J Neurosurg: Pediatrics / Volume 14 / November 2014 J Neurosurg Pediatrics 14:495–500, 2014 495 ©AANS, 2014 B rainstem dysfunction is the most common cause of death in newborns with myelomeningocele (MMC). 9 Most newborns with MMC have associ- ated Chiari Type II malformation. Brainstem dysfunction occurs in 20% of patients with MMC 23 and is tradition- ally considered to arise from brainstem dysgenesis in Chi- ari Type II malformation and resultant mechanical stress on the brainstem and cranial nerves. 7,24 However, similar symptoms have been reported in MMC in the absence of Chiari Type II malformation. 20 Surgical treatment of brain- stem dysfunction in these patients is controversial. 2,15,23 Some surgeons advocate early craniocervical decompres- sion in patients who become symptomatic, 23,25,26,31 par- ticularly because of the poor prognosis for children who develop bilateral vocal cord paralysis. 22,34 Unfortunately, prediction of symptom development remains diffcult. Past researchers have investigated the utility of audi- tory function testing for predicting symptom development in children with MMC. Most patients with MMC have abnormal results on auditory brainstem response (ABR) testing, with prolonged interpeak latencies (IPLs) 1,14,33 re- fecting slow peripheral 12,18,21 and central 5,6,18,19,21 auditory neurotransmission. Normalization of central auditory function over time, coupled with progressively worsening peripheral auditory function, suggests intrinsic brainstem improvement with simultaneous cranial nerve stretch- ing. 18,21 Some authors have had success correlating ABR results with symptoms, 14,30,33 though not in older children. 5 Case reports describe ABR improvement after craniocer- vical decompression. 10,29 Automated ABR testing is commonly used in the uni- versal newborn hearing screening (NHS) programs that were introduced throughout the United States in the mid- to late 1990s. 28 These programs were designed to identify hearing loss early by screening newborns before hospital discharge. Most studies of auditory impairment in MMC were published before the advent of universal NHS, and no prior research has examined whether NHS failure predicts development of brainstem dysfunction in these patients. Prognostic value of newborn hearing screening in patients with myelomeningocele Clinical article DaviD Satzer, B.a., anD Daniel J. Guillaume, m.D. Department of Neurosurgery, University of Minnesota, Minneapolis, Minnesota Object. Brainstem dysfunction occurs in a minority of patients with myelomeningocele (MMC), most of whom have Chiari Type II malformation. Some surgeons advocate early identifcation of these patients for craniocervical decompression to avoid signifcant 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 new- borns 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. (http://thejns.org/doi/abs/10.3171/2014.7.PEDS14168) Key WorDS • newborn hearing screening • myelomeningocele • congenital • brainstem dysfunction Abbreviations used in this paper: ABR = auditory brainstem response; EMR = electronic medical record; IPL = interpeak laten- cy; MMC = myelomeningocele; NHS = newborn hearing screening; NICU = neonatal intensive care unit; OAE = otoacoustic emissions.