Partridge Emily A, Bridge Christina, Donaher Joseph G, Herkert Lisa M, Grill Elena, Danzer Enrico, Gerdes Marsha, Hoffman Casey H, D'Agostino Jo Ann, Bernbaum Judy C, Rintoul Natalie E, Peranteau William H, Flake Alan W, Adzick N Scott, Hedrick Holly L
The Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
The Center for Childhood Communication, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
J Pediatr Surg. 2014 Jun;49(6):890-4; discussion 894. doi: 10.1016/j.jpedsurg.2014.01.019. Epub 2014 Jan 31.
The reported incidence of sensorineural hearing loss (SNHL) in long-term survivors of congenital diaphragmatic hernia varies widely in the literature. Conductive hearing loss (CHL) is also known to occur in CDH patients, but has been less widely studied. We sought to characterize the incidence and risk factors associated with SNHL and CHL in a large cohort of CDH patients who underwent standardized treatment and follow-up at a single institution.
We retrospectively reviewed charts of all CDH patients in our pulmonary hypoplasia program from January 2004 through December 2012. Categorical variables were analyzed by Fisher's exact test and continuous variables by Mann-Whitney t-test (p≤0.05).
A total of 112 patients met study inclusion criteria, with 3 (2.7%) patients diagnosed with SNHL and 38 (34.0%) diagnosed with CHL. SNHL was significantly associated with requirement for ECMO (p=0.0130), prolonged course of hospitalization (p=0.0011), duration of mechanical ventilation (p=0.0046), requirement for tracheostomy (p=0.0013), and duration of loop diuretic (p=0.0005) and aminoglycoside therapy (p=0.0003).
We have identified hearing anomalies in over 30% of long-term CDH survivors. These findings illustrate the need for routine serial audiologic evaluations throughout childhood for all survivors of CDH and stress the importance of targeted interventions to optimize long-term developmental outcomes pertaining to speech and language.
先天性膈疝长期存活者中感音神经性听力损失(SNHL)的报告发病率在文献中差异很大。传导性听力损失(CHL)在先天性膈疝(CDH)患者中也有发生,但研究较少。我们试图在一个在单一机构接受标准化治疗和随访的大型先天性膈疝患者队列中,描述SNHL和CHL的发病率及相关危险因素。
我们回顾性分析了2004年1月至2012年12月在我们肺发育不全项目中的所有先天性膈疝患者的病历。分类变量采用Fisher精确检验分析,连续变量采用Mann-Whitney t检验(p≤0.05)。
共有112例患者符合研究纳入标准,其中3例(2.7%)被诊断为SNHL,38例(34.0%)被诊断为CHL。SNHL与体外膜肺氧合(ECMO)的需求(p = 0.0130)、住院时间延长(p = 0.0011)、机械通气时间(p = 0.0046)、气管切开术的需求(p = 0.0013)以及襻利尿剂使用时间(p = 0.0005)和氨基糖苷类药物治疗时间(p = 0.0003)显著相关。
我们在超过30%的先天性膈疝长期存活者中发现了听力异常。这些发现表明,对于所有先天性膈疝幸存者,在儿童期进行常规系列听力评估的必要性,并强调了针对性干预对优化与言语和语言相关的长期发育结局的重要性。