A
Tumor limited to middle ear space
B
Tumor limited to the middle ear or mastoid without involvement of the infralabyrinthine space of the temporal bone
C
Tumor involving infralabyrinthine and apical spaces of temporal bone, with extension into the apex
C1
Tumor with limited involvement of vertical portion of the carotid canal
C2
Tumor invading vertical portion of carotid canal
C3
Tumor invasion of horizontal portion of carotid canal
D1
Tumor with intracranial extension less than 2 cm in diameter
D2
Tumor with intracranial extension greater than 2 cm in diameter
I | Small tumor involving the jugular bulb, middle ear, and mastoid |
II | Tumor extending under internal auditory canal; may have intracranial extension |
III | Tumor extending into the petrous apex; may have intracranial extension |
IV | Tumor extending beyond petrous apex into the clivus and infratemporal fossa; may have intracranial extension |
Symptoms
Hearing Loss
Hearing loss is seen in 60–85% of patients [1–3]. Hearing loss is most commonly conductive and results from tumor extension into the middle ear with involvement of the tympanic membrane, ossicular chain or round window. Less commonly, sensorineural hearing loss may result from tumor invasion of the bony labyrinth [1, 4, 5].
Pulsatile Tinnitus
Pulsatile tinnitus is the perception of auditory stimuli that is synchronous with the heartbeat [12]. It is commonly seen in JP as the tumor invades the petrous bone and compresses local neurovascular structures. The incidence of pulsatile tinnitus in patients with JPs is approximately 75% [1, 2].
There are a few hypotheses as to why this phenomenon occurs. It may result from nonlaminar, turbulent blood flow through regional blood vessels which transmit sound to the inner ear [12–14]. Another possibility is that the sound of normal flow is more intensely perceived, whether due to increased bone conduction in the inner ear or decreased conduction of external sounds [14]. Finally, pulsatile movement of the tympanic membrane, ossicles, or round window membrane may result in this symptom.
Cranial Nerve Paralysis
A number of other symptoms are associated with compression of the lower cranial nerves (CN) in the jugular foramen and surrounding structures. Overall, cranial nerve deficits are seen in approximately 40% of patients with JP at time of presentation [15–18]. Encasement and invasion of the facial nerve, most commonly in the mastoid segment, may result in sudden or progressive facial paralysis occurring in up to 25% of cases [2, 19, 20].
Cranial nerves IX, X, XI, and XII may also be involved due to their proximity to the jugular foramen [5]. Signs and symptoms include dysphagia (CN IX), nasal regurgitation, rhinolalia, hoarseness (CN X), shoulder droop (CN XI), and tongue weakness (CN XII). Tongue weakness is most commonly caused by medial growth of tumor into the region of the hypoglossal canal and can be seen in up to 20–40% of patients [2, 21].
Otalgia and Otorrhea
Several published series have described otalgia , occurring in 5–40% of patients [1, 2]. As the tumor expands to include the middle ear space, the Eustachian tube orifice or mastoid antrum may become blocked, resulting in effusion or symptomatic mastoid or middle ear inflammation. Involvement of the tympanic plexus, pressure on the tympanic membrane, or eruption into the external auditory canal may also incite fullness, pressure, or pain. JPs may erode through the tympanic membrane in 14–24% of patients, causing bloody or purulent otorrhea [1, 2].
Papilledema and Vision Loss
In rare instances, JPs may cause hydrocephalus, resulting in papilledema and vision loss . The development of elevated intracranial pressure may occur from intracranial tumor extension with brainstem compression (non-communicating hydrocephalus), occlusion of a dominant venous outflow system, or involvement of bilateral jugular venous systems in cases of multicentric disease (communicating hydrocephalus). If left untreated, elevated intracranial pressures may lead to vision loss, coma, or death [22, 23].
Cough Syncope
Cough syncope is another unusual presentation of JP. It has been described in a patient with elevated intracranial pressure from communicating hydrocephalus. It was hypothesized that coughing episodes resulted in transient elevations in baseline high intracranial pressure, leading to intermittent tonsillar herniation and syncope [24].
Vertigo
Catecholamine-Secreting Tumors
Histochemical studies have shown that all paragangliomas secrete catecholamines at some level, but less than 4% secrete enough to cause symptoms [1, 27]. The release of excess catecholamines may cause palpitations, headaches, and uncontrolled hypertension [5, 8, 28–32]. Patients with familial head and neck paragangliomas also have an increased risk of developing abdominal and thoracic pheochromocytomas [9]. Carcinoid syndrome is a rare manifestation of JP, resulting in symptoms of diarrhea, facial flushing, and headaches due to elevated 5-hydroxyindoleacetic acid, the main metabolite of serotonin [33].
Physical Exam
A pulsatile red or purple hypotympanic or mesotympanic mass is a characteristic of JP (Figs. 3.1 and 3.2). When the entire circumference of the tumor can be seen on otoscopy, the diagnosis of a glomus tympanicum can be made. However, it is impossible to distinguish a glomus tympanicum and JP on physical examination alone when the inferior aspect of the tumor extends below the tympanic ring into the hypotympanum—in such cases, additional imaging is required to determine the extent of the tumor. The “rising sun” sign has been used to describe the red appearance of a tumor seen on otoscopy when it ascends from the floor of the middle ear. The tumor may often develop prominent canal vasculature surrounding the mass, likened to sunrays. The retrotympanic mass may initially pulsate more vigorously, and then blanch with positive pressure using pneumatic otoscopy, a finding known as Brown’s sign (pulsation sign). Some tumors can erode through the tympanic membrane, whereby an inflamed polypoid growth in the medial external auditory canal can be seen [2, 5]. Aquino’s sign refers to blanching and decreased pulsation of the mass with manual compression of the ipsilateral carotid artery. Additionally, an audible bruit may be auscultated in the mastoid or infra-auricular region.
Fig. 3.1
Jugular paraganglioma presenting as retrotympanic mass involving most of the middle ear space (Courtesy of George Wanna, MD)
Fig. 3.2
Jugular paraganglioma involving the hypotympanum (Courtesy of George Wanna, MD)
Multicentric paragangliomas develop in 10–20% of sporadic tumors and in up to 80% of familial cases [24]. Head and neck paragangliomas may arise from other locations aside from the jugulotympanic region, including paraganglia of carotid bodies (most common), or along vagal, distal laryngeal or facial nerves or from the sympathetic chain. Cervical head and neck paragangliomas commonly present with an asymptomatic neck mass, which can be pulsatile [34].
Differential Diagnosis
Jugular paragangliomas are the most frequently occurring tumors of the jugular foramen, accounting for at least half of lesions of the jugular foramen [6]. The differential diagnosis includes benign and malignant neoplasms as well as vascular lesions that are most easily differentiated by neuroimaging [6, 35]. A case series of 106 patients who underwent surgery for jugular foramen lesions are identified, in order of incidence: paragangliomas, schwannomas, meningiomas, chondrosarcoma, carcinoma, aneurysmal bone cyst, chordoma, cholesteatoma, chondroma, lymphangioma, and inflammatory granuloma [6].
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