Receptive language acquisition in a pediatric population with Pendred syndrome and non-syndromic enlarged vestibular aqueduct

Abstract

Background: Children with Pendred syndrome (PS) and non-syndromic enlarged vestibular aqueduct (NSEVA) represent a group of pre-lingual hearing-impaired individuals with rehabilitation challenges.

Objective: To evaluate receptive language capabilities in a pediatric cohort with PS and NSEVA.

Materials and methods: Twenty-four (24) children diagnosed with either PS or NSEVA, were examined using the Peabody Picture Vocabulary Test (PPVT) and compared to a cohort of 55 Danish children with normal hearing, as well as to a mixed group of 29 children with hearing impairment of congenital and hereditary non-syndromal type. In addition, test results were compared to normative data (PPVT-4 US standard scores).

Results: PS/NSEVA children’s PPVT-4 test scores fall within the normative data for the PPVT-4 (US) but proved to be significantly lower statistically, when compared to Danish children with normal hearing (p<.0001) or to children with mixed non-syndromic hereditary hearing impairment (p=.006). Implantation age was significantly later for the PS/NSEVA group (median = 43 months), compared to the mixed non-syndromic hereditary congenial hearing impairment group (median = 11 months).

Conclusions and significance: Children with PS/NSEVA perform below age equivalent for receptive vocabulary outcome when compared to both children with normal hearing, and children with non-syndromic mixed hereditary congenital hearing impairment who receive cochlear implants earlier.

Chinese abstract

背景：Pendred综合征性（PS）和非综合征性前庭导水管扩大（NSEVA）的儿童是一类语言前听力障碍患者, 他们的康复具挑战性。

目的：评价PS和NSEVA儿童的接受性语言能力。

材料与方法：采用Peabody图片词汇测验（PPVT）, 对24名诊断为PS或NSEVA的儿童进行测试, 并与55名听力正常的丹麦儿童以及29名先天性和遗传性非综合征性听力障碍儿童进行比较。此外, 将测试结果与标准数据（PPVT-4US标准分）进行比较。

结果：PS/NSEVA儿童的PPVT-4测试分在PPVT-4（US）的标准数据范围之内, 但与听力正常的丹麦儿童（p<0.0001）或混合性非综合征遗传性听力障碍儿童（p=0.006）相比, 在统计学上显著较低。与混合性非综合征遗传性先天性听力损伤组（中位数=11个月）相比, PS/NSEVA组的植入年龄明显较晚（中位数=43 个月）

结论与意义：PS/NSEVA儿童与听力正常儿童和非综合征性混合遗传性先天性听力障碍儿童相比, 在接受词汇方面表现出低于同年龄儿童的水平。

Keywords:

• Receptive language outcomes
• pendred syndrome
• peabody picture vocabulary test
• pediatric cohort
• non-syndromic enlarged vestibular aqueduct

Acknowledgements

The authors thank Professor emeritus Jacob L. Mey III, for his kind revision of the manuscript with regard to the English language, and speech and language pathologist Anne-Marie Caron for assistance in testing the children.

Disclosure statement

No potential conflict of interest was reported by the authors.

Additional information

Funding

This work was supported by Oticon Foundation.