Symposium: Congenital anomalies of the middle ear. II. Vascular anomalies of the middle ear.

Of all the abnormal situations that one encounters in performing a tympanotomy, probably the last one to be thought of is vascular anomalies, and yet, potentially, this is one of the most hazardous of anomalous problems with which we have to deal. Most of the anomalies which have been reported concern the persistent stapedial artery. This artery arises from the stump of the second aortic arch (stapedio-hyoid artery), it traverses the developing stapes bone, leaving behind a monument to its existence - the obturator foramen of the stapes. The artery exits from the middle ear along the horizontal portion of the facial nerve, traversing this area for a variable distance and then usually divides into an intra-cranial branch and a sphenoidal branch. The stapedial artery in its development before its regression, gives rise to the anlage of many important cranial arteries. These vessels are usually anastomosed with and distributed by the branches of the internal maxillary artery and the ophthalmic artery. The hyoid artery is represented in the adult by the carotico tympanic artery. Anomalies involving the internal carotid artery are much more rare. The anomalies that have thus far been reported are: 1. Absence of the internal carotids. 2. Aneurysms. 3. Unusual courses. Venous anomalies are generally uncommon and are usually represented by an abnormally placed jugular bulb which can simulate a glomus jugulari tumor.     

Polytomography and congenital external and middle ear anomalies

The preciseness of the detail which can be demonstrated by multidirectional tomography makes it a useful and almost essential method of examination of congenital anomalies of the external and middle ear. Multiple examples are described which are of inestimable value to the surgeon in the diagnosis and surgical correction of anomalies of the temporal bone. Variations of the external canal, temporomandibular joint, pneumatization of the temporal bone, tegmen, tympanic cavity, ossicles, oval and round windows, jugular bulb and carotid artery and facial nerve as seen by polytomography are described.     

The facial nerve in congenital middle ear malformations

The two most common anomalies of the facial nerve encountered in patients with a congenital malformation of the middle ear are displacement of the nerve and lack of a bony cover, two conditions that place the nerve at risk of being injured by the unwary surgeon. Malformations of the stapes are often found in association with facial nerve anomalies and may range from underdevelopment to complete absence. A congenital absence of the oval window is not uncommon. The position of the facial nerve in relation to the location and maturation of the ossicles will determine the method of ossicular chain repair. Creation of a new oval window by drilling may require the surgeon to purposely displace the facial nerve to ensure a more direct alignment of the prosthesis with the vestibule. Any part of the incus or malleus may be contoured by drilling to accommodate the loop end of the wire-piston prosthesis. An aberrant course of the facial nerve was found in 13/54 (24%) ears having a congenital malformation of the middle ear. All 54 ears had a patent external ear canal and an identifiable tympanic membrane. Patients with atresia or stenosis of the external ear canal were specifically excluded from this study.     

Salivary gland choristoma of the middle ear: A case report and review of the literature

Salivary gland choristomata are heterotopic rests which have rarely been reported in the middle ear. A case report of a salivary gland choristoma of the middle ear is presented and the literature reviewed. The frequent association of ossicular chain and facial nerve anomalies is emphasized.     

Huge middle ear adenoma with delayed facial nerve paralysis

Middle ear adenoma is a rare disease that arises from the mucosa of the middle ear. Only a few cases of associated facial nerve paralysis have been reported. Facial nerve involvement is most likely related to nerve compression rather than tumor invasion of the nerve. We describe a case of a huge middle ear adenoma in a 63-year-old man. He presented with a 1-month history of right-sided otalgia, otorrhea, and facial palsy; he also had a 10-year history of right-sided hearing loss. A tympanomastoidectomy was performed. Intraoperatively, the tumor was found to fill the middle ear cavity as well as the entire diameter of the external auditory canal. The tumor had eroded the wall of the facial canal at the second genu, and it was tightly adherent to the epineurium. Focal inflammation around the tumor was observed at the exposed facial nerve. The tumor was removed and the facial nerve was decompressed. Immediately after surgery, the patient's aural symptoms resolved. The final pathology evaluation established the diagnosis of a middle ear adenoma. At the 3-year follow-up, the ear cavity was completely healed and facial nerve function was improved.     

Congenital defects of the middle ear - uncommon cause of pediatric hearing loss

IntroductionIn children, hypoacusis, or conductive hearing loss, is usually acquired; otitis media with effusion is the most common etiology. However, in some cases this condition is congenital, ranging from deformities of the external and middle ear to isolated ossicular chain malformations. The non-ossicular anomalies of the middle ear, for instance, persistent stapedial artery and anomaly of the facial nerve, are uncommon but may accompany the ossicular defects.ObjectiveThis study aimed to describe the clinical presentation, diagnostic tests, and therapeutic options of congenital malformations of the middle ear.MethodsThis was a retrospective study of cases followed in otolaryngologic consultations since 2007 with the diagnosis of congenital malformation of the middle ear according to the Teunissen and Cremers classification. A review of the literature regarding the congenital malformation of the middle ear and its treatment is presented.ConclusionMiddle ear malformations are rarely responsible for conductive hearing loss in children. As a result, there is often a late diagnosis and treatment of these anomalies, which can lead to delays in the development of language and learning.     

Vascular anomalies of the middle ear

The patient presenting with a red mass behind the eardrum and a pulsating tinnitus may well have a vascular tumor. One must be ever mindful, however, that the mass may represent a congential vascular anomaly. The most common one seen is the uncovered jugular bulb in which the vessel extends superiorly into the middle ear to or above the incudostapedial joint. More rare is the uncovered and posteriorly displaced carotid artery. The purpose of this paper is to review the literature on the subject of vascular anomalies of the middle ear and temporal bone and to discuss the diagnosis and management of these lesions. Illustrative case reports with color photographs are presented as well.     

Salivary gland choristoma of the middle ear. A review

While head and neck surgeons are accustomed to recognizing malignancies in their practices, developmental lesions are much less often encountered, and as such are not as likely to be included amongst other (more aggressive) differential diagnostic considerations. One such developmental lesion is the choristoma, defined as an architecturally normal arrangement of mature tissues found in a location not normally host to such tissues. Choristomas composed of recognizable salivary gland tissue may be found in a variety of locations including the middle ear. Middle ear salivary choristomas are distinctly unusual lesions and typically manifest as unilateral conductive hearing loss; while both adult and pediatric patients have been diagnosed with middle ear choristomas, most patients have been in the first two decades of life. As these are benign, nonprogressive lesions, treatment has been directed toward complete surgical excision whenever feasible, and biopsy for diagnosis with subsequent observation in the case of those lesions which are difficult to remove without endangering the facial nerve. There have been rare instances in which choristomas have been linked to the subsequent development of neoplasms, but in the main choristomas are regarded as self-limited developmental heterotopias.     

Salivary gland choristoma of the middle ear

Choristoma is a mass of tissue histologically normal for an organ or part of the body other than the site at which it is located. A rare case of ectopic salivary gland choristoma in the middle ear is described in a 14-year-old girl whose only symptom was a 55 dB conductive hearing loss. The left middle ear mass appeared whitish and was located behind the intact tympanic membrane. We first suspected it to be congenital middle ear cholesteatoma. Exploratory tympanotomy, however, revealed a salivary gland choristoma that adhered tightly to the facial nerve. Differential diagnosis and treatment of this rare condition are discussed.     

Primary middle ear schwannoma

Schwannomas of the middle ear may originate from the nerves of the middle ear cavity or by extensions from neighboring structures. We present a case of a 51-year-old female patient with primary middle ear schwannoma believed to arise from Jacobson's nerve. The tumor was easily divided from the facial nerve and the chorda tympani nerve. Erosion of the promontory was noted, and the jugular foramen and posterior wall of the ear canal were preserved without destruction. The mass was successfully removed by a canal down mastoidectomy procedure, while preserving the hearing and facial nerve functions of the patient. To our knowledge, this is only the second reported case of a patient with Jacobson's nerve schwannoma.     

Jacobson's nerve schwannoma: a rare middle ear mass.

The case of a 55-year-old woman with a middle ear mass is presented. The preoperative diagnostic workup, including an audiogram and imaging studies, and the histopathologic findings of the tumor are reviewed. The tumor, a schwannoma, arose from Jacobson's nerve in the middle ear. The surgical anatomy of Jacobson's nerve and the surgical approach to this tumor and to other tumors of the middle ear space are discussed. Tumors of the tympanic cavity are rare, with the exception of cholesteatoma; otherwise, the most common among them are paraganglioma and facial nerve neuroma. This report represents the first documented case of a schannoma arising from Jacobson's nerve in the tympanic cavity.     

Middle ear adenoma diagnosed by recurrent facial paralysis

Middle ear adenoma is a rare disease derived from the middle ear mucosa. It is usually lack specific findings and easily mistaken for other conditions, delaying a correct diagnosis. In particular, few cases with facial nerve paralysis have been reported. We describe a case of middle ear adenoma that caused hearing loss and recurrent facial paralysis in a 29-year-old woman. In an attempt to treat the facial nerve paralysis, we performed tympanomastoidectomy and facial nerve decompression. By pathological examination, we finally diagnosed it middle ear adenoma with neuroendocrine differentiation. Retrospectively, if we meet the antibiotic resistant mass in the middle ear, we should suspect the tumor. One year after surgery, the adenoma has not recurred but long-term observation is required.     

Tuberculosis of the middle ear in an infant

Tuberculosis of the middle ear is currently a rare disease. As most physicians are unfamiliar with the typical presenting features, the diagnosis is made too late, with resulting complications, such as irreversible hearing loss and facial nerve paralysis. A case report and review of the literature are presented, emphasizing that tuberculosis should be considered in the differential diagnosis of chronic ear infection.     

Salivary gland choristoma in the middle ear: a case report.

Salivary gland choristoma (ectopic or heterotopic salivary gland tissue) is a rare condition that occurs in various locations within the head and neck regions. We present a 13-year-old boy with a salivary gland choristoma in the middle ear associated with congenital alopecia around the auricle in addition to facial nerve and ossicular chain abnormalities. Because the lesions commonly involve the facial nerve, intraoperative facial nerve monitoring is helpful in allowing safe biopsy without facial nerve damage.     

Salivary gland choristoma of the middle ear in an infant: a case report

Salivary gland choristoma of the middle ear cavity is an extremely rare entity. Many patients with this entity usually present with conductive hearing loss. Many reports concentrate on the importance of conservative treatment of choristoma for fear of the possible injury of the facial nerve. We report another case of salivary choristoma of the middle ear in an 11-month-old girl. We performed a reconstruction surgery of the hearing mechanism, as well as tumor removal, and achieved satisfactory hearing results. If no remarkable facial nerve anomaly is detected in the preoperative evaluation, the reconstruction of the hearing mechanism with complete tumor removal might be considered.     

Salivary gland choristoma of the middle ear: case treated with KTP laser

Salivary gland choristoma of the middle ear is rare. It consists of non-malignant, non-growing, normal salivary gland tissue in the middle ear. It is a developmental abnormality that occurs around the proximal part of the second branchial arch before the fourth month of intrauterine life. The authors found the 25th recorded case in our centre and another 24 reported cases from a review of the literature between 1961 and 1999. Intratympanic salivary gland choristoma frequently occurs during the first and second decades of life and with a female preponderance (56 per cent). Nearly all the patients (96 per cent) in our review presented with a hearing loss, that had begun since birth, in infancy, or during childhood. Tinnitus (28 per cent), and serous otitis media (24 per cent) were also commonly present. One case complained of otorrhoea. Intratympanic and extratympanic anomalies were found in 96.2 per cent and 34.6 per cent of cases respectively. Of these anomalies, ossicular chain (88.5 per cent), facial nerve (65.4 per cent), middle-ear muscles (30.8 per cent) and labyrinthine windows (23 per cent) were the four most common sites. Therefore, salivary gland choristoma may represent a manifestation of a congenital ear anomaly. Diagnosis of salivary gland choristoma is generally not documented pre-operatively, but is based on surgical biopsy and histopathological investigations. Treatment of this rare lesion depends on the size, location and extent of the mass, degree of anatomical abnormality and expertise of the surgeon. In difficult cases where the mass is attached to the dehiscent or inferiorly placed facial nerve, only biopsy is recommended. However, complete surgical removal is advocated for a mass that is easy to remove. KTP laser use via a 200 micron fibre-optic light carrier can facilitate removal especially in cases with ossicular chain involvement.     

Adenomatous tumors of the middle ear and mastoid

Adenomatous tumors of the middle ear and mastoid have been called adenoma or adenocarcinoma. The clinical and pathologic distinction between the two has been difficult. The resultant pathologic ambiguity makes it difficult to decide whether conservative or radical management is appropriate. The Otologic Medical Group's (OMG) experience with glandular tumors of the middle ear and mastoid over the past 27 years was reviewed. Thirteen cases were found and analyzed with respect to signs and symptoms at presentation. Extensive histopathologic review with light and electron microscopy was performed on tumor specimens. Two distinctive histopathologic and clinical patterns were identified. The mixed type of tumor was always confined to the middle ear and mastoid, was commonly misdiagnosed as chronic otitis media, and rarely demonstrated otic capsule or facial nerve involvement. The papillary pattern always had extension to the petrous apex and frequently involved the middle and/or posterior cranial fossa. Papillary tumors were more frequent in females and usually involved the facial nerve. On the basis of the findings in this review as well as information from the literature, we have come to the following conclusions: 1. The correct general pathologic heading be Adenomatous Tumors of the Middle ear and Mastoid with each tumor then being subclassified into Mixed or Papillary tumor and adenocarcinoma when warranted by histology. 2. There is a high rate of local recurrence. 3. Long-term follow-up (at least 10 years) for all adenomatous tumors is necessary. 4. Primary surgical treatment is required.     

Burn injuries of the middle ear.

The present paper deals with rather rare burn injuries of the tympanic membrane and middle ear. Literary resources of this topic are scarce. On 36 thermal injuries of the ear, the conservative treatment results have been evaluated from the anatomical and functional points of view. The discussion is aimed at the mechanisms of injury, pathogenesis and results of treatment of middle ear burns. Importance of prevention is emphasized.     

Aggressive papillary middle ear tumour.

A rare middle ear tumour is reported. The clinical presentation was similar to that of a glomus tumour but the pathology that of an aggressive papillary middle ear tumour. This is a recently recognized subgroup of middle ear glandular tumours. Clinical findings, imaging, pathology and treatment are presented.     

Histopathology of spontaneous brain herniations into the middle ear.

Two patients with spontaneous brain herniation into the middle ear have been operated on with a combined otoneurological approach. In case No. 1, two 2 x 3 mm arachnoid tissue herniations were found in the tegmen antri of the left ear. Six years later, a 8 x 9 mm mass consisting of prolapsed brain was removed from the right ear. The histological examination showed normal but disorganized nervous tissue. The surface consisted of middle ear mucosa or modified glial cells. More deeply numerous well preserved neurons and synapses were observed. In case No. 2, a 2 x 1 cm herniation was found in contact with the ossicles and the bony walls of the middle ear. The herniation consisted of partly degenerated nervous tissue which could explain the episode of temporal lobe seizure the patient experienced 8 years before surgery. In the world literature during the last 40 years, 29 cases of spontaneous or idiopathic brain herniation into the middle ear and mastoid have been reported. In 10, the herniations were multiple, as in our case No. 1. Case No. 1 is interesting also because the spontaneous brain herniation was bilateral.