The primary purpose of this investigation is to study the auditory functioning of a population of patients with Sickle Cell Disease. A review of the literature indicates that patients with Sickle Cell Disease have a higher incidence of high frequency sensory neural type hearing loss. A study with children also suggests a higher incidence of central auditory dysfunction with normal peripheral hearing. Studies also show patients to have abnormally high or absent acoustic reflexes with normal auditory thresholds. None of the previsous studies offer conclusive evidence as to a specific site of lesion in these patients. It could be cochlear as evidenced by high frequency loss; it could be VIII never or lower brain stem as evidenced by acoustic reflexes; or it could be higher brain stem or cortical as evidenced by the SSW test. Through a battery of audiologic tests that have been chosen because of their ability to help identify more specifically the site of lesion it is hoped that the information obtained will (1) determine if there is a higher incidence of hearing loss in Sickle Cell patients; (2) determine the type and degree of the loss; (3) determine if those patients with normal peripheral hearing demonstrate any evidence of auditory dysfunction. An additional goal is to obtain information on Sickle Cell patients who are receiving a drug (desferol) which has been shown to cause hearing impairment. The test battery will include: pure tone and speech thresholds, speech discrimination at increasing intensity levels (PB/PI), acoustic reflex and decay, tympanometry, Staggered Spondee Word Test (SSW), and auditory Brainstem Response (ABR). The results of this study can have immediate and long term benefits to the patients. If the patients fail the screening test the medical intervention can begin immediately. The results of the test battery can yield specific information about the type and degree of loss. The patient can then be provided with additional services as needed; hearing aid evaluations, counseling and/or therapy. Test results can serve as baseline data for future comparisons. Monitoring of these patients seems profitable since they may be more susceptible to hearing loss due to their illness or medications.
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