Sensorineural Hearing Loss in Homozygous Sickle Cell Disease in Qatif, Saudi Arabia

Abstract

Sensorineural hearing loss (SNHL) is a known complication of sickle cell 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 group consisted of 100 healthy individuals. A detailed history, otologic and audiologic examinations were carried out on both groups. Further investigations to rule out local retrocochlear pathology were performed on those having unilateral SNHL. No case of SNHL was detected in the control group, while it was recorded in 19 (19%) of SCA patients, 13 males and six females. There was a significant association between the SNHL and the onset of first vaso-occlusive crisis at six years of age or less. There was no significant difference in the frequency of SNHL between children and adults. Twenty-one percent of patients who had SNHL completely recovered. Patients with SCD need a careful regular hearing assessment. Those detected to have SNHL need a careful plan implemented by both the clinician and otolaryngologist for proper care of this important complication.

Dysfunction of the auditory system in sickle cell anaemia: a systematic review with meta‐analysis

“…Of these 27 studies, six were excluded because they did not differentiate between homozygotes (SCA) and heterozygotes in the methods and/or in the results. Thus, 21 articles were included in this systematic review (Figure ).…”

“…All the studies were observational cross‐sectional studies. Among the 21 articles included, 14 primarily aimed to determine the prevalence of SNHL in SCA using PTA. Six studies used the OAE test for identifying sensorial dysfunctions.…”

“…Six studies used the OAE test for identifying sensorial dysfunctions. Four studies included in this systematic review evaluated retrocochlear involvement with the ABR test; however, two of these studies used audiometry and the ABR test, while one study used OAE and ABR tests.…”

“…The studies that analysed the prevalence of SNHL using PTA assessed a total of 884 participants with SCA. The samples ranged from 23 to 167 participants of both sexes; the age range was 4–56 years.…”

“…Retrocochlear involvement with pathology involving the brainstem structures in the lower central auditory pathways (CAP) was evaluated in four studies of this systematic review using the ABR test. A total of 100 participants of both sexes (age, 15–50 years) were evaluated.…”