Mosnier Isabelle, Rondini-Gilli E, Crosara Paulo Tormin Borges, Belmatoug N, Cyna-Gorse Françoise, Cazals-Hatem D, Abbey-Toby A, Bozorg-Grayeli A, Sterkers Olivier
Department of Otolaryngology, Hôpital Louis Mourier, AP-HP, Faculté Xavier Bichat, Université Parist, Colombes, France.
Otol Neurotol. 2004 Jan;25(1):27-32. doi: 10.1097/00129492-200401000-00006.
To describe and analyze three unusual cases of Langerhans' cell histiocytosis of the temporal bone in adults.
Retrospective case review.
A tertiary referral center.
Three adult patients with progressive sensorineural or mixed hearing loss, vertigo, and tinnitus as presenting symptoms of Langerhans' cell histiocytosis of the temporal bone.
Patients were evaluated by means of computed tomography and magnetic resonance imaging. All patients underwent complete surgical excision of the lesion via a transmastoid approach, extended to a translabyrinthine approach in one case. One patient with a multifocal disease underwent excision of a mandibular lesion 1 year later, followed-up by chemotherapy.
The Langerhans' cell histiocytosis was located adjacent to or within the area of the endolymphatic sac region in all cases. The middle ear was spared. Hearing function was preserved in the two patients who had serviceable hearing preoperatively. No complication occurred.
Langerhans' cell histiocytosis of the temporal bone is centered on or adjacent to the endolymphatic sac. The fact that the endolymphatic sac plays an immunologic role in the inner ear suggests that the infiltration of the Langerhans' cell may derive from it.
描述并分析三例成人颞骨朗格汉斯细胞组织细胞增多症的罕见病例。
回顾性病例分析。
三级转诊中心。
三名成年患者，以进行性感音神经性或混合性听力损失、眩晕和耳鸣为颞骨朗格汉斯细胞组织细胞增多症的主要症状。
通过计算机断层扫描和磁共振成像对患者进行评估。所有患者均通过经乳突入路对病变进行完整手术切除，其中一例扩展至经迷路入路。一名患有多灶性疾病的患者在1年后接受了下颌骨病变切除，随后进行化疗。
所有病例中，朗格汉斯细胞组织细胞增多症均位于内淋巴囊区域附近或该区域内。中耳未受累。术前听力尚可的两名患者听力功能得以保留。未发生并发症。
颞骨朗格汉斯细胞组织细胞增多症以内淋巴囊为中心或位于其附近。内淋巴囊在内耳中发挥免疫作用这一事实表明，朗格汉斯细胞的浸润可能源于此。
