Cochlear Implantation Can Improve Auditory Skills, Language and Social Engagement of Children With Autism Spectrum Disorder.

OBJECTIVE: To review outcomes of cochlear implantation (CI) in children diagnosed with autism spectrum disorder (ASD). STUDY DESIGN: Retrospective case review and parent survey. SETTING: Tertiary care children's hospital. PATIENTS: Thirty children with ASD who underwent CI between 1991 and 2018. Mean age at CI = 3.5 years (0.8-11.8), mean age at diagnosis of ASD = 5.1 years (2.0-15.0) (22/30 diagnosed after CI), mean follow-up = 10.5 years (1.4-21.6). Parents of 7 children returned a survey. INTERVENTION: Unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURES: Speech perception; expressive communication mode; educational placement; social engagement; consistency of CI use; parent survey of child behavior change. RESULTS: Thirty-three percent of all and 45% of the 22 consistent device users developed measurable open-set speech perception by an average of 4.5 years of device use. Educational placement at last follow-up included 13% mainstreamed without interpreter, 50% Special Education programs, 10% therapeutic residential or day programs, 23% total communication programs, and one home schooled. Spoken language alone was used by 31% and spoken plus sign by 14%, with the remainder using sign alone, augmentative communication devices or no mode of communication. By parent report, 86% showed improvement in social engagement compared to pre-CI. Survey results showed the behaviors most frequently ranked as most affected by CI were communication and attention, while awareness of environment had the lowest (most affected) mean ranking. CONCLUSIONS: Findings support a growing body of literature that cochlear implantation has the potential to improve auditory skills, language, and enhance social engagement in some deaf children with autism spectrum disorder.

Pediatric sleep outcomes after endoscopy-directed simultaneous lingual tonsillectomy and epiglottopexy.

BACKGROUND: The purpose of this study was to evaluate the efficacy of sleep endoscopy-directed simultaneous lingual tonsillectomy and epiglottopexy in patients with sleep disordered breathing (SDB), including polysomnography (PSG) and swallowing outcomes. METHODS: A retrospective review was performed of all patients undergoing simultaneous lingual tonsillectomy and epiglottopexy over the study period. PSG objective measures were recorded pre- and postoperatively, along with demographic data, comorbidities, and descriptive data of swallowing dysfunction in the postoperative setting. RESULTS: A total of 24 patients met inclusion criteria for consideration, with 13 having valid pre- and postoperative PSG data. Successful surgery was achieved in 84.6% of patients, with no difference based on presence of medical comorbidities including Trisomy 21. Median reduction in obstructive apnea-hypopnea index (oAHI) with the procedure was 69.9%. Four patients (16.7%) had postoperative concern for dysphagia, but all objective swallowing evaluations were normal and no dietary modifications were necessary. CONCLUSION: Combination lingual tonsillectomy and epiglottopexy in indicated patients has a high rate of success in this single-institutional study without new dysphagia in this population. These procedures are amenable to a combination surgery in appropriately selected patients determined by sleep state endoscopy in the setting of SDB evaluated with drug-induced sleep endoscopy.

Transcanal Endoscopic Ear Surgery for Congenital Cholesteatoma: A Multi-institutional Series.

OBJECTIVE: To assess outcomes of transcanal endoscopic ear surgery (TEES) for congenital cholesteatoma. STUDY DESIGN: Case series with chart review of children who underwent TEES for congenital cholesteatoma over a 10-year period. SETTING: Three tertiary referral centers. METHODS: Cholesteatoma extent was classified according to Potsic stage; cases with mastoid extension (Potsic IV) were excluded. Disease characteristics, surgical approach, and outcomes were compared among stages. Outcomes measures included residual or recurrent cholesteatoma and audiometric data. RESULTS: Sixty-five cases of congenital cholesteatoma were included. The mean age was 6.5 years (range, 1.2-16), and the mean follow-up was 3.9 years (range, 0.75-9.1). There were 19 cases (29%) of Potsic stage I disease, 10 (15%) stage II, and 36 (55%) stage III. Overall, 24 (37%) patients underwent a second-stage procedure, including 1 with Potsic stage II disease (10%) and 21 (58%) with Potsic stage III disease. Eight cases (12%) of residual cholesteatoma occurred. One patient (2%) developed retraction-type ("recurrent") cholesteatoma. Recidivism occurred only among Potsic stage III cases. Postoperative air conduction hearing thresholds were normal (<25 dB HL) in 93% of Potsic stage I, 88% of stage II, and 36% of stage III cases. CONCLUSION: TEES is feasible and effective for removal of congenital cholesteatoma not extending into the mastoid. Recidivism rates were lower with the TEES approach in this large series than in previously reported studies. Advanced-stage disease was the primary risk factor for recidivism and worse hearing result. As minimally invasive TEES is possible in the youngest cases, children benefit from early identification and intervention.

Impact of Cochlear Implant With Diametric Magnet on Imaging Access, Safety, and Clinical Care.

OBJECTIVES/HYPOTHESIS: Review safety and effectiveness of magnetic resonance imaging (MRI) of patients implanted with a cochlear implant (CI) containing a diametric magnet housed within the undersurface of the device. STUDY DESIGN: Retrospective chart review with additional review of MRI at a tertiary-care children's hospital. METHODS: Seven patients with mean age of 8.4 years (range = 1.3-19 years) with a diametric magnet in situ during MRI. The intervention comprised one or more sessions of 1.5 T or 3.0 T MRI without a head wrap. The main outcome measures were the occurrence of magnet-related complications including discomfort and magnet displacement, use of anesthesia or sedation, and clinical usefulness of MRI studies. RESULTS: Seven CI recipients underwent 17 episodes of 1.5 or 3.0 T MRI with an in situ diametric magnet. Thirteen of 17 (76%) MRI sessions were completed in awake patients. No patients had device-related discomfort. No magnet-related complications occurred. Thirteen of 14 (93%) brain studies were clinically useful despite artifacts. CONCLUSIONS: The diametric magnet enabled MRI with magnet in situ without the discomfort or magnet displacement associated with removable axial magnets. The reduction in MRI magnet-related complications occurred because torque is not directed perpendicular and outward from the plane of the magnet, and the magnet is securely contained within its housing. The design of this device increased access and reduced the need for sedation or anesthesia. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E952-E956, 2021.

Early Identification and Management of Congenital Cytomegalovirus.

Congenital cytomegalovirus (cCMV) is the most common nongenetic cause of sensorineural hearing loss. Despite its prevalence, universal screening for cCMV is not currently performed. Hearing loss caused by cCMV is most often severe to profound, often bilateral, and may be fluctuating or progressive. Infants with hearing loss at birth and confirmed cCMV might benefit from antiviral therapy. Roughly half of hearing loss cases owing to cCMV are delayed in onset, and consequently, these children pass newborn hearing screening. Children with cCMV require close audiologic monitoring, require appropriate management with hearing aids, and should be monitored for cochlear implant candidacy.

Congenital Cytomegalovirus Infection: Epidemiology, Timely Diagnosis, and Management.

Congenital cytomegalovirus (cCMV) infection is common because of the ubiquitous nature of the virus and the lack of an effective prevention strategy during pregnancy. Most infants with cCMV are asymptomatic, although a notable subset can have sequelae including, most commonly, sensorineural hearing loss and neurodevelopmental disability, which may not be present at birth. Timely screening for cytomegalovirus in the first weeks after birth is critical to appropriately diagnose congenital infection, evaluate affected infants, and determine the treatment course. Antiviral therapy with valganciclovir can optimize end hearing and neurodevelopmental outcomes in symptomatic infants. This review discusses the epidemiology and clinical manifestations of cCMV, targeted and universal screening approaches, and treatment and monitoring of infants with cCMV.

Telemedicine During the COVID-19 Pandemic: A Pediatric Otolaryngology Perspective.

The COVID-19 pandemic has created a situation unparalleled in our lifetime. As the medical community has attempted to navigate a sea of ever-changing information and policies, this uncertainty has instead bred creativity, community, and evolution. Necessity is the mother of invention, and one of the by-products of our rapidly changing environment is the increased reliance on telemedicine. Here, we discuss our experience with incorporating telemedicine into an urban academic pediatric otolaryngology practice, the challenges that we have encountered, and the principles unique to this population.

International Pediatric Otolaryngology Group (IPOG) Consensus Recommendations: Congenital Cholesteatoma.

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.

Transcutaneous Osseointegrated Implants for Pediatric Patients With Aural Atresia.

Importance: Patients with aural atresia typically have maximal conductive hearing loss, which can have negative academic and social consequences. Transcutaneous osseointegrated implants (TOIs) can potentially restore hearing on the affected side. Objectives: To review the demographic, audiological, and surgical outcomes of TOI placement in pediatric patients with aural atresia and to describe a modification in incision technique in anticipation of later auricular reconstruction. Design, Setting, and Participants: This retrospective case series reviewed 41 cases of TOI placement in pediatric patients between January 1, 2014, and September 30, 2016, at Lurie Children's Microtia and Aural Atresia Clinic. Patients, all younger than 18 years and with atresia or microtia, received at least 6 months of follow-up and underwent testing before and after surgery. Main Outcomes and Measures: Patient age, indication for procedure, ear sidedness, case length, incision type, complications, and other postoperative events. Audiological outcomes before and after implantation were measured using pure-tone averages and the Hearing In Noise Test for Children, presented in variable signal to noise ratios. Results: In total, 46 TOIs were performed in 38 pediatric patients, but only 41 implantations in 34 patients were included in this study. Of the 34 patients, 13 (38%) were males and 21 (62%) were females, with a mean age of 8.9 (range, 5-17) years at the time of TOI placement. Microtia on the implanted side was present in 39 cases (95%). A modified posterior-superior scalp incision technique was used in 30 (73%) of 41 ears, all in cases of microtia. One perioperative surgical complication occurred: a seroma requiring drainage. Two patients developed minor skin irritation and erythema at the magnet site related to the overnight use of the processor, which resolved when removed while sleeping. The mean (SD; range) score for the Speech In Noise test at 5 dB signal to noise ratio improved from 75.3% (14.4%; range, 50%-92%) correct in unaided/preoperative condition to 93.6% (6.95%; range, 80%-100%) correct in the aided/postoperative condition. The mean improvement in score was 18.3% (95% CI, 10.8%-25.9%), with an effect size of 1.62 (95% CI, 0.95-2.29). The mean pure-tone averages (SD; range) similarly improved from 63.7 (13.2; range, 25-11) dB to 9.6 (4.9; range, 5-15) dB. Conclusions and Relevance: Transcutaneous osseointegrated implantation has a low complication rate among pediatric patients with atresia or microtia and can provide excellent audiological results. It should be included as a treatment option for this population of patients who meet audiological criteria.

Endoscopic middle ear exploration in pediatric patients with conductive hearing loss.

OBJECTIVE: To describe our indications, findings, and outcomes for transcanal endoscopic middle ear exploration in pediatric patients with conductive hearing loss of unknown etiology, without effusions. METHODS: Prospective case series for all pediatric patients undergoing totally endoscopic transcanal middle ear exploration between April 2012 and October 2015 at a pediatric tertiary care referral hospital. Demographic data, operative findings, and hearing results were reviewed. RESULTS: 21 cases were performed in 20 ears (1 revision). Average age at surgery was 7.98 years and average follow up was 2.1 years. Middle ear pathology identified on CT imaging was confirmed in 55% of cases while identified in 45% of cases where pre-operative imaging was non-diagnostic. 6/20 patients (30%) had an ossicular deformity. 8/20(40%) had bony ossicular fixation. 5/20(25%) had ossicular discontinuity. 2/20(10%) had facial nerve dehiscence impinging on the stapes. 15% had adhesive myringosclerosis or severe granulation causing hearing loss. Prosthetic ossiculoplasty was done in 7/21 (33.3%) of the cases, with 1 TORP, 3 PORPs, and 3 IS joint replacements. Imaging was predictive of intra-operative findings in 13/20 cases (55%). Trainees assisted in 16/21(76%) of cases. The average improvement of PTA was 11.65 dB (range -10 to 36.25), and the average ABG improved 10.19 (range -11.25 to 28.75). There were no perioperative complications or adverse events. CONCLUSIONS: The endoscopic transcanal approach for middle ear exploration offers excellent visualization and is one of the best applications for the endoscopes in pediatric otology cases. This is particularly helpful for "unexplained" conductive hearing loss where ossicular deformity/fixation/discontinuity is suspected. The etiology of the conductive hearing loss was definitively found in 100% of cases, and can be repaired in the same sitting when applicable.

Endoscopic transcanal approach to the middle ear for management of pediatric cholesteatoma.

OBJECTIVES: To describe outcomes for endoscopic ear surgery (EES) for pediatric cholesteatoma at a tertiary pediatric hospital. METHODS: Retrospective case series of 65 pediatric cholesteatoma cases in 38 ears. Subgrouping based on cholesteatoma type and EES type. Surgical findings, outcomes, and demographic data were evaluated. RESULTS: Endoscopes were used in 65 pediatric cholesteatoma cases in 38 primary ears (34 patients), followed for an average of 2.6 years (9 months to 4.6 years). The endoscope was used as the primary visualization tool in 31 (81.6%) ears (EES 2 or 3), and as an adjunct to the microscope in seven ears (EES 1). Twenty-two (57.9%) ears and 35 (53.4%) cases were transcanal endoscopic ear surgery (EES 3 or TEES). Overall, there was recurrence in five (13.2%) ears and residual in four (10.5%) ears. Cholesteatoma was acquired in 27 ears, with average age 10.9 years; and congenital in 11 ears, with average age 3.8 years. Surgical time was longer for acquired cases (226 vs. 154 minutes). Hearing outcomes were comparable for both cholesteatoma types. Residual disease was seen in three (11.1%) acquired ears and one (9.1%) congenital ear. Overall, the lowest rates of recurrent and residual disease were seen in EES 3 cases, and relatively low rates in EES 2 and 3 ears, including four (12.9%) recurrences and two (6.5%) ears with residual disease. CONCLUSION: The endoscopes are a viable tool for resection of pediatric cholesteatoma and provide excellent visualization of the middle ear and associated recesses. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:2653-2658, 2017.

Outcomes and Time to Emergence of Auditory Skills After Cochlear Implantation of Children With Charge Syndrome.

OBJECTIVE: Review perioperative complications, benefits, and the timeframe over which auditory skills develop in children with CHARGE syndrome who receive a cochlear implant (CI). STUDY DESIGN: IRB-approved retrospective chart review of children with CHARGE syndrome who had at least 12 months of cochlear implant use. SETTING: Tertiary care children's hospital. PATIENTS: Twelve children, seven males and five females. Mean age implant = 3.5 years (1.7-8.2 yr); mean duration follow-up = 4.7 years (1.5-10.1 yr). INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Auditory skills categorized into four levels, temporal bone imaging findings, perioperative complications, time to emergence of speech perception, expressive communication mode. RESULTS: All children imaged with magnetic resonance imaging had cochlear nerve deficiency in at least one ear. Speech awareness threshold improved with the CI compared with aided preoperative in 83% of children, with means of 51.7 dB SAT preoperative and 27.1 dB with the CI (p ≤ 0.002). Overall, four children improved to auditory Level 2 (improved detection), three obtained Level 3 (closed-set speech perception), and three had open-set speech perception with their CIs (Level 4) that was first evident at 3.5, 3.3, and 0.8 years postimplant testing. Two children had minimal or limited improvement. One child with hypoplasia of the cochlear nerve obtained open-set levels. CONCLUSION: Auditory skills may develop slowly in children with CHARGE syndrome who receive a CI but most can achieve at least improved detection. In our series, half acquired some speech perception ability. Cochlear nerve deficiency is frequent, but should not be a contraindication to implantation.

Management of hearing loss and the normal ear in cases of unilateral Microtia with aural atresia.

OBJECTIVES/HYPOTHESIS: To identify the rate of hearing loss related to middle ear disease and the frequency of tympanostomy tube (TT) insertion in the contralateral ear of patients with unilateral microtia/aural atresia. STUDY DESIGN: Retrospective case series of patients less than 3 years of age with unilateral microtia/aural atresia treated at an urban, tertiary care children's hospital from 2008 to 2013. METHODS: Clinical and audiologic data were reviewed. Statistical analysis was performed to determine the relative risk of TT insertion in the normal ear. RESULTS: A total of 72 patients were included for analysis. The average age of patients at their initial otolaryngology visit was 3.3 months (range 0.08-1.67 years); 38 (52.8%) patients were males. Aural atresia involved the right ear in 43 (59.7%) cases. Five (6.9%) patients were syndromic. Abnormal audiometric testing of the normal ear was noted in 12 (16.7%), and 14 (19.4%) underwent TT during the first 3 years of life. Twelve children (85.7%) who had a TT placed were nonsyndromic. When compared to published norms for TT placement in the general population (6.8% of children < 3 year of age), a greater proportion of children with unilateral microtia/aural atresia had TT placement in the normal ear (z = 4.26, P < 0.0001). CONCLUSION: Patients with unilateral microtia/aural atresia have increased rates of hearing loss and middle ear effusion leading to TT in their normal ear at a higher rate versus the general population. This information can help guide more vigilant care and audiologic follow-up in affected children. LEVEL OF EVIDENCE: 4. Laryngoscope, 126:1470-1474, 2016.

Head and neck vascular lesions.

Vascular lesions of the head and neck are complex and diverse. These include infantile hemangioma, venous malformations, lymphatic malformations, and arteriovenous malformations, among others. Vascular malformations and tumors display different growth patterns and require different approaches to treatment. Therefore, accurate diagnosis is of utmost importance. This article is a guide for the diagnosis and management of vascular lesions of the head and neck.

The proximal bronchoplasty retrieval technique for removal of embedded distal airway foreign bodies.

OBJECTIVES: To describe a novel technique for removal of embedded distal airway foreign bodies with emphasis on procedural steps and management. METHODS: 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. RESULTS: The PBR technique was successfully used to remove the two described embedded distal airway foreign bodies after other attempts had failed. CONCLUSION: 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.

The transpalatal approach to repair of congenital Basal skull base cephaloceles.

Basal skull base herniations, including meningoceles and encephaloceles, are rare and may present with characteristic facial and neurologic features. The traditional craniotomy approach has known morbidity, and nasal endoscopy may not allow for control of large posterior basal defects, especially in newborns. We present two cases of successful repair of basal transsphenoidal meningoceles using an oral-transpalatal approach. The first patient with an intact palate presented with respiratory distress, and a palatectomy was performed for access to the skull base. The second patient had a large basal herniation that was reduced through a congenital midline cleft palate, and a calvarial bone graft was used to repair the defect. A literature search revealed 10 previous successful cases using the transpalatal repair, which allows for excellent access, low morbidity, and a team-oriented method to skull base surgery.

The "postcricoid cushion": observations on the vascular anatomy of the posterior cricoid region.

OBJECTIVE: To describe the cyclical vascular enlargement that occurs in the postcricoid region during the expiratory phase on an infant's cry, and to consider the anatomic, physiologic, and clinical implications of this phenomenon, which we term the "postcricoid cushion." DESIGN: A total of 125 consecutive office fiber-optic laryngoscopic examinations in children and infants were reviewed for engorgement and vascular discoloration of the postcricoid region. Presence of a postcricoid cushion in relation to patient age was reviewed. A comprehensive literature review was also performed. SETTING: Tertiary care pediatric hospital. PATIENTS: Patients from newborns to 17 years old undergoing laryngoscopy for any reason. RESULTS: Sixty-one percent of the videos showed a postcricoid cushion with cyclical enlargement during crying. Eighty-eight percent of children younger than 24 months had presence of a cushion compared with only 38% of children 24 months or older (P < .001). Twenty-five percent of the cushions had violaceous discoloration that resembled a vascular malformation. CONCLUSIONS: Anatomic studies have demonstrated a rich venous plexus in the postcricoid region of the larynx. During the expiratory phase of an infant's cry, there is a cyclical engorgement, occasionally with vascular discoloration, in the postcricoid region at the same level of the venous plexus-the "postcricoid cushion." We propose that during crying, with acute elevation in intrathoracic pressure, there is a filling of the plexus, causing apposition of the postcricoid cushion against the posterior pharyngeal wall, which may serve as a protective barrier to emesis in infants. Our observations relate and differentiate this normal physiologic phenomenon from the rare cases of postcricoid vascular anomalies.

Aspiration before and after Supraglottoplasty regardless of Technique.

Objective. To determine the incidence of preoperative and postoperative aspiration in infants who undergo supraglottoplasty. To determine the effect of cold steel and CO(2) laser supraglottoplasty on aspiration in infants with severe laryngomalacia. Design. Retrospective study. Setting. Tertiary pediatric hospital. Patients. Thirty-nine patients who underwent CO(2) laser-assisted supraglottoplasty (CLS) or cold steel supraglottoplasty (CSS) for severe laryngomalacia. Main Outcome Measures. Aspiration and upper-airway obstruction. Results. Thirty-nine patients met inclusion criteria (18 males, 21 females). Eighteen patients underwent CSS and 21 patients underwent CLS. 10/39 (25.6%) of the patients had preoperative aspiration, and 2/10 (20%) resolved after supraglottoplasty. New onset aspiration was found in 4/13 (30.8%) in the CSS group and 9/16 (56.3%) in the CLS group. Conclusions. There is no significant difference in the rate of postoperative new-onset aspiration or relief of upper-airway obstruction in the CLS or CSS, is temporary and can be managed with thickened diet or temporary tube feedings. The rate of persistent postoperative aspiration was statistically similar regardless of the method of surgery.

Supraglottoplasty outcomes in relation to age and comorbid conditions.

OBJECTIVE: To determine if age and comorbid conditions effect outcomes in children undergoing supraglottoplasty for severe laryngomalacia. DESIGN: Retrospective study. SETTING: Urban tertiary-care children's hospital. PATIENTS: Children undergoing supraglottoplasty for severe laryngomalacia between February 2004 and July 2008. 56 patients were identified. OUTCOME MEASURES: Persistence of upper airway obstruction, revision surgery (supraglottoplasty), and additional surgery (tracheostomy). RESULTS: 33/56 (58.9%) patients had no comorbid conditions and 23/56 (41.1%) patients had comorbid conditions. In noncomorbid patients, 36.4% of those less than 2 months of age at the time of surgery required revision supraglottoplasty, compared to 5.3% of patients between 2 and 10 months (p<0.05). Compared to the 2-10-month age group, there was a significantly higher percentage of patients with comorbid conditions in the >10-month group (32.1% vs. 79%, p<0.01). Patients with comorbid conditions were diagnosed at a significantly later age than those without (6 mo vs. 2 mo, respectively), and had significantly higher rates of revision supraglottoplasty (47.8% vs. 18.2%) and tracheostomy (39.1% vs. 0.0%). 70% of children with neurological conditions required revision surgery, with 60% requiring tracheostomy. The revision surgery and tracheostomy rates were significantly higher compared to the noncomorbid group (p<0.01 and p<0.0001). Children with cardiac conditions had a higher rate of tracheostomy than noncomorbid children (30% vs. 0%, p<0.01). 16.7% of children with genetic conditions required supraglottoplasty, and none required tracheostomy. CONCLUSIONS: In noncomorbid patients, those undergoing supraglottoplasty less than 2 months of age had a significantly higher rate of revision supraglottoplasty. Patients with neurologic and cardiac comorbidities require tracheostomy at a significantly higher rate than noncomorbid patients.