Patient:  J.S.

Age:  6 months

Gender: Female

JS did not pass neonatal screening and the report says she also “did not pass … a 90-dB brainstem response evaluation.”

JS has a twin. JS was discovered as having birth defects at 20 weeks gestation; JS and her twin were delivered at 34.5 weeks gestation via C-section.

JS was diagnosed with Dandy-Walker syndrome with hydrocephaly and she had surgery, with two shunts and a G-tube. She spent 3.5 months in the NICU; was treated for hyperbilirubinemia with phototherapy and for a staph infection with gentamicin and vancomycin.

• What is Dandy-Walker syndrome?  Could it affect ABR test results?
• What is a G-tube?
• Is the child at risk for progressive loss?  If so, why?

Tympanometry:

Type A bilaterally with 226 Hz and 1000 Hz probe tones.

1000 Hz ipsilateral reflex absent right, present left

OAEs absent bilaterally.

Click ABR at 95 dB nHL – no wave V seen; morphology especially poor left.

• Interpret these results and discuss your plan of action for this child.

Case donated by Gail Lim, AuD, Pediatrix Neonatal Group, presenting follow-up data on a child identified by Pediatrix through their neonatal screening program.