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一例桥小脑角恶性病变病例报告:突显单侧进行性听力损失情况下的多学科诊断挑战

A Case Report of Malignant Cerebellopontine Angle Lesion Highlighting the Interdisciplinary Diagnostic Challenge in the Case of Unilateral Progressive Hearing Loss.

作者信息

Marzolino Riccardo, Castro Veronica, Gambacorta Valeria, Tonon Eleonora, Cattaruzzi Elisabetta, Orzan Eva

机构信息

Otorhinolaryngology and Audiology Unit, Institute for Maternal and Child Health-IRCCS "Burlo Garofolo", Via dell'Istria 63, 34137 Trieste, Italy.

Section of Otorhinolaryngology, Department of Medicine and Surgery, University of Perugia, 06129 Perugia, Italy.

出版信息

J Clin Med. 2024 Jun 14;13(12):3483. doi: 10.3390/jcm13123483.

Abstract

The authors present the case of a young boy who experienced progressive unilateral hearing loss initially believed to be unrelated to any other medical condition. The patient received a thorough evaluation, which included a comprehensive battery of audiological tests, a CT scan, and a gadolinium-enhanced MRI. A repeated imaging investigation revealed the presence of a mass that mimicked a vestibular schwannoma (VS), but despite this, the boy was ultimately diagnosed with cerebral manifestations of B-cell acute lymphoblastic leukemia (B-ALL). Cerebral lesions originating from the internal auditory canal are rare in cases of B-ALL. In this case, the initial signs and symptoms of the disease were solely related to the audiovestibular system, making the diagnostic process particularly complicated. Unilateral hearing loss cases may indicate the presence of potentially life-threatening conditions, even if the hearing loss appears to be clinically non-syndromic. For these reasons, unilateral hearing losses necessitate a comprehensive interdisciplinary diagnostic approach from the very start of auditory manifestation and, in particular, if the hearing impairment demonstrates threshold progression.

摘要

作者介绍了一名小男孩的病例,该男孩起初出现进行性单侧听力损失,最初认为与任何其他疾病无关。患者接受了全面评估,包括一系列全面的听力学测试、CT扫描和钆增强MRI。重复的影像学检查发现了一个类似前庭神经鞘瘤(VS)的肿块,但尽管如此,该男孩最终被诊断为B细胞急性淋巴细胞白血病(B-ALL)的脑部表现。B-ALL病例中起源于内耳道的脑部病变很少见。在本病例中,该疾病的初始体征和症状仅与视听前庭系统有关,使得诊断过程特别复杂。单侧听力损失病例可能表明存在潜在的危及生命的疾病,即使听力损失在临床上看似非综合征性。出于这些原因,单侧听力损失从听觉表现一开始就需要全面的跨学科诊断方法,尤其是当听力障碍显示阈值进展时。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee29/11204955/bef8ff816a16/jcm-13-03483-g001.jpg

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