Department of Otorhinolaryngology, Head and Neck Surgery, Keio University Hospital, Tokyo, Japan.
Medicine (Baltimore). 2023 Nov 24;102(47):e36124. doi: 10.1097/MD.0000000000036124.
Hypertrophic pachymeningitis (HP) is a local or diffuse fibrous thickness of the dura mater of the brain or spinal cord, caused by infection or connective tissue disease. Headache is the most common clinical symptom, followed by various cranial nerve disorders such as visual impairment, diplopia, and hearing loss. HP can be classified into secondary and idiopathic. Here, we report a case of bilateral progressive profound sensorineural hearing loss diagnosed in a patient with idiopathic HP, where a cochlear implant was effectively used.
The patient was a 77-year-old woman. Hearing loss gradually progressed bilaterally, and magnetic resonance imaging showed a space-occupying lesion with a continuous contrast enhancement in the bilateral internal auditory canals, and diffused dural thickening from the middle to the posterior cranial fossa.
A trans-labyrinthine biopsy was conducted, and a definite diagnosis of idiopathic HP was made. Thickening of the dura mater in the bilateral internal auditory canals was thought to cause profound hearing loss.
A cochlear implant was implemented 4 months after biopsy, and a favorable hearing response was obtained postoperatively.
This is the first report of a cochlear implant in a patient with idiopathic HP. Cochlear implantation was considered a good treatment for profound hearing loss due to idiopathic HP, which provides a reference for patients to receive timely and correct treatment.
肥厚性硬脑膜炎（HP）是一种脑或脊髓硬脑膜的局部或弥漫性纤维性增厚，由感染或结缔组织疾病引起。头痛是最常见的临床症状，其次是各种颅神经障碍，如视力障碍、复视和听力损失。HP 可分为继发性和特发性。在这里，我们报告了一例特发性 HP 患者双侧进行性深度感觉神经性听力损失的病例，该患者成功使用了人工耳蜗。
患者为 77 岁女性，双侧听力逐渐下降，磁共振成像显示双侧内听道占位性病变，连续对比增强，从中颅窝到后颅窝弥漫性硬脑膜增厚。
进行经迷路活检，明确诊断为特发性 HP。双侧内听道硬脑膜增厚被认为导致了深度听力损失。
活检后 4 个月植入人工耳蜗，术后获得良好的听力反应。
这是首例特发性 HP 患者人工耳蜗植入的报告。人工耳蜗植入被认为是治疗特发性 HP 所致深度听力损失的一种良好治疗方法，为患者及时接受正确治疗提供了参考。
