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

Abstract

IMPORTANCE 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. OBJECTIVE To characterize the type and degree or SNHL in CDH survivors and better characterize perinatal risk factors. DESIGN, SETTING, AND PARTICIPANTSThis was a retrospective cohort study of CDH survivors who had a pure-tone audiogram available for review followed at a multidisciplinary clinic. INTERVENTIONS Treatment for CDH at birth.MAIN OUTCOMES AND MEASURES 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 (Ն60 dB), or profound (Ն80 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. RESULTS 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 Ն 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 (Ն14 days) (P = .001), and high neonatal furosemide exposure (P = .03).CONCLUSIONS AND RELEVANCE Sensorineural hearing loss is prevalent among children with CDH. As would be expected, significant SNHL (PTA Ն 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. associated with significant hearing loss. These included ECMO support (P = .02), nonprimary CDH repair (P = .01), prolonged ventilation (≥14 days) (P = .001), and high neonatal furosemide exposure (top quartile) (P = .03).

Audiological screening of high risk infants and prevalence of risk factors

“…Hearing impairment in these babies could be due to fetal hypoxia caused by these comorbid conditions which can lead to damage to cochlear cells and neuronal pathways. 16,20,21 In our study, 15.78% (n = 6) of hearing impaired babies were having meningitis / culture positive sepsis. ALHarbi M et al, KY Chan et al and Meyer A et al concluded that bacterial meningitis is significant factor associated with failed ABR in new born screening, probably due to vasculitis of inner ear or adhesions comprising eighth nerve.…”

Congenital diaphramatic hernia outcomes in East Africa: The Ethiopian experience

“…One of the patients with DS was noted to have Sensorineural hearing loss (SNHL) at birth. Though SNHL has a known association with CDH and is often diagnosed in the toddler years 7 , audiograms are not routinely performed at our institution Survival rates among CDH patients vary widely from institution to institution, but 80% is the most often quoted number for patients who are cared for at a tertiary referral center 8 , which is consistent with our overall survival rate of 83.3%, which is most likely slightly higher than the world average as most of our patients are late presenters. The clear majority of the literature comes from institutions with access to Extra Corporal Membrane Oxygenation (ECMO), which is not available in our setting.…”

Defining outcomes following congenital diaphragmatic hernia using standardised clinical assessment and management plan (SCAMP) methodology within the CDH EURO consortium

“…73 Although earlier studies tend to present a higher prevalence of SNHL, Amoils and coworkers report a prevalence of SNHL over 50% in 2015. 74 Controversies exist on the impact of the diagnosis of CDH on the risk of SNHL development. In a study on 111 ECMO graduates, Fligor and coworkers reported a 26% overall prevalence of SNHL in neonates with severe respiratory distress and described CDH as an independent risk factor.…”

“…Most studies with data from neonatal hearing screening, report normal findings. 73,74,[77][78][79][80][81] Therefore, the extreme variability in length of follow-up in available reports, precludes firm conclusions on the actual prevalence.…”

“…The most frequently reported factors associated with SNHL are ECMO treatment, 74,75,82,83 length of mechanical ventilation and/or stay in the NICU or in hospital, 74,78,79,[83][84][85] need for inhaled nitric oxide, 84 patch repair, 74 and dose and duration of specified drugs: loop diuretics, 74,78,[82][83][84] aminoglycosides, 75,83,84 and pancuronium bromide. 78,84 Overall, these factors suggest that the most critically ill CDH patients are at greatest risk.…”