The emerging role of hearing loss rehabilitation in patients with vestibular schwannoma treated with Gamma Knife radiosurgery: literature review

AbstractStereotactic radiosurgery (SRS) is currently the most common treatment for small- to medium-size vestibular schwannoma (VS). Despite favorable outcome, hearing deterioration still remains an underestimated problem, and the role of hearing rehabilitation is an underinvestigated topic. Among available technologies, cochlear implant (CI) should represent a valid alternative in sporadic VS with single-sided deafness and in neurofibromatosis (NF2) with bilateral profound hearing loss. A literature review of the current clinical data was performed searching scientific literature databases. From all of the articles found, 16 papers were selected. Forty-four subjects treated with radiosurgery (18 male, 19 female, and in 7 cases, sex were not specified; 43 NF2 and 1 sporadic VS) were included in the analysis. Epidemiological, clinical, tumor, treatment, and audiological data were collected. Clinical outcome at last follow-up showed an audiological improvement in 25 of the 44 patients. The audiological outcome was unchanged in 16 cases. Audiological deterioration was recorded in 3 cases. Severity of NF2 phenotype, long history of ipsilateral profound deafness before implantation, progressive tumor growth, and high radiation dose (20 and 40  Gy) were found in patients with a worst audiological outcome. Hearing rehabilitation can improve audiological results for VS patients following SRS in selected cases. Hearing rehabilitation with cochlear implant (CI) in SSD leads to par...
Source: Neurosurgical Review - Category: Neurosurgery Source Type: research

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Authors: Stević M, Vlajković M, Veličković F, Stanojević G, Nestorović M, Petrović F Abstract A 69 year old patient was admitted to hospital with massive gastrointestinal hemorrhage. The clinical presentation of the patient, except for bleeding, was dominated by the presence of neurofibromatosis type 1 - Von Recklinghausen disease. The patient was referred to multislice computed tomography (CT) angiography, magnetic resonance imaging (MRI), esophagogastroduodenoscopy and colonoscopy, which were performed without successful detection of the bleeding site. The MRI examination showed the existence of a tumor lo...
Source: Hellenic Journal of Nuclear Medicine - Category: Nuclear Medicine Tags: Hell J Nucl Med Source Type: research
Most heart failure patients undergoing ventricular assist device (VAD) implantation are managed with guideline-based therapy including the implantation of defibrillation capable device (ICD). Prior to surgery, programming allowing therapy with lower threshold could be lifesaving. After surgery, the defibrillator should be reprogrammed to protect the patient against painful, unnecessary shocks, as the circulation will be supported even in ventricular fibrillation. Difficulties in interrogation of ICD in patients with both HeartMate-II and HeartMate3 devices have been previously reported, related to the VAD-produced electrom...
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: (1016) Source Type: research
AbstractBackgroundThe incidence of malignant peripheral nerve sheath tumors (MPNSTs) is low in the general population, although individuals with Neurofibromatosis Type I (NF1) are particularly susceptible. These tumors generally have a high probability of metastasis. The rate and types of second malignancies (SMNs) after a primary diagnosis of MPNST are not well characterized. We aimed to quantify the rate of SMNs among individuals with a first primary MPNST using population-based data.MethodsWe estimated age-standardized incidence rates (SIRs) for SMNs among 1,579 primary MPNST cases between ages 0 –85+ using SEER 1...
Source: Journal of Neuro-Oncology - Category: Cancer & Oncology Source Type: research
Conclusion: STP is an extremely favorable surgical solution for a number of middle-ear pathologies that have previously caused major problems. The main reason for its growing popularity is that it can be combined with a number of modern, effective methods of hearing rehabilitation. Orv Hetil. 2020; 161(14): 544-553. PMID: 32223420 [PubMed - in process]
Source: Orvosi Hetilap - Category: General Medicine Authors: Tags: Orv Hetil Source Type: research
As the COVID-19 pandemic continues to affect the country, most everyone has had to adapt in some way to accommodate this strange new normal—audiologists and hearing researchers included. With calls and procedures for self-quarantine and guidance for small businesses and universities varying from state to state, hearing care professionals may find themselves thrust into new situations. Some are being asked to adopt telepractice for the foreseeable future, and others are quickly switching to virtual-only learning for their audiology students. ASHA is constantly monitoring the situation as it evolves daily. For more inf...
Source: American Speech-Language-Hearing Association (ASHA) Press Releases - Category: Speech-Language Pathology Authors: Tags: Academia & Research Audiology Health Care News Private Practice Slider audiologist COVID-19 Telepractice Source Type: blogs
Publication date: Available online 29 March 2020Source: Integrative Medicine ResearchAuthor(s): Bong Hyun Kim, Young Kyun Moon, Min Hee Kim, Hae Jeong Nam
Source: Integrative Medicine Research - Category: Complementary Medicine Source Type: research
We describe a 10-year-old boy, the third son of first-cousin parents, with global developmental delay, facial dysmorphism, and bilateral deafness. SNP-array analysis revealed regions of homozygosity (ROHs) in multiple chromosome regions. Whole-exome sequencing prioritized on gene-mapping into the ROHs showed homozygosity for the likely pathogenic c.1097_1098delAG p. (Arg366Thrfs*2) frameshift substitution in LARP7 and the likely pathogenic c.5743C>T p.(Arg1915*) nonsense variant in OTOG. Recessive variants in LARP7 cause Alazami syndrome, while variants in OTOG cause an extremely rare autosomal recessive form of neu...
Source: Genes - Category: Genetics & Stem Cells Authors: Tags: Article Source Type: research
This study, for the first time, shows that transplantation of non-autologous mitochondria from healthy skeletal muscle cells into normal cardiomyocytes leads to short-term improvement of bioenergetics indicating "supercharged" state. However, over time these improved effects disappear, which suggests transplantation of mitochondria may have a potential application in settings where there is an acute stress. Outlining Some of the Science Behind Partial Reprogramming at Tur...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
This report describes a rare presentation of perineal RMS associated with Neurofibromatosis Type 1 (NF1).
Source: Journal of Pediatric and Adolescent Gynecology - Category: OBGYN Authors: Source Type: research
Conclusions: We have reported three new cases with pathogenic variants in PEX6 presenting with milder forms of the Zellweger spectrum disorders (ZSD). The three cases showed distinct clinical features. Thus, expanding the phenotypic spectrum of PBDs and ascertaining exome sequencing is an effective strategy for an accurate diagnosis of clinically overlapping and genetically heterogeneous disorders such as deafness-blindness association. PMID: 32214787 [PubMed - in process]
Source: Molecular Vision - Category: Molecular Biology Tags: Mol Vis Source Type: research
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