Publications by authors named "Carlton J Zdanski"

40 Publications

Objective measurements for upper airway obstruction in infants with Robin sequence: what are we measuring? A systematic review.

J Clin Sleep Med 2021 May 7. Epub 2021 May 7.

Department of Plastic and Reconstructive Surgery, Amsterdam University Medical Centre, location AMC & VU, Emma Children's Hospital, Amsterdam, The Netherlands.

Study Objectives: Identifying optimal treatment for infants with Robin sequence (RS) is challenging due to substantial variability in the presentation of upper airway obstruction (UAO) in this population. Objective assessments of UAO and treatments are not standardized. A systematic review of objective measures of UAO was conducted as step towards evidence based clinical decision making for RS.

Methods: A literature search was performed in Pubmed and Embase databases (1990-2020) following PRISMA-guidelines. Articles reporting on RS and UAO-treatment were included if the following objective measures were studied: oximetry, polysomnography and blood gas. Quality was appraised by methodological index for non-randomized studies (MINORS, range:0-24).

Results: A total of 91 articles met inclusion criteria. Mean MINORS-score was 7.1 (range:3-14). Polysomnography was most frequently used (76%) followed by oximetry (20%) and blood gas (11%). Sleep position of the infant was reported in 35% of studies, with supine position most frequently, and monitoring time in 42%, including overnight recordings in more than half. Of 71 studies that evaluated UAO-interventions, the majority used polysomnography (90%), of which 61% did not specify the polysomnography technique. Reported polysomnography metrics included oxygen saturation (61%), apnea-hypopnea index (52%), carbon dioxide levels (31%), obstructive-apnea-hypopnea index (27%), and oxygen-desaturation-index (16%). Only 42 studies reported indications for UAO-intervention, with oximetry and polysomnography thresholds used equally (both 40%). In total, 34 distinct indications for treatment were identified.

Conclusions: This systematic review demonstrates a lack of standardization, interpretation and reporting of assessment and treatment indications for UAO in RS. An international, multidisciplinary consensus protocol is needed to guide clinicians on optimal UAO assessment in RS.
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May 2021

4D Computed Tomography for Dynamic Upper Airway Evaluation in Robin Sequence.

Otolaryngol Head Neck Surg 2021 Mar 9:1945998211002156. Epub 2021 Mar 9.

Division of Pediatric Otolaryngology, Seattle Children's Hospital, Seattle, Washington, USA.

Thorough assessment of dynamic upper airway obstruction (UAO) in Robin sequence (RS) is critical, but traditional evaluation modalities have significant limitations. Four-dimensional computed tomography (4D-CT) is promising in that it enables objective and quantitative evaluation throughout all phases of respiration. However, there exist few protocols or analysis tools to assist in obtaining and interpreting the vast amounts of obtained data. A protocol and set of data analysis tools were developed to enable quantification and visualization of dynamic 4D-CT data. This methodology was applied to a sample case at 2 time points. In the patient with RS, overall increases in normalized airway caliber were observed from 5 weeks to 1 year. There was, however, continued dynamic obstruction at all airway levels, though objective measures of UAO did improve at the nasopharynx and oropharynx. Use of 4D-CT and novel analyses provide additional quantitative information to evaluate UAO in patients with RS.
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March 2021

Systemic Bevacizumab for Treatment of Respiratory Papillomatosis: International Consensus Statement.

Laryngoscope 2021 06 6;131(6):E1941-E1949. Epub 2021 Jan 6.

Department of Otolaryngology-Head and Neck Surgery, University of Texas (UT) Southwestern Medical Center, Dallas, Texas, U.S.A.

Objectives/hypothesis: The purpose of this study is to develop consensus on key points that would support the use of systemic bevacizumab for the treatment of recurrent respiratory papillomatosis (RRP), and to provide preliminary guidance surrounding the use of this treatment modality.

Study Design: Delphi method-based survey series.

Methods: A multidisciplinary, multi-institutional panel of physicians with experience using systemic bevacizumab for the treatment of RRP was established. The Delphi method was used to identify and obtain consensus on characteristics associated with systemic bevacizumab use across five domains: 1) patient characteristics; 2) disease characteristics; 3) treating center characteristics; 4) prior treatment characteristics; and 5) prior work-up.

Results: The international panel was composed of 70 experts from 12 countries, representing pediatric and adult otolaryngology, hematology/oncology, infectious diseases, pediatric surgery, family medicine, and epidemiology. A total of 189 items were identified, of which consensus was achieved on Patient Characteristics (9), Disease Characteristics (10), Treatment Center Characteristics (22), and Prior Workup Characteristics (18).

Conclusion: This consensus statement provides a useful starting point for clinicians and centers hoping to offer systemic bevacizumab for RRP and may serve as a framework to assess the components of practices and centers currently using this therapy. We hope to provide a strategy to offer the treatment and also to provide a springboard for bevacizumab's use in combination with other RRP treatment protocols. Standardized delivery systems may facilitate research efforts and provide dosing regimens to help shape best-practice applications of systemic bevacizumab for patients with early-onset or less-severe disease phenotypes.

Level Of Evidence: 5 Laryngoscope, 131:E1941-E1949, 2021.
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June 2021

Readability analysis of pediatric otolaryngology patient-reported outcome measures.

Int J Pediatr Otorhinolaryngol 2021 Jan 8;140:110550. Epub 2020 Dec 8.

Department of Otolaryngology-Head and Neck Surgery, University of North Carolina, 170 Manning Drive, Campus Box #7070, Chapel Hill, NC, 27599, USA.

Objective: (s): Patient-reported outcome measures (PROMs) are tools that allow patients to directly share information about their health with their healthcare provider. Health literacy experts recommend that health information, such as PROMs, be written at a 6th grade level to ensure patients can read and comprehend it. As the readability of PROMs used in pediatric otolaryngology has yet to be studied, our goal was to analyze the readability of these PROMs and assess their compliance with readability recommendations.

Methods: The Gunning Fog, the Simple Measure of Gobbledygook (SMOG), and the FORCAST readability formulas were used to determine the readability of disease-specific PROMs for pediatric otolaryngology.

Results: Fourteen PROMs were reviewed in the study. Most were intended for caregiver completion (n = 13, 92.9%). Ten PROMs when measured by Gunning Fog (71.4%), 2 PROMs when measured by SMOG (14.3%), and 0 PROMs when measured by FORCAST (0.0%) were at or below the 6th grade reading level.

Conclusion: Most PROMs available for use in pediatric otolaryngology are above the recommended 6th grade reading level when measured by FORCAST, the most appropriate metric for assessing questionnaires. The high reading grade level needed to complete these PROMs can contribute to health disparities among underserved and vulnerable populations, such as children. Pediatric otolaryngology PROMs developed in the future should take readability into account in order to ensure equity in the delivery of care.
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January 2021

IVORY Guidelines (Instructional Videos in Otorhinolaryngology by YO-IFOS): A Consensus on Surgical Videos in Ear, Nose, and Throat.

Laryngoscope 2021 03 17;131(3):E732-E737. Epub 2020 Aug 17.

Department of Oto-Rhino-Laryngology-Head and Neck Surgery, Aix Marseille University, APHM, Language and Speech Laboratory, La Conception University Hospital, Marseille, France.

Objectives/hypothesis: Otolaryngology instructional videos available online are often of poor quality. The objective of this article was to establish international consensus recommendations for the production of educational surgical videos in otolaryngology.

Study Design: DELPHI survey.

Methods: Twenty-seven international respondents participated in this study from 12 countries. Consensus was reached after three rounds of questionnaires following the Delphi methodology. The proposals having reached the 80% agreement threshold in the third round were retained.

Results: The main recommendations are as follows: 1) Ethics: patients must be anonymized and unrecognizable (apart from plastic surgery if necessary). A signed authorization must be obtained if the person is recognizable. 2) Technical aspects: videos should be edited and in high-definition (HD) quality if possible. Narration or subtitles and didactic illustrations are recommended. 3) Case presentation: name of pathology and procedure must be specified; the case should be presented with relevant workup. 4) Surgery: surgical procedures should be divided into several distinct stages and include tips and pitfalls. Pathology should be shown if relevant. Key points should be detailed at the end of the procedure. 5) Organ-specific: type of approach and bilateral audiometry should be specified in otology. Coronal plane computed tomography scans should be shown in endonasal surgery. It is recommended to show pre- and postoperative videos in voice surgery and preoperative drawings and photos of scars in plastic surgery, as well as the ventilation method in airway surgery.

Conclusions: International recommendations have been determined to assist in the creation and standardization of educational surgical videos in otolaryngology and head and neck surgery.

Level Of Evidence: 5 Laryngoscope, 131:E732-E737, 2021.
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March 2021

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Management of suprastomal collapse in the pediatric population.

Int J Pediatr Otorhinolaryngol 2020 Dec 13;139:110427. Epub 2020 Oct 13.

Section of Pediatric ORL, Department of ORL, University Hospital Center S. Joao, University of Porto Faculty of Medicine and CINTESIS, Porto, Portugal.

Introduction: Suprastomal Collapse (SuStCo) is a common complication of prolonged tracheostomy in children. There is a paucity of literature on this subject, especially regarding how to manage significant suprastomal collapse that prevents safe decannulation.

Objective: Provide a definition, classification system, and recommend management options for significant suprastomal collapse in children with tracheostomy.

Methods: Members of the International Pediatric Otolaryngology Group (IPOG) who are experts in pediatric airway conditions were surveyed and results were refined using a modified Delphi method.

Results: Consensus was defined as > 70% agreement on a subject. The experts achieved consensus: CONCLUSION: This consensus statement provides recommendations for medical specialists who manage infants and children with tracheostomies with significant Suprastomal Collapse. It provides a classification system to facilitate diagnosis and treatment options for this condition.
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December 2020

Competency-Based Assessment Tool for Pediatric Esophagoscopy: International Modified Delphi Consensus.

Laryngoscope 2021 05 9;131(5):1168-1174. Epub 2020 Oct 9.

Department of Otolaryngology, Head and Neck Surgery, Stanford University, Lucile Salter Packard Children's Hospital, Palo Alto, California, U.S.A.

Objectives/hypothesis: Create a competency-based assessment tool for pediatric esophagoscopy with foreign body removal.

Study Design: Blinded modified Delphi consensus process.

Setting: Tertiary care center.

Methods: A list of 25 potential items was sent via the Research Electronic Data Capture database to 66 expert surgeons who perform pediatric esophagoscopy. In the first round, items were rated as "keep" or "remove" and comments were incorporated. In the second round, experts rated the importance of each item on a seven-point Likert scale. Consensus was determined with a goal of 7 to 25 final items.

Results: The response rate was 38/64 (59.4%) in the first round and returned questionnaires were 100% complete. Experts wanted to "keep" all items and 172 comments were incorporated. Twenty-four task-specific and 7 previously-validated global rating items were distributed in the second round, and the response rate was 53/64 (82.8%) with questionnaires returned 97.5% complete. Of the task-specific items, 9 reached consensus, 7 were near consensus, and 8 did not achieve consensus. For global rating items that were previously validated, 6 reached consensus and 1 was near consensus.

Conclusions: It is possible to reach consensus about the important steps involved in rigid esophagoscopy with foreign body removal using a modified Delphi consensus technique. These items can now be considered when evaluating trainees during this procedure. This tool may allow trainees to focus on important steps of the procedure and help training programs standardize how trainees are evaluated.

Level Of Evidence: 5. Laryngoscope, 131:1168-1174, 2021.
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May 2021

International Pediatric Otolaryngology Group (IPOG): Consensus recommendations on the prenatal and perinatal management of anticipated airway obstruction.

Int J Pediatr Otorhinolaryngol 2020 Nov 8;138:110281. Epub 2020 Aug 8.

Department of Otolaryngology, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.

Objective: To make recommendations on the identification, routine evaluation, and management of fetuses at risk for airway compromise at delivery.

Methods: Recommendations are based on expert opinion by members of the International Pediatric Otolaryngology Group (IPOG). A two-iterative Delphi method questionnaire was distributed to all members of the IPOG and responses recorded. The respondents were given the opportunity to comment on the content and format of the survey, which was modified for the second round. "Consensus" was defined by >80% respondent affirmative responses, "agreement" by 51-80% affirmative responses, and "no agreement" by 50% or less affirmative responses.

Results: Recommendations are provided regarding etiologies of perinatal airway obstruction, imaging evaluation, adjunct evaluation, multidisciplinary team and decision factors, micrognathia management, congenital high airway obstruction syndrome management, head and neck mass management, attended delivery procedure, and delivery on placental support procedure.

Conclusions: Thorough evaluation and thoughtful decision making are required to optimally balance fetal and maternal risks/benefits.
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November 2020

Competency-Based Assessment Tool for Pediatric Tracheotomy: International Modified Delphi Consensus.

Laryngoscope 2020 11 10;130(11):2700-2707. Epub 2019 Dec 10.

Department of Otolaryngology-Head and Neck Surgery, Oregon Health and Science University, Pediatric Otolaryngology, Doernbecher Children's Hospital, Portland, Oregon, U.S.A.

Objectives/hypothesis: Create a competency-based assessment tool for pediatric tracheotomy.

Study Design: Blinded, modified, Delphi consensus process.

Methods: Using the REDCap database, a list of 31 potential items was circulated to 65 expert surgeons who perform pediatric tracheotomy. In the first round, items were rated as "keep" or "remove," and comments were incorporated. In the second round, experts were asked to rate the importance of each item on a seven-point Likert scale. Consensus criteria were determined a priori with a goal of 7 to 25 final items.

Results: The first round achieved a response rate of 39/65 (60.0%), and returned questionnaires were 99.5% complete. All items were rated as "keep," and 137 comments were incorporated. In the second round, 30 task-specific and seven previously validated global rating items were distributed, and the response rate was 44/65 (67.7%), with returned questionnaires being 99.3% complete. Of the Task-Specific Items, 13 reached consensus, 10 were near consensus, and 7 did not achieve consensus. For the 7 previously validated global rating items, 5 reached consensus and two were near consensus.

Conclusions: It is feasible to reach consensus on the important steps involved in pediatric tracheotomy using a modified Delphi consensus process. These items can now be considered to create a competency-based assessment tool for pediatric tracheotomy. Such a tool will hopefully allow trainees to focus on the important aspects of this procedure and help teaching programs standardize how they evaluate trainees during this procedure.

Level Of Evidence: 5 Laryngoscope, 130:2700-2707, 2020.
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November 2020

Idiopathic, Refractory Sweet's Syndrome Associated with Common Variable Immunodeficiency: a Case Report and Literature Review.

Curr Allergy Asthma Rep 2019 05 14;19(6):32. Epub 2019 May 14.

Division of Allergy, Immunology, and Rheumatology, Department of Pediatrics, University of North Carolina at Chapel Hill, 3300 Thurston Building, CB 7280, Chapel Hill, NC, 27599-7280, USA.

Purpose Of Review: Sweet's syndrome (SS) is classically considered a hypersensitivity reaction often associated with autoimmune disorders and malignancy. SS has also been increasingly reported to occur with immunodeficiencies. We present a case of treatment-refractory, systemic SS as the initial manifestation in a young child with common variable immunodeficiency (CVID). We also review current literature about SS and concurrent immunodeficiencies and autoimmunity in CVID patients.

Recent Findings: Few case reports exist regarding the co-occurrence of Sweet's syndrome and primary immunodeficiencies. SS is characterized by a pro-inflammatory state with a neutrophil predominance resulting in a spectrum of clinical manifestations. CVID is a multifactorial antibody deficiency that can be associated with autoimmunity, which some studies have proposed to be secondary to altered CD21 expression. SS occurring in patients with CVID has been infrequently reported, and one case study demonstrated improvement of Sweet's associated skin lesions with immunoglobulin replacement. In our case, the patient had multi-system SS refractory to multiple immunomodulatory therapies. To our knowledge, this is the first report of the effective and safe use of intravenous tocilizumab and oral lenalidomide to treat SS in a child with CVID. Immunoglobulin replacement reduced the frequency of infections and may have contributed to the opportunity to wean the immunosuppressive therapies for Sweet's syndrome. Sweet's syndrome as an initial manifestation of co-occurring immunodeficiencies is rare, and providers need a high index of suspicion. In addition, treatment of SS associated with an immunodeficiency can be a challenge. Treatment with immunoglobulin replacement reduces the frequency of infections, and in some patients with concurrent SS may improve skin lesions and reduce the need for immunomodulator therapy. Further study is necessary to better understand the pathogenesis of CVID in patients with SS and to identify possible biomarkers that predict who with SS are at risk for developing hypogammaglobulinemia.
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May 2019

Considering a Weight Criterion for Neonatal Tracheostomy: An Analysis of the ACS NSQIP-P.

Laryngoscope 2019 02 8;129(2):500-505. Epub 2018 Sep 8.

Department of Otolaryngology/Head and Neck Surgery, University of North Carolina-Chapel Hill, Chapel Hill, North Carolina, U.S.A.

Objectives: Neonates weighing < 2.5 kg have known higher rates of surgical mortality and morbidity, but this remains unexamined specifically for tracheostomy. We present outcomes of neonates undergoing tracheostomy stratified by weight.

Methods: Retrospective review of the American College of Surgeons National Surgical Quality Improvement Program-Pediatric from 2012 to 2014. Patients undergoing tracheostomy were included. Thirty-day mortality and major/minor complication rates were stratified by weight (group 1: < 2.5 kg; group 2: ≥ 2.5 kg and < 4 kg; group 3: ≥ 4 kg). Patient comorbidities were assessed for independent risk factors of morbidity and mortality.

Results: Of 183,233 patients, 543 underwent tracheostomy. Forty-four patients were group 1 (mean: 2.2 kg ± 0.25); 170 patients were group 2 (mean: 3.31 kg ± 0.42); and 329 patients were group 3 (mean: 6.4 kg ± 2.7). Between groups 1 and 2, there were no significant differences in mortality (P = 0.47), major complication rates (P = 0.99), or minor complication rates (P = 0.64). In comparing all three groups, there were no significant differences in mortality (P = 0.47), major complication rates (P = 0.80), or minor complication rates (P = 0.77). The overall 30-day mortality for all patients was 4.24%. In a multivariate logistical regression model, weight group did not change the odds of all negative outcomes (group 1: odds ratio [OR] of 0.71; 95% confidence interval [CI], 0.33-1.53 and group 2: OR of 0.78; 95% CI, 0.50-1.22). Bronchopulmonary dysplasia was the only independent significant predictor of major complications (OR, 1.69; 95% CI, 1.02-2.79) (P = 0.04).

Conclusion: Our data indicate that 30-day mortality and morbidity outcomes for neonatal tracheostomy are not affected by weight. Overall 30-day mortality should be discussed with caregivers preoperatively.

Level Of Evidence: 4 Laryngoscope, 129:500-505, 2019.
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February 2019

Relationship between degree of obstruction and airflow limitation in subglottic stenosis.

Laryngoscope 2018 07 24;128(7):1551-1557. Epub 2017 Nov 24.

Department of Biomedical Engineering, Marquette University & The Medical College of Wisconsin, Milwaukee, Wisconsin, U.S.A.

Objectives: Subglottic stenosis (SGS) is one of the most common airway disorders in pediatric patients. Currently, treatment decisions rely primarily on the Cotton-Myer scale, which classifies SGS severity based on percentage reduction in airspace cross-sectional area (CSA). However, the precise relationship between upper airway resistance and subglottic CSA is unknown. We hypothesize that airway resistance can be described by the Bernoulli Obstruction Theory, which predicts that airway resistance is inversely proportional to airspace CSA ( R∝A-1) in cases of severe constriction.

Methods: Computed tomography (CT) scans of six healthy subjects and five SGS patients were used to create three-dimensional models of the respiratory tract from nostrils to carina. Cylindrical segments of varying lengths and varying diameters were digitally inserted in the subglottis of the healthy subjects to create simulated SGS models. Computational fluid dynamics simulations were run, and airway resistance was computed in the simulated SGS models and actual SGS models.

Results: Constriction diameter had a greater impact in airway resistance than constriction length. In agreement with the Bernoulli Obstruction Theory, airway resistance in the simulated SGS models was well represented by the power law R=aAb, where a is a constant and the exponent b ranged from -0.85 to -1.07. The percentage reduction in airflow (QOBSTRUCTIONQHEALTHY) at a constant pressure drop was found to be directly proportional to the percentage reduction in CSA (AOBSTRUCTIONAHEALTHY) in the limit of severe constrictions, namely QOBSTRUCTIONQHEALTHY=kAOBSTRUCTIONAHEALTHY, where k=2.25 ± 0.15. Airway resistances in the simulated SGS models were similar to resistances in models based on CT scans of actual SGS patients, suggesting that our simulated SGS models were representative of airway resistance in actual SGS patients.

Conclusion: Our computer simulations suggest that the degree of airflow limitation in SGS patients may be estimated based on anatomic measurements alone. Future studies are recommended to test these predictions in larger cohorts.

Level Of Evidence: 4. Laryngoscope, 128:1551-1557, 2018.
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July 2018

Electrocardiogram Screening in Children with Congenital Sensorineural Hearing Loss: Prevalence and Follow-up of Abnormalities.

Otolaryngol Head Neck Surg 2018 03 21;158(3):553-558. Epub 2017 Nov 21.

1 Department of Otolaryngology-Head and Neck Surgery, University of North Carolina, Chapel Hill, North Carolina, USA.

Objective The purpose is to determine the prevalence of electrocardiogram (ECG) abnormalities, including borderline and prolonged QT, among screened children with sensorineural hearing loss (SNHL) and to analyze their subsequent medical workup. Study Design Institutional Review Board-approved case series with chart review. Setting Tertiary academic center. Subjects and Methods Cases from 1996 to 2014 involving pediatric patients (N = 1994) with SNHL were analyzed. Abnormal ECGs were categorized as borderline/prolonged QT or other. A board-certified pediatric cardiologist retrospectively determined the clinical significance of ECG changes. For follow-up analysis, children with heart disease, known syndromes, or inaccessible records were excluded. Results Among 772 children who had ECGs, 215 (27.8%) had abnormal results: 35 (4.5%) with QT abnormalities and 180 (23.3%) with other abnormalities. For children with QT abnormalities meeting inclusion criteria (n = 30), follow-up measures included cardiology referral (46.6%), repeat ECG by ear, nose, and throat (ENT) specialist (20%), clearance by ENT specialist with clinical correlation and/or comparison with old ECGs (20%), and pediatrician follow-up (6.7%). Documentation of further workup by ENT or referral was absent for 6.7%. For children with other ECG changes meeting inclusion criteria (n = 136), abnormalities were documented for 57 (41.9%); normal QT without other abnormality was documented for 18 (13.2%). The most common follow-up referrals were to pediatricians (16.9%) and cardiologists (10.3%). Among patients with clinically significant non-QT abnormalities mandating further evaluation (n = 122), 38 (31.1%) had documented follow-up in medical records. Conclusion There is a high prevalence of ECG abnormalities among children with congenital SNHL. If findings are confirmed by future studies, screening should be considered for congenital unilateral or bilateral SNHL, regardless of severity. We describe a standardized protocol for ECG screening/follow-up.
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March 2018

Emergent airway management in the labor and delivery suite.

Int J Pediatr Otorhinolaryngol 2016 Aug 24;87:83-6. Epub 2016 May 24.

The North Carolina Children's Airway Center, The University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.

Introduction: Congenital airway obstruction is of varied etiology and uncommonly encountered. Prenatal care and imaging have enhanced detection of these abnormalities and allow for multi-disciplinary care planning for airway management at delivery. Despite the availability and advances in prenatal imaging, unanticipated airway obstruction may not be identified until the time of delivery.

Methods: Case series.

Results: Four airway emergencies were encountered in the labor and delivery suite over an eight-month period. Clinical history is correlated with autopsy findings. Congenital upper airway and laryngotracheal anomalies are reviewed. Recommendations to improve timely and efficient airway management in the labor and delivery suite are discussed and a protocol for a multi-disciplinary neonatal emergency airway response team is offered for consideration.

Conclusions: The development and implementation of a multi-disciplinary emergency newborn airway protocol is both realistic and feasible. While it did not improve survivability in our small group, it did reduce response time. It, or a protocol like it, is recommended for institutions caring for high-risk pregnancies and with Neonatal Intensive Care Units with high acuity patients.
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August 2016

Transoral robotic surgery for upper airway pathology in the pediatric population.

Laryngoscope 2017 01 19;127(1):247-251. Epub 2016 Jun 19.

Department of Otolaryngology/Head and Neck Surgery, University of North Carolina Hospitals, Chapel Hill, North Carolina.

Objectives/hypothesis: The purpose of this study is to present one of the largest case series of pediatric transoral robotic surgery (TORS) in the upper airway demonstrating a wide range of ages and indications.

Study Design: A retrospective case series at an academic tertiary referral center from August 2010 to September 2014.

Methods: The da Vinci surgical robot (Intuitive Surgical, Inc., Sunnyvale, CA) was used on 16 pediatric patients for 18 procedures. A variety of upper airway pathologies and reconstructions in children with a wide range of ages and weights were treated. No lingual tonsillectomies or base-of-tongue reductions were included.

Results: Sixteen children (6 males) underwent 18 TORS procedures, including resection of hamartoma (n = 1), repair of laryngeal cleft (n = 7), removal of saccular cyst (n = 2), release of pharyngeal or esophageal strictures (n = 2), and excision of lymphatic malformations (n = 4). Patient ages ranged from 14 days to 15 years. There were no intraoperative complications. All patients had successful robotic access, and no patients had conversions to open or traditional endoscopic surgery. Hospital courses varied with duration ranging from 1 to 20 days. The median follow up was 22 months.

Conclusion: Applying TORS to the pediatric population can be feasible and safe for appropriate airway pathologies. Because many patients are small in size, there is inherent risk in using robotic instruments and scopes transorally. Pearls in this series include a standardized two-robot experienced attending team and longitudinal airway follow-up.

Level Of Evidence: 4 Laryngoscope, 127:247-251, 2017.
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January 2017

The effect of interdevice interval on speech perception performance among bilateral, pediatric cochlear implant recipients.

Laryngoscope 2016 10 21;126(10):2389-94. Epub 2016 Apr 21.

Department of Otolaryngology, Washington University in St. Louis, St Louis, Missouri, U.S.A.

Objectives/hypothesis: To determine if prolongation of the interdevice interval in children receiving bilateral cochlear implants adversely affects speech perception outcomes.

Study Design: Retrospective chart review.

Methods: Retrospective review of our pediatric cochlear implant database was performed. Children who had undergone revision surgery or had less than 12 months listening experience with either the first or second implant were excluded. The interdevice interval, best Phonetically Balanced Kindergarten word lists (PBK) score from each ear, and demographic data about each patient were collected. A ratio of PBK was generated (PBK second side/PBK first side) to minimize potential confounding from other individual patient factors that affect speech outcomes.

Results: Two hundred forty children met the study criteria. Mean age at first cochlear implantation (CI) was 3.2 years (0.6-17.9), and the second was 6.6 years (0.8-22.4). Mean best PBK score from the first CI side was 83.8% (0-100), and the second was 67.5% (0-100) (P < .001). When the PBK ratio was plotted against interdevice interval, R(2) was 0.47 (P < .001). When analyzed for hearing stability, those with a progressive loss history demonstrated less influence of prolonged interdevice interval on performance. Multivariate analysis did not identify other factors influencing the ratio. A line of best fit for those with stable hearing loss suggested best outcomes were with an interdevice interval less than 3 to 4 years. Beyond 7 to 8 years, very few achieved useful speech recognition from the second CI.

Conclusions: Where possible, the second implant should be received within 3 to 4 years of the first to maximize outcome in those with stable, severe to profound sensorineural hearing loss.

Level Of Evidence: 4. Laryngoscope, 126:2389-2394, 2016.
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October 2016

A Multidisciplinary Children's Airway Center: Impact on the Care of Patients With Tracheostomy.

Pediatrics 2016 Feb 11;137(2):e20150455. Epub 2016 Jan 11.

Division of Pulmonology, Department of Pediatrics, and.

Background: Children with complex airway problems see multiple specialists. To improve outcomes and coordinate care, we developed a multidisciplinary Children's Airway Center. For children with tracheostomies, aspects of care targeted for improvement included optimizing initial hospital discharge, promoting effective communication between providers and caregivers, and avoiding tracheostomy complications.

Methods: The population includes children up to 21 years old with tracheostomies. The airway center team includes providers from pediatric pulmonology, pediatric otolaryngology/head and neck surgery, and pediatric gastroenterology. Improvement initiatives included enhanced educational strategies, weekly care conferences, institutional consensus guidelines and care plans, personalized clinic schedules, and standardized intervals between airway examinations. A patient database allowed for tracking outcomes over time.

Results: We initially identified 173 airway center patients including 123 with tracheostomies. The median number of new patients evaluated by the center team each year was 172. Median hospitalization after tracheostomy decreased from 37 days to 26 days for new tracheostomy patients <1 year old discharged from the hospital. A median of 24 care plans was evaluated at weekly conferences. Consensus protocol adherence increased likelihood of successful decannulation from 68% to 86% of attempts. The median interval of 8 months between airway examinations aligned with published recommendations.

Conclusions: For children with tracheostomies, our Children's Airway Center met and sustained goals of optimizing hospitalization, promoting communication, and avoiding tracheostomy complications by initiating targeted improvements in a multidisciplinary team setting. A multidisciplinary approach to management of these patients can yield measurable improvements in important outcomes.
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February 2016

Volumetric nasal cavity analysis in children with unilateral and bilateral cleft lip and palate.

Laryngoscope 2016 06 12;126(6):1475-80. Epub 2015 Aug 12.

Department of Otolaryngology-Head and Neck Surgery, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, U.S.A.

Objectives/hypothesis: Children with cleft lip and palate (CLP) often suffer from nasal obstruction that may be related to effects on nasal volume. The objective of this study was to compare side:side volume ratios and nasal volume in patients with unilateral (UCLP) and bilateral (BCLP) clefts with age-matched controls.

Study Design: Retrospective case-control study using three-dimensional (3D) nasal airway reconstructions.

Methods: We analyzed 20 subjects (age range = 7-12 years) with UCLP and BCLP from a regional craniofacial center who underwent cone beam computed tomography (CT) prior to alveolar grafting. Ten multislice CT images from age-matched controls were also analyzed. Mimics software (Materialise, Plymouth, MI) was used to create 3D reconstructions of the main nasal cavity and compute total and side-specific nasal volumes. Subjects imaged during active nasal cycling phases were excluded.

Results: There was no statistically significant difference in affected:unaffected side volume ratios in UCLP (P = .48) or left:right ratios in BCLP (P = .25) when compared to left:right ratios in controls. Mean overall nasal volumes were 9,932 ± 1,807, 7,097 ± 2,596, and 6,715 ± 2,115 mm(3) for control, UCLP, and BCLP patients, respectively, with statistically significant volume decreases for both UCLP and BCLP subjects from controls (P < .05).

Conclusions: This is the first study to analyze total nasal volumes in BCLP patients. Overall nasal volume is compromised in UCLP and BCLP by approximately 30%. Additionally, our finding of no major difference in side:side ratios in UCLP and BCLP compared to controls conflicts with pre-existing literature, likely due to exclusion of actively cycling scans and our measurement of the functional nasal cavity.

Level Of Evidence: 3b. Laryngoscope, 126:1475-1480, 2016.
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June 2016

Connexin-related (DFNB1) hearing loss: is routine computed tomography imaging necessary?

Otolaryngol Head Neck Surg 2015 May 12;152(5):889-96. Epub 2015 Jan 12.

Department of Otolaryngology/Head and Neck Surgery, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA

Objectives: Determine if routine computed tomography (CT) imaging is necessary in the workup for children with connexin-related (DFNB1) sensorineural hearing loss (SNHL).

Study Design: Case-control retrospective chart and imaging review.

Setting: Tertiary care otolaryngology practice.

Subjects And Methods: High-resolution temporal bone CT scans of children (n = 21) with DFNB1 SNHL were compared to age-matched controls with either conductive hearing loss (CHL, n = 33) or a nonsyndromic, non-DFNB1 SNHL (n = 33). Sixteen measurements of cochleo-vestibular structures were recorded. Statistical analysis was performed using a repeated analysis of variance model that controlled for both age and gender. Area under the curve (AUC) and multidimensional AUC (MAUC) analyses were also performed.

Results: Overall, no statistically significant differences were found between the 3 experimental groups. In addition, comparisons between the DFNB1 and CHL groups, DFNB1 and non-DFNB1 SNHL groups, and CHL and non-DFNB1 SNHL groups failed to demonstrate any statistically significant differences. AUC and MAUC analyses also failed to detect any significant differences between the 3 groups.

Conclusions: Patients with DFNB1 SNHL do not have significant anatomic differences on temporal bone CT scans when compared to non-DFNB1 SNHL and CHL control groups. Based on the above analysis, it is reasonable to avoid routine CT imaging of the temporal bones in children with known DFNB1 SNHL unless otherwise clinically indicated.
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May 2015

Comprehensive quality of life outcomes for pediatric patients undergoing endoscopic sinus surgery.

Rhinology 2014 Dec;52(4):327-33

Background: Limited quality of life data exist for pediatric chronic rhinosinusitis (CRS) patients undergoing endoscopic sinus surgery (ESS). Further exploration of the following areas will enhance understanding and support clinical decision-making: baseline and post-ESS general and disease-specific quality of life, parent vs. child report, and correlation of nasal endoscopy to sinus CT scores.

Methodology: A prospective cohort study evaluated CRS patients age 5-18 undergoing ESS. Surveys were completed at two timepoints: (1) pre-ESS and (2) 30-90 days post-ESS, with parents completing general (PedsQLTM) and CRS-specific (SNOT-16 and SN-5) quality of life surveys and children completing PedsQLTM and SNOT-16 surveys. Preoperative Lund-Kennedy nasal endoscopy and Lund-Mackay sinus CT scores were calculated. Where appropriate, outcomes were stratified by cystic fibrosis status.

Results: Impaired preoperative general quality of life was evidenced by parent proxy-report of PedsQLTM scores in 10 cystic fibrosis and 11 non-CF patients. ESS was associated with decreased sinus symptoms at 1-3 months postoperatively with SN-5 change scores of -1.85 and -2.2, in CF and non-CF patients, respectively. Parents reported worse CRS symptoms via higher preoperative SNOT-16 scores than their children did. Nasal endoscopy and sinus CT scores correlated with a Spearman correlation coefficient of 0.51. Scores not reaching statistical significance included CF-related CRS SNOT-16 change scores and PedsQLTM general quality of life change scores.

Conclusion: In pediatric patients with CRS electing ESS, general quality of life is impaired preoperatively and sinus symptoms improve significantly 1-3 months after sinus surgery. Parents report statistically worse CRS symptom scores than their children do. Nasal endoscopy scores in this cohort correlated with sinus CT scores.
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December 2014

Statistical atlas construction via weighted functional boxplots.

Med Image Anal 2014 May 29;18(4):684-98. Epub 2014 Mar 29.

University of North Carolina (UNC) at Chapel Hill, NC, USA; Biomedical Research Imaging Center, UNC-Chapel Hill, NC, USA.

Atlas-building from population data is widely used in medical imaging. However, the emphasis of atlas-building approaches is typically to estimate a spatial alignment to compute a mean/median shape or image based on population data. In this work, we focus on the statistical characterization of the population data, once spatial alignment has been achieved. We introduce and propose the use of the weighted functional boxplot. This allows the generalization of concepts such as the median, percentiles, or outliers to spaces where the data objects are functions, shapes, or images, and allows spatio-temporal atlas-building based on kernel regression. In our experiments, we demonstrate the utility of the approach to construct statistical atlases for pediatric upper airways and corpora callosa revealing their growth patterns. We also define a score system based on the pediatric airway atlas to quantitatively measure the severity of subglottic stenosis (SGS) in the airway. This scoring allows the classification of pre- and post-surgery SGS subjects and radiographically normal controls. Experimental results show the utility of atlas information to assess the effect of airway surgery in children.
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May 2014

Comparison of endoscopic versus 3D CT derived airway measurements.

Laryngoscope 2013 Sep;123(9):2136-41

Objectives/hypothesis: To understand: 1) how endoscopic airway measurements compare to three-dimensional (3D) CT derived measurements; 2) where each technique is potentially useful; and 3) where each has limitations.

Study Design: Compare airway diameters and cross-sectional areas from endoscopic images and CT derived 3D reconstructions.

Methods: Videobronchoscopy was performed and recorded on an adult-sized commercially available airway mannequin. At various levels, cross-sectional areas were measured from still video frames using a referent placed via the biopsy port. A 3D reconstruction was generated from a high resolution CT of the mannequin; planar sections were cut at similar cross-sectional levels; and cross-sectional areas were obtained.

Results: At three levels of mechanically generated tracheal stricture, the differences between the endoscopic measurement and CT-derived cross-sectional area were 1%, 0%, and 7% (1.8, 0.8, and 14 mm²). At the vocal folds, the difference was 9% (7.8 mm²). The tip of the epiglottis and width of the epiglottis differed by 27% and 10% (18.73 mm², 0.40 mm). The airway measurements at the base of tongue, minimal cross-sectional area of the pharynx, and choana differed by 26%, 36%, and 30% (101.40 mm², 36.67 mm², 122.71 mm²).

Conclusions: Endoscopy is an effective tool for obtaining airway measurements compared with 3D reconstructions derived from CT. Concordance is best in geometrically simple areas where the entire cross-section measured is visible within one field of view (trachea, round; vocal folds, triangular) versus geometrically complex areas that encompass more than one field of view (i.e. pharynx, choana).
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September 2013


Proc IEEE Int Symp Biomed Imaging 2013 Apr;2013:1206-1209

Kitware, Inc., Carrboro, NC, US.

Young children with upper airway problems are at risk for hypoxia, respiratory insufficiency and long term morbidity. Computational models and quantitative analysis would reveal airway growth patterns and benefit clinical care. To capture expected growth patterns we propose a method to build a pediatric airway atlas as a function of age. The atlas is based on a simplified airway model in combination with kernel regression. We show experimental results on children with subglottic stenosis to demonstrate that our method is able to track and measure the stenosis in pediatric airways.
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April 2013

Cochlear implantation in unique pediatric populations.

Curr Opin Otolaryngol Head Neck Surg 2012 Dec;20(6):507-17

Department of Otolaryngology/Head and Neck Surgery, University of North Carolina Hospitals, Chapel Hill, North Carolina 27599-7070, USA.

Purpose Of Review: Over the last decade, the selection criteria for cochlear implantation have expanded to include children with special auditory, otologic, and medical problems. Included within this expanded group of candidates are those children with auditory neuropathy spectrum disorder, cochleovestibular malformations, cochlear nerve deficiency, associated syndromes, as well as multiple medical and developmental disorders. Definitive indications for cochlear implantation in these unique pediatric populations are in evolution. This review will provide an overview of managing and habilitating hearing loss within these populations with specific focus on cochlear implantation as a treatment option.

Recent Findings: Cochlear implants have been successfully implanted in children within unique populations with variable results. Evaluation for cochlear implant candidacy includes the core components of a full medical, audiologic, and speech and language evaluations. When considering candidacy in these children, additional aspects to consider include disorder-specific surgical considerations and child/caregiver counseling regarding reasonable postimplantation outcome expectations.

Summary: Cochlear implants are accepted as the standard of care for improving hearing and speech development in children with severe-to-profound hearing loss. However, children with sensorineural hearing loss who meet established audiologic criteria for cochlear implantation may have unique audiologic, medical, and anatomic characteristics that necessitate special consideration regarding cochlear implantation candidacy and outcome. Individualized preoperative candidacy and counseling, surgical evaluation, and reasonable postoperative outcome expectations should be taken into account in the management of these children.
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December 2012

Influence of an intraoperative perilymph gusher on cochlear implant performance in children with labyrinthine malformations.

Otol Neurotol 2012 Dec;33(9):1489-96

Department of Otolaryngology/Head and Neck Surgery, University of North Carolina at Chapel Hill, North Carolina 27599, USA.

Objectives: To assess the effect of an intraoperative perilymph fluid gusher during cochlear implantation on speech perception abilities in pediatric patients with labyrinthine anomalies.

Setting: Tertiary care academic referral center.

Methods: Seventy subjects with labyrinthine malformations who received a cochlear implant were identified in our pediatric cochlear implant database. In 30 cases, an intraoperative perilymph fluid gusher was encountered during surgery. Fifteen children with GJB2 positive hereditary hearing loss served as controls. Multiple speech perception measures were obtained with the cochlear implant. The best score for each subject over time was determined as a speech reception index in quiet. This index was compared among groups and malformation types.

Results: The speech reception index in quiet demonstrated overall good performance scores of cochlear implantation in children with incomplete partitioning/enlarged vestibular aqueduct type malformations. Children with hypoplastic malformations, on the other hand, showed variable outcomes with many children demonstrating only limited long-term speech discrimination abilities. The presence or absence of a perilymph gusher did not significantly influence results after cochlear implantation.

Conclusion: This report documents the variable outcomes of pediatric cochlear implantation in children with inner ear malformations. More importantly, anatomic parameters, such as the classification of the anomaly and the presence of a viable cochlear nerve, seem to influence performance measures substantially. The presence of a perilymph gusher did not influence outcomes in both hypoplastic and incomplete partitioning/enlarged vestibular aqueduct type malformations.

Level Of Evidence: 2b Individual retrospective cohort study.
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December 2012

Pediatric airway abnormalities: evaluation and management.

Oral Maxillofac Surg Clin North Am 2012 Aug 1;24(3):325-36. Epub 2012 Jun 1.

Department of Otolaryngology/Head and Neck Surgery, University of North Carolina, 170 Manning Drive, Chapel Hill, NC 27599, USA.

Sleep disordered breathing syndromes in pediatric patients can lead to adverse effects in the cardiovascular system, neurocognitive function, growth, and behavior. These syndromes occur more frequently in patients with craniofacial disorders. A high index of suspicion as well as early recognition, detection, and treatment of these syndromes are considered integral to care of children with craniofacial disorders.
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August 2012

Esophageal foreign body mimicking a mediastinal mass.

Otolaryngol Head Neck Surg 2011 May;144(5):823-4

Department of Otolaryngology and Head and Neck Surgery, University of North Carolina at Chapel Hill, Chapel Hill, NC 27514, USA.

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May 2011

Transpalatal greater palatine canal injection: Radioanatomic analysis of where to bend the needle for pediatric sinus surgery.

Am J Rhinol Allergy 2010 Sep-Oct;24(5):385-8

Department of Otolaryngology–Head and Neck Surgery, University of North Carolina at Chapel Hill, USA.

Background: The greater palatine canal (GPC) local injection is used to limit posterior bleeding during sinus surgery in adults. Given the potential for causing iatrogenic damage to the intraorbital contents, this procedure is not commonly used in the pediatric population. No studies have described the anatomic development of the GPC during facial growth. By using age-stratified radioanatomic analysis, the dimensions of the GPC and the clinical implications are described for pediatric patients. An age-stratified radioanatomic study was performed.

Methods: High-resolution computed tomography measurements included the thickness of the mucosal plane overlying the GPC, the length of the GPC, and the distance between the base of the pterygopalatine fossa (PPF) and the orbital floor. Mean distance and standard deviation were calculated for each age cohort and compared using the one-way ANOVA test.

Results: The GPC length correlated directly with patient age. It varied from 9.14 ± 0.11 mm in the youngest age group (<2 years) to 19.36 ± 2.76 mm in adults (18-64 years). The height of the orbit relative to the hard palate approximated the adult dimensions described in the literature by 12-13 years (49.58 ± 1.72 mm).

Conclusion: These radioanatomic results suggest that the GPC injection described for adult patients may be safely administered to selected pediatric patients. For patients >12 years old, we recommend bending the needle 45° and inserting it 25 mm. For patients 6-12 years old, the needle should be inserted 20 mm to enter into the PPF. In patients <6 years old, the needle may safely be placed 12 mm into the GPC. Each of these descriptions is based on the minimal distance required to effectively access the PPF but with maximal safety in regard to the orbit. Further clinical correlation of these findings is necessary through future investigation.
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April 2011

Magnetic resonance imaging of guinea pig cochlea after vasopressin-induced or surgically induced endolymphatic hydrops.

Otolaryngol Head Neck Surg 2010 Feb;142(2):260-5

Department of Otolaryngology-Head and Neck Surgery, University of North Carolina School of Medicine, Chapel Hill, NC 27599-7070, USA.

Objective: To investigate the ability to detect the in vivo cochlear changes associated with vasopressin-induced and surgically induced endolymphatic hydrops using MRI at 3 tesla (T).

Study Design: Prospective, animal model.

Setting: Animal laboratory.

Subjects And Methods: In group 1, five guinea pigs underwent post-gadolinium temporal bone MRI before and after seven and 14 days of chronic systemic administration of vasopressin by osmotic pump. In group 2, five guinea pigs underwent temporal bone MRI eight weeks after unilateral surgical ablation of the endolymphatic sac. Three-tesla high-resolution T1-weighted sequences were acquired pre- and postcontrast administration. Region of interest signal intensities of the perilymph and endolymph were analyzed manually. Quantitative evaluation of hydrops was measured histologically.

Results: Gadolinium preferentially concentrated in the perilymph, allowing for distinction of cochlear compartments on 3.0-T MRI. The T1-weighted contrast MRI of vasopressin-induced hydropic cochlea showed significant increases in signal intensity of the endolymph and perilymph. Surgically induced unilateral hydropic cochlea also showed increased signal intensity, compared with the control cochlea of the same animal, but less of an increase than the vasopressin group. The histological degree of hydrops induced in the vasopressin group was comparable to previous studies.

Conclusions: In vivo postcontrast MRI of the inner ear demonstrated cochlear changes associated with chronic systemic administration of vasopressin and surgical ablation of the endolymphatic sac. Understanding the MRI appearance of endolymphatic hydrops induced by various methods contributes to the future use of MRI as a possible tool in the diagnosis and treatment of Ménière's disease.
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February 2010

Cochlear implantation in children with auditory neuropathy spectrum disorder.

Ear Hear 2010 Jun;31(3):325-35

W. Paul Biggers Carolina Children's Communicative Disorders Program, Department of Otolaryngology-Head and Neck Surgery and the Division of Audiology, University of North Carolina Hospitals, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599-7600, USA.

Objective: To report the patient's characteristics, preoperative audiological profiles, surgical outcomes, and postoperative performance for children with auditory neuropathy spectrum disorder (ANSD) who ultimately received cochlear implants (CIs).

Design: Prospective, longitudinal study of children with ANSD who received CIs after a stepwise management protocol that included electrophysiologic and medical assessment, documentation of behavioral audiometric thresholds and subsequent fitting of amplification according to Desired Sensation Level targets, auditory-based intervention with careful monitoring of skills development and communication milestones, and finally implantation when progress with the use of acoustic amplification was insufficient.

Results: Of 140 children with ANSD, 52 (37%) received CIs in their affected ears (mean duration of use of 41 mos). Many of these children were born prematurely (42%) and impacted by a variety of medical comorbidities. More than one third (38%) had abnormal findings on preoperative magnetic resonance imaging of the brain and inner ear, and 81% had a greater than severe (>70 dB HL) degree of hearing loss before implantation. Although 50% of the implanted children with ANSD demonstrated open-set speech perception abilities after implantation, nearly 30% of them with >6 months of implant experience were unable to participate in this type of testing because of their young age or developmental delays. No child with cochlear nerve deficiency (CND) in their implanted ear achieved open-set speech perception abilities. In a subgroup of children, good open-set speech perception skills were associated with robust responses elicited on electrical-evoked intracochlear compound action potential testing when this assessment was possible.

Conclusions: This report shows that children with ANSD who receive CIs are a heterogeneous group with a wide variety of impairments. Although many of these children may ultimately benefit from implantation, some will not, presumably because of a lack of electrical-induced neural synchronization, the detrimental effects of their other associated conditions, or a combination of factors. When preoperative magnetic resonance imaging reveals central nervous system pathology, this portends a poor prognosis for the development of open-set speech perception, particularly when CND is evident. These results also show that electrical-evoked intracochlear compound action potential testing may help identify those children who will develop good open-set speech perception. Instead of recommending CI for all children with electrophysiologic evidence of ANSD, the stepwise management procedure described herein allows for the identification of children who may benefit from amplification, those who are appropriate candidates for cochlear implantation, and those who, because of bilateral CND, may not be appropriate candidates for either intervention.
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June 2010