Roy Catherine F, Yang Luhe, Daniel Sam J
Faculty of Medicine, McGill University, 3605 Rue de la Montagne, Montreal, Quebec, H3G 2M1, Canada.
Department of Diagnostic Imaging, University of Saskatchewan, 107 Administration Place, S7N 5A2, Saskatoon, Saskatchewan, Canada.
Int J Pediatr Otorhinolaryngol. 2019 Nov;126:109640. doi: 10.1016/j.ijporl.2019.109640. Epub 2019 Aug 13.
Medulloblastoma is the most common pediatric malignant brain tumor and carries a relatively grim prognosis despite recent advances in multimodality therapy. Delays in diagnosis and treatment initiation may contribute to worst outcomes. Signs of increased intracranial pressure and ataxia are known presentations of posterior fossa tumors, but sensorineural hearing loss (SNHL) is a seldom reported symptom. We report the case of a 2-year-old girl who had progressive unilateral SNHL since birth, which was later found to have a posterior fossa medulloblastoma when a head magnetic resonance imaging was ordered following subsequent progression to bilateral hearing impair. We further report our review of 17 additional cases of medulloblastoma presenting with SNHL and their associated head and neck findings. The present study provides insight into the current state of the literature on this rare symptom of a commonly encountered diagnosis, while highlighting the need to consider dedicated brain imaging in pediatric unilateral SNHL when a syndromic etiology or inner ear anomaly is not readily identified on initial investigations.
髓母细胞瘤是最常见的儿童恶性脑肿瘤，尽管多模态治疗最近取得了进展，但其预后相对严峻。诊断和治疗开始的延迟可能导致更差的结果。颅内压升高和共济失调的体征是后颅窝肿瘤的已知表现，但感音神经性听力损失（SNHL）是一种很少报道的症状。我们报告了一名2岁女孩的病例，她自出生以来患有进行性单侧SNHL，在随后进展为双侧听力损害后进行头部磁共振成像检查时，后来发现患有后颅窝髓母细胞瘤。我们进一步报告了对另外17例表现为SNHL的髓母细胞瘤病例及其相关头颈部检查结果的回顾。本研究深入了解了关于这种常见诊断的罕见症状的当前文献状况，同时强调在初步检查中未轻易发现综合征病因或内耳异常时，对于儿童单侧SNHL需要考虑进行专门的脑部成像检查。
