Original Contributions Sensorineural Hearing Loss Among Sickle Cell Disease Patients From Southern Brazil Otavio Piltcher, MD, MSc, Luciana Cigana, Joao Friedriech, MD, Fernando de Andrade Quintanilha Ribeiro, MD, PhD, and Sady Selaimen da Costa, MD, MSc, PhD 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. (Am J Otolaryngol 2000;21:75-79. Copyright © 2000 by W.B. Saunders Company) Sickle cell disease (SCD) was first described by Herrick in 1910. A chronic hemolytic ane- mia and vaso-occlusive events that lead to a variety of signs and symptoms characterize the disease. It is caused by a single-point mutation in the 6th position of the hemoglo- bin's beta chain. The negative amino-acid glu- tamic acid is replaced by valine, a hydropho- bic amino acid. This substitution affects the physicochemical properties of the red blood From the Hospital de Clinicas de Porto Alegre; and the Faculdade de Ci6ncias M~dicas da Santa Casa de Sao Paulo, Brazil. Supported in part by Funda£Ao de Amparo a Pesquisa do Rio Grande do Sul (FAPERGS)--grant 96/60433.5. Address reprint requests to Otavio Piltcher, MD, MSc, Rua Miguel Tostes 533--201/202, Porto Alegre--RGS-- Brazil 90430-061. Copyright © 2000 by W.B. Saunders Company 0196-0709/00/2102-0001 $10.00/0 cell, causing morphological modifications that resemble a sickle shape. Affected red blood cells have a shorter life span and may cause microvascular occlusions. Indeed, all signs and symptoms of the disease are directly or indirectly correlated with these alterations. The prevalence of SCD is high among Afri- cans and their descendents, Arabs, Central and South Americans, and people of the Medi- terranean area. 1-3 In Brazil, SCD is the most common heredity disease, affecting from 0.1% to 0.3% of the black population and a rising number of whites. Approximately one of every 1,000 to 1,500 live Brazilian births is affected by or is a carrier of the SCD allele. 4 In 1983, the life expectancy of affected homozygotic indi- viduals in Brazil was reported to be 16 years, with a range of from 4 to 28 years. 5 More recently, a rising life expectancy has been American Journal of Otolaryngology,Vo121, No 2 (March-April), 2000: pp 75-79 75