RESUMEN
The objective of this study is to evaluate the impact of major sickle cell anaemia on the auditory function of the patients. It was a prospective survey conducted in the university hospital of Yopougon (Republic of Côte d'Ivoire). The auditory function of 112 major sickle cell anaemia patients 5- to 40-five-years-old, was compared with 112 healthy controls. 17% (19/112) of major sickle cell anaemia patients presented hearing loss from 30 dB to 65 dB versus 4% of controls. The average age of deaf patients was 26-years-old (7 years to 40-years-old). The sex-ratio was 0,73. The SC form with 47% (9/19) of deafness was the form the most frequently found followed by form SS, 37% (7/19) and form S beta+ thalassaemia 16% (3/19). The form S beta 0 thalassaemia did not record deafness patients. However the form having shown the highest rate of attack was the form S beta+ thalassaemia with 25% (3/12) followed by form SC 21% (9/43) and form SS 14% (7/51) of deafness. Deafness was generally light 73, 7% of the cases and often bilateral (52.6% of the cases). The audiometric loss affected both extremes of the hearing range, but was more significant in lower tones (58% of cases). Higher tones represented 5% of the cases. It was often sensorineural hearing loss 58% of the cases. Mixed hearing loss represented 42% of the cases.