Language assessment in Egyptian children with sickle cell disease

Abstract Background: Sickle cell disease (SCD) is a blood disorder; however, the central nervous system is one of the organs frequently affected by the disease. Brain insult can begin early in life and often leads to neurocognitive dysfunction. The progression of central nervous system abnormalities in SCD and its effect on language development have not been fully delineated. Aim: To determine the effect of SCD on language development in Egyptian children with SCD in order to delineate this group as a possible high-risk group for language disorders. In this way, further proper assessment of these children will aid the initiation of an early intervention and prevention of these problems. Participants and methods: A total of 24 children with SCD were subjected to the language assessment protocol of Ain Shams University Hospitals. These patients underwent language testing using the Standardized Arabic Language Test and hearing assessment including a basic audiological assessment and transient evoked otoacoustic emissions (TEOAEs) testing. The results obtained from this group were compared with the results of 17 normal children who were subjected to the same assessment protocol. Results: The results of this study showed that the age of acquisition of both the first word and sentence was significantly delayed in children with SCD than their control group. The scores of intelligence quotient and language parameters were significantly lower in the group of children with SCD. The language age deficit was significantly higher in the SCD group than the control group. The expressive language abilities and pragmatics were significantly lower than semantics and receptive abilities in children with SCD. The age of onset of the disease and number of vaso-occlusive crisis showed a significant correlation with the intelligence quotient score and all the language parameters. In terms of audiological assessment, all children of both groups had normal audiograms and tympanograms, but the group of children with SCD showed reduced TEOAEs echo levels in comparison with their control. Moreover, there was a significant correlation between TEOAEs echo levels and language parameters in children with SCD. Conclusion: SCD is considered as one of the important risk factor that can affect proper language development. Therefore, long-term follow-up of these children is necessary to detect deficits early in life to prevent delayed language development and poor academic achievement. © 2012, The Egyptian Oto - Rhino - Laryngological Society.