Introduction: Brainstem dysfunction occurs in a minority of patients with myelomeningocele (MMC), most of whom have Chiari II malformation. Some surgeons advocate early identification of these patients for craniocervical decompression to avoid significant mortality. The auditory brainstem response has been found to be abnormal in most children with MMC. The present study examines whether failure of routine newborn hearing screening (NHS) predicts brainstem dysfunction in MMC patients.
Methods: We reviewed the charts of 40 newborns with MMC and 50 newborns without MMC who stayed in the neonatal intensive care unit. We retrospectively examined NHS and brainstem symptom data, as well as birth demographics and surgical history. Differences in the presence and onset of brainstem symptoms by NHS result were assessed.
Results: NHS failure was more common among newborns with MMC who developed brainstem symptoms (31%, 4/13 patients) than newborns without MMC (0%, 0/50 patients; P=0.001). Among the 40 newborns with MMC, brainstem symptoms were more common in those who failed NHS (80%, 4/5 patients) than those who passed (26%, 9/35 patients; P=0.031). Respiratory symptom onset occurred later in patients who failed NHS (median 16 mo) than those who passed (median 0 mo; P=0.022). The positive and negative predictive values of NHS for brainstem dysfunction in MMC were 0.80 and 0.74, respectively.
Conclusions: NHS may be a useful tool for predicting future brainstem dysfunction in patients with MMC. Screening results may be incorporated into prognostic assessment and surgical decision-making.
Patient Care: NHS may be a useful tool for early identification of newborns with MMC who are at risk for brainstem dysfunction. Screening results may be considered during prognostic and surgical evaluation of these patients in order to reduce mortality from respiratory failure and other complications of brainstem dysfunction.
Learning Objectives: By the conclusion of this session, participants should understand (1) the rationale for early detection of brainstem dysfunction in MMC, (2) past studies of auditory brainstem response testing for brainstem dysfunction, and (3) our evidence for NHS as a predictor of brainstem dysfunction.