Polysomnographic Oxygen Saturation Findings for Preteen Children versus Adolescents.

Objective Home oximetry is commonly used to screen for obstructive sleep apnea (OSA) in children; however, normal oxygen desaturation levels by disease severity are not well known. It was our objective to determine if oxygen saturation levels differed by OSA severity category in children and if these differences were similar for preteen children and adolescents. Study Design Retrospective case series of children undergoing polysomnography from September 2011 to July 2015. Setting Tertiary pediatric hospital. Subjects and Methods Six- to 18-year-olds (preteen, 6-12 years old; adolescent, 13-18 years old). Chi-square, Wilcoxon rank sum test, and Kruskal-Wallis testing were used to compare variables between age groups. Results The study included 342 children with a mean age of 11.3 ± 2.4 years (range, 6.5-17.5) and a mean body mass index of 25.6 ± 9.2 kg/m2 (78 ± 29 percentile); 61% were white, 35% were black, and 4% were other or unknown. Of the children, 48% were female, and this was not a significant difference between age groups ( P = .81). Overall, 50% of the children had no OSA, 32% mild, 10% moderate, and 8% severe. When compared with the younger children, the adolescents had a longer sleep time ( P = .014) and a higher mean obstructive apnea-hypopnea index (3.53 ± 5.1 vs 3.03 ± 6.1 events per hour, P = .02). The 3% and 4% oxygen desaturation indices were not significantly different between age groups when accounting for OSA severity. Conclusion Adolescents have longer sleep times and higher obstructive apnea-hypopnea indexes than preteens, but oxygen saturations and desaturation indices were similar. This supports current triage algorithms for children with OSA, as we found no significant age-based differences.

Hearing loss in Down Syndrome revisited - 15 years later.

OBJECTIVE: In 2001, the senior authors published a study investigating hearing loss in young children (ages 11 months to 3.8 years) with Down Syndrome (DS). We re-visit this same study population to review current audiologic status, the incidence of pressure equalization tube (PET) placement(s), and rate of tympanic membrane (TM) perforations. We aim to better understand the natural history of ear disease and hearing loss in DS and assess potential complications. METHODS: This retrospective chart review included 57 children with DS who previously completed in 2006, a 5 year, longitudinal study investigating otolaryngologic problems in DS. Updated audiologic data was available for 54. Audiograms, age of ear specific testing, PET placement(s), and tympanic membrane(TM) descriptions were reviewed. RESULTS: Ages ranged from 14 to 18 years (mean 16.34 years). PET placement occurred in 88.8%, with mean of 3.5 procedures. 30% of PET's were placed after age 6. Ear specific testing was obtained in 92.5% (mean age 4.54 years). Normal hearing was present in 44% (right ear) and 38% (left ear). "Functional" hearing levels, defined as normal or mild hearing loss and speech reception threshold ≤ 30 dB, occurred in 83.3%. Sensorineural/mixed hearing loss was present in 11% (right ear) and 9% (left ear). TM perforations rate was 17%. No cholesteatomas were found. CONCLUSION: Chronic otitis media and indications for PET's persist as children with DS age. Although functional hearing occurred in 83.3%, there was an overall decrease in hearing levels as the children aged. Tympanic membrane perforations occurred in 17%. Continued surveillance of otologic and audiologic status in patients with Down syndrome is recommended.

Hearing loss progression and contralateral involvement in children with unilateral sensorineural hearing loss.

OBJECTIVES: We undertook this study to determine the rate of hearing loss progression in the affected ear of children with unilateral sensorineural hearing loss and without an enlarged vestibular aqueduct, and the rate of new-onset hearing loss in the contralateral ear. METHODS: We searched the database at our pediatric tertiary care center to identify patients who met the inclusion criteria, examining demographic variables, audiometric data, and presumptive causes. RESULTS: We identified 198 patients. At presentation, they showed slight left-sided and male predominances. Of 142 patients who had sufficient audiometric follow-up for us to evaluate progression, 21% showed ipsilateral progression and 10.6% developed new-onset hearing loss in the contralateral ear. Isolated high-frequency loss was identified in 11 patients (5.6%), 8 of whom had sufficient follow-up for us to identify progression. Two showed progression; 4 others with progression in the ipsilateral ear developed new-onset high-frequency loss in the contralateral ear. Temporal bone anomalies were identified in 26 children (13%), and these children were more likely to have profound hearing loss than were those without temporal bone anomalies (46% versus 23%). CONCLUSIONS: The findings suggest that unilateral sensorineural hearing loss may not always be a unilateral process, but that it may be the initial manifestation of bilateral auditory dysfunction.

Audiologic and temporal bone imaging findings in patients with sensorineural hearing loss and GJB2 mutations.

OBJECTIVES/HYPOTHESIS: Our objectives were to determine genotype-phenotype correlations in patients with sensorineural hearing loss (SNHL) who undergo testing for GJB2 mutations and to examine the relationship of temporal bone anomalies seen on computed tomography (CT) and GJB2 mutations. STUDY DESIGN: We conducted a retrospective review of all children diagnosed with SNHL and who underwent GJB2 testing from 1997 to 2006. RESULTS: Of 840 patients, 146 (17.4%) had mutations. Seventy-six (9.1%) had biallelic GJB2 mutations and 70 (8.3%) had heterozygous mutations. When biallelic mutations were categorized as missense or nonsense mutations, the presence of at least one missense mutation was associated with mild or moderate SNHL. Biallelic nonsense mutations were associated with severe to profound SNHL. Among patients with GJB2 mutations, those with heterozygous mutations (n = 14 [20%]) had a higher rate of asymmetric SNHL loss than those with biallelic mutations (n = 6 [7.9%], P = .03). Those with heterozygous mutations were more likely to experience progression than were those with biallelic mutations, though this difference was only marginally significant (26.5% vs. 12.3%, respectively; P = .06). Patients who were wild type for GJB2 were more likely to have an enlarged vestibular aqueduct (EVA) than were those with biallelic and heterozygous mutations (29% vs. 11.9%, respectively; P = .004). Compared to patients who were wild type, those with biallelic mutations had a significantly lower rate of EVA. CONCLUSIONS: This is the largest single-institution study of pediatric patients with GJB2 mutations and SNHL. The functional consequences of GJB2 mutations correlated with the degree of hearing loss. Patients with M34T mutations and/or mild SNHL had a low risk of progression. Temporal bone anomalies were uncommon in patients with GJB2 mutations.