Inner Ear Disorders in a Family With Sickle Cell Thalassemia

• We describe a family of four sisters, two of whom have sickle cell thalassemia. One has sickle cell trait; the other has normal hemoglobin. Two of the sisters with sickle cell thalassemia showed symptoms of vertigo or hearing involvement and vertigo after strenuous exercise. One sister with unilateral hearing loss subsequently developed a sudden, almost identical loss in the other ear. Her symptoms closely resembled those of Ménière disease and very likely represent sickle cell auditory crises. Inner ear involvement in sickle cell disease may occur more often than previously recognized. A theory as to pathogenesis is presented.