title: Prognostic value of newborn hearing screening in patients with myelomeningocele.
abstract: OBJECTIVE
  Brainstem dysfunction occurs in a minority of patients with myelomeningocele (MMC), most of whom have Chiari Type 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
  The charts of 40 newborns with MMC and 50 newborns without MMC who stayed in the neonatal intensive care unit were reviewed. Results of NHS, brainstem symptoms, birth demographics, and surgical history were retrospectively examined. Differences in the presence and onset of brainstem symptoms by NHS result were assessed.
  
  
  RESULTS
  Failure of NHS was more common among newborns with MMC who developed brainstem symptoms (31%, 4 of 13 patients) than among newborns without MMC (0%, 0 of 50 patients; p = 0.001). Among the 40 newborns with MMC, brainstem symptoms were more common in those who failed NHS (80%, 4 of 5 patients) than in those who passed (26%, 9 of 35 patients; p = 0.031). Respiratory symptom onset occurred later in patients who failed NHS (median 16 months) than among those who passed (median 0 months; p = 0.022). The positive and negative predictive values of NHS for brainstem dysfunction in MMC were 0.80 and 0.74, respectively.
  
  
  CONCLUSIONS
  Results of NHS may help predict future brainstem dysfunction in patients with MMC and may be useful to incorporate into prognostic assessment and surgical decision making.
authors.affiliation: Department of Neurosurgery, University of Minnesota, Minneapolis, Minnesota.
authors.forename: David
authors.initials: D
authors.lastname: Satzer
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Guillaume
mesh_terms: D001139:Arnold-Chiari Malformation / Q000503:physiopathology*; D001933:Brain Stem / Q000503:physiopathology*; D016057:Evoked Potentials, Auditory, Brain Stem; D005260:Female; D006311:Hearing Disorders / Q000175:diagnosis* / Q000209:etiology; D006320:Hearing Tests; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008499:Medical Records; D008591:Meningomyelocele / Q000150:complications*; D015997:Neonatal Screening / Q000379:methods; D017084:Otoacoustic Emissions, Spontaneous; D011237:Predictive Value of Tests; D011379:Prognosis; D012189:Retrospective Studies
keywords: ABR = auditory brainstem response; EMR = electronic medical record; IPL = interpeak latency; MMC = myelomeningocele; NHS = newborn hearing screening; NICU = neonatal intensive care unit; OAE = otoacoustic emissions; brainstem dysfunction; congenital; myelomeningocele; newborn hearing screening