Prevalence of auditory and vestibular symptoms in sickle cell disease

Abstract

Background and objectives: Sickle Cell Disease (SCD) is a generic term used to determine a group of genetic alterations characterized by hemoglobin S (Hb S) predominance. Sickle cell anaemia (SCA), the most common and severe SCD, is an autosomal recessive, inherited disorder of hemoglobin structure caused by a point mutation in the Beta²-globin chain of hemoglobin. Low blood oxygenation promotes the polymerization of hemoglobin, which distorts red blood cells into a sickle shape and reduces their elasticity. Consequently, these rigid and abnormally shaped blood cells are unable to deform as they pass through narrow capillaries, leading to vessel occlusion and ischaemia. Recent discoveries allowed SCD patients benefit from early diagnosis and better options of treatment. With longer longevity, the incidence of new complications of SCD became apparent and previously milder complications became more severe and more common. The ontological complications of SCD are examples of these changes that have become more common than before. However, there is a wide variety of data regarding the incidence, prevalence and management of these complications. Therefore, it is necessary a study covering the analysis of the patient stricken by this disorder and its prevalence among Brazilian patients with SCD. Besides, as the labyrinthine artery irrigates both the cochlea and the vestibule, it is also necessary to search for any symptom that is typical of its involvement (tinnitus and dizziness). Methods: This study has an analytical, transversal, clinical research plan. Will be included 50 SCD patients and 50 healthy individuals in the control group matched for age and sex. Analyzed aspects: 1. Hemoglobin type (SS, Sbeta ou SC) 2. Hemogram (hemoglobin level, hematocrit, DHL) 3. Hematologic questionnaire: a. Number of vaso-occlusive crisisb. Acute thoracic syndromic. Osteonecrosis 4.Audiological questionnaire: a. Hearing loss. Dizziness. Tinnitus 5. Audiometry 6.Impedanciometry 7.Otoacoustic emissions the size of the sample was calculated using POCOCK (1983) formula.