Disease sexually transmitted:

Lymphogranuloma (venereal)

Paraganglioma of the middle ear

  • What is a Paraganglioma of the middle ear
  • What triggers / Causes Paraganglioma of the middle ear
  • Pathogenesis (what is happening) during Paraganglioma of the middle ear
  • Symptoms of Paraganglioma of the middle ear
  • Diagnosis of Paraganglioma of the middle ear
  • Treatment of Paraganglioma of the middle ear
  • Which doctors should be consulted if You Paraganglioma of the middle ear

What is a Paraganglioma of the middle ear -

Paraganglioma of the middle ear (synonyms: non-chromaffin paraganglioma, chemodectoma) is paraganglioma., developing from adrenalynne cells adventitial sheath upper bulb of the internal jugular vein, located near the middle ear cavity.
Paragangliomas of the ear and skull base are considered relatively rare disease, however, to exclude this pathology and differential diagnosis have a significant number of patients suffering from hearing loss, noise in the ear, hoarseness, etc.

What triggers / Causes Paraganglioma of the middle ear:

It is assumed autosomal dominant inheritance, mainly affecting females (ratio female/male to 3.5:1 among adults and 1.4:1 in children.

Pathogenesis (what is happening) during Paraganglioma of the middle ear:

Glomal tumor of the middle ear, as established Rosenwasser, develops from detailed Guild glomal Taurus (non-chromaffin chromaffin body), the adventitia of the jugular vein (directly under the bottom of the tympanic cavity, these cells in the course of the tympanic nerve in the plexus on the medial wall of the tympanic cavity) and from glomal Taurus along the auricular branch of the vagus nerve (in the described case Bradley and Maxwell glomal tumor or paraganglioma located under the tip of the mastoid process, destroyed here the facial nerve). This tumor representing a tangle anastomosing arterial-venous capillaries and precapillaries enabled glomal cells, closely connected with the system of the vagus nerve. So far in the world literature is given more than 200 cases of tumors of the glomus jugular.
Glomal tumors occur 5 times more often in women than in men and mostly in middle age. The tumor penetrate into the tympanic cavity from the dome of the jugular vein or develop in the drum, the cavity then fill it, destroy the eardrum and act in the external auditory canal in the form of a heavily bleeding when touched polyp. The tumor may spread into the pyramid of the temporal bone, the inner ear and the cranial cavity. In observation Weile Lane and the tumor, destroying the whole pyramid of the temporal bone, sprouted V. the nasopharynx.
Most common glomal tumor of the middle ear and the jugular fossa, and in half of the cases the tumor is detected in the external ear canal (P. G. Weisinger). Glomal a tumor is causing the hearing loss of a conductive type and degree of violation. labyrinthine functions, until its loss. Ear symptoms usually precede a fairly frequent lesion of the facial nerve (according to Fuller, Brown, Harrison Siekert, paralysis of the facial nerve was observed in 19 of the 72 patients with glomal tumor)- less common, the opposite phenomenon. The defeat of IX-XII cranial nerves indicates the distribution process for the jugular fossa and is observed in a later stage of the tumor (E. P. FLAC).
Penetration of the tumor in the cranial cavity often marked in the posterior fossa through the mastoid or the jugular fossa.
Metastasis of the tumor is less than 4 occasions and the main localization of metastasis are regional lymph nodes, lungs and liver.Параганглиома среднего уха

Symptoms of Paraganglioma of the middle ear:

The clinical picture with the defeat of the tumor in the initial stage is very monotonous. The disease progresses slowly, almost imperceptibly. Patients usually complain of decreased hearing, ringing in the ear, mostly pulsatile nature, and in some cases appears, the symptoms of acute otitis media. If otomicroscopy eardrum during this period did not change and only after some time it turns pink, then red, for it reveals crimson education rounded, sometimes visible ripple. Large tumor impaneling glomus destroys the eardrum with the formation of persistent perforation, through which the tumor can spread into the ear canal and often gives the impression of an ordinary polyp, which often entails diagnostic errors. Patients often get used to the noise in the ear and don't go to the doctor until you develop such symptoms, like discharge from the ear, rarely pain in the late stages of the disease - neurological symptoms.

Diagnosis of Paraganglioma of the middle ear:

For the diagnosis of the disease L. Brown in 1953, proposed a method, known as "the symptom of a ripple." When introduced into the external auditory canal pneumatic funnel and a small pressure increase in the tumor tissue pale, and with further increase in pressure can be determined by its pulsation. This symptom is typical for tumors of the middle ear glomal nature.
Neurologistquarter symptoms usually appear after a long time (sometimes several years) after the defeat of the ear and is manifested by disorders of the facial, vestibulocochlear, and IX, X, XI cranial nerves. Hormoneproducing glomal tumors secreting catecholamines, are rare (1-3). The clinical picture in this case is accompanied by development of arterial hypertension, tachycardia.
In the diagnosis glomal tumors of the middle ear has high information content computed tomography (CT) of the temporal bone, allowing to assess the localization and prevalence of the pathological process. KT-glomal tumors symptoms include changes in soft tissue and bone-destructive nature that reflect pathological changes in the temporal bone at different tumor types.
So, when tumors impaneling glomus tympanic cavity, directly on promontorium, you can see soft-tissue tumor with rounded or polycyclic circuit. In some cases, the formation extends into the external auditory canal in the form of polypoid growths. With increasing tumor size and spread to the mastoid process on the CT there is a violation of his pneumatization due to the pathological substrate.
Jugulare tumor on CT are characterized by the presence of bone-destructive changes fossa of the jugular vein, internal carotid artery, the contours of which are purchased by the irregularity, and blurred.
To determine the boundaries of the tumor and identify its spread intracranially in the posterior or middle cranial fossa (SCA) and watched in the soft tissue of the neck allows magnetic resonance imaging (MRI). Less diagnostic capabilities in this respect has a CT scan with intravenous contrast. Carotid arteriography remains the most widely used method for preoperative diagnosis and embolization of the vessels feeding the tumor.
During the inspection of the eardrum is intact, and behind her is visualized region of pulsation. Larger paraganglioma bulges into the ear canal and resemble polyps. Although histologically they may be benign, to treat these tumors, especially when a significant size, it is difficult due to their vascularization and anatomical relationships.
When x-ray examination can be observed depending on the stage and direction of tumor growth, the expansion of the jugular fossa, its fuzzy edges, darkening of the lower and middle divisions of the tympanic cavity, the destruction of the bottom of the tympanic cavity, partial destruction of the lower and rear surfaces of the pyramid, thinning of the lateral masses of the occipital bone.
In the later stages of differential diagnosis more commonly done with other tumors of the temporal bone and skull base.
Differential diagnosis with diseases accompanied by pulsating noise. Pulsating tinnitus is one of the early major symptom of some diseases of the ear and vascular pathology. Among the diseases of the ear, in which there is a pulsating noise in the first place are paragangliomas - it occurs less frequently in otosclerosis, secretory otitis media, acoustic neuroma, inflammatory processes. From vascular disease, manifested pulsating noise, the most common aneurysm of the carotid arteries, stenosis of the main vessels, arterio-venous shunts. The noise may be due to the atypical location of vessels (high standing of the jugular vein, the passage of the internal carotid artery through the tympanic cavity, etc.). When paragangliomas and vascular pathology noise can be worn not only subjective, but also objective in nature, which allows you to register it using various instruments. A characteristic symptom in paragangliomas and vascular pathology is the disappearance or reduction of noise during compression of the carotid artery. Other diseases this symptom is not detected. For paragangliomas and vascular pathology characterized by the presence of noise until hearing loss, other diseases of the ear noise occurs after or simultaneously with hearing loss.
When the pulsating noise differential diagnosis is necessary to carry out mainly between paragangliomas and vascular pathology. Important importance of audiological research, in particular chemometry.
We examined 35 patients with paragangliomas and 19 with vascular pathology (9 - high standing of the jugular vein, 7 - arterio-venous shunts, 3 - aneurysm of the carotid arteries). When paragangliomas noise has been complicated and often superimposed on the monotonous sound of a frequency of 250-500 Hz. Himself humming noise had a frequency of 800-3000 Hz and a force of 10-20 dB. When vascular pathology noise wore a tonal nature, frequency - 80-500 Hz power usually exceeded 20 dB. Necessarily tonal audiometry. For paragangliomas are characterized by the progression of hearing loss, which is initially conductive, and then mixed. When vascular pathology auditory function remains normal or develops hearing loss of the sensorineural type. Great importance for the differential diagnosis has impedansometry. When paragangliomas disorders of the auditory tube (**tympanogram type C) or an increase of impedance due to the presence of fluid or tumors (**tympanogram type b). For vascular disease, these changes are not typical. Study of the vestibular function when paragangliomas reveals giovanbattista of the maze - when considering vascular pathology function of the labyrinth, as a rule, is not violated. The primary diagnosis is an x-ray study and especially angiography, which allows to detect well-perfused tumor or vascular pathology.
Differential diagnosis with secretory otitis media. As indicated above, one of the early signs of paragangliomas is secretory otitis media, clinic which differs little from the clinic banal secretory otitis media. Banal secretory otitis media is often bilateral in nature, when paraganglioma – sided. The main cause unilateral chronic secretory otitis media is dysfunction of the Eustachian tube associated with the pathology of the nose, nasopharynx (deviated septum, turbinate hypertrophy, polyps, adenoids, etc.). When paraganglioma secretory otitis media may develop without dysfunction of the auditory tube. For paraganglioma characterized by pulsating noise that appears to the hearing loss and secretory otitis media. Most often paraganglioma have to differentiate this kind of secretory otitis media, idiopathic hematodinium.
Important differential diagnostic method is to study the concentration of serotonin in the secret of the tympanic cavity. For paragangliomas are characterized by a high level of serotonin is more than 1 μmol/l In secretory otitis serotonin levels of less than 1 μmol/L.
Informative x-rays in these cases, MRI is that allows to detect even small tumors in the tympanic cavity and the base of the skull. Great importance is diagnostic tympanotomy in which it is possible to identify the tumor and to conduct a histological study.
Differential diagnosis with idiopathic unilateral paralysis of the larynx. Unilateral laryngeal paralysis is most often caused by dysfunction of the recurrent nerve in the pathology of the thyroid gland, mediastinum, aortic arch, etc. impaired function of the X nerve (vagus) in tumors of the jugular and vagal of glomus also leads to a unilateral restriction of mobility of the vocal folds. It should be noted that for tumors glomus jugular is characterized by multiple lesions of the nerves running in the region of the jugular foramen (IX, X, XI, XII), with the appropriate clinical picture. When tumors vagal glomus dysfunction of the vagus nerve for a long time may be the only manifestation of the disease, however, with the growth of tumors at the skull base and jugular foramen was also noted multiple nerve damage. The main diagnosis are x-ray methods: thyroid ultrasound, duplex scanning of neck vessels, CT, MRI and especially angiography.
Differential diagnosis with other tumors of the ear and skull base. Among tumors of the ear should be carried out differential diagnosis of hemangioma, a neuroma of the eighth nerve, rarely with malignant tumors. Hemangiomas grow more quickly in comparison with paragangliomas and is characterized by spontaneous bleeding from the ear that when paragangliomas are rare. Hemangiomas are not peculiar to the destruction of bone structures of the middle ear, detectable x-ray examination in cases of paragangliomas. The main method of diagnosis is histological examination.
Neuromas of the eighth nerve in some cases, especially with the growth in the caudal direction with lesions of IX, X, XI, XII cranial nerves, have a clinical picture similar to paragangliomas. The difference in the clinical course of the disease is that neuromas grow very slowly, you can stop growing, rarely germinate in the middle ear. For paragangliomas characterized by continued growth, if any source localization they can grow into the tympanic cavity. Important differential diagnostic importance of these audiological research. For neuromas is characterized by sensorineural impairment of the hearing function to retrocochlear level which can be detected by the evoked otoacoustic emissions (VAAA) and brainstem auditory evoked potentials (ABR). When paragangliomas hearing loss is conductive or mixed character and defeat neurosensory structures at the level of the cochlea. Neuromas ear noise tends to be high frequency tonal character, for paragangliomas characteristic pulsating noise. X-ray examination neuromas are manifested by the expansion of the internal auditory canal and the presence of a tumor in it, while paragangliomas rarely cause degradation of the internal auditory canal.
Malignant tumors of the ear, unlike paragangliomas, grow very quickly. However, they cause early destruction of the tympanic membrane and grow into the external auditory canal, which is accompanied by the accession of suppurative otitis media. Pain is an almost constant symptom in malignant tumors of the ear and not characteristic of paragangliomas. The basis for diagnosis is histological examination.
Among tumors of the skull base, growing in the area of the jugular foramen, meet neuroma, fibroma, meningioma, osteoma. Rare malignant neoplasm: cancer, sarcoma, rhabdomyosarcoma, etc. Possible metastatic lesion in tumors of breast, lung, prostate, kidney and other organs. For all tumors located in the region of the jugular foramen, is characterized by dysfunction of the cranial nerves passing through it. However, all other tumors, unlike paragangliomas rarely germinate in the middle ear and lead to hearing loss. The main method of differential diagnosis is an x-ray examination (CT scan, MRI, angiography. Paragangliomas, usually located in the lateral part of the jugular foramen and grow laterally, causing destruction of the posterior and inferior surfaces of the temporal bone, manifesting on CT uneven edges and extension holes. Neuroma, fibroma and other benign tumors lead to uniform expansion of the jugular foramen, which is characterized by smooth, well-rounded edges. Malignant neoplasms grow medially, destroying the medial side of the jugular foramen, clivus.
On MRI paragangliomas are characterized by a heterogeneous signal and have a "speckled" look that reflects the abundance of newly formed vessels, their raznokalibernoy, multi-directionality of the blood flow and partial thrombosis. Most reliable radiographic sign of paragangliomas is their visualization during angiography. Other tumors, visualized as paragangliomas, at MRI angiograms are not defined.
Thus, among the differential diagnostic methods are the most informative audiological study, determining the concentration of serotonin in the secret of the tympanic cavity and x-ray examination.
Параганглиома среднего уха
Paraganglioma of the middle ear

Treatment of Paraganglioma of the middle ear:

Treatment glomal of the tumor is reduced to surgery, radiation therapy or a combination of them. Surgeonalgebraic intervention is indicated for tumors not extending beyond the middle ear. The incomplete removal of the tumor is used additional irradiation.
B due to heavy bleeding (complicating the removal of the tumor) in recent times the operation is performed with controlled deep drop in blood pressure. After removal of the tumor together with the damaged it by the facial nerve, the nerve defect is replaced with an autograft.
Combined treatment is performed at glomal tumors of the middle ear and the jugular fossa. When the spread of the tumor in the cranial cavity and significant degradation of the temporal and occipital bones applies only radiotherapy (remote static humatrope).

Which doctors should be consulted if You Paraganglioma of the middle ear:


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