Department of Neuropaediatrics, Developmental Neurology, Social Paediatrics, University Children's Hospital, Tübingen, Germany.
Department of Radiology, Section of Diagnostic and Interventional Neuroradiology, Tübingen, Germany.
Neuropediatrics. 2020 Apr;51(2):170-172. doi: 10.1055/s-0039-3399528. Epub 2019 Nov 7.
Unilateral sensorineural hearing loss is a common symptom of vestibular schwannomas in adolescent patients with neurofibromatosis type 2 or sporadic vestibular schwannomas and is often the initial clinical feature. While rare cases of sensorineural impairment presenting as vision or hearing loss due to metastatic medulloblastoma are known, hearing loss as an isolated presenting symptom of primary malignant neuroepithelial tumors of the central nervous system has not been reported in the pediatric population so far. We present two adolescents with unilateral hearing loss due to cochlear nerve dysfunction as the only symptom of a primary nonmetastatic medulloblastoma of the WNT signaling pathway family members subgroup.
单侧感觉神经性听力损失是青少年患者神经纤维瘤病 2 型或散发性前庭神经鞘瘤中前庭神经鞘瘤的常见症状，也是最初的临床特征。虽然已知由于转移性髓母细胞瘤而导致单侧感觉神经性听力或视力丧失的罕见病例，但迄今为止，在儿科人群中，听力损失作为中枢神经系统原发性恶性神经上皮肿瘤的孤立表现症状尚未有报道。我们报告了两名青少年患者，他们因耳蜗神经功能障碍导致单侧听力损失，这是 WNT 信号通路家族成员亚组原发性非转移性髓母细胞瘤的唯一症状。
