The primary concern of this investigation is the consequential relationship of sickle cell anemia to sensorineural hearing loss and abnormal auditory function. The ultimate goal of this project is to investigate and define this relationship by determining the prevalence and predominate site of hearing loss and/or auditory dysfunction in sickle cell patients during sickle cell crisis, with the disease under control (non-crisis) and in the general sickle cell population. A comprehensive audiological evaluation process, including non-invasive auditory evoked responses and otacoustic emissions electrophysiological techniques will be utilized. Various investigators have assessed peripheral auditory sensitivity, with a wide disparity of results. A variety of case studies have documented middle ear, cochlear, VIII nerve, and central auditory involvement of sickle cell patients. Different patterns and degrees of hearing loss have been reported in association with sickle cell disease, ranging from profound losses bilaterally with partial recovery over time, to mild to moderate unilateral losses predominately in the high frequencies. Limited electrophysiological assessment of sickle cell patients has appeared in the literature.
The specific aims of this proposal are: to investigate the relationship between sickle cell status (control versus crisis); site of auditory deficit (cochlea versus eighth nerve); and neurological/developmental consequences related to abnormal auditory function; to effectively define a paradigm predictive of significant auditory system deficits in relation to the number, duration and severity of crisis episodes; and to develop an economical and efficient model for appropriate audiological assessment and treatment of sickle cell anemia patients.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Comprehensive Center (P60)
Project #
5P60HL038737-09
Application #
5213675
Study Section
Project Start
Project End
Budget Start
Budget End
Support Year
9
Fiscal Year
1996
Total Cost
Indirect Cost
Burch-Sims, G Pamela; Matlock, Valeria R (2005) Hearing loss and auditory function in sickle cell disease. J Commun Disord 38:321-9
Aguinaga, M D; Kutlar, F; Turner, E A et al. (2000) Hb Inkster [alpha85(F6)Asp-->Val] found in a caucasian male with polycythemia. Hemoglobin 24:333-9
Shokrani, M; Terrell, F; Turner, E A et al. (2000) Chromatographic measurements of hemoglobin A2 in blood samples that contain sickle hemoglobin. Ann Clin Lab Sci 30:191-4
Popp, R A; Shinpock, S G; Qopp, D M et al. (1998) Erythropoietin level and effect of rHuEPO in beta-thalassemic mice. Ann N Y Acad Sci 850:455-8
Iyamu, E W; Roa, P D; Kopsombut, P et al. (1998) New isocratic high-performance liquid chromatographic procedure to assay the anti-sickling compound hydroxyurea in plasma with ultraviolet detection. J Chromatogr B Biomed Sci Appl 709:119-26
Roa, D; Kopsombut, P; Aguinaga, M P et al. (1997) Hydroxyurea-induced denaturation of normal and sickle cell hemoglobins in vitro. J Clin Lab Anal 11:208-13
Roa, D; Turner, E A; Aguinaga, M D (1995) Reference ranges for hemoglobin variants by HPLC in African Americans. Ann Clin Lab Sci 25:228-35
Robbins, V; Aguinaga, M P; Valenzuela, M S (1995) Efficient isolation of whole genomic DNA from cell cultures and blood samples. Biotechniques 18:414-6, 418
Roa, P D; Turner, E A; Aguinaga, M del P (1993) Hemoglobin variant detection from dried blood specimens by high performance liquid chromatography. Ann Clin Lab Sci 23:433-8
Gu, H; Wilson, D; Inselburg, J (1992) Recovery of DNA from agarose gels using a modified Elutrap. J Biochem Biophys Methods 24:45-50

Showing the most recent 10 out of 17 publications