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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.

ObjectiveTo evaluate the reporting and rates of loss to follow‐up (LTFU) in head and neck cancer (HNC) randomized controlled trials based in the United States.Data SourcesPubmed/MEDLINE, Cochrane, Scopus databases.Review MethodsA systematic review of titles in Pubmed/MEDLINE, Scopus, and Cochrane Library was performed. Inclusion criteria were US‐based randomized controlled trials focused on the diagnosis, treatment, or prevention of HNC. Retrospective analyses and pilot studies were excluded. The mean age, patients randomized, publication details, trial sites, funding, and LTFU data were recorded. Reporting of participants through each stage of the trial was documented. Binary logistic regression was performed to evaluate associations between study characteristics and reporting LTFU.ResultsA total of 3255 titles were reviewed. Of these, 128 studies met the inclusion criteria for analysis. A total of 22,016 patients were randomized. The mean age of participants was 58.6 years. Overall, 35 studies (27.3%) reported LTFU, and the mean LTFU rate was 4.37%. With the exception of 2 statistical outliers, study characteristics including publication year, number of trial sites, journal discipline, funding source, and intervention type did not predict the odds of reporting LTFU. Compared to 95% of trials reporting participants at eligibility and 100% reporting randomization, only 47% and 57% reported on withdrawal and details of the analysis, respectively.ConclusionThe majority of clinical trials in HNC in the United States do not report LTFU, which inhibits the evaluation of attrition bias that may impact the interpretation of significant findings. Standardized reporting is needed to evaluate the generalizability of trial results to clinical practice.

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