Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes

xlomafota13 shared this article with you from Inoreader Cochlear Nerve Canal Stenosis: Association With MYH14 and MYH9 Genes Via ear nose throat Ear Nose Throat J. 2021 Mar 8:145561321996839. doi: 10.1177/0145561321996839. Online ahead of print. ABSTRACT The bony cochlear nerve canal transmits the cochlear nerve as it passes from the fundus of the internal auditory canal to the cochlea. Stenosis of the cochlear nerve canal, defined as a diameter less than 1.0 mm in transverse diameter, is associated with inner ear anomalies and severe to profound congenital hearing loss. We describe an 11-month-old infant with nonsyn dromic congenital sensorineural hearing loss with cochlear nerve canal stenosis. Next-generation sequencing revealed heterozygous mutations in MYH9 and MYH14, encoding for the inner ear proteins myosin heavy chain IIA and IIC. The patient's hearing was rehabilitated with bilateral cochlear implantation. PMID:33683976 | DOI:10.1177/0145561321996839 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Solitary Fibrous Tumor of the External Auditory Canal

xlomafota13 shared this article with you from Inoreader Solitary Fibrous Tumor of the External Auditory Canal Via ear nose throat Ear Nose Throat J. 2021 Mar 8:145561321997539. doi: 10.1177/0145561321997539. Online ahead of print. ABSTRACT Solitary fibrous tumors (SFTs) originating from the external auditory canal are uncommon; only few cases have been reported in the literature. In this article, we report a case of a 35-year-old man who presented with a 6-month history of a gradual swelling in the entrance of the left external auditory meatus associated with hearing loss. The tumor was surgically remo ved, and histological examination showed spindle-cell proliferation with a collagenous stroma. Immunohistochemically, the tumor cells were positive for CD34 confirming the diagnosis of an SFT. Although SFTs are benign, complications such as relapses and metastasis after excision were reported. Thus, a careful and long follow-up is recommended. PMID:33683977 | DOI:10.1177/0145561321997539 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Use of Off-Label Nasal Steroid Irrigations in Long-Term Management of Chronic Rhinosinusitis

xlomafota13 shared this article with you from Inoreader Use of Off-Label Nasal Steroid Irrigations in Long-Term Management of Chronic Rhinosinusitis Via ear nose throat Ear Nose Throat J. 2021 Mar 8:145561321998521. doi: 10.1177/0145561321998521. Online ahead of print. ABSTRACT OBJECTIVE: Chronic rhinosinusitis (CRS) is an inflammatory disease of the paranasal sinuses and mucosa. Topical nasal corticosteroids are a mainstay treatment for CRS by reducing sinonasal inflammation and improving mucociliary clearance. However, topical corticosteroids have limited paranasal distribution, and patient response to treatment has been variable in rando mized controlled trials (RCT). Thus, there is significant interest in evaluating the efficacy of nasal steroids delivered by nasal irrigation in order to improve penetration and absorption of topical steroids into the sinonasal mucosa. In this review, we discuss the use of off-label nasal steroid irrigations in the management of CRS. METHODS: A review of clinical trials evaluating the use of nasal steroid irrigations for CRS in the PubMed electronic database was performed. RESULTS: Of the 12 clinical studies identified, 10 evaluated budesonide irrigations while the remaining 2 focused on mometasone. The overwhelming majority of studies for both budesonide and mometasone supported the use of nasal irrigations with corticosteroids over nasal corticosteroid sprays alone. However, the heterogeneity in study design, patient cohort, and volume of steroid irrigation limit the interpretations of these studies. CONCLUSIONS: Nasal irrigation with corticosteroids is beneficia l and safe for the treatment of CRS. Future RCTs controlling for type of surgical intervention, CRS pheno- and endo-type, as well as dosing and duration of nasal corticosteroid irrigations are warranted. PMID:33683979 | DOI:10.1177/0145561321998521 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Reconstruction of Anterior Mandibular Defect Using Submental Island Flap Pedicled With Mental Artery

xlomafota13 shared this article with you from Inoreader Reconstruction of Anterior Mandibular Defect Using Submental Island Flap Pedicled With Mental Artery Via ear nose throat Ear Nose Throat J. 2021 Mar 8:145561320987039. doi: 10.1177/0145561320987039. Online ahead of print. ABSTRACT Gingival carcinoma is a common malignant tumor occurring in the anterior area of the mandible, which can be derived from the epithelium of gingival mucosa. Surgical extended resection is the main treatment of gingival cancer, which can lead to anterior mandibular defect including mouth floor and mandible and mucosa of lower lip. According to the size of the defect, t he common repair method is free musculocutaneous flap with vascular pedicle or pedicle flap. We present a method of repairing mandibular anterior tooth defect with an island flap pedicled with the mental artery. PMID:33683978 | DOI:10.1177/0145561320987039 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Patients with non-idiopathic sudden sensorineural hearing loss show hearing improvement more often than patients with idiopathic sudden sensorineural hearing loss

xlomafota13 shared this article with you from Inoreader Patients with non-idiopathic sudden sensorineural hearing loss show hearing improvement more often than patients with idiopathic sudden sensorineural hearing loss Via http://link.springer.com/journal/405,European archives of oto-rhino-laryngology,The European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino Eur Arch Otorhinolaryngol. 2021 Mar 8. doi: 10.1007/s00405-021-06691-y. Online ahead of print. ABSTRACT INTRODUCTION: To compare inpatient treated patients with idiopathic (ISSNHL) and non-idiopathic sudden sensorineural hearing loss (NISSNHL) regarding frequency, hearing loss, treatment and outcome. METHODS: All 574 inpatient patients (51% male, median age: 60 years) with ISSNHL and NISSNHL, who were treated in federal state Thuringia in 2011 and 2012, were included retrospectively. Univariate and multivariate statistical analyses were performed. RESULTS: ISSNHL was diagnosed in 490 patients (85%), NISSNHL in 84 patients (15%). 49% of these cases had hearing loss due to acute otitis media, 37% through varicella-zoster infection or Lyme disease, 10% through Menière disease and 7% due to other reasons. Patients with ISSNHL and NISSNHL showed no difference between age, gender, side of hearing loss, presence of tinnitus or ve rtigo and their comorbidities. 45% of patients with ISSNHL and 62% with NISSNHL had an outpatient treatment prior to inpatient treatment (p < 0.001). The mean interval between onset of hearing loss to inpatient treatment was shorter in ISSNHL (7.7 days) than in NISSNHL (8.9 days; p = 0.02). The initial hearing loss of the three most affected frequencies in pure-tone average (3PTAmax) scaled 72.9 dBHL ± 31.3 dBHL in ISSNHL and 67.4 dBHL ± 30.5 dBHL in NISSNHL. In the case of acute otitis media, 3PTAmax (59.7 dBHL ± 24.6 dBHL) was lower than in the case of varicella-zoster infection or Lyme disease (80.11 dBHL ± 34.19 dBHL; p = 0.015). Mean absolute hearing gain (Δ3PTAmaxabs) was 8.1 dB ± 18.8 dB in patients with ISSNHL, and not different in NISSNHL patients with 10.2 dB ± 17.6 dB. A Δ3PTAmaxabs ≥ 10 dB was reached in 34.3% of the patients with ISSNHL and to a significantly higher rate of 48.8% in NISSNHL patients (p = 0.011). CONCLUSIONS: ISSN HL and NISSNHL show no relevant baseline differences. ISSNHL tends to have a higher initial hearing loss. NISSHNL shows a better outcome than ISSNHL. PMID:33683448 | DOI:10.1007/s00405-021-06691-y View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

The impact of cochlear implantation on health-related quality of life in older adults, measured with the Health Utilities Index Mark 2 and Mark 3

xlomafota13 shared this article with you from Inoreader The impact of cochlear implantation on health-related quality of life in older adults, measured with the Health Utilities Index Mark 2 and Mark 3 Via http://link.springer.com/journal/405,European archives of oto-rhino-laryngology,The European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino Eur Arch Otorhinolaryngol. 2021 Mar 8. doi: 10.1007/s00405-021-06727-3. Online ahead of print. ABSTRACT PURPOSE: To determine the usefulness of the Health Utilities Index (HUI) in older cochlear implant (CI) recipients, the primary aims were: (1) to assess health-related quality of life (HRQoL), measured with HUI, in older CI candidates while comparing with age- and gender-matched normal-hearing controls; (2) to compare HRQoL after CI with the pre-operative situation, using HUI and the Nijmegen cochlear implant questionnaire (NCIQ). The difference between pre- and postoperative speech intelligibility in noise (SPIN) and in quiet (SPIQ) and the influence of pre-operative vestibular function on HRQoL in CI users were also studied. METHODS: Twenty CI users aged 55 years and older with bilateral severe-to-profound postlingual sensorineural hearing loss and an age- and gender-matched normal-hearing control group were included. HRQoL w as assessed with HUI Mark 2 (HUI2), HUI Mark 3 (HUI3) and NCIQ. The CI recipients were evaluated pre-operatively and 12 months postoperatively. RESULTS: HUI3 Hearing (p = 0.02), SPIQ (p < 0.001), SPIN (p < 0.001) and NCIQ (p = 0.001) scores improved significantly comparing pre- and postoperative measurements in the CI group. No significant improvement was found comparing pre- and postoperative HUI3 Multi-Attribute scores (p = 0.07). The HUI3 Multi-Attribute score after CI remained significantly worse (p < 0.001) than those of the control group. Vestibular loss was significantly related to a decrease in HUI3 Multi-Attribute (p = 0.037) and HUI3 Emotion (p = 0.021) scores. CONCLUSION: The HUI is suitable to detect differences between normal-hearing controls and CI users, but might underestimate HRQoL changes after CI in CI users over 55. PMID:33683447 | DOI:10.1007/s00405-021-06727-3 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Primary Calvarial Ewing Sarcoma: A Case Series

xlomafota13 shared this article with you from Inoreader Primary Calvarial Ewing Sarcoma: A Case Series Via Journal of Neurological Surgery Part B: Skull Base J Neurol Surg B Skull Base DOI: 10.1055/s-0041-1722900 Background Calvarial Ewing tumor is a relatively rare differential among bony neoplasms. We present our experience of managing primary calvarial Ewing sarcoma (EWS), highlighting their clinical and radiological findings. Method In a retrospective analysis, we evaluated our 12-year database for pathologically proven EWS. A literature search was conducted for the comparative presentation and update on the management and outcome. Result From January 2008 to December 2020, we managed eight patients (male:female = 5:3; age range 6 months to 19 years, mean 11.5 years) harboring primary calvarial EWS. All cases underwent wide local excision; two patients required intradural tumor resection, while one required rotation flap for scalp reconstruction. Mean hospital stay was 8 days. All patients received adjuvant chemo- and radiotherapy. Three patients remained asymptomatic at 5 years of follow-up, while two patients died. Conclusion Primary calvarial EWS is a rare entity. It usually affects patients in the first two decades of life. These tumors can be purely intracranial, causing raised intracranial pressure symptoms, which may exhibit rapidly enlarging subgaleal tumors with only cosmetic deformities or symptoms of both. Radical excision followed by adjuvant therapy may offer a favorable long-term outcome. [...] Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany Article in Thieme eJournals: Table of contents  |  Abstract  |  Full text View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

A Unique Subset: Idiopathic Intracranial Hypertension Presenting as Spontaneous CSF Leak of the Anterior Skull Base

xlomafota13 shared this article with you from Inoreader A Unique Subset: Idiopathic Intracranial Hypertension Presenting as Spontaneous CSF Leak of the Anterior Skull Base Via Journal of Neurological Surgery Part B: Skull Base J Neurol Surg B Skull Base DOI: 10.1055/s-0040-1716898 Background Spontaneous cerebrospinal fluid (CSF) leaks represent a unique clinical presentation of idiopathic intracranial hypertension (IIH), lacking classical features of IIH, including severe headaches, papilledema, and markedly elevated opening pressures. Methods Following a single-institution retrospective review of patients undergoing spontaneous CSF leak repair, we performed a literature review of spontaneous CSF leak in patients previously undiagnosed with IIH, querying PubMed. Results Our literature review yielded 26 studies, comprising 716 patients. Average age was 51 years with 80.8% female predominance, and average body mass index was 35.5. Presenting symptoms included headaches (32.5%), visual disturbances (4.2%), and a history of meningitis (15.3%). Papilledema occurred in 14.1%. An empty sella was present in 77.7%. Slit ventricles and venous sinus stenosis comprised 7.7 and 31.8%, respectively. CSF leak most commonly originated from the sphenoid sinus (41.1%), cribriform plate (25.4%), and ethmoid skull base (20.4%). Preoperative opening pressures were normal at 22.4 cm H2O and elevated postoperatively to 30.8 cm H2O. 19.1% of patients underwent shunt placement. CSF leak recurred after repair in 10.5% of patients, 78.6% involving the initial site. A total of 85.7% of these patients were managed with repeat surgical intervention, and 23.2% underwent a shunting procedure. Conclusion Spontaneous CSF leaks represent a distinct variant of IIH, distinguished by decreased prevalence of headaches, lack of visual deficits, and normal opening pressures. Delayed measurement of opening pressure after leak repair may be helpful to diagnose IIH. Permanent CSF diversion may be indicated in patients exhibiting significantly elevated opening pressures postoperatively, refractory symptoms of IIH, or recurrent CSF leak. [...] Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany Article in Thieme eJournals: Table of contents  |  Abstract  |  Full text View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Novel Focal Therapeutic Hypothermia Device for Treatment of Acute Neurologic Injury: Large Animal Safety and Efficacy Trial

xlomafota13 shared this article with you from Inoreader Novel Focal Therapeutic Hypothermia Device for Treatment of Acute Neurologic Injury: Large Animal Safety and Efficacy Trial Via Journal of Neurological Surgery Part B: Skull Base J Neurol Surg B Skull Base DOI: 10.1055/s-0040-1721818 Objective Therapeutic hypothermia is a potentially powerful and controversial clinical tool for neuroprotection following acute neurologic pathology, particularly vascular injury. Indeed, therapeutic hypothermia remains a standard of care for postcardiac arrest ischemia and acute neonatal hypoxic-ischemic encephalopathy, improving both survival and outcomes. Although therapeutic hypothermia remains promising for cellular and systems-based neuronal protection in other neurologic injury states, the systemic side effects have limited clinical utility, confounded analysis of potential neurologic benefits, and precluded the completion of meaningful clinical trials. Methods To address such limitations, we developed and tested a novel, minimally invasive, neurocritical care device that employs continuous circulation of cold saline through the pharyngeal region to deliver focal cerebrovascular cooling. We conducted a preclinical safety and efficacy trial in six adult porcine animals to assess the validity and functionality of the NeuroSave device, and assess cooling potential following middle cerebral artery occlusion (n = 2). Results NeuroSave consistently lowered brain parenchymal temperature by a median of 9°C relative to core temperature within 60 minutes of initiation, including in ischemic cerebral parenchyma. The core body temperature experienced a maximal reduction of 2°C, or 5% of body temperature, with no associated adverse effects identified. Conclusion The present study uses a large animal preclinical model to demonstrate the safety and efficacy of a novel, noninvasive device for the induction of robust and systemically safe hypothermia within the brain. [...] Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany Article in Thieme eJournals: Table of contents  |  Abstract  |  Full text View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Vision Deterioration and Hydrocephalus: Rare Presentations of Vestibular Schwannoma and Evolution of Management

xlomafota13 shared this article with you from Inoreader Vision Deterioration and Hydrocephalus: Rare Presentations of Vestibular Schwannoma and Evolution of Management Via Journal of Neurological Surgery Part B: Skull Base J Neurol Surg B Skull Base DOI: 10.1055/s-0041-1725032 Introduction The incidence of vestibular schwannoma is reported as 12 to 54 new cases per million per year, increasing over time. These patients usually present with unilateral sensorineural hearing loss, tinnitus, or vertigo. Rarely, these patients present with symptoms of hydrocephalus or vision changes. Objective The study aimed to evaluate the surgical management of vestibular schwannoma at a single institution and to identify factors that may contribute to hydrocephalus, papilledema, and the need for pre-resection diversion of cerebrospinal fluid. Patients and Methods A retrospective review examining the data of 203 patients with vestibular schwannoma managed with surgical resection from May 2008 to May 2020. We stratified patients into five different groups to analyze: tumors with a diameter of ≥40 mm, clinical evidence of hydrocephalus, and of papilledema, and patients who underwent pre-resection cerebrospinal fluid (CSF) diversion. Results From May 2008 to May 2020, 203 patients were treated with surgical resection. Patients with tumors ≥40 mm were more likely to present with visual symptoms (p < 0.001). Presentation with hydrocephalus was associated with larger tumor size (p < 0.001) as well as concomitant visual symptoms and papilledema (p < 0.001). Patients with visual symptoms presented at a younger age (p = 0.002) and with larger tumors (p < 0.001). Conclusion This case series highlights the rare presentation of vision changes and hydrocephalus in patients with vestibular schwannoma. We recommend urgent CSF diversion for patients with visual symptoms and hydrocephalus, followed by definitive resection. Further, vision may still deteriorate even after CSF diversion and tumor resection. [...] Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany Article in Thieme eJournals: Table of contents  |  Abstra ct  |  Full text View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Relationship between Signal Intensity of the Labyrinth and Cochleovestibular Testing and Morphologic Features of Vestibular Schwannoma

xlomafota13 shared this article with you from Inoreader Relationship between Signal Intensity of the Labyrinth and Cochleovestibular Testing and Morphologic Features of Vestibular Schwannoma Via Journal of Neurological Surgery Part B: Skull Base J Neurol Surg B Skull Base DOI: 10.1055/s-0041-1725035 Objectives The aim of this article was to evaluate the relationship between signal intensity of the labyrinth and vestibulocochlear function and morphologic features of vestibular schwannoma (VS). Design Cross-sectional Study. Setting Tertiary referral center. Participants Fifty-four patients with sporadic, untreated VS. Main Outcome Measure Signal intensity of the cochlea and vestibule (SIRc and SIRv: signal intensity of cochlea/vestibule compared with cerebellar signal intensity; AURc and AURv: SIRc/SIRv of the affected side compared with the unaffected side) in 1.5T T2-weighted images was correlated with size (Hannover classification), cystic status, distance from the fundus of the internal auditory canal, video head impulse test (vHIT), and audiometry. Results Signal intensity of the vestibule was higher than that of the cochlea (p < 0.01). Large tumors had lower SIRc than smaller tumors (p = 0.03); Hannover T1 tumors had higher SIRc (p < 0.01), SIRv (p < 0.01), AURc (p < 0.01) and AURv (p < 0.01) than the rest; heterogenous and cystic tumors had higher SIRv than solid large tumors (p = 0.02); superior vestibular nerve pattern on vHIT had higher SIRv and AURv than inferior vestibular nerve and mixed patterns (p = 0.03 and 0.004, respectively); and there was a weak correlation between AURv and speech discrimination (r = 0.33, p = 0.04). Conclusion A more abnormal signal intensity of the labyrinth is associated with larger size and solid status of VS. There was a positive relationship between signal intensity of the labyrinth and speech discrimination scores on audiogram. [...] Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany Article in Thieme eJournals: Table of contents  |  Abstract  |  Full text View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.