The goals of cleft palate repair are well-established; however, there does exist difference in practice patterns regarding the most appropriate patient age for palatoplasty. The optimal timing is debated and influenced by cleft type, surgical technique, and the surgeon's training. The objective of this study was to compare the rates of post-operative fistula formation and velopharyngeal insufficiency (VPI) in “early” versus “standard” cleft palate repair in a cohort of patients treated at a single craniofacial center. A retrospective chart review identified 525 patients treated for cleft palate from 2000 to 2017 with 216 meeting inclusion criteria. “Early repair” is defined as palatoplasty before 6-months of age (108 patients). “Standard repair” is palatoplasty at or beyond 6-months old (108 patients). Rates of fistula formation were found to be significantly higher in early repairs (Chi-square statistic 9.0536, P value = 0.0026). Development of VPI was not significantly different between the 2 groups (Chi-square statistic 1.2068, P value = 0.27196). As expected, the incidence of post-palatoplasty VPI was significantly higher in patients who had a post-operative fistula when compared to those who healed without fistula formation (Chi-square statistic 4.3627, P value = 0.0367). There is significant debate regarding the optimal timing of cleft repair to maximize speech outcomes and minimize risks. The authors’ data show that post-operative fistula formation occurs at a higher rate when performed prior to 6 months old. Furthermore, while the rate of VPI was not significantly affected by age at time of surgery, it was significantly higher in those who experienced a post-operative fistula.

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Objective To identify inner and middle ear anomalies in children with 22q11.2 deletion syndrome (22q11DS) and determine associations with hearing thresholds. Study Design Retrospective study. Setting Two tertiary care academic centers. Methods Children presenting with 22q11DS between 2010 and 2020 were included. Temporal bone imaging with computed tomography or magnetic resonance imaging was reviewed by 2 neuroradiologists. Results Twenty-two patients (12 female, 10 male) were identified. Forty-four ears were evaluated on imaging. There were 15 (34%) ears with abnormal semicircular canals, 14 (32%) with abnormal vestibules, 8 (18%) with abnormal ossicles, 6 (14%) with enlarged vestibular aqueducts, 4 (9.1%) with abnormal facial nerve canals, and 4 (9.1%) with cochlear anomalies. There were 25 ears with imaging and audiometric data. The median pure tone average (PTA) for ears with any structural abnormality was 41.0 dB, as compared with 28.5 dB for ears without any structural abnormality ( P = .21). Of 23 ears with normal imaging, 6 (26%) had hearing loss in comparison with 13 (62%) of 21 ears with abnormalities ( P = .02). Total number of anomalies per ear was positively correlated with PTA (Pearson correlation coefficient, R = 0.479, P = .01). PTA was significantly higher in patients with facial nerve canal anomalies ( P = .002), vestibular aqueduct anomalies ( P = .05), and vestibule anomalies ( P = .02). Conclusions Semicircular canal, ossicular, vestibular aqueduct, and vestibular anomalies were detected in children with 22q11DS, especially in the setting of hearing loss. Careful evaluation of anatomic anomalies is needed prior to surgical intervention in these patients.

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Objective To evaluate the relationship between intraoperative neural response telemetry (NRT) and postoperative auditory testing outcomes in children. Study Design Retrospective study. Setting Tertiary‐care academic center. Methods Children who underwent cochlear implantation using the Cochlear Corporation device between 2010 and 2019 were included. Associations of average NRT and the slope of amplitude with postoperative auditory outcomes including functional auditory measure Infant‐Toddler Meaningful Auditory Integration Scale (IT‐MAIS), and speech perception testing (consonant‐nucleus‐consonant [CNC], Pediatric AzBio [BABY BIO], Hearing In Noise Test [HINT], and Northwestern University Children's Perception of Speech [NU‐CHIPS]), measured between 6 and 57 months after implantation, were assessed using Spearman's rank correlation (ρ). Results Thirty‐eight patients (19 female, 19 male) and 54 ears were included. The median age of implantation was 20.6 months (range 9.6 months to 10.6 years). Eight (21%) children had neurologic disorders such as stroke, epilepsy, cerebral palsy, and other causes. Thirteen (34%) children had connexin mutations. Average NRT was not significantly correlated with postoperative auditory outcomes (IT‐MAIS [ρ = −0.08, p = .74], CNC [ρ = 0.19, p = .32], BABY BIO [ρ = 0.21, p = .29], HINT [ρ = 0.05, p = .83]) and NU‐CHIPS (ρ = 0.21, p = .28). The average slopes of amplitude and comfort level were not strongly correlated with any auditory outcomes (p > .05). Conclusions Intraoperative NRT was not correlated with any postoperative functional auditory outcomes. Patient counseling should include discussions that a subpar intraoperative cochlear response does not preclude favorable speech and auditory outcomes.

ObjectiveIn 2013, the American Academy of Otolaryngology‐Head and Neck Surgery (AAO‐HNS) published guidelines for Bell's palsy (BP), including recommendations for workup, management, and specialist referral. Patients with BP often present to primary care; however, adherence to guidelines may vary by setting. This study sought to evaluate the management of patients with BP presenting to primary care, emergency department (ED), and urgent care settings.Study DesignRetrospective cohort study.SettingTertiary care center.MethodsRetrospective chart review of patients identified by diagnosis code for BP.ResultsA total of 903 patients were included; 687 (76.1%) presented to ED, 87 (9.6%) to internal medicine, 77 (8.5%) to family medicine, and 52 (5.8%) to urgent care. On presentation, 804 (89.0%) patients were prescribed corticosteroids and 592 (65.6%) antiviral therapy. Steroid therapy ranged from 1 dose to greater than a 14‐day course, with 177 (19.6%) receiving an adequate duration of 10 days or greater. Referrals were provided to facial plastics and/or otolaryngology for 51 patients (5.6%). For all comers, 283 (31.3%) had complete resolution, 197 (21.8%) had an incomplete resolution, 62 (6.9%) had persistent palsy, and 361 (40.0%) lost to follow‐up. In assessing the association between clinic setting and management, appropriate corticosteroid therapy (p < .01), imaging (p < .01), and eye care (p < .01) were statistically significant.ConclusionAdherence to guidelines for BP management varies amongst providers. In our study cohort, 15.5% of patients received medical therapy in accordance with AAO‐HNS guidelines, and only 5.6% were referred to facial plastics. To facilitate more appropriate care, tertiary care institutions may benefit from system‐wide care pathways to manage acute BP.

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