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Tympanic and Jugular Paragangliomas

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Glomus tympanicum and glomus jugularis are hypervascular tumors of neurogenic origin which rarely have hormone activity when found in the head or neck. There are usually benign tumors, but sometimes, they can also be malignant. They develop in various sites of the head and neck and the same person may present various paragangliomata, especially if there is hereditary predisposition.

Their symptoms result mainly from the pressure they exert as they get bigger. This pressure may lead to the destruction of bone structures, paralysis of cerebral nerves or obstruction of big vessels. Glomus tympanicum and glomus jugularis occupy the same anatomic space thus presenting in the beginning the same symptomatology. Their treatment however is completely different. One of the first symptoms is a unilateral pulsatile tinnitus and a feeling of fullness, or ear congestion. Progressively, hearing loss is also observed and in advanced cases of glomus jugularis there are also symptoms of cerebral nerve paralysis such as voice hoarseness, swallowing difficulties, etc.

During ear examination with a microscope, we can observe a mass occupying a significant part or even the entire tympanic cavity. Upon closer examination, we can also observe pulses in the mass surface. In the case of glomus tympanicum we see a deep red hue whereas glomus jugularis presents a deep blue hue. All the above symptoms take time to develop thus not allowing us to misinterpret them as an acute ear inflammation.

 If there is a suspicion of glomus we must perform a computed tomography using a contrast agent. The tomography will helps us confirm the diagnosis and distinguish between glomus tympanicum and glomus jugularis. The presence of free blood flow from the sigmoid sinus to the internal jugular vein and the presence of bone shell between the jugular bulb and the mass in question, make us discard glomus jugularis. Computed tomography may be the only examination required to diagnose glomus tympanicum as there is no large enough feeding artery in order for preoperative angiography and embolism to be necessary.

 

On suspicion of glomus jugularis, we must proceed with the investigation performing a magnetic tomography with administration of paramagnetic substance, which will render more precisely the tumor’s borders and will trace its possible intracranial expansion. Then, classic angiography and, if necessary, embolism of the tumor will follow, once the patient has consented to its surgical removal. The embolism should be performed only one or two days before the intervention. It will reduce tumor perfusion and therefore its dimensions and its hemorrhagic predilection during surgery.

Main treatment for both types of paraganglioma/glomus is surgical removal. Glomus tympanicum is a rather easy case. We perform the intervention within the limits of the tympanic cavity and very rarely we have to proceed to the mastoid cavity.

Surgeon’s principal care is tumor removal. If even a minuscule element of neoplastic tissue remains this may lead to a relapse. The glomus tympanicum may expand to cells of the tympanic cavity but also inside the cochlea. For this reason the patient should be informed on the possibility of hearing impairment.

Surgery of the glomus jugularis is particularly demanding. The consequences of tumor removal may be important for the patient and surgical complications sometimes may be dangerous. In every case, however, surgical removal is the only radical treatment of a tumor which may become a threat even for the very life of the patient if it keeps growing progressively.

We must inform the patient about the hearing loss, the temporary paralysis of the facial nerve and the others cerebral nerves, about the leakage of cerebro-spinal fluid which are the inevitable or possible complications of the glomus jugularis surgery. Responsibility for this kind of intervention implies sufficient experience in head and neck and cranial base surgery, use of neuromonitoring of the cerebral function and cerebral nerves, use of navigator during the intervention and help of neurosurgeon in particular cases. It is also necessary that the patient remain at least one night in the intensive care unit once the operation is completed