Audiological profile in children with congenital inner ear anomalies.

IF 1.4 Q2 OTORHINOLARYNGOLOGY
Mohamed Mohamed El-Badry, Amira Fawzy, Mohamed Makhlouf Hasan, Fatma Refat
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引用次数: 0

Abstract

Objectives: The current study aims to describe subjects and audiological profiles of children with congenital inner ear anomalies (IEAs).

Materials and methods: A total of 193 children with sensorineural hearing loss (SNHL) and radiological evidence of one or more congenital IEAs were included.

Results and discussion: The most common IEAs in the current study was enlarged vestibular aqueduct (EVA) either isolated or associated with other IEAs. Incomplete partition (IP) with its three types (IP I, IP II, and IP III) was the second common anomaly, followed by cochlear hypoplasia (CH). At the time of radiological diagnosis, hearing loss degrees ranged from mild to profound in children with EVA, CH III, and CH IV with the majority having severe or profound degrees. The prevalence of severe and profound degree of hearing loss was higher in children with IP than children with isolated EVA or CH III and CH IV. In children with isolated EVA, hearing loss was asymmetric in 52.2% and progressive in 58%. In children with CH III and CH IV, hearing loss was asymmetric but stationary. Only children with EVA and IP III had air-bone gab (ABG) at low frequencies, while children with other IEAs did not have ABG except if there was an association with EVA. Children with severe anomalies such as CH I, CH II, common cavity, and cochlear nerve hypoplasia had profound degrees of hearing loss or just sound detection.

Conclusions: Knowing the audiological profile of children with IEAs has important clinical implications in the management of those children.

先天性内耳畸形儿童的听力学特征。
目的:本研究旨在描述先天性内耳畸形儿童的对象和听力学特征。材料和方法:共纳入193例伴有一种或多种先天性iea的感音神经性听力损失(SNHL)患儿。结果和讨论:目前研究中最常见的IEAs是前庭导水管(EVA)扩大,无论是孤立的还是与其他IEAs合并的。第二常见的异常是不完全性隔裂(IP),包括IP I、IP II和IP III三种类型,其次是耳蜗发育不全(CH)。在放射学诊断时,EVA、CH III和CH IV患儿的听力损失程度从轻度到重度不等,其中大多数为重度或重度。IP患儿中重度和重度听力损失的发生率高于孤立性EVA患儿或chiii和chiv患儿。在孤立性EVA患儿中,听力损失不对称的占52.2%,进行性听力损失的占58%。在chiii和chiv患儿中,听力损失不对称但稳定。只有患有EVA和IP III的儿童有低频率的气骨口漏(ABG),而患有其他IEAs的儿童除了与EVA相关外没有ABG。严重异常如CH I、CH II、共腔、耳蜗神经发育不全的患儿存在重度听力损失或仅能听到声音。结论:了解IEAs患儿的听力学特征对这些患儿的治疗具有重要的临床意义。
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来源期刊
COCHLEAR IMPLANTS INTERNATIONAL
COCHLEAR IMPLANTS INTERNATIONAL Medicine-Otorhinolaryngology
CiteScore
3.10
自引率
0.00%
发文量
29
期刊介绍: Cochlear Implants International was founded as an interdisciplinary, peer-reviewed journal in response to the growing number of publications in the field of cochlear implants. It was designed to meet a need to include scientific contributions from all the disciplines that are represented in cochlear implant teams: audiology, medicine and surgery, speech therapy and speech pathology, psychology, hearing therapy, radiology, pathology, engineering and acoustics, teaching, and communication. The aim was to found a truly interdisciplinary journal, representing the full breadth of the field of cochlear implantation.
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