Distortion product otoacoustic emissions in normal-hearing children with homozygous sickle cell disease

Area of Science:

• Otoacoustic Emissions
• Pediatric Audiology
• Hematology

Background:

• Sickle cell disease (SCD) can affect cochlear function due to vaso-occlusive events.
• Otoacoustic emissions (OAEs) are objective measures of outer hair cell function in the cochlea.
• Previous research has not extensively studied DPOAEs in children with SCD.

Purpose of the Study:

• To investigate distortion product otoacoustic emissions (DPOAEs) in young, normal-hearing children with SCD.
• To compare DPOAE prevalence and amplitude between children with SCD and those with normal hemoglobin.
• To assess potential cochlear function alterations in children with SCD using DPOAEs.

Main Methods:

• Distortion product otoacoustic emissions (DPOAEs) were measured in 20 children with SCD and 15 controls.
• DPOAEs were evoked using 13 primary tone pairs across a frequency range of 1000-4500 Hz.
• Stimuli were presented at two intensity levels: L1/L2 of 70/60 dB SPL and 50/40 dB SPL.

Main Results:

• The likelihood of detecting a DPOAE response did not differ between children with SCD and controls.
• Distortion product otoacoustic emission amplitudes were significantly larger in children with SCD (p =.01).
• Findings were contrary to the initial hypothesis of compromised cochlear function in SCD.

Conclusions:

• Cochlear outer hair cell function, as assessed by DPOAE amplitude, appears enhanced or unaffected in young children with SCD.
• The underlying mechanisms for larger DPOAE amplitudes in children with SCD require further investigation.
• Routine audiological screening using DPOAEs may not identify cochlear dysfunction solely based on SCD status in this population.