ERIC Number: EJ698176
Record Type: Journal
Publication Date: 2005-Jul
Reference Count: N/A
Hearing Loss and Auditory Function in Sickle Cell Disease
Burch-Sims, G.P.; Matlock, V.R.
Journal of Communication Disorders, v38 n4 p321-329 Jul 2005
Sickle cell disease was first reported in 1910 by J. Herrick, and since then, various associated conditions and complications have been described. Sickle cell disease is a hereditary disorder characterized by abnormality of the hemoglobin in the red blood cell. During periods of decreased oxygen tension in the red blood cell's environment, the abnormal hemoglobin within the red blood cell polymerizes and causes it to assume its sickled shape. This morphological change and its associated physiological changes drastically reduce the ability of red blood cells to navigate and deliver oxygen throughout the body. Sickle cell disease is a significant health problem affecting 1 in 400 African-Americans in the United States. One in 10 African-Americans in the United States has sickle cell trait. A variety of hemoglobinapathies are classified as sickle cell disease. Variants that simultaneously occur with hemoglobin S in high frequency are hemoglobins C and beta Thalassemia, and less frequently hemoglobin E. Sickle cell disease is characterized by chronic hemolytic anemia, end-organ damage, a heightened susceptibility to infections, and intermittent episodes of vascular occlusion causing both acute and chronic pain. Neurological symptoms are frequent in patients diagnosed with sickle cell disease. Considering the vaso-occlusive nature of sickle cell disease, the potential for auditory damage is not unexpected. However, the incidence of subjective hearing impairment among sickle cell anemia subjects is very low; therefore, the interest in hearing loss associated with the disease is not in its symptomatology, but in its pathogenesis. The relationship between sickle cell anemia and hearing loss is documented, but little is known about the relationship. Numerous investigations have assessed peripheral auditory sensitivity with a wide disparity of results. In the article, we will discuss: (1) The genetic characteristics and the pathophysiology of sickle cell disease; (2) The prevalence and predominate site of hearing loss and/or auditory dysfunction during sickle cell crisis and with the disease under control (non-crisis); (3) A model for appropriate audiological assessment and treatment of sickle cell disease patients, including published results of investigations utilizing this model. In view of the diversity of results and speculative etiology presented here and in the literature, the relationship between sickle cell anemia, auditory sensitivity, and auditory function warrants additional investigation. Learning outcomes: As a result of this activity, the participant will read descriptions of the genetic and pathophysiological characteristics of sickle cell disease. The participant will examine evidence of the prevalence of hearing loss and auditory dysfunction in the sickle cell population, as well as the overall hearing health risk for sickle cell patients in comparison to the risk for the normal hemoglobin population. The participant will examine a model for appropriate audiological assessment of treatment of patients with sickle cell disease.
Descriptors: Patients, Pathology, Investigations, Incidence, Etiology, Pain, Hearing Impairments, Hearing (Physiology), Genetics, Diseases
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Publication Type: Journal Articles
Education Level: N/A
Authoring Institution: N/A
Identifiers - Location: United States