UCL Ear Institute, London, UK.
Otol Neurotol. 2012 Feb;33(2):142-6. doi: 10.1097/MAO.0b013e318241c3bd.
Hearing loss in Paget's disease of bone (PDB) is typically mixed and bilateral. Although different mechanisms have been proposed, the pathophysiology of hearing impairment remains unclear.
The purpose of this study is to describe the histopathologic findings of temporal bones in patients with PDB and elucidate possible pathologic mechanisms related to hearing impairment.
This is an archival human temporal bone study of 8 subjects diagnosed with Paget's disease from the temporal bone collection of the UCL Ear Institute.
A fractured stapes footplate was observed in 1 temporal bone and stapes footplate fixation in 2 other specimens. Obliteration of Cotugno's canal by extensive bone remodeling was observed in 78% of temporal bones. An intracochlear vestibular schwannoma was observed in 1 specimen (previously reported in the literature). Other findings include microfissures and microfractures of the otic capsule, bleeding in the scalae, strial atrophy, and cystic lesions in the spiral ligament.
This study is the first to report a fractured stapes footplate, as a causative lesion of conductive hearing loss in PDB. Extensive bone remodeling around Cotugno canal also was a frequent finding, not reported before in the literature. We hypothesize that sensorineural hearing loss in patients with PDB of the temporal bone may, in some cases, be attributed to obliteration of Cotugno's canal by remodeling pagetoid bone, thus obstructing the venous drainage of the cochlea, with a subsequent effect on the function of stria vascularis and spiral ligament. This seems to be consistent with experimental studies in animals.
Paget 骨病（PDB）导致的听力损失通常为混合性和双侧性。尽管提出了不同的机制，但听力损伤的病理生理学仍不清楚。
本研究旨在描述 PDB 患者颞骨的组织病理学发现，并阐明与听力损伤相关的可能病理机制。
这是一项对 UCL 耳研究所颞骨库中 8 例 Paget 病患者的颞骨进行的存档人体颞骨研究。
在 1 例颞骨中观察到镫骨足板骨折，在另外 2 例标本中观察到镫骨足板固定。78%的颞骨观察到 Cotugno 管被广泛骨重塑所闭塞。1 例标本中观察到耳蜗内前庭神经鞘瘤（以前在文献中报道过）。其他发现包括耳囊的微裂缝和微骨折、scala 内出血、嵴萎缩和螺旋韧带囊性病变。
本研究首次报道了 PDB 中镫骨足板骨折这一导致传导性听力损失的病因病变。广泛的 Cotugno 管周围骨重塑也是一个常见的发现，以前的文献中没有报道过。我们假设，PDB 患者颞骨的感音神经性听力损失，在某些情况下，可能归因于 Pagetic 骨重塑导致的 Cotugno 管闭塞，从而阻塞耳蜗的静脉引流，随后影响血管纹和螺旋韧带的功能。这似乎与动物实验研究一致。
