Language Development and Disorder in Children with Sensorineural Hearing Loss: A UK Cohort Study

感音神经性听力损失（SNHL）是一种永久性听力损失，其严重程度从轻度（20-40 dB损失）到重度（> 90 dB损失）不等。大约0.2%的儿童出生时患有SNHL，0.6%的儿童在青少年时期患有SNHL。在过去，SNHL通常直到学龄前或更晚才被发现。然而，近年来，医学和技术的进步已经彻底改变了SNHL儿童的识别，诊断和治疗。自2000年以来，大多数在英国出生的儿童在出生时或出生后不久接受通用新生儿听力筛查（UNHS），筛查双耳> 40 dB的SNHL。这意味着SNHL通常比以前早得多（< 9个月）。因此，患有双侧、中度或重度SNHL的儿童可以在年幼时获得所需的帮助，无论是通过助听器、人工耳蜗植入物的安装，还是通过强化言语和语言治疗。这些进展使SNHL儿童的结局得到显著改善。如今，许多患有SNHL的儿童继续发展与听力正常的同龄人相称的言语和语言技能，并表现出良好的教育成就。然而，还有一些悬而未决的问题。首先，最近没有对英国SNHL儿童的结局进行大规模评估。因此，我们不知道今天在英国出生的SNHL儿童可能的结果是什么。其次，虽然UNHS检测到双侧、中度或更严重的SNHL，但它通常不会检测到轻度损失或仅影响一只耳朵的损失。因此，儿童先天性轻度或单侧SNHL通常在延迟后才被发现，有时根本无法发现。我们知道这对结果的影响。最后，尽管取得了这些进步，但相当一部分SNHL儿童在获得口语和学习阅读方面仍然存在困难。但是，我们不知道有多少人有困难，也不知道他们究竟有什么困难。此外，目前还无法预测哪些孩子会继续发展正常的语言，哪些不会。为了解决这些问题，我们将开展六项研究。在第一项研究中，我们将测试一大批早期发现的轻度至重度双侧SNHL儿童的沟通，语言，社交，情感和行为（SEB）以及学术能力。这将告诉我们这些孩子做得有多好。然后我们将在三年内跟踪这一组。这将有助于我们了解SNHL儿童的能力如何随时间变化，并确定哪些因素是导致这些变化的重要因素。在其他研究中，我们将测量患有（i）轻度SNHL，（ii）单侧SNHL和（iii）一种罕见形式的SNHL（称为听神经病谱系障碍（ANSD））的儿童的相同结局。这些研究将告诉我们这些群体可能的结果是什么，以及早期识别这些群体的结果是否有优势。最后，我们将识别那些在口语和/或阅读能力方面表现出特殊困难的SNHL儿童。我们将确定这些困难有多常见，以及这些困难与听力正常但口语和/或书面语言能力差的儿童相比如何。研究结果将告诉我们今天在英国出生的SNHL小学年龄儿童的可能结果，无论他们是否有轻度，中度，严重或严重的损失，双边或单边损失，或ANSD。他们还将告诉我们，患有轻度或单侧SNHL的儿童是否会从UNHS的识别中受益。最后，研究结果将提供更深入的了解一些SNHL儿童在获得口语和学习阅读方面所经历的困难。这些信息将为今后帮助这些儿童的新干预措施铺平道路。

Sensorineural hearing loss (SNHL) is a permanent hearing loss that can vary in severity from mild (20-40 dB loss) to profound (> 90 dB loss). Around 0.2% of children are born with SNHL, and 0.6% will have SNHL by the time they are in their teenage years. In the past, SNHL was often not detected until the preschool years or later. However, in recent years, medical and technological advances have revolutionised the identification, diagnosis, and treatment of children with SNHL. Since 2000, the majority of children born in the UK undergo a universal newborn hearing screen (UNHS) at or shortly after birth, which screens for SNHL of > 40 dB in both ears. This means that SNHL is typically identified much earlier than before (< 9 months). Consequently, children with bilateral, moderate or worse SNHL can receive the help they need to hear at a young age, whether that be through the fitting of hearing aids, cochlear implants, or access to intensive speech and language therapy. These advances have led to significant improvements in outcomes for children with SNHL. Nowadays, many children with SNHL go on to develop speech and language skills that are commensurate with their normally hearing peers, and show good educational attainments. However, there are a number of outstanding issues. First, there has been no recent large-scale assessment of outcomes in children with SNHL in the UK. Therefore, we do not know what the likely outcomes are for children born with SNHL in the UK today. Second, while the UNHS detects bilateral, moderate or worse SNHL, it does not routinely detect milder losses, or losses affecting one ear only. Therefore, congenital mild or unilateral SNHL in children is often detected after a delay, and sometimes not at all. We do know the effects of this on outcomes. Finally, despite these advances, a significant proportion of children with SNHL still have difficulties in acquiring spoken language, and in learning to read. However, we do not know how many have difficulties, and precisely what difficulties they have. Moreover, at present, it is not possible to predict which children will go on to develop normal language, and which will not. To address these issues we will carry out six studies. In the first study, we will test the communication, language, social, emotional and behavioural (SEB), and academic abilities of a large group of children with early-identified mild-to-profound bilateral SNHL. This will tell us how well these children are actually doing. We will then follow this group up over three years. This will help us to understand how the abilities of children with SNHL change over time, and identify what factors are important in causing these changes. In other studies we will measure the same outcomes in children with (i) mild SNHL, (ii) unilateral SNHL, and (iii) a rare form of SNHL known as auditory neuropathy spectrum disorder (ANSD). These studies will tell us what the likely outcomes for these groups are, and whether there is an advantage of early identification for outcomes in these groups. Finally, we will identify those children with SNHL who show particular difficulties in their oral language and/or reading abilities. We will determine how common these difficulties are, and how these difficulties compare to those of children with normal hearing who also have poor oral and/or written language. The findings will tell us about the likely outcomes for primary-school aged children born with SNHL in the UK today, whether they have mild, moderate, severe or profound losses, bilateral or unilateral losses, or ANSD. They will also tell us whether children with mild or unilateral SNHL would benefit from being identified at the UNHS. Finally, the findings will provide greater insight into the difficulties experienced by some children with SNHL in acquiring spoken language and in learning to read. This information will pave the way for new interventions that could help such children in the future.