To describe the occurrence of both peripheral and central auditory system dysfunction in sickle cell anaemia (SCA) patients and discuss the different mechanisms hypothesized to be responsible for these alterations.An electronic search was conducted using PubMed Central® (MEDLINE), LILACS® and Scopus® databases. This systematic review was performed in accordance with the PRISMA Statemen. Original observational studies that utilized audiological tests for auditory system evaluations in SCA were included.A total of 183 records were found in the databases searched. Twenty one of these studies met the inclusion criteria. Pooled prevalence of sensorineural hearing loss (SNHL) was 20.5% (CI: 10.3%-33%). Retrocochlear involvement was detected with the auditory brainstem response, which asseses integrity of the cochlea, 8 cranial nerve, and brainstem structures. In addition, the increase in otoacoustic emissions amplitudes in SCA patients indicates changes in cochlear micromechanics and precedes the expression of a detectable cochlear pathology.The prevalence of the SNHL is higher than in the general population. Dysfunction of the auditory system may be present in SCA patients, with the most probable mechanism being the presence of circulatory disturbances resulting from the chronic inflammatory state of the disease. This article is protected by copyright. All rights reserved.