Wilhelm Flatz

and 6 more

Objectives: Several studies proposed a loss of neural structures in Menière’s disease (MD). It has also been shown that VIIth and VIIIth cranial nerves are enlarged within MD patients compared to normal controls. We aimed to investigate potential differences in these two nerves in patients with MD. Setting: 71 patients with MD were included, 53 showed clinically unilateral affection. Constructive-interference-in-steady-state(CISS) and 3D-FLAIR-inversion-recovery(IR) sequences with two different slice thicknesses were acquired on a clinical 3T magnetic resonance imaging scanner. We evaluated morphometric properties of the VIIth and VIIIth cranial nerves passing from the cerebellopontine angle to the inner ear modiolus. In patients with clinically unilateral MD, we compared the unaffected to the clinically affected side. In addition, we evaluated the morphology of the nerves in correlation to symptom duration. Results: The clinically unilateral MD-patients showed no significant differences after Bonferroni correction when comparing the affected side to the non-affected side of VIIth and VIIIth cranial nerves. There was no significant difference between patients with different symptom durations. Conclusions: Our data showed no differences in nerve morphometry between the clinically non-affected and the clinically affected side in patients with clinically unilateral MD. There was also no correlation to duration of symptoms, in contrast to previously demonstrated correlations between clinical features and the extent of endolymphatic hydrops. A disease process starting before onset of clinical symptoms could be a potential explanation.

Richard Brill

and 25 more

Purpose: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor in children, which can be accompanied by life-threatening thrombocytopenia, referred to as Kasabach-Merritt Phenomenon (KMP). The mTOR inhibitor sirolimus is emerging as targeted therapy in KHE. As the sirolimus effect on KHE occurs only after several weeks we aimed to evaluate if additional transarterial embolization is of benefit for children with KHE and KMP. Methods: 17 patients with KHE and KMP acquired from 11 hospitals in Germany were retrospectively divided into two cohorts. Children being treated with adjunct transarterial embolization and systemic sirolimus, and those being treated with sirolimus without additional embolization. Bleeding rate as defined by WHO was determined for all patients. Response of the primary tumor at 6 and 12 months assessed by Magnetic Resonance Imaging (MRI), time to response of KMP defined as thrombocyte increase >150 x 103/µl, as well as rebound rates of both after cessation of sirolimus were compared. Results: N= 8 patients had undergone additive embolization to systemic sirolimus therapy, sirolimus in this group was started after a mean of 6.5 ± 3 days following embolization. N=9 patients were identified who had received sirolimus without additional embolization. Adjunct embolization induced a more rapid resolution of KMP within a median of 7 days vs 3 months, however tumor response as well as rebound rates were similar between both groups. Conclusion: Additive embolization may be of value for a more rapid rescue of consumptive coagulopathy in children with KHE and KMP compared to systemic sirolimus only.