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

描述（由申请人提供）：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智力迟钝和耳聋的儿童。目前，几乎没有令人信服的证据支持这种观点，即人工耳蜗植入对没有认知潜力的儿童有好处，无法发展正常的言语和语言。同样，儿童人工耳蜗植入也有一定的风险。因此，如果聋儿有严重的认知迟缓，许多中心不会给他们植入植入物。相反，治疗智力迟钝儿童听力损失的最流行的方法是使用强大的助听器来实现振动触觉或对低频声音的声学敏感性，就像在耳蜗植入发展之前为所有失聪儿童所做的那样。典型的临床结果是声音意识，即检测环境声音和声音的能力。人工耳蜗可以提高听觉阈值和单词识别能力，但由于智力低下患者的智力，这种额外信息的价值是值得怀疑的。由于目前尚不清楚在这一人群中助听器和人工耳蜗哪种治疗更合适；这个问题最好通过随机临床试验来回答。我们的初步数据表明，在人工耳蜗植入后，这类儿童的语言和认知发展有所增加。基于这些回顾性数据，我们假设，与使用助听器相比，使用人工耳蜗治疗的智力迟钝聋儿的发育和生活质量会得到更大的改善。我们将进行一项前瞻性随机临床试验，以回答哪种干预措施对这群儿童更有益的问题，使用经过验证的标准参考测试。所有在德州儿童医院接受人工耳蜗植入的儿童都将接受认知能力评估。符合智力迟钝标准的患者将给予助听器或人工耳蜗，并进行为期两年的纵向随访。如果我们的假设是正确的，并且人工耳蜗可以显著改善智力迟钝聋儿的发育和生活质量，那么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导方针，帮助选择智力迟钝儿童听力损失的治疗方法。这一建议意义重大，因为有特殊需要的儿童值得作为治疗决策依据的证据，但在医学文献中代表性不足，而且往往没有进行研究。这项研究旨在满足美国国立卫生研究院路线图的标准，因为它将产生这种可以直接改善患者护理的客观证据。

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.