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Vestibular paroxysmia (VP) is a disorder encountered in the pediatric population that etiology has been attributed to neurovascular cross-compression syndrome (NVCC). The purpose of this study was to report a new probable pathological condition, the narrowed internal auditory canal (IAC), which appears to be involved in the development of a clinical picture of VP in the pediatric population. A retrospective descriptive comparative study was conducted to compare clinical, electrophysiological, radiological, and therapeutic outcomes in both etiologies. Overall, 16 pediatric patients suffering from VP were included and divided into two groups: patients with narrowed internal auditory (Group 1) were compared to those with NVCC syndrome (Group 2). Patients in both groups were similar in terms of auditory complaints, as well as hearing, vestibular, and electrophysiological status. A narrowed IAC was encountered in the adolescent age category and females, especially those with rapid growth. The diagnosis requires a careful analysis of the shape and diameters of the IAC. Radiologic measurements in the axial plane do not seem to be sufficient to confirm the diagnosis, and, therefore, an analysis of diameters in the coronal plane is required. Treatment with sodium-channel blockers drugs showed promising results not only by relieving vertigo but also by normalizing the electrophysiological findings. In conclusion, a narrowed IAC can be considered in patients suffering from VP.

Objectives The aims of this study were to evaluate the agreement between cone-beam computed tomography (CBCT) and multislice computed tomography for the characterization of extremity fractures and to compare image quality, radiation dose, and patient tolerance. Methods Thirty-six patients with suspected fracture affecting distal extremities or who required preoperative fracture assessment were enrolled prospectively. Each patient underwent CBCT and multislice computed tomography the same day. Both examinations were evaluated independently twice by 2 trained radiologists using the Muller AO classification for fracture characterization. Results Cohen κ coefficient for agreement between the imaging techniques was almost perfect for fracture characterization, κ = 0.94 [95% confidence interval, 0.91-0.98]. There was substantial to almost perfect agreement for secondary findings. Cone-beam computed tomography was well tolerated and significantly less irradiant and had better subjective image. Conclusions An excellent agreement between both imaging techniques was found. This confirms the ability of CBCT to assess fractures and its potential in the management of patients with distal limb trauma.

Objectives: To determine whether temporal bone computed tomography (CT) features are linked to the presence and type of hearing loss in osteogenesis imperfecta (OI) when considering hearing-impaired OI patients and normally hearing (NH) OI ones. A secondary objective was to assess whether other factors influence CT features in a large sample: age, type of mutation, or bone mineral density (BMD). Methods: A total of 41 adults with OI underwent CTs and pure-tone audiometry in 82 ears. Hearing thresholds were normal in 64 out of 82 ears, and most had not been operated on for stapedectomy or stapedotomy. Ossicle density, footplates, oval and round windows, retrofenestral peri- and endolabyrinths, and temporal pneumatization were analyzed twice by an experienced radiologist. CT features were compared to hearing, age, collagen mutations, and bone mineral density. Results: Unexpectedly a high prevalence of footplate, ossicle, and otic capsule anomalies was observed, even in NH ears. Footplate hypodensity or thickening was mostly found in ears without conductive hearing loss. There were significantly more retrofenestral anomalies or window obstruction in ears with a sensorineural hearing loss component than in ears without. Age was significantly higher in ears with middle layer hypodensity than in ears without. Patients with mutations were expected to have reduced collagen quantity and had significantly more footplate or retrofenestral anomalies than those with qualitative mutations. BMD was significantly higher in ears without temporal hyperpneumatization. Conclusion: Temporal bone CT features in OI are present in a large proportion of patients, had they hearing loss or not, and might be determined more by collagen mutation type than by age or BMD.

To evaluate the diagnostic concordance between multislice computed tomography (MSCT) and cone beam computed tomography (CBCT) in the early postoperative assessment of patients after cochlear implantation.Prospective, randomized, single-center, interventional, pilot study on the diagnostic performance of a medical device.Tertiary referral center.Patients aged over 18 years requiring a computed tomographic (CT) scan after cochlear implant surgery.Nine patients were implanted with electrode arrays from three different manufacturers, including one bilateral. High-resolution MSCT and CBCT were then performed, and two experienced radiologists blinded to the imaging modality evaluated the randomized images, twice.Concordance between MSCT and CBCT for assessing the scalar position (tympani or vestibuli) of the electrodes. Secondary outcome measures were also studied: length of the intracochlear electrode array, percentage of implanted cochlea, number of intracochlear electrodes, and radiation doses.There was a good agreement between both CT scanners in determining the scalar position and estimating the number of implanted electrodes and percentage of implanted cochlea. CBCT had a lower radiation exposure.The CBCT appears to be a useful tool for postoperative assessment of cochlear implanted adult patients and is comparable to the conventional scanner in determining the scalar position, with lower radiation exposure.

Introduction Camurati-Engelmann disease is a rare autosomal dominant bone dysplasia belonging to the group of craniotubular hyperostoses. Genetic analysis classically shows mutation on TGFβ1 gene. Case report A young woman was hospitalized with intense pain in lower limbs, associated to radiographic hyperostosis and sclerosis of the long bones. Results Mutation on LRP6 has recently been associated to high bone mass. In this case report, a rare missense variant on LRP6 gene was associated to radiographic features of Camurati-Engelmann. Conclusions More studies should be conducted to assess the pathological role of this variant in Camurati-Engelmann-like disease.

To determine whether the diagnostic value of cone-beam computed tomography (CBCT) is equivalent or superior to that of multi-detector computed tomography (MDCT) for the radiological assessment of conductive hearing loss with intact tympanic membrane in adults. Evaluation of inter- and intraobserver variability and measurement of the radiation dosimetry associated with each technique were secondary objectives. Prospective, single-center study. Ten adults were included from April to June 2013. All patients underwent MDCT and CBCT with reconstruction of temporal bones. Two radiologists with ENT experience reviewed the results twice. Diagnostic agreement between MDCT and CBCT and inter- and intraobserver agreement was evaluated with the kappa statistic. Comparisons of dosimetry were evaluated by calculating the ratio of the CT dose index (CTDI) between MDCT and CBCT. Diagnostic agreement between MDCT and CBCT was satisfactory (kappa = 0.69). Inter- and intraobserver agreement was also acceptable, and the average ratio of the CTDI of MDCT and CBCT was 4.01. CBCT is a reliable method that uses a low dose of radiation to investigate conductive hearing loss with intact tympanic membrane in adults. Its relevance and potential superiority to MDCT in diagnosing middle ear pathologies such as otosclerosis remain to be demonstrated, but the preliminary data are promising.

European Annals of Otorhinolaryngology, Head and Neck Diseases - Vol. 135 - N° 2 - p. 149