Glomus jugulare tumor (GJT) is uncommon and has been shown to develop primarily between the fourth and seventh decades of life [1] [2]. It has a higher prevalence in females than males and is sometimes inherited familially [1].

Slow growing and non-malignant nature of GJT results in a negative outcome, as the presentation is delayed with gradual onset of symptoms [3]. Symptoms are due to the direct encroachment of the tumor into neighboring structures, and both the size and location of GJT is important for classification purposes [4]. As these tumors arise from the temporal bone, the middle ear is impinged on, causing tinnitus (pulsatile), sensorineural and conductive hearing loss, vertigo, ear discharge, pain, and a reddish-bluish discoloration of the eardrum by an inconspicuous mass on otoscopy. Due to their extensive vasculature, a bruit may be heard upon auscultation of the temporal bone.

The jugular foramen is the aperture through which the internal jugular vein, and cranial nerves IX, X, and XI exit the skull. Compression of the cranial nerves results in dysphagia, hoarseness, change in voice, weakness in the sternocleidomastoid and trapezius muscles leading to the jugular foramen syndrome [2] [5].

The spread of a glomus jugulare tumor into the cranial vault consequently manifests as headaches, accumulation of cerebrospinal fluid and other features of raised intracranial pressure [6]. The brain stem may be affected, giving rise to signs such as loss of coordination, ataxia, loss of balance, and involvement of multiple cranial nerves [6]. Unilateral facial weakness is unusual but has been reported [5].

Most GJTs do not release any hormones. In some instances, however, they secrete adrenalin, noradrenalin, and dopamine, and have been referred to as complex GJTs [7]. Ensuing sympathetic hyperstimulation can induce tachycardia, tachypnea, diaphoresis, and elevated blood pressure; similar to the presentation of adrenal gland tumors. Malignant change is uncharacteristic for these tumors, noted in only a minority of cases [8].

• Tachypnea

  Ensuing sympathetic hyperstimulation can induce tachycardia, tachypnea, diaphoresis, and elevated blood pressure; similar to the presentation of adrenal gland tumors. [symptoma.com]

• Sore Throat

  A 33-year-old woman presented a chronic headache and sore throat on the right side of her body, continuous pulsatile tinnitus with decreasing hearing in the right ear, and recurrent bleeding from the right ear. [ncbi.nlm.nih.gov]

• Respiratory Distress

  Postoperatively, 2 patients with acute respiratory distress syndrome required a temporary tracheotomy. Three patients developed partial ischemia of the wound, pinna, or both. [ncbi.nlm.nih.gov]

• Progressive Dysphagia

  Results: A 46-year-old female presented with progressive dysphagia with headaches and unilateral cranial nerve palsies demonstrating facial droop, tongue deviation and weakness of neck and shoulder muscles. [neurology.org]

• Explosive Diarrhea

  A patient with a long history of explosive diarrhea, facial flushing, and violent headaches, the symptom of carcinoid syndrome, was cured after removal of his large glomus jugulare tumor. [ncbi.nlm.nih.gov]

• Lacrimation

  Patients with active GJ tumors have vasomotor symptoms like headaches, excess lacrimation and episodic flushing. Dysphagia is an uncommon presentation of GJ tumor (6%). [neurology.org]

• Tinnitus

  Glomus jugulare tumor should be considered in the differential diagnosis of adult chronic otitis, deafness, tinnitus, and dysfunction of the lower cranial nerves. [ncbi.nlm.nih.gov]

  As these tumors arise from the temporal bone, the middle ear is impinged on, causing tinnitus (pulsatile), sensorineural and conductive hearing loss, vertigo, ear discharge, pain, and a reddish-bluish discoloration of the eardrum by an inconspicuous mass [symptoma.com]

• Pulsatile Tinnitus

  A 33-year-old woman presented a chronic headache and sore throat on the right side of her body, continuous pulsatile tinnitus with decreasing hearing in the right ear, and recurrent bleeding from the right ear. [ncbi.nlm.nih.gov]

  Forty had pulsatile tinnitus at the time of GKS. The median dose to the tumor margin was 15 Gy. The median of follow-up period was 50.5 months. [wjso.biomedcentral.com]

  Pulsatile tinnitus, conductive hearing loss and aural fullness are the most common presenting symptoms. Moreover, it may cause significant lower cranial nerve deficits due to mass effect. [tjn.org.tr]

  Patients with GJ tumor usually present with conductive hearing loss and pulsatile tinnitus. Growth of the tumor can cause facial nerve paralysis, vertigo, hoarseness, and paralysis of lower cranial nerves. [neurology.org]

• Hearing Problem

  Symptoms Symptoms may include: Difficulty swallowing (dysphagia) Dizziness Hearing problems or loss Hearing pulsations in the ear Hoarseness Pain Weakness or loss of movement in the face (facial nerve palsy) Signs and tests Glomus jugulare tumors are [coordinatedhealth.com]

  Symptoms may include: Difficulty swallowing (dysphagia) Dizziness Hearing problems or loss Hearing pulsations in the ear Hoarseness Pain Weakness or loss of movement in the face (facial nerve palsy) Glomus jugulare tumors are rarely cancerous and do not [medlineplus.gov]

  Symptoms Symptoms may include: Difficulty swallowing (dysphagia) Dizziness Hearing problems or loss Hearing pulsations in the ear Hoarseness Pain Weakness or loss of movement in the face (facial nerve palsy) Exams and Tests Glomus jugulare tumors are [mountsinai.org]

• Otalgia

  Symptoms of these tumors usually include pulsatile tinnitus, hearing loss, facial nerve paralysis, vertigo, otalgia, dysphagia, hoarseness, throat sore, episodic hypertension with headaches, and tachycardia ( 3 ). [journals.lww.com]

  Patients (%) Pulsatile tinnitus 36 (86) Hearing loss 29 (69) Otalgia 8 (19) Otorrhea 3 (7) Vertigo 9 (21) Twenty–nine patients (70 %) had no preoperative cranial nerve deficit. [ncbi.nlm.nih.gov]

  Other complications such as otalgia, vertigo, cofosis and facial palsy occurred only once. One patient eventually presented a basal cell carcinoma in the face and neck approximately 30 years after radiotherapy on the same irradiated side. [scielo.br]

  […] median follow-up time of 11 years (range 5.3–22.1 years), two patients were free of symptoms and eight patients showed an improvement of one or more of their symptoms (hyp-/anacusis, pulsatile tinnitus, vertigo/dizziness, cephalgia, cranial nerve palsy, otalgia [journals.plos.org]

• Unilateral Facial Weakness

  Unilateral facial weakness is unusual but has been reported. Most GJTs do not release any hormones. In some instances, however, they secrete adrenalin, noradrenalin, and dopamine, and have been referred to as complex GJTs. [symptoma.com]

Imaging is the mainstay of diagnosis and monitoring in this type of tumor. Computerized tomography (CT) and magnetic resonance imaging (MRI) scans are most frequently used. CT scans and MRI scans help to visualize tumor extension into the bone, as well as spread to adjacent intracranial structures respectively [6]. X-rays of the skull can also be done, and will show signs of abnormal growth around the jugular fossa, but are not as helpful as CT and MRI scans. The aforementioned rich blood supply of glomus jugulare tumors necessitates arteriography and is especially important if tumor resection is planned. Radionuclide scan using meta-iodobenzyl-guanidine (MIBG) has also been used with success, in assessing these masses [9]. If there are indications of sympathetic predominance, urine catecholamine levels can be measured to determine whether tumors are secretory or not [10].

1. Young WF Jr. Paragangliomas: clinical overview. Ann N Y Acad Sci. 2006;1073:21-29.
2. Woods CI, Strasnick B, Jackson CG. Surgery for glomus tumors: the Otology Group experience. Laryngoscope. 1993;103(11 Pt 2 Suppl 60):65–70.
3. Kuhweide R, Lanser MJ, Fisch U. Catecholamine secreting paragangliomas at the skull base. Skull Base Surg. 1996;6(1):35–45.
4. Offergeld C, Brase C, Yaremchuk S, et al. Head and neck paragangliomas: clinical and molecular genetic classification. Clinics (Sao Paulo). 2012;67(Suppl 1):19-28.
5. Leonetti JP, Anderson DE, Marzo SJ, et al. Facial paralysis associated with glomus jugulare tumors. Otol Neurotol. 2007;28(1):104-106.
6. Patel SJ, Sekhar LN, Cass SP, Hirsch BE. Combined approaches for resection of extensive glomus jugulare tumors. A review of 12 cases. J Neurosurg. 1994;80(6):1026–1038.
7. Al-Mefty O, Teixeira A. Complex tumors of the glomus jugulare: criteria, treatment, and outcome. J Neurosurg. 2002;97(6):1356–1366.
8. Sanna M, Jain Y, De Donato G, et al. Management of jugular paragangliomas: the Gruppo Otologico experience. Otol Neurotol. 2004;25(5):797–804
9. Hoefnagel CA, Voute PA, de Kraker J, Marcuse HR. Radionuclide diagnosis and therapy of neural crest tumours using iodine-131 metaiodobenzylguanidine. J Nucl Med. 1987;28(3):308–314.
10. Schwaber MK, Glasscock ME, Nissen AJ, et al. Diagnosis and management of catecholamine secreting glomus tumors. Laryngoscope. 1984;94(8):1008–1015