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A 70-year-old Japanese man undergoing remission induction therapy for acute monocytic leukemia (AML-M5b) developed fever and headache, and was started on antibiotics and liposomal amphotericin B (L-AMB). There was no improvement, and computed tomography and contrast-enhanced magnetic resonance imaging revealed acute rhinosinusitis and brain abscess. Successful endoscopic endonasal surgery was performed at this point, providing drainage for the rhinosinusitis and abscess. Histopathological findings showed the mucormycosis. [ABSTRACT FROM AUTHOR]

Purpose To retrospectively evaluate the outcomes of radiofrequency (RF) ablation of lung metastases from head and neck adenoid cystic carcinoma (ACC). Materials and Methods Nine patients (two men and seven women; mean age, 61.6 y) with 45 lung metastases (mean diameter, 1.1 cm; range, 0.4–2.7 cm) from head and neck ACC underwent RF ablation in 30 sessions. Primary endpoints were technical success, technique effectiveness, and procedural complications. Secondary endpoints included overall survival (OS). Results RF ablation was technically successful for all 45 metastases. The median tumor follow-up period was 37.1 months (range, 12.9–128.3 mo). Local progression occurred in six tumors, two of which were treated again and subsequently showed complete response. Major complications (pneumothorax requiring chest tube placement) occurred in five sessions (16.7%). The median patient follow-up period was 61.6 months (range, 20.5–134.5 mo). Two patients died of disease progression at 38.9 and 61.6 months after RF ablation, respectively, whereas the other seven remained alive at the end of the study. OS rates from the initial RF ablation were 100% at 3 years and 83.3% at 5 years (mean survival time, 106.4 mo). OS rates from the treatment of the primary site were 100% at 5 years and 62.5% at 10 years (mean survival time, 210.1 mo). Conclusions Radiofrequency ablation is an acceptable and effective local treatment for lung metastases from head and neck ACC. However, further study is needed to evaluate its effect on patient survival. [ABSTRACT FROM AUTHOR]

Objective: To investigate whether a novel laser-assisted stapedotomy technique is effective in treating congenital stapes fixation. Methods: With this surgical method, the suprastructure of the stapes is vaporized continuously from the superior half of the anterior crus to the inferior part of the posterior crus using a potassium-titanyl-phosphate (KTP) laser. The midmost point of the vaporization is the head of the stapes. This method was used to treat three patients with congenital stapes fixation (total of four ears). Results: The average hearing gain using this method was 32.2 dB, which lasted for at least 1 year. No complications were observed. Conclusions: This novel laser-assisted stapedotomy technique is an excellent method for the treatment of congenital stapes fixation. [Copyright &y& Elsevier]

Objective: To conduct a longitudinal national survey of cochlear implantation (CI) in Japan from 1985 through 2017.Methods: A retrospective analysis of adult and pediatric CI cases from 1985 to 2017 using the Oto-Rhino-Laryngological Society of Japan CI registration database was conducted. Surgical statistics were obtained, including number of surgeries, age, implanted side, facilities, and preoperative threshold levels for CI.Results: Since 1985, more than 11,100 CI surgeries have been conducted in Japan. The number exceeded 500 in 2005 and 1,000 in 2015. Since 2007, pediatric cases consistently surpassed adult cases until 2016. More facilities meet criteria for conducting CI as determined by Ministry of Health, Labor and Welfare and have now reached more than 100. Adult patients over 70 years old have become proportionally higher. Age at implantation steadily reduced in accordance with modifications of Japanese CI guidelines for children but is still not comparable to that in other developed countries. Although the number of CI surgeries for patients with moderate to severe hearing loss increased in the past ten years, the proportion was small.Conclusions: The CI database in Japan is quite unique in that it documents almost all the CI surgeries in Japan, allowing us to summarize the present situation as follows: fewer CI surgeries occur than are necessary, and children receiving implants are older than those in other developed countries. Continuous improvement of CI awareness is needed in Japan. [ABSTRACT FROM AUTHOR]

Background Recent studies have revealed that Staphylococcus aureus and its components participate in the pathogenesis of eosinophilic airway diseases, such as chronic rhinosinusitis with nasal polyps. Objective We sought to determine whether staphylococcal protein A (SpA) from S aureus regulated cellular responses in nasal polyps, especially when coupled to immunoglobulins in immune complexes (ICs). Methods Dispersed nasal polyp cells (DNPCs) or peripheral blood monocytes were cultured in vitro with SpA in the presence or absence of IgG, and IL-5, IL-13, IFN-γ, IL-17A, and IL-10 levels were measured in the supernatants. The effect of SpA exposure on staphylococcal enterotoxin B–induced cytokine production by DNPCs in the presence and absence of IgG, IgA, and autologous serum was also examined. Results Exposure to SpA induced DNPCs to produce significantly higher IL-10, IL-13, and IL-17A levels than DNPCs without SpA, although the magnitude of the IL-17A increase was less than that of IL-10 and IL-13. SpA induced IL-10 production mainly from adherent DNPCs, and this was significantly enhanced in the presence of IgG; similar results were observed in peripheral blood monocytes. IC formation between SpA and IgG (SpA-IgG ICs) was confirmed by using native polyacrylamide gel electrophoresis. SpA-IgG ICs, but not SpA alone, almost completely suppressed staphylococcal enterotoxin B–induced IL-5, IL-13, IFN-γ, and IL-17A production by DNPCs; similar inhibition was observed in DNPCs treated with SpA in the presence of either IgA or autologous serum. Conclusions Our results suggest that SpA can regulate the pathogenesis of enterotoxin-induced inflammation in patients with chronic rhinosinusitis with nasal polyps through coupling to immunoglobulins. [ABSTRACT FROM AUTHOR]

Background: Recent investigations have revealed that staphylococcal enterotoxins (SEs), COX metabolism, or both might participate in the pathogenesis of eosinophilic airway diseases, such as chronic rhinosinusitis with nasal polyposis. Objective: We sought to determine whether COX metabolism, especially prostaglandin (PG) E2, plays a significant role in SE-induced cellular responses in nasal polyps. Methods: Dispersed nasal polyp cells (DNPCs) were prepared from nasal polyps by means of enzymatic digestion. DNPCs were cultured with SEB in the presence or absence of COX inhibitors (diclofenac and indomethacin) for 72 hours; then the levels of IL-5, IL-13, RANTES, and eotaxin in the supernatants were measured. The effect of PGE2 on SEB-induced responses by diclofenac-treated DNPCs was examined, especially in terms of receptor specificity. Results: DNPCs produced significant amounts of IL-5, IL-13, and RANTES in response to SEB. COX inhibitors significantly increased the production of these cytokines. The degree of local eosinophilia was significantly and positively correlated with the changes in IL-5 production induced by diclofenac treatment. PGE2 significantly and dose-dependently inhibited SEB-induced IL-5, IL-13, and RANTES production by diclofenac-treated DNPCs. E-prostanoid (EP) 2 receptor–selective agonist strongly inhibited the production of all 3 cytokines. EP3 and EP4 receptor–selective agonists partially suppressed these responses, whereas EP1 receptor–selective agonist did not. Interestingly, all of the combined treatments with 2 of the 4 EP receptor–selective agonists significantly inhibited the SEB-induced responses by diclofenac-treated DNPCs. Conclusions: These results suggest that PGE2 inhibits the pathogenesis of SEB-induced eosinophilic inflammation primarily through the EP2-mediated pathway in patients with chronic rhinosinusitis with nasal polyposis. [Copyright &y& Elsevier]

Combination intranasal drugs with a corticosteroid and antihistamine are available in several countries with better effect than treatments with single agents. However, it remains unclear whether this effect is also seen in Japanese cedar pollinosis (JCP), the most prevalent seasonal allergic rhinitis in Japan. We investigated the effect of an add-on intranasal antihistamine with an intranasal corticosteroid in JCP during the pollen dispersal period. (UMIN000025508) We performed a double-blinded, randomized, placebo-controlled trial from March 1 to 14, 2017. Patients (n = 20 per group) received either a mometasone furoate nasal spray (MFNS) plus a levocabastine nasal spray (levocabastine group) or MFNS plus a placebo nasal spray (placebo group). The primary endpoint was the difference in the total nasal symptom score (TNSS) after treatment between the two groups. Differences in the total ocular symptom score, total symptom score, total medication score, total symptom-medication score, and five individual symptoms as well as safety were the secondary endpoints. The change in the TNSS from baseline was significantly greater in the levocabastine group than in the placebo group. A significant reduction in the TNSS was observed more than 6 days earlier in the levocabastine group than in the placebo group. Such add-on effects were also seen in the secondary endpoints. Both treatments were well-tolerated. The intranasal antihistamine provided better control of not only nasal symptoms, but also of ocular symptoms, and decreased the need for rescue medications when added to intranasal corticosteroid treatment in JCP patients. [ABSTRACT FROM AUTHOR]

To obtain new insights into research questions on how executive function and social interaction would be observed to change after the introduction of hearing aids (HAs) in older people with hearing impairment. Multi-institutional prospective single-arm observational study. Outpatients with complaints of hearing difficulty who visited HA clinics between October 18, 2017, and June 30, 2019, in 7 different university hospitals in Japan. The inclusion criteria of the study named Hearing-Aid Introduction for Hearing-Impaired Seniors to Realize a Productive Aging Society—A Study Focusing on Executive Function and Social Activities Study (HA-ProA study) were age ≥60 years and no history of HA use. A series of multi-institution common evaluations including audiometric measurements, the digit symbol substitution test to assess executive functions, convoy model as an index of social relations, and hearing handicap inventory for the elderly (HHIE) were performed before (pre-HA) and after 6 months of the HA introduction (post-HA). Out of 127 enrollments, 94 participants completed a 6-month follow-up, with a mean age of 76.9 years. The digit symbol substitution test score improved significantly from 44.7 at baseline to 46.1 at 6 months (P =.0106). In the convoy model, the social network size indicated by the number of persons in each and whole circles were not significantly different between pre- and post-HA; however, the total count for kin was significantly increased (P =.0344). In the analyses of HHIE, the items regarding the family and relatives showed significant improvement. HA use could benefit older individuals beginning to use HAs in executive function and social interaction, though the results should be interpreted cautiously given methodological limitations such as a single-arm short 6 months observation. Reduction in daily hearing impairment would have a favorable effect on relationships with the family. [ABSTRACT FROM AUTHOR]

Objectives: Sialolipoma has been classified as a benign soft tissue lesion in the 2017 World Health Organization classification of head and neck tumors. To our knowledge, only one case of laryngeal sialolipoma has been reported in the English literature. We conducted a retrospective study to identify clinical characteristics of supraglottic sialolipoma-like lesion and differentiate it from other supraglottic subepithelial masses.Methods: Medical records of 16 patients with supraglottic subepithelial benign mass lesions who underwent histological evaluation between 2003 and 2019 were retrospectively analyzed. Sialolipoma-like lesion was defined as a local finding of a well-circumscribed gross mass with pathological presence of salivary gland-like parenchymal lobules with evenly interspersed adipose tissue.Results: Eight patients showed histological positivity for sialolipoma-like lesion, 3 for amyloidosis, 2 for hemangioma, and 1 each for cyst, lymphoid hyperplasia, and chondrometaplasia. Sialolipoma-like lesion tended to be predominant among men; those affected had a mean age of 52.8 (range, 39-74) years. By contrast, among patients with amyloidosis, the ratio of men to women was 1:2 (100% vs. 33%; p = 0.055). Fiberscopic examination of all patients with sialolipoma-like lesions identified well-circumscribed, yellowish masses, closely resembling local amyloidosis findings. Sialolipoma-like lesion was associated with a significantly higher body-mass index (BMI; 27.4 ± 2.8 kg/m2) than amyloidosis (21.6 ± 1.4 kg/m2; p = 0.014). The transoral approach was used for lesion resection in all patients with sialolipoma-like lesion. No patient experienced postoperative recurrence.Conclusion: Laryngeal sialolipoma-like lesion might be more prevalent than was previously reported, and histological examination is important to differentiate it from amyloidosis. Supraglottic sialolipoma-like lesion must be differentially diagnosed in patients with high BMI presenting with well-circumscribed, yellowish supraglottic masses. [ABSTRACT FROM AUTHOR]

Objective: Infected mucocele of the paranasal sinuses can induce orbital infection, including orbital subperiosteal abscess, which may lead to life-threatening intracranial complications. Effective diagnosis is important, and treatment should be aggressive. This paper presents our experiences in endoscopic surgical management of orbital complications secondary to infected paranasal sinus mucoceles.Methods: From our retrospective review of the medical charts for 82 patients with 92 sides diagnosed with paranasal sinus mucoceles, we present 7 sides in 7 adult patients with orbital complications secondary to infected mucoceles. The collected data include the suggested etiology, side of sinus involvement, localization of abscess in the orbit, orbital wall bone defects caused by mucocele compression, ophthalmic symptoms, duration between symptom onset and initial visit, operation date, type of surgery performed, and follow-up.Results: The mucocele was located in the ethmoid-frontal region in 9.8% of the sides (9/92), in the frontal sinus in 7.6% (7/92), in the ethmoidal sinus in 9.8% (9/92), in the maxillary sinus in 67.4% (62/92), in the maxillary-ethmoidal sinus in 3.3% (3/92), and in the sphenoid sinus in 2.2% (2/92). The patients with ethmoid-frontal mucoceles had a significantly higher incidence of orbital complications (6/9) as compared with the other sub-types of mucoceles (frontal, 0/7; ethmoidal, 0/9; maxillary, 1/62; maxillary-ethmoidal, 0/3; sphenoid, 0/2). Chandler's classification showed Type I in one, Type II in three, and Type III in three. Sinus involvement was observed at the ethmoid-frontal sinuses in six cases and the maxillary sinus in one case. All seven cases had a partial defect of the orbital wall bone (lamina papyracea, or inferior orbital wall bone) by mucocele compression, and the patients underwent endoscopic marsupialization. For the cases with subperiosteal abscess, the lamina papyracea was also removed partially for draining the abscess. In all cases, symptoms were resolved without any recurrence of the mucocele.Conclusions: Infected ethmoid-frontal mucoceles with a defect of the lamina papyracea tend to induce orbital infection, so prompt surgery for the infected mucoceles should be considered early even with Types I and II, before visual acuity is impaired, because surgery is the only curative treatment for the mucoceles. [ABSTRACT FROM AUTHOR]

Objective: Since 2010, we have mainly performed surgical treatment following radiotherapy and concomitant intraarterial cisplatin (RADPLAT) for locally advanced maxillary sinus cancer (MSC). The present study investigated treatment results and pathological evaluations following RADPLAT for MSC.Methods: Pathological response to RADPLAT was evaluated using surgical specimens. Pathological response was graded in accordance with the classification method that Shimosato reported in 1964, as grade V (no tumor cells remain in any of section), grade IV, III, II, I, and 0. Five-year overall and disease-specific survival rates were estimated using Kaplan-Meier methods. Univariate analyses of correlations between recurrence of MSC and other clinicopathological parameters were evaluated using the chi-square or Fisher's exact tests.Result: 19 patients were enrolled in this study, 5 patients showed T3 disease and 14 had T4 disease. One patient demonstrated local recurrence and 3 patients experienced distant metastasis. The 5-year overall survival rate was 67.1% (T3, 50.0%; T4, 69.6%), and the 5-year disease-specific survival rate was 81.9% (T3, 100%; T4, 76.0%). Histological response was categorized as grade V in 9 cases. No significant risk factors for residual cancer were identified.Conclusion: Our study suggested that RADPLAT not only has a low risk of side effects, but also could represent an effective procedure for locally advanced MSC by pathological evaluation. Increasing the therapeutic intensity of RADPLAT might provide an effective modality to avoid highly invasive surgery. [ABSTRACT FROM AUTHOR]

As has been well recognized, methotrexate (MTX) leads to a state of immunosuppression and can provide a basis for the development of lymphoproliferative disorders (LPDs). MTX-associated LPDs can affect nodal sites as well as extranodal sites, though the manifestation of an LPD in the form of multiple pulmonary nodules is rare. Here, we report two cases of MTX-associated LPD with multiple bilateral pulmonary nodules, which was a finding suggestive of lung cancer, and bilateral cervical lymphadenopathy. After withdrawal of MTX, the multiple bilateral pulmonary nodules and bilateral cervical lymphadenopathy disappeared without chemotherapy in both cases. From these results, patients with pulmonary nodules and cervical lymphadenopathy should be examined for head and neck malignant tumors. Also, physicians should carefully check the administration of MTX. In patients with an MTX-associated LPD, we need to make an early diagnosis and consider discontinuing the administration of MTX as soon as possible. [ABSTRACT FROM AUTHOR]

Objective: The treatment of all forms of sinonasal inverted papilloma (IP) is a complete, wide, local resection. The main surgical purpose is to remove all diseased mucosa and mucoperiosteum, together with a cuff of normal-looking mucosa at the attachment site, followed by drilling and/or coagulation. Our aim is to present our experiences in endoscopic surgical management of IP by using attachment-oriented excision.Methods: We present 20 cases of sinonasal IP. The data collected includes the histopathological diagnosis, staging, extension of the tumor, tumor attachment site, approach to surgery, serum squamous cell carcinoma antigen (SCCA) level, and recurrences.Results: All patients underwent endoscopic surgery. A Caldwell-Luc operation was required in addition to the endoscopic surgery in one case. There was one case of recurrence (5%). After the additional operation, there was no recurrence. The tumor attachment sites vary, and the case of recurrence had a wide attachment site at the primary surgery. No major intra- or post-operative complications were observed.Conclusion: The present study shows that attachment-oriented excision for IP is useful for complete resection of IP. Surgeons should choose the surgical approach according to the location of the tumor attachment site rather than the Krouse staging system. [ABSTRACT FROM AUTHOR]

Objective: There is a close relationship between upper and lower respiratory tract diseases. Chronic rhinosinusitis patients frequently have lung diseases including asthma and chronic obstructive pulmonary disease. Eosinophilic chronic rhinosinusitis is considered a refractory and intractable subtype of chronic rhinosinusitis. However, there has been no report on pulmonary function in patients with eosinophilic chronic rhinosinusitis. The purpose of this study is to examine the pulmonary function in eosinophilic chronic rhinosinusitis patients and non-eosinophilic chronic rhinosinusitis patients, and evaluate clinical factors associated with the pulmonary function of these patients.Methods: Pulmonary function was measured in 53 patients with eosinophilic chronic rhinosinusitis with asthma, 58 patients with eosinophilic chronic rhinosinusitis without asthma, and 30 patients with non-eosinophilic chronic rhinosinusitis. The diagnosis of chronic rhinosinusitis was based on the definition in the European Position Paper on Rhinosinusitis and Nasal Polyps (EPOS) 2012. Eosinophilic chronic rhinosinusitis was diagnosed based on the Japanese Epidemiological Survey of Refractory Eosinophilic Chronic Rhinosinusitis (JESREC) scoring system. The relationship between pulmonary function and clinical parameters was assessed. These parameters included radiographic severity of chronic rhinosinusitis, peripheral blood eosinophil percentage, serum total immunoglobulin E level, and eosinophilic infiltration in nasal polyps.Results: The pulmonary function of the patients with eosinophilic chronic rhinosinusitis was significantly affected. The eosinophilic chronic rhinosinusitis patients had more peripheral airway obstruction as compared to the patients with non-eosinophilic chronic rhinosinusitis.Conclusion: Our findings indicated latent obstructive lung function changes in the eosinophilic chronic rhinosinusitis patients. The patients with eosinophilic chronic rhinosinusitis should be carefully monitored in order to detect lung diseases. [ABSTRACT FROM AUTHOR]

Objectives: Unlike glottic cancer, supraglottic cancer often presents with neck metastases. This different might be attributable to the location of the primary lesion. This study aimed to clarify the relationships between the sublocation of T1-2 supraglottic cancer, human papillomavirus (HPV) infection, neck metastasis, and prognosis of supraglottic cancer.Methods: This retrospective clinical study investigated 55 Japanese patients with T1-2 supraglottic cancer treated between 1994 and 2015.Results: Of 55 patients with T1-2 supraglottic cancer, neck metastasis was present at initial diagnosis in 14 patients (25.5%). Presence of neck metastasis was the only factor associated with worse prognosis of T1-2 supraglottic cancer (p=0.004). In multivariate analysis, age <70years (p=0.033) and sublocation of the primary lesion in the superior epilaryngeal portion (p=0.017) were significantly associated with presence of neck metastasis in multivariate analysis. Twelve (27.9%) of 43 patients showed positive results for human papillomavirus infection. However, human papillomavirus infection was not associated with prognosis, presence of neck metastasis, or primary lesion sublocation in T1-2 supraglottic cancer.Conclusion: Relatively young patients with supraglottic cancer at the superior epilaryngeal portion are more likely to show neck metastasis. Human papillomavirus infection was not associated with frequency of neck metastasis. [ABSTRACT FROM AUTHOR]

Transcanal endoscopic ear surgery (TEES) will become a very useful therapeutic option. A perilymphatic fistula (PLF) is defined as sudden sensorineural hearing loss and/or vertigo caused by leakage of the perilymph through a fistula from the oval window and/or round window. We report a case of PLF after electric acoustic stimulation (EAS), a kind of cochlear implant, successfully treated by TEES. A 38-year-old man presented to our hospital with vertigo and hearing loss (HL). His vertigo was induced by Valsalva maneuvers. Eight months ago, he underwent EAS for his right ear for congenital sensorineural HL. Although he maintained his hearing level after EAS, his pure tone audiogram this time showed deterioration of hearing at low frequencies in his right ear. A diagnosis of right PLF was made. After confirming the non-effectiveness of oral prednisolone treatment, PLF repair surgery to patch the oval and round windows by TEES was performed. His vertigo did not recur after the surgery. To the best of our knowledge, this is the first report of PLF repair surgery by TEES without a microscope. The wide-field view of the middle ear by TEES was useful to prevent electrode damage in a PLF patient with a cochlear implant. [ABSTRACT FROM AUTHOR]

Objective: Since most patients with peritonsillar abscess (PTA) can be successfully treated with surgical drainage and empirical antibiotic therapy, routine bacteriologic studies for all patients with PTA may be unnecessary. This study tried to evaluate which patients with PTA should certainly undergo bacteriologic studies.Methods: Hundred consecutive patients with PTA were treated and underwent culture tests of purulent contents obtained by surgical drainage between April 2008 and December 2013.Results: In 62 of the 100 patients, 71 pathogenic bacteria were identified; 61 (86%) were Gram-positive cocci (GPC), 8 (11%) were Gram-negative rods (GNR), and 6 (8%) were anaerobes. Normal flora were isolated in 27 patients, and culture results were negative in 11 patients. Although not significant, primary (without prior antibiotic therapy) case (odds ratio (OR)=2.19; 95% CI, 0.95-5.05) and laryngeal edema (OR=2.04; 95% CI, 0.82-5.03) showed a tendency of associations with detection of pathogenic bacteria. After taking into account interactions between smoking habit and laryngeal edema, the covariate-adjusted OR for non-smokers with laryngeal edema was significant and showed a strong relationship (OR=7.43; 95% confidence interval, 1.05-52.73) compared to non-smokers without laryngeal edema.Conclusion: Although empirical antibiotic therapy was effective for most of the PTA patients, bacteriologic studies might be indispensable for the patients with laryngeal edema considering the failure of the first treatments. Particularly, the culture tests may be useful for non-smokers with laryngeal edema. [ABSTRACT FROM AUTHOR]

Central auditory processing disorder (CAPD) is a condition in which dysfunction in the central auditory system causes difficulty in listening to conversations, particularly under noisy conditions, despite normal peripheral auditory function. Central auditory testing is generally performed in patients with normal hearing on the pure tone audiogram (PTA). This report shows that diagnosis of CAPD is possible even in the presence of an elevated threshold on the PTA, provided that the normal function of the peripheral auditory pathway was verified by distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), and auditory steady state response (ASSR). Three pediatric cases (9- and 10-year-old girls and an 8-year-old boy) of CAPD with elevated thresholds on PTAs are presented. The chief complaint was difficulty in listening to conversations. PTA showed elevated thresholds, but the responses and thresholds for DPOAE, ABR, and ASSR were normal, showing that peripheral auditory function was normal. Significant findings of central auditory testing such as dichotic speech tests, time compression of speech signals, and binaural interaction tests confirmed the diagnosis of CAPD. These threshold shifts in PTA may provide a new concept of a clinical symptom due to central auditory dysfunction in CAPD. [ABSTRACT FROM AUTHOR]

Charcot-Marie-Tooth disease (CMT) is the most common form of hereditary sensorimotor neuropathy and sometimes involves disorders of the peripheral auditory system. We present a case of steroid-dependent auditory neuropathy associated with CMT, in which the patient experienced 3 episodes of acute exacerbation of hearing loss and successful rescue of hearing by prednisolone. An 8-year-old boy was referred to the otolaryngology department at the University Hospital. He had been diagnosed with CMT type 1 (demyelinating type) at the Child Neurology Department and was suffering from mild hearing loss due to auditory neuropathy. An audiological diagnosis of auditory neuropathy was confirmed by auditory brainstem response and distortion-product otoacoustic emissions. At 9 years and 0 months old, 9 years and 2 months old, and 10 years and 0 months old, he had experienced acute exacerbations of hearing loss, each of which was successfully rescued by intravenous or oral prednisolone within 2 weeks. Steroid-responsive cases of CMT have been reported, but this is the first case report of steroid-responsive sensorineural hearing loss in CMT. The present case may have implications for the mechanisms of action of glucocorticoids in the treatment of sensorineural hearing loss. [ABSTRACT FROM AUTHOR]

Objective Permanent hearing impairment has a life-long impact on children and its early identification is important for language development. A newborn hearing screening (NHS) program has started in Okayama Prefecture, Japan, in 1999 to detect hearing impairment immediately after birth. We aim to examine the effect of this screening program on vocabulary development in pre-school children in a before and after comparative study design. Methods A total of 107 5-year-old children who graduated from Okayama Kanariya Gakuen (an auditory center for hearing-impaired children) between 1998 and 2011 were enrolled in this study. The pre-NHS group ( n = 40) was defined as those who graduated between 1998 and 2003, while the post-NHS group ( n = 67) was defined as those who graduated between 2004 and 2011. The primary outcome was receptive vocabulary, which was assessed by the Picture Vocabulary Test [score <18 (low) vs. score ≥18 (high)]. The secondary outcome was productive vocabulary, or the number of productive words, which was assessed by an original checklist [<1773 words (low) vs. ≥1773 (high)]. We calculated odds ratios and 95% confidence intervals for vocabulary development and compared both groups. Results The adjusted Picture Vocabulary Test score and number of productive words were significantly higher ( p < 0.01) in the post-NHS group than the pre-NHS group. Odds ratios were 2.63 (95% confidence interval: 1.17–5.89) for receptive vocabulary and 4.17 (95% confidence interval: 1.69–10.29) for productive vocabulary. Conclusions The introduction of NHS in Okayama Prefecture significantly improved both receptive and productive vocabulary development in hearing-impaired children. [ABSTRACT FROM AUTHOR]

Peritonsillar abscess is a frequently encountered otorhinolaryngological emergency, but the characteristics of patients with this disease have not been described in detail. The objective of this study was to delineate prognostic factors associated with peritonsillar abscess and the effects of early surgical drainage for the treatment of peritonsillar abscess. We conducted a retrospective analysis of the medical records of 240 consecutive patients with PTA during the period from 2007 to 2013. Univariate analysis indicated that the period between symptom onset and relief was significantly longer in patients with high levels of C-reactive protein (CRP) (>8.53 mg/dL, p = 0.0073) and without early surgical drainage of pus ( p < 0.0001). Multivariate analysis identified both of these values as independently associated with longer duration of symptoms (high CRP, P < 0.0001; no early drainage, P < 0.0001). Univariate analysis indicated that the duration between symptom onset and complete recovery from the disease was significantly longer with age ≥40 years ( P = 0.0004), no history of recurrent tonsillitis ( P = 0.022), high CRP level ( P = 0.0017), and no early surgical drainage of the abscess ( P = 0.0014). Multivariate analysis identified older age ( P = 0.0004), high CRP level ( P = 0.0001), and no early drainage ( P < 0.0001) as independently associated with longer duration between symptom onset and complete recovery. Early surgical drainage of the abscess is important for the treatment of peritonsillar abscess. Patients ≥40 years old with peritonsillar abscess and high CRP levels should be recognized as a high-risk group. [ABSTRACT FROM AUTHOR]

Abstract: Objective: The objective of this study was to delineate the frequency of delayed diagnosis in cases of subacute thyroiditis (SAT) and intervals between onset of clinical symptoms and appearance of abnormal laboratory findings. Methods: We reviewed the medical records of 27 patients (7 men and 20 women) with SAT who visited our hospital between 2007 and 2013. Results: On presentation to the hospital, 5 of 27 SAT cases (18.5%) showed normal laboratory findings. Among these 5 cases, the mean interval between symptom onset and thyrotropin (TSH) suppression was 6.3 weeks, and the mean interval to elevation of fT4 was 6.7 weeks. The longest interval from symptom onset to appearance of an abnormal laboratory finding was 11 weeks. Conclusion: Sometimes time-lag exists between onset of clinical symptoms and the appearance of abnormal laboratory findings in patients with SAT. The possibility of this disease should not be excluded from the differential diagnoses for patients with clinical symptoms consistent with SAT but showing normal laboratory findings. [Copyright &y& Elsevier]

Abstract: A case of thyroidal mucosa-associated lymphoid tissue (MALT) lymphoma mimicking IgG4-related disease is described. A 54-year-old male presented with acute swelling of the anterior neck. Anaplastic thyroid carcinoma (ATC), malignant lymphoma (ML), or acute deterioration of Hashimoto's thyroiditis were initially suspected, and an emergent tracheostomy was required for progressive airway obstruction; a simultaneous biopsy from the thyroid tissue was performed. Histopathologically, the lesion consisted of sclerotic fibrosis and diffuse and dense infiltration by small lymphoid cells without atypia and plasma cells, many of which were IgG4-positive. Blood examination also revealed high serum IgG4 levels. Riedel's thyroiditis was suspected. However, despite medical treatments, a firm swelling of the thyroid still remained. In an in situ hybridization study, IgG4-negative plasma cells showed immunoglobulin light-chain restriction (κ-monotype), and immunoglobulin heavy (IgH) chain gene monoclonal re-arrangement was detected by polymerase chain reaction. The lesion was finally diagnosed as MALT lymphoma. When IgG4-related disease is suspected, it is important to thoroughly exclude other possibilities. [Copyright &y& Elsevier]

Abstract: Learning disability combined with hearing impairment (LDHI) is a poor prognostic factor for the language development of hearing impaired children after educational intervention. A typical example of a child with LDHI and effective interventions provided by cochlear implants are presented in this report. A case of congenital cytomegaloviral infection that showed dysgraphia as well as profound deafness was reported and an underlying visual processing problem diagnosed in the present case caused the patient''s dysgraphia. The dysgraphia could be circumvented by the use of auditory memory fairly established by a cochlear implant. [Copyright &y& Elsevier]

Summary: Introduction: This study examines the ability and development in the comprehension of abstract words with hearing impaired children. The ability to understand abstract words is quite important for their academic learning and adaptation in their school life. Here, we qualitatively and quantitatively analyzed the development of abstract vocabulary in hearing impaired children using The Standardized Comprehension Test for Abstract Words (SCTAW). Subjects and methods: We examined 75 hearing impaired children (hearing aid users, 61; cochlear implant users, 14; 1st to 10th grade) and 188 children with normal hearing (1st to 6th grade) using the Picture Vocabulary Test (PVT) and SCTAW. Results: The PVT and SCTAW results closely correlated (r =0.87). The SCTAW scores of the hearing impaired group were lower than those of their peers with normal hearing, but the scores improved as their school grade advanced. In particular, their abstract ability began to catch up from the fifth grade. The error trends of abstract vocabulary in the two groups did not significantly differ. Conclusions: The SCTAW was useful as an abstract lexical evaluation of hearing impaired children. The development of an abstract vocabulary did not qualitatively differ between children with or without a hearing impairment. [Copyright &y& Elsevier]

Abstract: Development of a gene-delivery method to the inner ear is an essential step for studies of hearing function and gene therapy. Application of liposomes or adenoviral vectors onto the intact round window membrane (RWM) offers the possibility of atraumatic exogenous gene transfer. GJB2 encodes the gap junction protein Connexin26, which plays a crucial role in potassium recycling in the inner ear. The R75W allele of GJB is a well-characterized mutation that causes deafness at the DFNA3 through a dominant-negative mechanism of action. In this study, a plasmid vector, pGJB2 R75W-eGFP, was lipocomplexed with N-[1-(2,3-Dioleoloxy)propyl]N,N,N-trimethylammonium methylsulfate: cholesterol and applied onto mouse RWM. At 3 days (3d) post-treatment, immunohistochemistry demonstrated GJB2R75W-eGFP transgene expression in the cochlea in: inner and outer pillar cells, outer hair cells, Claudius cells and, in the spiral limbus and ligament. Significant hearing loss was detected by auditory brainstem response testing after 1, 2 and 3d post-treatment; hearing levels returned to control levels at 5d post-treatment. These data confirm that GJB2 R75W induces functional impairment in the mature cochlea through a dominant negative effect, and importantly, that RWM application of exogenous genes is a feasible method to test their impact on hearing. [Copyright &y& Elsevier]

Summary: Introduction: The purpose of this investigation was to describe the correlation between vocal and hearing development by longitudinal analysis of sound spectrograms, as a basic system for evaluating progress in vocal development. Subjects and methods: Two school-aged children with prelingual deafness were evaluated diachronically to assess speech perception and speech intelligibility after cochlear implantation. One child had non-syndromic hearing impairment without any known neurological deficit except for hearing loss, while the other had hearing impairment accompanied by mild mental retardation and attention deficit disorder. Their voices were recorded for monthly follow-up after cochlear implantation; these were used for formant analysis and compared with their mother''s voice, and alteration of the formant data was also compared with monosyllable speech perception. Results: Formant analysis demonstrated high concordance was observed between monosyllable speech perception and speech intelligibility. F1–F2 forms of the patients more closely resembled those of their mothers after 1 year''s follow-up. The time point at which speech development altered was very similar in both cases although the final outcomes were different. Conclusion: Fair improvement of articulation after cochlear implant was demonstrated by the F1–F2 gram analysis. This procedure can be used for data sharing and cooperation between medical and educational specialists. [Copyright &y& Elsevier]

Summary: Objective: While investigators have reported that patients with GJB2-associated deafness and cochlear implants have preferable language development, the mechanisms of this phenomenon remains unknown. The goal of the present study was to assess higher brain functions of patients with GJB2-related and GJB2-unrelated deafness as a method of evaluating language development. Methods: Eight children with cochlear implants were subjected to genetic testing for GJB2 and underwent the Raven colored progressive matrices test, Rey''s auditory verbal learning test, Rey''s complex figure test, the standardized language test for aphasia, the picture vocabulary test, and the standardized comprehension test for abstract words. Results: Three children were diagnosed with GJB2-related deafness, and five children were diagnosed with GJB2-unrelated deafness. All three GJB2-related cases demonstrated normal range higher brain functions and fair language development. By contrast, one GJB2-unrelated case showed a semantic disorder, another demonstrated a visual cognitive disorder with dyslexia, and another had attention deficit-hyperactivity disorder. Conclusions: Children with GJB2-unrelated deafness showed a high frequency of heterogeneous disorders that can affect proper language development. This difference between children with GJB2-related and GJB2-unrelated deafness may account for the improved language development in children with GJB2-related deafness and cochlear implants. Further, genetic diagnosis of the non-syndromic hearing loss represents a useful tool for the preoperative prediction of outcomes following a cochlear implant procedure. [Copyright &y& Elsevier]

Abstract: Objective: Adenocarcinoma ex pleomorphic adenoma is a rare tumor, and thus the management of the tumor has not been established. Methods: The retrospective review of the clinical records at Okayama University was performed. Results: We present five cases of adenocarcinoma ex pleomorphic adenoma of the head and neck, including a rare case with nasopharyngeal adenocarcinoma ex pleomorphic adenoma. There was no response to chemotherapy with nedaplatin and 5-FU, but the nasopharyngeal adenocarcinoma ex pleomorphic adenoma showed a remarkable regression after the administration of docetaxel. Conclusion: The combination therapy that includes docetaxel may be a promising treatment for adenocarcinoma ex pleomorphic adenoma of the head and neck. [Copyright &y& Elsevier]