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BACKGROUND: Fibrous dysplasia of the bone is a disease caused by a somatic GNAS mutation that affects craniofacial bones and can have a mass effect on different neurovascular structures. The authors present the first case of primary jugular foramen fibrous dysplasia with occlusion of the transverse and sigmoid sinuses. OBJECTIVE: A 33-year-old man presented with a history of dizziness and occasional dysphagia over the past year. Magnetic resonance imaging showed a uniform enhanced mass in the left jugular foramen, with complete blockage of the adjacent transverse and sigmoid sinuses. The computed tomography scan revealed a cystic bone lesion of the jugular foramen. The patient underwent a gross-total removal of the tumor through an infratemporal transjugular approach with complete preservation of the lower cranial nerves. LESSONS: This first reported case of primary jugular foramen fibrous dysplasia highlights the importance of considering this diagnosis when evaluating jugular fossa lesions. Understanding the anatomy of the infratemporal and jugular fossae, along with proficiency in microsurgical techniques, is essential for removing such tumors while preserving cranial nerve functions and the patient's quality of life. https://thejns.org/doi/10.3171/CASE24396.

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Paragangliomas are the most common tumors at jugular foramen and pose a great surgical challenge. Careful clinical history and physical examination must be performed to adequately evaluate neurological deficits and its chronologic evolution, also to delineate an overview of the patient performance status. Complete imaging evaluation including MRI and CT scans should be performed, and angiography is a must to depict tumor blood supply and sigmoid sinus/internal jugular vein patency. Screening for multifocal paragangliomas is advisable, with a whole-body imaging. Laboratory investigation of endocrine function of the tumor is necessary, and adrenergic tumors may be associated with synchronous lesions. Preoperative prepare with alpha-blockage is advisable in norepinephrine/epinephrine-secreting tumors; however, it is not advisable in exclusively dopamine-secreting neoplasms. Best surgical candidates are young otherwise healthy patients with smaller lesions; however, treatment should be individualized each case. Variations of infratemporal fossa approach are employed depending on extensions of the mass. Regarding facial nerve management, we avoid to expose or reroute it if there is preoperative function preservation and prefer to work around facial canal in way of a fallopian bridge technique. If there is preoperative facial nerve compromise, the mastoid segment of the nerve is exposed, and it may be grafted if invaded or just decompressed. A key point is to preserve the anteromedial wall of internal jugular vein if there is preoperative preservation of lower cranial nerves. Careful multilayer closure is essential to avoid at most cerebrospinal fluid leakage. Residual tumors may be reoperated if growing and presenting mass effect or be candidate for adjuvant stereotactic radiosurgery.

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The jugular foramen (JF) is located between the temporal and occipital bones. The JF is a primary pathway for venous outflow from the skull and passage of nerves. Variations are common in this region and may have clinical and surgical implications. To analyze the sexual dimorphism and JF morphology in skulls from Northeastern Brazil. 128 human skulls from the Anatomy Laboratory of the Federal University of Paraíba, 64 male and 64 female, were selected and the JFs analyzed for bone septation and the presence of a dome. Data analysis considered P<0.05 as significant. On at least one side, complete septation was observed in 26 skulls (20.3%), incomplete septation in 93 skulls (72.6%) and 61 skulls (47.6%) did not present septation. In 114 skulls (89%), 47.6% female and 41.4% male, have a unilateral presence of the dome and 71 (55.4%) have it bilaterally. Posterolateral compartment diameters and JF area had higher values on the right side in the total sample and separated by sex (P<0.05). Most morphometric variables of the anteromedial compartment were higher in male than in female (P<0.05), fact that was not observed in the posterolateral compartment (P>0.05). This study showed a higher prevalence of complete septation in males compared to females. Morphometric analysis presented a peculiar morphology of the JF in this study. These results suggests that the surgical approach to diseases that affect the JF may be peculiar to the studied population, confirming the importance of morphological analysis of the skull base.

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Abstract Introduction The surgical management that achieves minimal morbidity and mortality for patients with glomus and non-glomus tumors involving the jugular foramen (JF) region requires a comprehensive understanding of the complex anatomy, anatomic variability, and pathological anatomy of this region. Objective The aim of this study is to propose a rational guideline to expose and preserve the lower cranial nerves (CNs) in the lateral approach of the JF. Methods The technique utilized is the gross and microdissection of 4 fixed cadaveric heads to revise the JF's surgical anatomy and high part of the carotid sheath compared with surgical cases to understand and preserve the integrity of lower CNs. The method involves radical mastoidectomy, microdissection of the JF, facial nerve, and high neck just below the carotid canal and the JF. The CNs IX, X, XI, and XII are microscopically dissected and kept in sight up to the JF. Results This study realized well the surgical and applied anatomy of the lower CNs with relation to the facial nerve and JF. Conclusions The JF anatomy is complicated, and the key to safely operate on it and preserving the lower CNs is to find the posterior belly of the digastric muscle, to skeletonize the facial nerve, to remove the mastoid tip preserving the stylomastoid foramen, to skeletonize the sigmoid sinus and posterior fossa dura not only anterior but also posteroinferior to reach and drill the jugular tubercle.

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Introduction The surgical management that achieves minimal morbidity and mortality for patients with glomus and non-glomus tumors involving the jugular foramen (JF) region requires a comprehensive understanding of the complex anatomy, anatomic variability, and pathological anatomy of this region. Objective The aim of this study is to propose a rational guideline to expose and preserve the lower cranial nerves (CNs) in the lateral approach of the JF. Methods The technique utilized is the gross and microdissection of 4 fixed cadaveric heads to revise the JF's surgical anatomy and high part of the carotid sheath compared with surgical cases to understand and preserve the integrity of lower CNs. The method involves radical mastoidectomy, microdissection of the JF, facial nerve, and high neck just below the carotid canal and the JF. The CNs IX, X, XI, and XII are microscopically dissected and kept in sight up to the JF. Results This study realized well the surgical and applied anatomy of the lower CNs with relation to the facial nerve and JF. Conclusions The JF anatomy is complicated, and the key to safely operate on it and preserving the lower CNs is to find the posterior belly of the digastric muscle, to skeletonize the facial nerve, to remove the mastoid tip preserving the stylomastoid foramen, to skeletonize the sigmoid sinus and posterior fossa dura not only anterior but also posteroinferior to reach and drill the jugular tubercle.

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Context Tumors of the jugular foramen present a challenge to skull base surgeons. Their rarity, coupled with the complex anatomy of the region require exquisite knowledge and surgical technique. We present the history of the craniocervical approach to the jugular foramen, as well as surgical advice on how to manage the different extensions these tumors may present. The surgical nuances come from the experience of our skull base team managing over 150 tumors of the jugular foramen over the past 30 years. The history of the craniocervical approach was obtained by reviewing articles on surgery of the jugular foramen published on PUBMED over the past 100 years. History The first craniocervical approach may be attributed to Gardner et al. in 1891, with posterior contributions from Shapiro and Neues, Gejrot, Kempe et al, Hilding and Greenberg, and Glasscock. Nuances Cervical dissection with identification of the jugular vein, carotid bifurcation, and IX to XII cranial nerves was performed. Mastoidectomy with exposition of the fallopian canal, labyrinth, middle ear, sigmoid sinus, followed by a trans-sigmoid craniotomy with transposition of the vertebral artery gave access to the temporal and intracranial region. Conclusion Approaching the jugular foramen is the epitome of skull base surgery. Several modifications of the standard approach may be necessary depending on the extension of the tumor.

Contexto Os tumores do forame jugular apresentam um desafio para os cirurgiões da base do crânio. A sua raridade e a anatomia complexa da região requerem conhecimento específico da técnica cirúrgica. O presente manuscrito apresenta a história do acesso crâniocervical ao forame jugular, assim como dicas cirúrgicas para manejar as diferentes extensões que os tumores podem apresentar. As dicas são derivadas da experiência da nossa equipe em mais de 150 tumores do forame jugular nos últimos 30 anos. A história do acesso crâniocervical foi obtida da revisão de artigos em PUBMED dos últimos 100 anos. História A primeira abordagem craniocervical pode ser atribuída a Gardner et al. em 1891, com contribuições posteriores de Shapiro e Neues, Gejrot, Kempe et al, Hilding e Greenberg e Glasscock. Descrição A dissecção cervical necessita identificação da veia jugular, bifurcação carotídea, e dos nervos cranianos de IX a XII. A mastoidectomia com exposição do canal de falópio, labirinto, ouvido médio e seio sigmoide foi seguida por uma craniotomia transsigmoide com transposição da artéria vertebral, permitindo abordar a região temporal e intracraniana. Conclusão Acessar o forame jugular é o epítome da cirurgia da base do crânio. Múltiplas modificações do acesso tradicional podem ser necessárias dependendo da extensão do tumor.

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BACKGROUND: Jugular foramen paragangliomas (JFP) treatment represents a challenge for surgeons due to its close relationship with facial nerve (FN), lower cranial nerves (LCN), and internal carotid artery. Due to its hypervascularization, preoperative tumor embolization has been indicated. METHODS: Retrospective analysis of the clinical evolution of 26 patients with JFP class C/D previously embolized treated through infratemporal/cervical access without FN transposition. RESULTS: Total and subtotal resections were 50% each, regrowth/recurrence were 25%, and 23%, respectively, and mortality was 3.9%. Postoperatively, 68.4% of patients had FN House and Brackmann (HB) Grades I/II. New FN deficits were 15.4% post embolization and 30.7% postoperatively. Previous FN deficits worsened in 46.1%. Tumor involved the FN in 30.8% and in 62.5% of them these nerves were resected and grafted (60% of them had HB III). Lateral fall, ear murmur, and vertigo improved in all patients. Tinnitus improved in 77.8% and one patient developed tinnitus after surgery. Hearing loss did not improve, eight partial hearing loss remained unchanged and four worsened. New postoperative LCN deficits were 64.3%. Postoperative KPS between 80 and 100 dropped 8.3%. Two patients with secretory paragangliomas with arterial hypertension difficult to control had better postoperative blood pressure control. CONCLUSION: Although still with significant morbidity due to FN and LCN injuries, the treatment of patients with JFP Fisch C/D has good long-term results. Surgical techniques without FN transposition have less intraoperative nerve damage, lower rates of total resection, and higher recurrence. Preoperative embolization of JFP reduces the intraoperative blood loss but can cause FN deficit.

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INTRODUCTION: Anomalous intracranial venous anatomy is described in patients with syndromic craniosynostosis and is of significant importance when it comes to surgical morbidity. However, it is still controversial its origin, type of circulation in each syndrome, how it behaves over time, when it can be interrupted and wether it needs to be studied. The purpose of this paper is to discuss these issues by reviewing the literature. METHODS: A literature search was performed using the PubMed database with a focus on papers including detailed descriptions of the venous outflow in complex and syndromic craniosynostosis. Search details used were the following: ("veins"[MeSH Terms] OR "veins"[All Fields] OR "venous"[All Fields]) AND ("abnormalities"[Subheading] OR "abnormalities"[All Fields] OR "anomalies"[All Fields]) AND syndromic[All Fields] AND ("craniosynostoses" [MeSH Terms] OR "craniosynostoses"[All Fields] OR "craniosynostosis"[All Fields]). Studies that exposed details of venous anomalies found in syndromic or complex craniosynostosis were selected. RESULTS: Of a total of 211 articles found, 11 were selected for this review. Of these, 5 were case reports, 5 retrospective studies, and only 1 prospective study. From the 6 series of cases presented, 5 discussed the relationship between jugular foramen stenosis (JFS) and collateral venous drainage. The authors discuss data from the literature for each leading question presented: 1-collateral circulation: is it an intrinsic trouble, a consequence of stenosis of the cranial base foramina or related to raised intracranial pressure (ICP)?; 2-what venous anomalies should we search for, and what is the best exam to study them?; 3-collateral circulation changes with time?; 4-can neurosurgeons interrupt the collateral circulation?; 5-should we study all complex types of craniosynostosis? CONCLUSION: The importance of the study of the venous outflow in patients with complex craniosynostosis is evident in the literature. The real relationship between intracranial hypertension, hypoplastic skull base foramen, Chiari I malformation, hydrocephalus, and venous collateral circulation remains unknown. Prospective studies focusing on molecular biology analysis will possibly solve all of these leading questions.

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SUMMARY: The objective of this study was to determine the occurrence of anatomical variants in the exocranial surface of the jugular foramen, specifically, the presence of single or double and complete or incomplete septation. A cross-sectional anatomical study was performed using 96 Brazilian dry human skulls (53 male and 43 female). One examiner determined the number (single or double) and type (i.e. complete or incomplete) of osseous septation at the outer surface of jugular foramens. Data went through statistical analysis on GraphPad Prism 6.01. Our results shown that Male individuals where more likely to present normal jugular foramens (male = 71.69%, female = 34.88%; p = 0.003). However, one incomplete septation occurred more often on the right side of female individuals (1 incomplete septation, male = 16.98%; 1 incomplete septation, female = 34.88%; p = 0.044). Similarly, one complete septation (i.e. the presence of two fully divided jugular compartments) also occurred more often on the right side of female individuals (1 complete septation, male = 9.43%; 1 complete septation, female = 25.58%; p = 0.038). Anatomical variants of the jugular foramen regarding single or double complete or incomplete septations were more likely to be found on the right side of female individuals, whose also presented a higher rate of jugular foramens with any type of septation than regular non-altered jugular foramens.

RESUMEN: El objetivo de la presente investigación fue determinar la presencia de variaciones anatómicas en la superficie exocraneal del foramen yugular, especificamente, la presencia de septos únicos o dobles, completos o incompletos. El estudio fue realizado en 96 cráneos secos (53 masculinos y 43 femeninos) de indivíduos Brasileños. Se determinaron septos óseos completos o incompletos y número de ellos. Los resultados obtenidos fueron tratados estadísticamente con el programa GraphPad Prism 6.01. Los sujetos de sexo masculino fueron más propensos a presentar forámenes yugulares normales (sexo masculino: 71,69%; sexo femenino: 34,88%, p= 0,003). Sin embargo, se observaron septos incompletos con mayor frecuencia en el lado derecho y en el sexo femenino (sexo masculino: 16,98%; sexo femenino: 34,88%, p=0,044). Adicionalmente, una septación completa (presencia de dos compartimientos yugulares, divididos completamente), se presentaron más frecuentemente en el lado derecho de indivíduos femeninos (sexo masculino: 9,43%; sexo femenino: 25,58%, p= 0,038). Las variantes anatómicas del foramen yugular, en relación a septos simples o dobles, completos o incompletos, se encontraron con mayor frecuencia en el lado derecho de las mujeres, las que presentaron un alto rango de forámenes yugulares con algún tipo de septos respecto a los forámenes yugulares regulares no alterados.

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The jugular foramen remains one of the most complex regions of the human body. Approaching lesions in this area requires extensive anatomical knowledge and experience, due to the many critical neurovascular structures passing through or around the jugular foramen. Here, we present a concise review of the microsurgical anatomy of the jugular foramen in relation to the craniocervical approach.

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Glomus tumors, also called paragangliomas, are challenging lesions, demanding accurate knowledge of complex anatomy and pertinent approaches. We present the case of a 39-year-old male presenting with headache, vertigo, tinnitus, hearing loss, and hoarseness. Neurological assessment showed facial paralysis House-Brackmann IV and lower cranial nerves deficits. Preoperative magnetic resonance imaging (MRI) demonstrated two large lesions, suggestive of a glomus jugulare, and carotid body paragangliomas. Considering worsening of the symptoms and the important mass effect of both lesions over the neurovascular structures, microsurgical excision was offered, after preoperative tumor embolization. We preferred to approach both lesions in the same operation, starting by the cervical tumor. Initially there was not an easily identifiable dissection plane between the tumor and the carotid artery, but it was achieved after performing a subadventitial dissection, being possible to resect the entire lesion. The jugular foramen lesion was approached through a postauricular transtemporal approach, skeletonizing the sigmoid sinus, jugular bulb, and facial nerve, following a complete mastoidectomy. The tumor, extending to the intradural compartment, middle ear, internal auditory canal, petrous internal carotid artery, and internal jugular vein was completely removed. Postoperative MRI demonstrated complete resection of both lesions, and pathology confirmed to be paragangliomas. In the immediate postoperative period, the facial paralysis evolved to House-Brackmann grade VI, improving to grade III during follow-up. The patient underwent a vocal cord medialization in order to improve voice quality and swallowing. These are challenging lesions and extensive laboratory training is mandatory to be familiarized with the regional anatomy and its various surgical approaches. The link to the video can be found at: https://youtu.be/gA_ckwFq_9c .

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BACKGROUND: The retrosigmoid suprajugular approach provides a less-aggressive approach for a subset of tumors of the jugular foramen. METHOD: We described the retrosigmoid suprajugular approach with its advantages, caveats, and indications. A Samii-B2 glossopharyngeal nerve schwannoma is shown to exemplify the procedure. CONCLUSION: The retrosigmoid suprajugular approach provides an excellent option for tumors with a variable extension into the cerebellopontine cistern and limited extension into the jugular foramen. It is less destructive than the other approaches and allows a good exposure to the posterior part of the jugular foramen.

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Introduction Preserving cranial nerve (CN) function during tumor removal at the jugular foramen is challenging. No anatomical study has better defined the relevant dural septations on the inner surface of the jugular foramen. This study was undertaken to elucidate this anatomy. Methods Fourteen cadaveric heads (28 sides) were dissected, and relationships of the meningeal coverings of the jugular foramen and adjacent CNs documented. A classification scheme was created to better describe the dural septations of the inner surface of the jugular foramen. Results Four types of dural septations were noted. Type I: 10 sides (36%) where a dural septation was seen between CNs IX anteriorly and X and XI posteriorly. Of these, the septum was ossified in 20%. Type II (32%) was defined as a jugular foramen with no dural septation. Type III (7%) was defined as septation between CNs IX and X anteriorly and XI posteriorly. Type IV (7 sides, 25%) or the chaotic form was defined as multiple septations within the jugular foramen that housed and divided CN rootlets. Conclusions The dural septations defined here can be used in future studies to help correlate operative strategy to meningeal morphology within the jugular foramen.

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The jugular foramina (JF) are bilateral openings situated between the lateral part of the occipital bone and the petrous part of the temporal bones in the human skull. It is a bony canal transmitting neurovascular structures from the posterior cranial fossa through the base of the skull to the carotid space. Since the JF depicts variations in shape, size, height and volume between different racial and gender groups, along with distinctive differences in laterality from its intracranial to extracranial openings, knowledge of the JF may be necessary to understand intracranial pathologies. Therefore, the purpose of this study was to evaluate the morphometric measurements of the jugular foramen. Various morphometric parameters of the JF and its relation to surrounding structures were measured and assessed in 73 dry skull specimens (n=146). Each of the morphometric parameters measured were statistically analyse using SPSS to determine the existence of a possible relationship between the parameters and sex, race, age and laterality. The comparisons of sex and age with the distance between the JF and lateral pterygoid plate and distance between the JF and foramen magnum yielded statistically significant p values of 0.0049 and 0.036, respectively. The results of this study correlated with that of previous studies indicating that measurements regarding the JF are greater on the right side. The provision of morphometric data pertaining to the JF and surrounding structures may assist surgeons and clinicians during operative procedures.

Los forámenes yugulares (FY) son aberturas bilaterales situadas entre la parte lateral del hueso occipital y la porción petrosa del hueso temporal del cráneo humano. Se trata de un canal óseo que da paso a estructuras neurovasculares de la fosa craneal posterior a través de la base del cráneo hasta el espacio carotídeo. Ya que el FY representa variaciones en la forma, tamaño, altura y volumen entre diferentes grupos raciales y sexo, además de las diferencias distintivas en la lateralidad desde sus aperturas intracraneales a las aperturas extracraneales, se hace necesario su conocimiento con el objetivo de comprender las patologías intracraneales. El propósito de este estudio fue evaluar las mediciones morfométricas del FY. Se midieron y evaluaron muestras de 73 cráneos secos, bilateralmente (n=146). Se estudiaron varios parámetros morfométricos del FY y su relación con las estructuras circundantes. Cada uno de los parámetros morfométricos medidos fue analizado estadísticamente con el programa SPSS para determinar la existencia de una posible relación entre los parámetros y sexo, raza, edad y lateralidad. Las comparaciones de sexo y edad con la distancia entre la placa pterigoidea lateral y el FY, y la distancia entre el FY y el foramen magno, arrojaron valores de p significativos de 0,0049 y 0,036, respectivamente. Los resultados de este estudio se correlacionaron con estudios previos que indican que las mediciones relativas al FY son mayores en el lado derecho. El suministro de datos morfométricos relativos al FY y las estructuras circundantes puede ayudar a los cirujanos y clínicos durante los procedimientos quirúrgicos.

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Objective To compare the right and left sides of the same skulls as far as the described landmarks are concerned, and establish the craniometric differences between them. Method We carried out measurements in 50 adult dry human skulls comparing both sides. Results The sigmoid sinus width at the sinodural angle level was larger on the right side in 78% of the cases and at the level of the digastric notch in 72%. The jugular foramen width was also larger on the right side in 84% of the cases. The sigmoid sinus distance at the level of the digastric notch was larger on the right side in 64% of the cases, and the sigmoid sinus distance at the level of the digastric notch to the jugular foramen was larger on the right side in 70% of the cases. Conclusion Significant craniometric differences were found between both sides of the same skulls. .

Objetivo Comparar os lados direito e esquerdo no mesmo crânio nos pontos referenciais descritos e definir as diferenças craniométricas entre ambos. Método Realizamos mensurações em 50 crânios secos de humanos adultos comparando os lados direito e esquerdo. Resultados Como resultado, obtivemos as medidas da largura do seio sigmóideo na altura do ângulo sinodural maiores no lado direito em 78% dos casos e na altura do ponto digástrico em 72%. A largura do forame jugular foi também maior no lado direito em 84% dos casos. A distância do seio sigmóideo na altura do ângulo sinodural até a altura do ponto digástrico foi maior do lado direito em 64% dos casos, e a distância do seio sigmóideo na altura do ponto digástrico até o forame jugular foi maior do lado direito em 70% dos casos. Conclusão Diferenças craniométricas significativas foram encontradas entre os dois lados do crânio. .

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A case of unusual anatomical variation of the jugular foramen (JF) with doubled posterior condylar canal (PCC) is reported. According to the presence of bridging, the JF can be defined as Type I (one septation, two compartments) on the right side and Type IV (three septations, four compartments) on the left side. The dome of the jugular fossa is present on the right, absent on the left. The jugular foramen shows a canal-like structure with an external and an internal opening. The lengths of the longest and widest axes of the JFs are measured as 21.93 x 16.56 mm on the right and 16.75 x 15.14 mm on the left side. The right JF is larger. The PCC is doubled on the right side and there is only one on the left side. It is essential not only to know compartments per se but also to know the structures passing through the compartments, in order to achieve desired surgical outcomes and avoid complications.

Es reportado el caso de una inusual variación anatómica del foramen yugular (FY), con el canal condilar posterior duplicado (CCP). De acuerdo con la presencia de los puentes, el FY se puede definir como Tipo I (una tabicación, dos compartimientos) en el lado derecho y Tipo IV (tres tabiques, cuatro compartimientos) en el lado izquierdo. El domo de la fosa yugular está presente en el lado derecho, y ausente en el izquierdo. El foramen yugular mostró una estructura igual a un canal con una apertura externa y otra interna. Las longitudes de los ejes más largos y anchos de la FY fueron 21,93x16,56mm al lado derecho y 16,75x15,14mm al lado izquierdo. El FY derecho fue más grande. El CCP se observó duplicado en el lado derecho y único en el lado izquierdo. Es esencial no sólo conocer los compartimientos en sí, sino también las estructuras que pasan a través de los compartimientos con el fin de lograr los resultados deseados y evitar las complicaciones quirúrgicas.

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The jugular foramen (JF) lies between the occipital bone and the petrosal portion of the temporal bone, andit allows for the passage of important nervous and vascular elements, such as the glossopharyngeal vagusand accessory nerves, and the internal jugular vein. Glomic tumors, schwannomas, metastatic lesions andinfiltrating inflammatory processes are associated with this foramen, which can account for injuries of relatedstructures. Variatons of the JF were already reported regarding shape, size and laterality in one only skull,besides differences related to sex, race and laterality domain, which makes the study of these parameters in thepopulation of southern Brazil significant. Objective: this paper wants to conduct the morphometric analysisof the JF of 111 dry skulls belonging to males and females. Results: the latero-medial the anteroposteriormeasurements showed significant differences when genera were compared and side was compared, respectively.Of the total amount of the investigated skulls, 0.9% showed a complete septum on both sides; 0.9% showedincomplete septum, and 83.8% lacked the septum. The presence of a domed bony roof was noticed in 68.5%of skulls on both sides. Conclusion: the obtained results presented variations regarding some parameterswhen compared to previous studies, thus making it evident the significance of race in the morphometricmeasurements and characteristics of the JF, besides the relevance of studying the kind of impairment whichcan jeopardize important functions, as the cardiac innervation of the vagus nerve.

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Introdução: As lesões do forame jugular são raras, sendo os paragangliomas os tumores mais comuns desta região seguidos pelos schwannomas. Os schwannomas são tumores benignos de crescimento lento e origem principalmente nos nervos cranianos sensitivos. Quando localizados no forame jugular, apresentam-se clinicamente com perda auditiva, ataxia e cefaleia, sendo comuns as alterações dos nervos cranianos IX, X e XI. A excisão cirúrgica é a opção terapêutica de escolha. Relato do Caso: Relatamos o caso de um paciente adulto jovem que procurou o serviço de Otorrinolaringologia do Hospital Universitário de Brasília com quadro de otalgia e otorreia piossanguinolenta no ouvido esquerdo, associada a zumbido, hipoacusia e tumor no conduto auditivo externo. Iniciada investigação diagnóstica com tomografia computadorizada e biópsia, ambas com resultados inconclusivos. Realizadas então nova biópsia e ressonância nuclear magnética, com diagnóstico definitivo de schwannoma do foram jugular. Na ocasião a lesão foi definida como tipo D pela classificação de Kaye-Pellet, por envolver o forame jugular, apresentar formato de halter e componentes intra e extracraniano. Realizada cirurgia por craniotomia com excisão parcial do tumor. Não houve sequelas do procedimento, apesar da persistência dos sintomas otológicos. Comentários Finais: O caso é um exemplo de uma doença rara, com pouco mais de duzentos casos relatados na literatura, e apresentação clínica incomum. O desafio diagnóstico e terapêutico é enfrentado pelas equipes de Otorrinolaringologia e Neurocirurgia, que programam para breve nova abordagem cirúrgica combinada para extirpação completa do tumor.

Introduction: Lesions of the jugular foramen are uncommon, and the paragangliomas are the most common tumors in this region, followed by schwannomas. Schwannomas are benign tumors, of slow growth and origin mainly in the sensitive cranial nerves. When located in the jugular foramen, the patients present with clinically auditory hearing loss, ataxia and headache, and cranial nerves IX, X and XI alterations are also common. Surgical excision is the therapeutic choice. Case Report: We reported the case of a young adult patient who sought the Otorhinolaryngology service at the School Hospital of Brasília for investigation of otalgia and chronic infection in the left ear, associated with tinnitus, hypacusis and tumor in the external ear canal. Initial diagnostic investigation by computed tomography and biopsy was inconclusive. New biopsy and magnetic resonance imaging were performed and definitive diagnosis of jugular foramen schwannoma was given. The lesion was then defined as type D by the classification of Kaye-Pellet, for it involved the jugular foramen, presented format of dumbbell and intra and extracranial components. The patient underwent a craniotomy and partial excision of the tumor. He did not have sequels of the procedure, although the otologic symptoms persisted. Final Comments: This case is an example of a rare disease. No more than two hundred cases have been reported in the literature and the clinical presentation is uncommon. The diagnostic and therapeutic challenge is faced by Otorhinolaryngology and Neurosurgery teams who are scheduling a new combined surgical procedure for a complete excision of the tumor.

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