Spiral ganglion deficiency in adult-onset deafness-dystonia syndrome.
Spiral ganglion deficiency in adult-onset deafness-dystonia syndrome.
复制标题
成人发病的耳聋肌张力障碍综合征中的螺旋神经节缺陷。
DOI：
10.1097/mao.0b013e3182a09b3b
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发表时间：
2013
期刊：
影响因子：
--
通讯作者：
LinthicumJr,FredH
中科院分区：
文献类型：
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作者：
Hoa,Michael;LinthicumJr,FredH
癫痫-肌张力障碍综合征患者可能表现为成人型耳聋，并伴有听神经病样图像（1）。我们的患者表现为成人发作的双侧深度SNHL，螺旋神经节细胞几乎完全丧失，耳蜗和前庭神经结构严重退化，尽管耳蜗Corti结构正常。此外，患者的肌张力障碍症状为轻度和节段性。之前有报道称，DDON患者的人工耳蜗植入性能很差（4）。有类似成人发病听神经病样表现的成人患者可能具有类似的组织学图像。在成人听神经病样图像的鉴别诊断中，应考虑耳聋-肌张力障碍综合征。此外，应考虑这些患者的潜在边缘人工耳蜗植入性能，但不应阻止人工耳蜗植入的尝试。边缘人工耳蜗植入性能，在未来，提示考虑听觉脑干植入在这一人群的患者。
DISCUSSIONPatient with deafness-dystonia syndrome may present with adult-onset deafness with an auditory neuropathylike picture (1). Our patient presented with adult-onset bilateral profound SNHL and exhibited near-total loss of spiral ganglion cells and severely degenerated architecture of the cochlear and vestibular nerves, despite normal-appearing cochlear organ of Corti architecture. Furthermore, the patient’s dystonia symptoms were mild and segmental. It has been previously reported that cochlear implant performance in DDON is marginal (4). It is possible that adult patients with a similar an adult-onset auditory neuropathy-like presentation may possess a similar histologic picture. Deafness-dystonia syndromes should be considered in the differential diagnosis of adults presenting with an auditory neuropathy-like picture. Furthermore, consideration of the potential marginal cochlear implant performance in these patients should be considered but should not deter attempts at cochlear implantation. Marginal cochlear implant performance may, in the future, prompt consideration of auditory brainstem implantation in this population of patients.