音的能力。人工耳蜗植入可以提高听力阈值和单词识别能力，但由于智力低下患者的智商较低，这种额外信息的价值值得怀疑。因为，目前还不清楚在这一人群中，使用助听器或人工耳蜗治疗更合适；这个问题最好通过随机临床试验来回答。我们的初步数据显示，植入人工耳蜗后，这群儿童的语言和认知发展都有所提高。基于这些回顾数据，我们假设，与使用助听器治疗相比，植入人工耳蜗会更多地改善智力低下聋儿的发育和生活质量。我们将进行一项前瞻性、随机的临床试验，以回答哪种干预措施对这群儿童更有利的问题，使用经过验证的常模参考试验。得克萨斯儿童医院所有有资格植入人工耳蜗的儿童都将接受认知评估。符合精神发育迟滞标准的患者将接受助听器或人工耳蜗植入，并纵向跟踪两年。如果我们的假设是正确的，并且人工耳蜗植入显著改善了智力低下聋儿的发育和生活质量，我们的研究将为支持这一人群的临床决策提供必要的证据。因此，我们的长期目标是制定指南，在选择智力低下儿童听力损失的治疗时可能有所帮助。这一建议意义重大，因为有特殊需要的儿童应该得到证据，作为治疗决策的基础，但在医学文献中的代表性仍然不足，而且往往没有得到研究。这项研究旨在满足NIH路线图的标准，因为它将产生这种类型的客观证据，可以直接改善患者护理。 公共卫生相关性：这项研究与人类健康相关，因为它将提供证据来回答是否用人工耳蜗或助听器来治疗智力低下的聋儿的问题。

DESCRIPTION (provided by applicant): Children with special needs require complex, individualized therapy to maximize their long-term quality of life. One subset of children with special needs includes those with both mental retardation and deafness. Currently, there is little compelling evidence supporting the idea that cochlear implantation provides benefit to children that don't have the cognitive potential to develop normal speech and language. As well, pediatric cochlear implantation does entail a certain level of risk. Therefore, many centers will not implant deaf children if they have severe cognitive delays. Instead, the most popular method of treating hearing loss in children with mental retardation is to use powerful hearing aids to achieve vibrotactile sensations or acoustic sensitivity to low frequency sounds in a similar fashion to what was done for all deaf children prior to the development of cochlear implants. The typical clinical outcome is sound awareness, i.e. the ability to detect environmental sounds and voices. A cochlear implant can improve auditory thresholds and word recognition ability, but because of the lower intelligence of patients with mental retardation, this value of this additional information is questionable. Since, it is unknown whether treatment with hearing aids or a cochlear implant is more suitable in this population; this question is best answered by a randomized clinical trial. Our preliminary data demonstrate that linguistic and cognitive development increase in this population of children after cochlear implantation. Based on these retrospective data, we hypothesize that development and quality of life will improve more in deaf children with mental retardation when treated with a cochlear implant compared to those treated with hearing aids. We will perform a prospective, randomized clinical trial to answer the question of which intervention provides more benefit to this population of children using validated, norm-referenced tests. All children eligible for cochlear implantation seen at Texas Children's Hospital will undergo a cognitive evaluation. Patients that meet the criteria for mental retardation will be given either hearing aids or a cochlear implant and followed longitudinally for two years. If our hypothesis is correct and cochlear implants significantly improve development and quality of life in deaf children with mental retardation, our study will provide essential evidence to support clinical decision-making in this population. Thus, our long-term goal is to develop guidelines that may help when selecting a treatment for hearing loss in a child with mental retardation. This proposal is significant because children with special needs are deserving of evidence upon which to base treatment decision-making, but remain under-represented in the medical literature and are often not studied. This research is designed to meet the criteria for the NIH roadmap because it will generate this type of objective evidence that can directly improve patient care. PUBLIC HEALTH RELEVANCE: This research is relevant to human health because it will provide evidence to answer the question of whether to treat a deaf child with mental retardation with a cochlear implant or hearing aids.