Aggressive papillary tumor of the middle ear: a true entity or an endolymphatic sac neoplasm?

Aggressive papillary tumors of the middle ear are rare, and their true origin is not clear. We describe the clinical, radiologic, genetic, and histopathologic features of a papillary epithelial tumor filling the middle ear of a 68-year-old woman. Imaging revealed no evidence of petrous temporal bone apex involvement, nor did genetic studies demonstrate the von Hippel-Lindau mutation. A literature search revealed 24 previously reported cases of such a papillary epithelial tumor of the middle ear. All except 2 cases demonstrated apical petrous temporal bone invasion on imaging, and it has been suggested that they arose from a primary endolymphatic sac tumor, which has a similar papillary epithelial histology. Substantial numbers of cases of papillary epithelial tumors involving the middle ear are reported to be associated with von Hippel-Lindau disease, as are known cases of endolymphatic sac tumor. This is the third reported case of papillary epithelial tumor of the middle ear that does not show apical petrous temporal bone invasion on imaging, suggesting that such neoplasms do not always arise from a primary in the endolymphatic sac.     

Epithelial tumors of the middle ear--are middle ear carcinoids really distinct from middle ear adenomas?

Primary tumors of the middle ear are much less commonly encountered in clinical practice than non-neoplastic lesions such as inflammatory polyps (aural polyps) or cholesteatomas. The rarity of such tumors can complicate attempts, by both clinicians and pathologists, to correctly classify them. It has been customary for many authors to segregate middle ear adenomas (MEAs) from middle ear carcinoids as two discrete benign neoplastic entities. It has become apparent, however, that MEAs and carcinoids of the middle ear share a sufficient number of overlapping pathologic features and similarities of clinical behavior to warrant their collapse into a single diagnostic category. It is proposed that these tumors should be designated as MEAs, which are defined as benign, indolent epithelial tumors of the middle ear that do not invade or erode bone and do not metastasize. The individual tumor cells are cytologically bland and polygonal, columnar or plasmacytoid-shaped; they may be arranged in islands, glandular formations or trabeculae, but not in papillary structures. They are typically keratin- and vimentin-positive immunohistochemically, and are often positive as well with antibodies for chromogranin A, synaptophysin, neuron-specific enolase, Leu-7, serotonin, pancreatic polypeptide and S-100 protein. Dense core neurosecretory granules may be identifiable by electron microscopy. Conservative surgical excision is the treatment of choice, and local recurrence following complete excision is quite uncommon.     

Gain and maximum output of two electromagnetic middle ear implants: are real ear measurements helpful?

We compared the output of two electronic middle ear implants: the Otologics MET device and the Vibrant Soundbridge device. Both devices were programmed in the linear amplification mode. Aided minus unaided sound pressure levels recorded in the ear canal (objective gain) were compared to unaided minus aided soundfield thresholds (functional gain) in 13 patients with severe sensorineural hearing loss. In addition, input/output characteristics were studied with the help of ear canal measurements. Objective gain was consistently lower than functional gain, with wide variation between patients and frequencies. Using input/output data measured in the ear canal in combination with functional gain data, the mean maximum output of the two devices was estimated, expressed in dB SPL. In comparison to NAL-R target values, (functional) gain was adequate; however, the maximum output was low, especially for the Vibrant Soundbridge device.     

Is virtual endoscopy of the middle ear useful?

Virtual endoscopy is a relatively new imaging technology in otology, and therefore data on its efficacy in clinical situations are limited. We conducted a prospective study to evaluate the clinical relevance of radiologic diagnoses based on virtual endoscopy of the middle ear. Our patient population was made up of 30 adults who were scheduled to undergo surgery to correct conductive hearing loss of unknown etiology. Virtual endoscopy was performed on three-dimensional images that were constructed from images obtained with conventional two-dimensional computed tomography (CT). Findings on virtual endoscopy were then compared with the subsequent surgical findings. Virtual endoscopy suggested a middle ear pathology in 19 patients and a normal middle ear in 11 patients. Postoperatively, we found that the virtual diagnoses correlated moderately well with the surgical findings in the group of patients with predicted pathology; 13 of these 19 patients were found to have middle ear problems such as ossicular chain anomalies, otosclerosis, and cholesteatoma (positive predictive value: 68%). However, among the 11 patients whose middle ear structures were radiologically predicted to be normal, only 2 had negative middle ear findings on surgical exploration; of the remaining 9 patients, 8 had otosclerosis and 1 had malleus fixation (negative predictive value: 18%). Thus, the sensitivity and specificity of virtual endoscopy were 59 and 25%, respectively. Virtual endoscopy provides images from a surgeon's perspective, and so it has the potential to be useful in the preoperative evaluation of the middle ear cavity. With ongoing advancements in computer systems and imaging techniques, the cost, reliability, and efficacy of virtual endoscopy may improve. However, further clinical validation and cost-benefit analysis are required before we can determine if it has any additional advantages over conventional two-dimensional CT.     

Papillary tumor of the inner ear: adenoma or carcinoma?

A 46-year-old female patient presented relapsed left-sided hearing loss and progressive left facial nerve palsy. At first, the computed tomography (CT) scan and magnetic resonance (MR) imaging did not indicate a diagnosis of a tumorous lesion. Nine years after the first symptoms, a destructive cystic neoplasm of the pars petrosa of the temporal bone was then evident. The lesion was surgically removed by radical resection. Histologically, a papillary tumor of the endolymphatic sac was diagnosed. These tumors tend to become destructive and more or less aggressive, but they do not metastasize. Therefore, the best term for them should be "aggressive papillary tumors of the endolymphatic sac" to avoid classification as an adenoma or adenocarcinoma.     

Open vs closed type congenital cholesteatoma of the middle ear: Two distinct entities or two aspects of the same phenomenon?

ObjectiveThe clinical features and surgical results of “closed type” versus “open type” congenital cholesteatoma were compared in order to analyse the differences between the two forms; whether the morphology of the disease may have a role in the staging systems has been also evaluated.Patients and methodsWe reviewed retrospectively 95 patients (96 ears) who underwent surgery for congenital cholesteatoma over a 15-year period focusing on the clinical differences between open and closed type congenital cholesteatoma.ResultsSeventy-one patients (74%) had a closed-type and 25 (26%) an open type congenital cholesteatoma. Our study confirmed the higher prevalence of the closed type, as well as, a younger age at initial diagnosis compared with the open type congenital cholesteatoma. Other differences between the two forms were: modality of diagnosis (pathognomonic otoscopy in 100% of the closed type and in 40% of the open type), positive history for otitis media with effusion (51.4% in closed type vs 20% in open type), involvement of the tympanic membrane quadrants (anterior quadrants were more frequently involved in the closed forms, whereas posterior quadrants were more frequently involved in the open forms), disease extension and aggressiveness. A residual cholesteatoma was found in 6 out of the 71 patients (8.4%) with a closed type congenital cholesteatoma and in 10 out of the 25 patients (40%) with an open type congenital cholesteatoma. After adjusting for potential confounders, open-type congenital cholesteatoma was significantly associated with residual cholesteatoma compared to the closed-type (odds ratio [OR] 7.39, 95% confidence interval [CI] 1.10–49.77, p = 0.03).ConclusionThis study confirmed that the open congenital cholesteatoma has global clinical features that are uniquely different from the classical closed form. These differences could reflect a distinct pathogenesis, but there is no proof of this to date. The classification of the congenital cholesteatoma could be further refined by adding the morphologic type of the disease.     

Sinonasal inverted papilloma with malignant transformation in the middle ear: a multicentric origin?

Inverted papilloma is a rare, benign tumour representing only 0.5 to 4 per cent of all sinonasal neoplasms; its involvement of the middle ear is extremely rare. We present a case of multicentric inverted papilloma in the sinonasal region and middle ear in a 54-year-old man. The patient later developed neck metastasis secondary to malignant transformation of the inverted papilloma in the middle ear.     

pediatric middle ear congenital cholesteatoma: a case report

Congenital cholesteatoma (CC)is a rarely seen benign tumor of the temporal bone. There are five general sites of extradural occurrence: the midd leear, external auditory meatus, mastoid, squamous portion and the petrous apex of the temporal bone. CC grows slowly and presents no symptoms at the early stage. Delayed and mis-diagnosis are common with this condition 1.     

Glial lesion of the infratemporal fossa presenting as a soft tissue middle ear mass - rudimentary encephalocele or neural crest remnant?

We report about ectopic glial tissue of the skull base and the parapharyngeal space presenting as a soft tissue mass in the middle ear. An 11-year-old boy presented with bilateral conductive hearing loss since early childhood. The history included previously removed lesions consistent with ectopic neuroglial tissue of the tongue and the parapharyngeal space soon after birth, as well as surgery for cleft palate. High resolution computed tomography of the petrous bone and magnetic resonance imaging were useful in identifying the skull base defect and in characterizing the lesion's relation to the brain. There was no clinical, radiological or surgical evidence of any associated dural defect. The lesion was removed via a modified infratemporal approach. Histology revealed neuroglial tissue with calcifications without any signs of mesodermal or entodermal origin. On the basis of this case the pathogenesis and diagnosis of ectopic brain tissue and its relation to the more commonly encountered meningoencephalic herniations are reviewed. Furthermore therapeutical implications are discussed.     

Obstruction of the eustachian tube orifice and pressure changes in the middle ear: are they correlated?

OBJECTIVES: We performed a cross-sectional study to investigate whether the obstruction of the eustachian tube orifice due to adenoid hyperplasia changes the pressures in the middle ear. METHOD: Fifty consecutive children 2 to 12 years of age with nasal obstruction were examined from May to October 2005. Adenoid size and status of the eustachian tube orifice were assessed with nasal flexible fiberoptic endoscopy. Tympanometry was used to evaluate the middle ear. RESULTS: In children with occlusion of the eustachian tube orifice by adenoid tissue, 87% had abnormal pressure in the middle ear according to tympanograms. When orifices were not occluded, 86% of the tympanograms were normal (p < .001). CONCLUSIONS: Obstruction of the eustachian tube orifice by adenoid tissue was associated with tympanograms suggestive of abnormal pressure in the middle ear. Future studies with a larger sample size are necessary to clarify this association.     

To pack or not to pack? A contemporary review of middle ear packing agents

Middle ear packing agents are used in otologic surgery to provide support to the middle ear structures, maintain aeration of the middle ear, and promote hemostasis. However, there is currently a lack of standardization regarding the use of different types of packing agents. The choice of materials and how they are used remain controversial. In fact, some have recently advocated for no packing. In view of this, this review focuses on the types of materials available, a brief historical account of each material, characteristics of an ideal packing agent, and a discussion on the techniques of insertion to optimize surgical outcomes.     

Fibro-osseous lesion of the middle turbinate: ossifying fibroma or fibrous dysplasia?

Benign fibro-osseous tumours of the head and neck region seldom appear in the midface and nasal cavity. Correct differential diagnosis of fibro-osseous tumours is crucial for adequate therapy, as their clinical aggressiveness seems to differ. The rather uncommon case of a 14-year-old boy with a tumour of the middle turbinate is presented. The radiologic appearance of the tumour, on computed tomography and magnetic resonance imaging scans, was consistent with fibrous dysplasia. Angiography revealed extensive vascularisation of the tumour from both the internal and external carotid arteries. To avoid ipsilateral blindness following embolisation, a superselective embolisation of the supplying blood vessels was performed. The tumour was completely resected via an endoscopic approach. Histopathology revealed an ossifying fibroma. This case emphasises the importance of interpretation of the clinical, radiological and histological features before planning definitive treatment. Moreover, when fibro-osseous tumours are suspected, the possibility of extensive, complicated vascularisation must be considered. This case underwent radical resection, with no recurrence after four years' follow up.     

The use of middle ear endoscopy: has residual cholesteatoma been eliminated?

Oto-endoscopes allow surgeons to control blind pockets within the middle ear. Many surgeons credit the reduction of residual cholesteatoma in their practice to the use of oto-endoscopes. Since 1988, the author has been using rigid endoscopes in combination with the operating microscope in cholesteatoma surgery. Between 1988 and 1999, 231 primary cholesteatoma operations were performed. The operations included closed cavity mastoidectomy (53 patients), small cavity mastoidectomy (115 patients), open mastoidectomy with primary canal wall reconstruction (44 patients) or mastoid obliteration (19 patients). The median follow-up period was 6.5 years. The incidence of residual cholesteatoma is closed cavity mastoidectomy (9.4 per cent) was found to be similar to that of open cavity mastoidectomy (8.7 per cent). Site analysis of the residual cholesteatoma revealed an incidence of 3.6 per cent in the epitympanum, 10.5 per cent in the sinus tympani and 0.7 per cent in the mastoid bowl. This study showed that residual cholesteatoma has not been eliminated with the use of middle-ear endoscopy, although the incidence has reduced the figures for closed cavity mastoidectomy to single figures.     

How do metallic middle ear implants behave in the MRI?

BACKGROUND: Magnetic resonance imaging (MRI) has gained in frequency and importance as a diagnostic procedure. In respect to the close anatomical relationship in the temporal bone it is necessary to know whether it is hazardous to patients with metallic middle ear implants regarding displacement and rise in temperature. For the MR image quality artefacts caused by metallic prostheses should be low. METHODS: Four different stapes prostheses made from titanium, gold, teflon/platinum and teflon/steel, a titanium total ossicular reconstruction prosthesis (TORP) and two ventilation tubes (made from titanium and gold) were tested in a 1.5 Tesla MRI machine regarding their displacement. All objects were first placed in a petri dish, then suspended from a thread and finally immersed in a dish filled with Gadolinium. Temperature changes of the implants were recorded by a pyrometer. RESULTS: None of the implants moved when they were placed in the petri dish or suspended from the thread. On the water surface the teflon/platinum and the teflon/steel pistons adjusted their direction with their axis longitudinally to the MRI scanner opening and the teflon/steel piston floated towards the MRI-machine when put close enough to the scanner opening. No rise in temperature was recorded. All implants showed as little artefacts that would still make an evaluation of the surrounding tissue possible. CONCLUSION: Patients with any of the metallic middle ear implants that were examined in this study may undergo MRI-investigations without significant adverse effects.     

middle ear implants: historical and futuristic perspective

Technological advancements are continuously changing the paradigm of human existence.Human beings are constantly engaging in various measures to reduce the extent of sensory and motor impairment.This h...     

Does Helicobacter pylori colonize the nasopharynx of children and contribute to their middle ear disease?

OBJECTIVE: There is growing interest in studying the presence of HP in the upper aerodigestive tract. It was shown in several pilot studies that it colonizes the area, while other authors found no evidence of its presence there and a third group of authors believed that it had only a transient presence there. In this study we investigated a possible role for HP in middle ear disease in children. MATERIAL AND METHODS: Consecutive patients undergoing myringotomy and adenoidectomy for chronic otitis media with effusion or recurrent otitis media were enrolled. Middle ear fluids were cultured on three types of agar plate (Brucella + laked horse blood; Brucella + sheep blood; and chocolate). A double polymerase chain reaction (PCR) was run to detect urease-C and adhesion subunit genes. Rapid urease enzyme testing and PCR were used on the adenoid specimens. Parents were interviewed regarding symptoms suggestive of gastroesophageal reflux in their children. RESULTS: Eighteen patients were enrolled in the study (mean age 4.4 years; age range 3-8 years) with an equal gender distribution. All 28 middle ear fluid cultures were negative in all 3 media. Twenty-one of the 28 samples contained DNA, yet PCR revealed that none of them belonged to HP. Ten of the 13 adenoid specimens obtained were positive on rapid urease testing, but none on PCR. Seven of the 18 patients had at least 1 symptom suggestive of gastroesophageal reflux during the 6 months preceding the study but this did not have an impact on any of the results. CONCLUSION: There was no evidence from this study that Helicobacter pylori (HP) colonizes the nasopharynx of children with middle ear disease, whether dyspeptic or not. There is also no apparent role for this bacterium in middle ear pathology.