Risk factors and outcomes in patients with cochlear nerve deficiency.

INTRODUCTION: Cochlear nerve deficiency (CND) is a cause of sensorineural hearing loss made by radiologic criteria. There is sparse literature involving audiological outcomes and cochlear implantation (CI) success in patients with CND. METHODS: A retrospective chart review of all patients with sensorineural hearing loss at a tertiary children's hospital from 2000 to 2020 was conducted. Patients with CND on radiographic imaging were included and categorized as hypoplastic, aplastic, or indeterminate. RESULTS: In this study, 53 patients were identified with CND, totaling 70 ears. Of the 53 patients, 30 (56.6 %) were male, 8 (16.0 %) had a family history of childhood hearing loss, 6 (11.3 %) were born preterm, and 11 (23.4 %) required neonatal intensive care admission. The median maternal age was 29 years old [IQR: 27, 35], and 8 (15 %) patients were born to mothers with diabetes. Of the 70 ears, 49 (70 %) utilized conventional hearing aids, 12 (17.1 %) utilized a bone-anchored hearing aid, and 10 (14.3 %) underwent CI. Of the 10 ears implanted, 4 (40 %) ears had nerves classified as hypoplastic, 3 (30 %) as aplastic, and 3 (30 %) as indeterminate. Improvement in pure tone averages compared to preoperative testing was demonstrated in 8 (80 %), and 6 (60 %) displayed improved speech awareness thresholds. CONCLUSION: This study demonstrates that there may be an association between CND and maternal diabetes and NICU admission. There are variable results with hearing amplification options in patients with CND, and further research is needed to better describe the role of CI, bone-anchored hearing aids and conventional hearing aids in patients with CND.

Update on congenital ear molding.

PURPOSE OF REVIEW: Congenital ear deformities are characterized by distorted, but still present, landmarks of the auricle. Interventions include early treatment with ear molding or delayed surgical treatment. The purpose of this review is to provide an update on the latest information related to ear molding for congenital ear deformities. RECENT FINDINGS: Various ear molding techniques date back to the 1980s, but with the availability of commercially available molding systems, interest and use has blossomed in recent years. As more longitudinal research results are obtained, ear molding has been proven to be a highly effective treatment, although the best technique and length of treatment remains unclear. SUMMARY: While it is clear that earlier intervention is ideal, due to lack of public awareness, patients often present later, and it remains controversial what age to offer ear molding interventions to and still expect success. Ear molding interventions are becoming increasingly covered by insurance due to evidence of preventing long-term psychological morbidities and the need for future surgical interventions.

Pediatric Otolaryngology fellowship virtual interview sentiments.

BACKGROUND: Interviews for Pediatric Otolaryngology fellowship rapidly transitioned to virtual interviews mid-cycle in March 2020 due to the COVID-19 pandemic. OBJECTIVE: This study aims to describe perspectives on virtual versus in-person interviews for both applicants and program directors. METHODS: Cross-sectional study. Surveys were conducted of all Pediatric Otolaryngology fellowship applicants participating in the San Francisco Match and program directors in 2020 and 2021. RESULTS: Out of 32 U.S. trained fellowship applicants, 24 completed the survey in 2020 and 18 in 2021. While 70% of applicants felt they did not get the same experience with virtual interviews, 75% did not feel it changed how they ranked programs. Applicant perception of virtual interviews improved in 2021, with the majority (56%) preferring virtual interviews if provided an option. Twenty out of 36 fellowship directors completed the survey in 2020, and eighteen in 2021. While fellowship directors continued to prefer in-person in 2021, an increased number (10% in 2020, 30% in 2021) felt continuing with virtual interviews may increase the number of applicants in the future. CONCLUSION: Based on the survey, both applicants and fellowship directors had a less favorable perception of virtual interviews compared to in-person interviews initially; however, applicant perception favored virtual interviews in 2021, while Program Directors continued to prefer in-person.

Percutaneous Enteral Feeding in Patients With 22q11.2 Deletion Syndrome.

OBJECTIVE: To describe the incidence of percutaneous enteral feeding in patients with 22q11.2 deletion syndrome (22q11.2 DS) and determine factors associated with the need for percutaneous enteral feeding tube placement. DESIGN: Retrospective chart review. METHODS: The records of a 22q11.2 DS clinic and pediatric otolaryngology clinic at a tertiary pediatric hospital were reviewed from January 1, 2009, to December 31, 2019. All patients with confirmed 22q11.2 deletion were identified. Cardiac, otolaryngological, and feeding characteristics were recorded along with surgical history. A patient was defined to have a G-tube if the history was significant for any percutaneous gastric feeding tube placement, including a gastrostomy tube, gastrostomyjejunostomy tube, or a Mickey button. RESULTS: One hundred ninety patients with confirmed 22q11.2 DS by genetic testing were included. Thirty-three percent (n = 63) required G-tube placement. G-tube placement was associated with cardiac diagnosis (P < .01), history of cardiac surgery (P < .01), aspiration (P < .01), nasopharyngeal reflux (P < .01), subglottic stenosis (P < .01), laryngeal web (P = .003), and tracheostomy (P < .01). This suggests these conditions are associated with higher rates of G-tube placement in the 22q11.2 DS population. CONCLUSIONS: Patients with 22q11.2 DS often require supplemental nutritional support in the form of G-tube feeding, most often in the first year of life. Congenital heart abnormalities and surgery along with tracheostomy, subglottic stenosis, laryngeal web, aspiration, and nasopharyngeal reflux are significantly associated with the need for G-tube placement. Understanding associations between comorbid conditions and G-tube placement, especially those involving the head and neck, may assist with counseling of patients with 22q11.2 DS.

Incidence of mandibular distraction osteogenesis in Stickler Syndrome: Variation due to COL2A1 and COL11A1.

OBJECTIVE: To determine whether the two most common genetic mutations seen in Stickler Syndrome (SS) (COL2A1 and COL11A1) affect the incidence of mandibular distraction osteogenesis (MDO) and what impact Robin sequence (RS) has on diagnosis. SS is an autosomal dominant connective tissue disorder characterized by almost complete penetrance. COL2A1 and COL11A1 are the two most common mutations seen in SS patients. SS often presents at birth with RS, which is characterized by the triad of micrognathia, glossoptosis, and tongue-based airway obstruction. MDO is one surgical intervention that has been shown to be successful in relieving tongue base obstruction and is the surgical intervention of choice for this condition. METHODS: A retrospective chart review was performed on all patients with a diagnosis of SS at a tertiary pediatric hospital between January 1, 2003 and December 31, 2018. The included patient charts were reviewed for demographic information, SS mutation, and history of MDO. Forty-six patients had a clinical diagnosis of SS. Of those, 31 met inclusion criteria which involved having a molecular diagnosis of SS and sufficient follow up information to determine if MDO was indicated or performed. Twenty-two of the 31 included patients had a diagnosis of RS (70.96%). Thirteen of the 31 patients (41.94%) included in this study required MDO as a neonate. RESULTS: Fifty-percent of patients with type I (COL2A1) required MDO as a neonate compared to only 31% of patients with type II (COL11A1), though the difference between the two groups was not statistically significant. CONCLUSION: The findings of this study suggest that patients with type I mutation may have a higher incidence of MDO than patients with a type II mutation, though further research with larger sample sizes is needed. This information is helpful in counseling those with SS or family history of SS about what they can expect related to RS and need for MDO based on genetic findings. LEVEL OF EVIDENCE: 3.

Submucous Cleft Palate Repair in Patients With 22q11.2 Deletion Syndrome.

OBJECTIVE: To determine whether surgical intervention for submucous cleft palate (SMCP) is more common in children with 22q11.2 deletion syndrome (22q DS) compared to children without 22q DS. DESIGN: Retrospective chart review. SETTING: Tertiary pediatric hospital and 22q11.2 DS specialty clinic. PARTICIPANTS: One hundred forty-two children seen at the tertiary hospital or clinic during a 20-year period (June 1999-June 2019) with documented SMCP with and without 22q DS. MAIN OUTCOME MEASURE: Percentage of children with SMCP with and without 22q DS requiring surgical intervention for velopharyngeal insufficiency. RESULTS: Patients with 22q DS had a significantly higher frequency of SMCP repair than those without 22q DS (89.7% vs 32.0%, P < .001, χ2 = 37.75). The odds of requiring SMCP repair were 18.6 times higher in those with 22q DS compared to those without (odds ratio = 18.6, CI = 6.1-56.6). CONCLUSIONS: This study provides new evidence suggesting patients with 22q DS require SMCP surgical repair for velopharyngeal insufficiency at a significantly higher rate than those without 22q DS. As the majority of patients with 22q DS with SMCP require surgical intervention, future prospective studies looking at early versus late repair of SMCP in patients with 22q DS are needed to guide the surgical repair timeline in this population.

Malnutrition as a Risk Factor in Cleft Lip and Palate Surgery.

OBJECTIVES/HYPOTHESIS: To assess the prevalence of acute and chronic malnutrition at the time of surgery in patients with cleft lip and/or palate (CLP) at our institution, and to quantify nutrition as a risk factor for postsurgical complications following CLP surgery. STUDY DESIGN: Retrospective cohort study. METHODS: Retrospective review of 855 children undergoing initial cleft lip or palate surgery, or revision surgery after fistula/dehiscence of initial cleft repair. We measured acute and chronic malnutrition using World Health Organization Z-scores of weight-for-age and height-for-age, respectively, and noted any postsurgical fistula or dehiscence. RESULTS: Among patients with cleft lip, 22.3% were at least moderately chronically malnourished at the time of initial repair, and 17.5% were at least moderately acutely malnourished. Among patients undergoing initial repair of cleft palate, 20.9% were at least moderately chronically malnourished, and 8.1% were at least moderately acutely malnourished. Increasing nutritional status, as measured by height-for-age, predicts decreased odds of fistula (OR 0.78, P = .01) after cleft palate surgery. CONCLUSIONS: Chronic malnutrition significantly increases the risk of fistula formation in patient with cleft palate. Preoperative strategies to manage this risk and influence surgical timing can avoid morbid and costly postoperative complications. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2060-E2065, 2021.

Comparing autologous versus allogenic rib grafting in pediatric cleft rhinoplasty.

BACKGROUND: Patients with cleft lip with or without cleft palate suffer from varying degrees of nasal deformity, often requiring nasal reconstruction to provide improved form and function. Rib cartilage is an excellent source of grafting material for nasal reconstruction and is available either as an autologous or allogenic graft. There is a paucity of literature comparing outcomes of autologous and allogenic rib grafts in pediatric cleft rhinoplasty. METHODS: A retrospective chart review was performed on patients who underwent cleft rhinoplasty with autologous or allogenic rib grafting at a tertiary pediatric hospital between January 1, 2003 and December 31, 2017. Outcome data were gathered over a 6-month postoperative period. RESULTS: There were 23 cleft rhinoplasties performed with rib graft, 12 with autologous rib and 11 with allogenic rib. Those in the autologous group tended to be older than those in the allogeneic group (15.6 ± 4.4 v 12.4 ± 5.2 years, p = 0.13). The most common types of grafts used were columellar strut (20/23), shield graft (9/23), and unilateral or bilateral batten grafts (7/23). Length of stay was significantly longer for patients who underwent autologous rib grafting compared with those with allogenic rib grafting (25.8 ± 4.7 v 11.9 ± 7.2 h, p < 0.05). Each group reported one complication. CONCLUSION: Autologous and allogenic rib grafts are safe and effective in pediatric rhinoplasty. The most common grafts used in this sample were columellar strut, batten, and shield grafts. Autologous rib grafts were more likely to be used in older patients and require longer hospital stay compared to allogenic grafts.

Associated syndromes in patients with Pierre Robin Sequence.

OBJECTIVES: Classically, Pierre Robin Sequence (PRS) is a triad of micrognathia, glossoptosis, and airway obstruction, although frequently associated with cleft palate. Current literature reports that Stickler syndrome is the most common syndrome associated with PRS, and 22q11 deletion syndrome (22q11 DS) as the second most common. This study identifies associations between PRS and genetic syndromes. METHODS: A retrospective chart review was performed to identify patients diagnosed with PRS over a 10-year period from 4/1/2007 to 4/1/2017 at a tertiary children's hospital. RESULTS: 4,052 consecutive charts were reviewed and 234 patients had a diagnosis of PRS confirmed with the triad of micrognathia, glossoptosis, and airway obstruction. Of note, all of these patients had cleft palate. Of the 234 patients with PRS, 65 patients had syndromic diagnoses (28%). One patient had 22q11 DS (0.43%), and 31 patients had Stickler syndrome (13.2%). Additionally, 3 patients had central hypoventilation syndrome, 3 patients had Duane syndrome, 2 patients had Cornelia de Lange syndrome, 2 patients had Emanuel syndrome, 2 patients had Gordon syndrome, 2 patients had Mobius syndrome, 2 patients had Nager syndrome. Multiple other syndromes were identified, but occurred in isolated cases. CONCLUSION: This study supports literature that PRS is most commonly associated with Stickler Syndrome but rarely associated with 22q11 DS given that only 1 patient had both PRS and 22q11 DS.

Vincristine-induced vocal cord paresis and paralysis in children.

OBJECTIVES: To describe three new cases of vincristine-induced vocal cord paresis or paralysis (VIVCPP) in children and to review the diagnosis and management of this neuropathy. METHODS: Retrospective case series. Diagnosis of VIVCPP was confirmed by laryngoscopy in all children. RESULTS: Less than 20 cases of VIVCPP in children have been previously documented in the literature. Of the three children in our case series, one had unilateral vincristine-induced vocal cord paresis and two had bilateral VIVCPP. The first two patients each had two separate episodes of paresis, lasting 4 months and 1 month respectively. In the last patient, whose medical course was complicated by many additional factors, vocal cord paralysis persisted for over three years. CONCLUSIONS: Clinicians must evaluate children with suspected VIVCPP for concomitant symptoms and signs of vincristine neuropathies and examine the vocal cords via laryngoscopy. The effects of vincristine neurotoxicity can be waxing and waning, demonstrate delayed onset and persist well beyond drug cessation. Further studies are needed to identify effective neuroprotectants and delineate appropriate vincristine dosing in patients with vincristine neurotoxicity and cancer.

Upper airway obstruction in neonates: Does sleep exacerbate symptoms?

OBJECTIVE: Describe the factors that exacerbate upper airway obstructions (UAOs) in neonates. STUDY DESIGN: Retrospective chart review. SETTING: Pediatric tertiary care hospital. SUBJECTS AND METHODS: All neonates hospitalized between 1/1/2010 and 12/31/2014 diagnosed with either: 1) laryngomalacia, 2) Pierre Robin sequence, or 3) vocal cord paralysis were included in this study. Patient charts were reviewed to determine factors that exacerbated symptoms of airway obstruction. The independent variable was patient diagnosis, and the outcome measure was exacerbating factors. RESULTS: In patients with laryngomalacia (n = 31), 41.9% worsened with agitation, 38.7% worsened with feeding, 16.1% worsened with positioning, 0.0% worsened during sleep, and 25.8% had no aggravating factors. In Pierre-Robin patients (n = 31), 48.4% worsened with agitation, 16.1% worsened with feeding, 61.3% worsened with positional changes, 0.0% worsened during sleep, and 12.9% had no aggravating factors. In vocal cord paralysis patients (n = 25), 72.0% worsened with agitation, 8.0% worsened with feeding, 20.0% worsened with positional changes, 4.0% worsened during sleep, and 24.0% had no aggravating factors. CONCLUSION: Airway obstruction was not reliably exacerbated during sleep for any of the diagnoses studied in this review. Our findings show that agitation exacerbates airway obstruction in most patients with vocal cord paralysis, and positioning exacerbates airway obstruction in the majority of patients with PRS. Aggravating factors in laryngomalacia are variable. These findings question the utility of polysomnography as a diagnostic tool for hospitalized neonates with these conditions. LEVEL OF EVIDENCE: 4.

Congenital and iatrogenic laryngeal and vocal abnormalities in patients with 22q11.2 deletion.

BACKGROUND: Voice abnormalities often go unrecognized in patients with 22q11.2 deletion because speech abnormalities become the focus of evaluation. OBJECTIVE: To analyze voice and vocal fold abnormalities in patients with 22q11.2 deletion by examining voice, not speech. METHODS: This is a case series with chart review from 2009 to 2016. Records of both a velocardiofacial syndrome (VCF) clinic and pediatric otolaryngology clinic at a tertiary pediatric hospital were reviewed. All patients with confirmed 22q11.2 deletion were identified. Cardiac, otolaryngological, speech, and voice characteristics were recorded along with surgical history. Main outcomes included voice characteristics based on speech therapy assessment, along with vocal fold or laryngeal abnormalities. Co-morbidities and surgical history were also recorded. RESULTS: 109 patients were identified with 22q11.2 deletion by genetic testing. Eighteen percent (n = 20) displayed a vocal fold or laryngeal abnormality, either congenital or iatrogenic. The most common congenital abnormalities were laryngeal web (n = 10) and subglottic stenosis (n = 7). The most common iatrogenic abnormality was paralyzed left vocal fold (n = 4). Perceptual analysis by speech therapy showed 65% (n = 71) with a voice within normal limits while 17% (n = 19) were not within normal limits. Of the 20 patients with a vocal fold abnormality, 15% (n = 3) presented a voice within normal limits, while 65% (n = 13) were not within normal limits. Of the 19 patients with a voice outside normal limits, 68% (n = 13) had a diagnosed vocal fold abnormality. Sixteen percent (n = 3) of these patients had an abnormal voice as a result of other issues. CONCLUSION: Out of 109 patients with 22q11.2 deletion, 18% displayed a laryngeal abnormality and 17% had abnormal vocal quality. This suggests that voice and vocal fold abnormalities are important to consider in this population. Distinguishing between speech and voice abnormalities in patients with 22q11.2 deletion can help ensure appropriate intervention.

Treatment of juvenile recurrent parotitis of childhood: an analysis of effectiveness.

IMPORTANCE: Juvenile recurrent parotitis (JRP) is characterized by recurrent painful swelling of the parotid gland that occurs in the pediatric population. Sialendoscopy with and without ductal corticosteroid infusion (DCI) has been found to be effective in the treatment of JRP and autoimmune parotitis. OBJECTIVE: To determine the utility of instrumentation vs pharmacotherapy alone for juvenile recurrent parotitis. DESIGN, SETTING, AND PARTICIPANTS: A retrospective medical record review of pediatric patients undergoing DCI without sialendoscopy at a tertiary pediatric hospital was conducted. The medical records were reviewed to determine the frequency of parotitis events before and after treatment. A multiquestion telephone survey of patients and their parents who underwent the procedure was then conducted to determine patient satisfaction. INTERVENTIONS: Ductal corticosteroid infusion with hydrocortisone through catheter inserted in the parotid duct. MAIN OUTCOMES AND MEASURES: Frequency of symptoms before and after treatment and parental satisfaction with the treatment. RESULTS: Twelve patients with a mean age of 6.7 years were identified. The mean duration and frequency of symptoms before the procedure were 22 months and every 2 months, respectively. Five patients had a recurrence, on average 4 months after the procedure. Four patients underwent repeated surgical procedures. All had a longer duration between episodes compared with before DCI. Eight parents participated in the survey on satisfaction with the procedure, and 75% reported improvement in their child's life postprocedure. CONCLUSIONS AND RELEVANCE: Current literature shows sialendoscopy with corticosteroid application is successful in treating JRP, but it is unclear whether corticosteroid application alone would treat JRP equally. This study shows that DCI alone has similar results as sialendoscopy with corticosteroid application, indicating that it is the corticosteroid application and not the sialendoscopy causing improvement in symptoms. Because JRP must be differentiated from sialolithiasis, we recommend ultrasonography of the involved parotid(s) prior to using DCI alone to ensure no stone is present. Sialendoscopy should be reserved to rule out other parotid ductal pathologic conditions.

Granulocytic sarcoma of pediatric head and neck: an institutional experience.

OBJECTIVE: To demonstrate a case series of granulocytic sarcoma of the head and neck found in the pediatric population and review long-term outcomes. METHODS: A pathology database at a tertiary hospital was searched for patients with biopsy specimens from the head and neck diagnosed as granulocytic sarcoma. RESULTS: There were 6 cases between 1992 and 2004 that met inclusion criteria. Subjects' age ranged from 22 months to 14 years old. In three cases, the patients were diagnosed with acute myeloid leukemia (AML) based on biopsy results; 2 patients were already diagnosed with AML when diagnosed with granulocytic sarcoma, and in 1 case, a relapse of AML was diagnosed. In all cases, patients began induction chemotherapy. Two patients died during induction chemotherapy from infection. The remaining 4 patients underwent bone marrow transplants. One patient had a relapse post-transplant and died. Only one patient was healthy two years post-transplant. CONCLUSION: The results of this series suggest granulocytic sarcoma must be on the differential when tumors present in the head and neck region in pediatric patients. In our series, 100% of the patients with granulocytic sarcoma had underlying AML. The long-term prognosis of patients with AML who developed granulocytic sarcoma is quite poor.

Prevalence of positional plagiocephaly in teens born after the "Back to Sleep" campaign.

OBJECTIVES: To determine the prevalence of positional plagiocephaly and brachycephaly in teenagers born after the "Back to Sleep" campaign but before orthotic helmet treatment became widely available and to provide long-term outcomes data on those children with plagiocephaly who were not treated with remolding therapy. STUDY DESIGN: Cross-sectional analysis. SETTING: Local high schools in the Minneapolis-St Paul Metro area. SUBJECTS AND METHODS: Subjects were selected if they attended participating high schools and were born after the Back to Sleep campaign began. Skull measurements, including transcranial diameter, length, and width, were taken. Overall facial appearance was inspected for any apparent abnormal characteristics. Cranial vault asymmetry and cephalic index were calculated for each participant. Plagiocephaly was diagnosed if cranial vault asymmetry was >1 cm. Brachycephaly was diagnosed if the cephalic index was >0.90. RESULTS: There were 1045 participants, ranging from 12 to 17 years old, with an average age of 15.7 years. The prevalence of plagiocephaly was 1.1%. The prevalence of brachycephaly was 1.0%. The overall prevalence of a deformational cranial abnormality was 2.0%. Of those who met diagnostic criteria of plagiocephaly or brachycephaly, 38.1% were noted to have abnormal facial characteristics. CONCLUSIONS: The prevalence of plagiocephaly and brachycephaly in teenagers is significantly lower than the 20% to 48% prevalence found in infants in previous studies, suggesting most children will outgrow the condition without intervention. Additional studies are needed to determine which patients might benefit from treatment and which will likely have resolution without intervention, since treating all infants who meet criteria results in significant overtreatment.

Symptom timeline preceding cochlear implant failure: an institutional experience.

OBJECTIVE: Review cochlear explants and determine the incidence of device and medical failures and develop a pattern of symptoms indicating probable implant failure. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Subjects were selected if they underwent cochlear explantation. Data were reviewed to determine initial symptom and symptom timeline preceding implant failure. RESULTS: A total of 847 implants were performed from 1988 to 2008, with 128 devices explanted. Of total implants, 72% were Advanced Bionic devices, and 28% were Cochlear Nucleus devices. Overall failure rate was 128 of 847 (15%), with 51 (6%) medical failures and 77 (9%) device failures. Patients with Advanced Bionic devices underwent 102 explants (16.7% failure rate), with 35% medical failures and 65% device failures. Patients with Nucleus devices underwent 26 explants (11% failure rate), with 58% medical failures and 42% device failures. Medical failures included infected devices and wound dehiscence, with the pattern averaging 4.5 months to explantation. Hard device failures included sudden malfunction and slow decline in function, with pattern of failure over 4.2 months. Soft failures included tinnitus and discomfort, with failure over 8 months. CONCLUSIONS: There were common patterns when evaluating both medical and device failures. By recognizing patterns of symptoms that may indicate probable implant failure, otolaryngologists can better counsel patients on what to expect with their implants when they present with certain symptoms.