Division of Otolaryngology-Head and Neck Surgery, The George Washington University, Washington DC.
Otol Neurotol. 2021 Sep 1;42(8):e1056-e1057. doi: 10.1097/MAO.0000000000003184.
To present a case of progressive sensorineural hearing loss (SNHL) caused by labyrinthine erosion secondary to expanding geniculate ganglion cerebrospinal fluid (CSF) diverticulum extending along the path of the facial nerve.
Thirteen-year-old man with no past medical history or risk factors presented with unilateral progressive SNHL and no other otologic or neurologic symptoms.
Audiological data as well as imaging studies including a magnetic resonance imaging (MRI) of the brain and internal auditory canal and a temporal bone computed tomography (CT) are presented. Partial labyrinthectomy, CSF leak closure, and cochlear implantation were performed. A lumbar puncture was obtained 2 months postoperatively.
Closure of the CSF leak.
Mastoidectomy revealed a wide area of CSF leak through the geniculate ganglion and fallopian canal with partial destruction of the superior and lateral semicircular canals by an expanding diverticulum.
This is an unusual presentation of progressive SNHL due to partial labyrinthine erosion due to a meningocele of the geniculate ganglion along the fallopian canal. It is likely that the patient had a congenital open pathway through the labyrinthine segment of the facial nerve and CSF pulsations created the meningocele over a few years. This case argues for early closure of such CSF diverticula to prevent possible SNHL due to labyrinthine erosion.
介绍一例由沿面神经迷路段延伸的扩大膝状神经节脑脊髓液（CSF）憩室引起的进行性感音神经性听力损失（SNHL）的病例。
一名 13 岁男性，无既往病史或危险因素，表现为单侧进行性 SNHL，无其他耳科或神经科症状。
呈现了听力数据以及影像学研究，包括脑和内听道磁共振成像（MRI）以及颞骨计算机断层扫描（CT）。进行了部分迷路切除术、CSF 漏封闭和耳蜗植入术。术后 2 个月进行了腰椎穿刺。
CSF 漏的闭合。
乳突切除术显示广泛的 CSF 漏通过膝状神经节和输卵管，扩大的憩室使上半规管和外侧半规管部分破坏。
这是一例由于膝状神经节脑膜膨出沿着输卵管导致部分迷路侵蚀引起的进行性 SNHL 的不常见表现。患者可能存在面神经迷路段先天性开放通路，CSF 搏动在数年内形成了脑膜膨出。该病例表明，应尽早封闭此类 CSF 憩室，以防止可能因迷路侵蚀导致的 SNHL。
