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Jugular diverticulum: Clinical significance
Jugular diverticulum: Clinical significance HUSSAM K. EL-KASHLAN, MD, H. ALEXANDER ARTS, MD, and STEPHEN GEBARSKI, MD, Ann Arbor, Michigan
A
high-riding jugular bulb extending into the middle ear or a large jugular bulb without significant bone remodeling is not uncommon. A jugular diverticulum (JD), considered to be a true venous anomaly, is rare. A recent report reviewed the English language literature and added 4 cases, resulting in a total of 18 reported cases of JD.1 The authors commented that none of the reported cases presented with pulsatile tinnitus. This article reports 3 additional cases of patients who had pulsatile tinnitus as their only symptom on presentation. CASE REPORTS
Case 1. A 43-year-old woman had a 6-month history of left pulsatile tinnitus that was most pronounced with the head turned to the left. Physical examination including auscultation was normal. An audiogram revealed normal hearing bilaterally. High-resolution CT (HRCT) of the temporal bone (Fig 1A) showed a JD on the left. Magnetic resonance angiography (Fig 1B) confirmed the diagnosis and excluded other sources of pulsatile tinnitus on the left. Case 2. At presentation, a 60-year-old woman with a 10year history of left pulsatile tinnitus that could be stopped by applying gentle pressure on the neck or by turning the head to the left. Physical examination including auscultation was normal, and the audiogram showed normal hearing bilaterally. An HRCT demonstrated a JD on the left. Case 3. A 68-year-old man presented with left pulsatile tinnitus of approximately 1 year’s duration. This tinnitus was not affected by changes in head position or by pressure on the neck. An audiogram showed symmetric high-frequency sensorineural hearing loss (SNHL), and findings of the physical examination, including auscultation, were normal. An HRCT demonstrated a JD on the left side.
DISCUSSION A JD is an out-pouching of the jugular bulb that extends superiorly, medially, and posteriorly in the petrous bone.
From the Departments of Otolaryngology and Radiology, University of Michigan. Presented at the Annual Meeting of the American Academy of Otolaryngology–Head and Neck Surgery, San Antonio, TX, September 13-16, 1998. Reprint requests: Hussam K. El-Kashlan, MD, Department of Otolaryngology, 1500 E Medical Center Dr, Ann Arbor, MI 48109-0312. Otolaryngol Head Neck Surg 2000;122:575-6. Copyright © 2000 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/2000/$12.00 + 0 23/4/104640 doi:10.1067/mhn.2000.104640
These are not common lesions, and only 18 cases have been reported in the English language literature.1 JD is reported to be more common in women, which is also true in the current series. The left side was involved in all 3 patients. This agrees with findings of previous reports of more common left side involvement, despite the known dominance of the jugular venous system on the right in 75% of the cases.2 None of the previously reported patients had pulsatile tinnitus, which was the only presenting symptom in the subjects of the current article. The symptoms produced by a JD are related to its size and extension. Depending on the degree of extension, the cochlear and vestibular aqueducts, the endolymphatic sac, the posterior semicircular canal, and the internal auditory canal (IAC) can be involved. Presenting symptoms can include SNHL, vertigo, and tinnitus. SNHL could result from encroachment of the JD on the IAC or the posterior semicircular canal. Involvement of the vestibular aqueduct or the endolymphatic sac can result in Meniere-like symptoms with vertigo, SNHL, tinnitus, and aural fullness.3 Tinnitus can be constant or, as in all patients in this series, pulsatile. The pulsatile tinnitus is likely due to turbulent blood flow in the diverticulum. At our institution, pulsatile tinnitus is usually evaluated with HRCT of the temporal bone with intravenous contrast. If CT is negative, angiography or MRI is used in selected cases. A high-resolution bone algorithm CT with 1.0- or 1.5-mmthick images is the method of choice for evaluating the vascular anatomy within the temporal bone. A JD is characterized by its continuity with the jugular bulb, its smooth edges, and the lack of aggressive pattern of bone destruction. The differential diagnosis includes jugular foramen lesions and petrous apex lesions. JDs were rarely reported before CT. Because a significant number of JD are likely asymptomatic, they are probably underreported. They are usually discovered at surgery or as an incidental finding on CT examination of the temporal bone. Management of JD should be individualized. Patients with Meniere-like symptoms and CT evidence of involvement of the endolymphatic system by the JD may benefit from surgical intervention, as suggested in one reported series.3 In patients with hearing loss or tinnitus and evidence of IAC or posterior semicircular canal dehiscence, a decision for surgery should be based on careful evaluation of the risks and benefits in each case. The knowledge and prompt recognition of this vascular anomaly help the otologist to avoid hemorrhage and surgical compromise. The presence of this anomaly can create marked 575
576
Otolaryngology– Head and Neck Surgery April 2000
EL-KASHLAN et al
B A
Fig 1. A, Coronal CT section shows the diverticulum remodeling of the otic capsule, which is thinned along the basal portion of the posterior semicircular canal but is not violated (arrow). B, Coronal source image from intravenous contrast-enhanced magnetic resonance venography shows a large left jugular bulb with a diverticulum (arrow).
technical difficulties in transtemporal approaches to the IAC and posterior cranial fossa. Thus careful review of temporal bone HRCT is recommended in surgical planning of transtemporal approaches. Whether the diverticulum represents a stable congenital anomaly or a pathologic progressive process that is capable of expanding is still unclear. Follow-up imaging studies are necessary to clarify this issue.
REFERENCES 1. Pappas DG Jr, Hoffman RA, Cohen NL, et al. Petrous jugular malposition (diverticulum). Otolaryngol Head Neck Surg 1993;109:847-52. 2. Gejrot T. Retrograde jugulography in the diagnosis of abnormality of the superior bulb of the internal jugular vein. Acta Otolaryngol 1964;57:177-80. 3. Grayeli AB, Bouccara D, Julien N, et al. Traitement chirurgical des vertiges induits par une ectasie du golfe de la jugulaire. Rev Laryngol Otol Rhinol 1995;116:31-7.
A
high-riding jugular bulb extending into the middle ear or a large jugular bulb without significant bone remodeling is not uncommon. A jugular diverticulum (JD), considered to be a true venous anomaly, is rare. A recent report reviewed the English language literature and added 4 cases, resulting in a total of 18 reported cases of JD.1 The authors commented that none of the reported cases presented with pulsatile tinnitus. This article reports 3 additional cases of patients who had pulsatile tinnitus as their only symptom on presentation. CASE REPORTS
Case 1. A 43-year-old woman had a 6-month history of left pulsatile tinnitus that was most pronounced with the head turned to the left. Physical examination including auscultation was normal. An audiogram revealed normal hearing bilaterally. High-resolution CT (HRCT) of the temporal bone (Fig 1A) showed a JD on the left. Magnetic resonance angiography (Fig 1B) confirmed the diagnosis and excluded other sources of pulsatile tinnitus on the left. Case 2. At presentation, a 60-year-old woman with a 10year history of left pulsatile tinnitus that could be stopped by applying gentle pressure on the neck or by turning the head to the left. Physical examination including auscultation was normal, and the audiogram showed normal hearing bilaterally. An HRCT demonstrated a JD on the left. Case 3. A 68-year-old man presented with left pulsatile tinnitus of approximately 1 year’s duration. This tinnitus was not affected by changes in head position or by pressure on the neck. An audiogram showed symmetric high-frequency sensorineural hearing loss (SNHL), and findings of the physical examination, including auscultation, were normal. An HRCT demonstrated a JD on the left side.
DISCUSSION A JD is an out-pouching of the jugular bulb that extends superiorly, medially, and posteriorly in the petrous bone.
From the Departments of Otolaryngology and Radiology, University of Michigan. Presented at the Annual Meeting of the American Academy of Otolaryngology–Head and Neck Surgery, San Antonio, TX, September 13-16, 1998. Reprint requests: Hussam K. El-Kashlan, MD, Department of Otolaryngology, 1500 E Medical Center Dr, Ann Arbor, MI 48109-0312. Otolaryngol Head Neck Surg 2000;122:575-6. Copyright © 2000 by the American Academy of Otolaryngology– Head and Neck Surgery Foundation, Inc. 0194-5998/2000/$12.00 + 0 23/4/104640 doi:10.1067/mhn.2000.104640
These are not common lesions, and only 18 cases have been reported in the English language literature.1 JD is reported to be more common in women, which is also true in the current series. The left side was involved in all 3 patients. This agrees with findings of previous reports of more common left side involvement, despite the known dominance of the jugular venous system on the right in 75% of the cases.2 None of the previously reported patients had pulsatile tinnitus, which was the only presenting symptom in the subjects of the current article. The symptoms produced by a JD are related to its size and extension. Depending on the degree of extension, the cochlear and vestibular aqueducts, the endolymphatic sac, the posterior semicircular canal, and the internal auditory canal (IAC) can be involved. Presenting symptoms can include SNHL, vertigo, and tinnitus. SNHL could result from encroachment of the JD on the IAC or the posterior semicircular canal. Involvement of the vestibular aqueduct or the endolymphatic sac can result in Meniere-like symptoms with vertigo, SNHL, tinnitus, and aural fullness.3 Tinnitus can be constant or, as in all patients in this series, pulsatile. The pulsatile tinnitus is likely due to turbulent blood flow in the diverticulum. At our institution, pulsatile tinnitus is usually evaluated with HRCT of the temporal bone with intravenous contrast. If CT is negative, angiography or MRI is used in selected cases. A high-resolution bone algorithm CT with 1.0- or 1.5-mmthick images is the method of choice for evaluating the vascular anatomy within the temporal bone. A JD is characterized by its continuity with the jugular bulb, its smooth edges, and the lack of aggressive pattern of bone destruction. The differential diagnosis includes jugular foramen lesions and petrous apex lesions. JDs were rarely reported before CT. Because a significant number of JD are likely asymptomatic, they are probably underreported. They are usually discovered at surgery or as an incidental finding on CT examination of the temporal bone. Management of JD should be individualized. Patients with Meniere-like symptoms and CT evidence of involvement of the endolymphatic system by the JD may benefit from surgical intervention, as suggested in one reported series.3 In patients with hearing loss or tinnitus and evidence of IAC or posterior semicircular canal dehiscence, a decision for surgery should be based on careful evaluation of the risks and benefits in each case. The knowledge and prompt recognition of this vascular anomaly help the otologist to avoid hemorrhage and surgical compromise. The presence of this anomaly can create marked 575
576
Otolaryngology– Head and Neck Surgery April 2000
EL-KASHLAN et al
B A
Fig 1. A, Coronal CT section shows the diverticulum remodeling of the otic capsule, which is thinned along the basal portion of the posterior semicircular canal but is not violated (arrow). B, Coronal source image from intravenous contrast-enhanced magnetic resonance venography shows a large left jugular bulb with a diverticulum (arrow).
technical difficulties in transtemporal approaches to the IAC and posterior cranial fossa. Thus careful review of temporal bone HRCT is recommended in surgical planning of transtemporal approaches. Whether the diverticulum represents a stable congenital anomaly or a pathologic progressive process that is capable of expanding is still unclear. Follow-up imaging studies are necessary to clarify this issue.
REFERENCES 1. Pappas DG Jr, Hoffman RA, Cohen NL, et al. Petrous jugular malposition (diverticulum). Otolaryngol Head Neck Surg 1993;109:847-52. 2. Gejrot T. Retrograde jugulography in the diagnosis of abnormality of the superior bulb of the internal jugular vein. Acta Otolaryngol 1964;57:177-80. 3. Grayeli AB, Bouccara D, Julien N, et al. Traitement chirurgical des vertiges induits par une ectasie du golfe de la jugulaire. Rev Laryngol Otol Rhinol 1995;116:31-7.