Sensorineural hearing loss among sickle cell disease patients from southern Brazil

Sensorineural hearing loss in sickle cell disease—A prospective study from Oman

“…[7][8][9]15 Given its size, this tiny organ receives a disproportionate percentage of the body's blood. 16 Ischemia of the stria vascularis, with secondary hypoxia of the organ of Corti, as a direct result of sickle cell crisis, therefore poses a significant risk to the auditory system.…”

“…In SCD, hearing loss manifests first in the basal turn (high frequencies) and then in the apical turn (low frequencies) and then throughout the entire cochlea. [5][6][7][8][9] The inner ear structures are largely supplied by the labyrinthine artery, which divides into the vestibular, vestibulocochlear, and cochlear branches, all of which are terminal arteries. Even brief ischemia can damage the inner ear because of minimal collaterals to these terminal arteries.…”

“…4 SCD has been frequently associated with sensorineural hearing loss (SNHL) with a variable degree of severity when compared with the rest of the general population. [5][6][7][8][9] The basal turn of the cochlea is generally sensitive to anoxia, due to high oxygen consumption rate of the stria vascularis and the poor capacity for anaerobic metabolism. Therefore, with anoxia, the basal turn, which records high frequencies, is the first affected before the apical turn, which is the place that records the lower frequencies.…”

Insights into elevated distortion product otoacoustic emissions in sickle cell disease: Comparisons of hydroxyurea-treated and non-treated young children

“…Indeed, histopathological changes in the temporal bone and degenerative changes in the organ of Corti consonant with hypoxia have been observed with SCD (Morgenstein and Mance, 1969). Also consistent with this speculation is the observation of a higher prevalence of sensorineural hearing loss among those with SCD (Adams and Benson, 1992;Ajulo et al, 1993;Ashoor and Al-Awamy, 1985;Atsina and Ankra-Badu, 1988;Crawford et al, 1991;Forman-Franco et al, 1982;Friedman, et al, 1980;Piltcher et al, 2000;Todd et al, 1973). Hearing loss has a progressive nature in SCD as the prevalence is lower in children than adults (Gentry et al, 1997;Koussi et al, 2001;MacDonald et al, 1999;Odetoyinbo and Adekile, 1987) 4 and the degree becomes more advanced with increasing age (Onakoya et al, 2002;Piltcher et al, 2000).…”

Clinical and Radiologic Findings of Inner Ear Involvement in Sickle Cell Disease

“…3 SNHL is a well-recognized complication of SCD. [4][5][6][7][8][9][10][11] LH and LO have been reported to affect the inner ear in SCD; however, the association between inner ear involvement and imaging findings has not been fully described. Our purpose was to examine the prevalence of inner ear involvement of LH and LO and to assess the relationship between clinical and imaging findings of LH and LO in SCD.…”