Sickle Cell anemia and hearing loss among children and youngsters: literature review

Silva, Luzia Poliana Anjos da; Vila-Nova, Camila; Lucena, Rita

doi:10.1590/s1808-86942012000100020

Cited by 17 publications

(10 citation statements)

References 16 publications

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“…However, the hypoxia resulting from abnormally shaped blood cells in the circulation may lead to the absence of blood supply to the neurons responsible for the transmission of the electrical impulses to central auditory pathway . This may lead to changes in the MOC efferent and emergence of reflexive processes of synaptic growth and hyperactivity of the ventral cochlear nucleus, which is responsible for control of the OHC; thus, the electromotility of the OHC is elevated with the purpose of improving cochlear sensitivity and increasing the gain of the signal in the cochlea.…”

Section: Discussionmentioning

confidence: 99%

“…Since HU is a drug that diminishes the inflammatory process in SCA by reducing the adhesion of erythrocytes and leucocytes to the vascular endothelium and reduces myelosuppression and vasodilatation by means of nitric oxide release [44], it is possible that the return of the amplitudes of the OAE to a normal level reflects a reduction in the mechanism of the undetermined cause of the increase in the amplitude of OAE. However, the hypoxia resulting from abnormally shaped blood cells in the circulation may lead to the absence of blood supply to the neurons responsible for the transmission of the electrical impulses to central auditory pathway [8,9]. This may lead to changes in the MOC efferent and emergence of reflexive processes of synaptic growth and hyperactivity of the ventral cochlear nucleus, which is responsible for control of the OHC; thus, the electromotility of the OHC is elevated with the purpose of improving cochlear sensitivity and increasing the gain of the signal in the cochlea.…”

Section: Discussionmentioning

confidence: 99%

“…Concerning the effects of SCD on the auditory system, evidence documented since the past century has revealed the presence of SCD-related symptoms and auditory pathway dysfunction [6][7][8][9]. The physiopathological mechanism appears to be linked to circulatory changes in the auditory system as a result of the abnormally shaped blood cells, which cause hypoxia in the structures of auditory system [5][6][7]10].…”

Section: Introductionmentioning

confidence: 99%

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

Lago

Fernandes

Alves³

et al. 2019

Tropical Med Int Health

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Lago

Fernandes

Alves³

et al. 2019

Tropical Med Int Health

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“…SNHL was unilateral in all our cases, suggesting that SNHL in SCD may result from local tissue ischemia. The basal turn of the cochlea is particularly sensitive to hypoxia, given the high oxygen consumption rate of the stria vascularis and the high metabolic activity necessary for maintaining the endolymph's ionic and electrical balance . Secondary hypoxia to the organ of Corti may cause further cochlear damage .…”

Section: Discussionmentioning

confidence: 99%

“…The basal turn of the cochlea is particularly sensitive to hypoxia, given the high oxygen consumption rate of the stria vascularis and the high metabolic activity necessary for maintaining the endolymph's ionic and electrical balance . Secondary hypoxia to the organ of Corti may cause further cochlear damage . Repeated cycles of ischemia and reperfusion, due to vaso‐occlusion by sickled RBCs or inadequate blood oxygen content, may result in labyrinthine hemorrhage, causing fibrosis, sclerosis, and labyrinthitis ossificans .…”

Section: Discussionmentioning

confidence: 99%

Prevalence and nature of hearing loss in a cohort of children with sickle cell disease

Towerman

Hayashi

et al. 2018

Pediatric Blood & Cancer

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Background: Sickle cell disease (SCD) may cause injury to any organ, including the auditory system. Although the association of SCD and hearing loss has been described, the nature of this complication is unknown. We sought to establish the prevalence and nature of hearing loss in a referred cohort of children with SCD and to identify correlating disease-or treatment-associated factors. Procedure:We conducted a retrospective review of patients with SCD < 22 years of age who had hearing evaluations between August 1990 and December 2014. Demographics, audiograms, and disease and treatment variables were analyzed.Results: Two hundred and ten audiograms among 81 patients were reviewed, and 189 were evaluable. Seventy-two children constituted the referred cohort. Fourteen (19.4%) had hearing loss documented on at least one audiogram. Seven (9.7%) patients had only conductive hearing loss, and the loss persisted for up to 10.3 years. The median age of first identification was eight years.Six (8.3%) patients had hearing loss that was at least partially sensorineural. One patient's hearing loss was ambiguous. All sensorineural hearing losses were unilateral and 4/6 patients had prior documented normal hearing, indicating acquired loss. No correlations were identified. Conclusions:Both conductive and sensorineural hearing losses are more prevalent in our study population than those observed in the general pediatric population. In children with SCD, sensorineural hearing loss appears to be acquired and unilateral. Conductive hearing loss was identified in older children and can persist. Serial screening is needed for early detection and more prompt intervention in this population.

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Sudden Sensorineural Hearing Loss Associated With Iron-Deficiency Anemia

Chung

Chen

Lin

et al. 2014

JAMA Otolaryngol Head Neck Surg

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IMPORTANCE Vascular events play a big part in the development of sudden sensorineural hearing loss (SSNHL), but only those associated with sickle-cell anemia have been previously associated with SSNHL. This study demonstrates an association between SSNHL and prior iron-deficiency anemia (IDA).OBJECTIVE To evaluate the association between IDA and SSNHL using a nationwide population-based database.DESIGN, SETTING, AND PARTICIPANTS In this case-control study in Taiwan, participants with SSNHL (n = 4004) were identified, and controls (n = 12 012) were randomly selected.MAIN OUTCOMES AND MEASURES Conditional logistic regression was used to calculate the ORs (95%CIs) for IDA in participants with SSNHL vs controls.RESULTS Of the 16 016 sampled participants, 533 (3.3%) had previously been diagnosed with IDA, including 172 (4.3%) participants with SSNHL and 361 (3.0%) controls. The χ2 test revealed a significant difference (P < .001) in the prevalence of prior IDA between participants with SSNHL and controls. By conditional logistic regression, we found that the OR for previous IDA among the participants with SSNHL was 1.34 (95%CI, 1.11-1.61) (P < .01)after adjusting for monthly income, geographic region, urbanization level, and comorbidities(ie, hypertension, diabetes, hyperlipidemia, renal disease, and coronary heart disease). The significant relationship between SSNHL and prior IDA was most pronounced among those 44 years or younger (adjusted OR, 1.91; 95%CI, 1.35-2.72) (P < .001) for the participants with SSNHL compared with controls, and the strength of this relationship decreased with age.CONCLUSIONS AND RELEVANCE There is an association between SSNHL and prior IDA.Patients with IDA, especially those younger than 60 years, should be more aggressively surveyed and managed to reduce hearing-related morbidities.

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Sickle Cell anemia and hearing loss among children and youngsters: literature review

Cited by 17 publications

References 16 publications

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

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

Prevalence and nature of hearing loss in a cohort of children with sickle cell disease

Sudden Sensorineural Hearing Loss Associated With Iron-Deficiency Anemia

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