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.     

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.     

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.     

Hearing loss associated with ear infections in Nord-Trøndelag, Norway

OBJECTIVE: We have assessed the effect of recurrent childhood ear infections on adult hearing. We also examined whether adult hearing is poorer with an early age of onset of ear infections compared with later onset. DESIGN: A population-based cohort of 50,398 subjects, 20 yr of age or older, were examined with air-conduction, pure-tone audiometry and reported if they had had recurrent ear infections (EI) in childhood (or subsequently) with the age of onset. RESULTS: There were poorer hearing thresholds associated with recurrent EI for all frequency ranges from 0.25 kHz to 8 kHz, regardless of age or gender. The effect increased with age from approximately 2 dB among younger subjects (20 to 44 yr old) to approximately 5 to 6 dB among older subjects (65 yr or older). Among younger subjects, the effect of EI was somewhat stronger for men, whereas among older subjects the effect was somewhat stronger for women. Early age of onset for EI was associated with poorer hearing thresholds than late onset. The mean loss was close to 9 to 10 dB for all frequency ranges among older subjects reporting onset of EI before 2 yr of age. The mean loss was only about 4 dB among older subjects reporting onset of EI after 7 yr of age. CONCLUSIONS: Reported EIs are associated with similar consequences in terms of reduced hearing across frequencies 0.25 to 8 kHz for male and female subjects. The observed effects are stronger among older than among younger subjects, perhaps because effects increase with age or possibly because EI affected the hearing more before 1940 than during subsequent decades. Reported early age of onset of EI increases the risk of a substantially reduced hearing level later in life.     

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.     

Palatoplasty decreases the re-insertion rate of middle ear ventilation tube in cleft palate children – A population-based birth cohort study

Conclusion: Palatoplasty can significantly decrease their middle ear re-intubation rate with a relatively lower hazard ratio compared to children who underwent VTI only. Objectives: In children with cleft palate, questions remain about the overall effect of ventilation tube insertion (VTI) and palatoplasty for their OME. A large-scale study might offer more evidence for the roles of palatal surgery. Subjects and methods: This was a retrospective birth cohort study based upon a national database. We analyzed children born between 1999–2004 and diagnosed as cleft palate and/or lips. These children, according to their surgeries, were separated into two groups: (1) VTI only, and (2) VTI and palatoplasty. Kaplan-Meier analysis and log-rank test were used to calculate their cumulative tube re-insertion rates. Their hazard ratios of tube re-insertion were also analyzed. Results: In total, 1205 cleft children were collected, with 151 in the VTI only group and 1054 in the VTI?+?palatoplasty group. Ventilation tube re-insertion rates were significantly lower in the VTI?+?palatoplasty group (p?=?0.002). The cumulative re-insertion rates also showed a significant difference (p?=?0.001). When compared to the VTI only group, the adjusted hazard ratio was 0.528 in the VTI?+?palatoplasty group (p?=?0.001).     

Do middle ear muscles trigger attacks of Menière's disease?

OBJECTIVE: Current surgical methods for treating Ménière's disease (MD), e.g. endolymphatic sac surgery, mastoidectomy or insertion of middle ear ventilation tubes, only improve vertigo and fail to influence the hearing threshold. In this retrospective study we examined the long-term effects of tenotomy on the symptoms of MD. MATERIAL AND METHODS: A total of 45 patients suffering from definite MD underwent middle ear muscle tenotomy, a treatment that is very rarely considered nowadays. Of these 45 patients, 20 were observed postoperatively for a period of > 2 years. The disease was scored pre- and postoperatively and the results were evaluated according to the American Academy of Otolaryngology-Head and Neck Surgery Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy for MD. RESULTS: Sectioning of the tensor tympani and stapedius muscle tendons significantly reduced the frequency and intensity of vertigo and improved both the functional profile and tinnitus. Pure-tone audiometry at frequencies between 500 and 3000 Hz substantiated the improvement in hearing threshold, as did the pure-tone average. The therapeutical outcome was stable in cases where both tendons remained persistently disconnected. Intratympanal inflammatory reactions were observed in most patients during surgery. CONCLUSION: Our results prove that tenotomy is a successful and enduring therapeutic approach for treating the auditory and vestibular symptoms of MD and strongly suggest that it should be reconsidered as a promising surgical treatment for the symptoms of MD.     

Expression levels of receptor activator of nuclear factor-κB ligand and osteoprotegerin are associated with middle ear cholesteatoma risk

Conclusion: Overexpression of receptor activator of nuclear factor-κB ligand (RANKL) and low expression of osteoprotegerin (OPG) are typical features in middle ear cholesteatoma patients. The altered RANKL/OPG protein ratio suggests that alterations in the RANKL-OPG pathway may be major factors in the pathogenesis of middle ear cholesteatoma. Objective: Our meta-analysis explored the contribution of one important cytokine pathway, the RANKL and OPG pathway, in the development of middle ear cholesteatoma. Methods: We screened Embase, the Cochrane Library, CISCOM, CINAHL, Google Scholar, China BioMedicine, China National Knowledge Infrastructure, PubMed, and Web of Science for relevant articles. RANKL expression and ratio of RANKL/OPG were analyzed using Comprehensive Meta-Analysis Version 2 software. Results: The electronic literature search identified five studies that contained information on the correlation of RANKL and OPG expression with middle ear cholesteatoma. Increased RANKL expression positively correlated with middle ear cholesteatoma, while OPG expression showed an inverse association (p < 0.05). The ratio of RANKL/OPG in middle ear cholesteatoma cases was higher than in healthy controls, indicating that our observations are applicable to each individual case. Subgroup analysis based on country of study revealed that OPG levels decreased in China and Korea, and high RANKL expression was found in Poland, China, and Korea (all p < 0.05).     

Why do some children have good hearing results following type III and IV tympanoplasty? Current theories of middle ear mechanics

INTRODUCTION: Middle ear reconstruction in children following tympanomastoidectomy for cholesteatoma is commonly limited to a Wullstein type III or IV reconstruction owing to ossicular erosion. The hearing outcomes of this procedure have been unpredictable. Nevertheless, there are children who have remarkably good hearing results despite having extensive and aggressive cholesteatoma surgery and limited reconstruction. METHODS: The current theories of middle ear mechanics following tympanoplasty and ossicular reconstruction are reviewed. In addition, a selective retrospective chart review of pediatric type III and IV tympanoplasty at The Hospital for Sick Children between 1998 and 2003 is presented. RESULTS: Nine patients were reconstructed with a type III (n = 3) or IV (n = 6) tympanoplasty. The mean pre- and postoperative air-bone gaps were 43.6 and 24.9 dB. Speech reception threshold improved from 37.5 to 22.8 dB. The changes were statistically significant (p < .05). CONCLUSIONS: This series of patients demonstrated a statistically significant hearing improvement at long-term follow-up. The improvements are consistent with optimal hearing outcomes predicted by current theories of middle ear mechanics.     

Teratoma of the middle ear: a real entity or a non-entity?

Space-occupying lesions of the middle ear range from wholly benign developmental anomalies to highly virulent and aggressive malignancies. Amongst the benign entities classed with the family of developmental anomalies, the middle ear choristoma is well known. But what of the middle ear teratoma? Recent reports suggest that, while rare, teratomas of the middle ear do exist. Middle ear teratomas are defined as tumors consisting of benign tissues derived from all three embryologic layers: endoderm, mesoderm and ectoderm. They are lesions of the neonatal period and early infancy. While complete surgical excision will be curative, a thorough histologic examination of the specimen is recommended to exclude the possibility of immature and/or frankly malignant elements being admixed with the benign teratomatous elements.     

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.     

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.     

Lysis of the long process of the incus secondary to Vibrant SounBridge® middle ear implants,treated with hydroxyapatite bone cement

We report two patients presenting with delayed complicationsafter Vibrant SounBridge® middle ear implant surgery: in both cases, a revision surgery was performed and lysis of the long process of the incus was highlighted. A re-assembly of the clip around the remaining long process of the incus was performed, associated with hydroxyapatite bone cement application, on the clip and the incudo-stapedial joint. Both patients had a satisfying result, with a mean follow up of 12 months.     

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.     

Do Swiftlets have an ear for echolocation? The functional morphology of Swiftlets' middle ears

The Oilbird and many Swiftlet species are unique among birds for their ability to echolocate. Echolocaters may benefit from improved hearing sensitivity. Therefore, morphological adaptations to echolocation might be present in echolocating birds' middle ears. We studied the functional morphology of the tympano-ossicular chain of seven specimens of four echolocating Swiftlet species and one specimen each of five non-echolocating species. Three dimensional (3D) reconstructions were made from micro-Computer-Tomographic (muCT) scans. The reconstructions were used in functional morphological analyses and model calculations. A two dimensional (2D) rigid rod model with fixed rotational axes was developed to study footplate output-amplitudes and to describe how changes in the arrangement of the tympano-ossicular chain affect its function. A 3D finite element model was used to predict ossicular-chain movement and to investigate the justification of the 2D approach. No morphological adaptations towards echolocation were found in the middle-ear lever system or in the mass impedance of the middle ear. A wide range of middle-ear configurations result in maximum output-amplitudes and all investigated species are congruent with these predicted best configurations. Echolocation is unlikely to depend on adaptations in the middle ear tympano-ossicular chain.