Progressive sensorineural hearing loss in children with congenital diaphragmatic hernias.

Abstract

T he increased prevalence of sensorineural hearing loss (SNHL) in survivors of congenital diaphragmatic hernia (CDH) has been recognized in the literature for the past 10 years. Previously reported rates have varied from 18 to 60%. The onset of hearing loss is most often delayed, and the nature of the hearing loss is progressive. Although the delay of onset of SNHL in these children is well documented, very little is known about the approximate age at onset. In a previous study of hearing loss after severe neonatal respiratory failure, children were followed from birth to 4 years of age. All survivors of CDH who had SNHL by 4 years of age were already diagnosed by 2 years of age, suggesting that, for CDH survivors destined to develop hearing loss, the onset is prior to age 2. It is also well recognized that the hearing loss in these children has a progressive nature. However, the rate and characteristics of this progression have not been well studied to date. Several possible etiologies of SNHL in these children have been explored. These include the various treatments for CDH and the inherent hypoxia experienced by children in respiratory distress and failure at birth. Previous studies have focused on the effects of such treatments as extracorporeal membrane oxygenation, high-frequency oscillatory (HFO) therapy, pancuronium bromide, ototoxic drugs including loop diuretics and aminoglycosides, and inhaled nitric oxide. However, to date, no definite etiology for SNHL in survivors of CDH has been discovered. It is hypothesized that a multifactorial etiology exists. The purpose of this study was to determine the approximate age at onset of SNHL in survivors of CDH, characterize its progression, and examine a possible correlation between various modes of ventilation used for the treatment and development of SNHL later in life.