Sensorineural hearing loss among sickle cell disease patients from southern Brazil.

PURPOSE: A case-control study was done to confirm the higher prevalence of sensorineural hearing loss (SNHL) among sickle cell patients (HbSS) from southern Brazil.

PATIENTS AND

METHODS

Twenty-eight patients and 28 matched normal controls were studied. Besides complete anamneses and physical otolaryngological examination, all individuals had audiometric and tympanometric testings to determine the presence of SNHL or middle ear problems. The only significant difference between groups was the presence of a positive history of otologic symptoms (hearing loss, dizziness or tinnitus) and neurological sequelae in the HbSS patients.

RESULTS

Six patients (21.4%) presented with SNHL compared with one control (3.6%) (Fisher's one-tailed P = .05; OR, 7.36 (95% CI, 0.82, 65.83). The average age of HbSS diagnosis was higher among the patients with SNHL (10 years +/- 8.3) than those without SNHL (5.43 years +/- 5.65), but this was not significant (analysis of variance P > .05). More patients 25 years and older on the examination date had SNHL than younger patients (Fisher's 2-tailed P < .05). Sixty-seven percent of the patients with SNHL had alterations in the acoustic reflex, and a surprising 27% of the patients without NSHL also presented with some elevation or an absence of acoustic reflex.

CONCLUSIONS

These data indicate that patients with HbSS from southern Brazil are more predisposed to the development of sensorineural hearing problems than the general population.