Levy C E, Lash A T, Janoff H B, Kaplan F S
Ohio State University, Columbus, USA.
Am J Audiol. 1999 Jun;8(1):29-33. doi: 10.1044/1059-0889(1999/011).
Fibrodysplasia ossificans progressiva (FOP) is a very rare genetic disorder that is characterized by progressive heterotopic ossification of soft tissues and congenital malformation of the great toes. Although previous case studies have reported hearing loss in individuals with FOP, there have been no large-scale studies regarding the nature or cause of the hearing loss. Here, we report the findings of a two-part study. In Part I, we report the findings of a postal survey regarding hearing loss that was sent to 102 individuals with FOP. In Part II, we report the findings of on-site hearing evaluations of eight individuals with FOP. The findings of both studies indicate that individuals with FOP are at risk for hearing loss and that the type of loss is predominantly conductive in nature, similar to that seen in individuals who have otosclerosis.
进行性骨化性纤维发育不良（FOP）是一种非常罕见的遗传性疾病，其特征是软组织进行性异位骨化和大脚趾先天性畸形。尽管先前的病例研究报告了FOP患者存在听力损失，但尚未有关于听力损失的性质或原因的大规模研究。在此，我们报告一项分为两部分的研究结果。在第一部分中，我们报告了一项针对102名FOP患者的关于听力损失的邮政调查结果。在第二部分中，我们报告了对8名FOP患者进行现场听力评估的结果。两项研究的结果均表明，FOP患者有听力损失风险，且损失类型主要为传导性，类似于耳硬化症患者的情况。
