SENSORINEURAL HEARING-LOSS IN HOMOZYGOUS SICKLE-CELL DISEASE IN QATIF, SAUDI-ARABIA

Sensorineural hearing loss (SNHL) is a known complication of sickle ce ll disease (SCD). There is a paucity of information on this problem in Saudi SCD patients. A prospective controlled study was conducted over 27 months in Qatif Central Hospital. One hundred patients with sickle cell anemia (SCA), aged five to 40 years, were studied. The control g roup consisted of 100 healthy individuals. A detailed history, otologi c and audiologic examinations were carried out on both groups. Further investigations to rule out local retrocochlear pathology were perform ed on those having unilateral SNHL. No case of SNHL was detected in th e control group, while it was recorded in 19 (19%) of SCA patients, 13 males and six females. There was a significant association between th e SNHL and the onset of first vaso-occlusive crisis at six years of ag e or less. There was no significant difference in the frequency of SNH L between children and adults. Twenty-one percent of patients who had SNHL completely recovered. Patients with SCD need a careful regular he aring assessment. Those detected to have SNHL need a careful plan impl emented by both the clinician and otolaryngologist for proper care of this important complication.