Spectral Resolution and Speech Perception in Cochlear Implanted School-Aged Children.

OBJECTIVE: Cochlear implantation of prelingually deaf infants provides auditory input sufficient to develop spoken language; however, outcomes remain variable. Inability to participate in speech perception testing limits testing device efficacy in young listeners. In postlingually implanted adults (aCI), speech perception correlates with spectral resolution an ability that relies independently on frequency resolution (FR) and spectral modulation sensitivity (SMS). The correlation of spectral resolution to speech perception is unknown in prelingually implanted children (cCI). In this study, FR and SMS were measured using a spectral ripple discrimination (SRD) task and were correlated with vowel and consonant identification. It was hypothesized that prelingually deaf cCI would show immature SMS relative to postlingually deaf aCI and that FR would correlate with speech identification. STUDY DESIGN: Cross-sectional study. SETTING: In-person, booth testing. METHODS: SRD was used to determine the highest spectral ripple density perceived at various modulation depths. FR and SMS were derived from spectral modulation transfer functions. Vowel and consonant identification was measured; SRD performance and speech identification were analyzed for correlation. RESULTS: Fifteen prelingually implanted cCI and 13 postlingually implanted aCI were included. FR and SMS were similar between cCI and aCI. Better FR was associated with better speech identification for most measures. CONCLUSION: Prelingually implanted cCI demonstrated adult-like FR and SMS; additionally, FR correlated with speech identification. FR may be a measure of CI efficacy in young listeners.

Behavioral characterization of the cochlear amplifier lesion due to loss of function of stereocilin (STRC) in human subjects.

Loss of function of stereocilin (STRC) is the second most common cause of inherited hearing loss. The loss of the stereocilin protein, encoded by the STRC gene, induces the loss of connection between outer hair cells and tectorial membrane. This only affects the outer hair cells (OHCs) function, involving deficits of active cochlear frequency selectivity and amplifier functions despite preservation of normal inner hair cells. Better understanding of cochlear features associated with mutation of STRC will improve our knowledge of normal cochlear function, the pathophysiology of hearing impairment, and potentially enhance hearing aid and cochlear implant signal processing. Nine subjects with homozygous or compound heterozygous loss of function mutations in STRC were included, age 7-24 years. Temporal and spectral modulation perception were measured, characterized by spectral and temporal modulation transfer functions. Speech-in-noise perception was studied with spondee identification in adaptive steady-state noise and AzBio sentences with 0 and -5 dB SNR multitalker babble. Results were compared with normal hearing (NH) and cochlear implant (CI) listeners to place STRC-/- listeners' hearing capacity in context. Spectral ripple discrimination thresholds in the STRC-/- subjects were poorer than in NH listeners (p < 0.0001) but remained better than for CI listeners (p < 0.0001). Frequency resolution appeared impaired in the STRC-/- group compared to NH listeners but did not reach statistical significance (p = 0.06). Compared to NH listeners, amplitude modulation detection thresholds in the STRC-/- group did not reach significance (p=  0.06) but were better than in CI subjects (p < 0.0001). Temporal resolution in STRC-/- subjects was similar to NH (p = 0.98) but better than in CI listeners (p = 0.04). The spondee reception threshold in the STRC-/- group was worse than NH listeners (p = 0.0008) but better than CI listeners (p = 0.0001). For AzBio sentences, performance at 0 dB SNR was similar between the STRC-/- group and the NH group, 88 % and 97 % respectively. For -5 dB SNR, the STRC-/- performance was significantly poorer than NH, 40 % and 85 % respectively, yet much better than with CI who performed at 54 % at +5 dB SNR in children and 53 % at + 10 dB SNR in adults. To our knowledge, this is the first study of the psychoacoustic performance of human subjects lacking cochlear amplification but with normal inner hair cell function. Our data demonstrate preservation of temporal resolution and a trend to impaired frequency resolution in this group without reaching statistical significance. Speech-in-noise perception compared to NH listeners was impaired as well. All measures were better than those in CI listeners. It remains to be seen if hearing aid modifications, customized for the spectral deficits in STRC-/- listeners can improve speech understanding in noise. Since cochlear implants are also limited by deficient spectral selectivity, STRC-/- hearing may provide an upper bound on what could be obtained with better temporal coding in electrical stimulation.

Predictors of Time Spent in Special Education Among Students With Severe-to-Profound Hearing Loss.

OBJECTIVE: Determine if students with severe-to-profound hearing loss with cochlear implants (CIs) mainstream (transition to general education) more than students with hearing amplification at the population level. STUDY DESIGN: Cross-sectional secondary analysis of data from the National Center of Education Statistics. SETTING: Special education (SpEd) students in the United States who had severe to profound "hearing impairment" and were 6 to 16 years old at enrollment from 2000 to 2001. METHODS: We weighted the data to produce national estimates, performed multiple imputations for missingness, and built a multivariate linear regression model, which was cross-validated with a multivariate Poisson regression model. We used a theory-based approach to model-building using a directed acyclic graph to identify the minimally sufficient adjustment set of variables, which included school district urbanicity, student's age when they started SpEd, other disabilities, home language, and caregiver education. RESULTS: We identified 7267 students with CIs and 28,794 students with hearing amplification. CI users mainstreamed more than peers using hearing amplification during secondary school (40.29% less daily time in special education, p = .004) but not during primary school (9.19% less daily time in SpEd, p = .155). Additional significant predictors of mainstreaming varied between the primary and secondary school cohorts and included school district urbanicity and the student's age when they started SpEd. CONCLUSION: CI status predicts daily time spent in SpEd among a secondary school cohort. These findings do not establish causation. The National Center of Education Statistics should consider linking to clinical databases in future studies.

Association of Genetic Diagnoses for Childhood-Onset Hearing Loss With Cochlear Implant Outcomes.

Importance: In the US, most childhood-onset bilateral sensorineural hearing loss is genetic, with more than 120 genes and thousands of different alleles known. Primary treatments are hearing aids and cochlear implants. Genetic diagnosis can inform progression of hearing loss, indicate potential syndromic features, and suggest best timing for individualized treatment. Objective: To identify the genetic causes of childhood-onset hearing loss and characterize severity, progression, and cochlear implant success associated with genotype in a single large clinical cohort. Design, Setting, and Participants: This cross-sectional analysis (genomics) and retrospective cohort analysis (audiological measures) were conducted from 2019 to 2022 at the otolaryngology and audiology clinics of Seattle Children's Hospital and the University of Washington and included 449 children from 406 families with bilateral sensorineural hearing loss with an onset younger than 18 years. Data were analyzed between January and June 2022. Main Outcomes and Measures: Genetic diagnoses based on genomic sequencing and structural variant analysis of the DNA of participants; severity and progression of hearing loss as measured by audiologic testing; and cochlear implant success as measured by pediatric and adult speech perception tests. Hearing thresholds and speech perception scores were evaluated with respect to age at implant, months since implant, and genotype using a multivariate analysis of variance and covariance. Results: Of 406 participants, 208 (51%) were female, 17 (4%) were African/African American, 32 (8%) were East Asian, 219 (54%) were European, 53 (13%) were Latino/Admixed American, and 16 (4%) were South Asian. Genomic analysis yielded genetic diagnoses for 210 of 406 families (52%), including 55 of 82 multiplex families (67%) and 155 of 324 singleton families (48%). Rates of genetic diagnosis were similar for children of all ancestries. Causal variants occurred in 43 different genes, with each child (with 1 exception) having causative variant(s) in only 1 gene. Hearing loss severity, affected frequencies, and progression varied by gene and, for some genes, by genotype within gene. For children with causative mutations in MYO6, OTOA, SLC26A4, TMPRSS3, or severe loss-of-function variants in GJB2, hearing loss was progressive, with losses of more than 10 dB per decade. For all children with cochlear implants, outcomes of adult speech perception tests were greater than preimplanted levels. Yet the degree of success varied substantially by genotype. Adjusting for age at implant and interval since implant, speech perception was highest for children with hearing loss due to MITF or TMPRSS3. Conclusions and Relevance: The results of this cross-sectional study suggest that genetic diagnosis is now sufficiently advanced to enable its integration into precision medical care for childhood-onset hearing loss.

Spectrotemporal Modulation Discrimination in Infants With Normal Hearing.

OBJECTIVES: Spectral resolution correlates with speech understanding in post-lingually deafened adults with cochlear implants (CIs) and is proposed as a non-linguistic measure of device efficacy in implanted infants. However, spectral resolution develops gradually through adolescence regardless of hearing status. Spectral resolution relies on two different factors that mature at markedly different rates: Resolution of ripple peaks (frequency resolution) matures during infancy whereas sensitivity to across-spectrum intensity modulation (spectral modulation sensitivity) matures by age 12. Investigation of spectral resolution as a clinical measure for implanted infants requires understanding how each factor develops and constrains speech understanding with a CI. This study addresses the limitations of the present literature. First, the paucity of relevant data requires replication and generalization across measures of spectral resolution. Second, criticism that previously used measures of spectral resolution may reflect non-spectral cues needs to be addressed. Third, rigorous behavioral measurement of spectral resolution in individual infants is limited by attrition. To address these limitations, we measured discrimination of spectrally modulated, or rippled, sounds at two modulation depths in normal hearing (NH) infants and adults. Non-spectral cues were limited by constructing stimuli with spectral envelopes that change in phase across time. Pilot testing suggested that dynamic spectral envelope stimuli appeared to hold infants' attention and lengthen habituation time relative to previously used static ripple stimuli. A post-hoc condition was added to ensure that the stimulus noise carrier was not obscuring age differences in spectral resolution. The degree of improvement in discrimination at higher ripple depth represents spectral frequency resolution independent of the overall threshold. It was hypothesized that adults would have better thresholds than infants but both groups would show similar effects of modulation depth. DESIGN: Participants were 53 6- to 7-month-old infants and 23 adults with NH with no risk factors for hearing loss who passed bilateral otoacoustic emissions screening. Stimuli were created from complexes with 33- or 100-tones per octave, amplitude-modulated across frequency and time with constant 5 Hz envelope phase-drift and spectral ripple density from 1 to 20 ripples per octave (RPO). An observer-based, single-interval procedure measured the highest RPO (1 to 19) a listener could discriminate from a 20 RPO stimulus. Age-group and stimulus pure-tone complex were between-subjects variables whereas modulation depth (10 or 20 dB) was within-subjects. Linear-mixed model analysis was used to test for the significance of the main effects and interactions. RESULTS: All adults and 94% of infants provided ripple density thresholds at both modulation depths. The upper range of threshold approached 17 RPO with the 100-tones/octave carrier and 20 dB depth condition. As expected, mean threshold was significantly better with the 100-tones/octave compared with the 33-tones/octave complex, better in adults than in infants, and better at 20 dB than 10 dB modulation depth. None of the interactions reached significance, suggesting that the effect of modulation depth on the threshold was not different for infants or adults. CONCLUSIONS: Spectral ripple discrimination can be measured in infants with minimal listener attrition using dynamic ripple stimuli. Results are consistent with previous findings that spectral resolution is immature in infancy due to immature spectral modulation sensitivity rather than frequency resolution.

Sensitivity to Deaf Culture Among Otolaryngology and Audiology Trainees.

OBJECTIVE: The Deaf community is an ethnolinguistic minority group. Low sensitivity to Deaf culture contributes to health disparities among Deaf patients. This study determines the level of sensitivity to Deaf culture among otolaryngology-head and neck surgery (OHNS) and audiology trainees. METHODS: Cross-sectional survey study of OHNS and audiology trainees from 10 large US institutions. Trainees were queried on their exposure to and comfort with Deaf patients and their education on, attitude toward, and awareness and knowledge of Deaf culture. Sensitivity to Deaf culture was operationalized as awareness and knowledge of Deaf culture. These were assessed using a 35-item instrument that was previously developed using a d/Deaf community-based participatory approach to research. We used T-tests to compare the sample to previous samples of medical students with training in Deaf culture (MS-TDCs) and general practitioners (GPs). RESULTS: There were 91 completed surveys (response rate 44.5%). Almost all were aware of Deaf culture (97.8%). The mean knowledge score was 55.0% (standard deviation (SD) 13.4%), which was significantly higher than that for GPs at 43.0% (SD 15.0%) (95% confidence interval 8.1%, 15.8%, P < .0001) but significantly lower than that for MS-TDCs at 69.0% (SD 13.0%)(CI -20.3%, -7.6%, P < .0001). Knowledge scores were comparable for OHNS and audiology trainees (P = .09). CONCLUSION: This sample of OHNS and audiology trainees was more sensitive to Deaf culture than GPs but less sensitive than MS-TDCs. Developing specialty-specific education may be warranted. LEVEL OF EVIDENCE: 4.

Beyond Laryngeal Clefts: Interarytenoid Injection Augmentation to Predict Success of Suture Augmentation in Children.

OBJECTIVE: To assess the efficacy of interarytenoid injection augmentation (IAIA) and the ability of IAIA to predict response to interarytenoid suture augmentation (IASA) based on diet advancement on video fluoroscopic swallow studies (VFSS). METHODS: Retrospective cohort analysis of patients with persistent pharyngeal dysphagia at a tertiary children's hospital with VFSS pre- and post-IAIA were included between March 2011 and June 2019. RESULTS: Median age of the 229 patients was 2.2 years (5.8 months-19 years). Interarytenoid mucosal height (IAMH) was found to be above the false vocal folds in 112 patients (53.4%) and at true vocal folds in 10 (4.9%) patients. On VFSS post-IAIA, 95 (41.5%) patients were successfully advanced in recommended diet consistency, 115 (50.2%) were stable, and 19 (8.3%) needed thicker consistency. Paired t-tests on pre- and post-operative consistency scores showed significant improvement, p-value of <0.0001, 95% confidence interval (CI; 0.50-0.85). Poisson regression found no covariates with significant association with improvement on IAIA. For IASA patients, 35/60 (58.3%) improved on post-op VFSS. Paired t-tests on pre- and post-operative consistency scores showed significant improvement, p-value of <0.0001, 95% CI (0.63-1.33). Positive predictive value for IAIA predicting response to IASA was 77% with positive likelihood ratio of 2.3. The response to IAIA versus no response to IAIA likelihood ratios were found to have a statistically significant difference (p < 0.05). CONCLUSIONS: Our study suggests IAIA yields objective improvement in swallow function on VFSS in nearly half of our patients and may be a reliable diagnostic tool to predict response to IASA in patients with persistent pharyngeal dysphagia with or without a laryngeal cleft. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:1749-1756, 2023.

Educational Environments and Secondary School Outcomes Among Students Who Are D/deaf and Hard of Hearing in Special Education.

PURPOSE: The purpose of this study is to provide updated national estimates on the annual number, educational environments, and secondary school outcomes of students who are D/deaf and hard of hearing (D/HH) receiving special education (SpEd) and related services in the United States. METHOD: We performed a retrospective cross-sectional descriptive analysis of Individuals with Disabilities Education Act, Part B, Section 618 data from 2012 to 2018. Participants included students 6-21 years old in SpEd with "hearing impairment" reported as their primary disability. The general population of students in secondary school served as a comparator, via Current Population Survey data. We described the annual number of students (a) overall, (b) by educational environment, and (c) by reason for exiting SpEd, including the proportion graduating from and dropping out of secondary school. We described variation over time. RESULTS: The median annual number of students was 67,655, with minimal variation by year. The proportion in general education (GenEd) for ≥ 80% of the day increased by 4.2% over 6 years from 57.8% to 62.0%, whereas the proportions in GenEd for < 40% and 40%-79% of the day decreased by 1.6% and 1.3%, respectively. Proportions in the remainder of the environments changed < 1.0% each. Of exiters, 86.8% of students graduated, whereas 3.9% dropped out, compared to a dropout rate of 5.0% in the general population. CONCLUSION: From 2012 to 2018, students who are D/HH receiving SpEd in the United States have spent increasingly more time in GenEd, most graduated from high school, and few dropped out, with dropout patterns appearing similar to the general population.

Spectral Resolution Development in Children With Normal Hearing and With Cochlear Implants: A Review of Behavioral Studies.

PURPOSE: This review article provides a theoretical overview of the development of spectral resolution in children with normal hearing (cNH) and in those who use cochlear implants (CIs), with an emphasis on methodological considerations. The aim was to identify key directions for future research on spectral resolution development in children with CIs. METHOD: A comprehensive literature review was conducted to summarize and synthesize previously published behavioral research on spectral resolution development in normal and impaired auditory systems. CONCLUSIONS: In cNH, performance on spectral resolution tasks continues to improve through the teenage years and is likely driven by gradual maturation of across-channel intensity resolution. A small but growing body of evidence from children with CIs suggests a more complex relationship between spectral resolution development, patient demographics, and the quality of the CI electrode-neuron interface. Future research should aim to distinguish between the effects of patient-specific variables and the underlying physiology on spectral resolution abilities in children of all ages who are hard of hearing and use auditory prostheses.

Validation of the Seattle Suprastomal Safety Score (5S): A Novel Measure in Pediatric Tracheostomy-Dependent Patients.

OBJECTIVE: Suprastomal collapse and granulation are common sequelae of pediatric tracheostomy. We present the first measure of suprastomal obstructive pathology, the Seattle Suprastomal Safety Score (5S), an instrument with 2 domains: collapse and granulation. STUDY DESIGN: Cross-sectional repeated testing survey. SETTING: Electronic survey. METHODS: A library of images was assembled from still pictures of the suprastomal area in 50 patients who previously underwent trachea-bronchoscopy at a quaternary children's hospital. Five pediatric otolaryngologists and 2 pediatric pulmonologists reviewed the images in random, blinded fashion and provided 5S scores. Participants repeated this process 2 to 4 weeks later. Interrater agreement was calculated with an intraclass correlation coefficient (ICC) with a 2-way random-effects model and Fleiss's κ. Intrarater agreement was measured with an ICC using a 2-way mixed-effects model as well as with test-retest correlations using Spearman rank coefficient. All measures were performed separately on collapse and granulation domains. RESULTS: ICC for interrater agreement was 0.88 (95% CI, 0.82-0.93) for collapse and 0.97 (95% CI, 0.96-0.98) for granulation, indicating almost perfect agreement. Fleiss's κ demonstrated moderate agreement for collapse and almost perfect agreement for granulation. ICC for intrarater agreement was 0.95 (95% CI, 0.93-0.97) and 0.99 (95% CI, 0.98-0.99) for collapse and granulation, respectively, indicating almost perfect agreement. Spearman rank correlation for test-retest demonstrated substantial agreement for collapse and almost perfect agreement for granulation. CONCLUSION: The 5S demonstrates excellent interrater and intrarater agreement, making it highly reliable as a novel measure of suprastomal collapse and granulation in tracheostomy-dependent pediatric patients.

Drug-induced sleep endoscopy directed surgery improves polysomnography measures in overweight and obese children with obstructive sleep apnea.

BACKGROUND: Obstructive sleep apnea affects approximately 1-4% of all children, with increased prevalence amongst overweight and obese children. OBJECTIVE: To assess the effects of drug-induced sleep endoscopy (DISE)-directed surgery on polysomnography parameters in obese and overweight children. MATERIAL/METHODS: A retrospective case-series was performed on obese and overweight pediatric patients who underwent clinically indicated DISE-directed surgery. Forty children met the inclusion criteria, including: body mass index ≥85%, DISE-study, and pre- and post-DISE polysomnography. Patients were divided into surgically naïve (n = 23) and prior adenotonsillectomy (n = 17) groups. Demographic and clinical characteristics were examined with chi-square and Wilcoxon rank-sum test. Polysomnography parameters were compared with Wilcoxon signed rank test. RESULTS: Of 40 children with mean BMI 94% and mean age 8 ± 6 years old, 17 (43%) underwent a previous adenotonsillectomy. Overall, significant improvements were observed in the apnea-hypopnea index (AHI; 25.0 to 9.9 events/hour, p < .01) and oxygen nadir (82.7% to 88.5%, p < .01). A similar pattern was observed among the surgically naïve (AHI: 35.9 to 12.7 events/hour, p = .04; oxygen nadir: 79.7% to 86.4%, p = .2) and post-adenotonsillectomy groups (AHI: 10.4 to 6.2 events/hour, p = .02; oxygen nadir: 86.7% to 91.2%, p < .01). CONCLUSIONS/SIGNIFICANCE: Polysomnography parameters significantly improved following DISE-directed interventions in obese and overweight children with obstructive sleep apnea.

Reliability of Measuring Insertion Depth in Cochlear Implanted Infants and Children Using Cochlear View Radiography.

OBJECTIVES: Cochlear implant depth of insertion affects audiologic outcomes and can be measured in adults using plain films obtained in the "cochlear view." The objective of this study was to assess interrater and intrarater reliability of measuring depth of insertion using cochlear view radiography. STUDY DESIGN: Prospective, observational. SETTING: Tertiary referral pediatric hospital. SUBJECTS AND METHODS: Patients aged 11 months to 20 years (median, 4 years; interquartile range [IQR], 1-8 years) undergoing cochlear implantation at our institution were studied over 1 year. Children underwent cochlear view imaging on postoperative day 1. Films were deidentified and 1 image per ear was selected. Two cochlear implant surgeons and 2 radiologists evaluated each image and determined angular depth of insertion. Images were re-reviewed 6 weeks later by all raters. Inter- and intrarater reliability were calculated with intraclass correlation coefficients (ICCs). RESULTS: Fifty-seven ears were imaged from 42 children. Forty-nine ears (86%) had successful cochlear view x-rays. Median angular depth of insertion was 381° (minimum, 272°; maximum, 450°; IQR, 360°-395°) during the first round of measurement. Measurements of the same images reviewed 6 weeks later showed median depth of insertion of 382° (minimum, 272°; maximum, 449°; IQR, 360°-397°). Interrater and intrarater reliability ICCs ranged between 0.81 and 0.96, indicating excellent reliability. CONCLUSIONS: Postoperative cochlear view radiography is a reliable tool for measurement of cochlear implant depth of insertion in infants and children. Further studies are needed to determine reliability of intraoperatively obtained cochlear view radiographs in this population.

Defining Essential Services for Deaf and Hard of Hearing Children during the COVID-19 Pandemic.

COVID-19 is a rapidly growing global pandemic caused by a novel coronavirus. With no vaccine or definitive treatment, public health authorities have recommended a strategy of "social distancing," reducing individual interaction, canceling elective procedures, and limiting nonessential services. Health care providers must determine what procedures are considered "elective," balancing risk of treatment delays with that of coronavirus exposure to patient, family, and providers. Given critical periods for language development and the long-term impact of auditory deprivation, some audiologic and otologic services should be considered essential. In this article, we describe the experience of a quaternary referral pediatric hospital in Seattle, the epicenter of COVID-19 in the United States, and share strategies for risk minimization employed by Seattle Children's Hospital. We hope that this work can be a reference for other centers continuing care for children who are deaf and hard of hearing during the COVID-19 and future resource-limiting crises.

Pediatric Otolaryngology Divisional and Institutional Preparatory Response at Seattle Children's Hospital after COVID-19 Regional Exposure.

Coronavirus disease 2019 (COVID-19) is a novel coronavirus resulting in high mortality in the adult population but low mortality in the pediatric population. The role children and adolescents play in COVID-19 transmission is unclear, and it is possible that healthy pediatric patients serve as a reservoir for the virus. This article serves as a summary of a single pediatric institution's response to COVID-19 with the goal of protecting both patients and health care providers while providing ongoing care to critically ill patients who require urgent interventions. A significant limitation of this commentary is that it reflects a single institution's joint effort at a moment in time but does not take into consideration future circumstances that could change practice patterns. We still hope dissemination of our overall response at this moment, approximately 8 weeks after our region's first adult case, may benefit other pediatric institutions preparing for COVID-19.

Spectral aliasing in an acoustic spectral ripple discrimination task.

Spectral ripple discrimination tasks are commonly used to probe spectral resolution in cochlear implant (CI), normal-hearing (NH), and hearing-impaired individuals. In addition, these tasks have also been used to examine spectral resolution development in NH and CI children. In this work, stimulus sine-wave carrier density was identified as a critical variable in an example spectral ripple-based task, the Spectro-Temporally Modulated Ripple (SMR) Test, and it was demonstrated that previous uses of it in NH listeners sometimes used values insufficient to represent relevant ripple densities. Insufficient carry densities produced spectral under-sampling that both eliminated ripple cues at high ripple densities and introduced unintended structured interference between the carriers and intended ripples at particular ripple densities. It was found that this effect produced non-monotonic psychometric functions for NH listeners that would cause systematic underestimation of thresholds with adaptive techniques. Studies of spectral ripple detection in CI users probe a density regime below where this source of aliasing occurs, as CI signal processing limits dense ripple representation. While these analyses and experiments focused on the SMR Test, any task in which discrete pure-tone carriers spanning frequency space are modulated to approximate a desired pattern must be designed with the consideration of the described spectral aliasing effect.

Polysomnography outcomes of sleep endoscopy-directed intervention in surgically naïve children at risk for persistent obstructive sleep apnea.

PURPOSE: Drug-induced sleep endoscopy (DISE) is useful in children with obstructive sleep apnea (OSA) that persists after adenotonsillectomy (AT), but its utility in surgically naïve children is unclear. We report polysomnography outcomes of surgically naïve children who underwent DISE-directed intervention because they were considered high risk for persistent OSA after adenotonsillectomy. METHODS: This study is a case series of 62 surgically naïve children with OSA who were considered high risk for persistence after AT and underwent DISE-directed intervention with pre- and postoperative polysomnography between 2012 and 2016. Analysis was performed with the paired t test. RESULTS: Children were on average 5.9 (± 5.5, 0.2-18.6) years old at the time of surgery, 68% male, 18% obese, and 60% white. Thirty-eight percent had a syndromic diagnosis: 19% trisomy 21, 11% hypotonic neuromuscular disorder, and 8% craniofacial condition. The remaining 62% were non-syndromic but underwent DISE because they had at least one risk factor for OSA persistence after AT (age > 7 years, black race, 1+ tonsils, obesity, and/or severe OSA). Forty-two percent underwent AT, while 58% underwent treatment other than AT, including 18% who had multilevel surgery. Children improved significantly in 4 out of 5 polysomnography parameters tested, including obstructive apnea-hypopnea index (oAHI; 22.2 to 7.2, p < 0.01) and oxygen nadir (82 to 87, p < 0.01). Thirty-eight (61%) had a postoperative oAHI < 5; 16 (21%) had a postoperative oAHI < 2. CONCLUSION: DISE resulted in intervention other than AT in 58% of surgically naïve children at high risk for persistent OSA after AT. DISE-directed intervention resulted in significant mean improvement in postoperative OSA.

Auditory comprehension outcomes in children who receive a cochlear implant before 12 months of age.

OBJECTIVE: The U.S. Food and Drug Administration guidelines for cochlear implantation (CI) include age greater than 12 months. Studies have suggested that implantation in children younger than 12 months with congenital deafness may be associated with better spoken language outcomes. Compare auditory comprehension (AC) outcomes for children with congenital deafness who received CI less than 12 months of age to those implanted at 12 to 24 months of age. METHODS: Retrospective review of prospectively collected data in consecutively implanted patients under 2 years of age who received CI and had post-CI Preschool Language Scale (PLS)-AC scores. Receptive language was assessed with the AC subtest of the PLS. Patients without pre-CI PLS-AC scores were excluded. The association between age at implantation and post-CI PLS-AC scores up to 2 years after CI surgery was modeled using a linear mixed-effects model. Time from CI surgery, number of implants, risk factors for language delay, pre-CI PLS-AC score, and sex were included in the model. Patients implanted less than 12 months of age were compared to those implanted between 12 and 24 months. RESULTS: Twenty-nine patients who had CI surgery by 12 months and 82 who had CI surgery between 12 and 24 months were included in the analysis. Younger age at implantation and better pre-CI PLS-AC scores were significantly associated with better post-CI PLS-AC scores. CONCLUSION: Cochlear implantation in children with congenital deafness less than 12 months of age was associated with better PLS-AC than in children implanted over 12 months of age up to 2 years after implantation. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:776-781, 2020.

Reliability and Construct Validity of the Penetration-Aspiration Scale for Quantifying Pediatric Outcomes after Interarytenoid Augmentation.

OBJECTIVE: To assess the reliability and construct validity of the Penetration-Aspiration Scale in children. STUDY DESIGN: This was a retrospective cohort study of pre- and postoperative video modified barium swallow studies from children who underwent interarytenoid injection augmentation for unexplained persistent pharyngeal dysphagia. Two pediatric speech and language pathologists reviewed each study twice in a blinded and randomized fashion. SETTING: Tertiary academic pediatric hospital. SUBJECTS AND METHODS: Thirty children were identified with adequate pre- and postoperative modified barium swallow studies within 4 weeks of intervention. Children were separated into clinical outcome groups based on ability to advance to thinner diet consistencies postoperatively. Construct validity was assessed with a mixed linear model to test the hypothesis that only the clinically improved group would receive better Penetration-Aspiration Scale scores after surgery. Reliability was assessed by calculating chance-corrected agreement between raters (interrater) and raters' repeat evaluations (intrarater). RESULTS: Inter- and intrarater reliabilities (Cohen's κ) were both excellent. Results of the mixed model revealed a significant interaction between outcome group and pre- and postoperative time interval. As hypothesized, this involved a significant improvement in Penetration-Aspiration Scale score only in the improved group. CONCLUSIONS: These findings suggest that the Penetration-Aspiration Scale is a reliable and valid measure of clinical response to interarytenoid injection augmentation in children.

Advances in Management of Pediatric Sensorineural Hearing Loss.

The work-up and management of sensorineural hearing loss in children has been an area of rapid evolution. With the availability of genetic and cytomegalovirus testing, the diagnostic process is continuously refined. Aural rehabilitation should be provided to children in a timely manner. At present, the main surgical options for the treatment of sensorineural hearing loss are bone conduction sound processors and cochlear implants. Investigations into modalities such as auditory brainstem implants are ongoing. With further technological and medical advancements, the evaluation and management of pediatric sensorineural hearing loss will undoubtedly continue to change.