Patterns and Predictors of Sensorineural Hearing Loss in Children With Congenital Diaphragmatic Hernia

Although congenital diaphragmatic hernia (CDH) may be associated with sensorineural hearing loss (SNHL), to our knowledge, no studies have yet identified the pattern of this hearing loss or definitive independent risk factors that may place this population at higher risk.

To characterize the type and degree or SNHL in CDH survivors and better characterize perinatal risk factors.

This was a retrospective cohort study of CDH survivors who had a pure-tone audiogram available for review followed at a multidisciplinary clinic.

Treatment for CDH at birth.

Audiograms were used to calculate pure-tone average (PTA) for the most severely affected side and classify SNHL as normal (<20 dB), mild (20-39 dB), moderate (>40-59 dB), severe (≥60 dB), or profound (≥80 dB). Clinically significant SNHL was defined as a PTA of at least 40 dB. Perinatal data evaluated included demographics, characteristics of CDH and neonatal respiratory course, peak total and direct bilirubin levels, exposure to ototoxic drugs, need for supplemental oxygen at discharge, and results of newborn hearing screen. Univariate analysis was conducted to examine the relationship between these variables and (1) any SNHL or (2) significant SNHL.

Fifty patients were included. Audiograms were performed at age 2.7 ± 2.3 years (range, 0.5-10.7 years). Of the 50 patients, 28 (56%) had any SNHL and 9 of the 28 (32%) had significant SNHL (PTA ≥ 40 dB). Any SNHL was not significantly different (P = .42) by newborn hearing screen results: 5 of 7 children (71%) who failed the screening were classified as having any SNHL, and 20 of 40 children (50%) who passed were classified as having any SNHL. While no variables were significantly associated with any SNHL, multiple significant associations were found with significant SNHL: extracorporeal membrane oxygenation support (P = .02), nonprimary CDH repair (P = .01), prolonged ventilation (≥14 days) (P = .001), and high neonatal furosemide exposure (P = .03).

Sensorineural hearing loss is prevalent among children with CDH. As would be expected, significant SNHL (PTA ≥ 40 dB) in this cohort was associated with markers of more severe CDH with a more complicated neonatal hospital course. Overall close follow-up for hearing and speech development, including routine audiology testing, is indicated in children with CDH.