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Objective : To evaluate short and long term results of stapes surgery in patients with Osteogenesis imperfecta (OI). Design: Retrospective case series. Setting: Tertiary referral center Patients: 18 primary stapes surgeries were performed in 11 hearing-impaired OI patients with evidence of stapes fixation. Intervention: Mainly CO2 laser stapedotomy with Teflon piston prosthesis. Main Outcome Measures: Pre-operative and post-operative audiograms at one month and at least one year Results: The main operative findings were stapes fixation, thickened footplate and fragile or fractured stapes crura. No revision surgery was necessary. Hearing improvement was achieved in 94% of the cases. We obtained an air-bone gap closure to within 10 dB in 46% of the cases and to within 15dB in 92% of the cases at one year follow up. The mean hearing gain in air conduction (at 0.5, 1, 2 and 4 kHz) was 18.4 dB at one month and 22.4 dB at one year. Conclusion: Stapes surgery in OI gives good results with few complication in our series. A hearing gain is often obtained in spite of the sensorineural hearing loss caused by the natural progression of the disease

Patients with superior semicircular canal dehiscence syndrome, which can only be treated by surgery, present cochleo-vestibular symptoms related to a third-mobile window but also endolymphatic hydrops. Since cVEMP and oVEMP are disturbed by the presence of the dehiscence, the aim of the study is to assess the value of MRI for the diagnosis of endolymphatic hydrops in patients with superior semicircular canal dehiscence syndrome in comparison with cVEMP and oVEMP. In this retrospective cohort study we enrolled 33 ears in 24 patients with superior semicircular dehiscence syndrome who underwent a 4-h delayed intravenous Gd-enhanced 3D-FLAIR MRI and pure tone audiometry, cVEMP and oVEMP. For each patient MRI images were evaluated by two radiologists who used a compartmental endolymphatic hydrops grading system in comparison with cVEMP and oVEMP. Endolymphatic hydrops was found on MRI in 9 out of 33 SCDS ears (27.3%). We found no significant correlation between the presence of endolymphatic hydrops on MRI and cVEMP and oVEMP (p = 0.36 and p = 0.7, respectively). However, there was a significant correlation between the presence of endolymphatic hydrops on MRI and the degree of sensorineural hearing loss, Air Conduction-Pure Tone Average level (p = 0.012) and Bone Conduction-Pure Tone Average level (p = 0.09), respectively. We demonstrated that EH might be observed in 27.3% of superior semicircular dehiscence syndrome ears. The role of inner ear MRI is important to detect endolymphatic hydrops, since cVEMP and oVEMP are disturbed by the presence of the dehiscence, because these patients could benefit from a medical treatment. Level 3.

PURPOSE The purpose of this study was to describe the MRI characteristics of intralabyrinthine schwannoma (ILS) on post contrast three-dimensional (3D) fluid-attenuation-inversion-recovery (FLAIR) images obtained four hours after intravenous administration of a gadolinium-based contrast agent (4h-3D-FLAIR). MATERIALS AND METHODS This IRB-approved retrospective multi-center study included patients presenting with typical ILS from January 2016 to October 2020. All medical charts were systematically collected. All MRI examinations, including 4h-3D-FLAIR images, were reviewed by two board-certified neuroradiologists. Main outcome measures were location, signal intensity and associated anomalies of ILS. RESULTS Twenty-seven out of 8730 patients (0.31%) referred for the investigation of a cochleovestibular disorder had a final diagnosis of ILS. There were 13 men and 14 women with a mean age of 52 ± 17 (SD) years (age range: 20-86 years). The most common clinical presentation was unilateral progressive sensorineural hearing loss (16/27; 59%). All ILS were unilateral and 15 (15/27; 55%) were intracochlear. All ILS presented as a hypointense filling defect within the labyrinth on T2-weighted images that enhanced on post-contrast T1-weighted images. On 4h-3D-FLAIR images, all ILS presented as a hypointense filling defect, associated with diffuse perilymphatic hyperintensity. Two patients (2/27; 7%) presented with ipsilateral endolymphatic hydrops. CONCLUSION ILS displays consistent features on post-contrast 4h-3D-FLAIR images. ILS should not be confused with endolymphatic hydrops and requires a systematic analysis of the corresponding T2-weighted images.

3D-FLAIR sequences with delayed acquisition after contrast medium injection have demonstrated new insights into blood-labyrinthine barrier (BLB) abnormalities in various diseases. The aim of this study was to assess the BLB in patients referred with unilateral acute vestibular syndrome (UAVS). In this retrospective multicenter imaging study, we performed 3D-FLAIR and steady-state free precession (SSFP) sequences 4 h after contrast medium administration in 26 healthy volunteers and in 30 patients with UAVS. Two radiologists, blinded to the clinical data, independently assessed the asymmetrical enhancement of the labyrinthine structures and the vestibular nerve on 3D-FLAIR sequences, and the signal of the labyrinthine structures on SSFP sequences. Inter-reader agreement tests were performed. An asymmetrical enhancement of the semicircular canals was observed in 26 out of 30 ears (86.6%, p

Purpose Due to the increased protein concentration in the perilymph liquid, FIESTA-C has recently been proposed to assess endolymphatic spaces without administration of contrast media in patients with vestibular schwannoma. The aim of this study was to assess whether vestibular endolymphatic space dilatation, as evaluated with 3D-FIESTA-C sequences, is related to the degree of hearing loss in patients with schwannoma. Materials and methods In this retrospective study (IRB 5995), we performed 3D-FIESTA-C sequences in 32 patients with schwannoma, as diagnosed on the basis of typical image findings. Two radiologists independently checked the visualization and evaluated the volume of the utricle and saccule. Pearson and Spearman tests were used to explore the correlation between the volume of the tumor, the volume of the vestibular endolymphatic space and the degree of hearing loss – as evaluated with the levels of pure-tone average (PTA) and speech recognition threshold (SRT). Results The endolymphatic space can be evaluated in 72% (23 out of 32 patients) of patients with schwannoma on non-contrast 3D-FIESTA-C sequences. The mean saccular, utricular and tumor volume were 3.17 mm3, 14.55 mm3 and 1.74 cm3 respectively. There was a significant correlation between the volume of the utricle and the degree of hearing loss as evaluated with the levels of PTA (p = 0.015) and SRT (p = 0.004). There was no significant correlation between the saccular and tumor volumes and the degree of hearing loss. Conclusion The volume of the utricle in schwannoma patients is related to the degree of hearing loss.

The need to treat an abdominal aortic aneurysm (AAA) in kidney transplanted patient is a rare event. To date, no method to protect the kidney during the aneurysm treatment has been identified as undeniably relevant. On the other hand, the advantage of endovascular treatment of the aneurysm (EVAR) is to avoid transplanted kidney injury. Unfortunately, EVAR is not always available leading to open repair and then aortic cross clamping. We report here 3 cases of AAA open repair in kidney transplanted patients using a temporary axillofemoral bypass to protect the renal function.