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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