Academic Outcomes with Hearing Amplification Devices in Children with Unilateral Hearing Loss: A Systematic Review and Narrative Synthesis.

BACKGROUND: Many studies have shown increased academic problems in children with unilateral hearing loss (UHL). However, whether hearing devices can ameliorate the educational difficulties associated with UHL is not well studied. Therefore, the objective of the current systematic review was to answer the question: do nonsurgical amplification devices, bone-anchored hearing aids, and/or cochlear implants improve academic outcomes in school-aged children and adolescents with UHL? METHODS: Embase, MEDLINE, Scopus, CINAHL, APA PsycInfo, ClinicalTrials.gov, and Cochrane databases were searched from inception to December 21, 2022. Published, peer-reviewed studies comparing academic outcomes in patients with UHL aged ≥5 and ≤19 years with and without hearing devices (nonsurgical amplification devices, bone-anchored hearing aids, or cochlear implants) were included. Results of studies were qualitatively synthesized, and the risk of bias was evaluated with the Effective Public Health Practice Project (EPHPP) Quality Assessment Tool. RESULTS: A total of 5,644 non-duplicate publications were identified by the search, and four studies were included for synthesis, every one of which was investigating nonsurgical amplification. One small, single-arm study demonstrated significant improvement in subjective classroom listening difficulties after a 3- to 4-month trial with a behind-the-ear hearing aid. The other three studies of nonsurgical amplification devices showed no benefit across multiple academic outcomes with FM systems and conventional and CROS-style hearing aids. DISCUSSION: The small sample sizes, heterogeneous and/or ill-defined study samples, and overall low quality of the available literature ultimately make it hard to draw definitive conclusions regarding nonsurgical amplification devices' effectiveness in improving academic outcomes in children with UHL. No articles were identified that studied cochlear implants or bone-anchored hearing aids. Further studies with high-quality study design, large sample sizes, and long-term follow-up are needed to answer this clinically important question.

Frequency of QTc Interval Prolongation in Children and Adults with Williams Syndrome.

QTc prolongation (≥ 460 ms), according to Bazett formula (QTcB), has been identified to be increased in Williams syndrome (WS) and suggested as a potential cause of increased risk of sudden cardiac death. The Bazett formula tends to overestimate QTc in higher heart rates. We performed a retrospective chart review of WS patients with ≥ 1 electrocardiogram (EKG) with sinus rhythm, no evidence of bundle branch blocks, and measurable intervals. A total of 280 EKGs from 147 patients with WS were analyzed and 123 EKGs from 123 controls. The QTc was calculated using Bazett formula. The average QTcB for individuals with WS and controls was 444 ± 24 ms and 417 ± 26 ms, respectively (p < 0.001). In our WS cohort 34.4% had at least 1 EKG with a QTcB ≥ 460 ms. The mean heart rate (HR) from patients with WS was significantly higher than controls (96 bpm vs 76 bpm, p < 0.001). Linear regression showed that HR contributed 27% to QTcB prolongation in the patients with WS. Patients with WS have a mean QTcB in the normal range but higher than controls, and a higher than expected frequency of QTc ≥ 460 ms compared to the general population. HR is also higher in WS and contributes modestly to the WS QTcB prolongation. Future studies are needed to assess if these findings contribute risk to sudden cardiac death but in the interim we recommend routine EKG testing, especially when starting QTc prolonging medications.

Hypercalcemia in Patients with Williams-Beuren Syndrome.

OBJECTIVE: To evaluate the timing, trajectory, and implications of hypercalcemia in Williams-Beuren syndrome (WBS) through a multicenter retrospective study. STUDY DESIGN: Data on plasma calcium levels from 232 subjects with WBS aged 0-67.1 years were compared with that in controls and also with available normative data. Association testing was used to identify relevant comorbidities. RESULTS: On average, individuals with WBS had higher plasma calcium levels than controls, but 86.7% of values were normal. Nonpediatric laboratories overreport hypercalcemia in small children. When pediatric reference intervals were applied, the occurrence of hypercalcemia dropped by 51% in infants and by 38% in toddlers. Across all ages, 6.1% of the subjects had actionable hypercalcemia. In children, actionable hypercalcemia was seen in those aged 5-25 months. In older individuals, actionable hypercalcemia was often secondary to another disease process. Evidence of dehydration, hypercalciuria, and nephrocalcinosis were common in both groups. Future hypercalcemia could not be reliably predicted by screening calcium levels. A subgroup analysis of 91 subjects found no associations between hypercalcemia and cardiovascular disease, gastrointestinal complaints, or renal anomalies. Analyses of electrogradiography data showed an inverse correlation of calcium concentration with corrected QT interval, but no acute life-threatening events were reported. CONCLUSIONS: Actionable hypercalcemia in patients with WBS occurs infrequently. Although irritability and lethargy were commonly reported, no mortality or acute life-threatening events were associated with hypercalcemia and the only statistically associated morbidities were dehydration, hypercalciuria, and nephrocalcinosis.

Are children with unilateral hearing loss more tired?

OBJECTIVE: To determine whether children with unilateral sensorineural hearing loss (USNHL) and unilateral conductive hearing loss (UCHL) have higher levels of fatigue than literature reported normal hearing (LRNH) children. METHODS: This was a cross-sectional survey utilizing the PedsQL™ Multidimensional Fatigue Scale administered to children with unilateral hearing loss (UHL) and their parents at two tertiary care academic medical centers and a nationwide microtia/atresia conference. The PedsQL™ Multidimensional Fatigue Scale was used to compare child and parental proxy reports of fatigue among USNHL, UCHL, and LRNH children. ANOVA and post-hoc Tukey Honest Significant Difference testing were used for statistical analysis. RESULTS: Of 69 children included in the study, 42 had UCHL (61%) and 27 (39%) had USNHL. Children with USNHL reported more total fatigue (mean 69.1, SD 19.3) than LRNH children (mean 80.5, SD 13.3; difference -11.4; 95% CI: -19.98 to -2.84) and children with UCHL (mean 78.0, SD 14.5; difference -8.95; 95% CI: -17.86 to 0.04). Children with UCHL reported similar levels of fatigue compared to LRNH children (difference -2.5; 95% CI: -9.95 to 5.03). Parents of children with USNHL reported greater levels of fatigue (mean 67.6, SD 22.6) in their children than parents of LRNH children (mean 89.6, SD 11.4; difference -22.0; 95% CI: -29.8 to -14.3) and parents of children with UCHL (mean 76.2, SD 17.3; difference -8.6; 95% CI: -17.5 to 0.21). Parents of children with UCHL also report higher levels of fatigue than parents of LRNH children (difference -13.4; 95% CI: -19.98 to -6.84). CONCLUSIONS: Children with USNHL reported greater levels of fatigue than LRNH children and children with UCHL. Results implicate cognitive load as an important consideration in children with hearing loss. The measurement of fatigue may be a useful indicator to determine the benefit of intervention (e.g., amplification) for these children.

Overview of Medical Evaluation of Unilateral and Bilateral Hearing Loss in Children.

Early identification, treatment, and medical evaluation of childhood hearing loss are essential to promoting language and social development, regardless of their age of presentation. Evaluation of hearing loss in children should prioritize reversible and treatable causes. Multiple algorithms have been established to address the changing prevalence of genetic or infectious contributions to hearing loss and include recommendations on laboratory testing, imaging, and genetic testing. Despite these recommendations, significant practice variation remains on assessing the etiology of hearing loss in children.

Fatigue in Children With Unilateral and Bilateral Hearing Loss.

OBJECTIVE: To determine whether children with unilateral hearing loss (UHL) experience similar levels fatigue as children with bilateral hearing loss (BHL) or normal-hearing (NH). DESIGN: Cross-sectional study. SETTING: Two tertiary care otolaryngology practices. PARTICIPANTS: Children, 5 to 18 years old, with UHL or BHL and their parents. MAIN OUTCOME MEASURES: PedsQL Multidimensional Fatigue Scale (MFS) survey. RESULTS: Overall response rate was 90/384 (23%). Mean age of child participants was 10.7 years old (standard deviations [SD] 3.1); 38 (42%) were men and 52 (58%) were women. Sixty-nine (77%) children had UHL, 21 (23%) had BHL. Children with BHL (mean 65, SD 21) and UHL (mean 75, SD 17) reported greater levels of fatigue than children with NH (BHL difference -15, 95% confidence interval [CI] -25 to -5; UHL difference -6, 95% CI -13-1.2). Parent-proxy reports for children with BHL (mean 67, SD 20) and UHL (mean 76, SD 20) reported more fatigue than NH (BHL difference -22, 95% CI -33 to -12; UHL difference -14; 95% CI -20 to -8). Sub-section scores for general, sleep, and cognitive fatigue were higher for children with BHL and UHL than NH. CONCLUSION AND RELEVANCE: Children with UHL and BHL reported significantly more fatigue than children with NH, and children with BHL reported more fatigue than UHL. These findings underscore the need to increase auditory rehabilitation and educational resources for children with UHL and support the use of the PedsQL MFS questionnaire as a measure to follow disability experienced by children with HL as they undergo hearing rehabilitation.

Mild macrocytosis in Williams-Beuren syndrome.

OBJECTIVE: To evaluate the occurrence and estimate the frequency of macrocytosis in Williams-Beuren syndrome (WBS). STUDY DESIGN: Complete blood count (CBC) data from 179 subjects with WBS aged 1-69 were collected, with common parameters assessed for trends. Z-transformed mean corpuscular volume (MCV) was compared with each laboratory's reference range as well as with control data from the National Health and Nutrition Examination Survey (NHANES) 2013-2014 data archives. RESULTS: Just over a third (35%) subjects had at least one recorded incidence of macrocytosis. In comparisons of CBC parameters with an expected population mean, MCV and MCH were greater than, while Hct and RDW were lower than, expected values. The distribution of erythrocyte MCV is shifted to the right in WBS compared to controls, as was the mean value. Despite this, anemia was absent, except in a single medically complex WBS subject. Though there was a paucity of data available of variables that could potentially cause an elevated MCV, no obvious etiology could be elucidated. CONCLUSIONS: Mild macrocytosis without anemia affects a moderate subset of WBS patients, leading to a rightward shift in the MCV distribution curve. Providers encountering isolated mild macrocytosis in WBS can consider observation over further workup.

Association of Preoperative Functional Performance With Outcomes After Surgical Treatment of Head and Neck Cancer: A Clinical Severity Staging System.

Importance: Patients with head and neck cancers have comorbidities and other constitutional symptoms known to be associated with adverse postoperative outcomes, but the role of functional performance is not well studied. Objective: To explore the addition of functional performance to other clinical factors for association with 3 patient outcomes: 30-day unplanned readmission (UR), 90-day medical complications, and overall survival (OS). Design, Setting, and Participants: This retrospective cohort study was conducted in a single tertiary care center with patients surgically treated for squamous cell cancer of the lip, oral cavity, pharynx, or larynx from January 2012 to December 2016. All analysis took place between January 2018 and November 2018. Data from 2 registries were analyzed, supplemented with medical record review. Logistic regression analysis was used to explore association of preoperative functional performance with outcomes. Conjunctive consolidation was used to create a useful clinical severity staging system, which included functional performance (estimated from metabolic equivalent [MET] score: <4, light-intensity activities; ≥4 at least moderate-intensity activities); overall comorbidity severity; preoperative weight loss; and TNM tumor staging. Logistic regression was used to assess the prognostic accuracy of the clinical severity staging system for 30-day UR and 90-day complications, and Cox proportional hazard regression for OS. Exposures: All patients underwent surgical treatment for head and neck cancer. Main Outcomes and Measures: The primary outcomes were 30-day UR and 90-day complications; the secondary outcome was OS. Results: For the 657 patients included, the mean (SD) age was 62.0 (11.3) years; 73% were men (n = 477), and 88% were white (n = 580). A total of 75 (11%) had a 30-day UR; 204 (31%) developed a 90-day complication; and 127 (19%) patients died during the observation period. Individually, poor functional performance (<4 METs), high comorbidity burden, preoperative weight loss, and advanced TNM stage were associated with all 3 outcomes; the increased risk for each outcome ranged from 1.5 to 3.0 times the reference range. Using these 4 variables in combination, the 4-category clinical severity staging system demonstrated a strong association between severity stage and all 3 adverse outcomes: 30-day UR (C statistic, 0.63), 90-day complications (C statistic, 0.63), and OS (C statistic, 0.68). Conclusions and Relevance: Poor preoperative functional performance, high comorbidity burden, preoperative weight loss, and advanced tumor stage were all associated with worse patient outcomes after head and neck cancer surgery. The model incorporating all 4 of these factors developed in this study may facilitate patient-centered risk assessment and patient-physician shared preoperative decision making.

Identification of Retinal Vascular Lesions Using Ultra-Widefield Angiography in Hereditary Hemorrhagic Telangiectasia Patients.

PURPOSE: To determine the presence and to characterize location of retinal vascular lesions in patients with hereditary hemorrhagic telangiectasia (HHT). DESIGN: Prospective cross-sectional pilot descriptive study. PARTICIPANTS: Eighteen patients (age range, 22-65 years) with a clinical diagnosis of HHT. METHODS: Patients completed the 25-item National Eye Institute Visual Function Questionnaire and underwent a single study visit with dilated ophthalmic examination, OCT angiography (OCTA), and fluorescein angiography (FA) with widefield imaging. MAIN OUTCOME MEASURES: Presence of retinal vascular abnormalities in 1 or more quadrants identified on widefield FA, Visual Function Questionnaire scores, retinal vessel architecture on FA and OCTA, and dilated ophthalmic examination findings. RESULTS: Of the 18 patients recruited, fine telangiectatic vessels with capillary dilation and tortuosity were identified in 78% by FA imaging. CONCLUSIONS: In the first FA and OCTA analysis of the retina of unrelated HHT patients, we found a high rate of temporal and nasal telangiectasias. These telangiectasias were more apparent in older patients, suggesting that they may appear in later stages of HHT development. No abnormalities of the macular vasculature and architecture were identified, explaining the generally well-preserved visual acuity. Temporal and nasal telangiectasias may have clinical significance in a patient's risk for retinal hemorrhage and likely warrant periodic surveillance by annual FA imaging.