Description
Sickle cell disease is an autosomal recessive
haemoglobinopathy, common in people of African-Caribbean descent.
A single point mutation in the beta chain of the haemoglobin
produces the abnormal haemoglobin, which is characteristic
of this condition. The defect involves substitution of valine
or lysine for glutamic acid in the 6th position of the beta
chain of haemoglobin A (HbA), resulting in HbS or HbC, respectively.
Meningitis and sickle cell disease are separate risk
factors for hearing impairment. We report a case of bilateral
profound sensorineural hearing impairment following pneumococcal
meningitis in a patient with sickle cell disease. The clinical
findings and the pathomechanisms of hearing loss are discussed
along with the role of early rehabilitation and steroids in
the treatment
