Surgical management of pulsatile tinnitus secondary to jugular bulb or sigmoid sinus diverticulum with review of literature

Introduction

Jugular bulb and sigmoid sinus anomalies are well-known causes of vascular pulsatile tinnitus. Common anomalies reported in the literature include high-riding and/or dehiscent jugular bulb, and sigmoid sinus dehiscence. However, cases of pulsatile tinnitus due to diverticulosis of the jugular bulb or sigmoid sinus are less commonly encountered, with the best management option yet to be established. In particular, reports on surgical management of pulsatile tinnitus caused by jugular bulb diverticulum have been lacking in the literature.

The effect of jugular bulb–vestibular aqueduct dehiscence on hearing and balance

Conclusion: This study suggests that jugular bulb–vestibular aqueduct dehiscence (JBVAD) does not affect the hearing or balance functions of the inner ear. Objectives: Since JBVAD may cause impairment of the inner ear and induce hearing loss, tinnitus, and vertigo, this study was to investigate the effect of JBVAD on hearing and balance. Method: Patients undergoing temporal bone computed tomography (TBCT) scans from May 2013 to December 2013 at a tertiary referral hospital were reviewed. The topographic relationship between the jugular bulb (JB) and the vestibular aqueduct was assessed. The findings were classified as normal when there was always a bony structure between the two structures and as dehiscent when the bony coverage separating the two structures was absent. Clinical data were collected via electronic records and clinical follow-up. Results: A total of 1313 out of 8325 patients were diagnosed with a HJB. Forty-six patients showed JBVAD. The prevalence of dehiscence was 0.6% in patients undergoing temporal bone CT scans and 3.5% in patients with HJB. Of the 46 patients with JBVAD, 23 (50%) had sensorineural hearing loss, three (6.5%) had tinnitus, and two (4.3%) had vertigo. The correlation between JBVAD and these clinical symptoms did not achieve statistical significance.     

Audiological signs in pediatric cases with dehiscence of the bony labyrinth caused by a high jugular bulb

Clinical findings were assessed in three pediatric cases of dehiscence of the bony labyrinth caused by a high jugular bulb (HJB). Two children had two dehiscent lesions, which included posterior semicircular canal dehiscence (PSCD) and vestibular aqueduct dehiscence (VAD). One child had VAD alone. Two subjects with PSCD, but not with VAD alone, had mixed hearing loss and showed wave motion of the baseline on tympanometry and acoustic reflex testing, and a reduced response on otoacoustic emission. These findings may reflect jugular venous pulsations transmitted through the PSC and represent characteristics of cases with PSCD caused by HJB.     

高位颈静脉球及术中出血的处理

目的：研究慢性中耳炎行鼓室成形术的患者高位颈静脉球（HJB）的发生率及HJB裸露并发术中破裂出血的处理。方法：对2005年1月～2006年8月287例慢性中耳炎行鼓室成形术的患者进行回顾性研究,结合高分辨率CT（HRCT）所见及术中处理加以分析。结果：287例慢性中耳炎患者中,HRCT检查发现HJB65例,左侧14例,右侧31例,双侧20例。术中发现颈静脉球顶部骨壁缺损5例,其中1例发生颈静脉球出血,占0．35％（1／287）。以胶原蛋白海绵片封闭静脉裂口,再以合适大小颞肌肌瓣填压后出血控制效果好。发生颈静脉球出血者经止血后,顺利完成了清除病灶、听骨链重建等全部手术步骤。结论：鼓室成形术术前需注意颈静脉球情况,手术中遇到HJB,尤其在颈静脉球与下鼓室间骨壁缺损时易发生颈静脉破裂出血时,颞肌肌瓣及胶原蛋白海绵片是较好的止血材料,同时短暂控制性低血压及术中冷静、及时、恰当的处理也是减少出血、保证安全的重要措施,并可继续完成既定手术。     

Pulsatile tinnitus arising from jugular megabulb deformity: A treatment rationale

Pulsatile tinnitus is a rare presenting symptom in patients with enlarged jugular bulbs. We will describe three young women presenting with right pulsatile tinnitus associated with a megabulb deformity of the temporal bone. After extensive radiologic and audiologic evaluation, no vascular or bony abnormalities could be identified. All three patients demonstrated pulsatile bruits over the right temple. Pulsatile tinnitus disappeared with ipsilateral jugular compression suggesting flow rather than pressure to be responsible for abnormal auditory symptoms. This observation formed the basis of our treatment recommendations. Because of progressively debilitating pulsatile tinnitus, two patients elected right internal jugular vein ligations under local anesthesia. Both patients were relieved of tinnitus. We conclude that jugular vein ligation can be a safe and effective surgical treatment for pulsatile tinnitus resulting from a jugular megabulb deformity, provided two criteria are met: 1, The presence of an expanding tumor is ruled out. 2. The presence of contralateral venous drainage is established by angiography or brain scan. Anatomic and functional hemodynamic considerations of brain circulation will be emphasized in the discussion of our treatment rationale.     

颈内静脉源性耳鸣的诊断和治疗

目的 :探讨颈内静脉哼鸣性耳鸣的诊断途径与治疗方法。方法 :4例搏动性耳鸣患者经耳镜及全身体检 ,听力学、电生理、超声及影像学检查确诊为颈内静脉哼鸣性耳鸣 ,进行了颈内静脉结扎术。结果 :3例耳鸣完全消失 ,1例明显减轻 ,不影响日常生活。结论 :颈内静脉哼鸣性耳鸣是一种无明显器质性病变的客观性耳鸣 ,但有时可严重干扰患者的正常生活。一经确诊 ,可行颈内静脉及其属支结扎、切断术。可获迅速和永久的治疗效果。     

Surgical treatment of the high jugular bulb by compressing sinus sigmoideus: two cases

If the jugular bulb normally surrounded by a bony layer in jugular fossa is anatomically over the inferior surface of the bony annulus, in the middle ear or over the basal turn of cochlea, it is then named as high jugular bulb (HJB). It may be dehiscent or aberrant. It is reported to occur in 5% of the temporal bone specimens. In accordance with the literature jugular bulb compression, jugular vein ligation and embolization are suggested in such cases. In both of the presented cases, there was bleeding from jugular bulb during surgery and jugular bulb was compressed with bone wax and Surgicel, but sigmoid sinus has been compressed after failure to stop bleeding through jugular bulb compression. Venous MR angiographies showed no flow in postoperative controls. Although it is very rarely seen clinically, we present two HJB cases and different treatment perspectives accompanied by literature.     

Petrous high jugular bulb: a histological study

PURPOSE: A high jugular bulb (JB) is thought to affect structures of the inner ear and possibly cause symptoms there, but clear histological findings of an anatomical relationship between a high JB and the inner ear have not yet been described. MATERIALS AND METHODS: We surveyed horizontal sections of 1,591 temporal bones from the collection of the Otopathology Laboratory at the University of Minnesota in Minneapolis, Minnesota, defining a high JB as a JB extending above the inferior margin of the basal cochlear turn. RESULTS: In 65 specimens (16%), we found a high JB with its vascular wall obviously thinner than that of a low JB. Bony resorption was occasionally observed around high JBs. Sixteen specimens showed a bony deshiscence between the JB and the endolymphatic sac. Clinical charts showed no obvious symptoms associated with a high JB. CONCLUSIONS: Our findings suggest that the JB may have potential to expand upward postnatally. Although our study confirmed occasional bony dehiscence between the JB and the endolymphatic sac, JBs with this involvement may have only a minor effect on function in the inner ear.     

颞骨解剖变异的高分辨率CT研究

目的 分析颞骨解剖变异发生率,为耳科手术提供参考依据。方法 回顾性分析健康志愿者（76例152耳）和病理证实慢性化脓性中耳炎患者（66例96耳）颞骨横断面、冠状面高分辨率CT（HRCT）,统计以下解剖变异发生率：①颈静脉窝高位;②颈静脉球裸露;③乙状窦前位：④颅中窝脑板低位;⑤鼓室盖不连续;⑥鼓窦缺如;⑦异位颈内动脉：⑧大鼓室窦。结果 正常对照组、病变组、气化良好组和气化不良组中,颈静脉窝高位发生率右侧均高于左侧（P〈0．05）,发生率分别为46．0％和26．2％。病变组乙状窦前位（32．3％）发生率高于正常对照组（15．8％,P〈0．05）,气化不良组（43．5％）高于气化良好组（11．3％,P〈0．05）。气化良好组鼓室盖不连续（18．0％）发生率高于气化不良组（8．7％,P〈0．05）。颈静脉球裸露、颅中窝脑板低位、鼓窦缺如、异位颈内动脉、大鼓室窦发生率在组内和组间均无显著性差异（P〉0.05）,发生率分别为2．0％、21．8％、1．2％、4‰、8‰。结论 术前HRCT可明确颞骨解剖变异,对减少手术并发症发生率有重要临床价值。     

Another etiology for vertigo due to idiopathic lateral semicircular canal bony defect

Superior semicircular canal dehiscence syndrome (SSCDS) is a set of symptoms, related to vestibular and/or auditory, which dues to a dehiscence of bone at the superior semicircular canal. We reported an extremely rare case with idiopathic bony dehiscence at the lateral semicircular canal, which presents the similar symptoms with SSCDS, and performed a pertinent literature review. A 76-year-old male patient complained experiencing vertigo and autophony caused by loud noises, with a sign of horizontal eye movements for the stimuli of loud noise to his right ear. HRCT scanning presented a solitary 2.0 mm bony defect at the right lateral semicircular canal. For a chief complaint of sound-/pressure-induced vertigo and/or oscillopsia and/or auditory symptoms, semicircular canal dehiscence syndrome should be considered. Then the doctor could presume the location of the dehiscence via the way of the oscillopsia. If the dehiscence lying the lateral semicircular canal, in clinical radiology, both axial and coronal images to be produced though the lateral semicircular canal plane in a parallel and perpendicular way should be carefully assessed for making diagnosis.     

Anatomical study of the superior jugular bulb by use of high-resolution computed tomography

Using high-resolution computed tomography, the relationship between the superior jugular bulb and the tympanic cavity was analysed in 40 subjects. Until now, the superior jugular bulb was thought to lie below the floor of the hypotympanum, however, the following observations were made from this study: 1) In 48 (60%) out of 80 temporal bones (40 cases), the top of the superior jugular bulb was situated in the higher level than the floor of the hypotympanum. 2) In 9 cases, the jugular bulb was separated from the tympanic cavity by only thin bony wall. This finding was noticed on the right side in 8 cases and was bilateral in a case. 3) In 21 cases (52.5%), the right jugular bulb was situated in higher level than the left, and the reverse finding was noticed only in 4 cases (10.0%). The reason why the right jugular bulb is frequently higher than the left was thought to be related to its function. Namely, the jugular bulb is useful to absorb the pulsation of the carotid artery, therefore, it was considered that the anatomical and physiological differences between the right and left carotid arteries induce the asymmetry of the jugular bulbs.     

A high placed jugular bulb in the middle ear: A clinical and temporal bone study

Anatomically, the superior boundary of the jugular bulb lies below the floor of the hypotympanum of the middle ear space. In a rare instance, it can extend upward, elevate the floor of the hypotympanum and present in the middle ear. This elevation is an anomaly well known but rarely witnessed or documented in the literature. This may result in serious hemorrhage at the time of surgery if unrecognized by the surgeon and if it is injured during temporal bone surgery. Two hundred fifty-seven histologically prepared temporal bones were examined microscopically for anomalous positions of the jugular bulb. This survey represents 189 patients. Thirteen temporal bones were found in which the jugular bulb extended into the middle ear space above the inferior rim of the bony annulus. Although this anatomical variation occurs infrequently in the general population, a 6 percent incidence of high placed jugular bulb in the middle ear was found in the temporal bones studied in this series.     

Surgical treatment of the high jugular bulb in patients with Ménière’s disease and pulsatile tinnitus

The aim of this retrospective study was to evaluate the functional results of surgical lowering of the high jugular bulb in the treatment of patients with Ménière’s disease and pulsatile tinnitus. Fifteen patients with disabling Ménière’s disease associated with pulsatile tinnitus and a high and medial jugular bulb were included in this study. As treatment a complete mastoidectomy was performed, after which the jugular bulb was freed by an infralabyrinthine and subfacial approach. The bulb was then displaced downwards with surgical wax. Functional results of surgery were assessed by a questionnaire according to the 1995 guidelines of the United States American Academy Committee on Hearing and Equilibrium, audiometric and vestibular tests, and by magnetic resonance and computed tomographic imaging with vascular sequences. Surgical treatment was contraindicated in two cases: one had hypoplasia of the contralateral sigmoid sinus and the other a small petrous hemangioma located around the jugular bulb that was discovered peroperatively. Among the 13 patients treated by definitive surgery, attacks of vertigo were reported as disabling in 12 cases preoperatively (92%) versus 1 (8%) after surgical treatment. No significant change in hearing was observed after surgery. Tinnitus had been reported in all patients preoperatively and decreased in intensity in four (31%) and disappeared in three (23%) after surgery. Received: 24 April 1998 / Accepted: 13 October 1998     

A rare case of the high jugular bulb associated with only hearing ear

The jugular bulb may be present in different positions and dimensions within the temporal bone. In general, high jugular bulbs were classified into 2 types: lateral in which the jugular bulb protrudes into the middle ear and up into the tympanic cavity and medial in which the jugular bulb is abnormally placed more superiorly and medial to the cochlea. We report, a unique case of a high jugular bulb which came round from behind of the internal auditory canal and the cochlea protruding into the posterosuperior part of the mesotympanum. It was a very rare pattern of a high jugular bulb which varies in position. The occurrence of adhesive otitis media caused the high jugular bulb to bleed easily in the only hearing ear. There would be risks of making the patient suffer severe bilateral healing impairment due to only one hearing ear and excessive hemorrhage in surgical treatment. With only one hearing ear, we should therefore select transcatheter interventional angiography when the quantity and frequency of bleeding from the jugular bulb increase so.     

Pulsatile tinnitus alleviated by contralateral neck compression: a case report

A case of a 58-year-old man with right pulsatile tinnitus originating from a small dural arteriovenous fistula (DAVF) of the jugular bulb is described. The tinnitus was alleviated by contralateral neck compression. This unusual observation ruled out venous pulsatile tinnitus, although a temporal bone CT scan showed a high jugular bulb. The fistula was confirmed by angiographic study. The patient was treated by transarterial embolization of the fistula and remains free of symptoms 1 year after treatment.     

Endovascular embolization of a hemorrhagic jugular bulb diverticulum

A jugular bulb diverticulum (JBD) is considered to be a rare venous anomaly. In exceptional cases it extends into the middle ear cavity with no bony covering, and slight oozing to massive bleeding might be encountered during routine middle ear surgical procedures. We report a case of massive bleeding from a dehiscent jugular bulb diverticulum that appeared during myringotomy and was successfully managed by endovascular embolization. This report emphasizes the value of interventional neuroradiology in otosurgical cases in which preoperative control of blood vessels or vascular malformations is crucial to avoid severe complications.     

A temporal bone study of the jugular fossa]

This study was performed to elucidate the anatomic variations of the jugular fossa (JF) on the basis of examination of 120 human temporal bones. Observations were made of temporal bones sectioned along a plane including the cochlea, the JF, and long axis of the internal auditory canal. The position of the upper margin of the JF was classified according to its relation to the tympanic cavity, the cochlea, and the internal auditory canal. The height of the upper margin of the JF was classified as follows. Low type; inferior to the level of the external auditory canal. Middle type; between the level of the external auditory canal and the cochlea. High type; superior to the level of the cochlea. The results revealed that 58 ears were of low type, 46 middle type, and 16 high type. Middle and high type comprised 62 ears, thus in 52% of ears the upper margin of the JF was situated superior to the external auditory canal. The relation between the JF and the cochlea was classified as follows. Medial type; medial to the medial margin of the cochlea. Lateral type; lateral to the medial margin of the cochlea. The results showed that 74 ears were of medial type, and 46 lateral type. Therefore, in 62% of ears the upper margin of the JF was situated medial to the medial margin of the cochlea. Forty-seven ears of medial type were of middle or high type. Ears of lateral type included none of high type. The jugular bulb diverticulum was observed in 32 ears, which consisted of 3 of low type, 13 middle type, 16 high type. When the upper margin of the JF was positioned higher, the JF was in a more medial position. However, no defect of the bony labyrinth was observed. In conclusion, it is considered that protrusion of the jugular bulb into the tympanic cavity was not caused by the abnormally high position of the JF, but by its lateral displacement. Distances from the JF to the surrounding structures were as follows; to the tympanic membrane 5.58 +/- 2.43mm (mean +/- S.D.), to the tympanic cavity 2.94 +/- 1.92mm, to the cochlea 4.93 +/- 2.20mm, and the internal auditory canal 5.82 +/- 2.38mm.     

Diagnosis and cure of venous hum tinnitus

Sounds arising from abnormalities of or abnormal communications between blood vessels in the neck or cranial cavity may result in objective tinnitus. It is audible to patient and examiner alike. Contrary to the usual subjective tinnitus of non-vascular origin, it is low pitched and pulsatile in character. That tinnitus which arises from and within the internal jugular vein is particularly important, as it may be loud enough to interfere with sleep, and result in some loss of hearing. Diagnosis is important as it can be cured by simple ligation of the internal jugular vein. Such a case is reported.     

Surgical treatment of posterior semicircular canal dehiscence syndrome caused by jugular diverticulum

Objective: We report a rare case of posterior semicircular canal dehiscence caused by a jugular diverticulum, and we describe its surgical treatment using a dehiscence resurfacing manoeuvre. Method: The clinical findings, surgical procedure and outcomes are presented. Results: A 66-year-old man presented with disequilibrium, sound-induced vertigo, a reduced ocular vestibular evoked myogenic potential threshold, and pressure-induced vertical and torsional nystagmus. Computed tomography revealed a right posterior semicircular canal dehiscence caused by a diverticulum of the jugular bulb. The defect in the posterior semicircular canal was localised and resurfaced with bone paté, temporalis muscle fascia and conchal cartilage, under direct visualisation. Post-operatively, the patient's symptoms disappeared and his ocular vestibular evoked myogenic potential threshold normalised. Conclusion: This case illustrates that posterior semicircular canal dehiscence can be surgically managed by resurfacing the defect site via a transmastoid approach.     

与侧颅底手术有关的颈静脉球区解剖研究

目的为了提高对侧颅底手术中颈静脉球空间形态的认识。方法对20具成人尸头40侧颈静脉球高度、顶部骨质气化、毗邻结构进行了解剖观测。结果发现颈静脉球与面神经垂直段最短距离为2.67±1.23mm(0～4.62mm),左右侧无显著差异;与鼓室最小间隔为右侧3.12±1.46mm(0～6.16mm),左侧4.86±2.04mm(1.02～9.26mm),P<0.05;高位颈静脉球出现率为17.5%(7/40),右侧为30%(6/20),左侧为5%(1/20),P<0.05;右侧颈静脉球高度为9.29±1.78mm(6.58～14.26mm),左侧为6.04±2.98mm(0～11.84mm),P<0.001;颈静脉球顶部骨质硬化时其高度为5.33±3.21mm(0～9.54mm),非硬化时高度为8.90±2.08mm(2.68～14.26),P<0.001;颈静脉球到内耳道最短距离为右侧5.82±1.64mm(3.16～8.62mm),左侧8.02±2.38mm(4.26～17.12mm)P<0.05。结论正常人左、右侧颈静脉球区解剖有一定规律及差异,熟悉颈静脉球解剖,有助于侧颅底手术顺利进行。