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Malignant tumors of the middle ear
Last reviewed: 05.07.2025
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Malignant tumors of the middle ear are a rare disease in otolaryngology. Interesting statistics are provided by the Romanian author Cornelia Paunescu. According to her data, in the Bucharest (Romania) Colcius Hospital, the ratio of malignant tumors to other diseases of the middle ear by 1960 was 1:499. According to the summary statistics of Adams and Morrisson (1955), collected in Birmingham hospitals over the entire period of their existence, out of 29,727 different ear diseases, there were only 18 patients with malignant tumors of the ear, which was 0.06%. Sarcomas of the middle ear are even more rare.
Malignant tumors of the middle ear occur in equal proportions in both sexes, epitheliomas occur between 40 and 50 years of age, and sarcomas - before the age of 10.
Malignant tumors of the middle ear are divided into primary and secondary.
Primary tumor is the most common and typical disease of the middle ear of all malignant tumors that arise here. Primary cancer can develop from the epithelium of the mucous membrane of the tympanic cavity, and sarcoma - from the fibrous tissue of the periosteum, and most often it is preceded by either benign connective tissue tumors or long-term purulent processes in the middle ear.
A secondary tumor of the middle ear occurs as a result of the penetration of tumors from neighboring anatomical structures (base of the skull, nasopharynx, parotid region) or metastasis from distant tumors.
[ What causes malignant tumors of the middle ear?
The most common cause of middle ear cancer is long-term chronic epitympanitis complicated by bone caries and cholesteatoma. The development of primary malignant tumors of the middle ear is facilitated by repeated use of silver nitrate solution to extinguish granulations in the tympanic cavity, and secondary ones - eczema, exostoses, benign tumors of the external auditory canal, leading to the formation of malignant tumors of the external auditory canal and their spread to the tympanic cavity.
Among the causes of sarcomas, a number of authors cite trauma and the presence of remnants of gelatinous mesenchymal tissue in the epitympanic space of newborns, the resorption of which is delayed, which leads to its malignant degeneration. The mechanism of development of malignant tumors of the middle ear is explained by metaplasia of the mucous membrane of the epitympanic space during a long-term inflammatory process in this area or as a result of migration of the cutaneous epithelium of the external auditory canal in the middle ear through the marginal perforation of the tympanic membrane.
Pathological anatomy of malignant tumors of the middle ear
The most common form of middle ear cancer is spinocellular epithelioma. It is followed by basal cell, cylindrical cell and glandular epitheliomas. Middle ear sarcomas are undifferentiated, fibrosarcomas, rhabdomyosarcomas, angiosarcomas, osteosarcomas, lymphoreticulosarcoma.
Symptoms of Malignant Tumors of the Middle Ear
Usually the first symptoms of malignant tumors of the middle ear are drowned out by signs of chronic purulent inflammation, and suspicion of the presence of a tumor arises only when an X-ray examination reveals an atypically extensive destruction of bone tissue that extends beyond the tympanic cavity, epitympanic space and cave.
The main symptom is hearing loss, which, however, has no specific significance for diagnosis. The only sign that can alert the doctor is an unusually rapidly progressing hearing loss, up to complete deafness in one ear. Constant symptoms of malignant tumors of the middle ear are non-tonal tinnitus, and when the tumor spreads towards the medial wall of the tympanic cavity and windows, signs of vestibular disorders appear (mechanical pressure on the base of the stapes, penetration of cancer toxins through the windows) and the addition of perceptual hearing loss.
Many authors consider facial nerve paralysis to be a pathognomonic symptom for malignant tumors of the middle ear. Indeed, in advanced cases, when there is destruction of the facial canal in the area of the medial wall of the tympanic cavity, passing between the vestibular window below and the arch of the lateral semicircular canal above, paresis or paralysis of the nerve occurs, but this complication often accompanies a chronic purulent process in the middle ear with a carious process and cholesteatoma, so this sign should not be considered "too" pathognomonic. Suppuration, even of a serous nature, can also be attributed to banal chronic purulent inflammation. Pain syndrome, perhaps, can play a decisive role in the diagnosis of a malignant tumor of the middle ear: it is distinguished by a special persistence, not characteristic of banal chronic otitis; the pain is of a constant deep nature, intensifies at night, sometimes reaching excruciating paroxysms. Even with abundant discharge from the ear, indicating that there is no delay, this pain does not subside, but on the contrary, steadily intensifies.
During otoscopy, a fleshy red formation protrudes through the completely destroyed eardrum in the external auditory canal, usually "bathing" in purulent-bloody masses, often mistaken for granulation. Palpation of this formation with a button probe of Voyachek reveals its friability, bleeding, and the probe penetrates almost unhindered into the deep sections of the tympanic cavity, the contents of which look like a homogeneous bleeding mass. It is better to avoid palpation of the medial wall with a probe, since it is easy to penetrate through the destructively changed bone into the vestibule or the main curl of the cochlea and thereby cause severe labyrinthitis with unpredictable consequences.
When the process spreads into the retroauricular space, a dense tumor-like formation can be visually and palpated, smoothing the postauricular fold and protruding the auricle. Secondary infection of the exteriorized tumor leads to a periauricular inflammatory infiltrate, increased pain, and endaural infection with malacia of the medial wall leads to rapidly developing labyrinthitis and multiple intracranial complications.
In other cases, malignant tumors of the middle ear proceed without particularly bright manifestations under the guise of chronic banal otitis media, and only during surgical intervention can an experienced otosurgeon suspect the presence of a tumor in the reddish and dense homogeneous tissue, therefore, during surgical interventions on the middle ear for chronic purulent inflammation, all removed pathologically altered tissues should be sent for histological examination.
In advanced cases, malignant tumors of the middle ear can be complicated by damage to the parotid salivary gland, ethmoid bone, nasopharyngeal formations, ear labyrinth, pyramid of the temporal bone, nerve trunks of the middle ear (lacerated foramen, Gradenigo, Colle-Sicard syndromes, etc.).
The general condition of the patient progressively worsens (anemia, cachexia). In untreated or incurable cases, patients die in a state of cachexia or as a result of complications such as bronchopneumonia, meningitis, profuse erosive bleeding from the internal carotid artery, sigmoid sinus or internal jugular vein.
Diagnosis of malignant tumors of the middle ear
Diagnosis of malignant tumors of the middle ear includes collecting anamnesis, assessing subjective and objective clinical data, conducting histological and radiological (CT and MRI) studies. Radiological examination is carried out in projections according to Schuller, Stenvers, Shosse II and Shosse III. At the initial stages, more or less intense shading of the middle ear cavities appears without signs of bone tissue destruction (if this destruction has not occurred earlier, as a result of previous chronic purulent epitympanitis). Further development of the process leads to bone destruction, manifested in bone tissue resorption, uneven osteolysis with the formation of defects with uneven and unclear edges.
In some cases, the destructive process can spread to the entire petromastoid massif, and the boundaries limiting the lost bone tissue are characterized by indistinctness, blurring, which distinguishes the radiographic picture of malignant tumors of the middle ear from that of cholesteatoma or glomus tumor, in which the resulting cavities have a smooth surface and clear boundaries. In epitheliomas, bone destruction occurs much earlier and progresses much faster than in benign tumors of the middle ear. Sarcomas of the middle ear do not have significant differences either in the clinical course or in the results of radiographic examination. They are identified based on histological examination data.
Differential diagnostics of malignant tumors of the middle ear is carried out with chronic purulent otitis media, glomus tumor and other benign tumors of the outer and middle ear, which were mentioned above. In many cases, the final diagnosis can only be made on the operating table when taking a biopsy for histological examination, which is of fundamental importance both for the prognosis and for developing subsequent treatment tactics. It should be noted that the intracranial spread of a glomus tumor with damage to the VII, IX and XII cranial nerves significantly complicates differential diagnostics.
As Cornelia Paunescu (1964) notes, the aural manifestation of some systemic diseases can simulate a malignant tumor of the middle ear, especially sarcomas, such as leukemia, leukosarcomatosis, myeloma, eosinophilic granulomas, etc. Among these diseases, we especially note the Hand-Schüller-Christian syndrome described above (and Letterer-Siwe disease).
At first, the ear manifestation is often observed in children between 2 and 5 years of age and is characterized by primary localization in the mastoid process and can be mistaken for sarcoma. However, multiple enlightenments in the bones of the skull and other bones, along with other symptoms (exophthalmos, pituitary symptoms, liver enlargement, etc.), serve as differential diagnostic signs that exclude a malignant neoplasm.
In Letterer-Siwe disease, an exceptionally severe reticuloendotheliosis in children, the primary manifestation in the region of the sacrum may simulate sarcoma, but the characteristic changes in the cytological picture of the blood, increased body temperature, and histological picture of the infiltrate (monocytosis, giant malformed cells) typical of this disease - all this determines the final diagnosis.
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Treatment of malignant tumors of the middle ear
Treatment of malignant tumors of the middle ear includes surgical, chemotherapeutic and radiation methods, the sequence of application of which is determined mainly by the prevalence of the tumor process. Symptomatic treatment of malignant tumors of the middle ear includes the use of analgesics, normalization of acid-base balance and red blood composition, and prevention of superinfection.
Today, the main method of treatment is surgical, and in those cases where the surgical method can not only eliminate the disease, but also prolong the life of the patient, preventing at least for a while the spread of the tumor process and the occurrence of a relapse.
Indications for surgical intervention and its nature, according to Cornelia Paunescu (1964), are determined as follows.
In case of limited tumors manifested by conductive hearing loss, extended petromastomastoideal extirpation is performed with exposure of the dura mater and sigmoid, and in some cases transverse sinus. At the same time, the cervical and submandibular lymph nodes on the affected side are removed.
If a malignant tumor of the middle ear is affected by a secondary infection and infiltrates the tragus area, then the operation is supplemented by removal of the parotid salivary gland.
If the tumor affects the epitympanic space, the entire anatomical structure called the auricular region, which includes the middle ear, mastoid region, external auditory canal, and auricle, is removed en bloc; all cervical and submandibular lymph nodes on the affected side are also removed. The patient is then subjected to radiation therapy.
If there is facial nerve paralysis at the level of the epitympanic space, signs of damage to the ear labyrinth (deafness, loss of the vestibular apparatus), then the previous surgical interventions are supplemented by the removal of the entire mass of the labyrinthine node and the pyramid of the temporal bone, using the Ramadier method.
In this operation, separation of the labyrinthine ganglion and pyramid is facilitated by total removal of the middle ear structures, during which the medial wall of the tympanic cavity is completely removed, as well as preliminary resection of the inferior and anterior bone walls of the external auditory canal. As a result, access to the bone canal of the auditory tube is opened, the wall of which is also resected. This achieves access to the canal of the internal carotid artery, which is trepanned with exposure of the latter. The carotid artery lies here between the bone wall of the auditory tube and the cochlea. The carotid artery is pulled forward, after which the mass of the pyramid of the temporal bone is exposed. The path to the apex of the pyramid is laid from the side of the exposed medial wall of the bone canal of the internal carotid artery. After careful separation of the internal carotid artery, preliminarily taken on a provisional ligature, the pyramid of the temporal bone is removed together with the remains of the labyrinthine ganglion and the vestibulocochlear nerve located in it. Then the sigmoid sinus is exposed, right up to the bulb, and the dura mater and adjacent structures are inspected. The resulting postoperative defect is closed with a pedicled skin flap taken from the surface of the head. Antibiotics are prescribed and, if indicated, appropriate symptomatic and rehabilitation treatment. Radiation therapy begins after 3 weeks.
What is the prognosis for malignant tumors of the middle ear?
Malignant tumors of the middle ear usually have a serious or pessimistic prognosis (due to the often late recognition of the disease), especially in the case of sarcomas that arise in childhood.