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Paeds Casesfetal-neonatal-and-perinatal

Paeds Cases · fetal-neonatal-and-perinatal

Hearing loss in high-risk neonates — clinical case

Clinical case of an extremely preterm NICU graduate who refers on AABR screening and is found to have auditory neuropathy spectrum disorder, illustrating the screening rationale, diagnostic confirmation, and early-intervention pathway.

neonatal long case2 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalABP General Pediatrics
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Target exams

RACP DCEMRCPCH ClinicalABP General Pediatrics
Prompt
A 27-week-gestation girl, birth weight 880 g, spent 72 days in the neonatal intensive care unit for respiratory distress syndrome, receiving two courses of gentamicin and furosemide and undergoing a double-volume exchange transfusion at peak bilirubin of 380 micromoles per litre. Before discharge she refers on automated auditory brainstem response screening in both ears. The parents ask whether their daughter is deaf.

Case summary

This extremely preterm infant carries a heavy stack of JCIH risk indicators — prolonged NICU stay, repeated ototoxic exposure, severe hyperbilirubinaemia requiring exchange, and prolonged ventilation — and she has correctly been screened with automated auditory brainstem response rather than otoacoustic emissions. The clinical insight the case tests is that a bilateral refer is not yet a diagnosis but a commitment to diagnostic follow-up, and that the high-risk infant is the one in whom auditory neuropathy spectrum disorder must be suspected and for whom surveillance continues even if a later screen passes. [1] [2]

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References5ShowHide
  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]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
  4. [4]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
  5. [5]Yoshinaga-Itano C, Sedey AL, Coulter DK, Mehl AL Language of early- and later-identified children with hearing loss. Pediatrics, 1998.PMID 9794949
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