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

Abstract

Objectives To describe the occurrence of both peripheral and central auditory system dysfunction in sickle cell anaemia (SCA) patients and discuss the different mechanisms hypothesised to be responsible for these alterations. Methods An electronic search was conducted using PubMed Central® (MEDLINE), LILACS® and Scopus® databases. This systematic review was performed in accordance with the PRISMA statement. Original observational studies that utilised audiological tests for auditory system evaluations in SCA were included. Results 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 assesses integrity of the cochlea, 8th cranial nerve and brainstem structures. In addition, the increase in otoacoustic emission amplitudes in SCA patients indicates changes in cochlear micromechanics and precedes the expression of a detectable cochlear pathology. Conclusion 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.

Association between hearing impairment, school performance and cognitive function in children and adolescents with sickle cell disease

“…The occurrence of SNHL is similar to that reported in other studies, which indicates a high occurrence of SNHL in this population at an early age with mild severity [2,24]. In the comparisons performed in studies that included SCD patients with different genotypes, the prevalence and characteristics of SNHL were similar to those of patients with HBSS and HbSC [25].…”

Low-Frequency Sensorineural Hearing Loss Associated With Iron-Deficiency Anemia

“…However, many studies have shown bilateral and unilateral SNHL at various frequencies in patients with sickle cell anemia (SCA). 14 Onakoya et al 15 reported both bilateral (62%) and unilateral (left ear, 18%; right ear, 20%) SNHL in patients with SCA. While high-frequency SNHL was observed in most patients, low-frequency SNHL was observed in some patients, particularly in the right ear (5.6%).…”

Numb Chin Syndrome in Sickle Cell Disease: A Systematic Review and Recommendations for Investigation and Management

“…An increased prevalence in the early onset of the neurocognitive disorder is observed, even in those without ischemic lesions, possibly because of reduced cerebrovascular reserve [ 9 , 10 ]. Patients with the SC genotype also commonly report auditory disturbances [ 11 ]. Despite peripheral neuropathies being relatively rare in SCD, there have been several cases associated with numb chin syndrome (NCS).…”