Purpose: Sickle cell disease (SCD) is a genetically inherited red blood cell disorder that affects people all over the world but is more common among blacks of African ancestry than other races. The condition is linked to sensorineural hearing loss (SNHL). This scoping review aims to evaluate studies that reported SNHL in SCD patients and to identify demographic and contextual risk factors for SNHL in SCD patients.
Methods: We conducted scoping searches for relevant studies in PubMed, Embase, Web of Science, and Google Scholar. All articles were evaluated independently by two authors. The checklist Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) was used. SNHL was detected at hearing levels above 20 decibels.
Results: In terms of methodology, the studies reviewed were diverse, with 15 being prospective and four being retrospective. Fourteen of the 19 articles chosen from 18,937 search engine results were case-control studies. Sex, age, foetal haemoglobin (HbF), SCD type, painful vaso-occlusive crisis (PVO), blood parameters, flow-mediated vasodilation (FMV), and hydroxyurea use were all extracted. Few studies investigated SNHL risk factors with noticeable knowledge gaps. Age, PVO, and certain blood parameters appear to predispose to SNHL, whereas decreased FMV, the presence of HbF, and the use of hydroxyurea appear to have an inverse relationship with the development of SNHL in SCD.
Conclusion: There is a clear gap in the existing literature regarding the knowledge of demographic and contextual risk factors that is required for the prevention and management of SNHL in SCD.
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Purpose: Sickle cell disease (SCD) is a genetically inherited red blood cell disorder that affects people all over the world but is more common among blacks of African ancestry than other races. The condition is linked to sensorineural hearing loss (SNHL). This scoping review aims to evaluate studies that reported SNHL in SCD patients and to identify demographic and contextual risk factors for SNHL in SCD patients.
View Article and Find Full Text PDFAssociation between hearing impairment, school performance and cognitive function in children and adolescents with sickle cell disease.
Trop Med Int Health
March 2022
College of Speech Therapy, Department of the Life Science, State University of Bahia, Salvador, Brazil.
Objective: To investigate the association among hearing impairment, school performance, and cognitive function in children and adolescents with sickle cell disease.
Methods: Thirty-one participants with sickle cell disease (SCD) and 31 healthy participants in the control-comparison group (CG), both aged 8-17 years underwent auditory system evaluation (pure tone audiometry and acoustic reflex), were screened for the risks of (central) auditory processing disorder and dysfunction of cognitive function using the Scale of Auditory Behaviors (SAB) and the Mini-Mental State Examination (MMSE), respectively, and were interviewed to obtain clinical data and data on school performance.
Results: In the SCD group, eight (25.
Prevalence of Sensorineural Hearing Loss in Pediatric Patients with Sickle Cell Disease: A Meta-analysis.
Laryngoscope
May 2021
Division of Otolaryngology-Head and Neck Surgery, George Washington University, Washington, District of Columbia, U.S.A.
Objectives: To determine the prevalence of Sensorineural Hearing Loss (SNHL) attributable to Sickle Cell Disease (SCD) in the global pediatric population and to identify factors contributing to its severity.
Study Design: Meta-analysis.
Methods: We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations under 18 years of age with excluding studies analyzing patients on iron chelation therapy, adults, or those without objective audiological analysis.
Enhanced Otolith Function Despite Severe Labyrinthine Damage in a Case of Pneumolabyrinth and Pneumocephalus Due to Otogenic Meningitis Associated With Superior Canal Dehiscence.
Otol Neurotol
January 2021
ENT Unit, Department of Surgery, Azienda USL - IRCCS di Reggio Emilia.
Objective: To describe a rare case of pneumolabyrinth (PNL) and pneumocephalus (PNC) due to otogenic meningitis in a patient with superior canal dehiscence (SCD) resulting in profound sensorineural hearing loss (SNHL), semicircular canals impairment but preservation of SCD-related enhanced otolith function.
Patient: A 65-year-old woman with otogenic meningitis.
Intervention: Temporal bone high-resolution computed tomography (CT) scans, brain-magnetic resonance imaging, audiometry, bedside examination, video-head impulse test, and vestibular-evoked myogenic potentials (VEMPs).
Characterization of Sensorineural Hearing Loss in Adult Patients With Sickle Cell Disease: A Systematic Review and Meta-analysis.
Otol Neurotol
January 2021
Division of Otolaryngology, George Washington University School of Medicine and Health Sciences, Washington, DC.
Objectives And Main Outcome Measures: To determine the prevalence of sensorineural hearing loss (SNHL) attributable to sickle cell disease (SCD) in the global adult population and to identify factors contributing to its severity.
Study Design: Systematic Review and Meta-analysis.
Methods: We performed a comprehensive literature search for scientific articles in PubMed, Scopus, CINAHL, Web of Science, and the Cochrane Library that reported the incidence of hearing loss in populations over 18 years of age with SCD.
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