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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. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis: To describe outcomes of advanced head and neck cutaneous squamous cell carcinoma (cSCC) with clinical perineural invasion (cPNI) treated with immune checkpoint inhibitor (ICI) therapy, and to describe post‐treatment radiographic findings in the context of clinical response to treatment using a new grading system. Study Design: Retrospective chart review. Methods: Retrospective chart review was performed for 11 patients treated with ICI for head and neck cSCC with cPNI of large named nerves. The primary outcome was response to treatment as defined by radiographic and clinical evidence. Clinical responses were defined as improvement in symptoms of neuropathic pain, hypoesthesia, nerve weakness, or decrease in visible tumor. Imaging studies were graded based on a new classification system for perineural invasion and reviewed by two neuroradiologists since RECISTv1.1 is inadequate to adjudicate response in these patients. Results: Nine (82%) patients had radiographic perineural disease control on ICI. Eight patients had improved radiographic perineural disease and one had stable disease. Of these, complete resolution of radiographic evidence of perineural disease was seen in only one patient. Seven (64%) patients had clinical responses, with either improved or stable radiographic disease. Conclusions: ICI therapy is a viable treatment option for head and neck cSCC with cPNI. Radiographic and clinical evidence of response correlate well, with improvement in neuropathic pain being the most sensitive clinical marker of response. Even with favorable findings on repeat imaging and stable clinical course, complete resolution of perineural thickening and enhancement is rare. A grading system for classifying changes in perineural disease over time is proposed. Level of Evidence: 4 Laryngoscope, 132:1213–1218, 2022 [ABSTRACT FROM AUTHOR]

Objective: To characterize the prevalence, imaging characteristics, and cochlear implant candidacy of pediatric patients with single-sided deafness (SSD). Methods: An audiometric database of patients evaluated at a large tertiary academic medical center was retrospectively queried to identify pediatric patients (<18 years old) with SSD, defined as severe to profound sensorineural hearing loss in one ear and normal hearing in the other. Medical records of identified patients were reviewed to characterize the prevalence, etiology, and cochlear implant candidacy of pediatric patients with SSD. Results: We reviewed audiometric data obtained from 1993 to 2018 for 52,878 children at our institution. 191 (0.36%) had the diagnosis of SSD. Cochlear nerve deficiency (either hypoplasia or aplasia) diagnosed on MRI and/or CT was the most common etiology of SSD and was present in 22 of 88 (25%) pediatric SSD patients with available imaging data. 70 of 106 (66%) pediatric SSD patients with available imaging had anatomy amenable to cochlear implantation. Conclusions: Pediatric SSD is a rare condition and the most common etiology based on radiology is cochlear nerve deficiency. High resolution imaging of the temporal bone is essential to determine cochlear nerve morphology prior to consideration of cochlear implantation. [ABSTRACT FROM AUTHOR]

Objective  Transcanal endoscopic operative approaches provide for a minimally invasive surgical portal to the lateral skull base. Traditional preoperative imaging evaluation involves computed tomography (CT) acquisition in the axial and coronal planes that are not optimized for the transcanal surgical corridor. Herein, we describe a novel CT-based "transcanal view" for preoperative surgical planning and intraoperative navigation. Study Design  Present study is a cadaveric imaging study. Methods  Cadaveric temporal bones (n  = 6) from three specimens underwent high-resolution CT (0.625 mm slice thickness). Using three-dimensional (3D) Slicer 4.8, reformatted "transcanal" views in the plane of the external auditory canal (EAC) were created. Axial and coronal reformats were used to compare and measure distances between anatomic structures in the plane of the EAC. Results  The degree of oblique tilt for transcanal CT reformats was 6.67 ± 1.78 degrees to align the EAC in axial and coronal planes. Anticipated critical landmarks were identified easily using the transcanal view. Mean values were 8.68 ± 0.38 mm for annulus diameter, 9.5 ± 0.93 mm for isthmus diameter, 10.27 ± 0.73 mm for distance between annulus and isthmus, 2.95 ± 0.13 mm for distance between annulus and stapes capitulum, 5.12 ± 0.35 mm for distance between annulus and mastoid facial nerve, and 19.54 ± 1.22 mm for EAC length. Conclusion  This study is the first to illustrate a novel "transcanal" CT sequence intended for endoscopic lateral skull base surgery. Future studies may address how incorporation of a transcanal CT reformat may influence surgical decision making. [ABSTRACT FROM AUTHOR]