Hidden hearing loss in children and adolescents with sickle cell anemia

Area of Science:

• Audiology
• Hematology
• Pediatrics

Background:

• Sickle cell anemia (SCA) is a genetic blood disorder with potential systemic complications.
• Hidden hearing loss (HHL) can affect auditory function without significant changes in standard hearing tests.
• Endothelial dysfunction (ED) is a known complication in SCA.

Purpose of the Study:

• To investigate auditory system function in children and adolescents with SCA.
• To assess for hidden hearing loss (HHL) in SCA patients.
• To explore the association between auditory changes, clinical variables, and endothelial dysfunction (ED) in SCA.

Main Methods:

• Evaluated 37 SCA patients and 44 healthy controls (aged 6-18 years) with normal hearing thresholds.
• Utilized pure tone audiometry, tympanometry, acoustic reflex, otoacoustic emission, and auditory evoked potentials.
• Assessed lipid profile, C-reactive protein, and brachial artery flow-mediated dilation for endothelial function.

Main Results:

• SCA patients showed increased contralateral acoustic reflex thresholds compared to controls (p<0.05).
• Significant differences in brainstem auditory evoked potentials were observed in SCA patients, including increased wave latencies and interpeak intervals.
• No association was found between audiological measures and clinical/metabolic variables, ED, or SCA complications.

Conclusions:

• Auditory system damage, particularly in retrocochlear structures, can be present in SCA patients.
• These functional deficits in the auditory system may occur without a measurable decline in hearing sensitivity.
• Further research is needed to understand the mechanisms and implications of auditory system involvement in SCA.