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To calculate the prevalence of sinonasal and ear involvement in an Erdheim-Chester disease (ECD) population, to describe the different ear, nose and throat (ENT) manifestations and to study the association between ENT involvement, other organ involvement, and BRAF mutations. We led a retrospective monocentric study in the national referral center for ECD. One hundred and sixty-two patients with ECD and ENT data were included between January 1, 1980 and December 31, 2020. Ear and nose clinical and radiological findings were noted. We described and studied the prevalence of ENT involvement in ECD population. The association between sinonasal and ear involvement, other organ involvement, and BRAF mutations was calculated. The prevalence of ENT manifestations is around 45%. No clinical rhinologic or otologic signs were specific to ECD. Sinus imaging was abnormal in 70% of cases. A bilateral maxillary sinus frame osteosclerosis was highly specific of ECD. Associations were found between the sinus MRI imaging type and BRAF status, central nervous system involvement, cerebellum involvement and xanthelasma. Sinonasal or ear involvement is frequent in ECD and has specific imaging features for sinuses. Trial registration: #2011-A00447-34.
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Doença de Erdheim-Chester , Humanos , Doença de Erdheim-Chester/complicações , Doença de Erdheim-Chester/diagnóstico por imagem , Estudos Retrospectivos , Proteínas Proto-Oncogênicas B-raf/genética , MutaçãoRESUMO
A 48-year-old man presented to the ENT department of a general hospital after a traumatic event that occurred at work. During arc welding, an incandescent metallic projectile entered the left external auditory canal immediately causing earache, tinnitus, hearing loss, vertigo, and completed peripheral facial palsy on the left side. A burnt middle ear is a rare situation for which very few cases are described in the literature. We describe and discuss our therapeutic strategy in an emergency setting. This can be of interest to any ENT surgeon who may face such a case of ear burn. Then we report the first repair of the facial nerve by interposition of an anastomosed intermediate graft associated with cochlear implantation during a single surgical intervention. This case illustrates the multimodal rehabilitation of the damage that can be a consequence of petrous trauma. The patient recovered hearing and facial motor skills.
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Queimaduras , Implante Coclear , Masculino , Humanos , Pessoa de Meia-Idade , Nervo Facial/cirurgia , Orelha Média , Anastomose CirúrgicaRESUMO
PURPOSE: To evaluate functional results of facial nerve repair by direct nerve suture or grafting, compare results between a traumatic and a tumoral group and identify prognostic factors. METHODS: A retrospective monocentric cohort study was studied in a university ENT department. Thirty-one patients who benefited from facial nerve suture or grafting, with at least 12 months postoperative follow-up were included. Patients were divided into two groups according to the lesion type: traumatic (accident of the public road or iatrogenic) and tumoral. Preoperative data studied were sex, side, etiology documented by CT and/or MRI, facial palsy duration and grade according to House Brackmann grading system. Intraoperative data included: surgeon, age of patient, surgical technique, graft type, use of biological glue, facial nerve derivation, lesioned site. Postoperative data included: histological diagnosis, radiotherapy history, time to onset of the first signs of reinnervation, follow-up duration, and final facial function. RESULTS: Success rate, including grade III and IV, was 68% in the whole cohort, 79% in the traumatic group and 59% in the tumoral group. Patients presenting with facial palsy evolving less than 6 months had better recovery results than those evolving longer than 6 months (p = 0.02). No other prognostic factors were identified. CONCLUSIONS: The best outcome that can be achieved by suture or grafting of the facial nerve in traumatic or tumoral lesions is a grade III. Preoperative facial palsy duration is a prognostic factor and must be considered when establishing an operative indication.
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Nervo Facial , Paralisia Facial , Estudos de Coortes , Nervo Facial/cirurgia , Paralisia Facial/diagnóstico , Paralisia Facial/etiologia , Humanos , Prognóstico , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: Oro-facial myofunctional praxis assesses the muscular coordination and the degree of motor impairment of the lingual, mandibular and facial muscles necessary for articulation, mimicry and swallowing. PURPOSE: The objective of this study was to create and validate the MBLF protocol, a French oro-facial myofunctional assessment in order to quantify patient's impairment and to specify the motor and functional deficit for an adapted management. METHODS: The MBLF was validated against the Sunnybrook Facial Grading System (criterion validity). The construct validity was tested by comparing healthy subjects (n = 102) from patients with facial palsy (n = 60). Internal and external consistency of face symmetry were reported. Normative data was provided. RESULTS: There was a statistically significant correlation between the MBLF protocol and the Sunnybrook Facial Grading System [F(59) = 310.51, p < 0.001, R2 = 0.843]. Significant differences were observed in Student's t test between healthy volunteers and patients with facial palsy [t(74.13) = 14,704, p < 0.001, r = 0.863]. A significant effect of the severity grades of facial palsy on the MBLF_TOTAL scores was found [F(158) = 268.469, p < 0.001]. The more severe the facial palsy, the lower the motor scores were. CONCLUSION: This MBLF French validation provides a baseline for comparing and quantifying the performance of subjects. The MBLF protocol is valid for assessing facial symmetry in peripheral facial palsy. A prospective study is needed to validate its role in dynamic evaluation of facial palsy.
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Paralisia de Bell , Paralisia Facial , Face , Músculos Faciais , Paralisia Facial/diagnóstico , Humanos , Estudos Prospectivos , Reprodutibilidade dos TestesAssuntos
Limiar Auditivo , Condução Óssea , Otosclerose/cirurgia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , PrognósticoAssuntos
Adenoma/cirurgia , Neoplasias da Orelha/cirurgia , Orelha Média , Tumores Neuroendócrinos/cirurgia , Adenoma/diagnóstico por imagem , Adenoma/patologia , Adulto , Diagnóstico Diferencial , Neoplasias da Orelha/diagnóstico por imagem , Neoplasias da Orelha/patologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/diagnóstico por imagem , Tumores Neuroendócrinos/patologia , Estudos Retrospectivos , Tomografia Computadorizada por Raios XAssuntos
Nervo Facial , Neurilemoma/terapia , Adolescente , Adulto , Idoso , Anastomose Cirúrgica , Tratamento Conservador , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos RetrospectivosAssuntos
Paralisia de Bell/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Meios de Contraste , Feminino , Humanos , Imageamento Tridimensional , Imageamento por Ressonância Magnética , Masculino , Interpretação de Imagem Radiográfica Assistida por Computador , Estudos RetrospectivosAssuntos
Paralisia de Bell/diagnóstico , Paralisia Facial/diagnóstico , Tomografia Computadorizada Multidetectores/métodos , Osso Petroso/diagnóstico por imagem , Paralisia de Bell/complicações , Paralisia Facial/etiologia , Seguimentos , Humanos , Imageamento Tridimensional , Prognóstico , Curva ROC , Estudos RetrospectivosRESUMO
Long-standing facial palsy sequelae cause functional, aesthetic, and psychological problems in patients. Botulinum toxin is an effective way to manage them, but no standardized recommendations exist. Through this non-systematic review, we aimed to guide any practitioner willing to master the ins and outs of this activity. We reviewed the existing literature and completed, with our experience as a reference center, different strategies of botulinum toxin injections used in facial palsy patients, including history, physiopathology, facial analysis, dosages, injection sites, and techniques, as well as time intervals between injections. The reader will find all the theorical information needed to best guide injections according to the patient's complaint, which is the most important information to consider.
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Paralisia de Bell , Toxinas Botulínicas , Paralisia Facial , Humanos , Face , Progressão da DoençaRESUMO
(1) Background: Sequels of facial palsy lead to major psychosocial repercussions, disrupting patients' quality of life (QoL). Botulinum toxin (BoNT) injections can permit us to treat long-standing facial palsy, improving facial symmetry and functional signs including synkinesis and contractures. (2) Methods: The main aim of this study was to assess the evolution of the QoL for patients with long-standing facial palsy before, at 1 month, and at 4 months after BoNT injections by using three questionnaires (HFS-30, FaCE, and HAD). The other goals were to find clinical factors associated with the improvement in the QoL and to assess the HFS-30 questionnaire for patients with unilateral facial palsy (3) Results: Eighty-eight patients were included in this study. There was a statistically significant improvement in QoL at 1 month after injections, assessed using the three questionnaires. This improvement was sustained at 4 months after the injections, with a statistically significant difference for the HFS-30 and FaCE questionnaires. (4) Conclusions: This study showed that the BoNT injections lead to a significant increase in the QoL of patients with unilateral facial palsy. This improvement is sustained 4 months after the injections.
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Paralisia de Bell , Toxinas Botulínicas , Paralisia Facial , Humanos , Qualidade de VidaRESUMO
OBJECTIVES: Head & Neck Paragangliomas have been historically relying on surgery mostly, with worsened quality of life and major sequelae. Conventional external radiation therapy seems to offer an equivalent control rate with a low toxicity profile. The aim of this study was to assess the safety and efficiency of intensity-modulated radiation therapy in Head & Neck paragangliomas. METHODS: This is a retrospective monocentric study conducted in a referral center, including all patients treated with IMRT, whether as an exclusive or post-operative treatment for a tympanic and jugular, carotid, or vagal paraganglioma. Data collection was performed through the manuscript and computerized medical files, including consultation, operative, imaging, pathological analyses, delineation, and treatment planning reports. Success was defined as the complete or partial regression or stabilization without progression, or relapse in accordance with the RECIST criteria. Acute toxicities and long-term sequelae were assessed. RESULTS: Our cohort included 39 patients included between 2011 and 2021: 18 patients treated for a TJ PG (45.9%), 11 patients for a carotid PG (28.4%), and 9 for a vagal PG (23.1%). Twenty-nine patients had IMRT as an exclusive treatment (74.4%), whereas 10 patients had a post-operative complementary treatment (25.6%). Median follow-up in our cohort was 2318 days (average = 2200 days, 237-5690, sd = 1281.9). Among 39 patients, 37 were successfully controlled with IMRT (94.8%), and the toxicity profile was low without any major toxicity. CONCLUSION: IMRT seems an ideal treatment, whether exclusive or post-operative for Head & Neck paragangliomas. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:607-614, 2023.
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Neoplasias de Cabeça e Pescoço , Paraganglioma , Radioterapia de Intensidade Modulada , Humanos , Radioterapia de Intensidade Modulada/efeitos adversos , Radioterapia de Intensidade Modulada/métodos , Estudos Retrospectivos , Qualidade de Vida , Neoplasias de Cabeça e Pescoço/radioterapia , Recidiva Local de Neoplasia , Paraganglioma/radioterapia , Paraganglioma/patologiaRESUMO
BACKGROUND: Elongation of the styloid process associated with oropharyngeal pain and dysphagia is known as Eagle's syndrome, a condition whose pathophysiology is still a matter of debate. Given its low prevalence and complex symptomatology, this syndrome is often misdiagnosed, leading to chronic pain and medical nomadism. A 51-year-old woman of African origin with 3-year history of left-side oropharyngeal pain and worsening dysphagia consulted several health professionals. Medical and surgical treatments, including a sinus surgery and the extraction of three healthy teeth, did not improve her symptoms. Evaluation in an orofacial pain clinic revealed an asymmetrically elongated styloid process. Surgical shortening of the elongated styloid process provided complete pain relief and recovery of normal swallowing function. CONCLUSION: Based on this case report, the pathophysiology of Eagle's syndrome is discussed, and the need for specific follow-up in a subpopulation of patients with asymptomatic styloid process elongation is highlighted.
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Dor Crônica , Transtornos de Deglutição , Ossificação Heterotópica , Dor Crônica/etiologia , Feminino , Humanos , Pessoa de Meia-Idade , Ossificação Heterotópica/complicações , Ossificação Heterotópica/diagnóstico , Ossificação Heterotópica/cirurgia , Osso Temporal/anormalidades , Osso Temporal/diagnóstico por imagem , Osso Temporal/cirurgiaRESUMO
Objective: The role of antibiotics in ear surgery is still controversial. The aim of this study was to assess their need in cholesteatoma surgery when performing obliteration with S53P4 bioactive glass, a biocompatible material with antibacterial properties. Methods: This retrospective cohort study was conducted in a tertiary referral center between January 2017 and May 2019. Sixty-nine consecutive patients, who underwent surgery for cholesteatoma removal and/or rehabilitation of canal-wall-down mastoidectomy with mastoid and epitympanic obliteration using S53P4 granules were included. Before 2019, antibiotics were routinely used (group "w/AB"). Patients received intravenous antibiotics during surgery, oral treatment was continued for 7 days and topical antibiotics for 1 month. After 2019, no antibiotics were administered (group "w/oAB"). The primary outcome was the occurrence of early surgical site infection. Secondary outcomes were late infection, anatomic and functional results at 3 and 12 months. Results: Twenty-three patients were included in group "w/oAB" and 46 in group "w/AB", with no significant differences in demographics, medical history or follow-up. Five ears (22%) in group "w/oAB" developed an early infection compared with 2 (4%) in group "w/AB" (p = .03). The relative risk was 6.11, 95CI%[1.09;31.96]. Infections were successfully treated with antibiotics, and no patient underwent surgical removal of the granules. No late infections or complications were observed. There was no difference in graft failure or air-bone gap closure at 1 year. Conclusion: Peri-/post-operative antibiotics prevent early infection in obliteration surgery with S53P4 granules. Infections can be treated medically without complications or require removal of the implanted material. Level of evidence: 4.
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Vestibular schwannomas (VS) are benign tumors of the vestibular nerve that may trigger hearing loss, tinnitus, rotatory vertigo, and dizziness in patients. Vestibular and auditory tests can determine the precise degree of impairment of the auditory nerve, and superior and inferior vestibular nerves. However, balance is often poorly quantified in patients with untreated vestibular schwannoma, for whom validated standardized assessments of balance are often lacking. Balance can be quantified with the EquiTest. However, this device was developed a long time ago and is expensive, specific, and not sensitive enough to detect early deficits because it assesses balance principally in the sagittal plane on a firm platform. In this study, we assessed postural performances in a well-defined group of VS patients. We used the Dizziness Handicap Inventory (DHI) and a customized device consisting of a smartphone, a mask delivering a fixed or moving visual scene, and foam rubber. Patients were tested in four successive sessions of 25 s each: eyes open (EO), eyes closed (EC), fixed visual scene (VR0), and visual moving scenes (VR1) delivered by the HTC VIVE mask. Postural oscillations were quantified with sensors from an android smartphone (Galaxy S9) fixed to the back. The results obtained were compared to those obtained with the EquiTest. Vestibulo-ocular deficits were also quantified with the caloric test and vHIT. The function of the utricle and saccule were assessed with ocular and cervical vestibular-evoked myogenic potentials (o-VEMPs and c-VEMPs), respectively. We found that falls and abnormal postural oscillations were frequently detected in the VS patients with the VR/Foam device. We detected no correlation between falls or abnormal postural movements and horizontal canal deficit or age. In conclusion, this new method provides a simpler, quicker, and cheaper method for quantifying balance. It will be very helpful for (1) determining balance deficits in VS patients; (2) optimizing the optimal therapy indications (active follow-up, surgery, or gamma therapy) and follow-up of VS patients before and after treatment; (3) developing new rehabilitation methods based on balance training in extreme conditions with disturbed visual and proprioceptive inputs.
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OBJECTIVE: Based on their clinical and radiological patterns, idiopathic CSF rhinorrhea and idiopathic intracranial hypertension can represent different clinical expressions of the same underlying pathological process. Transverse sinus stenoses are associated with both diseases, resulting in eventual restriction of the venous CSF outflow pathway. While venous sinus stenting has emerged as a promising treatment for idiopathic intracranial hypertension, its efficiency on idiopathic CSF leaks has not been very well addressed in the literature so far. The purpose of this study was to report the results of transverse sinus stenting in patients with spontaneous CSF rhinorrhea associated with transverse sinus stenoses. METHODS: From a prospectively collected database, the authors retrospectively collected the clinical and radiological features of the patients with spontaneous CSF leakage who were treated with venous sinus stenting. RESULTS: Five female patients were included in this study. Transverse sinus stenoses were present in all patients, and other radiological signs of idiopathic intracranial hypertension were present in 4 patients. The median transstenotic pressure gradient was 6.5 mm Hg (range 3-9 mm Hg). Venous stenting resulted in the disappearance of the leak in 4 patients with no recurrence and no subsequent meningitis during the follow-up (median 12 months, range 6-63 months). CONCLUSIONS: According to the authors' results, venous sinus stenting may result in the disappearance of the leak in many cases of idiopathic CSF rhinorrhea. Larger comparative studies are needed to assess the efficiency and safety of venous stenting as a first-line approach in patients with spontaneous CSF rhinorrhea associated with transverse sinus stenoses.
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Visible and immediate trauma, facial palsy (FP) covers functional but also psychological damage and it is essential to evaluate before a comprehensive therapeutic care tailored. Few patients, however, are emerging with a prescription for rehabilitation after a consultation. Why? This rehabilitation is it ignored? Is it absolutely necessary? It is evident in the extension of medical care to minimize the effects. Yet the foundation of rehabilitation is sadly little known and often poorly enforced. In addition to its specificity, this therapy preceded by a report called "pretreatments offers a prognosis for recovery to patient" regardless of the origin and degree of involvement of the PF.
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Paralisia Facial/reabilitação , Terapia por Exercício , Paralisia Facial/classificação , Humanos , MassagemRESUMO
It is well-established that botulinum toxin (BT) injections improve quality of life in patients with postparalytic hemifacial spasm. Nevertheless, injection-related pain and contracture-related pain have not yet been studied. The primary objective of our study was to evaluate injection-related pain in patients with facial palsy sequelae, and to compare the standard technique (syringe) with the Juvapen device. The secondary objective was to evaluate the improvement of contracture-related pain one month after BT injection. METHODS: We conducted an observational, prospective, monocentric study based on 60 patients with facial palsy sequelae who received BT injections in our university ENT (ear, nose throat) department. There were 30 patients in the Juvapen group (J) and 30 in the standard technique group (ST). All patients completed Numerical Rating Scale (NRS) questionnaires immediately after the injections and one month later. RESULTS: The average NRS score was 1.33/10 with Juvapen and 2.24/10 with the standard technique (p = 0.0058; Z = 2.75). In patients with contracture-related pain, the average NRS score was 3.53 before BT injection, and 0.41 one month after BT injection (p = 0.0001). CONCLUSIONS: Juvapen is a less-painful injection technique than the standard one. BT reduces contracture-related pain one month after injection.
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Toxinas Botulínicas/uso terapêutico , Espasmo Hemifacial/tratamento farmacológico , Fármacos Neuromusculares/uso terapêutico , Adulto , Idoso , Feminino , França , Humanos , Masculino , Pessoa de Meia-Idade , Dor , Estudos Prospectivos , Qualidade de Vida , Adulto JovemRESUMO
OBJECTIVE: To evaluate the surgical results and complications in a cohort of patients operated on to repair a tegmen bony defect using either transmastoid approach with obliteration using S53P4 bioactive glass granules (TMA-O), or the classic middle cranial fossa approach (MCFA). STUDY DESIGN: A retrospective monocentric study. METHODS: Twenty-five cases (24 patients) were included. Data regarding patient demographics, etiology, intraoperative findings, complications, recurrences, audiometric data, and follow-up were analyzed. RESULTS: Seven patients were operated with MCFA and 17 patients with TMA-O. One patient was operated on using a combined approach (MCFA + TMA-O). In the preoperative HRCT scan, the size of the defect was estimated to be 6 ± 3.8 mm in the TMA-O group and 6 ± 3.5 mm in the MCFA group (P = .969). Intraoperatively, in the MCFA group, the location of the defect was mostly anterior (86%) with an intact ossicular chain (86%). A discontinuous chain was observed in 15 patients (88%) in the TMA-O group. The mean follow-up time was 22 ± 14 months in the TMA-O group and 24 ± 15 months in the MCFA group (P = .762). In both groups, there were no early postoperative complications or recurrences during follow-up. CONCLUSION: Repair of a tegmen bony defect with S53P4 bioactive glass granules seems to be safe and effective, limiting the use of the middle cranial fossa approach to cases with epitympanic defects and with an intact ossicular chain, and it could be used whatever the size of the defect and/or the presence of meningoencephalocele. LEVEL OF EVIDENCE: 4.