Abstract
A 72-year-old woman with recurrent bouts of vertigo and syncope was found to have a glomus tympanicum tumor. Surgical removal of this tumor resulted in complete symptom resolution. This report summarizes the presentation, workup, treatment, and clinical significance of this case when dealing with these presenting symptoms.
Presenting Symptoms
Mrs M. is a pleasant 72-year-old woman who presented with vertigo and syncope. She had prior mild episodes of vertigo every three years, which subsided spontaneously, or with an oral antihistamine. She now presented with more severe episodes, resulting in syncope.
Chart Notes
Mrs M. is a 72-year-old woman who presents today with a bruised right eye. She suffers from vertigo. Four days ago she had a vertigo episode, fell, passed out and hit the right side of her face/eye and her left knee on a concrete floor. The fall was unwitnessed. The patient was getting orange juice from refrigerator at the time, turned, felt dizzy, and passed out. She is taking “motion sickness pills.” Vertigo attacks occur once every three years. This is the first time that she has actually passed out. There was no antecedent headache, palpitations, or chest pain. The patient has been asymptomatic since event; no headache, no visual changes, no weakness, no numbness, no paresthesias. She has been ambulating well, without dizziness since then.
Initial Workup
Physical exam - negative
24 hour Holter monitor - negative
Referral and Subsequent Evaluation
Referred to otolaryngology.
CT of posterior fossa revealed a mass in the right tympanic (middle ear) canal.
Surgical pathology revealed a glomus tumor.
Patient's symptoms completely resolved after surgical removal of the tumor.
Glomus Tumor Overview
Glomus tumors are generally benign, vascular tumors arising from glomus bodies, which arise from chemoreceptor cells in paraganglionic bodies, and assist with temperature, pressure, and chemical regulatory functions in the body.1
The most common location is in the distal extremities, most commonly subungual, where it may be seen as a bluish discoloration under the fingernail.2
Glomus tumors may also be found in the head and neck, with the most common sites being the jugular foramen, middle ear cavity, carotid body bifurcation, and vagal nerve. Glomus tumors represent 0.6% of neoplasms of the head and neck and 0.03% of all neoplasms. Glomus jugulare tumors are the most common head and neck glomus tumor; the rarest are glomus tympanicum tumors. Glomus tumors are the most common tumors of the inner ear. Tumors in the middle ear may appear as a small red spot visualized behind the tympanic membrane on otoscopy.3
Most glomus tumors are asymptomatic. However, they may cause pain (especially in the subungual location), hearing loss (ear), pulsatile tinnitus, dizziness, or cranial nerve palsies (ear or cranial nerves). If the paraganglioma cells of the glomus tumor are functional, which is rare, catecholamine secretion may cause systemic symptoms.4
The peak age incidence of glomus tumors of the head and neck occurs between 50 and 60 years, with a slight female predominance.
Imaging is usually accomplished by contrast CT or MRI; because of the high vascularity in these tumors, contrast material is helpful in their visualization and distinction from adjacent structures, particularly lymph nodes.5
Treatment of glomus tumors involves local excision, when possible. Other modalities include radiation treatment (though these tumors are generally not very radiosensitive), particularly with intracranial tumors or in elderly patients. Selective arterial embolization is another treatment modality, which is also often used prior to surgery, to reduce intraoperative bleeding in these vascular tumors.
Excision may be particularly difficult if the tumor is growing on a nerve, as excision might result in nerve damage. Removal is also difficult when the tumor is intracranial, such as in the jugular foramen, and may involve multiple subspecialist skills during surgery, i.e, neuroradiologist, otolaryngologist, neurosurgeon.
Successful tumor removal generally results in symptom resolution.6
Case Relevance
This case highlights the need to consider glomus tumor of the ear when considering the differential diagnosis of a patient with dizziness and syncope, particularly when the patient complains of hearing loss or pulsatile tinnitus. Many patients with dizziness and syncope present with vague and varied complaints, so evaluation is often difficult. In patients with dizziness, the differential diagnosis is vast, and peripheral (i.e, labrynthitis, Meniere's disease, benign paraxysmal positional vertigo) causes must be differentiated from central (i.e, brain tumor or stroke) causes. Patients with syncope, particularly the elderly, often require a cardiac workup, to rule out dysrhythmia (i.e, heart block, rapid atrial fibrillation) or valvular dysfunction (i.e, aortic stenosis) as potential causes. Those with associated focal neurological signs or symptoms may benefit from intracranial and carotid artery imaging.7 This case also revealed the lack of sensitivity of general head CT scan in making this particular diagnosis; only the CT with attention to the posterior fossa, as ordered by the otolaryngologist, was able to successfully reveal the tumor. Because the tumor, in this case, was limited to the tympanic canal without extension into the mastoid or jugular foramen, the otolaryngologist was able to resect the tumor via excision of the tympanic membrane.
The patient returned for follow up symptom-free, and was delighted with her successful outcome.
Footnotes
Disclosure: There was no grant funding for this case report.
References
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