Vanderbilt University School of Medicine, Nashville, Tennessee.
Department of Otolaryngology-Head and Neck Surgery, San Antonio Uniformed Services Health Education Consortium, San Antonio, Texas.
Otol Neurotol. 2020 Apr;41(4):489-493. doi: 10.1097/MAO.0000000000002554.
Several reports have suggested that the incidence of otosclerosis is declining. However, due to the limitations of epidemiologic databases within the United States, little is known about the incidence of congenital stapes footplate fixation (CSFF), much less epidemiological trends over the past 50 years. The current study was conceived to characterize the epidemiology of CSFF over the past five decades using the unique resources of the Rochester Epidemiology Project (REP).
Population-based study.
The medical records of every resident of Olmsted County, Minnesota who was diagnosed with CSFF was reviewed from January 1, 1970 to December 12, 2017 using the REP. The population of Olmsted County on January 1, 2017 was 159,689 persons.
Incidence of CSFF.
Sixteen cases of CSFF were identified at a median age at diagnosis of 8 years (IQR, 6-17), eight of whom confirmed by exploratory tympanotomy. Three of the 16 (19%) cases were bilateral, and the median pure-tone average (500, 1k, 2k, 3k Hz) at time of diagnosis was 37 dB (IQR, 31-48). Two (13%) cases were identified in patients bearing a syndromic condition and one (6%) case demonstrated a malformed external ear on physical examination. The incidence rate of surgically confirmed cases among residents of Olmsted County was 0.31 per 100,000 person-years from 1970 to 1979 and 0.28 from 2000 to 2009. No incident cases among residents of Olmsted County were identified since 2010.
CSFF constitutes a rare disease. Unlike epidemiological trends in otosclerosis, the incidence of surgically confirmed cases of CSFF remained stable over the past 50 years.
有几份报告表明耳硬化症的发病率正在下降。然而，由于美国流行病学数据库的局限性，人们对先天性镫骨底板固定（CSFF）的发病率知之甚少，更不用说过去 50 年来的流行病学趋势了。本研究旨在利用罗切斯特流行病学项目（REP）的独特资源，描述过去五十年 CSFF 的流行病学特征。
基于人群的研究。
通过使用 REP，对 1970 年 1 月 1 日至 2017 年 12 月 12 日期间明尼苏达州奥姆斯特德县的每一位被诊断为 CSFF 的居民的医疗记录进行了回顾。2017 年 1 月 1 日，奥姆斯特德县的人口为 159689 人。
CSFF 的发病率。
在中位诊断年龄为 8 岁（IQR，6-17）时，共发现 16 例 CSFF 病例，其中 8 例经探查鼓室切开术确诊。16 例中有 3 例（19%）为双侧，确诊时纯音平均听阈（500、1k、2k、3k Hz）为 37 dB（IQR，31-48）。2 例（13%）患者患有综合征，1 例（6%）患者体格检查发现外耳畸形。奥姆斯特德县居民经手术确诊的病例发病率在 1970 年至 1979 年期间为 0.31/100000 人年，在 2000 年至 2009 年期间为 0.28/100000 人年。自 2010 年以来，奥姆斯特德县居民中未发现新发病例。
CSFF 是一种罕见疾病。与耳硬化症的流行病学趋势不同，过去 50 年来，经手术确诊的 CSFF 发病率保持稳定。
