Pediatric middle ear cholesteatoma: the comparative study of congenital cholesteatoma and acquired cholesteatoma.

This study examined the differences between congenital cholesteatoma (CC) and acquired cholesteatomas (AC) in children by comparing clinical features and treatment courses. This was a retrospective study which retrospectively evaluated 127 children with middle ear cholesteatomas using medical records from January 1999 to December 2012 in the Department of Otolaryngology, Niigata University Hospital. The study comprised 69 and 58 cases of CC and AC, respectively. The main outcome measures include patient backgrounds, the opportunities for consultations, mastoid cell development, intraoperative finding of stapes, surgical procedure and number of surgeries. The average age at operation was 6.4 and 9.8 years in CC and AC, respectively. AC was more prevalent in boys. Mastoid development was better in CC than in AC. We adopted a two-stage operation in 17 cases (25 %) of CC and in 22 cases (38 %) of AC. The repeat surgery rate was 11.6 % in CC and 27.6 % in AC. Three times as many operations were required for three cases (4.3 %) of CC and 10 cases (17.2 %) of AC. The lesions in AC were more difficult to control. In the treatment of pediatric middle ear cholesteatoma, we had to keep the outcome in mind.

A Survey of the Otorhinolaryngological Screening of Children in Niigata Prefecture: Kindergarten to High School.

We conducted the first survey of otorhinolaryngological (ORL) screening at educational institutions, from kindergarten to high school, in Niigata Prefecture. The survey results showed that ORL screening is not performed in 62.1% of kindergartens and nursery schools, and that screening was conducted by non-ENT doctors in 23.9% of them. At elementary school entry health check-ups, ORL screening was performed by otorhinolaryngologists in only 4.2% of children overall. ORL screening was conducted in students in all grades by 51.7% of all elementary schools and 31.6% of all junior high schools. Audiometry was performed in students in all grades by over 80% of elementary schools and junior high schools. With regard to high schools, ORL screening was performed in students in all grades at only three schools among 105 high school; ORL screening and audiometry were conducted primarily in the first-year students. In addition to the above results, the survey revealed that no ORL screening whatsoever was performed during a period of 9 years at an elementary school and a junior high school in a certain municipality; as a result of discussion with the relevant municipality, it was decided that screening will be conducted starting at 2016. From the viewpoint of reinforcing the structure of conducting ORL screening in infants in whom ORL findings are identified at a high frequency, we requested that Niigata City conduct ORL screening at private kindergartens in Niigata City where the ORL screening rate is low. We consider this survey to have been productive, as it identified meaningful new facts and measures that could be devised to address the relevant issues. It is our aim to become more proactively than ever involved in school health including health check-ups.

[Clinical Study of Skull Base Osteomyelitis].

Typical osteomyelitis is reportedly caused by Pseudomonous aeruginosa in elderly diabetic patients after malignant external otitis. Recently, complications have arisen due to the emergence of atypical osteomyelitis. We have experiensed 5 cases of skull base osteomyelitis at our hospital. All patients were male with a mean age of 75.2 years. Four patients had diabetes. Regarding the clinical and radiographic findings, patients 1, 2, and 3 had typical osteomyelitis after malignant external otitis, whereas patients 4 and 5 had atypical osteomyelitis without temporal bone findings. Sample culturing revealed Pseudomonas aeruginosa in 4 cases and Aspergillus in one. Intravenous antibiotics were administered to all patients. Two patients responded positively and survived, while 3 died. Typical osteomyelitis is reportedly caused by P. aeruginosa in elderly diabetic patients after malignant external otitis. Recently, complications have arisen due to the emergence of atypical osteomyelitis. The prognosis of skull base osteomyelitis is still poor in Japan. Early diagnosis and long-term antibiotic administration is required to improve outcome.

[A Case of the Relapsing Polychondritis Limited to the Trachea].

A 62-year-old woman, who had dysesthesia in the throat, and polyposia the previous year, was admitted in a coma because of respiratory failure. Computed tomography scans demonstrated dilatation of the esophagus and stenosis of the trachea. After emergency intubation, extubation was not possible due to a collapsed trachea, so we performed a tracheostomy. The tracheoscopy from the stoma showed an esophagus-like trachea due to disappearance of the tracheal cartilage and the straight pattern on the membranous portion, and the lumen deformed with coughing. With the continuous dyspnea episode, the patient was diagnosed as having tracheomalacia. Anti-type II collagen antibody and pathological findings of the trachea led us to the diagnosis of Relapsing Polychondritis.

Surgical management of retropharyngeal nodes metastases from papillary thyroid carcinoma.

BACKGROUND: Papillary thyroid carcinoma (PTC) often metastasizes to the central and lateral neck lymph nodes, but rarely affects retropharyngeal nodes (RPN). METHODS: We retrospectively reviewed 12 patients (three men, nine women) with PTC who underwent dissection of RPN metastases between 1994 and 2012. Mean age at dissection was 65 years (range 23-77). RESULTS: Dissection was performed with the initial surgery for PTC in eight patients, while the remaining four patients underwent dissection as secondary surgery. RPN metastases arose from an ipsilateral primary in nine patients, bilateral in two, and contralateral in one, with primaries located at the superior pole of the thyroid lobe in nine patients. All patients showed simultaneous or previous lymph node metastases in the upper jugular chain. A transcervical approach was applied for RPN dissection in 11 patients, while a transcervical-transparotid approach was applied in the remaining patient. No patients needed mandibulotomy or showed severe complications. Median duration of follow-up after RPN dissection was 48 months (range 3-206). No recurrences in the retropharyngeal space were identified. Two patients died of the disease, one died from other carcinoma, and five survived with distant metastases from PTC. The remaining four patients remain free of the disease as at the time of writing. CONCLUSIONS: If lymph node metastases are identified in the upper jugular chain, the possibility of RPN metastases should be checked. Most RPN metastases from PTC can be dissected safely without mandibulotomy. In particular, low-risk patients can expect favorable outcomes.

Prevalence of human papillomavirus in mobile tongue cancer with particular reference to young patients.

The carcinogenetic role of human papillomavirus (HPV) in mobile tongue cancer remains unclear because of conflicting results reported in the literature. This disparity is likely to be due to variations in the samples and methods used. Furthermore, despite a tendency for increased prevalence of mobile tongue cancer in young adults, only a few reports specifically in young patients have been published. In the present study on 32 patients, including six in their 20s, we genotyped the prevalence of HPV using a highly sensitive detection tool in fresh-frozen samples from surgical specimens and a novel detection device with electrochemical DNA chip and loop-mediated isothermal amplification. In addition, we confirmed HPV prevalence by in situ hybridization and immunohistochemistry for the p16(INK4a) protein, regarded as a biomarker of HPV-associated cancers. The frequency of 13 genotypes of high-risk HPV was 0/32 (0%), which was further confirmed by in situ hybridization. Overexpression of p16(INK4a) protein was observed in six of the 32 patients (19%), with four (67%) also overexpressing p53. Because there is usually a lack of p53 overexpression in HPV-associated cancer, it is unlikely that p16(INK4a) protein overexpression is correlated with HPV infection. Consequently, it is unlikely that HPV infection plays an important role in mobile tongue carcinogenesis, in particular in young adults. In addition, our data suggest that the overexpression of p16(INK4a) protein is not an appropriate biomarker for HPV association in mobile tongue carcinogenesis.

[Concurrent chemoradiotherapy with either CDDP or CDGP, plus 5-FU for squamous cell carcinoma of the oropharynx and hypopharynx].

Clinical outcomes of 53 patients with oropharyngeal and hypopharyngeal squamous cell carcinoma, treated from January, 2000 to December, 2008 by concurrent chemoradiotherapy of either CDDP or CDGP, plus 5-FU were investigated. Patients were treated with either CDDP (70 mg/m2) or CDGP (100 mg/m2) on day 1 of the chemotherapy regime, with 5-FU (700 mg/m2/day) as a continuous infusion for 5 days. Each regimen was administered as two courses in the first and final weeks of radiotherapy. Radiotherapy was administered at a daily dose of 2 Gy for five days a week, with patients receiving a total of 70 Gy by the end of seven weeks. The primary cancer was located in the oropharynx and hypopharynx in 21 and 32 patients, respectively. Twenty-six patients (49.1%) had stage IVA disease and 10 patients (18.9%) had overall stage I to II disease. Acute adverse events such as pharyngeal mucositis and leucopenia occurred in 49.1% and 43.4% of patients, respectively, and second round chemotherapy was not commenced in 56.6% of patients (n=30) due to significant adverse events. Mean weight loss following treatment was 4.1 kg. After a median follow-up of 30.0 months, 3-year overall survival was 53.0% for advanced carcinoma of the oropharynx and hypopharynx. Five-year overall survival was 46.4%. Patients receiving two courses of chemotherapy had an improved 5-year survival compared to patients receiving one course (67.0% vs 32.8%). Results indicate a significant benefit from two courses of chemotherapy. As such, minimizing the incidence of adverse effects and thereby reducing treatment discontinuation will likely improve overall treatment outcomes.

[MRI-CT fusion image navigation during surgery for inverted papilloma].

We report two cases of intraoperative computer-aided surgery using magnetic resonance imaging (MRI)/computed tomography (CT) fusion imaging for inverted papilloma (IP). Case 1: IP had spread to the frontal recess/sinus so we chose a combined endoscopic sinus surgery (ESS) and external approach. Case 2: For a maxillary sinus tumor, we combined ESS and the adjuvant external (Caldwell-Luc) procedure. This is helpful to surgeons when, shifting fusion imaging when opening the frontal sinus bone to obtain CT data, and shifting to MRI to detect the tumor pedicle, then approaching the bone defect using MRI-CT fusion imaging (50-50%).

[A case of difficult-to-diagnose hereditary angioedema].

Hereditary angioedema (HAE) due to an inherited C1-inhibitor (C1-INH) deficiency causes localized swelling of the oral cavity, pharynx, larynx, and face, that may be life-threatening when the larynx is involved. A 26-year-old woman seen 3 times previously for pharyngeal or laryngeal edema while in her teens, and seen this time for dyspnea was found in computed tomography (CT) to have esophageal edema and pleural effusion. Her C1-INH activity was low, yielding a definitive diagnosis of HAE for her 10-year-plus-disease history. n mind in th e f idiopathicedema. should be kept i While it is comparatively rare, HAE differential diagnosis o

Characterisation of N-glycans in the epithelial-like tissue of the rat cochlea.

Membrane proteins (such as ion channels, transporters, and receptors) and secreted proteins are essential for cellular activities. N-linked glycosylation is involved in stability and function of these proteins and occurs at Asn residues. In several organs, profiles of N-glycans have been determined by comprehensive analyses. Nevertheless, the cochlea of the mammalian inner ear, a tiny organ mediating hearing, has yet to be examined. Here, we focused on the stria vascularis, an epithelial-like tissue in the cochlea, and characterised N-glycans by liquid chromatography with mass spectrometry. This hypervascular tissue not only expresses several ion transporters and channels to control the electrochemical balance in the cochlea but also harbours different transporters and receptors that maintain structure and activity of the organ. Seventy-nine N-linked glycans were identified in the rat stria vascularis. Among these, in 55 glycans, the complete structures were determined; in the other 24 species, partial glycosidic linkage patterns and full profiles of the monosaccharide composition were identified. In the process of characterisation, several sialylated glycans were subjected sequentially to two different alkylamidation reactions; this derivatisation helped to distinguish α2,3-linkage and α2,6-linkage sialyl isomers with mass spectrometry. These data should accelerate elucidation of the molecular architecture of the cochlea.

Transcranial photo-inactivation of neural activities in the mouse auditory cortex.

Flavoprotein fluorescence in the brain is intimately coupled with neuronal aerobic energy metabolism. If flavoproteins are photobleached, neural activities may be affected owing to dysfunction in aerobic energy metabolism in mitochondria. We tested this possibility in cortical slices from mice, and found that exposure to blue light (lambda = 475 nm) derived from a 20 mW diode laser for 50 min suppresses trans-synaptic components of field potentials. This finding formed the basis of a transcranial photo-inactivation technique, that was used to investigate auditory signal transmission between the anterior auditory field (AAF) and the primary auditory cortex (AI) in anesthetized mice. Cortical responses in AAF and AI, elicited by 5 kHz tonal stimuli, were visualized using transcranial flavoprotein fluorescence imaging. After determining responsive areas in AAF and AI, the auditory cortex was exposed to the blue diode laser via the intact skull, while either AAF or AI was protected with a piece of carbon paper. Although the photo-inactivation of AI had no significant effect on the fluorescence responses in AAF, the photo-inactivation of AAF significantly reduced the fluorescence responses in AI, indicating the presence of auditory signal transmission from AAF to AI.

Head-tilting stabilometry in patients with benign paroxysmal positional vertigo.

OBJECTIVE: One of the pathologic conditions underlying benign paroxysmal positional vertigo (BPPV) is degeneration of the otolith organs. In this study, we examined changes in the parameters of stabilometry under an upright condition and head-tilt conditions in patients with BPPV. METHODS: We performed stabilometry on 21 patients with right BPPV, on 21 patients with left BPPV and on 21 controls. First, the subject stood barefoot in an upright position with both feet together on the platform with eyes closed. Next, tilting of the head about 30 degrees to the left was added. Then, tilting about 30 degrees to the right was performed. RESULTS: In right BPPV patients, the total length of velocity vectors in the right or left direction on right or left head-tilt were significantly smaller than those in an upright position. The enveloped area and total length of velocity vectors in the right or left direction were significantly larger than those in controls. In left BPPV patients, there were no parameters that showed any significant difference. CONCLUSIONS: In this study, lesions of right BPPV patients were coincidentally more severe than those in left BPPV patients, and velocity vectors with head-tilts were significantly smaller than in an upright position. Using the total length of the velocity vectors, head-tilting stabilometry has the potential to become a reliable otolith function examination method.

Molecular diversity of clustered protocadherin-α required for sensory integration and short-term memory in mice.

Clustered protocadherins (Pcdhs) are neuronal cell adhesion molecules characterized by homophilic adhesion between the tetramers of 58 distinct isoforms in mice. The diversity of Pcdhs and resulting highly-specific neuronal adhesion may be required for the formation of neural circuits for executing higher brain functions. However, this hypothesis remains to be tested, because knockout of Pcdh genes produces abnormalities that may interfere with higher brain functions indirectly. In Pcdh-α1,12 mice, only α1, α12 and two constitutive isoforms are expressed out of 14 isoforms. The appearance and behavior of Pcdh-α1,12 mice are similar to those of wild-type mice, and most abnormalities reported in Pcdh-α knockout mice are not present in Pcdh-α1,12 mice. We examined Pcdh-α1,12 mice in detail, and found that cortical depression induced by sensory mismatches between vision and whisker sensation in the visual cortex was impaired. Since Pcdh-α is densely distributed over the cerebral cortex, various types of higher function are likely impaired in Pcdh-α1,12 mice. As expected, visual short-term memory of space/shape was impaired in behavioral experiments using space/shape cues. Furthermore, behavioral learning based on audio-visual associative memory was also impaired. These results indicate that the molecular diversity of Pcdh-α plays essential roles for sensory integration and short-term memory.

Daytime hypoxemia, sleep-disordered breathing, and laryngopharyngeal findings in multiple system atrophy.

BACKGROUND: The mechanism underlying nocturnal sudden death in patients with MSA remains unclear. It may be explained by upper airway obstruction, such as vocal cord abductor paralysis; an impairment of the respiratory center, such as Cheyne-Stokes respiration; or an impaired hypoxemic ventilatory response. OBJECTIVE: To investigate the mechanism of sleep-disordered breathing in multiple system atrophy (MSA). DESIGN: We recruited 21 patients with probable MSA who were admitted sequentially to our hospital, and performed daytime blood gas analysis, pulmonary function tests, polysomnography, and fiberoptic laryngoscopy during wakefulness and with the patient under anesthesia. RESULTS: A decrease in arterial oxygen pressure and an increase in alveolar-arterial oxygen gradient significantly correlated with disease duration (P = .045 and .046, respectively). Polysomnography demonstrated Cheyne-Stokes respiration in 3 (15%) of 20 patients. Fiberoptic laryngoscopy during wakefulness showed that 3 (14%) of the 21 patients exhibited vocal cord abductor paralysis, and laryngoscopy under anesthesia showed that 9 (45%) of 20 patients exhibited vocal cord abductor paralysis. Laryngoscopy under anesthesia also revealed that 11 (55%) of 20 patients showed upper airway obstruction in places other than the vocal cords, including obstruction at the base of the tongue or soft palate. In addition, it demonstrated novel laryngopharyngeal findings, such as floppy epiglottis and airway obstruction at the arytenoid. CONCLUSIONS: We observed daytime hypoxemia with an increased alveolar-arterial oxygen gradient, Cheyne-Stokes respiration, and novel abnormal laryngopharyngeal movements in patients with MSA. We also found that laryngoscopy under anesthesia might be useful for evaluating upper airway obstruction. The significance of these findings to the mechanism of sudden death in those with MSA needs to be examined.

Investigation into dizziness before and after Epley's maneuver for benign paroxysmal positional vertigo using stabilometry.

OBJECTIVE: To investigate the cause of dizziness after Epley's maneuver using stabilometry. METHODS: Subjects were 35 patients with posterior canal type BPPV. First we performed stabilometry, next Epley's maneuver, then stabilometry was performed again. The enveloped area and the locus length per second of stabilometry were compared. RESULTS: Seventeen patients reported a feeling of dizziness after the treatment, whereas the other 18 patients did not. The patients were divided into two groups: 17 patients with dizziness (WD) and 18 patients without dizziness (WOD) after the treatment. In the WD group, the enveloped area (P=0.0495) and the locus length per second (P=0.0099) before Epley's maneuver were significantly lower than those after Epley's maneuver. In the WOD group, there was no significant difference in the stabilometry parameter between values obtained before and after Epley's maneuver. There were no significant differences found in either parameter between these two groups either before or after applying Epley's maneuver. CONCLUSIONS: Epley's maneuver is a treatment for the posterior semicircular canal. Malfunction of the otolith organ remains. It is considered that the cause of dizziness after Epley's maneuver comes from otolith dysfunction.

[Clinical evaluation of patients with vocal cord palsy--rate and timing of phonation recovery by disease differences].

Vocal cord palsy (VCP) is common in otolaryngological clinics, as are postoperative patients with VCP at general and university hospitals. Although VCP treatment strategies have been discussed, decisions on treatment methods and timing are complicated by the spontaneous recovery exhibited by some patients. We studied clinical features of VCP patients and their histories. Subjects were 171 VCP patients seen at our phonation clinic during a 15-year period-18% of all phonation clinic cases. By gender, 69% were male and 31% were female, ranging in age from 4 to 89 (mean, 58) years. Some 18% were bilateral and 82% unilateral, with 71% of unilateral cases on the left. Postoperative VCP occurred in 59% of patients in common surgical procedures for esophageal and thyroid tumors. During follow-up, 58% spontaneously recovered phonation. Postintubation VCP had the best recovery at 82%. Half with phonation recovery had spontaneous recovery from VCP and the other half had compensation. Most VCP resolved within 1 year, but compensation occurred after 1 year, meaning that in treatment planning and explanation, to VCP should be considered curable within 1 year and compensation attained 1 year after VCP.

[Clinical evaluation of patients with pyriform sinus fistula].

Thirteen patients with pyriform sinus fistula treated surgically at our department were clinically evaluated. Twelve (92%) fistulae occurred in the left pyriform sinus, and one (8%) in the right. There were a mean of 4 infectious episodes before the final diagnosis. The median age at the first infection was 5 years, and the median age at surgery was 13 years, although there were 2 elderly patients (over 60 years old) in this series. Association with pharyngeal foreign body was suspected in the case with onset at 81 years of age. There was a history of previous surgery for cervical disease in 54% of the patients. The final diagnosis was based on delineation of the fistula by hypopharyngography, although coronal section of CT and MRI were also useful for understanding the morphopathology. The surgical procedure consisted of identification and staining of the fistula under direct hypopharyngoscopy, followed by identification and extirpation of the fistula until the distal end via a cervical approach. There was no recurrence of infections, although resection of the thyroid gland and identification of the recurrent laryngeal nerve were not always performed. The most important surgical principle is considered to be complete disconnection of the lesion from the hypopharynx, and complete identification and extirpation of the fistula by the procedure used at our department is considered to be a useful surgical strategy.

Risk Factors of Recurrence in Pediatric Congenital Cholesteatoma.

OBJECTIVE: To examine the risk factors of recurrence in pediatric congenital cholesteatoma. STUDY DESIGN: Retrospective chart review. SETTING: University hospital. PATIENTS: Sixty-seven patients having tympanic type of congenital cholesteatoma under 15-years old at surgery. INTERVENTIONS: Canal wall-up tympanomastoidectomy (n = 30) or transcanal atticotomy/tympanoplasty (n = 37) was performed depending on cholesteatoma extension, 16 of which were followed by second-look surgery. Preoperative computed tomography (CT) before second-look surgery or follow-up CT was performed to detect residual recurrence 1 year after the surgery. Cholesteatoma found at the second surgery was also included in the recurrence. All patients had no recurrent cholesteatoma at the last follow-up (median, 61 mo after surgery). MAIN OUTCOME MEASURES: Possible predictive factors were compared between the groups. RESULTS: Residual cholesteatoma and retraction cholesteatoma occurred in 21 and 6%, respectively. There was no significant difference in age, sex, and type of cholesteatoma (open or closed) between the groups; however, Potsic stage and status of stapes involvement were more advanced in the residual cholesteatoma group. All residual lesions could be detected by follow-up CT or by second-look surgery. All of four retraction cholesteatoma patients were male, young at the surgery and in stage IV. CONCLUSION: Recurrence mostly occurred as residual cholesteatoma, suggesting that CT is recommended as a follow-up tool for congenital cholesteatoma. Advanced lesions had the risk of residual cholesteatoma, suggesting that complete removal of epithelium is important. Although rare, young advanced-stage patients had risk of retraction cholesteatoma and therefore normal mucosa should be preserved as much as possible for these patients.

Creating an Optimal 3D Printed Model for Temporal Bone Dissection Training.

OBJECTIVE: Making a 3-dimensional (3D) temporal bone model is simple using a plaster powder bed and an inkjet printer. However, it is difficult to reproduce air-containing spaces and precise middle ear structures. The objective of this study was to overcome these problems and create a temporal bone model that would be useful both as a training tool and for preoperative simulation. METHODS: Drainage holes were made to remove excess materials from air-containing spaces, ossicle ligaments were manually changed to bony structures, and small and/or soft tissue structures were colored differently while designing the 3D models. The outcomes were evaluated by 3 procedures: macroscopic and endoscopic inspection of the model, comparison of computed tomography (CT) images of the model to the original CT, and assessment of tactile sensation and reproducibility by 20 surgeons performing surgery on the model. RESULTS: Macroscopic and endoscopic inspection, CT images, and assessment by surgeons were in agreement in terms of reproducibility of model structures. Most structures could be reproduced, but the stapes, tympanic sinus, and mastoid air cells were unsatisfactory. Perioperative tactile sensation of the model was excellent. CONCLUSIONS: Although this model still does not embody perfect reproducibility, it proved sufficiently practical for use in surgical training.

Inactivation of tumor suppressor Dlg1 augments transformation of a T-cell line induced by human T-cell leukemia virus type 1 Tax protein.

BACKGROUND: The interaction of human T-cell leukemia virus type 1 (HTLV-1) Tax1 protein with the tumor suppressor Dlg1 is correlated with cellular transformation. RESULTS: Here, we show that Dlg1 knockdown by RNA interference increases the ability of Tax1 to transform a mouse T-cell line (CTLL-2), as measured interleukin (IL)-2-independent growth. A Tax1 mutant defective for the Dlg1 interaction showed reduced transformation of CTLL-2 compared to wild type Tax1, but the transformation was minimally affected by Dlg1 reduction. The few Tax1DeltaC-transduced CTLL-2 cells that became transformed expressed less Dlg1 than parental cells, suggesting that Dlg1-low cells were selectively transformed by Tax1DeltaC. Moreover, all human T-cell lines immortalized by HTLV-1, including the recombinant HTLV-1-containing Tax1DeltaC, expressed less Dlg1 than control T-cell lines. CONCLUSION: These results suggest that inactivation of Dlg1 augments Tax1-mediated transformation of CTLL-2, and PDZ protein(s) other than Dlg1 are critically involved in the transformation.