High-frequency sensorineural hearing loss in children.

OBJECTIVES/HYPOTHESIS: Determine the prevalence of high-frequency sensorineural hearing loss (HFSNHL) in our hearing loss population and a diagnostic algorithm for these patients. STUDY DESIGN: Retrospective case series. METHODS: We identified patients diagnosed with sensorineural hearing loss (SNHL) at our pediatric tertiary care institution from 1981 to 2010. Based on audiometric profiles, these patients were subdivided into those with a flat SNHL configuration and those with HFSNHL. Imaging and genetic testing data and data regarding age at diagnosis, laterality, and risk factors were obtained for both groups. Comparisons were then made between the two groups. RESULTS: Of 2,867 patients included in the study, 7.6% had HFSNHL. Age at diagnosis was significantly higher in HFSNHL patients (8.3 years vs. 6.1 years; P < .0001). These patients also had a significantly higher proportion of unilateral versus bilateral loss (49.1% vs. 26.1%; P < .0001); unilateral losses were also less severe. Genetic testing showed no significant difference between groups in the proportion of patients tested or in those who tested positive. Similarly, imaging data revealed no difference in the proportion of patients tested in the two groups; however, overall diagnostic yield was significantly higher in flat SNHL patients (29.5% vs.17.3; P = .02). CONCLUSIONS: The positive predictive value of simple genetic testing is similar to that of imaging studies. However, given cost differences between genetic testing and imaging, it is prudent to perform genetic testing as the initial diagnostic test. Determination of whether high-throughput, multigene diagnostic platforms offer an added benefit in the evaluation of children requires further study. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:1236-1240, 2016.

Is the OSA-18 predictive of obstructive sleep apnea: comparison to polysomnography.

OBJECTIVES/HYPOTHESIS: To examine the ability of the OSA-18 to predict Obstructive Sleep Apnea (OSA) in a racially diverse population when compared to overnight polysomnography (PSG). STUDY DESIGN: Cross-sectional retrospective. METHODS: Children 2 to 12 years of age diagnosed with OSA who were treated at a tertiary care institution between 2008 and 2013 and had complete PSG and OSA-18 data were included. We performed logistic regression with OSA as the dependent variable and the OSA-18 total symptom score (TSS), age, gender, race, asthma, and body mass index (BMI) as independent variables. RESULTS: Seventy-nine children (32 females) were included (mean age 5.2 ± 2.4 years). The positive predictive value (PPV) was greater than 90 for an obstructive apnea-hypopnea index (oAHI) ≥ 1. The PPV and specificity were higher for white than for nonwhite children; however, sensitivity and negative predictive value (NPV) of OSA-18 TSS were low for mild, moderate, and severe OSA regardless of race. Age, race, and BMI were not significantly associated with oAHI. CONCLUSIONS: This study, conducted in a racially diverse cohort, examined the ability of the OSA-18 to predict OSA when compared to PSG-the gold standard-and found that sensitivity and NPV were extremely low for both white and nonwhite children. This suggests that the OSA-18 is not sufficiently sensitive to detect OSA nor sufficiently specific to determine the absence of OSA. The OSA-18 should be used as a quality-of-life indicator and is not a reliable substitute for PSG. LEVEL OF EVIDENCE: 4.

A diagnostic paradigm for childhood idiopathic sensorineural hearing loss.

OBJECTIVE: Our objective was to determine the diagnostic yield of laboratory testing, radiological imaging, and GJB2 mutation screening in a large cohort of patients with differing severities of idiopathic sensorineural hearing loss (SNHL). DESIGN AND SETTING: We undertook a retrospective study of patients presenting with SNHL at our institution from 1993 to 2002. RESULTS: Laboratory testing had an extremely low yield. Patients with unilateral SNHL had a significantly higher imaging yield than those with bilateral. The diagnostic yield of GJB2 screening was significantly higher in patients with severe to profound SNHL than in those with less severe SNHL. However, a relatively large number of patients with mild to moderate SNHL had positive GJB2 screens. CONCLUSIONS: Based on diagnostic yields, we propose a cost-effective stepwise diagnostic paradigm to replace the more commonly used and costly simultaneous testing approach. EBM RATING: C.