E.A. is age 5 months.  She has Trisomy 18 (Edwards sydrome).  She is seen by the same audiologist who tested the child described in Case 7.

• Is Edwards associated with hearing loss?

E.A. did not pass neonatal screening, left ear.  She was born 3 weeks prematurely, breech, and with fetal distress. The mother indicates that it was an induced delivery.  An emergency C-section was completed.  The child weighed 4 lbs 4 ounces and was in the NICU for 2 months.

• It seems odd that they would induce when ultrasound showed the baby was so small.  Do you think it would be acceptable to ask the child’s mother why they induced labor?  Or, if the delivery was induced due to the mother’s being uncomfortable or herself at medical risk, would that lead to maternal guilt?  Would it be equated with your implying that the obstetrician was negligent?  Is this an area where it’s better not to inquire?

She  has low-set ears, a small lower jaw.  She has heart defect, she is still small in size and has feeding / swallowing problems.  She has sluggish passage of urine from the kidneys to the uretrha and is on amoxicillin and a diuretic prophylacticly.

• Are these typical with Edward’s syndrome?

Click-evoked ABR testing was conducted in natural sleep and quiet wakefulness.  The click response was observed down to 40 dB nHL, which was interpreted as indicating mild hearing loss in the 2-4 kHz region.  The report states “For the right ear ear waveforms indicated slightly delayed latency for I with normal inter-wave latencies for E.A.’s gestational age. For the left ear wave morphology was poor with wave I appearing to be present at a very delayed latency. Wave V was noted to be present with normal latency period for gestational age. Reversed polarity recordings showed no evidence of auditory dys-synchrony.”

The report states “Otoscopic examination of the right ear was unremarakble. The left ear canal was very narrow and the tympanic membrane could not be visualized. Tympanometry revealed abnormal middle ear mobility bilaterally using a 1000 Hz probe tone. Acoustic reflexes could not be evaluated do to the reduced mobility.”

• Describe the ABR findings further.  What exactly does the click threshold mean?  Could the threshold of visualizing the response and the response morphology be influenced by the child’s state?  Discuss.  Given the report, what is the I-V interpeak latency, and what does that finding suggest?
• Critique the immittance test results/report.

The following was recommended:

1. Bone conduction click ABR testing

2. Otological evaluation and management.

3. Consideration of bone conduction hearing aid if medical management is not successful

• Do you concur? Would your recommendations have differed?

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