The emergence and prevalence of hearing loss in children with homozygous sickle cell disease

Stuart, Andrew; Smith, Madeline R.

doi:10.1016/j.ijporl.2019.04.032

Cited by 4 publications

(1 citation statement)

References 40 publications

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“…The prevalence of SNHL in children with SCD is higher than that in normal children, ranging from 3.8% to 30.9% [2,[4][5][6][7][8][9][10]. In Brazil, the prevalence of SNHL is 28.8% in children with sickle cell anaemia (SCA) [2], and in the USA, the prevalence of SNHL is 8.3% [10] in children with SCD.…”

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confidence: 99%

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

Bomfim

Bacelar

Neto

et al. 2022

Tropical Med Int Health

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Sickle cell disease (SCD) is the result of a mutation in haemoglobin S, which causes physical and chemical changes. Haemoglobin S is prone to premature destruction, culminating in chronic haemolytic anaemia. In addition, this structure, being more rigid and not flexible, fixes more easily to the wall of the vascular endothelium, contributing to thrombotic phenomena and culminating in blood vessel stenosis [1].All these processes of chronic haemolytic anaemia and vaso-occlusive processes generate changes in several organs and systems, including changes in the auditory system, typically sensorineural hearing loss (SNHL), and in structures

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mentioning

confidence: 99%

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

Bomfim

Bacelar

Neto

et al. 2022

Tropical Med Int Health

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Limiares auditivos em frequências altas e emissões otoacústicas em pacientes com anemia falciforme

Weigert

Teixeira

Geyer

et al. 2021

RSD

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Objetivo: Verificar limiares auditivos em frequências convencionais e altas frequências e presença de emissões otoacústicas por produto de distorção em pacientes falcêmicos, analisando a variável faixa etária. Método: Coorte observacional, prospectivo, analítico, de fins diagnósticos. Realizada audiometria convencional, audiometria em frequências altas (AAF) e emissões otoacústicas por produto de distorção (EOAPD) em pacientes portadores da doença. Resultados: Foram avaliados 38 pacientes, sendo 16 (42,1%) na faixa etária de 7 a 20 anos de idade e 22 (57,9 %), de 21 a 35 anos. A alteração auditiva na audiometria convencional foi de 6,3% na faixa etária de 7 a 20 anos e 13, 6%, entre 21 e 35 anos. Não houve diferença estatisticamente significativa entre as faixas etárias (p=0,624). Na AAF, alteração em 25% das crianças e adolescentes e em 68,2% dos adultos. Os adultos apresentaram maior perda auditiva (p=0,021). Não houve diferença significativa na comparação das medidas de relação sinal/ruído das EOAPD entre as orelhas, tanto na amostra total quanto por faixa etária (p>0,05). Não houve associação significativa entre os resultados das alterações das EOAPD e a perda auditiva em frequências ultra-altas tanto nas crianças e adolescentes (p=0,250) quanto nos adultos (p=0,121). Na comparação entre as sensibilidades dos exames AAF e EOAPD, houve diferença significativa entre eles somente nos adultos (p=0,004). Conclusões: Os adultos com maior duração da exposição à doença apresentaram elevação dos limiares em AAF, bem como alteração das EOAPD.

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Prevalence and pattern of sensorineural hearing loss among children and adolescents with sickle cell disease in a tertiary health facility, Northwest Nigeria

Solomon,

Nurudeen Adebola,

Abdulkadir

et al. 2023

Ann Afr Med Res

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Sickle Cell Disease (SCD) can result in painful vascular occlusion crises, anoxia, and ischemia, which can occasionally cause damage to tissues and organs, including the auditory system, particularly the blood-rich cochlea. Despite being underreported, Sensorineural Hearing Loss (SNHL) is a well-known consequence of SCD globally. The study’s objective was to determine the prevalence and pattern of hearing loss in children and adolescents aged 5 to 16 years old with SCD who were in steady state and were seen at the hematology and pediatric outpatient clinics at Ahmadu Bello University Teaching Hospital (ABUTH), Zaria, Nigeria. A prospective hospital-based case-control study was conducted with 125 children with SCD in steady state, aged 5 to 16, who were enrolled in the hematology clinic at the Ahmadu Bello University Teaching Hospital in Zaria, Nigeria, and 125 ‘age- and sex-matched healthy controls, who were selected from the ABUTH’s pediatric outpatient clinics. An organized questionnaire was used to collect fundamental data on the sociodemographic characteristics and medical history of the participants and controls. In a sound-treated booth, the participant’s hearing was evaluated using tympanometry and pure tone audiometry. Overall, 68 (54.4%) and 57 (45.6%) out of the 125 assessed participants were male and female, respectively, with a male-to-female ratio of 1.2:1 and the mean age 10.17±3.55 years. Due to age-sex matching, the subject’s age and sex distributions match those of the controls. Children with SCD experienced bilateral SNHL ≥25 dB in 32/125 (25.6%) cases (21 males;11 females). The control group, which had a HbAA phenotype that was normal, did not exhibit any hearing loss. 21/32 (65.6%) of the participants had mild (26-40 dB) hearing loss, and SNHL occurred more frequently in males (21/32 (65.6%) than in females (11/32 (34.4%). In comparison to 3(9.4%) HbSC and 1(3.1%) HbSS+F, SNHL was more common in individuals with 28(87.5%) HbSS phenotypes. While diverse frequencies were impacted in the affected participants, there was no consistency in the frequency pattern of hearing loss. The current study showed that SNHL is a frequent complication in children and adolescents with SCD. About 25% of children and adolescents with SCD experienced SNHL, which disproportionately affected males. Frequent audiometry should be carried out to check the children’s hearing levels and identify any early hearing losses so that interventions can be made to perhaps prevent associated speech and language issues that might cause educational challenges.

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The emergence and prevalence of hearing loss in children with homozygous sickle cell disease

Cited by 4 publications

References 40 publications

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

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

Limiares auditivos em frequências altas e emissões otoacústicas em pacientes com anemia falciforme

Prevalence and pattern of sensorineural hearing loss among children and adolescents with sickle cell disease in a tertiary health facility, Northwest Nigeria

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