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Cancer transformation regarding neurofibromatosis-1 directly into low-grade malignant peripheral lack of feeling sheath tumor: an instance document.
OBJECTIVE To conduct a systematic review of posterior semicircular canal dehiscence (PSCD) and to present a series of patients with PSCD with and without classic third-window symptoms. DATA SOURCES PubMed, Scopus, and the Cochrane Library from inception until April 2019. Case series of five patients seen in a multidisciplinary, vestibular-focused, neurotology clinic. STUDY SELECTION Inclusion criteria PSCD studies of symptomatology, diagnostic testing, radiology, and histopathology. EXCLUSION CRITERIA non-English articles, reviews, letters, animal studies. DATA EXTRACTION Quality evaluated according to Oxford Center for Evidence-Based Medicine criteria and funnel plot via the Stern and Egger method. DATA SYNTHESIS Two hundred five studies were found, and 58 studies were included. In 47 total patients, sound-induced vertigo, mixed hearing loss, and tinnitus were the most common presenting symptom. A meta-analysis of proportions using eight radiological and histopathological studies revealed an incidence of 0.38% adult ears [95% CI 0.08, 0.89] and 2.16% of adult patients [0.64, 4.54]. The incidence in pediatric patients ranged from 1.3 to 43%. Jugular bulb abnormalities were common. Compstatin Complement System inhibitor In our case series, four of five patients presented without third-window symptoms, while one had sound- and pressure-induced vertigo. Hearing loss in these patients was not salvageable. CONCLUSIONS PSCD is a rare phenomenon most commonly presenting with third-window type symptoms. However, PSCD might also present with dizziness and hearing loss inconsistent with third-window symptomatology. One should be conscious of potentially poorer prognosis for hearing recovery in these patients.OBJECTIVE To evaluate the efficacy of on demand and low dose intratympanic gentamicin (ITG) in patients with intractable Meniere's disease (MD). STUDY DESIGN Clinical chart review. SETTING Secondary care center. PATIENTS Subjects with MD who failed conventional treatment and underwent on demand ITG infiltration from June 2013 to December 2018. INTERVENTION 0.4 to 0.5 ml of buffered gentamicin were administered through an intratympanic route. A total of 5 mg in case of low dose and 20 mg as a standard dose. MAIN OUTCOME MEASURES Vertigo control, Meniere's Disease Functional Level Scale (MDFLS), Dizziness Handicap Inventory (DHI), and pure tone audiometry pre and posttreatment. RESULTS Thirty-one patients, 16 women and 15 men with a mean age of 52.81 (22-79) years were included. The number of ITG injections ranged from 1 to 7, with a mean of 2.52 applications per patient. Mean interval between doses was 212.15 (21-1442) days. Average follow-up was 24.03 months. An improvement on MDFLS was seen on 77.4% (n = 24) patients. DHI score improved after gentamicin treatment (mean 55.23 versus 24.06, p ≤ 0.001). Thirty patients (96.8%) reached complete or substantial vertigo control. Only one patient did not achieve control. Hearing was preserved in 43.5% (n = 10) of analyzed audiograms, whereas 17.4% (n = 4) developed hearing loss greater than 20 dB, which was not statistically significant (p = 0.099). CONCLUSIONS In our study, on demand and low dose ITG was effective for vertigo control in patients with intractable MD. Individualized therapy is recommended in all patients to minimize vestibular and cochlear toxicity.OBJECTIVE The cochlear nucleus (CN) is the target of the auditory brainstem implant (ABI). Most ABI candidates have Neurofibromatosis Type 2 (NF2) and distorted brainstem anatomy from bilateral vestibular schwannomas. The CN is difficult to characterize as routine structural MRI does not resolve detailed anatomy. We hypothesize that diffusion tensor imaging (DTI) enables both in vivo localization and quantitative measurements of CN morphology. STUDY DESIGN We analyzed 7 Tesla (T) DTI images of 100 subjects (200 CN) and relevant anatomic structures using an MRI brainstem atlas with submillimetric (50 μm) resolution. SETTING Tertiary referral center. PATIENTS Young healthy normal hearing adults. INTERVENTION Diagnostic. MAIN OUTCOME MEASURES Diffusion scalar measures such as fractional anisotropy (FA), mean diffusivity (MD), mode of anisotropy (Mode), principal eigenvectors of the CN, and the adjacent inferior cerebellar peduncle (ICP). RESULTS The CN had a lamellar structure and ventral-dorsal fiber orientation and could be localized lateral to the inferior cerebellar peduncle (ICP). This fiber orientation was orthogonal to tracts of the adjacent ICP where the fibers run mainly caudal-rostrally. The CN had lower FA compared to the medial aspect of the ICP (0.44 ± 0.09 vs. 0.64 ± 0.08, p  less then  0.001). CONCLUSIONS 7T DTI enables characterization of human CN morphology and neuronal substructure. An ABI array insertion vector directed more caudally would better correspond to the main fiber axis of CN. State-of-the-art DTI has implications for ABI preoperative planning and future image guidance-assisted placement of the electrode array.OBJECTIVE Validation of the preoperative sound simulation test for vibroplasty-hearing so-called "Direct Drive Simulation" (DDS) in case of mixed hearing loss. STUDY DESIGN Retrospective data analysis. SETTING Tertiary referral center with a large hearing implant program. MAIN OUTCOME MEASURE Comparison of sound impression during preoperative Direct Drive Simulation, and postoperative testing with the activated active middle ear implant (AMEI) under free-field condition and in daily routine. PATIENTS Fifty-four data sets from 18 patients with mixed hearing loss with a mean age of 60.78 ± 3.18 were included. RESULTS Comparing the sound impression during DDS preoperatively versus free-field testing with the implanted AMEI, no significant differences were found. DDS offers a slightly better sound quality than the AMEI in daily routine, fitting well to the ideal listening situation in DDS versus some background noise in daily routine. CONCLUSION The DDS offers the possibility of a realistic preoperative sound simulation of the "vibroplasty-hearing" in cases of mixed hearing loss. This probably facilitates patient's decision towards a vibroplasty. The audiologist as well as the surgeon get additional information regarding the indication especially when audiologic inclusion criteria are critical. Thus, the DDS is a useful extension of preoperative diagnostics before vibroplasty.
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