Objectives: To determine the prevalence of sensorineural hearing loss (SNHL) at the age of 4 years among survivors of severe neonatal respiratory failure with and without congenital diaphragmatic hernia and to document the occurrence of late-onset or progressive SNHL among the survivors.

Design: Prospective, longitudinal secondary outcome study.

Setting: Multicenter Canadian study in 9 tertiary referral centers.

Patients: Eighty-one (89%) of ninety 4-year-old survivors born from 1994 to 1996 of > or =34 weeks gestation at birth with severe neonatal respiratory failure (2 oxygenation indices > or =25 at least 15 minutes apart).

Main outcome measures: Repeated audiologic measurements from birth to the age of 4 years with documentation of the entire cohort at 2 and 4 years of age.

Results: Forty-three (53%) of 81 tested 4-year-old survivors had SNHL; 28 (42%) of 66 without congenital diaphragmatic hernia and 15 (100%) of 15 with congenital diaphragmatic hernia. High-frequency SNHL occurred in 65% of the patients. Of the 43 children with SNHL at 4 years, 30 (70%) had loss at 2 years, and 18 (60%) of these 30 had progressive loss between 2 and 4 years of age. For 13 children with SNHL onset after 2 years of age, the loss was less severe with lesser involvement of the lower frequencies.

Conclusion: Survivors of severe neonatal respiratory failure frequently develop late-onset SNHL that may be progressive. Urgent investigation is required to enable further understanding and prevention of this problem. Severe neonatal respiratory failure should be an indication for long-term audiologic surveillance.