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小儿患者内耳道单侧重复畸形:狭窄与不完全横向巨嵴

Unilateral Duplication of the Internal Auditory Canal: Stenosis and Incomplete Transverse Megacrest in a Pediatric Patient.

作者信息

Dang Brittany Q, Abebe Abel, Camacho Alvin

机构信息

Radiology, University of Texas Medical Branch, Galveston, USA.

出版信息

Cureus. 2024 Nov 19;16(11):e74000. doi: 10.7759/cureus.74000. eCollection 2024 Nov.

Abstract

Based on the available literature, duplicated internal auditory canals (DIACs) represent an exceedingly rare temporal bone anomaly that can result in sensorineural hearing loss (SNHL) in the pediatric population. Often associated with a hypoplastic or aplastic cochlear nerve, DIAC poses limitations on treatment options, such as cochlear implants, for affected patients. Accurate diagnosis and optimal management necessitate a thorough assessment of inner ear structures and potential neural abnormalities with high-resolution computed tomography and magnetic resonance imaging of the temporal bones. However, diagnosis of DIACs poses a significant challenge and may be underrecognized during imaging procedures due to similarities with non-duplicated stenotic cochlear abnormalities and unfamiliar characteristics. This case report describes pertinent radiologic features of DIAC in the case of a pediatric patient who presented with SNHL during late childhood and was subsequently found to have an incomplete bony megacrest within the internal auditory canal, resulting in a unilateral DIAC.

摘要

根据现有文献,重复内耳道(DIAC)是一种极其罕见的颞骨异常,可导致儿童人群出现感音神经性听力损失(SNHL)。DIAC常与发育不全或未发育的蜗神经相关,这对受影响患者的治疗选择(如人工耳蜗植入)造成了限制。准确的诊断和最佳管理需要通过颞骨的高分辨率计算机断层扫描和磁共振成像对内耳结构和潜在的神经异常进行全面评估。然而,DIAC的诊断面临重大挑战,在成像过程中可能因与非重复狭窄蜗异常相似以及特征不熟悉而未被充分认识。本病例报告描述了一名儿童患者的DIAC相关影像学特征,该患者在儿童晚期出现SNHL,随后被发现内耳道内有不完全的骨性巨嵴,导致单侧DIAC。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0dea/11658892/801c937cd069/cureus-0016-00000074000-i01.jpg

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