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1.
J Clin Med ; 12(18)2023 Sep 13.
Artigo em Inglês | MEDLINE | ID: mdl-37762888

RESUMO

This study delves into the absence of prognostic or predictive markers to guide rehabilitation in patients afflicted with vestibular schwannomas. The objective is to analyze the reweighting of subjective and instrumental indicators following surgery, at 7 days and 1 month postoperatively. This retrospective cohort encompasses 32 patients who underwent unilateral vestibular schwannoma surgery at the Marseille University Hospital between 2014 and 2019. Variations in 54 indicators and their adherence to available norms are calculated. After 1 month, one-third of patients do not regain the norm for all indicators. However, the rates of variation unveil specific responses linked to a preoperative error signal, stemming from years of tumor adaptation. This adaptation is reflected in a postoperative visual or proprioceptive preference for certain patients. Further studies are needed to clarify error signals according to lesion types. The approach based on variations in normative indicators appears relevant for post-surgical monitoring and physiotherapy.

2.
Otolaryngol Head Neck Surg ; 168(4): 707-713, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-35727632

RESUMO

OBJECTIVE: The aim of our study was to describe the diagnostic performances of tubomanometry (TMM) and to determine tubomanometric parameter thresholds for the diagnosis of patulous eustachian tube (PET). STUDY DESIGN: We performed a retrospective, monocentric study, including patients treated for PET vs control group. SETTING: This study was performed at the Otolaryngology Department of a tertiary-care hospital in the south of France. METHODS: We collected epidemiologic and clinical data, as well as adjusted opening latency index ("R"-index), rhinopharyngeal pressure threshold of tubal opening (Po), and velar contraction index (IVC) on TMM. Receiver operating characteristic (ROC) curves were used for determination of R index and Po thresholds. RESULTS: Twenty-one patients (26 patulous ears) and 14 controls (24 normal ears) were included. The R index values and Po values were significantly lower in the PET group vs controls (0.46 vs 0.80, respectively; P < .05 for R index and 13.89 vs 26.42 mbar, respectively; P < .05 for Po). No significant difference was reported between the 2 groups on IVC measurement (P = .784). After ROC curve analysis, R index was the most discriminating factor to classify PET patients with 89% specificity and 76% sensitivity with a threshold ≤0.6. Po value ≤10 mbar could support this diagnosis with more than 83% specificity. CONCLUSION: TMM is a reliable noninvasive method for positive diagnosis of PET. TMM could provide an accurate positive PET diagnosis and an objective evaluation for PET management.


Assuntos
Otopatias , Tuba Auditiva , Otite Média , Humanos , Tuba Auditiva/diagnóstico por imagem , Tuba Auditiva/cirurgia , Estudos Retrospectivos , Nasofaringe , Curva ROC , Otopatias/diagnóstico
3.
Eur Arch Otorhinolaryngol ; 280(3): 1101-1109, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35932313

RESUMO

PURPOSE: Balloon dilatation of the Eustachian tube (BDET) is an option for treating chronic obstructive Eustachian tube dysfunction (COETD). In this prospective multicentric study, the main objective was to evaluate the results of BDET in unilateral COETD refractory to medical treatment. METHODS: Adults with unilateral COETD whose Eustachian Tube Score (ETS) was less than 5 despite medical pressure therapy were included. The primary endpoint was the change in ETS measured at 2, 6, and 12 months after BDET. Secondary objectives were the evolution of clinical symptoms assessed by the Eustachian Tube Questionnaire (ETDQ-7), audiometry, tympanometry, and otoscopy changes after BDET. RESULTS: Twenty-eight patients were included between May 2014 and December 2017 and were treated with BDET without adverse effects in three different referral centers. Population's median age was 52 (Q25; Q75: 24, 82) years. The median follow-up time was 381 (Q25; Q75: 364; 418) days. The median ETS was 2 (Q25; Q75: 1; 4) before BDET. There was a significant improvement in ETS at 2 and 6 months and 1 year after BDET (score at 1 year: 6 (Q25; Q75: 2; 8) (p < 0.0001)). There was a significant, sustained improvement in the ETDQ-7 with a score of 4.21 (Q25; Q75-3.50; 4.79) before BDET and 3.43 (Q25; Q75-2.43; 4.14) (p = 0.0012) at 1 year. There was a suggestive improvement in tympanometry results at 1 year (p = 0.025). CONCLUSION: BDET provides an improvement in symptoms and objective measures assessed at 1 year in patients with COETD who have failed medical treatment. TRIAL REGISTRATION: NCT02123277 (April 25, 2014).


Assuntos
Otopatias , Tuba Auditiva , Adulto , Humanos , Pessoa de Meia-Idade , Tuba Auditiva/cirurgia , Dilatação/métodos , Estudos Prospectivos , Endoscopia , Otoscopia , Otopatias/terapia , Otopatias/cirurgia , Resultado do Tratamento
4.
J Neurol ; 269(8): 4333-4348, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-35306619

RESUMO

During navigation, humans mainly rely on egocentric and allocentric spatial strategies, two different frames of reference working together to build a coherent representation of the environment. Spatial memory deficits during navigation have been repeatedly reported in patients with vestibular disorders. However, little is known about how vestibular disorders can change the use of spatial navigation strategies. Here, we used a new reverse T-maze paradigm in virtual reality to explore whether vestibular loss specifically modifies the use of egocentric or allocentric spatial strategies in patients with unilateral (n = 23) and bilateral (n = 23) vestibular loss compared to healthy volunteers (n = 23) matched for age, sex and education level. Results showed that the odds of selecting and using a specific strategy in the T-maze were significantly reduced in both unilateral and bilateral vestibular loss. An exploratory analysis suggests that only right vestibular loss decreased the odds of adopting a spatial strategy, indicating an asymmetry of vestibular functions. When considering patients who used strategies to navigate, we observed that a bilateral vestibular loss reduced the odds to use an allocentric strategy, whereas a unilateral vestibular loss decreased the odds to use an egocentric strategy. Age was significantly associated with an overall lower chance to adopt a navigation strategy and, more specifically, with a decrease in the odds of using an allocentric strategy. We did not observe any sex difference in the ability to select and use a specific navigation strategy. Findings are discussed in light of previous studies on visuo-spatial abilities and studies of vestibulo-hippocampal interactions in peripheral vestibular disorders. We discuss the potential impact of the history of the disease (chronic stage in patients with a bilateral vestibulopathy vs. subacute stage in patients with a unilateral vestibular loss), of hearing impairment and non-specific attentional deficits in patients with vestibular disorders.


Assuntos
Vestibulopatia Bilateral , Navegação Espacial , Doenças Vestibulares , Realidade Virtual , Feminino , Humanos , Masculino , Aprendizagem em Labirinto , Transtornos da Memória , Percepção Espacial , Doenças Vestibulares/diagnóstico
5.
Neurosurg Rev ; 45(1): 751-761, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34269934

RESUMO

In order to verify whether a previous gamma knife surgery (GKS) treatment could influence the oncological and functional outcome in large vestibular schwannoma (VS) surgery, we have compared group of patients operated on for large VS after failed GKS to a group of genuine VS that underwent the same functional nerve-sparing resection technique regimen in the same period. Single center retrospective cohort study of 23 consecutive GKS failure and 170 genuine VS patients operated on between April 2003 and March 2019. After resection, patients were allocated to a Wait-&-rescan or an upfront GKS policy. At last follow-up examination, the facial nerve function was good (House-Brackmann grades I or II) in 95% of the GKS failure and 84% of the genuine VS patients (p = .25). The median volume of tumor residue was .56 cc in the GKS failure group and .62 cc in the genuine VS group (p = .70). Tumor control was achieved in 91% and 83% of cases with a mean follow-up of 74 and 63 months in the GKS failure and the genuine VS populations, respectively. The 1-, 5-, and 7-year progression-free survival were 100%, 95%, and 85% respectively in the GKS failure group and 97%, 80%, and 81% in the genuine VS group (p = .27). Despite significant modifications of the microsurgical environment associated to salvage surgery after GKS failure, a functional nerve-sparing resection is an effective strategy to optimize the results on facial nerve function, with similar long-term tumor control to those observed in the genuine VS population.


Assuntos
Neuroma Acústico , Radiocirurgia , Nervo Facial/cirurgia , Seguimentos , Humanos , Neuroma Acústico/cirurgia , Estudos Retrospectivos , Resultado do Tratamento
6.
Front Syst Neurosci ; 15: 733684, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34776883

RESUMO

Introduction: The unilateral vestibular syndrome results in postural, oculomotor, perceptive, and cognitive symptoms. This study was designed to investigate the role of vestibular signals in body orientation representation, which remains poorly considered in vestibular patients. Methods: The subjective straight ahead (SSA) was investigated using a method disentangling translation and rotation components of error. Participants were required to align a rod with their body midline in the horizontal plane. Patients with right vestibular neurotomy (RVN; n =8) or left vestibular neurotomy (LVN; n = 13) or vestibular schwannoma resection were compared with 12 healthy controls. Patients were tested the day before surgery and during the recovery period, 7 days and 2 months after the surgery. Results: Before and after unilateral vestibular neurotomy, i.e., in the chronic phases, patients showed a rightward translation bias of their SSA, without rotation bias, whatever the side of the vestibular loss. However, the data show that the lower the translation error before neurotomy, the greater its increase 2 months after a total unilateral vestibular loss, therefore leading to a rightward translation of similar amplitude in the two groups of patients. In the early phase after surgery, SSA moved toward the operated side both in translation and in rotation, as typically found for biases occurring after unilateral vestibular loss, such as the subjective visual vertical (SVV) bias. Discussion and Conclusion: This study gives the first description of the immediate consequences and of the recovery time course of body orientation representation after a complete unilateral vestibular loss. The overall evolution differed according to the side of the lesion with more extensive changes over time before and after left vestibular loss. It is noteworthy that representational disturbances of self-orientation were highly unusual in the chronic stage after vestibular loss and similar to those reported after hemispheric lesions causing spatial neglect, while classical ipsilesional biases were reported in the acute stage. This study strongly supports the notion that the vestibular system plays a major role in body representation processes and more broadly in spatial cognition. From a clinical point of view, SSA appeared to be a reliable indicator for the presence of a vestibular disorder.

7.
Eur J Radiol ; 143: 109911, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34508941

RESUMO

PURPOSE: The aim of this study is to identify quantitative MR biomarkers in head and neck paragangliomas. METHODS: The study was approved by an institutional review board. A retrospective review of patients with head and neck paragangliomas (HNPGL) evaluated by time-resolved MRA sequences between 2009 and 2019 was performed. A control group investigated during the same period was analyzed, including nerve sheath tumors and metastatic lymph nodes from squamous cell carcinomas or undifferentiated nasopharyngeal cancer (UCNT). A gold standard was obtained for all cases. Semi-quantitative parameters of enhancement were extracted from time-intensity curves on time-resolved MRA sequences and diffusion weighted imaging/DWI was assessed for each lesion. RESULTS: Sixty head and neck paragangliomas (HNPGLs) were included from 50 patients. The control group consisted of 30 parapharyngeal space lesions (27 patients), which included nerve sheath tumors (n = 12) and metastatic lymph nodes (n = 18) from squamous cell carcinomas or UCNT. PGLs showed a shorter time-to-peak value compared to other groups, measured at 25.0 +/- 29 sec. The wash-in and wash-out ratios were also significantly higher for PGLs, respectively measured at 5.34 ± 2.99 (p < 0,001) and 1.24 ± 0.80 (p < 0.001). On DWI sequences, the mean ADC value for PGLs (1.17 ± 0.19 10^-3 mm2/s) was significantly different than the other tumor groups (p < 0.001). HNPGLs were clearly distinguishable from other tumors on classification with regression tree based on TTP and ADC values. These distinct group features were also consistent on principal component analysis. CONCLUSION: Our study identifies a multiparametric signature for disease subtyping, providing a strong impetus for switching from qualitative to quantitative analysis of deep soft-tissue tumors of the neck.


Assuntos
Neoplasias de Cabeça e Pescoço , Imageamento por Ressonância Magnética Multiparamétrica , Neoplasias Nasofaríngeas , Paraganglioma , Biomarcadores , Imagem de Difusão por Ressonância Magnética , Neoplasias de Cabeça e Pescoço/diagnóstico por imagem , Humanos , Paraganglioma/diagnóstico por imagem , Estudos Retrospectivos
8.
Otol Neurotol ; 42(8): e1072-e1076, 2021 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-34238895

RESUMO

IMPORTANCE: An infective etiology of acute peripheral vestibulopathy (APV) has long been hypothesized. In the context of coronavirus disease 2019 (COVID-19), we examined the possible comorbidity between these two entities. OBJECTIVES: APV is the second most common cause of vestibular disorders and results from a sudden and unilateral loss of vestibular inputs. The characteristic signs and symptoms include sudden and prolonged vertigo, absence of auditory symptoms, and absence of other neurological symptoms. An infective etiology of APV has long been hypothesized on the basis of its association with respiratory tract infections and its frequent occurrence in epidemics. Possible comorbidity with herpes simplex virus type 1 reactivation or influenza virus infection has also been proposed. This study was designed to assess the possible comorbidity between APV and COVID-19. DESIGN/SETTING/PARTICIPANTS: Quantification of the number of hospital admissions for APV over the period from February to May 2020 was carried out in 5 French hospitals. A comparison with 2018 and 2019 entries over the same period was made. Comorbidity between APV and COVID-19 infection was investigated. RESULTS: No significant increase in admission for APV was noticed over the examination period. No significant difference was noticed among hospitals located in COVID-19 high- and low-risk zones for SARS-CoV-2. No significant increase in the severity of the APV cases was noticed. No case of comorbidity between APV and SARS-CoV-2 infection was reported. Based on our observations, no correlation was made between APV and COVID-19. CONCLUSION: Based on our observations, COVID-19 is not statistically correlated with APV.


Assuntos
COVID-19 , Neuronite Vestibular , Comorbidade , Hospitalização , Humanos , SARS-CoV-2
9.
Otol Neurotol ; 42(7): 1089-1094, 2021 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-34260513

RESUMO

OBJECTIVES: Evaluate intra-rater and inter-rater variabilities of the Sunnybrook Facial Grading System (SFGS) and explore potential factors of variability. STUDY DESIGN: Prospective test of hypothesis. SETTING: University tertiary referral center. PARTICIPANTS/METHODS: Facial video recordings of 20 patients with variable degrees of peripheral facial palsy (PFP) were anonymized then presented to 31 independents raters in 2 trials. The raters were senior and junior professionals involved in the management of PFP: ENT specialists, physiotherapists, and speech therapists. The SFGS was used for grading paralysis. MAIN OUTCOME MEASURE: Intra-rater and inter-rater variabilities were estimated by intraclass correlation coefficient (ICC [95% confidence interval]) for the composite score and the three subscores of the SFGS. Factors of variability studied were: rater professions and rater experience (senior vs junior). RESULTS: For the total population, the intra-rater ICC was 0.915[0.900-0.929] for the composite score considered to represent almost perfect repeatability. Repeatability was important for symmetry at rest (0.694[0.646-0.737]), almost perfect for voluntary movements (0.903[0.886-0.918]), and important to almost perfect for synkinesis (0.810[0.778-0.838]). The inter-rater ICC for the composite score was 0.847[0.755-0.923] indicating important to almost perfect agreement between all raters. Agreement between raters was important to almost perfect for voluntary movements (0.839[0.746-0.919]), but moderate to important for symmetry at rest (0.553[0.408-0.730]) and weak to important for synkinesis (0.476[0.333-0.666]). Some variability was found between raters groups; however, repeatability and agreement were good for all raters. CONCLUSIONS: The SFGS is a reproducible scale. It can be used with good reproducibility by both novices and experts, and by all professionals involved in the management of PFP.


Assuntos
Paralisia Facial , Face , Paralisia Facial/diagnóstico , Humanos , Variações Dependentes do Observador , Estudos Prospectivos , Reprodutibilidade dos Testes , Gravação em Vídeo
10.
PLoS One ; 16(5): e0251122, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33974628

RESUMO

BACKGROUND: To assess the risk of postoperative SARS-CoV-2 infection during the COVID-19 pandemic. METHODS: The CONCEPTION study was a cohort, multidisciplinary study conducted at Conception University Hospital, in France, from March 17th to May 11th, 2020. Our study included all adult patients who underwent minor surgery in one of the seven surgical departments of our hospital: urology, digestive, plastic, gynecological, otolaryngology, gynecology or maxillofacial surgery. Preoperative self-isolation, clinical assessment using a standardized questionnaire, physical examination, nasopharyngeal RT-PCR and chest CT scan performed the day before surgery were part of our active prevention strategy. The main outcome was the occurrence of a SARS-CoV-2 infection within 21 days following surgery. The COVID-19 status of patients after discharge was updated during the postoperative consultation and to ensure the accuracy of data, all patients were contacted again by telephone. RESULTS: A total of 551 patients from six different specialized surgical Departments in our tertiary care center were enrolled in our study. More than 99% (546/551) of included patients underwent a complete preoperative Covid-19 screening including RT-PCR testing and chest CT scan upon admission to the Hospital. All RT-PCR tests were negative and in 12 cases (2.2%), preoperative chest CT scans detected pulmonary lesions consistent with the diagnosis criteria for COVID-19. No scheduled surgery was postponed. One patient (0.2%) developed a SARS-CoV-2 infection 20 days after a renal transplantation. No readmission or COVID-19 -related death within 30 days from surgery was recorded. CONCLUSIONS: Minor surgery remained safe in the COVID-19 Era, as long as all appropriate protective measures were implemented. These data could be useful to public Health Authorities in order to improve surgical patient flow during a pandemic.


Assuntos
COVID-19/diagnóstico , Cuidados Pré-Operatórios , Idoso , COVID-19/virologia , Estudos de Coortes , Feminino , França , Humanos , Masculino , Pessoa de Meia-Idade , Procedimentos Cirúrgicos Menores , Nasofaringe/virologia , Período Perioperatório , RNA Viral/análise , Reação em Cadeia da Polimerase Via Transcriptase Reversa , SARS-CoV-2/genética , SARS-CoV-2/isolamento & purificação , Centros de Atenção Terciária , Tórax/diagnóstico por imagem , Tomografia Computadorizada por Raios X
11.
Eur J Neurol ; 28(5): 1779-1783, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33369817

RESUMO

BACKGROUND AND PURPOSE: In this study, the question of whether egocentric representation of space is impaired in chronic unilateral vestibulopathies was examined. The objective was to test current theories attributing a predominant role to vestibular afferents in spatial cognition and to assess whether representational neglect signs are common in peripheral vestibular loss. METHODS: The subjective straight-ahead (SSA) direction was investigated using a horizontal rod allowing the translation and rotation components of the body midline representation to be dissociated in 21 patients with unilateral vestibular loss (right, 13; left, eight) and in 12 healthy controls. RESULTS: Compared to the controls, the patients with unilateral vestibulopathy showed a translation bias of their SSA, without rotation bias. The translation bias was not lateralized towards the lesioned side as typically found for biases reported after unilateral vestibular loss. Rather, the SSA bias was rightward whatever the side of the vestibular loss. The translation bias correlated with the vestibular loss, as measured by caloric response and vestibulo-ocular reflex gain, but not with the subjective visual vertical or the residual spontaneous nystagmus. CONCLUSION: The present data suggest that the dysfunctions of neural networks involved in egocentred and allocentred representations of space are differentially compensated for in unilateral vestibular defective patients. In particular, they suggest that asymmetrical vestibular inputs to cortical regions lead to representational spatial disturbances as does defective cortical processing of vestibular inputs in spatial neglect after right hemisphere stroke. They also highlight the predominant role of symmetrical and unaltered vestibular inputs in spatial cognition.


Assuntos
Transtornos da Percepção , Lateralidade Funcional , Humanos , Transtornos da Percepção/etiologia , Reflexo Vestíbulo-Ocular
12.
Proc Natl Acad Sci U S A ; 117(49): 31278-31289, 2020 12 08.
Artigo em Inglês | MEDLINE | ID: mdl-33229591

RESUMO

Presbycusis, or age-related hearing loss (ARHL), is a major public health issue. About half the phenotypic variance has been attributed to genetic factors. Here, we assessed the contribution to presbycusis of ultrarare pathogenic variants, considered indicative of Mendelian forms. We focused on severe presbycusis without environmental or comorbidity risk factors and studied multiplex family age-related hearing loss (mARHL) and simplex/sporadic age-related hearing loss (sARHL) cases and controls with normal hearing by whole-exome sequencing. Ultrarare variants (allele frequency [AF] < 0.0001) of 35 genes responsible for autosomal dominant early-onset forms of deafness, predicted to be pathogenic, were detected in 25.7% of mARHL and 22.7% of sARHL cases vs. 7.5% of controls (P = 0.001); half were previously unknown (AF < 0.000002). MYO6, MYO7A, PTPRQ, and TECTA variants were present in 8.9% of ARHL cases but less than 1% of controls. Evidence for a causal role of variants in presbycusis was provided by pathogenicity prediction programs, documented haploinsufficiency, three-dimensional structure/function analyses, cell biology experiments, and reported early effects. We also established Tmc1N321I/+ mice, carrying the TMC1:p.(Asn327Ile) variant detected in an mARHL case, as a mouse model for a monogenic form of presbycusis. Deafness gene variants can thus result in a continuum of auditory phenotypes. Our findings demonstrate that the genetics of presbycusis is shaped by not only well-studied polygenic risk factors of small effect size revealed by common variants but also, ultrarare variants likely resulting in monogenic forms, thereby paving the way for treatment with emerging inner ear gene therapy.


Assuntos
Surdez/genética , Genes Dominantes , Mutação/genética , Presbiacusia/genética , Fatores Etários , Idade de Início , Animais , Estudos de Casos e Controles , Estudos de Coortes , Heterozigoto , Humanos , Proteínas de Membrana/genética , Camundongos , MicroRNAs/genética , Mitocôndrias/genética , Sequenciamento do Exoma
13.
Clin Nucl Med ; 45(12): 982-983, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33031243

RESUMO

We report the case of a 72-year-old woman presenting with a progressive left peripheral facial paralysis and a facial canal mass extending through the stylomastoid foramen along the posterior edge of the parotid gland. On MRI, the early and intense enhancement was highly suggestive of paraganglioma but could not rule out a nonossifying hemangioma. Laboratory analysis showed normal plasma metanephrines. On F-FDOPA PET/CT, the mass exhibited a typical paraganglioma feature with a marked tumor uptake. Our case demonstrates that F-FDOPA plays a vital role in this rare entity and can avoid any further confirmatory invasive procedure.


Assuntos
Nervo Facial/patologia , Paraganglioma/diagnóstico por imagem , Idoso , Nervo Facial/diagnóstico por imagem , Feminino , Humanos , Paraganglioma/patologia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada
14.
Eur Arch Otorhinolaryngol ; 277(8): 2209-2217, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32279104

RESUMO

PURPOSE: To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making. METHODS: A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients' demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered. RESULTS: At presentation, transient or persistent facial palsy was the most common symptom, followed by hearing loss. The schwannoma involved more than one segment in the majority of the patients with the geniculate ganglion being most commonly involved. Initial treatment consisted of a wait and scan approach in 67.5%, surgery in 30% and radiation therapy in 2.5% of the patients. Tympanic segment schwannomas caused mainly conductive hearing loss and were more prone to develop facial palsy at follow-up. Internal auditory canal or cerebellopontine angle schwannomas presented with significantly more sensorineural hearing loss. CONCLUSIONS: Although modern imaging has improved diagnosis of this tumor, choosing the best treatment modality remains a real challenge. Based on the literature review and current findings, more insights into the clinical course and the management of facial nerve schwannomas are provided.


Assuntos
Neoplasias dos Nervos Cranianos , Doenças do Nervo Facial , Paralisia Facial , Neurilemoma , Neoplasias dos Nervos Cranianos/diagnóstico , Neoplasias dos Nervos Cranianos/cirurgia , Europa (Continente) , Nervo Facial , Doenças do Nervo Facial/diagnóstico , Doenças do Nervo Facial/etiologia , Paralisia Facial/diagnóstico , Paralisia Facial/etiologia , Humanos , Neurilemoma/diagnóstico , Neurilemoma/cirurgia , Estudos Retrospectivos
15.
High Alt Med Biol ; 21(1): 99-104, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31985271

RESUMO

Background: Middle ear (ME) pressure regulation has been suggested as a physiological mechanism that maintains pressure equilibrium between the ME and the ambient environment. This mechanism would be based on a complex sensorineural reflex loop composed of mechanoreceptors, an integrative center, and efferent neural pathways. Our aim was to demonstrate that hypoxic conditions, which would inhibit mechanoreceptors in general, similarly participate in the inhibition of the opening of the Eustachian tube (ET), and thus, to suggest that such receptors are involved in the overall regulation of ME pressure. Materials and Methods: Among 14 healthy volunteers, tubomanometry was performed in normoxia followed by hypoxia, and 3 parameters were evaluated for each ear under each condition, allowing the evaluation of the reactivity of the system: ET opening latency index (R), the Index of Velum Contraction (IVC), and the latency of pressure instauration (C2-C1). Results: Hypoxia induced a significant increase in the opening latency index of ET opening, without deleterious effects on the quality (IVC) and latency (C2-C1) of soft palate contraction. Conclusions: Our study supports the theory of a sensorineural reflex loop and provides evidence for the existence of mechanoreceptors, whose function is modified by changes in oxygen partial pressure, able to collect information on pressure variations between the ME and the external environment.


Assuntos
Tuba Auditiva , Humanos , Hipóxia , Pressão , Reflexo
16.
Neurosurg Rev ; 43(4): 1191-1199, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31388842

RESUMO

In order to verify whether neurofibromatosis type 2 (NF2) could influence the oncological and functional outcome in large vestibular schwannoma (VS) surgery, we compared a group of NF2 patients operated with a facial nerve-sparing technique to a group of sporadic VSs of similar volume that underwent the same treatment regimen in the same period. Single-center retrospective cohort study about 12 consecutive NF2 and 69 non-NF2 patients operated on for large VS between September 2006 and November 2016. After resection, patients were allocated to an upfront Gamma Knife surgery policy of the tumor residue. At last follow-up examination, the facial nerve function was good (House-Brackmann grades I or II) in 92% of the NF2 and 83% of the non-NF2 patients (p = .90). The median volume of tumor residue was .92 cc in the NF2 group and .54 cc in the non-NF2 group (p = .14). Tumor control was achieved in 83% and 81% of cases in the NF2 and the non-NF2 populations, respectively, with a mean follow-up of 73 months in both groups. The 1-, 5-, and 7-year progression-free survival were 92%, 83%, and 83% respectively in the NF2 group, and 99%, 83%, and 80% in the non-NF2 group (p = .96). Our analysis of 12 NF2 and 69 non-NF2 patients operated on by the same surgical team with the same treatment regimen did not show any functional or tumor control difference between those groups.


Assuntos
Neurofibromatose 2/complicações , Neuroma Acústico/complicações , Neuroma Acústico/cirurgia , Procedimentos Neurocirúrgicos/métodos , Radiocirurgia/métodos , Adolescente , Adulto , Estudos de Coortes , Terapia Combinada , Nervo Facial/fisiopatologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasia Residual/cirurgia , Intervalo Livre de Progressão , Resultado do Tratamento , Adulto Jovem
17.
Otol Neurotol ; 41(1): e89-e93, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31789798

RESUMO

OBJECTIVE: We describe the effect of long-term tacrolimus delivery on sporadic vestibular schwannoma (VS). PATIENT: A 48-year-old woman who was diagnosed a right VS. INTERVENTION: Long-term tacrolimus delivery following liver transplant. MAIN OUTCOME MEASURES: Clinical examination, audiometric test, and gadolinium-contrasted magnetic resonance imaging. RESULTS: During the reported follow-up period, the patient felt hearing improvement especially in noisy environment. This was consistent with a clear audiometric improvement. Over a 26 months follow-up period, we noticed a 50% volume regression and a 40% decrease in the intensity of enhancement of the tumor after gadolinium injection. CONCLUSION: Tacrolimus, an immunosuppressant used to prevent rejection in organ transplant, could lead to VS volume shrinkage and hearing improvement. Tacrolimus could be an interesting new therapeutic weapon, especially for VS in Neurofibromatosis type 2.


Assuntos
Imunossupressores/uso terapêutico , Transplante de Fígado , Neuroma Acústico/patologia , Tacrolimo/uso terapêutico , Feminino , Rejeição de Enxerto/prevenção & controle , Humanos , Achados Incidentais , Pessoa de Meia-Idade , Resultado do Tratamento
18.
Clin Endocrinol (Oxf) ; 91(6): 879-884, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31479526

RESUMO

OBJECTIVES: Nuclear imaging findings by virtue of phenotyping disease heavily depend on genetic background, metabolites, cell membrane specific targets and signalling pathways. PPGL related to succinate dehydrogenase subunits mutations (SDHx mutations) are less differentiated than other subgroups and therefore may lack to concentrate 18 F-FDOPA, a precursor of catecholamines biosynthesis. However, this 18 F-FDOPA negative phenotype has been reported mostly in SDHx-PPGL of sympathetic origin, suggesting that both genotype status and location (from sympathetic vs parasympathetic paraganglia; adrenal vs extra-adrenal) could influence 18 F-FDOPA uptake. The aim of this study was to test if SDHx drives 18 F-FDOPA uptake in presence of normal epinephrine/norepinephrine concentrations. DESIGN: Retrospective study PATIENTS: A cohort of 86 head and neck PPGL patients (including three metastatic) with normal metanephrines underwent 18 F-FDOPA PET/CT. The relationships between 18 F-FDOPA uptake and tumour genotype were evaluated. RESULTS: In nonmetastatic HNPGL (50 non-SDHx/33 SDHx), no significant difference was observed between these two groups for SUVmax (P = .256), SUVmean (P = .188), MTV 42% (P = .596) and total lesion uptake (P = .144). Metastatic HNPGL also had high elevated uptake values. CONCLUSIONS: Our results suggest that SDH deficiency or metastatic behaviour have no influence on 18 F-FDOPA uptake in HNPGL probably due to their very-well differentiation status, even at metastatic stage. The potential prognosticator value of 18 F-FDOPA uptake would need to be further explored in the setting of metastatic PPGL of sympathetic origin.


Assuntos
Neoplasias de Cabeça e Pescoço/diagnóstico por imagem , Neoplasias de Cabeça e Pescoço/enzimologia , Paraganglioma/diagnóstico por imagem , Paraganglioma/enzimologia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Succinato Desidrogenase/deficiência , Adulto , Feminino , Genótipo , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Cintilografia , Estudos Retrospectivos , Succinato Desidrogenase/metabolismo
19.
J Neurol ; 266(Suppl 1): 149-159, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31230115

RESUMO

A growing number of studies indicate that cognitive complaints are common in patients with peripheral vestibular disorders. A better understanding of how vestibular disorders influence cognition in these patients requires a clear delineation of the cognitive domains affected by vestibular disorders. Here, we compared the consequences of left and right vestibular neurectomy on third-person perspective taking-a visuo-spatial task requiring mainly own-body mental imagery, and on 3D objects mental rotation imagery-requiring object-based mental imagery, but no perspective taking. Patients tested 1 week after a unilateral vestibular neurectomy and a group of age- and gender-matched healthy participants played a virtual ball-tossing game from their own first-person perspective (1PP) and from the perspective of a distant avatar (third-person perspective, 3PP). Results showed larger response times in the patients with respect to their controls for the 3PP taking task, but not for the 1PP task and the 3D objects mental imagery. In addition, we found that only patients with left vestibular neurectomy presented altered 3PP taking abilities when compared to their controls. This study suggests that unilateral vestibular loss affects mainly own-body mental transformation and that only left vestibular loss seems to impair this cognitive process. Our study also brings further evidence that vestibular signals contribute to the sensorimotor bases of social cognition and strengthens the connections between the so far distinct fields of social neuroscience and human vestibular physiology.


Assuntos
Cognição/fisiologia , Estimulação Luminosa/métodos , Desempenho Psicomotor/fisiologia , Percepção Espacial/fisiologia , Doenças Vestibulares/diagnóstico , Vestíbulo do Labirinto/fisiologia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças Vestibulares/fisiopatologia , Doenças Vestibulares/cirurgia
20.
World Neurosurg ; 127: e599-e608, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30930324

RESUMO

OBJECTIVE: The preservation of acceptable facial nerve (FN) function after surgery is the key concern for most patients with vestibular schwannomas (VS). To assess predictive factors of early postoperative and long-term FN function in patients harboring large VS operated with a FN-sparing technique. METHODS: Single-center retrospective cohort study with 169 consecutive large VS operated on between January 2003 and May 2015. Clinical, radiologic, and intraoperative factors were assessed according to FN function. RESULTS: At last follow-up examination, among the 145 patients without preoperative FN palsy, FN function was good (House-Brackmann [HB] grades I or II) in 84% and moderate (HB grade III) in 15% of patients. Only 1 patient presented with poor HB grade IV function. Multivariate logistic regression model showed the mean preoperative VS extrameatal diameter as being an independent predictor of an unfavorable initial FN outcome (odds ratio [OR], 1.062; P = 0.038). Surgical anatomic preservation of the cochlear nerve was associated with better FN outcomes (OR, 0.237; P = 0.012). A history of previous surgery seemed to be related to long-term impaired FN function (OR, 71.405; P = 0.042), as well as early postoperative FN function (OR, 19.068; P = 0.000). No correlation was found between a history of previous Gamma Knife surgery treatment (P = 0.225) or the extent of resection (P = 0.438) and impaired FN outcomes. History of previous surgery was identified as an unfavorable predictive recovery factor of impaired postoperative FN function (P = 0.034). CONCLUSIONS: As long as the extent of resection or additional Gamma Knife surgery have not been identified as predictive risk factors of postoperative FN palsy, we suggest that optimal resection is the main option for patients harboring large VS.


Assuntos
Traumatismos do Nervo Facial/cirurgia , Nervo Facial/cirurgia , Paralisia Facial/cirurgia , Neuroma Acústico/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Traumatismos do Nervo Facial/etiologia , Paralisia Facial/etiologia , Feminino , Humanos , Masculino , Microcirurgia/métodos , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos/métodos , Tempo , Resultado do Tratamento , Adulto Jovem
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