Childhood Hearing Loss in Patients With Sickle Cell Disease in the United States

Schopper, Heather K.; D’esposito, Christopher F.; Muus, John S.; Kanter, Julie; Meyer, Ted A.

doi:10.1097/mph.0000000000001373

Cited by 10 publications

(5 citation statements)

References 26 publications

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“…Between 13% and 62% of tested patients are affected by hearing loss, with 6.6% to 13% having a component of sensorineural hearing loss. [50][51][52] FIGURE 3: Functional connectivity matrices per cohort. The intra-network spherical parcellations (on the diagonal) have a strong positive correlation compared with the inter-network spherical parcellations, showing that the functional network architecture is similar between the control (left) and sickle cell disease (SCD) cohort (middle).…”

Section: Discussionmentioning

confidence: 99%

“…We also found decreased connectivity within the auditory network, which is supported by mounting evidence that patients with SCD suffer hearing loss. Between 13% and 62% of tested patients are affected by hearing loss, with 6.6% to 13% having a component of sensorineural hearing loss 50–52 . Hearing loss has been associated with fcMRI changes, 53 and undiagnosed and untreated hearing loss can potentially impact cognitive development.…”

Section: Discussionmentioning

confidence: 99%

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Functional Connectivity Decreases with Metabolic Stress in Sickle Cell Disease

Fields

Mirro

Guilliams

et al. 2020

Annals of Neurology

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Objective: Children with sickle cell disease (SCD) experience cognitive deficits even when unaffected by stroke. Using functional connectivity magnetic resonance imaging (MRI) as a potential biomarker of cognitive function, we tested our hypothesis that children with SCD would have decreased functional connectivity, and that children experiencing the greatest metabolic stress, indicated by elevated oxygen extraction fraction, would have the lowest connectivity. Methods: We prospectively obtained brain MRIs and cognitive testing in healthy controls and children with SCD. Results: We analyzed data from 60 participants (20 controls and 40 with sickle cell disease). There was no difference in global cognition or cognitive subdomains between cohorts. However, we found decreased functional connectivity within the sensory-motor, lateral sensory-motor, auditory, salience, and subcortical networks in participants with SCD compared with controls. Further, as white matter oxygen extraction fraction increased, connectivity within the visual (p = 0.008, parameter estimate = −0.760 [95% CI = −1.297, −0.224]), default mode (p = 0.012, parameter estimate = −0.417 [95% CI = −0.731, −0.104]), and cingulo-opercular (p = 0.009, parameter estimate = −0.883 [95% CI = −1.517, −0.250]) networks decreased. Interpretation: We conclude that there is diminished functional connectivity within these anatomically contiguous networks in children with SCD compared with controls, even when differences are not seen with cognitive testing. Increased white matter oxygen extraction fraction was associated with decreased connectivity in select networks. These data suggest that elevated oxygen extraction fraction and disrupted functional connectivity are potentially presymptomatic neuroimaging biomarkers for cognitive decline in SCD.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Functional Connectivity Decreases with Metabolic Stress in Sickle Cell Disease

Fields

Mirro

Guilliams

et al. 2020

Annals of Neurology

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“…For example, electronic health records (EHRs) combine encounter records with problem lists of patient diagnoses recorded by clinicians, and studies have demonstrated the accuracy of EHR diagnosis codes for the identification of people living with SCD 7,8 . Similarly, health system databases can identify those with clinical diagnoses of SCD 9 . This critical review is intended to help researchers choose among algorithms in stand‐alone administrative databases lacking clinical or laboratory records to identify individuals, especially children and adolescents, living with SCD.…”

Section: Introductionmentioning

confidence: 99%

“…clinical diagnoses of SCD. 9 This critical review is intended to help researchers choose among algorithms in stand-alone administrative databases lacking clinical or laboratory records to identify individuals, especially children and adolescents, living with SCD.…”

mentioning

confidence: 99%

Administrative data identify sickle cell disease: A critical review of approaches in U.S. health services research

Grosse

Green

Reeves

2020

Pediatric Blood & Cancer

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To identify people living with sickle cell disease (SCD) and study their healthcare utilization, researchers can either use clinical records linked to administrative data or use billing diagnosis codes in stand-alone administrative databases. Correct identification of individuals clinically managed for SCD using diagnosis codes in claims databases is limited by the accuracy of billing codes in outpatient encounters. In this critical review, we assess the strengths and limitations of claims-based SCD case-finding algorithms in stand-alone administrative databases that contain both inpatient and outpatient records. Validation studies conducted using clinical records and newborn screening for confirmation of SCD case status have found that algorithms that require three or more nonpharmacy claims or one inpatient claim plus two or more outpatient claims with SCD codes show acceptable accuracy (positive predictive value and sensitivity) in children and adolescents. Future studies might seek to assess the accuracy of case-finding algorithms over the lifespan.

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“…Audiological data analyses were adapted from previous AudGenDB studies . Pure‐tone air‐ and bone‐conduction audiometry and sound‐field testing were used to evaluate hearing outcomes.…”

Section: Methodsmentioning

confidence: 99%

Characteristics and progression of hearing loss in children with turner's syndrome

et al. 2019

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Objectives/Hypothesis: Evaluate patterns of hearing impairment in children with Turner's syndrome (TS) and determine factors influencing severity and progression.Study Design: Retrospective database review. Methods: Demographic, audiological, and medical data for children with TS were extracted from the Audiological and Genetic Database to analyze patterns of hearing loss with comorbidities, demographics, and interventions.Results: Two hundred seventy-two children with TS were identified; 213 had audiological data. Of these children, 72.3% (N = 154) had hearing loss in which 84.4% (N = 130) was bilateral. In individual ears, conductive hearing loss was most common (28.7%, n = 73), followed by mixed (22.0%, n = 93) and sensorineural (7.9%, n = 20). Otitis media (odds ratio [OR] = 2.7, 95% confidence interval [CI]: 1.2-6.5), eustachian tube dysfunction (OR = 9.5, 95% CI: 3.2-35.2), and aortic valve anomalies were also associated with higher rates of hearing loss (OR = 3.6, 95% CI: 1.3-11.5). Of ears with quantifiable severity, 16.3% (n = 40) had moderate or worse hearing loss. Aortic coarctation (36.3 vs. 21.3 dB, P < .001), seizures (40.6 vs. 21.3 dB, P = .006), facial anomalies (32.5 vs. 21.3 dB, P = .029), and hypertension (36.3 vs. 21.3 dB, P = .015) portended more severe loss.Conclusions: Children with TS have high rates of hearing loss. High rates of sensorineural loss were unexpected. Nonotologic comorbidities, including seizures, hypertension, and anomalies of the kidney and aorta were associated with greater prevalence, severity, and progression of loss. Children with TS, particularly with the above comorbidities, are at risk for poor hearing outcomes, and should be offered audiological intervention to efficiently direct patient care.

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Childhood Hearing Loss in Patients With Sickle Cell Disease in the United States

Cited by 10 publications

References 26 publications

Functional Connectivity Decreases with Metabolic Stress in Sickle Cell Disease

Functional Connectivity Decreases with Metabolic Stress in Sickle Cell Disease

Administrative data identify sickle cell disease: A critical review of approaches in U.S. health services research

Characteristics and progression of hearing loss in children with turner's syndrome

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