Kay W. Chang, MD

All Publications

• Tympanostomy Tube Insertion With and Without Adenoidectomy. JAMA otolaryngology-- head & neck surgery Qian, Z. J., Truong, M. T., Alyono, J. C., Valdez, T., Chang, K. 2024

  Abstract

  The benefit of adenoidectomy on otologic outcomes after tympanostomy tube (TT) insertion is unclear. Results from prior work are challenging to interpret due to small sample sizes, heterogeneous study designs, and varying outcome measures.To evaluate the association between adenoidectomy and otologic outcomes using a US population-level sample of children who received TTs, producing generalizable results for widespread clinical application.A matched cohort study was conducted using claims data from the Merative MarketScan Research Databases. The study included 601 848 children who received TTs between January 1, 2007, and December 31, 2021. Children who received adenoidectomy and TTs simultaneously (Ad+TT) were identified irrespective of the number of prior TTs. Control participants who received TTs without adenoidectomy were matched based on sex, age at the time of the procedure, and the number of prior TT procedures.Adenoidectomy without tonsillectomy was the primary exposure.The primary outcomes were repeat TT insertion and subsequent oral antibiotic prescriptions after TT insertions. Multivariable logistic regression was used to quantify the effects of adenoidectomy and covariates on each outcome. Stratified analyses were performed in children younger than 4 years and 4 years or older.Overall, 601 848 children (median [IQR] age, 2 [1-4] years; range, 0-11 years; 351 078 [58.3%] male) who received TTs were identified. The Ad+TT cohort included 201 932 children, with an equal number in the matched cohort. In children younger than 4 years, Ad+TT was common and was associated with lower odds of subsequent oral antibiotics (odds ratio [OR], 0.59; 95% CI, 0.58-0.60) but higher odds of repeat TT insertions (OR, 1.24; 95% CI, 1.22-1.27). In children 4 years or older, Ad+TT was associated with lower odds of repeat TT insertions (OR, 0.78; 95% CI, 0.75-0.81) and subsequent oral antibiotics (OR, 0.63; 95% CI, 0.62-0.65).This study found that in children younger than 4 years, Ad+TT was commonly performed and may have had a secondary benefit of reducing subsequent oral antibiotic courses; however, it was not associated with a reduction in the risks of repeat TT insertions. In children 4 years or older, Ad+TT was associated with a reduction in the risk of repeat TT insertions and subsequent oral antibiotics. Given these findings, Ad+TT may be offered in children 4 years or older to improve otologic outcomes.

  View details for DOI 10.1001/jamaoto.2024.3584

  View details for PubMedID 39418047

• Principles of Cholesteatoma Management. Otolaryngologic clinics of North America Angeli, S. I., Chang, K. W. 2024

  Abstract

  Surgery for cholesteatoma should be tailored to individual patients, considering demographic and disease factors, to obtain a dry, safe, and functional ear. The EAONO/JOS classification and staging system provide a valuable framework for data collection and outcome assessment. Canal wall-up and canal wall-down surgical approaches each have their advantages and disadvantages, though it is not definitive that one approach is clearly more advantageous than the other. Mastoid obliteration techniques show promise in reducing recidivistic disease rates but require further research and standardization. Endoscopic ear surgery further augments our surgical capabilities to visualize and eradicate cholesteatoma.

  View details for DOI 10.1016/j.otc.2024.08.002

  View details for PubMedID 39266390

• Endoscopic-Assisted Cochlear Implantation via a "Microfacial Recess". The Laryngoscope Din, T. F., Abdulkader, F., Chang, K. W. 2024

  Abstract

  Syndromic patients can have severely anomalous anatomy significantly challenging conventional cochlear implant (CI) surgery. This case report describes a 20-year-old with brachio-oto-renal syndrome with a severely anomalous facial nerve completely covering the round window and preventing a traditional posterior tympanotomy CI. This is the first report to illustrate the performance of an endoscopic trans-canal bony cochleostomy with insertion of the CI performed via a "microfacial recess." We describe the performance of an endoscopic trans-canal bony cochleostomy and a modification of the conventional wide posterior tympanotomy usually performed into a 1 mm "microfacial recess," which allowed a full insertion to take place. Laryngoscope, 2024.

  View details for DOI 10.1002/lary.31562

  View details for PubMedID 38837783

• Gaze Patterns of Normal and Microtia Ears Pre- and Post-Reconstruction. The Laryngoscope Losorelli, S., Chang, J. K., Chang, K. W., Most, S. P., Truong, M. T. 2024

  Abstract

  To understand attentional preferences for normal and microtia ears.Eye-tracking technology was used to characterize gaze preferences. A total of 71 participants viewed images of 5 patients with unilateral microtia. Profile images of patient faces and isolated ears including normal, microtia, and post-reconstruction microtia ears were shown. Total time of fixation in predefined areas of interest (AOI) was measured. Inferential statistics were used to assess significance of fixation differences between AOIs within and between facial or auricular features.The ear received most visual attention in lateral view of the face (1.91 s, 1.66-2.16 s) [mean, 95% CI], followed by features of the "central triangle"-the eyes (1.26 s, 1.06-1.46), nose (0.48 s, 0.38-0.58), and mouth (0.15 s, 0.15-0.20). In frontal view, microtia ears received less attention following surgical reconstruction (0.74 s vs. 0.4 s, p < 0.001). The concha was the most attended feature for both normal (2.97 s, 2.7-3.23) and reconstructed microtia ears (1.87 s, 1.61-2.13). Scars on reconstructed ears altered the typical visual scanpath.The ear is an attentional gaze landmark of the face. Attention to microtia ears, both pre- and post-reconstruction, differs from gaze patterns of normal ears. The concha was the most attended to subunit of the ear. Attentional gaze may provide an unbiased method to determine what is important in reconstructive surgery.N/A Laryngoscope, 2024.

  View details for DOI 10.1002/lary.31331

  View details for PubMedID 38334225

• Prevalence of Cochlear Nerve Deficiency and Hearing Device Use in Children With Single-Sided Deafness. Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery Ward, K. M., Coughran, A. J., Lee, M., Fitzgerald, M. B., Cheng, A. G., Chang, K. W., Ahmad, I. N. 2023

  Abstract

  This study aimed to assess the prevalence of cochlear nerve deficiency (CND) in a cohort of pediatric patients with single-sided deafness (SSD). A secondary objective was to investigate trends in intervention and hearing device use in these children.Case series with chart review.Pediatric tertiary care center.Children ages 0 to 21 years with SSD (N = 190) who underwent computerized tomography (CT) and/or magnetic resonance imaging (MRI) were included. Diagnostic criteria for SSD included unilateral severe-to-profound sensorineural hearing loss with normal hearing sensitivity in the contralateral ear. Diagnostic criteria for CND included neuroradiologist report of an "aplastic or hypoplastic nerve" on MRI or a "stenotic cochlear aperture" on CT.The prevalence of CND was 42% for children with CT only, 76% for children with MRI only, and 63% for children with both MRI and CT. Of the children with MRI and CT, there was a 90% concordance across imaging modalities. About 36% of children with SSD had hearing devices that routed sound to the normal hearing ear (ie, bone conduction hearing device/contralateral routing of signal), while only 3% received a cochlear implant. Approximately 40% did not have a hearing device. Hearing device wear time averaged 2.9 hours per day and did not differ based on cochlear nerve status.There is a high prevalence of CND in children with SSD. Cochlear nerve status should be confirmed via MRI in children with SSD. The limited implementation and use of hearing devices observed for children with SSD reinforce the need for increased support for early and continuous intervention.

  View details for DOI 10.1002/ohn.255

  View details for PubMedID 36939463

• Single Versus Multigene Testing for Hereditary Hearing Loss: Use and Costs in a Commercially Insured Cohort. Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery Moon, P. K., Qian, Z. J., Stevenson, D. A., Chang, K. W. 2023

  Abstract

  The objectives of this study were to describe trends in single-gene GJB2/6 (connexin 26/30) and multigene hearing loss panel (HLP) testing for hereditary hearing loss using real-world evidence.Retrospective study using insurance claims data.Optum Data Mart database from 2015 to 2020.Rates of overall and hearing-specific genetic testing and costs to insurers and patients were reported. Linear regression models were used to assess the proportion of single-gene GJB2/6 testing over time. Additional linear regression models were used to assess changes in costs over time.From 2015 to 2020, 91,986 children received genetic testing for any indication, of which 601 (0.65%) received hearing-specific tests. The proportion of single-gene GJB2/6 testing remained similar over time (mean difference [MD]: -1.3% per year; 95% confidence interval [CI]: -4.3%, 1.7%), while multigene HLP use increased over time (MD: 4.0% per year; 95% CI: 0.4%, 7.5%). The median charge for single-gene GJB2/6 testing remained constant during the study period (MD: -$34; 95% CI: -$86, $18), while the median charge for multigene HLP decreased during the study period (MD: -$145 per year; 95% CI: -$278, -$12).Compared to molecular testing for GJB2/6, HLPs are becoming more common for hereditary hearing loss. The comprehensiveness of HLP and decreasing costs provide justification for its more widespread adoption moving forward.

  View details for DOI 10.1002/ohn.204

  View details for PubMedID 36939467

• Author's Response JOURNAL OF INTERNATIONAL ADVANCED OTOLOGY Zhang, T., Bulstrode, N., Chang, K. W., Cho, Y., Frenzel, H., Jiang, D., Kesser, B. W., Siegert, R., Triglia, J. 2023; 19 (1): 75

  View details for Web of Science ID 001027317000017

• Integrated microtia and aural atresia management. Frontiers in surgery Truong, M. T., Liu, Y. C., Kohn, J., Chinnadurai, S., Zopf, D. A., Tribble, M., Tanner, P. B., Sie, K., Chang, K. W. 2022; 9: 944223

  Abstract

  To present recommendations for the coordinated evaluation and management of the hearing and reconstructive needs of patients with microtia and aural atresia.A national working group of 9 experts on microtia and atresia evaluated a working document on the evaluation and treatment of patients. Treatment options for auricular reconstruction and hearing habilitation were reviewed and integrated into a coordinated care timeline.Recommendations were created for children with microtia and atresia, including diagnostic considerations, surgical and non-surgical options for hearing management and auricular reconstruction, and the treatment timeline for each option. These recommendations are based on the collective opinion of the group and are intended for otolaryngologists, audiologists, plastic surgeons, anaplastologists, and any provider caring for a patient with microtia and ear canal atresia. Close communication between atresia/hearing reconstruction surgeon and microtia repair surgeon is strongly recommended.

  View details for DOI 10.3389/fsurg.2022.944223

  View details for PubMedID 36636584

  View details for PubMedCentralID PMC9831057

• Infectious Complications Following Cochlear Implant: Risk Factors, Natural History, and Management Patterns. Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery Moon, P. K., Qian, Z. J., Ahmad, I. N., Stankovic, K. M., Chang, K. W., Cheng, A. G. 2022: 1945998221082530

  Abstract

  OBJECTIVE: To describe the natural history, detail the treatment patterns, and identify the risk factors for cochlear implant (CI) infections in a large US cohort.STUDY DESIGN: Retrospective study based on insurance claims.SETTING: Optum Data Mart database: 6101 patients who received CIs from 2003 to 2019.METHODS: Infections, treatments patterns, and timelines were described. A multivariable logistic regression model was used to assess the association between postoperative oral antibiotics and CI infection.RESULTS: The cohort includes 4736 (77.6%) adults and 1365 (22.4%) children. Between adult and pediatric patients, rates of CI infection (5.1% vs 4.5%, P = .18) and explantation (1.2% vs 0.8%, P = .11) were not significantly different. Infections typically occurred within 5 months of surgery. Children were diagnosed with CI infection earlier than adults (median difference, -1.5 months; P = .001). Postoperative oral antibiotic supply was not associated with lower risk of CI infection in either children or adults. However, among adults, otitis media was associated with higher odds of CI infection (odds ratio, 1.41; P < .001), while higher income was associated with lower odds of CI infection (odds ratio, 0.71; P = .03).CONCLUSIONS: Postoperative oral antibiotics were not associated with lower risk of infection or interventions. Otitis media episodes and lower income were associated with increased risk of infection among adults as well as intervention overall. Infection typically presented within the first 6 months after surgery, with children presenting earlier than adults. Overall, our findings serve as a resource for providers to consider in their care of patients with CIs.

  View details for DOI 10.1177/01945998221082530

  View details for PubMedID 35192408

• Comments on Use of Diagnostic Testing and Intervention for Sensorineural Hearing Loss in US Children-Reply. JAMA otolaryngology-- head & neck surgery Qian, Z. J., Chang, K. W., Cheng, A. G. 2021

  View details for DOI 10.1001/jamaoto.2021.2162

  View details for PubMedID 34436534

• Trends and Healthcare Use Following Different Cholesteatoma Surgery Types in a National Cohort, 2003-2019. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology Qian, Z. J., Tran, E. D., Alyono, J. C., Cheng, A. G., Ahmad, I. N., Chang, K. W. 2021

  Abstract

  OBJECTIVE: To describe national trends in cholesteatoma management.STUDY DESIGN AND SETTING: Retrospective analysis Optum Clinformatics Database from 2003 to 2019.PATIENTS: 16,179 unique adult and pediatric patients who received cholesteatoma surgery.INTERVENTIONS AND MAIN OUTCOME MEASURES: Patients were categorized into three groups by initial surgical modality: canal wall down (CWD), canal wall up (CWU), and tympanoplasty without mastoidectomy (TnoM). Three major comparisons between groups were performed: 1) temporal trends, 2) clinical and sociodemographic determinants, and 3) healthcare use in terms of total costs and incidence of postoperative imaging and subsequent surgery.RESULTS: Overall, 23.2% received initial CWD surgery, 44.3% CWU, and 32.5% TnoM. 1) The incidence of initial CWD surgery decreased (odds ratios [OR] = 0.98, 95% confidence intervals [CI] [0.97,0.99]), while CWU increased (OR = 1.02, 95% CI [1.01,1.03]), and TnoM remained stable over the study period (OR = 0.99, 95% CI [0.98,1.00]). 2) Relative to CWU, TnoM surgery was less likely in adults, patients with prior complications, and non-White patients, while being more likely in patients with higher household income. CWD was more likely than CWU in adults, patients with prior complications, and non-White patients, while income had no effect. 3) Postoperative costs for CWU and CWD were similar. In 2 years following initial surgery, postoperative imaging and/or subsequent surgery was performed in 45.48% of CWD, 57.42% of CWU, and 41.62% of TnoM patients.CONCLUSIONS: Incidence of initial CWD surgery decreased and social disparities in cholesteatoma management were observed. Postoperative imaging or second-look surgery were performed in less than 60% of patients with initial CWU surgery and over 40% of patients with initial CWD.

  View details for DOI 10.1097/MAO.0000000000003284

  View details for PubMedID 34310551

• Social Perceptions of Pediatric Hearing Aids. The Laryngoscope Qian, Z. J., Nuyen, B. A., Kandathil, C. K., Truong, M., Tribble, M. S., Most, S. P., Chang, K. W. 2021

  Abstract

  OBJECTIVES: To determine whether hearing aid (HA) use affects social perceptions of general public adults and age-matched peers and if so, determine if effects are modulated by lack of societal representation of pediatric HAs.METHODS: A 10-year-old boy was presented in six photographic conditions with and without HAs and eyeglasses (a worn sensory aid with wider societal representation). HAs were presented in neutral skin tone and bright blue colors. Photographic conditions were embedded into web-based surveys with visual analog scales to capture social perceptions data and sourced to 206 adults (age 18-65) and 202 peers (age 10) with demographic characteristics representative of the general US population. Mean differences in scores for each condition compared to control images were computed using two-tailed t-tests.RESULTS: In both adult and child respondents, HAs were associated with decreased athleticism, confidence, health, leadership, and popularity. Glasses were associated with decreased athleticism and popularity but increased intelligence, overall success, and in the child respondents, friendliness. When worn in combination, the beneficial effects of glasses were mitigated by brightly colored but not neutrally colored HAs.CONCLUSION: Negative effects of pediatric HAs on social perceptions may be influenced by poor societal representation of HAs. These results suggest that greater representation of pediatric HAs is necessary to make society more inclusive for children with hearing loss.LEVEL OF EVIDENCE: 4 Laryngoscope, 2021.

  View details for DOI 10.1002/lary.29369

  View details for PubMedID 33405290

• Recommendations for Age-Appropriate Testing, Timing, and Frequency of Audiologic Monitoring During Childhood Cancer Treatment: An International Society of Paediatric Oncology Supportive Care Consensus Report. JAMA oncology Meijer, A. J., van den Heuvel-Eibrink, M. M., Brooks, B., Am Zehnhoff-Dinnesen, A. G., Knight, K. R., Freyer, D. R., Chang, K. W., Hero, B., Papadakis, V., Frazier, A. L., Blattmann, C., Windsor, R., Morland, B., Bouffet, E., Rutkowski, S., Tytgat, G. A., Geller, J. I., Hunter, L. L., Sung, L., Calaminus, G., Carleton, B. C., Helleman, H. W., Foster, J. H., Kruger, M., Cohn, R. J., Landier, W., van Grotel, M., Brock, P. R., Hoetink, A. E., Rajput, K. M. 2021

  Abstract

  Ototoxicity is an irreversible direct and late effect of certain childhood cancer treatments. Audiologic surveillance during therapy as part of the supportive care pathway enables early detection of hearing loss, decision-making about ongoing cancer treatment, and, when applicable, the timely use of audiologic interventions. Pediatric oncologic clinical practice and treatment trials have tended to be driven by tumor type and tumor-specific working groups. Internationally accepted standardized recommendations for monitoring hearing during treatment have not previously been agreed on.To provide standard recommendations on hearing loss monitoring during childhood cancer therapy for clinical practice.An Ototoxicity Task Force was formed under the umbrella of the International Society of Paediatric Oncology, consisting of international audiologists, otolaryngologists, and leaders in the field of relevant pediatric oncology tumor groups. Consensus meetings conducted by experts were organized, aimed at providing standardized recommendations on age-directed testing, timing, and frequency of monitoring during cancer treatment based on literature and consensus. Consensus statements were prepared by the core group, adapted following several videoconferences, and finally agreed on by the expert panel.The consensus reached was that children who receive ototoxic cancer treatment (platinum agents, cranial irradiation, and/or brain surgery) require a baseline case history, monitoring of their middle ear and inner ear function, and assessment of tinnitus at each audiologic follow-up. As a minimum, age-appropriate testing should be performed before and at the end of treatment. Ideally, audiometry with counseling before each cisplatin cycle should be considered in the context of the individual patient, specific disease, feasibility, and available resources.This is an international multidisciplinary consensus report providing standardized supportive care recommendations on hearing monitoring in children undergoing potentially ototoxic cancer treatment. The recommendations are intended to improve the care of children with cancer and facilitate comparative research on the timing and development of hearing loss caused by different cancer treatment regimens.

  View details for DOI 10.1001/jamaoncol.2021.2697

  View details for PubMedID 34383016

• Use of Diagnostic Testing and Intervention for Sensorineural Hearing Loss in US Children From 2008 to 2018. JAMA otolaryngology-- head & neck surgery Qian, Z. J., Chang, K. W., Ahmad, I. N., Tribble, M. S., Cheng, A. G. 2020

  Abstract

  Importance: Early detection and intervention of pediatric hearing loss is critical for language development and academic achievement. However, variations in the diagnostic workup and management of pediatric sensorineural hearing loss (SNHL) exist.Objective: To identify patient and clinician factors that are associated with variation in practice on a national level.Design, Setting, and Participants: This cross-sectional study used the Optum claims database to identify 53 711 unique children with SNHL between January 1, 2008, and December 31, 2018.Main Outcomes and Measures: National use rates and mean costs for diagnostic modalities (electrocardiogram, cytomegalovirus testing, magnetic resonance imaging, computed tomography, and genetic testing) and interventions (speech-language pathology, billed hearing aid services, and cochlear implant surgery) were reported. The associations of age, sex, SNHL laterality, clinician type, race/ethnicity, and household income with diagnostic workup and intervention use were measured in multivariable analyses.Results: Of 53 711 patients, 23 735 (44.2%) were girls, 2934 (5.5%) were Asian, 3797 (7.1%) were Black, 5626 (10.5%) were Hispanic, 33 441 (62.3%) were White, and the mean (SD) age was 7.3 (5.3) years. Of all patients, 32 200 (60.0%) were seen by general otolaryngologists, while 7573 (14.10%) were seen by pediatric otolaryngologists. Diagnostic workup was received by 14 647 patients (27.3%), while 13 482 (25.1%) received intervention. Use of genetic testing increased (odds ratio, 1.22 per year; 95% CI, 1.20-1.24), whereas use of computed tomography decreased (odds ratio, 0.93 per year; 95% CI, 0.92-0.94) during the study period. After adjusting for relevant covariables, children who were seen by pediatric otolaryngologists and geneticists had the highest odds of receiving workup and intervention. Additionally, racial/ethnic and economic disparities were observed in the use of most modalities of diagnostic workup and intervention for pediatric SNHL.Conclusions and Relevance: This cross-sectional study identified factors associated with disparities in the diagnostic workup and intervention of pediatric SNHL, thus highlighting the need for increased education and standardization in the management of this common sensory disorder.

  View details for DOI 10.1001/jamaoto.2020.5030

  View details for PubMedID 33377936

• Prevention of cisplatin-induced ototoxicity in children and adolescents with cancer: a clinical practice guideline. The Lancet. Child & adolescent health Freyer, D. R., Brock, P. R., Chang, K. W., Dupuis, L. L., Epelman, S., Knight, K., Mills, D., Phillips, R., Potter, E., Risby, D., Simpkin, P., Sullivan, M., Cabral, S., Robinson, P. D., Sung, L. 2019

  Abstract

  Despite ototoxicity being a prevalent consequence of cisplatin chemotherapy, little guidance exists on interventions to prevent this permanent and progressive adverse event. To develop a clinical practice guideline for the prevention of cisplatin-induced ototoxicity in children and adolescents with cancer, we convened an international, multidisciplinary panel of experts and patient advocates to update a systematic review of randomised trials for the prevention of cisplatin-induced ototoxicity. The systematic review identified 27 eligible adult and paediatric trials that evaluated amifostine, sodium diethyldithiocarbamate or disulfiram, systemic sodium thiosulfate, intratympanic therapies, and cisplatin infusion duration. Regarding systemic sodium thiosulfate, the panel made a strong recommendation for administration in non-metastatic hepatoblastoma, a weak recommendation for administration in other non-metastatic cancers, and a weak recommendation against its routine use in metastatic cancers. Amifostine, sodium diethyldithiocarbamate, and intratympanic therapy should not be routinely used. Cisplatin infusion duration should not be altered as a means to reduce ototoxicity. Further research to determine the safety of sodium thiosulfate in patients with metastatic cancer is encouraged.

  View details for DOI 10.1016/S2352-4642(19)30336-0

  View details for PubMedID 31866182

• International Pediatric Otolaryngology Group (IPOG) Consensus Recommendations: Congenital Cholesteatoma. Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology Denoyelle, F., Simon, F., Chang, K. W., Chan, K. H., Cheng, A. G., Cheng, A. T., Choo, D. I., Daniel, S. J., Farinetti, A., Garabedian, E., Greinwald, J. H., Hoff, S. R., Hone, S., Licameli, G. R., Papsin, B. C., Poe, D. S., Pransky, S., Smith, R. J., Triglia, J., Walton, J., Zalzal, G., Leboulanger, N. 2019

  Abstract

  OBJECTIVE: To provide recommendations to otolaryngologists and allied physicians for the comprehensive management of children who present with signs and symptoms of congenital cholesteatoma.METHODS: A two-iterative Delphi method questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group, on the preoperative work-up, the perioperative considerations, and follow-up.RESULTS: Twenty-two members completed the survey, in 14 tertiary-care center departments representing 5 countries. The main consensual recommendations were: a precise otoscopic description of the quadrants involved, extensive audiological workup (bilateral tonal, vocal audiometry, and BERA), and a CT scan are required. Facial nerve monitoring and a combination of microscope and telescope are recommended for surgical removal. Clinical and audiological follow-up should be pursued yearly for at least 5 years. First MRI follow-up should be done at 18 months postoperatively if the removal violated the matrix. MRI follow-up duration depends on the initial extent of the cholesteatoma.CONCLUSION: The goal of preoperative and follow-up consensus from International Pediatric Otolaryngology Group participants is to help manage infants and children with congenital cholesteatoma. The operative techniques may vary, and experienced surgeons must perform these procedures.

  View details for DOI 10.1097/MAO.0000000000002521

  View details for PubMedID 31851066

• Updates in Pediatric Cholesteatoma: Minimizing Intervention While Maximizing Outcomes. Otolaryngologic clinics of North America Luu, K., Chi, D., Kiyosaki, K. K., Chang, K. W. 2019

  Abstract

  Advances in pediatric otology have provided new tools to diagnose and manage complex otologic disease. Diffusion-weighted MRI provides the surgeon with a tool to detect recurrent or residual cholesteatoma that may obviate a second look surgery. Surgical trends move toward less invasive surgery. The literature provides good evidence to advocate for preserving the posterior ear canal and single stage ossiculoplasty. The growing popularity and increasing evidence in the literature supports otoendoscopy as a means to minimize invasiveness while eradicating disease in previously difficult to reach anatomic locations. These advances have allowed the otologic surgeon to improve surgical outcomes while minimizing intervention.

  View details for DOI 10.1016/j.otc.2019.05.003

  View details for PubMedID 31280890

• Autoimmune Lymphoproliferative Syndrome with Histopathologic Features of Castleman Disease Lo, R., Gratzinger, D., Keiser, E., Chang, K., Gernez-Goldhammer, Y. SPRINGER/PLENUM PUBLISHERS. 2019: S103–S104

  View details for Web of Science ID 000463709600168

• Recommendations for ototoxicity surveillance for childhood, adolescent, and young adult cancer survivors: a report from the International Late Effects of Childhood Cancer Guideline Harmonization Group in collaboration with the PanCare Consortium LANCET ONCOLOGY Clemens, E., van den Heuvel-Eibrink, M. M., Mulder, R. L., Kremer, L. M., Hudson, M. M., Skinner, R., Constine, L. S., Bass, J. K., Kuehni, C. E., Langer, T., van Dalen, E. C., Bardi, E., Bonne, N., Brock, P. R., Brooks, B., Carleton, B., Caron, E., Chang, K. W., Johnston, K., Knight, K., Nathan, P. C., Orgel, E., Prasad, P. K., Rottenberg, J., Scheinemann, K., de Vries, A. H., Walwyn, T., Weiss, A., Zehnhoff-Dinnesen, A., Cohn, R. J., Landier, W., Kadan-Lottick, N., Levitt, G., Hoetink, A., Mussman, J., Int Guideline Harmonization Grp 2019; 20 (1): E29–E41

  View details for DOI 10.1016/S1470-2045(18)30858-1

  View details for Web of Science ID 000454901700022

• International Consensus Recommendations on Microtia, Aural Atresia and Functional Ear Reconstruction. The journal of international advanced otology Zhang, T. Y., Bulstrode, N. n., Chang, K. W., Cho, Y. S., Frenzel, H. n., Jiang, D. n., Kesser, B. W., Siegert, R. n., Triglia, J. M. 2019; 15 (2): 204–8

  Abstract

  The aim of this report is to provide international recommendations for functional ear reconstruction in patients with microtia and aural atresia. All patients with microtia and external auditory atresia should be seen in the setting of a multidisciplinary team and agreed treatment outcomes should be measured, so that techniques, approaches, and results can be compared. The methods are expert opinion from the members of the International Microtia and Atresia Workgroup (IMAW). The consensus recommendations reported herein take into account the variability in practice patterns present among experts in the field; the degree of consensus was quantified by presenting the percentage of above authors who agree or partially agree with each statement. Recommendations include the definition and classification of microtia/atresia, treatment of microtia, treatment of congenital aural atresia, flowchart of functional ear reconstruction, and future research directions. Patients with microtia and aural atresia can be guided by the consensus recommendations provided herein.

  View details for DOI 10.5152/iao.2019.7383

  View details for PubMedID 31418720

• Exploring the Medical and Psychosocial Concerns of Adolescents and Young Adults With Craniofacial Microsomia: A Qualitative Study CLEFT PALATE-CRANIOFACIAL JOURNAL Hamilton, K. V., Ormond, K. E., Moscarello, T., Bruce, J. S., Merrell, S., Chang, K. W., Bernstein, J. A. 2018; 55 (10): 1430–39

  View details for DOI 10.1177/1055665618768542

  View details for Web of Science ID 000446871400011

• Renal ultrasound abnormalities in children with syndromic and non-syndromic microtia. International journal of pediatric otorhinolaryngology Koenig, J. L., Amoils, M., Grade, M. M., Chang, K. W., Truong, M. T. 2018; 113: 173–76

  Abstract

  OBJECTIVE: Renal abnormalities are commonly considered in the work up of pediatric patients with external ear malformations. However, there is little consensus regarding an appropriate renal screening protocol for patients with microtia. We sought to characterize renal abnormalities detected on ultrasonography in pediatric patients with microtia.METHODS: We conducted a retrospective cohort study of pediatric patients diagnosed with microtia who underwent renal ultrasound from 1991 to 2014 at a single tertiary academic institution. Renal ultrasound reports and medical records were reviewed to assess for renal abnormalities and to determine whether patients required specialist follow-up or interventions. Audiograms and otolaryngology notes were used to determine patterns of hearing loss. The following additional information was recorded from the electronic medical records: patient sex, microtia grade (I-IV), microtia laterality, and known associated syndromes. Characteristics were compared between those who did and did not have renal ultrasound findings using Fisher's exact test. Univariate logistic regression analysis was performed to determine factors associated with renal ultrasound findings.RESULTS: The majority of patients in this cohort were syndromic (n = 51, 64%) with grade III microtia (n = 46, 58%) and conductive hearing loss (n = 58, 72%). Syndromic children with microtia demonstrated a higher crude rate of renal ultrasound abnormalities (22%) than children with isolated microtia (7%). Of these patients, 69% required specialist follow-up. Univariate logistic regression analysis did not identify predictors that were significantly associated with renal ultrasound findings.CONCLUSION: Fairly high rates of abnormalities in syndromic and non-syndromic patients may warrant screening renal ultrasound in all patients with microtia, especially given the high percentage of findings requiring renal follow-up. A prospective study to formally evaluate screening efficacy is needed.

  View details for PubMedID 30173979

• Prevention of cisplatin-induced hearing loss in children: Informing the design of future clinical trials. Cancer medicine Minasian, L. M., Frazier, A. L., Sung, L. n., O'Mara, A. n., Kelaghan, J. n., Chang, K. W., Krailo, M. n., Pollock, B. H., Reaman, G. n., Freyer, D. R. 2018

  Abstract

  Cisplatin is an essential chemotherapeutic agent in the treatment of many pediatric cancers. Unfortunately, cisplatin-induced hearing loss (CIHL) is a common, clinically significant side effect with life-long ramifications, particularly for young children. ACCL05C1 and ACCL0431 are two recently completed Children's Oncology Group studies focused on the measurement and prevention of CIHL. The purpose of this paper was to gain insights from ACCL05C1 and ACCL0431, the first published cooperative group studies dedicated solely to CIHL, to inform the design of future pediatric otoprotection trials. Use of otoprotective agents is an attractive strategy for preventing CIHL, but their successful development must overcome a unique constellation of methodological challenges related to translating preclinical research into clinical trials that are feasible, evaluate practical interventions, and limit risk. Issues particularly important for children include use of appropriate methods for hearing assessment and CIHL severity grading, and use of trial designs that are well-informed by preclinical models and suitable for relatively small sample sizes. Increasing interest has made available new funding opportunities for expanding this urgently needed research.

  View details for PubMedID 29846043

• Diagnosis and Management of Congenital Sensorineural Hearing Loss Current Treatment Options in Pediatrics Kiyosaki, K., Chang, K. W. 2018; 4 (2): 174-182

  View details for DOI 10.1007/s40746-018-0119-y

• Evaluation and Management of Hearing Loss in Survivors of Childhood and Adolescent Cancers: A Report From the Children's Oncology Group PEDIATRIC BLOOD & CANCER Bass, J. K., Knight, K. R., Yock, T. I., Chang, K. W., Cipkala, D., Grewal, S. S. 2016; 63 (7): 1152-1162

  Abstract

  Hearing loss (HL) is common in childhood cancer survivors exposed to platinum chemotherapy and/or cranial radiation and can severely impact quality of life. Early detection and appropriate management can mitigate academic, speech, language, social, and psychological morbidity resulting from hearing deficits. This review is targeted as a resource for providers involved in aftercare of childhood cancers. The goal is to promote early identification of survivors at-risk for HL, appropriate evaluation and interpretation of diagnostic tests, timely referral to an audiologist when indicated, and to increase knowledge of current therapeutic options.

  View details for DOI 10.1002/pbc.25951

  View details for PubMedID 26928933

• Hearing Loss in Patients Who Received Cranial Radiation Therapy for Childhood Cancer. Journal of clinical oncology Bass, J. K., Hua, C., Huang, J., Onar-Thomas, A., Ness, K. K., Jones, S., White, S., Bhagat, S. P., Chang, K. W., Merchant, T. E. 2016; 34 (11): 1248-1255

  Abstract

  Patients treated with cranial radiation therapy (RT) are at risk for sensorineural hearing loss (SNHL). Although SNHL is often characterized as a delayed consequence of anticancer therapy, longitudinal reports of SNHL in childhood cancer survivors treated with contemporary RT are limited. We report the incidence, onset, severity, and long-term trajectory of SNHL among children receiving RT. Potential risk factors for SNHL were also identified.Serial audiologic testing was conducted on 235 pediatric patients who were treated with conformal or intensity-modulated RT as part of an institutional phase II trial for localized primary brain tumors, including craniopharyngioma, ependymoma, and juvenile pilocytic astrocytoma. All but one patient had measurable cochlear radiation dose (CRD) greater than 0 Gy. The median follow-up from RT initiation to latest audiogram was 9 years with a median of 11 post-RT audiograms per patient. Audiograms were classified by the Chang Ototoxicity Grading Scale. Progression was defined by an increase in Chang grade from SNHL onset to the most recent evaluation.At last evaluation, SNHL was prevalent in 14% of patients: 2.1% had mild and 11.9% had significant SNHL requiring hearing aids. Median time from RT to SNHL onset was 3.6 years (range, 0.4 to 13.2 years). Among 29 patients with follow-up evaluations after SNHL onset, 65.5% experienced continued decline in hearing sensitivity in either ear and 34.5% had no change. Younger age at RT initiation (hazard ratio [HR], 2.32; 95% CI, 1.21 to 4.46), higher CRD (HR, 1.07; 95% CI, 1.03 to 1.11), and cerebrospinal fluid shunting (HR, 2.02; 95% CI, 1.07 to 3.78) were associated with SNHL.SNHL is a late effect of RT that likely worsens over time. Long-term audiologic follow-up for a minimum of 10 years post-RT is recommended.

  View details for DOI 10.1200/JCO.2015.63.6738

  View details for PubMedID 26811531

• Postoperative Complications in Pediatric Tonsillectomy and Adenoidectomy in Ambulatory vs Inpatient Settings JAMA OTOLARYNGOLOGY-HEAD & NECK SURGERY Amoils, M., Chang, K. W., Saynina, O., Wise, P. H., Honkanen, A. 2016; 142 (4): 344-350

  Abstract

  A large-scale review is needed to characterize the rates of airway, respiratory, and cardiovascular complications after pediatric tonsillectomy and adenoidectomy (T&A) for inpatient and ambulatory cohorts.To identify risk factors for postoperative complications stratified by age and operative facility type among children undergoing T&A.This retrospective review included 115 214 children undergoing T&A in hospitals, hospital-based facilities (HBF), and free-standing facilities (FSF) in California from January 1, 2005, to December 31, 2010. The analysis used the State of California Office of Statewide Health Planning and Development private inpatient data and Emergency Department and Ambulatory Surgery public data. Inpatient (n = 18 622) and ambulatory (n = 96 592) cohorts were identified by codes from the International Classification of Diseases, Ninth Revision, and Current Procedural Terminology. Data were collected from September 2011 to March 2012 and analyzed from March through May 2012.Rates of airway, respiratory, and cardiovascular complications.A total of 18 622 inpatients (51% male; 49% female; mean age, 5.4 [range, 0-17] years) and 96 592 ambulatory patients (37% male; 35% female; 28%, masked; mean age, 7.6 [range, 0-17] years) underwent analysis. The ratio of ambulatory to inpatient procedures was 5:1. Inpatients demonstrated more comorbidities (≤8,compared with ≤4 for HBF and ≤3 for FSF patients) and, in general, their complication rates were 2 to 5 times higher (seen in 1% to 12% of patients) than those in HBFs (0.2% to 5%), and more than 10 times higher than those in the FSFs (0% to 0.38%), with rates varying markedly by age range and facility type. Tonsillectomy and adenoidectomy was associated with increased risk for all complication types in both settings, reaching an odds ratio of 8.5 (95% CI, 6.6-11.1) for respiratory complications in the ambulatory setting. Inpatients aged 0 to 9 years experienced higher rates of airway and respiratory complications, peaking at an odds ratio of 7.5 (95% CI, 3.1-18.2) for airway complications in the group aged 0 to 11 months.Large numbers of pediatric patients undergo T&A in ambulatory settings despite higher rates of complications in younger patients and patients with more comorbidities. Fortunately, a high percentage of these patients has been appropriately triaged to the inpatient setting. Further research is needed to elucidate the subgroups that warrant postoperative hospitalization.

  View details for DOI 10.1001/jamaoto.2015.3634

  View details for PubMedID 26915058

• A modified technique for firm elevation of the reconstructed auricle. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery Shan, J. n., Guo, Y. n., Chang, K. W., Zhang, T. n. 2016

  Abstract

  Reconstruction of the external ear for microtia remains one of the most challenging clinical problems in reconstructive surgery. Whether the reconstructive effort replicates a normal ear relates to making a delicate ear cartilage framework and achieving a firm projection of the constructed auricle. In this study, we present a new technique to create a firm elevation. 46 patients with congenital microtia, 30 male and 16 female, are included in this series. We used a modified Nagata's two-stage technique. In the second stage, instead of using a temporoparietal fascial flap to wrap a cartilage wedge, we developed a new technique using the retroauricular fascial flap wrapping a porous polyethylene (Medpor) wedge as the strut. None of the patients demonstrated any necrosis for the implanted auricle and grafted skin. None of the patients had postoperative exposure of the porous polyethylene wedge. The height of the protrusion for point 1 (the protrusion at superaurale level) and point 2 (the inferior end of the helix, where it connects with the lobule) ranged from 1.1 to 1.6 cm with the median of 1.23 cm, and from 0.8 to 1.1 cm with the median of 0.93 cm, respectively. The advantages of this technique are safe, practical and straightforward. The retroauricular fascial flap can help to fix the wedge and provide good vascular supply to the grafted skin. The porous polyethylene wedge provides excellent projection for the reconstructed auricle.

  View details for PubMedID 26781330

• Genetics of Hearing Loss-Nonsyndromic. Otolaryngologic clinics of North America Chang, K. W. 2015; 48 (6): 1063-1072

  Abstract

  Eighty percent of nonsyndromic hearing losses are caused by autosomal-recessive (AR) inheritance, while most of the other 20% are caused by autosomal-dominant (AD) inheritance. Although AR nonsyndromic SNHL is most commonly caused by GJB2 and SLC26A4, there is no single gene that accounts for any significant proportion of AD SNHL. High-throughput sequencing techniques, also called next-generation sequencing (NGS) or massively parallel sequencing (MPS), may allow for routine definitive diagnosis of all possible genetic causes for hearing loss in the not-too-distant future.

  View details for DOI 10.1016/j.otc.2015.06.005

  View details for PubMedID 26275501

• Hearing evaluation of patients with head and neck cancer: Comparison of Common Terminology Criteria for Adverse Events, Brock and Chang adverse event criteria in patients receiving cisplatin. Head & neck Colevas, A. D., Lira, R. R., Colevas, E. A., Lavori, P. W., Chan, C., Shultz, D. B., Chang, K. W. 2015; 37 (8): 1102-1107

  Abstract

  The purpose of this study was to compare Common Terminology Criteria for Adverse Events (CTCAE), Brock and Chang hearing loss grading in patients with head and neck cancer receiving cis-diamminedichloroplatinum (CDDP). Endpoints were baseline distribution of hearing loss, interobserver consistency, and sensitivity to hearing loss after CDDP treatment.Four hundred sixty single ear audiograms in 110 patients with head and neck cancer were graded. Hearing loss at baseline, interobserver agreement rates, and changes in hearing loss after CDDP were evaluated.The Chang and Brock tools' baseline hearing loss distribution was concentrated at grade 0 (57% and 41%, respectively), whereas 47%, per the CTCAE, had grade 3 baseline hearing loss. Interobserver agreement was highest for the Brock scale (≥90%) followed by the Chang (≥89%) and CTCAE (≥75%) scales. Detection of change after CDDP was highest for Chang (48%) followed by Brock (45%) and the CTCAE (32%).The Brock and Chang tools may be superior to the CTCAE in patients with head and neck cancer receiving CDDP using baseline hearing loss distribution, interobserver agreement, and detection of hearing loss grade change as performance indicators. © 2014 Wiley Periodicals, Inc. Head Neck, 2014.

  View details for DOI 10.1002/hed.23714

  View details for PubMedID 24737682

• Catch-up growth in infants with laryngomalacia after supraglottoplasty. International journal of pediatric otorhinolaryngology Czechowicz, J. A., Chang, K. W. 2015; 79 (8): 1333-1336

  Abstract

  Laryngomalacia, the most common congenital anomaly of the infant airway, can lead to poor feeding and failure to thrive. The decision to perform the standard surgical treatment, supraglottoplasty, is often based upon a sustained period of poor weight gain or weight loss.To characterize patterns of growth in infants with laryngomalacia, preceding and following supraglottoplasty.Retrospective chart review. Bioinformatics techniques were used to procure data from a clinical data warehouse based on the HL7 Reference Information Model consisting of all infants who underwent supraglottoplasty from June 1, 2005 to October 31, 2013. Height and weight measurements were obtained from 76 operated patients allowing for characterization of growth changes from the time of surgery to an average of 9 months following surgery. Logistic regression analysis was performed to examine the following variables for correlations with changes in weight, height and body mass index percentiles: patient age at surgery, preoperative weight, gender, and ethnic background.Academic pediatric tertiary medical center.Patients under 2 years of age at the time of surgery, who underwent supraglottoplasty for laryngomalacia, with height and weight measurements recorded within 3 months preceding surgery and greater than 3 months following surgery.Supraglottoplasty.Change in weight, height and body mass index (BMI) percentile from pre-op to post-op.BMI increased from a mean of 15.4 to 18.0 and BMI percentile from a mean of 34 to 51 from pre-op to post-op. The largest BMI percentile increases were observed in infants that were 3 months or younger at the time of surgery, as well as in those under 12 months of age, who were in the lowest BMI quintile.Infants who underwent supraglottoplasty tended to be small in weight and stature, and gained weight after surgery. Most of the weight gain occurred within 6 months of surgery. The largest increases in BMI percentile were observed in infants who were younger and smaller at the time of surgery while the infants older than 18 months had no significant gain.

  View details for DOI 10.1016/j.ijporl.2015.06.005

  View details for PubMedID 26096747

• Analysis of Growth Curves in Children After Adenotonsillectomy JAMA OTOLARYNGOLOGY-HEAD & NECK SURGERY Czechowicz, J. A., Chang, K. W. 2014; 140 (6): 491-496

  Abstract

  Several studies have documented that children gain more weight than expected after adenotonsillectomy.To examine patterns of change in weight and stature percentiles in children after adenotonsillectomy and to analyze clinical and demographic correlates of shifts in the growth curve.In this retrospective medical record review, we studied patients 18 years and younger who underwent adenotonsillectomy at an academic pediatric tertiary medical center and had at least one height and weight measurement recorded at each of the following time points: within 3 months before surgery, within 3 to 9 months after surgery, and within 12 to 27 months after surgery. Data were procured from all children from January 1, 2007, through October 31, 2012, and initially included 2893 surgical patients and 161,458 height and weight measurements. The final database consisted of 815 patients with adequate growth data and multiple time points. Logistic regression analysis was performed to examine patient age at surgery, preoperative weight, sex, and ethnic background for correlations with changes in weight, height, and body mass index percentiles.Change in weight, height, and body mass index percentile before and after surgery.At 18 months after surgery, weight percentiles in the study group increased by a mean of 6.3 percentile points (P < .001). Body mass index percentiles increased by a mean of 8.0 percentile points (P < .001). The greatest increases in weight percentiles were observed in children who were between the 1st and 60th percentiles for weight (P < .001) and younger than 4 years at the time of surgery (P < .001). An increase in weight percentile was not observed in children who preoperatively were already above the 80th percentile in weight (P = .15).Weight gain after adenotonsillectomy occurs primarily in patients who are smaller and younger at the time of surgery and does not correlate with increased rates of obesity.

  View details for DOI 10.1001/jamaoto.2014.411

  View details for Web of Science ID 000337888400001

• Concordance between the chang and the International Society of Pediatric Oncology (SIOP) ototoxicity grading scales in patients treated with cisplatin for medulloblastoma. Pediatric blood & cancer Bass, J. K., Huang, J., Onar-Thomas, A., Chang, K. W., Bhagat, S. P., Chintagumpala, M., Bartels, U., Gururangan, S., Hassall, T., Heath, J. A., McCowage, G., Cohn, R. J., Fisher, M. J., Robinson, G., Broniscer, A., Gajjar, A., Gurney, J. G. 2014; 61 (4): 601-605

  Abstract

  Reporting ototoxicity is frequently complicated by use of various ototoxicity criteria. The International Society of Pediatric Oncology (SIOP) ototoxicity grading scale was recently proposed for standardized use in reporting hearing loss outcomes across institutions. The aim of this study was to evaluate the concordance between the Chang and SIOP ototoxicity grading scales. Differences between the two scales were identified and the implications these differences may have in the clinical setting were discussed.Audiological evaluations were reviewed for 379 patients with newly diagnosed medulloblastoma (ages 3-21 years). Each patient was enrolled on one of two St. Jude clinical protocols that included craniospinal radiation therapy and four courses of 75 mg/m(2) cisplatin chemotherapy. The latest audiogram conducted 5.5-24.5 months post-protocol treatment initiation was graded using the Chang and SIOP ototoxicity criteria. Clinically significant hearing loss was defined as Chang grade ≥2a and SIOP ≥2. Hearing loss was considered serious (requiring a hearing aid) at the level of Chang grade ≥2b and SIOP ≥3.A strong concordance was observed between the Chang and SIOP ototoxicity scales (Stuart's tau-c statistic = 0.89, 95% CI: 0.86, 0.91). Among those patients diagnosed with serious hearing loss, the two scales were in good agreement. However, the scales deviated from one another in classifying patients with less serious or no hearing loss.Although discrepancies between the Chang and SIOP ototoxicity scales exist primarily for patients with no or minimal hearing loss, the scales share a strong concordance overall. Pediatr Blood Cancer 2014;61:601-605. © 2013 Wiley Periodicals, Inc.

  View details for DOI 10.1002/pbc.24830

  View details for PubMedID 24504791

• GJB2-Associated Hearing Loss: Systematic Review of Worldwide Prevalence, Genotype, and Auditory Phenotype LARYNGOSCOPE Chan, D. K., Chang, K. W. 2014; 124 (2): E34-E53

  Abstract

  To perform a systematic review of GJB2-associated hearing loss to describe genotype distributions and auditory phenotype.230 primary studies identified from Pubmed.Pubmed was searched systematically to screen broadly for any study reporting on genotype and carrier frequencies for biallelic GJB2-associated hearing loss in defined populations around the world. Genotype and audiometric data were extracted and subjected to meta-analysis to determine genotype distributions, carrier frequencies, rates of asymmetric or progressive hearing loss, and imaging abnormalities.A total of 216 articles comprising over 43,000 hearing-loss probands were included. The prevalence of biallelic GJB2-associated hearing loss was consistent across most of the 63 countries examined, with different mutations being predominant in different countries. Common mutations were found in greater than 3% of the general population worldwide. Meta-analysis of 48 case-control studies demonstrated a two-fold higher carrier frequency among hearing-impaired individuals compared to normal-hearing controls for truncating alleles, but not V37I. Progression, asymmetry, and imaging abnormalities were present in 14% to 19% of individuals with GJB2-associated hearing loss.GJB2 mutations are highly prevalent around the world. The multiple predominant mutations present in different populations attest to the importance of this gene for normal cochlear function and suggests an evolutionary heterozygote advantage. The unusually high carrier rate for truncating mutations among hearing-impaired individuals is consistent with either the presence of complementary mutations or a carrier phenotype. The significant rate of asymmetry and progression highlights the importance of diagnostic workup and close follow-up for this highly variable condition. Laryngoscope, 124:E34-E53, 2014.

  View details for DOI 10.1002/lary.24332

  View details for Web of Science ID 000329929900003

  View details for PubMedID 23900770

• The proximal bronchoplasty retrieval technique for removal of embedded distal airway foreign bodies INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY Hoff, S. R., Chang, K. W. 2014; 78 (1): 148-151

  Abstract

  To describe a novel technique for removal of embedded distal airway foreign bodies with emphasis on procedural steps and management.The proximal bronchoplasty retrieval (PBR) technique is described including indications, rationale, surgical technique, and clinical decision making. Two representative cases are described in detail in order to demonstrate the technique.The PBR technique was successfully used to remove the two described embedded distal airway foreign bodies after other attempts had failed.Airway foreign bodies which have been present for several days or weeks are associated with edema and granulation tissue which often embeds the foreign body and may prevent removal due to proximal airway narrowing. We present a novel technique using endoscopic balloon dilation bronchoplasty to enlarge the airway proximal to the foreign body and enable removal with two example cases presented herein. The PBR technique allowed for removal of otherwise "stuck" distal airway foreign bodies.

  View details for DOI 10.1016/j.ijporl.2013.10.051

  View details for PubMedID 24315084

• Hearing outcomes of atresia surgery versus osseointegrated bone conduction device in patients with congenital aural atresia: a systematic review. Otology & neurotology Nadaraja, G. S., Gurgel, R. K., Kim, J., Chang, K. W. 2013; 34 (8): 1394-1399

  Abstract

  To perform a systematic review, comparing hearing outcomes of atresiaplasty versus osseointegrated bone conduction device (OBCD) in congenital aural atresia (CAA) patients.Approximately 107 studies, published from 1975 to 2012, evaluating hearing outcomes after atresiaplasty and/or OBCD in CAA patients were identified through a PubMed search.Articles that evaluated external auditory canal stenosis alone, did not report speech reception threshold (SRT), pure tone average (PTA), hearing gain, or air-bone gap (ABG) or had less than 5 patients were excluded. For authors or institutions with multiple reports, the largest or most recent study was included. Forty-one articles satisfied our inclusion and exclusion criteria.The number and percentage of ears with a postoperative SRT, PTA, ABG less than 30 dB, and/or average hearing gain were extracted. The total number of ears and the timing of the postoperative audiogram were also noted.Of the atresiaplasty ears, 73.8% (95% CI, 62.2%-85.4%), had a SRT less than 30 dB (338 ears), 60.3% (95% CI, 45.8%-74.8%) had a PTA less than 30 dB (390 ears), and 68.9% (95% CI, 59.4%-78.3%) had an ABG less than 30 dB (852 ears). The average hearing gain was 24.1 dB (95% CI, 21.62-26.51) for 516 ears. Hearing outcomes deteriorated with time. Of OBCD patients, 95.9% (95% CI, 91.5%-100.0%) had a PTA less than 30 dB (77 ears), and 98.2% (95% CI, 94.5%-100.0%) had an ABG less than 30 dB (47 ears); the average hearing gain was 38.0 dB (95% CI, 33.14-45.22) in 100 ears.The OBCD has better hearing outcomes compared with atresiaplasty in patients with CAA.

  View details for DOI 10.1097/MAO.0b013e3182a36065

  View details for PubMedID 24005171

• VALIDATION OF THE SIOP BOSTON OTOTOXICITY SCALE- AN INTERNATIONAL SCALE FOR THE EVALUATION OF PLATINUM-INDUCED OTOTOXICITY IN CHILDREN Sefi, E., Brock, P., Chang, K., Neuwelt, E., Knight, K., Rajput, K. WILEY-BLACKWELL. 2013: 167

  View details for Web of Science ID 000324735501166

• Lip Abscess Associated With Isotretinoin Treatment of Acne Vulgaris JAMA DERMATOLOGY Huoh, K. C., Chang, K. W. 2013; 149 (8): 960-961

  Abstract

  IMPORTANCE Isotretinoin is frequently prescribed for the treatment of acne vulgaris. Among the numerous documented adverse effects, most common are xerostomia and cheilitis. Lip abscesses as a consequence of cheilitis present dramatically and may pose a diagnostic challenge. OBSERVATIONS We present a case of a 15-year-old boy with a severe lip abscess requiring incision and drainage and hospital admission for intravenous antibiotic treatment of methicillin-resistant Staphylococcus aureus. We discuss the pathophysiologic characteristics of isotretinoin therapy and the likely causative role that the medication played in the development of the lip abscess. CONCLUSIONS AND RELEVANCE Although rare, lip abscesses related to isotretinoin therapy present with substantial morbidity and should be promptly recognized. Misdiagnosis of mucositis and angioedema may delay appropriate therapy.

  View details for DOI 10.1001/jamadermatol.2013.4066

  View details for PubMedID 23760516

• beta-Adrenergic receptor expression in vascular tumors MODERN PATHOLOGY Chisholm, K. M., Chang, K. W., Truong, M. T., Kwok, S., West, R. B., Heerema-McKenney, A. E. 2012; 25 (11): 1446-1451

  Abstract

  Propranolol has recently emerged as an effective therapy for infantile hemangiomas causing regression. The β-adrenergic receptor (AR) antagonist is thought to cause vasoconstriction by its effect on nitric oxide, block angiogenesis by its effect on vascular endothelial growth factor (VEGF), and induce apoptosis. In a prior report, we identified expression of β2-AR (B2-AR) and its phosphorylated form (B2-ARP) in a case of infantile hemangioma that responded to propranolol treatment. We now explore the expression of βARs on a variety of vascular lesions utilizing a tissue microarray containing 141 lesions, including infantile hemangiomas, angiosarcomas, hemangiomas, hemangioendotheliomas, and various vascular malformations. The array was immunostained for B2-AR, B2-ARP, and β3-AR (B3-AR), and the results scored for the intensity of endothelial cell expression as negative, weak positive, or strong positive. All phases of infantile hemangiomas had strong expression of all three receptors, with the exception of only weak expression of B2-ARP in the proliferative phase infantile hemangioma. Strong expression of all three receptors was present in many hemangiomas, hemangioendotheliomas, and vascular malformations. Absent to weak expression of all three receptors was seen in glomus tumor, hobnail hemangioendothelioma, pyogenic granuloma, and reactive vascular proliferations. This is the first study to report β-AR expression in a variety of vascular lesions. Although immunohistochemical expression of the receptors does not necessarily indicate that similar pathways of responsiveness to β-blockade are present, it does raises the possibility that β-blockade could potentially affect apoptosis and decrease responsiveness to VEGF. Additional study is warranted, as therapeutic options are limited for some patients with these lesions.

  View details for DOI 10.1038/modpathol.2012.108

  View details for PubMedID 22743651

• Platinum-Induced Ototoxicity in Children: A Consensus Review on Mechanisms, Predisposition, and Protection, Including a New International Society of Pediatric Oncology Boston Ototoxicity Scale JOURNAL OF CLINICAL ONCOLOGY Brock, P. R., Knight, K. R., Freyer, D. R., Campbell, K. C., Steyger, P. S., Blakley, B. W., Rassekh, S. R., Chang, K. W., Fligor, B. J., Rajput, K., Sullivan, M., Neuwelt, E. A. 2012; 30 (19): 2408-2417

  Abstract

  The platinum chemotherapy agents cisplatin and carboplatin are widely used in the treatment of adult and pediatric cancers. Cisplatin causes hearing loss in at least 60% of pediatric patients. Reducing cisplatin and high-dose carboplatin ototoxicity without reducing efficacy is important.This review summarizes recommendations made at the 42nd Congress of the International Society of Pediatric Oncology (SIOP) in Boston, October 21-24, 2010, reflecting input from international basic scientists, pediatric oncologists, otolaryngologists, oncology nurses, audiologists, and neurosurgeons to develop and advance research and clinical trials for otoprotection.Platinum initially impairs hearing in the high frequencies and progresses to lower frequencies with increasing cumulative dose. Genes involved in drug transport, metabolism, and DNA repair regulate platinum toxicities. Otoprotection can be achieved by acting on several these pathways and generally involves antioxidant thiol agents. Otoprotection is a strategy being explored to decrease hearing loss while maintaining dose intensity or allowing dose escalation, but it has the potential to interfere with tumoricidal effects. Route of administration and optimal timing relative to platinum therapy are critical issues. In addition, international standards for grading and comparing ototoxicity are essential to the success of prospective pediatric trials aimed at reducing platinum-induced hearing loss.Collaborative prospective basic and clinical trial research is needed to reduce the incidence of irreversible platinum-induced hearing loss, and optimize cancer control. Wide use of the new internationally agreed-on SIOP Boston ototoxicity scale in current and future otoprotection trials should help facilitate this goal.

  View details for DOI 10.1200/JCO.2011.39.1110

  View details for Web of Science ID 000306182300020

  View details for PubMedID 22547603

• Is It Valid to Calculate the 3-Kilohertz Threshold by Averaging 2 and 4 Kilohertz? OTOLARYNGOLOGY-HEAD AND NECK SURGERY Gurgel, R. K., Popelka, G. R., Oghalai, J. S., Blevins, N. H., Chang, K. W., Jackler, R. K. 2012; 147 (1): 102-104

  Abstract

  Many guidelines for reporting hearing results use the threshold at 3 kilohertz (kHz), a frequency not measured routinely. This study assessed the validity of estimating the missing 3-kHz threshold by averaging the measured thresholds at 2 and 4 kHz. The estimated threshold was compared to the measured threshold at 3 kHz individually and when used in the pure-tone average (PTA) of 0.5, 1, 2, and 3 kHz in audiometric data from 2170 patients. The difference between the estimated and measured thresholds for 3 kHz was within ± 5 dB in 72% of audiograms, ± 10 dB in 91%, and within ± 20 dB in 99% (correlation coefficient r = 0.965). The difference between the PTA threshold using the estimated threshold compared with using the measured threshold at 3 kHz was within ± 5 dB in 99% of audiograms (r = 0.997). The estimated threshold accurately approximates the measured threshold at 3 kHz, especially when incorporated into the PTA.

  View details for DOI 10.1177/0194599812437156

  View details for PubMedID 22301102

• CT Analysis Demonstrates That Cochlear Height Does Not Change with Age AMERICAN JOURNAL OF NEURORADIOLOGY Mori, M. C., Chang, K. W. 2012; 33 (1): 119-123

  Abstract

  CH measurements can often be useful in the diagnosis of inner ear malformations associated with SNHL. Our aim was to establish the relationship between CH and age by using analysis of CT images in patients who underwent coronal CT scans of the temporal bone between 2001 and 2007.We measured CH on coronal CT scans of the temporal bone of 422 ears scanned from 2001 to 2007 in 211 patients, 1 month to 23 years of age. Using multivariate linear regression analysis, we determined the relationship of CH to age, sex, and HL type. In addition, 11 patients with multiple scans at different ages were assessed for change in CH with age.Average CH was 5.3 mm (normal range, 4.4-6.2 mm). Analysis showed no statistically significant change in CH across ages from 1 month to 23 years (95% CI for regression line slope = -0.003, 0.013). Likewise, there were no statistically significant differences in CH for patients with multiple scans at different ages. ICW increased with age as expected with increased cranial size. A small difference in CH between sexes was noted with males having greater CHs than females (P < .01). All patients with hypoplastic cochleas, defined by a CH <2 SDs from the mean (4.48 mm for males and 4.25 mm for females), had HL with a positive predictive value of 86%.CH does not change from 1 month of age to adulthood and is slightly greater in males than in females.

  View details for DOI 10.3174/anjr.A2713

  View details for PubMedID 21960502

• Clinically Accurate Assessment and Grading of Ototoxicity LARYNGOSCOPE Chang, K. W. 2011; 121 (12): 2649-2657

  Abstract

  To design a grading scale and audiologic protocols to better reflect clinical impact than the currently widely used classifications.Retrospective.Audiologic studies from 130 patients receiving cisplatin chemotherapy were analyzed. Pure-tone audiograms were evaluated using the newly proposed grading criteria, Brock criteria, and Common Terminology Criteria for Adverse Events (CTCAE). The resulting grades were then compared to recommended audiologic interventions. Auditory brainstem response (ABR) data and data on incidences of conductive hearing loss from other multi-institutional studies that the author participated in were compared to data from this cohort.Although the newly proposed, Brock, and CTCAE ototoxicity grades were significantly related to audiologist recommendations for assistive devices (P < .0001), the newly proposed criteria were more specific and allowed better delineation of different patients into distinct subgroups requiring either FM system (grade 2a) or hearing aid (grade 2b or higher). Multi-institutional data review indicated significant problems with ABR evaluations and separating out conductive hearing losses from ototoxicity.The newly proposed grading system needs to be paired with specific recommendations regarding audiologic monitoring protocols as well as active participation by the audiologists implementing the protocol to provide clinically accurate assessment and grading of ototoxicity.

  View details for DOI 10.1002/lary.22376

  View details for PubMedID 22109766

• Wegener's granulomatosis in an adolescent presenting with pachymeningitis, mastoid effusion and Horner's syndrome INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY EXTRA Kohlberg, G. D., Mai Thy Truong, Chang, K. W. 2011; 6 (2): 80–84

  View details for DOI 10.1016/j.pedex.2010.03.009

  View details for Web of Science ID 000435153000009

• Diagnostic Yield in the Workup of Congenital Sensorineural Hearing Loss Is Dependent on Patient Ethnicity OTOLOGY & NEUROTOLOGY Chan, D. K., Schrijver, I., Chang, K. W. 2011; 32 (1): 81-87

  Abstract

  Diagnostic yield on GJB2 sequencing and computed tomography in the workup for idiopathic congenital sensorineural hearing loss is related to patient ethnicity.GJB2 sequencing and computed tomography of the temporal bones are important initial diagnostic tests in the workup of idiopathic congenital sensorineural hearing loss. Previous studies showed an association between mild or unilateral hearing loss and positive imaging findings and between severe or bilateral deafness and GJB2 mutations. Recent studies on connexin 26-associated deafness demonstrate a wide range of phenotypes that vary with ethnicity.We present a retrospective case series of 271 consecutive ethnically diverse patients evaluated for idiopathic congenital sensorineural hearing loss. Results of genetic testing and imaging were correlated with audiologic findings and ethnicity.All patients with asymmetric hearing loss had more positive findings on imaging. With respect to the severity of hearing loss, however, differences were noted between ethnic groups. Whereas white patients conformed to previous findings, Hispanics with severe hearing loss had similar rates of positive imaging and genetic testing results. Asians with mild hearing loss had significantly greater yield on genetic testing rather than on imaging. This reflects the high prevalence of the p.V37I mutation in GJB2 among Asians, which gives rise to a mild, frequently progressive phenotype.Ethnicity should be considered when determining the optimal sequence of diagnostic testing for idiopathic congenital sensorineural hearing loss. Asian patients, in particular, should all be screened for mutations in GJB2, especially in the case of mild hearing loss.

  View details for DOI 10.1097/MAO.0b013e3181fc786f

  View details for PubMedID 21042228

• Propranolol for the treatment of airway hemangiomas: A case series and treatment algorithm INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY Truong, M. T., Perkins, J. A., Messner, A. H., Chang, K. W. 2010; 74 (9): 1043-1048

  Abstract

  (1) To present six patients with symptomatic airway hemangiomas treated with oral propranolol. (2) To review the diagnostic and treatment options for airway hemangiomas and propose a new management protocol.Retrospective review.Tertiary care children's hospital.Pediatric patients diagnosed with obstructive airway hemangiomas treated with oral propranolol. Patients were followed for symptomatic improvement and relief of airway obstruction on imaging or laryngoscopy.Seven patients presenting with airway obstruction were treated with propranolol. One patient had a focal hemangioma confined to the subglottis. Four patients had airway hemangiomas that extended beyond the confines of the larynx and trachea. A sixth patient had a bulky supraglottic hemangioma. A seventh patient with an extensive maxillofacial lesion failed propranolol therapy and was found to have a pyogenic granuloma on final pathology after excision. Six patients had failed standard medical therapy and/or surgical interventions and were treated successfully with oral propranolol with improvements in airway symptoms and oral intake, requiring no further surgical intervention. Treatment was initiated as early as 1.5 months of age, and as late as 22 months. No adverse side effects of propranolol were noted.Oral propranolol was successfully used to treat airway hemangiomas, resulting in rapid airway stabilization, obviating the need for operative intervention, and reducing the duration of systemic corticosteroid therapy while causing no obvious adverse effects. These outstanding results enable the possibility of use of a standardized diagnostic and treatment algorithm for airway hemangiomas that incorporates systemic propranolol.

  View details for DOI 10.1016/j.ijporl.2010.06.001

  View details for Web of Science ID 000281615300015

  View details for PubMedID 20674045

• Practical Grading System for Evaluating Cisplatin Ototoxicity in Children JOURNAL OF CLINICAL ONCOLOGY Chang, K. W., Chinosornvatana, N. 2010; 28 (10): 1788-1795

  Abstract

  We present a new ototoxicity grading system that has clearly defined and frequency-specific audiometric criteria. The purpose of this study was to validate this grading system by assessing its correspondence to audiology treatment recommendations and comparing it with the currently utilized Common Terminology Criteria for Adverse Events (CTCAE).A retrospective chart review was conducted using audiologic, demographic, and clinical data from 134 children receiving 149 courses of chemotherapy consisting of cisplatin and/or carboplatin. Pure-tone audiograms were evaluated using both our proposed grading criteria and the CTCAE criteria. The resulting grades were then compared with charted audiologic interventions and a number of clinical parameters to assess the clinical validity of the grading scale.Chang grade 2a or higher predicted audiologic intervention. Although both the Chang and CTCAE ototoxicity grades were significantly related to audiologist recommendations for assistive devices such as hearing aids and/or frequency modulated systems (P < .0001), the Chang scale was more specific, with the CTCAE scale diverging from clinical recommendation at higher grades. As expected, patients receiving cisplatin had more severe hearing loss with concurrent carboplatin administration, radiation therapy exposure, younger age, smaller body-surface area, longer treatment exposure, and more severe disease.This grading system provides robust and clinically useful criteria to represent clinical hearing loss induced by ototoxicity with regard to the impact on speech and language and the need for assistive hearing devices. It is both more specific and more sensitive than the traditional CTCAE criteria for identifying clinically significant ototoxicity.

  View details for DOI 10.1200/JCO.2009.24.4228

  View details for PubMedID 20194861

• Connexin-26-associated deafness: Phenotypic variability and progression of hearing loss GENETICS IN MEDICINE Chan, D. K., Schrijver, I., Chang, K. W. 2010; 12 (3): 174-181

  Abstract

  To evaluate genotype-phenotype correlation over time for a cohort of children with connexin-26 (GJB2)-associated autosomal recessive hearing loss.Fifty-two children were identified from a database of individuals with homozygous or compound heterozygous mutations in GJB2 and subjected to chart review of their otolaryngologic and serial audiometric evaluations. Genotype-phenotype correlations were identified among the members of this group by appropriate statistical analyses.Hearing loss was most severe in individuals with two truncating mutations in GJB2 and mildest in those with two nontruncating mutations. Progressive hearing loss was seen directly by serial audiometry in 24% of all subjects, and suggested in a total of 28% when those with normal newborn hearing screens and subsequent hearing loss were included. Progression was particularly common among carriers of the p.V37I allele either in homozygosity or in compound heterozygosity with a truncating allele; these children are primarily of Asian descent and demonstrate mild, slowly progressive hearing loss.Phenotype in GJB2-associated hearing loss is correlated with genotype, with truncating mutations giving rise to more severe hearing loss. Progression of hearing loss is not uncommon, especially in association with the p.V37I allele. These results suggest that close audiometric follow-up is warranted for patients with GJB2-associated recessive hearing loss.

  View details for DOI 10.1097/GIM.0b013e3181d0d42b

  View details for PubMedID 20154630

• Propranolol for the Treatment of a Life-Threatening Subglottic and Mediastinal Infantile Hemangioma JOURNAL OF PEDIATRICS Truong, M. T., Chang, K. W., Berk, D. R., Heerema-McKenney, A., Bruckner, A. L. 2010; 156 (2): 335-338

  Abstract

  An infant with a subglottic hemangioma remained in respiratory distress after multiple treatments failed and was found to have an enlarging mediastinal infantile hemangioma compressing the trachea. Treatment with oral propranolol resulted in resolution of symptoms within 2 days and a 50% reduction in lesion size within 1 week.

  View details for DOI 10.1016/j.jpeds.2009.10.010

  View details for PubMedID 20105647

• Amifostine Does Not Prevent Platinum-Induced Hearing Loss Associated With the Treatment of Children With Hepatoblastoma CANCER Katzenstein, H. M., Chang, K. W., Krailo, M., Chen, Z., Finegold, M. J., Rowland, J., Reynolds, M., Pappo, A., London, W. B., Malogolowkin, M. 2009; 115 (24): 5828-5835

  Abstract

  The current study was conducted to determine whether amifostine is effective in reducing the toxicities associated with the administration of platinum-containing regimens in children with hepatoblastoma (HB).Patients were enrolled on P9645 beginning in March of 1999. Patients who had stage I/II disease received treatment with 4 cycles of combined cisplatin, 5-fluorouracil, and vincristine (C5V) with or without amifostine. Patients who had stage III/IV disease were randomized to receive treatment with 6 cycles of either C5V with or without amifostine or carboplatin alternating with cisplatin (CC) with or without amifostine. Patients who were randomized to receive amifostine were given a dose of 740 mg/m2 intravenously over 15 minutes before each administration of a platinum agent.Eighty-two patients were considered in a special interim analysis of the incidence of toxicity. The disease outcome for patients who received amifostine was similar to the outcome for patients who did not receive amifostine (P=.22). The incidence of significant hearing loss (>40 dB) was similar for patients who did or did not receive amifostine (38% [14 of 37 patients] vs 38% [17 of 45 patients], respectively; P=.68). There were no differences in the incidence of renal or bone marrow toxicities evaluated. Patients who received amifostine had a higher incidence of hypocalcemia (5% vs 0.5%; P=.00006).Amifostine in the doses and schedule used in this study failed to significantly reduce the incidence of platinum-induced toxicities in patients with HB.

  View details for DOI 10.1002/cncr.24667

  View details for Web of Science ID 000272545400028

  View details for PubMedID 19813275

  View details for PubMedCentralID PMC2795100

• Salivary Gland Anlage Tumor in a Neonate Presenting with Respiratory Distress: Radiographic and Pathologic Correlation AMERICAN JOURNAL OF NEURORADIOLOGY Mogensen, M. A., Lin, A. C., Chang, K. W., Berry, G. J., Barnes, P. D., Fischbein, N. J. 2009; 30 (5): 1022-1023

  Abstract

  We present a case of congenital salivary gland anlage tumor (SGAT) of the nasal septum in a 2-week-old infant who had difficulty breathing through her nose since birth. CT and MR imaging demonstrated a circumscribed mass within the nasal cavity that did not communicate with the intracranial compartment. Differential diagnosis and clinical significance of recognizing this rare lesion are reviewed.

  View details for DOI 10.3174/ajnr.A1364

  View details for PubMedID 19112069

• Evaluation of unilateral referrals on neonatal hearing screening JOURNAL OF MEDICAL SCREENING Chang, K. W., O-Lee, T. J., Price, M. 2009; 16 (1): 17-21

  Abstract

  Examination of neonatal hearing screening practices around the world suggests that more attention is placed on infants who fail bilaterally on their hearing screen than infants who refer (fail) in one ear. Some programmes only report bilateral failures as positive hearing screens. This study investigates how limitations of the screening techniques demand continued audiologic evaluations in unilateral referrals.The study sample consisted of all infants born at a single academic paediatric hospital between February 1998 and February 2002.There were 16,007 infants screened using ALGO automated auditory brainstem response. Eighteen of the infants who failed the screen in one ear but passed in the other ear were found to have permanent hearing loss, and had their subsequent clinical course and audiologic management analysed. The final audiologic outcome after four years in both the pass and fail ear were examined.One group of unilateral referrals (n = 6) had obvious anatomic reasons for the ear failing the screen (canal atresia/stenosis). There were five patients in which the ear that passed the screen was later found on more extensive audiologic evaluation to have significant hearing loss. Review of recent literature was also completed to examine the methods by which unilateral screening referrals are commonly reported and whether or not this affected follow-up diagnostic evaluation.Infants who pass one ear and refer one ear on neonatal hearing screening still need to have thorough and prompt evaluations. In many cases, the ear that passed can be found to have significant hearing loss.

  View details for DOI 10.1258/jms.2009.007113

  View details for PubMedID 19349526

• Otorrhea in infants with tympanostomy tubes before and after surgical repair of a cleft palate Arch Otolaryngol Head Neck Surg Curtin, G., Messner, A. H., Chang, K. W. 2009; 135 (8): 748-51

  View details for DOI 10.1001/archoto.2009.106

• Intracapsular versus subcapsular coblation tonsillectomy OTOLARYNGOLOGY-HEAD AND NECK SURGERY Chang, K. W. 2008; 138 (2): 153-157

  Abstract

  To compare the postoperative recovery of patients who undergo intracapsular to subcapsular Coblation tonsillectomy.This was a prospective, randomized, double-blinded study. A total of 69 children, aged 2 to 16 years, were randomized to intracapsular (n = 34) or subcapsular (n = 35) tonsillectomy. The Coblation technique was used with both groups. Outcomes measures were assessed on postoperative day 1 or 2 and 5 or 6. These included child and parental rating of pain with the Wong Faces pain scale, analgesic use, oral intake, and activity level.Intracapsular tonsillectomy patients had similar levels of pain to subcapsular tonsillectomy patients on day 1 or 2. However, at day 5 or 6, intracapsular tonsillectomy patients had significantly less pain than the subcapsular tonsillectomy patients. Intracapsular patients ate more and were more active at both time points.Children with obstructive sleep apnea who undergo tonsillectomy demonstrate better postoperative recovery after intracapsular tonsillectomy. The intracapsular versus subcapsular difference may be best appreciated at a delayed time point (day 5 or 6) rather than early (day 1 or 2).

  View details for DOI 10.1016/j.otohns.2007.11.006

  View details for PubMedID 18241707

• Utility of temporal bone computed tomographic measurements in the evaluation of inner ear malformations ARCHIVES OF OTOLARYNGOLOGY-HEAD & NECK SURGERY Chen, J. L., Gittleman, A., Barnes, P. D., Chang, K. W. 2008; 134 (1): 50-56

  Abstract

  To investigate whether normative inner ear measurements can assist in the evaluation of sensorineural hearing loss (SNHL).Retrospective cohort review.A tertiary care hospital.Computed tomography or magnetic resonance imaging was performed on 188 ears with SNHL and 220 ears without SNHL (204 children) between 2001 and 2004.Two readers measured the basal turn of cochlea (BTC) lumen, lateral semicircular canal (LSCC) bony island width, superior semicircular canal (SSCC) bony island width, and cochlear height (CH).A t test was performed comparing measurements in patients with and without SNHL. Interobserver variability was characterized by intraclass correlation coefficients and Bland-Altman plots.The t test results demonstrated no statistically significant differences between inner ear measurements in those with and without SNHL. The intraclass correlation coefficients for BTC lumen, CH, LSCC bony island width, and SSCC bony island width measurements was 0.612, 0.632, 0.869, and 0.912, respectively. Bland-Altman plots revealed systematic biases of 1%, 8%, 10%, and 21% for the BTC lumen, SSCC bony island width, LSCC bony island width, and CH measurements, respectively.Inner ear measurements in children with and without SNHL are not statistically different. Moreover, the measurements are difficult to interpret because while they demonstrate good reproducibility, they are susceptible to systematic biases. However, use of inner ear measurements is more sensitive in identifying vestibulocochlear dysplasias and should be considered to complement visual analysis.

  View details for PubMedID 18209137

• Third branchial cleft fistula infected with Actinomyces INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY EXTRA Chang, K. W., Lee, B. G., Gutierrez, K. M. 2008; 3 (1): 20–23

  View details for DOI 10.1016/j.pedex.2007.08.007

  View details for Web of Science ID 000435038800006

• Recovery from cisplatin-induced ototoxicity. Int J Pediatr Otorhinolaryngol Truong, M. T., Winzelberg, J., Chang, K. W. 2007; 71 (10): 1631-8
• Two patients with the V371/235delC genotype: Are radiographic cochlear anomalies part of the phenotype? INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY Schrijver, I., Chang, K. W. 2006; 70 (12): 2109-2113

  Abstract

  We present two East Asian patients with sensorineural hearing loss (SNHL) and compound heterozygosity for the 235delC and V37I mutations in the GJB2 gene. One patient has a unilaterally enlarged vestibular aqueduct, which underscores the importance of routine CT examination in children with SNHL, even if GJB2 (connexin 26) mutations have been identified. The second patient was not available for evaluation by CT. The pathogenic role of the V37I mutation has been controversial. We review the literature and present evidence in support of pathogenicity. Larger studies in compound heterozygous individuals and co-transfection studies will allow better genotype-phenotype correlations and prognostication.

  View details for DOI 10.1016/j.ijporl.2006.07.015

  View details for PubMedID 16952406

• Familial laryngomalacia in two siblings with syndromic features INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY Chen, J. L., Messner, A. H., Chang, K. W. 2006; 70 (9): 1651-1655

  Abstract

  We present two siblings with severe laryngomalacia requiring surgical intervention during the newborn period, microcephaly, developmental delay, cleft palate, preaxial polydactyly, dysplastic nails and conductive hearing loss (persistent after tympanostomy tube placement). In addition the girl has microopthalmia and the boy was born with a patent ductus arteriosus, mild pelviectasis, and hypospadias. This combination of multiple congenital anomalies has not been described previously and may represent a previously undescribed syndrome with autosomal inheritance.

  View details for DOI 10.1016/j.ijporl.2006.04.010

  View details for PubMedID 16774790

• Posttransplantation lymphoproliferative disorder of the paranasal sinuses in a child INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY EXTRA Rosen, F. S., Bingham, D., Chang, K. W. 2006; 1 (1): 22–26

  View details for DOI 10.1016/j.pedex.2005.11.005

  View details for Web of Science ID 000435033000007

• Amifostine does not protect against the ototoxicity of high-dose cisplatin combined with etoposide and bleomycin in pediatric germ-cell tumors - A children's Oncology Group study CANCER Marina, N., Chang, K. W., Malogolowkin, M., London, W. B., Frazier, A. L., Womer, R. B., Rescorla, F., Billmire, D. F., DAVIS, M. M., Perlman, E. J., Giller, R., Lauer, S. J., Olson, T. A. 2005; 104 (4): 841-847

  Abstract

  High-dose cisplatin combined with etoposide and bleomycin (HDPEB) improves event-free survival (EFS) in advanced pediatric germ-cell tumors (PGCT), but has significant ototoxicity. Amifostine appears to protect against toxicity. The authors combined amifostine with HDPEB and evaluated the efficacy and toxicity, specifically whether ototoxicity decreased.Eligibility criteria included age < 15 years and unresectable Stage III/IV extracranial, extragonadal PGCT. Patients received bleomycin 15 IU/m(2) on Day 1, then etoposide 100 mg/m(2) per day, amifostine 825 mg/m(2) per day, and cisplatin 40 mg/m(2)per day on Days 1-5, intravenously. The cycles were repeated every 3-4 weeks with imaging evaluation after 4 cycles. Patients with residual radiographic abnormalities underwent resection. Patients with residual tumor received two additional HDPEB cycles. Hearing evaluations were required at diagnosis and after two and four cycles. Audiologic results were reviewed and compared with historical controls treated with HDPEB.Twenty-five eligible patients were enrolled between April 2000 and April 2002. Their median age was 1.6 years (range, 0.64-13.9 years), 17 patients were female, 11 had metastases, and 24 had a yolk sac carcinoma component histologically. Primary sites included sacrococcygeal area/pelvis (n = 15), vagina (n = 5), and other (n = 5). Two-year EFS and overall survival were 83.5% +/- 12.8% and 85.6% +/- 12.3%, respectively. Eight patients were removed from the study (four had progressive disease/disease recurrence and four had ototoxicity). Grade 3/4 toxicities included neutropenia (n = 20), thrombocytopenia (n = 14), electrolyte imbalances (n = 14), and gastrointestinal toxicity (n = 12). Twenty-four of 25 patients received hearing evaluations, and 75% had significant hearing loss.Amifostine did not protect against HDPEB-associated ototoxicity.

  View details for DOI 10.1002/cncr.21218

  View details for PubMedID 15999362

• Not your routine foreign body: Endobronchial tuberculosis in an infant PEDIATRICS Agarwal, S., Hong, D. K., Soslow, J., Chang, K. W. 2005; 116 (1): 246-248

  Abstract

  Foreign-body aspiration is a common cause of respiratory distress among children. Here we describe an 8-month-old, previously 34-week premature, male patient who presented with a 1-day history of fever and increased work of breathing. Of note, 3 weeks before presentation, the patient had been treated with orally administered amoxicillin for presumed pneumonia and exhibited good clinical response. No chest radiograph was obtained at that time. A current chest radiograph revealed hyperexpansion of the left lung, with a mediastinal shift. Although the patient was referred because of possible foreign-body aspiration, no clear history of an aspiration event was obtained, and computed tomographic scans of the chest were recommended. These showed extensive hilar and mediastinal lymphadenopathy, resulting in obstruction of the left bronchus. Bronchoscopy revealed a cheesy granulomatous mass in the left mainstem bronchus, which was ball-valving into the upper bronchus. Removal resulted in improvement of the patient's respiratory status. Pathology, bronchial lavage, and gastric aspirate specimens all revealed acid-fast bacilli, consistent with Mycobacterium tuberculosis infection. This unusual presentation of tuberculosis may become more common in the United States as the incidence of immigrants carrying tuberculosis increases.

  View details for PubMedID 15995065

• Randomized controlled trial of Coblation versus electrocautery tonsillectomy Otolaryngol Head Neck Surg Chang, K. W. 2005; 132 (2): 273-280

  View details for DOI 10.1016/j.otohns.2004.11.002

• Prevention of cisplatin ototoxicity using transtympanic N-acetylcysteine and lactate OTOLOGY & NEUROTOLOGY Choe, W. T., Chinosornvatana, N., Chang, K. W. 2004; 25 (6): 910-915

  Abstract

  Transtympanic administration of the antioxidant N-acetylcysteine or lactated Ringer's solution onto the round window membrane will prevent cisplatin ototoxicity in the guinea pig model.Cochlear ototoxicity is a well-known side effect of cisplatin administration, with the mechanism of injury thought to rest in oxidative damage to the outer hair cells. However, previous attempts at transtympanic antioxidant delivery have met with varied success. We present an effective method of counteracting cisplatin ototoxicity via the transtympanic application of lactated Ringer's solution or N-acetylcysteine.Baseline distortion product otoacoustic emission measurements were obtained. Intraperitoneal cisplatin was administered to a cumulative dose of 20 mg/kg. The middle ears were either untreated (control) or filled with normal saline (negative control), 2%N-acetylcysteine diluted in normal saline (treatment), or lactated Ringer's solution (treatment) via anterosuperior quadrant myringotomies. Posttreatment distortion product otoacoustic emissions were obtained.Animals in the untreated control group and the negative control normal saline group demonstrated consistent obliteration of distortion product otoacoustic emissions. However, those receiving either lactated Ringer's solution or 2%N-acetylcysteine diluted in normal saline demonstrated significant preservation of distortion product otoacoustic emissions. The treatment regimen was well tolerated, with minimal animal loss.We have demonstrated the efficacy of transtympanic lactated Ringer's solution and N-acetylcysteine in the prevention of cisplatin ototoxicity using a guinea pig model. The possible mechanisms for the high efficacy of lactated Ringer's solution are discussed in detail.

  View details for PubMedID 15547419

• A randomized study of platinum-based chemotherapy with or without amifostine for the treatment of children with hepatoblastoma: A report of the Intergroup Hepatoblastoma Study P9645. Proc Am Soc Clin Oncol Katzenstein HM, Chang K, Krailo M, et al 2004; 22: 8518
• Pediatric residents' clinical diagnostic accuracy of otitis media PEDIATRICS Steinbach, W. J., Sectish, T. C., Benjamin, D. K., Chang, K. W., Messner, A. H. 2002; 109 (6): 993-998

  Abstract

  Pediatric resident physicians' clinical diagnostic accuracy of otitis media is unknown. We attempted to correlate the clinical examination of pediatric house staff with pediatric otolaryngologists and tympanometry.Pediatric residents evaluated patients who were scheduled in the pediatric acute care clinic and completed a provider examination form detailing their otoscopic findings, interpretation, and treatment plan. Patients were then immediately reevaluated by a pediatric otolaryngologist using an identical form. Tympanometry was also performed by a pediatric audiologist. We used kappa statistics to calculate correlation of clinical findings and interpretation.A total of 103 patients consented for the study; 70 patients were examined by 27 different pediatric residents with 43 patients (86 ears) examined by all 3 providers. Correlation of clinical findings between all pediatric residents and the pediatric otolaryngologists was a kappa statistic of 0.30 (fair agreement). The individual diagnostic finding with the greatest correlation was tympanic membrane erythema (kappa statistic: 0.40 [fair agreement]), and the worst correlate was tympanic membrane position (kappa statistic: 0.16 [slight agreement]). Resident interpretation and tympanometry yielded a kappa statistic of 0.20 (slight agreement), and the otolaryngologist interpretation and tympanometry yielded a kappa statistic of 0.32 (fair agreement).Otitis media is the most common disease seen by practicing general pediatricians, but there is a paucity of formalized resident education. We demonstrated only a slight to moderate correlation between the clinical diagnostic examinations of pediatric residents and pediatric otolaryngologists and tympanometry.

  View details for PubMedID 12042534

• Efferently mediated changes in the quadratic distortion product (f2-f1) JOURNAL OF THE ACOUSTICAL SOCIETY OF AMERICA Chang, K. W., Norton, S. J. 1997; 102 (3): 1719-1733

  View details for Web of Science ID A1997XW37200049

• The effects of continuous versus interrupted noise exposures on distortion product otoacoustic emissions in guinea pigs HEARING RESEARCH Chang, K. W., Norton, S. J. 1996; 96 (1-2): 1-12

  Abstract

  Distortion product otoacoustic emissions (DPOAE) were measured serially in guinea pigs before and following 4-h exposures to a half-octave band of noise centered at 6 kHz. Stimulus parameters used to elicit the DPOAE were f2/f1 = 1.26 and L2 = L1-10. The 80 dB SPL exposures resulted in attenuation of emissions, which was maximal at the frequency one-half octave above the exposure when referenced to the f2 stimulus, and which recovered back to baseline after 2 days. The 90 dB SPL exposures resulted in a permanent deficit in emissions elicited by high-frequency stimuli, as measured after 8 days of recovery. A statistically significant difference was also found between animals exposed continuously for 4 h versus animals given two 2-h exposures separated by a 1-h break. Measures of f2-f1 and 3f1-2f2 indicated that they were more sensitive than 2f1-f2 to alterations in cochlear function after noise exposure.

  View details for Web of Science ID A1996UX13500001

  View details for PubMedID 8817301

• Evaluation and management of hoarseness in children. Current Opinion Oto Head Neck Surg Chang, K. W., Inglis, A. F. 1996; 4: 396-400
• IATROGENIC HYPONATREMIA - A CAUSE OF DEATH FOLLOWING PEDIATRIC TONSILLECTOMY INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY MCRAE, R. G., WEISSBURG, A. J., Chang, K. W. 1994; 30 (3): 227-232

  Abstract

  Death following pediatric tonsillectomy is very rare. If deaths occur, they are most commonly due to bleeding or aspiration. In this presentation, we would like to illustrate another potentially lethal complication following the pediatric tonsillectomy, iatrogenic hyponatremia. We have encountered 3 patients who have developed post-operative hyponatremia. This has resulted in 2 deaths. The third patient was successfully treated and developed no permanent sequela. We will discuss the etiology and pathophysiology of post-operative hyponatremia including guidelines for administering fluid and electrolytes intra-operatively and post-operatively. We expect that fatal post-operative hyponatremia can be avoided in the pediatric tonsillectomy patients.

  View details for Web of Science ID A1994PR62600007

  View details for PubMedID 7836036

• EXTERNAL AND MIDDLE-EAR STATUS RELATED TO EVOKED OTOACOUSTIC EMISSION IN NEONATES ARCHIVES OF OTOLARYNGOLOGY-HEAD & NECK SURGERY Chang, K. W., Vohr, B. R., Norton, S. J., LEKAS, M. D. 1993; 119 (3): 276-282

  Abstract

  Screening auditory status prior to neonatal hospital discharge to identify newborns with severe hearing impairment is an important pediatric care priority. Evoked otoacoustic emission (OAE) testing is a quick noninvasive method. The purpose of this study was to determine the relationship between external auditory canal and middle ear status with click-evoked OAE. It was hypothesized that vernix caseosa, debris in the ear canal, and middle ear fluid contribute to the OAE fail rate.All neonates had an initial OAE examination. A second investigator, "blinded" to the results, examined all ears otoscopically, cleaned any obstructing debris, and repeated with a second OAE test.All neonates were tested in a designated nursery at a mean age of 43 +/- 21 hours.Forty-one full-term neonates were prospectively enrolled.The ear canals with debris were cleaned under direct vision with a pediatric swab dampened by an alcohol wipe.The primary outcome measure was the postcleaning OAE pass rate.The preotoscopic examination pass rate of 82 ears was 76%. The OAE pass rate improved to 91% after debris removal.The results indicate that the examination and cleaning of the external ear canal are important components of the neonatal screening process.

  View details for Web of Science ID A1993KQ55900003

  View details for PubMedID 8435165