Otorhinolaryngology and Audiology, Institute for Maternal and Child Health IRCCS "Burlo Garofolo", Trieste, Italy.
Radiology, Institute for Maternal and Child Health IRCCS "Burlo Garofolo", Trieste, Italy.
Sci Rep. 2021 Feb 8;11(1):3338. doi: 10.1038/s41598-021-82818-9.
The use of neonatal hearing screening has enabled the identification of congenital unilateral sensorineural hearing loss (USNHL) immediately after birth, and today there are several intervention options available to minimize potential adverse effects of this disease, including cochlear implantation. This study aims to analyze the characteristics of the inner ear of a homogeneous group of congenital non-syndromic USNHL to highlight the features of the inner ear, which can help in clinical, surgical, and rehabilitative decision-making. A retrospective chart review was carried out at a tertiary referral center. Systematic diagnostic work-up and rigorous inclusion-exclusion criteria were applied to 126 children with unilateral hearing impairment, leading to a selection of 39 strictly congenital and non-syndromic USNHL cases, undergoing computed tomography (CT) and magnetic resonance (MR) imaging studies. The frequency and type of malformations of the inner ear in USNHL and unaffected contralateral ears were assessed, with an in-depth analysis of the deficiency of the cochlear nerve (CND), the internal auditory canal (IAC) and the cochlear aperture (CA). Inner ear anomalies were found in 18 out of 39 (46%) of the USNHL patients. In 1 subject, the anomalies were bilateral, and the CND resulted in the predominant identified defect (78% of our abnormal case series), frequently associated with CA stenosis. Only 3 out of 14 children with CND presented stenosis of the IAC. CND and CA stenosis (and to a much lesser extent IAC stenosis) are a frequent association within congenital and non-syndromic USNHL that could represent a distinct pathological entity affecting otherwise healthy infants. In the context of a diagnostic work-up, the evaluation with CT and MRI measurements should take place in a shared decision-making setting with thorough counseling. Both imaging techniques have proven useful in differentiating the cases that will most likely benefit from the cochlear implant, from those with potentially poor implant performance.
新生儿听力筛查使先天性单侧感觉神经性听力损失（USNHL）能在出生后立即得到诊断，目前有多种干预措施可供选择，以尽量减少这种疾病的潜在不良影响，包括人工耳蜗植入。本研究旨在分析一组先天性非综合征性 USNHL 患者内耳的特征，以突出内耳的特征，这有助于临床、手术和康复决策。在一家三级转诊中心进行了回顾性图表审查。对 126 名单侧听力障碍儿童进行了系统的诊断性检查，并应用严格的纳入和排除标准，最终选择了 39 例严格的先天性和非综合征性 USNHL 病例，对其进行了计算机断层扫描（CT）和磁共振（MR）成像研究。评估了 USNHL 及对侧未受影响耳的内耳畸形的频率和类型，深入分析了蜗神经（CND）、内听道（IAC）和耳蜗口（CA）的缺失。在 39 例 USNHL 患者中，有 18 例（46%）存在内耳畸形。1 例患者为双侧畸形，CND 是主要的异常（占我们异常病例系列的 78%），常伴有 CA 狭窄。在有 CND 的 14 名儿童中，只有 3 名 IAC 狭窄。CND 和 CA 狭窄（以及程度较小的 IAC 狭窄）是先天性和非综合征性 USNHL 的常见关联，可能代表一种影响其他健康婴儿的独特病理实体。在诊断性检查中，应在充分咨询的情况下，进行 CT 和 MRI 测量评估。这两种成像技术都已被证明有助于区分最有可能受益于人工耳蜗植入的病例，以及那些可能植入效果不佳的病例。
