先天性颞骨胆脂瘤的诊断和治疗

Diagnosis and treatment of congenital temporal bone cholesteatoma

先天性颞骨胆脂瘤是耳鼻喉科的罕见疾病,具有侵袭性,可能导致包括听力损失(传导性或感音神经性)、颞骨破坏以及颅内侵犯等严重并发症。本文回顾了先天性颞骨胆脂瘤的临床表现、影像学特征、分期和目前的治疗方案,以期提高认识,及时进行手术治疗,避免胆脂瘤对于颞骨及周围结构的破坏,从而减少并发症。

Congenital temporal bone cholesteatoma is a rare lesion in otolaryngology.The disease is locally invasive and may lead to significant complications, including hearing loss(conductive or sensorineural), temporal bone destruction and intracranial invasion. This article reviews the characteristic symptoms of congenital temporal bone cholesteatoma, testing and imaging of the disease, stage and the current treatment options in order to promote awareness to this rare disease entity and perform early surgical treatment, effectively avoid the destruction of the temporal bone and its surrounding structures, thereby reducing the occurrence of complications. By improving the understanding of the disease and performing early surgical treatment, the destruction of the temporal bone and its surrounding structures can be effectively avoided, thereby reducing the occurrence of complications.