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Paeds Vivasfetal-neonatal-and-perinatal

Paeds Vivas · fetal-neonatal-and-perinatal

Hearing loss in high-risk neonates — structured oral (viva)

Branching structured oral on a NICU infant who refers on newborn hearing screening, testing the OAE-versus-AABR rationale, the JCIH risk indicators, congenital CMV, and the early-intervention pathway.

branching clinical structured oral3 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Study tools

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A 29-week-gestation infant spent 54 days in the neonatal intensive care unit for respiratory distress syndrome, received two courses of gentamicin and furosemide, and required a partial exchange transfusion for severe hyperbilirubinaemia. Before discharge the infant refers on automated auditory brainstem response screening in both ears. The parents are anxious and ask what this means.

Branch 1 — Screening rationale

Examiner: "Why was this infant screened with AABR rather than otoacoustic emissions?" Candidate: Automated auditory brainstem response was used because the NICU population carries the highest burden of auditory neuropathy spectrum disorder. Otoacoustic emissions test only the cochlear outer hair cells and return a normal result in auditory neuropathy, where the cochlea is intact but the nerve or brainstem misfires. AABR tests the entire pathway from sound to brainstem and therefore detects auditory neuropathy, which is why it is the mandated screen for the NICU infant. [1] [2]

Examiner: "How do you interpret a bilateral refer?" Candidate: A refer means the screen did not detect a clear brainstem response in either ear. It is not a diagnosis of hearing loss — a proportion of refers reflect transient causes such as middle-ear fluid or ambient noise. The protocol is to repeat the screen once, and if it refers again, to refer for a full diagnostic assessment. The priority is to ensure the infant is not lost to follow-up, because loss to follow-up is the commonest failure of the pathway. [1]

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References4ShowHide
  1. [1]American Academy of Pediatrics, Joint Committee on Infant Hearing Year 2007 position statement: Principles and guidelines for early hearing detection and intervention programs. Pediatrics, 2007.PMID 17908777
  2. [2]Vohr BR, Widen JE, Cone-Wesson B, Sininger YS, Gorga MP, Folsom RC, Norton SJ Identification of neonatal hearing impairment: characteristics of infants in the neonatal intensive care unit and well-baby nursery. Ear Hear, 2000.PMID 11059699
  3. [3]Goderis J, De Leenheer E, Smets K, Van Hoecke H, Keymeulen A, Dhooge I Hearing loss and congenital CMV infection: a systematic review. Pediatrics, 2014.PMID 25349318
  4. [4]Morlet T, Parkes W, Pritchett C, Venskytis E, DeVore B, O'Reilly RC A 15-Year Review of 260 Children With Auditory Neuropathy Spectrum Disorder: I. Demographic and Diagnostic Characteristics. Ear Hear, 2023.PMID 37036288
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