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 African-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 position 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 hemoglobin polymerizes, 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 susceptibility 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

Sudden Sensorineural Hearing Loss in Patients Aged from 15 to 40 Years

“…Our data emphasized the need for individuals presenting with SSNHL, especially women of reproductive age with a history of vascular thrombosis and/or pregnancy loss, to be tested for anti-β-2-glycoprotein-1 antibodies, lupus anticoagulant, and anti-cardiolipin antibodies; this recommendation stems from the potential role of antiphospholipid syndrome in the pathogenesis of SSNHL, as a consequence of microthrombus formation in the labyrinthine vasculature [ 42 , 43 ]. The present study also confirmed that sickle cell disease, a genetic disorder characterized by abnormal hemoglobin production, is another possible cause of SSNHL in individuals younger than 40 years of age [ 44 , 45 ]. Indeed, these patients have sickle-shaped red blood cells that can obstruct small blood vessels in the inner ear, leading to ischemia and subsequent SSNHL; in addition, the breakdown of sickle cells releases hemoglobin, which can be toxic to the cochlear structures [ 46 ].…”