An 87‐year‐old woman presented with one year of progressively worsening left‐sided hearing loss and left ear fullness, 6 months of hoarseness, and 1 month of dysphagia. She also reported an abnormal appearance of her tongue, persistent left‐sided tongue deviation, and inability to “lick (her) lips right.” She sought medical attention after a fit of coughing and choking which occurred while eating. The left side of her tongue was atrophied and deviated to the left with protrusion. The left soft palate did not elevate. Left‐sided hearing was diminished. Left otoscopic exam revealed an erythematous, non‐pulsatile mass behind the left tympanic membrane. Bedside flexible fiberoptic laryngoscopy revealed left vocal cord paralysis. Computed tomographic scan of the neck revealed a hypervascular left skull base mass, centered on the jugular foramen, extending medially to the hypoglossal canal and laterally to the left tympanic cavity. The clinical and radiographic findings were consistent with glomus jugulare tumor (paraganglioma), causing deficits of cranial nerves 9, 10, and 12 due to mass effect. Given the patient’s age and multiple chronic medical problems, a non‐surgical approach to management of the tumor was recommended. She was referred for consideration of radiation therapy.
Paragangliomas are rare, highly vascular neuroendocrine tumors which arise from extra‐adrenal sympathetic or parasympathetic paraganglia. Most are diagnosed in the third to fifth decades. In about one‐third to one‐half of cases, a paraganglioma is a component of an inherited syndrome; most are sporadic. Sympathetic paragangliomas usually secrete catecholamines and are located in the sympathetic paravertebral ganglia of the thorax, abdomen, and pelvis. Most parasympathetic paragangliomas are benign, nonfunctional, and located in the neck and skull base along the branches of the glossopharyngeal and vagus nerves. They arise most commonly from the carotid body, less commonly from jugulotympanic and vagal paraganglia, and rarely from laryngeal paraganglia. Jugulotympanic paragangliomas are slow‐growing lesions that usually present with pulsatile tinnitus, conductive hearing loss, a bluish pulsating mass behind the tympanic membrane, and lower cranial nerve deficits. Management options for symptomatic parasympathetic gangliomas include surgery and radiation, both of which can provide adequate local disease control.
Hospitalists frequently manage patients who present with neoplasm‐related cranial nerve or other neurologic deficits which are associated with primary tumors, metastatic lesions, or paraneoplastic syndromes. The purpose of reporting this case is to highlight an unusual neoplastic etiology of symptoms often encountered by hospitalists.