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DATA SYNTHESIS Pooled estimates of preserved Class A/B, I/II hearing at last postoperative follow-up. CONCLUSIONS Of 1323 reports, 14 were utilized in analyses yielding data from 2,977 patients. Mean follow-up was 52.5 months (SD = 19.9). Class A/B, 1/2 hearing was preserved at last follow-up in 57% of patients. Meta-regression revealed that resection through the middle cranial fossa was associated with preservation of serviceable hearing. Moreover, when preserved in the immediate postoperative period, it seems to be stable over time.HYPOTHESIS Endolymphatic hydrops (EH) associated with cochlear implantation are associated with vestibular dysfunction. BACKGROUND Vestibular dysfunction is a known risk after cochlear implantation (CI). CI has been shown to cause cochlear hydrops due to fibrosis surrounding the ductus reuniens. However, the association of cochlear hydrops with vestibular hydrops and the relationship to vestibular symptoms remain unknown. METHODS Histopathological analysis and clinical evaluation of the vestibular end organs of 17 human temporal bones (HTB)s exhibiting cochlear hydrops from 15 CI recipients. RESULTS Eight of 15 patients with cochlear hydrops due to CI had complaints of dizziness, vertigo, or imbalance following CI. In all 17 HTBs with cochlear hydrops, there was fibrosis, atrophy, or obstruction of the ductus reuniens, and all had straight electrode CI via cochleostomy. In one of the eight reporting postoperative dizziness, labyrinthitis ossificans was deemed causative. Six of the seven remaining patients had EH of both the saccule and utricle. Fifteen of 17 HTBs (88.2%) had saccular EH. In contrast, 8 of 17 HTBs (47.0%) in 7 patients had utricular EH, of which 6 patients had postoperative vertigo spells. It seems that hydrops of the utricle closely corresponds to postoperative vertigo spells and vestibular complaints. CONCLUSION Implantation of the CI, when complicated by ductus reuniens fibrosis, may cause both cochlear hydrops and vestibular endolymphatic hydrops. Hydrops of the vestibular periphery when involving the utricle seems to be more likely associated with disabling vertigo symptoms. This study supports the round window technique of insertion rather than cochleostomy.OBJECTIVES To investigate false-positive findings on non-echoplanar (non-EPI) diffusion-weighted magnetic resonance imaging (DWI) in patients under surveillance post-cholesteatoma surgery. STUDY DESIGN, SETTING, SUBJECTS, AND METHODS A retrospective review was performed on patients diagnosed with cholesteatoma who underwent surgical resection and were then followed by serial non-EPI DWI using half-Fourier acquisition single-shot turbo spin echo (HASTE) sequence. All patients had at least two annual follow-up imaging studies. RESULTS False-positive findings were identified in four patients. The size of the suspected lesions was 4 to 12 mm. Otoendoscopy was used during all primary cases and Argon laser was used in one case. In all cases, the entire cholesteatoma was removed, and no residual disease was detected at the end of the procedures. Nrf2 activator One patient underwent revision surgery but only cartilage graft was found in the area of concern. All patients had stable or resolved hyperintense areas in the subsequent HASTE sequences. CONCLUSION False positive findings can occur with non-EPI DWI MRI and patients need to be counseled accordingly before revision surgery. Decreasing intensity and dimension of a suspected lesion and a positive finding in an area other than the location of the initial cholesteatoma may favor a false positive. If a false positive finding is suspected when the surgeon is confident of complete resection of the cholesteatoma, an MRI can be repeated in 6 to 12 months to assess changes in the dimension and intensity of the area of concern. Cartilage grafts may cause restricted diffusion on DWI sequences.OBJECTIVES In this review, we discuss current knowledge about the genetics and epigenetics of vestibular schwannoma (VS) in relation to hearing loss. A multistep and sequential genetic algorithm suitable for the identification of Neurofibromatosis Type 2 (NF2) constitutional and somatic mutations is discussed. DATA SOURCES, STUDY SELECTION A review was performed of the English literature from 1990 to 2019 using PubMed regarding genetics and epigenetics of vestibular schwannoma and NF2. CONCLUSION NF2 is a genetic disorder characterized by NF2 mutations that affect the function of a tumor suppressor called merlin. In particular, individuals with NF2 develop bilateral VS that can lead to hearing loss and even deafness. Recent advances in genetic and epigenetic studies have improved our understanding of the genotype-phenotype relationships that affect hearing in NF2 patients. Specific constitutional NF2 mutations including particular truncating, deletion, and missense mutations have been associated with poorer hearing outcomes and more severe clinical manifestations. Epigenetic events, such as DNA methylation and histone modifications, also contribute to the development and progression of hearing loss in NF2 patients. Furthermore, the accumulation of multiple NF2 and non-NF2 genetic and epigenetic abnormalities at the level of the tumor may contribute to worse hearing outcomes. Understanding genetic and epigenetic signatures in individual NF2 patients and particularly in each VS will allow us to develop novel gene therapies and precision medicine algorithms to preserve hearing in NF2 individuals.OBJECTIVE To investigate the impact of configurations of the vertebrobasilar system on the incidence of idiopathic sudden sensorineural hearing loss (ISSNHL) and canal paresis (CP). STUDY DESIGN Retrospective case review. SETTING Tertiary referral center. PATIENTS Two hundred and forty-eight consecutive patients diagnosed with ISSNHL and 152 patients with unilateral CP of an uncertain cause who were managed between January 2011 and December 2017. The contralateral side of 144 patients with Bell's palsy or cerebellopontine angle tumor served as a control. INTERVENTIONS All patients underwent magnetic resonance cisternography. CP was diagnosed based on caloric testing. MAIN OUTCOME MEASURES 1) Branching patterns of the anterior/posterior inferior cerebellar artery (AICA/PICA) in the cerebellopontine angle area. 2) The direction of the basilar artery (BA) curvature. 3) Vertebral artery (VA) dominance. RESULTS The incidence of vascular loops of the AICA/PICA entering the internal acoustic canal was significantly higher on both the affected and unaffected sides in patients with ISSNHL and CP in comparison to controls (p  less then  0.

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