Sudden Hearing Loss in Sickle Cell Disease: A Case Report
Abstract
S i c k l e cell disease (SCD) is an inherited hematologic disorder that may involve sensorineural hearing loss (SNHL) and that is common in those of African descent. It is an autosomal recessive hemoglobinopathy in which the carrier is said to have sickle cell trait. In Afr ican-Americans the prevalence of sickle cell trait is 7% to 9%, and that of SCD is approximately 0.25%. 1,2 Conventions of nomenclature define SCD as the family of hemoglobinopathies , including the homozygous form, sickle cell anemia (Hb SS), and multiple sickle cell variants including Hb SC. The hemoglobinopathy involves substitution of valine or lysine for glutamic acid at the sixth posit ion on the beta chain of Hb A, resulting in Hb S or Hb C, respectively. SCD is characterized by sickling of red blood cells when exposed to low oxygen tension. When deoxygenated, the abnormal hemoglob in polymer izes , deforming the red blood cells into a rigid, sickle shape. These sickled cells occlude the capillary bed causing further local tissue hypoxia and consequent sickling, leading to a vaso-occlusive crisis. 3 All tissues are subject to acute and chronic injury from such capillary bed occlusion. In addition, the clinical course is complicated by hemolytic anemia and susceptibili ty to infection caused by splenic infarction. This article presents a case of sudden hearing loss (SHL) as the first manifestation of Hb SC disease in a young adult
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