Introduction: Sickle cell anemia is a disease characterized by a wide vaso-occlusive incident from micro-vascular incident to muscularactivity. The cochlear function can also get affected by this vaso-occlusion. Objective: It is aimed at determining what kind of effects sickle cell anemia has on hearing and balance system. Methods: This study has been conducted on 46 patients with sickle cell anemia and 45 healthy individuals. For all participants, their pure tone hearing thresholds and videonystagmography (VNG) findings have been determined in 17 frequencies between 125–16.000 Hz. Results: All hearing thresholds between 125 and 16,000 Hz, pure tone averages of patients with sickle cell anemia have been found statistically significant to be higher than the corresponding values in the control group(p < 0.05). The normal hearing rate of patients with sickle cell anemia has been determined to be 71.1% conductive hearing loss (CHL) to be 4.4%, sensorineural hearing loss (SNHL) to be 22.2%, and mixed type hearing loss to be 2.2% in right ear; the normal hearing rate has been determined to be 71.1%, CHL to be 2.2%, SNHL to be 22.2%, and mixed type hearing loss to be 4.4% in left ear. Statistically significant difference has not been found between head shake, spontaneous nystagmus, optokinetic, tracking test batteries, static and dynamic positional tests used in VNG, saccade accuracy and saccade peak velocity, which are saccadic test findings of 2 groups. However, saccadic latency, which is a saccadic test finding, has been determined to be longer in patients with sickle cell anemia in comparison to the control group. Conclusion: While sickle cell anemia causes hearing deficits, it does not have any effect on the central or peripheral vestibular system.

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