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OBJECTIVE Posttonsillectomy hemorrhage can be life-threatening, so we investigated whether patients are at increased risk with an inexperienced surgeon. There is scant information on how surgical experience affects outcomes in pediatric tonsillectomy. We hypothesized that supervised residents would have longer operative times but no difference in complication rates compared to attending surgeons. STUDY DESIGN Retrospective case series of children who underwent tonsillectomy from July 2014 to April 2017 at a tertiary pediatric medical center. METHODS We assessed outcomes and operative times, based on the primary surgeon's level of training, for children (14 months to 22 years) who underwent tonsillectomy. RESULTS A total of 7,606 children were included (mean age 7.0 ± 4.1 years, 51% female) with a mean body mass index (BMI) of 18.6 ± 5.48 kg/m2 ; 76% were white; and 13% were black. Residents assisted with tonsillectomy in 43% of cases. The readmission rate (5%-6%) was not different (P = 0.48) by level of experience. Similarly, return to the operating room for control of hemorrhage (3.3%-3.5%) did not differ by level of experience (P = 0.95). The median procedure time for adenotonsillectomy was shortest for attendings (9 minutes), followed by fellows (13 minutes), and residents (14 minutes, P

Objectives/hypothesis Over the past decade, thoracic slide tracheoplasty (TST) has become the principal operation in the management of congenital tracheal stenosis. The purpose of this report was to describe our experience with revision TST following unsuccessful prior tracheal reconstruction. Study design Retrospective analysis at an academic children's hospital. Methods Patients undergoing TST on cardiopulmonary bypass between January 2005 and May 2014 were reviewed. Patients with a history of prior airway surgery were extracted for further analysis. Preoperative patient variables and postoperative outcomes were evaluated and compared between patients undergoing revision slide tracheoplasty (RTST) and a control group of 26 matched patients undergoing primary surgery TST. Results Twenty-six revision patients (25 referrals, one primary patient) of 162 patients reviewed over the study period met inclusion criteria. Twenty-three patients had a history of complete tracheal rings, and three patients had cartilaginous deficiency. A total of 41 airway reconstruction procedures had been performed prior to RTST. When compared to primary TST, patients undergoing RTST required fewer cardiac procedures intraoperatively, and fewer mean ventilator hours (P = .01) postoperatively. There was no significant difference in the median length of stay, requirement of >48 hours ventilation, or postoperative complications between groups. There was one nonsurgical postoperative mortality following RTST. Conclusions Despite some differences in the postoperative management when compared to nonrevision cases, revision TST can be successfully performed after prior tracheal reconstruction with good postoperative outcomes. Level of evidence 4. Laryngoscope, 128:2181-2186, 2018.

OBJECTIVE Determine if overnight stenting is warranted after pharyngeal flap for management of velopharyngeal insufficiency (VPI) in pediatric patients METHODS: This is a retrospective age-matched cohort study from a single tertiary pediatric facility. Patients who underwent a posterior pharyngeal flap for VPI from January 1, 2013, to December 31, 2016, were included. Two equal groups were constructed based on when their nasal stents were removed, that is, postoperative day (POD) 0 or POD 1. Primary outcome measure was oxygen desaturation after surgery. Secondary outcome measures were total length of stay, need for narcotics, and oral intake based on POD. Additional information was gathered and evaluated as possible prognostic variables. RESULTS There were 27 matched pairs included in the study. One patient from each group required supplemental oxygen during the night of surgery. No escalation of care was needed in either case. The group whose stents were removed on POD 0 were discharged earlier than the group whose stents were removed on POD 1, P < 0.0001. The POD 0 group took significantly more oral intake on POD 0 than the POD 1 group, P = 0.03. The POD 0 group had significantly more genetic syndromes than the POD 1 group, P = 0.02. CONCLUSION There was no benefit with overnight stenting. Earlier discharge from the hospital was achieved in the POD 0 group. No patient experienced need for reintubation, escalation of clinical care, postoperative hemorrhage, or death. LEVEL OF EVIDENCE 4. Laryngoscope, 128:750-755, 2018.

Objectives/Hypothesis Tracheotomy is a common procedure. A reliable method of securing the tracheotomy tube is essential to minimize accidental decannulation. However, skin breakdown has been reported in ∼30% of patients. We sought to evaluate rates of skin-related complications and accidental decannulation with the use of Velcro ties compared to twill ties. Study Design A nonblinded, randomized controlled trial comparing Velcro versus twill ties in patients undergoing tracheotomy between July 1, 2014 and January 31, 2016. Methods Patients ≤21 years of age were recruited and randomized to receive either Velcro or twill ties. The primary outcome measure was skin-related complications. The secondary outcome measure was accidental decannulation. Outcome measures were followed through postoperative day 5. Results Ninety-three patients were eligible, and 63 were enrolled. No patients were withdrawn. Fifty-seven patients were included in the analysis. Twenty-seven (47.4%) received Velcro, and 30 (52.6%) received twill. Five enrolled patients did not undergo tracheotomy (one Velcro, four twill). One was diagnosed with a genetic skin condition after enrollment but prior to undergoing tracheotomy. Patient characteristics were similar between groups. No significant differences were found with respect to skin-related complications (P = .59). Six patients (20%) with twill ties required early tie change compared to two (7.4%) with Velcro ties (P = .5). Two accidental decannulations occurred in the twill group compared to one in the Velcro group (P = 1.0). Conclusions Our study demonstrated no differences in skin-related complications or accidental decannulation between Velcro and twill tracheotomy ties in the immediate postoperative period following tracheotomy. Our study suggests that Velcro ties are a viable alternative to twill ties. Level of Evidence 1b Laryngoscope, 127:1996–2001, 2017

Objectives/Hypothesis Lingual tonsil hypertrophy is a common cause of persistent airway obstruction in patients with Down syndrome (DS) following adenotonsillectomy (TA however, little is known about the effect of lingual tonsillectomy (LT) on polysomnographic outcomes in these patients. Our objective was to describe changes in sleep-related respiratory outcomes following LT in children with DS and persistent obstructive sleep apnea (OSA) following T&A. Study Design Retrospective case series. Methods We included all children with DS who underwent polysomnography before and after LT at a tertiary care center from 2003 to 2013. Nonparametric analysis of variables was performed. Results Forty patients with DS underwent LT; 21 met inclusion criteria. The mean age at surgery was 9.3 ± 4.3 years and 47.6% were female. The median apnea-hypopnea index (AHI) was 9.1 events/hour (range, 3.8 to 43.8 events/hour) before surgery and 3.7 events/hour (range, 0.5 to 24.4 events/hour) after surgery. The median improvement in overall AHI and the obstructive AHI (oAHI) were 5.1 events/hour (range, −2.9 to 41) and 5.3 events/hour (range, −2.9 to 41), respectively (P 50 mm Hg, central index, and percentage of rapid eye movement sleep were not significantly different. After surgery, the oAHI was

Objectives/Hypothesis To establish the prevalence of abnormal vestibular test findings in children with enlarged vestibular aqueduct (EVA) and determine if these findings correlate with clinical symptoms, radiographic findings (EVA size and laterality), audiometric findings, and genetic testing in these patients. Study Design Prospective cohort. Methods Patients 3 to 12 years of age with hearing loss and imaging findings consistent with EVA treated at our tertiary care institution were sequentially enrolled from 2009 to 2011. The following six outcome measurements were analyzed: audiometric findings, EVA laterality, temporal bone measurements, genetic testing, vestibular testing (cervical-evoked myogenic potentials, posturography, rotational chair, and calorics), and vestibular symptoms. Results Twenty-seven patients with EVA (mean age 9.2 years, 48% female) were enrolled in and completed the study. Vertigo was reported in six patients. Twenty-four of 27 (89%) had at least one abnormal vestibular test result. Midpoint and operculum size correlated with directional preponderance (P = .042 and P = .032, respectively). Also, high-frequency pure tone average (HFPTA) correlated with unilateral weakness (P = .002). Walking at a later age correlated with abnormal posturography results. There was no correlation between EVA laterality and vestibular test findings. Conclusion We found a high rate of vestibular pathology in children with EVA; however, the prevalence of abnormal vestibular test findings in this patient population was not correlated with vestibular symptoms. Enlarged vestibular aqueduct size, HFPTA, and walking at a later age were correlated with abnormal vestibular test findings. In view of these results, it may be prudent to consider vestibular testing in children with these clinical characteristics. Level of Evidence 2b. Laryngoscope, 126:2344–2350, 2016

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

Objectives/Hypothesis With the prevalence of head and neck squamous cell carcinoma (HNSCC) nearly twice as high in veterans (6%) than general populations (3%), the noted problems of long waits and access to care in United States Veterans Affairs (VA) hospitals across the country are pressing. We examined primary outcome measures of timeliness and access to care for our patients with HNSCC assessing a multidisciplinary team approach at our VA hospital. Study Design Retrospective chart review. Methods Our patients newly diagnosed with HNSCC were identified from two 24-month periods: diagnosis before (group 1, 2005–2006) and after (group 2, 2008–2009) implementing our multidisciplinary team in 2007. No significant differences in age (P = .13) or disease stage (P = .18) occurred between groups. Primary and secondary outcomes (i.e., treatment modality, imaging, completion of treatment, survival) were compared. Results Timeliness to care improved for all measures. Improvement was significant for times from consult placed to seen in clinic (27.5–16.5 days; P < 0.0001) and from positive biopsy reported to date of initiating definitive treatment (35–27 days; P = 0.04). Pretreatment consults to various services represented by the multidisciplinary team increased from one to four (P < 0.0001). Two-year mortality was approximately the same between group 1 (33%) and group 2 (36%) (P = 0.035). Five-year mortality was slightly better in group 2 (50%) versus group 1 (61%), although not statistically significant. Conclusion Our veteran population with HNSCC had improved timeliness and access to care with our multidisciplinary approach. Level of Evidence 4. Laryngoscope, 2015

Objectives/Hypothesis Although adenotonsillectomy is accepted as a first-line therapy for pediatric obstructive sleep apnea (OSA), there is currently no consensus regarding optimal methods for identifying the sites of obstruction or treatment of children with persistent disease after surgery. With this in mind, our aim was to systematically review the English-language literature pertaining to these issues. Study Design Systematic review. Methods We searched all indexed years of Pubmed, Cochrane CENTRAL, DynaMed, UpToDate, CINAHL, and Scopus for English-language articles containing original human data, with ≥ 7 participants, all

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. Laryngoscope, 125:1491–1495, 2015

Over the past decade, thoracic slide tracheoplasty (TST) has become the principal operation in the management of congenital tracheal stenosis. The purpose of this report was to describe our experience with revision TST following unsuccessful prior tracheal reconstruction.Retrospective analysis at an academic children's hospital.Patients undergoing TST on cardiopulmonary bypass between January 2005 and May 2014 were reviewed. Patients with a history of prior airway surgery were extracted for further analysis. Preoperative patient variables and postoperative outcomes were evaluated and compared between patients undergoing revision slide tracheoplasty (RTST) and a control group of 26 matched patients undergoing primary surgery TST.Twenty-six revision patients (25 referrals, one primary patient) of 162 patients reviewed over the study period met inclusion criteria. Twenty-three patients had a history of complete tracheal rings, and three patients had cartilaginous deficiency. A total of 41 airway reconstruction procedures had been performed prior to RTST. When compared to primary TST, patients undergoing RTST required fewer cardiac procedures intraoperatively, and fewer mean ventilator hours (P = .01) postoperatively. There was no significant difference in the median length of stay, requirement of48 hours ventilation, or postoperative complications between groups. There was one nonsurgical postoperative mortality following RTST.Despite some differences in the postoperative management when compared to nonrevision cases, revision TST can be successfully performed after prior tracheal reconstruction with good postoperative outcomes.4. Laryngoscope, 128:2181-2186, 2018.

Determine if overnight stenting is warranted after pharyngeal flap for management of velopharyngeal insufficiency (VPI) in pediatric patients METHODS: This is a retrospective age-matched cohort study from a single tertiary pediatric facility. Patients who underwent a posterior pharyngeal flap for VPI from January 1, 2013, to December 31, 2016, were included. Two equal groups were constructed based on when their nasal stents were removed, that is, postoperative day (POD) 0 or POD 1. Primary outcome measure was oxygen desaturation after surgery. Secondary outcome measures were total length of stay, need for narcotics, and oral intake based on POD. Additional information was gathered and evaluated as possible prognostic variables.There were 27 matched pairs included in the study. One patient from each group required supplemental oxygen during the night of surgery. No escalation of care was needed in either case. The group whose stents were removed on POD 0 were discharged earlier than the group whose stents were removed on POD 1, P0.0001. The POD 0 group took significantly more oral intake on POD 0 than the POD 1 group, P = 0.03. The POD 0 group had significantly more genetic syndromes than the POD 1 group, P = 0.02.There was no benefit with overnight stenting. Earlier discharge from the hospital was achieved in the POD 0 group. No patient experienced need for reintubation, escalation of clinical care, postoperative hemorrhage, or death.4. Laryngoscope, 128:750-755, 2018.

Objectives/Hypothesis The objective was to describe trends in the annual prevalence of hospitalization for pediatric acute mastoiditis since introduction of the 7-valent pneumococcal vaccine in 2000 and the 13-valent vaccine in 2010. Study Design Cross-sectional retrospective data analysis. Methods The Kids' Inpatient Database from years 2000 to 2012 was analyzed. To determine the annual prevalence of hospitalization for acute mastoiditis, nationally weighted frequencies of hospitalization for children

Tracheotomy is a common procedure. A reliable method of securing the tracheotomy tube is essential to minimize accidental decannulation. However, skin breakdown has been reported in ∼30% of patients. We sought to evaluate rates of skin-related complications and accidental decannulation with the use of Velcro ties compared to twill ties.A nonblinded, randomized controlled trial comparing Velcro versus twill ties in patients undergoing tracheotomy between July 1, 2014 and January 31, 2016.Patients ≤21 years of age were recruited and randomized to receive either Velcro or twill ties. The primary outcome measure was skin-related complications. The secondary outcome measure was accidental decannulation. Outcome measures were followed through postoperative day 5.Ninety-three patients were eligible, and 63 were enrolled. No patients were withdrawn. Fifty-seven patients were included in the analysis. Twenty-seven (47.4%) received Velcro, and 30 (52.6%) received twill. Five enrolled patients did not undergo tracheotomy (one Velcro, four twill). One was diagnosed with a genetic skin condition after enrollment but prior to undergoing tracheotomy. Patient characteristics were similar between groups. No significant differences were found with respect to skin-related complications (P = .59). Six patients (20%) with twill ties required early tie change compared to two (7.4%) with Velcro ties (P = .5). Two accidental decannulations occurred in the twill group compared to one in the Velcro group (P = 1.0).Our study demonstrated no differences in skin-related complications or accidental decannulation between Velcro and twill tracheotomy ties in the immediate postoperative period following tracheotomy. Our study suggests that Velcro ties are a viable alternative to twill ties.1b Laryngoscope, 127:1996-2001, 2017.

Lingual tonsil hypertrophy is a common cause of persistent airway obstruction in patients with Down syndrome (DS) following adenotonsillectomy (TA); however, little is known about the effect of lingual tonsillectomy (LT) on polysomnographic outcomes in these patients. Our objective was to describe changes in sleep-related respiratory outcomes following LT in children with DS and persistent obstructive sleep apnea (OSA) following TA.Retrospective case series.We included all children with DS who underwent polysomnography before and after LT at a tertiary care center from 2003 to 2013. Nonparametric analysis of variables was performed.Forty patients with DS underwent LT; 21 met inclusion criteria. The mean age at surgery was 9.3 ± 4.3 years and 47.6% were female. The median apnea-hypopnea index (AHI) was 9.1 events/hour (range, 3.8 to 43.8 events/hour) before surgery and 3.7 events/hour (range, 0.5 to 24.4 events/hour) after surgery. The median improvement in overall AHI and the obstructive AHI (oAHI) were 5.1 events/hour (range, -2.9 to 41) and 5.3 events/hour (range, -2.9 to 41), respectively (P.0001). The mean oxygen saturation nadir improved from 84% to 89% (P =.004). The mean time with COIn children with DS, persistent OSA after TA and lingual tonsil hypertrophy, LT significantly improved AHI, oAHI, and O4 Laryngoscope, 2016 127:520-524, 2017.

Objectives/Hypothesis: Evaluate sexual dysfunction in patients after treatment for head and neck cancer. Study Design: Single-institution cross-sectional study. Methods: Our modified Sexual Adjustment Questionnaire was administered to 42 patients (mean age, 55.1 years) and included seven questions (total score, 7–35). Based on sexual satisfaction ratings, we categorized three groups as unsatisfied (7–16), satisfied (17–25), and very satisfied (26–35). Clinical information was obtained by reviewing medical records. Bivariate analysis tested associations between sexual satisfaction and patient-related factors (e.g., sex, age, site/cancer stage, treatment, time between treatment and survey, partner, alcohol/tobacco consumption). Pearson correlation was used to analyze two continuous variables, and multivariate logistic regression analysis was used to evaluate the independent impact of each factor. Results: All 42 patients rated that head and neck cancer negatively impacted their sexual relationships, including 21 (50%) rating effects as negative or extremely negative. Men reported higher satisfaction scores with sexual function (mean ± standard deviation) than women (19.9 ± 5.0 vs. 16.3 ± 6.5, respectively; P = .06). Respondents with partners reported higher scores than those without partners (19.9 ± 5.3 vs. 14.1 ± 4.4, respectively; P = .01). When the survey was administered (median, 12 months; range, 4–33 months) after the first treatment, mean score was 19; 57% of respondents were sexually satisfied, 31% were unsatisfied, and 12% were very satisfied. Instrument reliability was .82 (Cronbach alpha). Conclusions: Patients who are male and ≤60 years have a higher probability of sexual satisfaction during recovery. Our sexual dysfunction questionnaire will be administered in further prospective studies in patients with head and neck cancer. Laryngoscope, 2012

Objectives: Cricotracheal resection (CTR) is an effective treatment for moderate-to-severe laryngotracheal stenosis (LTS) in adults. However, one of the potentially significant sequela of this procedure is postoperative dysphonia and permanent voice alteration. The objective of this study is to characterize voice changes in adult patients with subglottic stenosis who have undergone CTR. Study Design: Retrospective case-series. Methods: Acoustic, aerodynamic, and consensus auditory-perceptual evaluation of voice (CAPE-V) data were reviewed for consecutive adult patients undergoing CTR and perioperative voice evaluation from 2000 to 2010. Results: Sixteen patients (median age 44 years, 94% female) underwent CTR and voice evaluation during the study period. Thirteen patients underwent postoperative evaluation and had a mean overall CAPE-V score of 47.5/100, mean fundamental frequency (F0) of 156.7 Hz, average estimated subglottic pressure of 10.0 cm H2O, and an average airflow of 224 mL/sec. Seven patients underwent pre- and postoperative evaluations and had a significant reduction in mean F0 (206.5 vs. 151.1 Hz, P = .002) and mean F0 for connected speech (194.9 vs. 152.7 Hz, P = .047), but not in median intensity, range, estimated subglottic pressure, or airflow. Median overall CAPE-V scores worsened, but did not reach statistical significance (12 vs. 61.5, P = .28). Seventy-one percent of patients underwent an increase in acoustic signal typing postoperatively. Conclusions: Although CTR is an effective treatment for adult LTS, it results in significant alteration of the adult voice. In particular, CTR decreases the fundamental frequency of connected speech and vowel phonation and changes the acoustic signal type. Patients should be counseled about these likely voice alterations prior to undergoing surgery.

Objectives/Hypothesis: Quantify post-cochlear implant (CI) language among children with disabilities and determine the role of nonverbal cognitive quotients (NVCQ) in predicting language. Study Design: Small cohort study in pediatric tertiary care center. Methods: Children (n = 20) with CIs and developmental disabilities were enrolled. Receptive and expressive language was reported as language quotients (LQs). Pre- and post-CI LQs were compared using the signed-rank test. Multiple regression models analyzed language while controlling for possible confounders. Results: Five subjects had symptomatic cytomegalovirus, and four subjects had CHARGE syndrome with hearing loss etiology. Seventy-five percent had cognitive deficits, and 55% had motor delays. Median age of CI was 24 months; median CI duration was 27.7 months. The range of NVCQs for the study cohort was 27 to 115. Fifteen subjects had NVCQs

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. Laryngoscope, 119:554–558, 2009

Determine the prevalence of high-frequency sensorineural hearing loss (HFSNHL) in our hearing loss population and a diagnostic algorithm for these patients.Retrospective case series.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.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).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.4. Laryngoscope, 126:1236-1240, 2016.

Objectives/Hypothesis To describe the clinical phenotype of pediatric patients with unilateral enlarged vestibular aqueduct (EVA) and then to compare the findings to two clinically related phenotypes: bilateral EVA and unilateral hearing loss without EVA. In view of clinical observations and previously published data, we hypothesized that patients with unilateral EVA would have a much higher rate of contralateral hearing loss than patients with unilateral hearing loss without EVA. Study Design Retrospective cohort study. Methods Patients with unilateral or bilateral EVA were identified from a database of children with sensorineural hearing loss who were seen at a tertiary care institution between 1998 and 2010. Those with imaging findings consistent with well-established EVA criteria were identified. A comparative group of patients with unilateral hearing loss without EVA was also identified. The following specific outcome measurements were analyzed: 1) hearing loss phenotype, 2) laterality of EVA and hearing loss, 3) midpoint and operculum vestibular aqueduct measurements, and 4) genetic test results. Results Of the 144 patients who met our inclusion criteria, 74 (51.4%) had unilateral EVA. There was a strong correlation between the presence of hearing loss and ears with EVA. Fifty-five percent of patients with unilateral EVA had hearing loss in the contralateral ear; in most of these patients, the hearing loss was bilateral. Contralateral hearing loss occurred in only 6% of patients with unilateral hearing loss without EVA. No significant differences were found in temporal bone measurements between the ears of patients with unilateral EVA and ipsilateral hearing loss and all ears with EVA and normal hearing (P = .4). There was no difference in the rate of hearing loss progression in patients with unilateral EVA between ears with or without EVA (16 of 48 [33.3%] vs. 9 of 27 [33.3%], respectively; P = 1.0). There was no difference in the rate of hearing loss progression in patients with bilateral and unilateral EVA (41 of 89 ears [46.1%] vs. 25 of 75 ears [33.3%], respectively; P = .1); however, both EVA groups had higher rates of progression compared to patients with unilateral hearing loss without EVA. There was a strong correlation between the presence of hearing loss at 250 Hz and the risk of more severe hearing loss and progressive hearing loss. Patients with bilateral EVA and SLC26A4 mutations had a higher rate of progression than patients who had no mutations (P = .02). No patients with unilateral EVA had Pendred syndrome. Conclusions Children with unilateral EVA have a significant risk of hearing loss progression. Hearing loss in the ear contralateral to the EVA is common, suggesting that unilateral EVA is a bilateral process despite an initial unilateral imaging finding. In contrast to bilateral EVA, unilateral EVA is not associated with Pendred syndrome and may have a different etiology. Temporal bone measurements, hearing loss severity, and hearing loss at 250 Hz were all correlated with the risk of progressive hearing loss. Clinicians should become knowledgeable regarding the implications of this disease process so that families can be counseled appropriately. Level of Evidence 2b.

Cricotracheal resection (CTR) is an effective treatment for moderate-to-severe laryngotracheal stenosis (LTS) in adults. However, one of the potentially significant sequela of this procedure is postoperative dysphonia and permanent voice alteration. The objective of this study is to characterize voice changes in adult patients with subglottic stenosis who have undergone CTR.Retrospective case-series.Acoustic, aerodynamic, and consensus auditory-perceptual evaluation of voice (CAPE-V) data were reviewed for consecutive adult patients undergoing CTR and perioperative voice evaluation from 2000 to 2010.Sixteen patients (median age 44 years, 94% female) underwent CTR and voice evaluation during the study period. Thirteen patients underwent postoperative evaluation and had a mean overall CAPE-V score of 47.5/100, mean fundamental frequency (F0) of 156.7 Hz, average estimated subglottic pressure of 10.0 cm H(2) O, and an average airflow of 224 mL/sec. Seven patients underwent pre- and postoperative evaluations and had a significant reduction in mean F0 (206.5 vs. 151.1 Hz, P = .002) and mean F0 for connected speech (194.9 vs. 152.7 Hz, P = .047), but not in median intensity, range, estimated subglottic pressure, or airflow. Median overall CAPE-V scores worsened, but did not reach statistical significance (12 vs. 61.5, P = .28). Seventy-one percent of patients underwent an increase in acoustic signal typing postoperatively.Although CTR is an effective treatment for adult LTS, it results in significant alteration of the adult voice. In particular, CTR decreases the fundamental frequency of connected speech and vowel phonation and changes the acoustic signal type. Patients should be counseled about these likely voice alterations prior to undergoing surgery.

Quantify post-cochlear implant (CI) language among children with disabilities and determine the role of nonverbal cognitive quotients (NVCQ) in predicting language.Small cohort study in pediatric tertiary care center.Children (n = 20) with CIs and developmental disabilities were enrolled. Receptive and expressive language was reported as language quotients (LQs). Pre- and post-CI LQs were compared using the signed-rank test. Multiple regression models analyzed language while controlling for possible confounders.Five subjects had symptomatic cytomegalovirus, and four subjects had CHARGE syndrome with hearing loss etiology. Seventy-five percent had cognitive deficits, and 55% had motor delays. Median age of CI was 24 months; median CI duration was 27.7 months. The range of NVCQs for the study cohort was 27 to 115. Fifteen subjects had NVCQs80. Age at implantation, income, and number of siblings were not correlated with language. Although children had significant increases in language age pre- to post-CI, median LQs did not significantly change after implantation. NVCQ, age at hearing loss diagnosis, implant duration, and number of different therapies attended were significant in models. NVCQ contributed the most unique variance (67%; P = .0003). Pre-CI language performance did not predict post-CI performance.This study is the first step in addressing the effects of CIs on language among children with disabilities. Progress in language skills occurred for all participants, although rates of progress were slow and highly variable. NVCQ was the strongest predictor of language, although cognition is not always sufficient for good language development. Adapting therapeutic strategies may be essential to impact greater language progress in these complex children.