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Recurrent otitis media and persistent otitis media with effusion in early childhood may cause an atelectatic eardrum and adhesive otitis media, which sometimes progress to pars tensa cholesteatoma. When and how children with adhesive otitis media should be operated on remain controversial. Therefore, this study aimed to analyze the clinical characteristics of children with adhesive otitis media and pars tensa cholesteatoma, and to determine the risk factors of progression to cholesteatoma.Seventeen ears of 15 children with adhesive otitis media (adhesive group) and 14 ears of 13 children with pars tensa cholesteatoma (tensa cholesteatoma group) who underwent tympanoplasty were included in this study. We analyzed the following clinical characteristics of children in both groups: medical and life history, associated diseases, sites of the adhesion, and development and aeration of mastoid air cells as shown by temporal bone computed tomography.Most of the children in both groups had a history of recurrent otitis media and/or persistent otitis media with effusion. They showed a male predominance and a frequent association of allergic rhinitis. The number of ears showing undeveloped mastoid air cells in the tensa cholesteatoma group was significantly larger than that in the adhesive otitis media group (P=0.0068). A lack of aeration of the middle ear, including the eustachian tube, was more frequently found in ears with pars tensa cholesteatoma than in ears with adhesive otitis media (P=0.0012). Using multivariate logistic regression, the presence of otorrhea (odds ratio [OR], 14.847; 95% confidence interval [CI], 0.834-264.184), total adhesion (OR, 28.550; 95% CI, 0.962-847.508), and undeveloped mastoid air cells (OR, 19.357; 95% CI, 1.022-366.589) were related to pars tensa cholesteatoma.Children with adhesive otitis media should be carefully followed up in the outpatient setting. Ears with poor mastoid development may develop pars tensa cholesteatoma. Additionally, ears with middle ear effusion, total adhesion, and the presence of otorrhea tend to be at risk of pars tensa cholesteatoma. Tympanoplasty or tympanostomy tube insertion should be considered for children with adhesive otitis media who have these risk factors to prevent progression to pars tensa cholesteatoma.

Eosinophilic otitis media (EOM) is an intractable otitis media with highly viscous middle ear effusion and is usually associated with bronchial asthma. Since the diagnostic criteria of EOM were established in 2011, the concept of EOM has been known worldwide. EOM is caused by Type 2 inflammation in the respiratory tract, similar to bronchial asthma and eosinophilic rhinosinusitis. With the appreciation of Type 2 inflammatory diseases, EOM is no longer considered to be a rare disease and should be specifically treated to improve quality of life. The diagnosis of EOM needs to be reconsidered because many reports have described varying pathogenesis and mechanisms of rare middle ear conditions. Systemic and topical administration of corticosteroids is presently the most effective treatment to control EOM. However, EOM treatments are developing because various biologics have been used to treat patients with bronchial asthma with and without eosinophilic rhinosinusitis and EOM. Surgical intervention is also no longer contraindicated with the use of biologics. These advances represent the beginning of a new stage of basic and clinical research for EOM. This review focuses on the diagnosis and treatment of EOM based on the most recent advances regarding EOM.

This is an update of the 2015 Guidelines developed by the Japan Otological Society and Oto-Rhino-Laryngeal Society of Japan defining otitis media with effusion (OME) in children (younger than 12 years old) and describing the disease rate, diagnosis, and method of examination. Recommended therapies that received consensus from the guideline committee were updated in consideration of current therapies used in Japan and based on available evidence.Regarding the treatment of OME in children, we developed Clinical Questions (CQs) and retrieved documents on each theme, including the definition, disease state, method of diagnosis, and medical treatment. In the previous guidelines, no retrieval expression was used to designate a period of time for literature retrieval. Conversely, a literature search of publications from March 2014 to May 2019 has been added to the JOS 2015 Guidelines. For publication of the CQs, we developed and assigned strengths to recommendations based on the collected evidence.OME in children was classified into one group lacking the risk of developing chronic or intractable disease and another group at higher risk (e.g., children with Down syndrome, cleft palate), and recommendations for clinical management, including follow-up, is provided. Information regarding management of children with unilateral OME and intractable cases complicated by adhesive otitis media is also provided.In clinical management of OME in children, the Japanese Clinical Practice Guidelines recommends management not only of complications of OME itself, such as effusion in the middle ear and pathologic changes in the tympanic membrane, but also pathologic changes in surrounding organs associated with infectious or inflammatory diseases.

Objective To determine whether myringoplasty can be an effective tool to control eosinophilic otitis media and improve hearing. Methods Severity scores for patients with eosinophilic otitis media with or without myringoplasty were retrospectively analyzed. Surgical outcomes were assessed and compared to those of patients with control: chronic otitis media with tympanic membrane perforation. Results The average eosinophilic otitis media severity score for the patients with myringoplasty was significantly lower after than before surgery (P = 0.004), although the score for the group without myringoplasty remained stable during the study period. The success rate for graft uptake was 87.5%, and a statistically significant improvement was observed in the postoperative air conduction hearing level within the speech range (mean ± standard deviation; 41.5 ± 12.3 to 34.6 ± 9.22 dB; P = 0.0026) for patients with eosinophilic otitis media. Conclusion Myringoplasty contributed to decreased postoperative eosinophilic otitis media severity scores and should be considered to protect the patients from recurrent bacterial infections and pathogenic antigen parasitism in the middle ear.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of ANCA-related vasculitis. Asthma and sinusitis occur first in the course of EGPA, followed by vasculitis symptoms such as fever, weight loss, and peripheral neuropathy. Otitis media with effusion and sensorineural hearing loss occur occasionally in EGPA patients. Here we report a case of a 39-years-old female patient with asthma that developed at age 37 and sinusitis. The patient was diagnosed with EGPA and treatment was started with oral corticosteroids. During the course of treatment, otitis media with effusion and sensorineural hearing loss developed. Benralizumab was administered for severe asthma. After treatment with benralizumab, the symptoms of asthma, otitis media with effusion and sinusitis dramatically improved. This is the first reported case in which benralizumab was used for treating otitis media and sinusitis associated with EGPA. The findings suggest that benralizumab may be effective for otitis media and sinusitis associated with EGPA.

Atelectatic eardrum and adhesive otitis media in children are related to persistent otitis media with effusion (OME) and eustachian tube dysfunction in infancy. However, the pathogenesis of these diseases is not fully understood, and some cases even progress to pars tensa cholesteatoma. This study analyzed the clinical characteristics of children with atelectatic eardrum and adhesive otitis media to clarify associated causes of and risk factors for progression of these middle ear pathologies in children.This retroactive study included 38 ears of 31 children with atelectatic eardrums (atelectasis group), and 19 ears of 17 children with adhesive otitis media (adhesive OM group). Thirty-two contralateral non-cholesteatoma ears of 32 children with congenital cholesteatoma were also examined as a control group. Participants were aged 15 or younger. Life history (obtained via questionnaire), associated diseases, hearing acuity, aeration and development of the temporal bone were investigated.All children in the atelectasis and adhesive OM groups had a history of recurrent acute otitis media (rAOM) and/or OME. The prevalence of perennial allergic rhinitis was 18 cases (58%) in the atelectasis group and 16 cases (94%) in the adhesive OM group. The prevalence of perennial allergic rhinitis was significantly higher in the adhesive OM group compared to the control group (P 0.01). The development of mastoid air cells, categorized by MC classification, showed MC0 + MC1 (poor pneumatization) in 19 ears (50.0%) with atelectatic eardrums and 12 ears (63.2%) with adhesive OM. Poor pneumatized mastoid was more frequently observed in the ears of the atelectasis and adhesive OM groups compared to the control group (P 0.01). No significant difference was found in regards to aeration of the middle ear between the two groups; however, aeration was significantly poorer in both groups compared to the control group (P 0.01). Three characteristics were analyzed using multivariate logistic regression: perennial allergic rhinitis (odds ratio [OR] 4.319, P = 0.013), poor mastoid pneumatization (OR 8.457, P = 0.012), and pars flaccida retraction pocket (OR 20.897, P = 0.006). These characteristics were shown to be significant risk factors for atelectatic eardrums and adhesive OM. In addition, the predisposition to perennial allergic rhinitis was shown to be the most important factor in the progression from atelectatic eardrum to adhesive otitis media (OR 16.615, P = 0.012).Children with perennial allergic rhinitis, poor development of mastoid air cells, poor aeration of the temporal bone, and with pars flaccida retraction pocket were at an increased risk of developing an atelectatic eardrum and adhesive OM. In particular, perennial allergic rhinitis was shown to be a significant risk factor in the progression from atelectatic eardrum to adhesive otitis media. Allergic inflammation may affect not only the nasal passages but also the eustachian tube, resulting in persistent middle ear dysfunction. Therefore, children with rAOM/OME who have these risk factors should be carefully monitored and treat over time in effort to prevent progression of pathology.

To examine the evidence for treating children with otitis media with effusion with pharmacotherapy.For the systematic review, data were retrieved from PubMed, Cochrane database, and the Japan Medical Abstracts Society Database (1st January 1995 through 31th May 2019).Articles addressing pharmacotherapy for the management of otitis media with effusion in children were selected in English.The database was searched using the keywords "Otitis Media with effusion or secretory otitis media" and the following medical agents: carbocysteine, antihistamines, leukotriene receptor antagonist, and steroid nasal spray.After a critical review of 18 studies, studies addressing steroid nasal spray were eligible for quantitative synthesis. Intranasal steroids for OME showed no benefit with OR 1.155 (95% CI 0.834-1.598) within one month. Conversely, intranasal steroids have effects for OME with OR 1.858 (95% CI 1.240-2.786) for more than one month.We found evidence of benefit from treatment of OME in children with intranasal steroids and S-carboxymethylcysteine at longer-term follow-up.

Eosinophilic otitis media (EOM) is an intractable otitis media that is mostly associated with bronchial asthma. Middle ear effusion is highly viscous and contains many eosinophils. Surgical interventions, such as tympanoplasty and mastoidectomy, are usually contraindicated for patients with EOM because of the high incidence of deafness and relapse of EOM after the operation. We report here a patient who was successfully treated with tympanoplasty with the use of dupilumab therapy. The patient was a 50-year-old woman with the granulation type of EOM who had a poor response to treatments for EOM, including systemic and topical use of corticosteroids and other biological drugs. After switching to add-on dupilumab therapy, the granulation tissue from the mesotympanum gradually decreased and no middle ear effusion was found. The severity score of EOM and temporal bone computed tomographic findings were dramatically improved after dupilumab therapy. Tympanoplasty was then performed 13 months after the initiation of dupilumab therapy. The postoperative course was excellent without deterioration of the bone conduction hearing level or relapse of EOM for 1 year. Tympanoplasty/mastoidectomy for EOM is currently indicated if patients show a good response to biological drugs for a long time.

Objective Eosinophilic otitis media (EOM) is an intractable otitis media characterized by a highly viscous effusion containing eosinophils, and it is mostly associated with bronchial asthma. Recently, anti-IL-5 therapy using mepolizumab has been reported to be effective for patients with severe and refractory eosinophilic bronchial asthma. EOM shows accumulation of eiosinophils in the middle ear effusion and most EOM patients have high numbers of peripheral blood eosinophils. Therefore, we carried out a retrospective study to determine whether anti-IL-5 therapy is also effective in the treatment of EOM. Patients and methods Nine patients with EOM associated with bronchial asthma received the anti-IL-5 agent mepoliumab as an add-on therapy for at least 6 months (mepolizumab group). They were evaluated by EOM severity scores, symptom scores, bone conduction hearing levels, and surrogate markers before and after receiving the anti-IL-5 therapy. Thirteen EOM patients associated with bronchial asthma who did not receive the anti-IL-5 therapy were also included as controls (control group). Results The severity scores of most patients in the mepolizumab group were dramatically reduced at 3 months after the initiation of this therapy and, as therapy continued, they further decreased to levels significantly lower than the baseline. However, two patients with a granulation type of EOM showed minimal improvement from the therapy. The severity scores of control patients showed no significant changes during the study period. Significant deterioration of the bone conduction hearing levels was not observed in either group. The number of peripheral blood eosinophils was significantly reduced, and eosinophils were scarcely observed in the middle ear effusion and middle ear mucosa after the mepolizumab therapy. Conclusions Anti-IL-5 therapy using mepolizumab was effective at inhibiting eosinophilic recruitment to the middle ear in patients with EOM. However, this therapy showed minimal effect on patients with the granulation type of EOM. Therefore, this therapy may be a viable treatment option for refractory EOM without severe mucosal change.

Objectives Eosinophilic otitis media (EOM) is an intractable otitis media mostly associated with bronchial asthma. Dupilumab, an anti-interleukin (IL)-4 receptor (R)α, is effective and has been approved for use in patients with moderate to severe bronchial asthma, atopic dermatitis and chronic rhinosinusitis with nasal polyposis, whose diseases are not controlled by previous treatments including other molecular targeted drugs. We aimed to assess efficacy of dupilumab in three EOM patients with associated bronchial asthma, who were poor responders to previous topical and systemic corticosteroid therapy and molecular targeted therapies. Patients and methods Three patients with severe, refractory EOM (two with a granulation type) associated with bronchial asthma received dupilumab as add-on therapy for at least 6 months. The efficacy of dupilumab therapy was evaluated using severity scores, symptom scores, hearing acuities, temporal bone computed tomography (CT) scores, and surrogate markers before and after therapy. Results Severity scores in all patients were dramatically reduced to 2 points or less (full score: 16 points) after initiation of therapy. Air conduction hearing levels were improved in all patients. Temporal bone CT scores in two patients were reduced, and serum IgE levels in all three patients also decreased following therapy. Conclusion We provide the first report that add-on dupilumab therapy was effective in patients with severe, refractory EOM who did not respond to the treatments including other molecular targeted therapy. Patients with severe middle ear mucosal change may benefit particularly from dupilumab therapy.

Eosinophilic otitis media (EOM) is an intractable type of otitis media in which sensorineural hearing loss (SNHL) progresses over time. Clinically, bacterial infection complicates the course of EOM, making it challenging to control otorrhea/middle ear effusion (MEE) from infected ears, and accelerates the progression of SNHL. In this study, we focused on infection, one of the risk factors for SNHL in EOM, and analyzed factors associated with it.In this cohort study, we evaluated 144 ears of 72 patients diagnosed with bilateral EOM. Patients visited our hospital once every 1-3 months and received intratympanic or systematic administration of steroids when otorrhea/MEE was observed. Several investigations, including blood tests, otorrhea/MEE cytology, bacterial culture tests, and respiratory function tests, were performed. In the otorrhea/MEE cytology, the leukocyte fraction was measured.Two risk factors for SNHL in EOM were middle ear mucosal thickening (p0.01) and infection (p0.05). Compared to the group with40% neutrophils in otorrhea/MEE samples, groups with 40-70% and ≥70% neutrophils had a significantly higher bone conduction hearing level (p0.01, p0.05, respectively). Two risk factors associated with the occurrence of infection in EOM were tympanic membrane (TM) perforation (p0.01) and the coincidence of otorrhea/MEE and rhinorrhea in bacterial culture test results (p0.001). A positive correlation was observed between TM perforation and infection (p0.001). Our analysis of the relationship between the frequency of intratympanic corticosteroids administration and the time-period until the occurrence of TM perforation showed that4 intratympanic administrations/year significantly increased the risk of perforation (p0.001). Pseudomonas aeruginosa was isolated from otorrhea/MEE samples, while Pseudomonas aeruginosa and fungi, detected in cultures of rhinorrhea samples, were significantly related to the deterioration of bone conduction hearing levels.The risk factors associated with the occurrence of infection in patients with EOM were TM perforation and the coincidence of otorrhea/MEE and rhinorrhea in bacterial culture test results. Since TM perforation is likely to occur even due to intratympanic corticosteroids administration, it is necessary to confirm whether the frequency of treatment is appropriate and try a less invasive technique of administration. Furthermore, Pseudomonas aeruginosa infection poses a high risk for the development of SNHL, and clinicians should be alert to this possibility, even if the bacteria were identified only in cultures of rhinorrhea samples.

We aimed to analyze clinical features and treatment outcomes of otitis media caused by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), i.e. otitis media with AAV (OMAAV).This survey was performed between December 2013 and February 2014. The study began with a preliminary survey to 123 otolaryngology institutions in Japan to inquire about their experiences with OMAAV patients during the past 10 years, and was followed by a questionnaire survey to investigate clinical and laboratory findings. OMAAV was defined using the criteria described in the text.Two hundred and thirty-five patients classified as OMAAV were enrolled in this study. They were characterized as follows: (1) disease onset with initial signs/symptoms due to intractable otitis media with effusion or granulation, which did not respond to ordinary treatments such as antibiotics and insertion of tympanic ventilation tubes, followed by progressive hearing loss; (2) predominantly female (73%) and older (median age: 68 years); (3) predominantly myeloperoxidase (MPO)-ANCA-positive (60%), followed by proteinase 3 (PR3)-ANCA-positive (19%) and both ANCAs-negative (16%); (4) frequently observed accompanying facial palsy (36%) and hypertrophic pachymeningitis (28%); and (5) disease often involving lung (35%) and kidney (26%) lesions. Four factors associated with OMAAV were found to be related to an unfavorable clinical course threatening the patient's hearing and/or lives, namely facial palsy, hypertrophic pachymeningitis, both ANCAs-negative phenotype, and disease relapse. The occurrence of hypertrophic pachymeningitis was associated with facial palsy (p 0.05), both ANCAs-negative phenotype (p 0.001), and headache (p 0.001). The administration of corticosteroid together with an immunosuppressant was an independent predicting factor for lack of disease relapse (odds ratio [OR] = 1.90, p = 0.03) and an improvement in hearing loss (OR =2.58, p = 0.0002).Since OMAAV has novel clinical features, the disease may be categorized as a subentity for the classification of AAV.

Intratympanic membrane cholesteatoma (ITMC) is uncommon. We report a case of ITMC in the right ear of a four-month-old male infant. The lesion was incidentally discovered during a routine ear examination when the boy was admitted for bacterial meningitis. The majority of previously reported cases of ITMC were seen in ears with a history of surgery or trauma, but our patient’s affected ear had experienced neither of these. His ITMC was excised at our clinic under local anaesthesia without any adverse events. We resected the ITMC using a procedure similar to myringotomy. This surgery is simple, minimally invasive, and can be performed in most otolaryngology clinics with commonly available devices. Small white masses found on the surface of the tympanic membrane should be carefully examined for the possibility of ITMC, because untreated ITMCs can grow and destroy the surrounding bone structure, in the same manner as any other acquired cholesteatoma.

We classified eosinophilic otitis media (EOM) into three grades (G1-G3) based on the middle ear mucosal thickness and aimed to establish a treatment strategy for EOM based on these pathological categories.Retrospective cohort study.Tertiary referral center.We evaluated 136 ears of 68 patients (38 women, 30 men; average age, 56.1 yr; range, 32-80 yr) with bilateral EOM, including 14 surgically treated ears.Diagnostics and treatment.Treatment responses to intratympanic instillation of triamcinolone acetonide (TA) and surgery.G1 (mild) cases of EOM responded well to intratympanic TA. G2 (moderate) cases required both TA and systemic glucocorticoids. G3 (severe) cases with granulation were unresponsive to but tolerated the treatment. In 14 ears, granulation tissue was surgically removed and the area was covered with a gelatin sponge containing TA. After the surgery, the severity level of EOM decreased from G3 to G2 in 12 ears, and hearing improved in 4 ears. Three ears showed normalization of the tympanic membrane, and seven ears had less otorrhea. Risk factors for granulation (G3) were the presence of bacterial infection (p = 0.017) and diabetes mellitus (HbA1c of ≥6.5%) (P = 0.039) (odds ratios of 4.55 and 3.95, respectively).This study showed that G3 EOM was unresponsive to conservative glucocorticoid treatment and required granulation tissue removal. Classification based on the middle ear pathology is useful for determining the most appropriate and successful treatment for EOM.

Background Chronic rhinosinusitis (CRS) can be classified into CRS with nasal polyps (CRSwNP) and CRS without nasal polyps (CRSsNP). CRSwNP displays more intense eosinophilic infiltration and the presence of Th2 cytokines. Mucosal eosinophilia is associated with more severe symptoms and often requires multiple surgeries because of recurrence; however, even in eosinophilic CRS (ECRS), clinical course is variable. In this study, we wanted to set objective clinical criteria for the diagnosis of refractory CRS. Methods This was a retrospective study conducted by 15 institutions participating in the Japanese Epidemiological Survey of Refractory Eosinophilic Chronic Rhinosinusitis (JESREC). We evaluated patients with CRS treated with endoscopic sinus surgery (ESS), and risk of recurrence was estimated using Cox proportional hazard models. Multiple logistic regression models and receiver operating characteristics curves were constructed to create the diagnostic criterion for ECRS. Results We analyzed 1716 patients treated with ESS. To diagnose ECRS, the JESREC scoring system assessed unilateral or bilateral disease, the presence of nasal polyps, blood eosinophilia, and dominant shadow of ethmoid sinuses in computed tomography (CT) scans. The cutoff value of the score was 11 points (sensitivity: 83%, specificity: 66%). Blood eosinophilia (>5%), ethmoid sinus disease detected by CT scan, bronchial asthma, aspirin, and nonsteroidal anti-inflammatory drugs intolerance were associated significantly with recurrence. Conclusion We subdivided CRSwNP in non-ECRS, mild, moderate, and severe ECRS according to our algorithm. This classification was significantly correlated with prognosis. It is notable that this algorithm may give useful information to clinicians in the refractoriness of CRS before ESS or biopsy.

We report endonasal ultrasonography (US)-assisted neuroendoscopic transsphenoidal surgery (TSS) in seven patients. With sagittal and coronal US images, internal carotid arteries, anterior cerebral arteries, residual tumor, and lateral ventricles were recognized, and the tumors were removed without leakage of cerebrospinal fluid in patients with pituitary adenoma. US images clearly depicted the carotid arteries, anterior cerebral arteries, middle cerebral arteries, chiasmatic cistern, and residual tumor. Endonasal US images can provide real-time animated information and may help neuroendoscopic TSS, whenever needed during TSS.

Additional treatment with clarithromycin (CAM) reduced persistent middle ear inflammation after acute otitis media (AOM) caused by Haemophilus influenzae in children. CAM is a treatment option for persistent inflammation following AOM and to prevent continuing otitis media with effusion.We conducted a clinical study to evaluate a new method of treatment for persistent inflammation after AOM in children.H. influenzae-infected children with AOM were treated acutely with antimicrobial agents, after which those still demonstrating effusion of the middle ear cavity received additional treatment with carbocysteine (S-CMC) alone or S-CMC combined with clarithromycin (CAM) for 1 week. The two regimens were compared in terms of clinical effects.After the initial acute treatment, many patients still showed abnormal otoscopic findings. At the completion of additional treatment, there were no significant differences between the two treatment groups. However, 1 week after completion of additional treatment, the prevalence of a diminished light reflex was significantly lower in the CAM + S-CMC group than in the S-CMC group (p = 0.017). The prevalence of redness of the tympanic membrane also tended to be lower in the combined treatment group than in those receiving a single drug (p = 0.097).

OBJECTIVE We aimed to analyze preoperative and intraoperative clinical characteristics of chronic perforated otitis media by patient age. MATERIALS AND METHODS We analyzed 137 tympanoplasties for chronic perforated otitis media in 133 patients. Exclusion criteria were ear adhesions, cholesteatoma, and a history of tympanoplasty. Patients were divided into a younger group (aged 15-39 years), a middle group (40-64 years), and an elderly group (≥65 years). We compared tympanoplasty technique (using Wullstein classification), mastoid air-cell area measured by temporal bone computed tomography, preoperative aeration of the temporal bone, mean preoperative hearing (at 500 Hz, 1000 Hz, and 2000 Hz), and patch effect calculated by the pre- and post-patch air-bone gap at frequencies of 250 Hz and 500 Hz among the three age groups. RESULTS Although type I tympanoplasty was performed most frequently in all age groups, it was performed significantly less often in the elderly group (p

OBJECTIVE To investigate preoperative clinical findings of chronic otitis media (COM) in patients with Down and without (non-Down) syndrome. STUDY DESIGN Retrospective. SETTING Referral hospital, otolaryngology department. PATIENTS Patients with COM who underwent tympanoplasty were included. There were 10 ears of 8 patients (mean age, 14.9 yr) in the Down group and 44 ears of 41 patients (mean age, 14.7 yr) in the non-Down group. MAIN OUTCOME MEASURES Clinical characteristics, including the frequency of preoperative persistent otorrhea, cause of COM, and mastoid pneumatization (maturation), were compared between the 2 groups using clinical records and temporal bone computed tomography (CT). RESULTS Preoperative persistent otorrhea was more frequent in the Down group (60%) than in the non-Down group (27.2%; p < 0.05). Perforation due to tympanostomy tube insertion also occurred more frequently in the Down group (100%) than in the non-Down group (53.3%; p < 0.05). Mastoid pneumatization occurred significantly less in the Down group than in the non-Down group (p < 0.01). There was no significant difference in mastoid pneumatization regardless of the presence or absence of a past history of tympanostomy tube insertion in the Down group (p = 0.3, t test) unlike that in the non-Down group (p < 0.05, t test). All ears attained a dry condition with no perforated eardrums. CONCLUSION Frequent draining ear and extremely immature mastoid pneumatization, regardless of the presence or absence of a past history of tympanostomy tube insertion, were clinically important characteristics of COM in patients with Down syndrome.

Eosinophilic otitis media (EOM) is an intractable otitis media characterized by a highly viscous effusion that contains eosinophils. We previously reported that high levels of IgE were detected in middle ear effusion (MEE) of patients with EOM.To examine antigen-specific IgE in MEE of patients with EOM to clarify the pathogenesis of EOM.Twenty-six patients with EOM associated with bronchial asthma were included in this study. Antigen-specific IgE against inhalant and bacterial antigens were measured in the serum and MEE of these patients.In patients with EOM, 1 or more antigen-specific IgEs were detected from the MEE of 16 of 26 patients (62%), whereas 1 of 9 control patients (11%) had antigen-specific IgE (P.01). Total serum IgE concentrations were not different between the groups who tested positive (16 patients) and negative (10 patients) for antigen-specific IgE. None of the fungi-specific IgEs were detected in serum even though 11 patients tested positive for 1 or more fungal antigens detected in MEE. The severity score of EOM in the antigen-specific IgE-positive group was significantly higher than that in the antigen-specific IgE-negative group (P.05).Antigen-specific IgE against inhalant and bacterial antigens may be locally produced in the middle ear mucosa in patients with EOM. In particular, local sensitization against fungi together with Staphylococcus aureus could result in local IgE production in the middle ear and may be responsible for the severity of EOM.

To (1) indicate the definition, the disease state, methods of diagnosis, and testing for otitis media with effusion (OME) in childhood (12 years); and (2) recommend methods of treatment in accordance with the evidence-based consensus reached by the Subcommittee of Clinical Practice Guideline for Diagnosis and Management of OME in Children.We produced Clinical Questions (CQs) concerning the treatment of OME and searched the literature published until April 2014 according to each theme including CQ, the definition, the disease state, the method of diagnosis, and examination. The recommendations are based on the results of the literature review and the expert opinion of the Subcommittee.Because children with Down's syndrome and cleft palate are susceptible to OME, we categorized OME into low-risk and high-risk groups (e.g., Down's syndrome and cleft palate), and recommended the appropriate treatment for each group.In the clinical management of OME in children, Japanese Clinical Practice Guidelines recommend management not only of OME itself, such as effusion in the middle ear and pathological changes in the tympanic membrane, but also pathological abnormality in surrounding organs, such as infectious or inflammatory diseases.

This study investigated the difference in clinical characteristics of fallopian canal dehiscence associated with pars flaccida and pars tensa cholesteatomas for the purpose of increasing the preoperative detectability of dehiscence. A total of 189 ears of patients 7-80 years of age (mean 42 years) with pars flaccida cholesteatoma and 63 ears of patients 9-84 years of age (mean 50 years) with pars tensa cholesteatoma were studied. All patients had undergone prior surgical management at our institution from January 2006 to April 2012. The incidence of fallopian canal dehiscence and its location were compared between pars flaccida and pars tensa cholesteatomas. Intraoperative findings of coexistent pathologies, including destruction of the stapes superstructure, labyrinthine fistula, and dural exposure, were compared between the dehiscence and no-dehiscence groups for the two types of cholesteatomas. The incidence of dehiscence was significantly higher in patients with pars tensa cholesteatoma (55.6 %) than in patients with pars flaccida cholesteatoma (26.5 %). Dehiscence located posterior to the cochleariform process occurred slightly more frequently in patients with pars tensa cholesteatoma than in those with pars flaccida cholesteatoma. In patients with pars flaccida cholesteatoma, labyrinthine fistulas and dural exposure were significantly more frequent in the dehiscence group than in the no-dehiscence group. Fallopian canal dehiscence is more frequent in patients with pars tensa cholesteatoma than in those with pars flaccida cholesteatoma. Especially in patients with pars flaccida cholesteatoma, paying special attention to these coexisting pathologies is important to increase preoperative detectability of dehiscence.

To investigate the different pathways of progression to the middle ear in keratosis obturans (KO) and external auditory canal cholesteatoma (EACC).Retrospective case review.Referral hospital otolaryngology department.Patients with KO or EACC and middle ear disease who underwent surgical management were included. Four ears of 4 patients (mean age, 41.25 yr) were the KO group, and 5 ears of 4 patients (mean age, 49.5 yr) were the EACC group.Intraoperative findings of the middle ear cavity were investigated in KO and EACC groups.In the KO group, 3 patients had a perforated tympanic membrane and cholesteatoma in the tympanic cavity. The other patient had preoperative right facial palsy. Removal of the keratin plug revealed an adherent tympanic membrane. In intraoperative findings, the tympanic segment of the fallopian canal was found to be eroded because of inflammation. No case initially progressed to the mastoid cavity. Four patients had external auditory canal cholesteatoma with middle ear disease. In EACC group, all patients had initial progression to the mastoid cavity.KO tends to progress initially to the tympanic cavity via a diseased tympanic membrane. EACC tends to progress to the mastoid cavity via destruction of the posterior bony canal. This is the first report to investigate differences in pathway of progression to the middle ear cavity in these 2 diseases.

We report three patients with pars flaccida-type cholesteatoma (attic cholesteatoma) with closure of the entrance to the cholesteatoma at the time of surgery. These patients were diagnosed with attic cholesteatoma requiring surgery on the basis of abnormal findings of the pars flaccida, audiometry, and temporal bone computed tomography during the clinical course. Intraoperatively, cholesteatoma matrix and granulation tissue were observed behind the intact pars flaccida epithelium, which suggested that the entrance had apparently closed and the continuity with the cholesteatoma matrix disappeared after resolution of inflammation at the pars flaccida. In such patients, a normal pars flaccida may cause cholesteatoma to be initially overlooked, or misdiagnosed as congenital cholesteatoma. The diagnosis should be carefully made on the basis of the clinical course and the results of various examinations.

ObjectiveEosinophilic otitis media (EOM) is an intractable otitis media characterized by a highly viscous effusion containing eosinophils, and high levels of immunoglobulin (Ig) E are detected in the middle ear effusion (MEE). We carried out a pilot study to determine whether anti-IgE therapy is eff

To investigate the benefits and problems of tympanoplasty in elderly patients older than 70 years.Retrospective case review.Referral hospital otolaryngology department.Among 1,014 patients who underwent tympanoplasty for chronic otitis media from 2006 to 2011, those aged over 70 years were eligible for inclusion.Clinical characteristics including tympanosclerosis and preoperative severe complications were investigated. In chronic otitis media group, hearing outcomes (air-bone gap at 500-Hz and 1- and 2-kHz frequency) and postoperative ear condition were investigated. In chronic otitis media with cholesteatoma, clinical characteristics including postoperative bone conduction hearing threshold (at 500-Hz and 1- and 2-kHz frequency) were investigated.Ninety-seven ears of 83 patients were included. Eighteen ears had obvious findings of tympanosclerosis (18/97 = 18.6%). In chronic otitis media without cholesteatoma (52 ears/47 patients), no preoperative complications were noted. The mean air-bone was 30.8 and 16.1 dB before and after the operation, respectively (p0.001). Otorrhea disappeared in 51 ears (98.1%). In chronic otitis media with cholesteatoma (42 ears/33 patients), we noted preoperative severe complications including labyrinthine fistula (7/97 = 7.22%), widely exposed dura (1/97 = 1.03%), and facial palsy 1 (1/97 =1.03%). The mean bone conduction hearing threshold was 39.6 dB and 40.89 dB, respectively (p = 0.7). Three ears of 3 patients had operated ears with open mastoid and underwent canal wall reconstruction tympanoplasty.Tympanoplasty in elderly patients older than 70 years seems to be as safe as when performed in younger patients.

OBJECTIVE To investigate differences in middle ear ventilation mechanisms between pars flaccida and pars tensa cholesteatoma. STUDY DESIGN Retrospective case review. SETTING A referral hospital otolaryngology department. PATIENTS Sixty-six ears with pars flaccida cholesteatoma (mean age, 45.7 yr) and 19 ears with pars tensa cholesteatoma (mean age, 58.8 yr) were included. Patients with totally adhesive tympanic membranes or mixed-type cholesteatoma were excluded. INTERVENTIONS Patients underwent canal wall down tympanoplasty with canal reconstruction. The canal wall was reconstructed with tragal or conchal cartilage and cortical bone grafts. MAIN OUTCOME MEASURES Habitual sniffing, preoperative sonotubometry results, mastoid pneumatization (maturation), and postoperative aeration around the stapes were investigated. Preoperative mastoid pneumatization and postoperative aeration around the stapes were measured on computed tomography scans. RESULTS Ten (15.4%) of 65 patients with pars flaccida cholesteatoma and 3 (15.7%) of 19 patients with pars tensa cholesteatoma were habitual sniffers (p = 0.5). Preoperative sonotubometry indicated that a patulous pattern was more common in ears with pars flaccida than pars tensa cholesteatoma (42.8% versus 7.1%, p < 0.05), and a stenotic pattern was more common in ears with pars tensa than pars flaccida cholesteatoma (85.8% versus 42.8%, p < 0.01). Preoperative mastoid pneumatization and postoperative aeration around the stapes were significantly better in ears with pars flaccida than pars tensa cholesteatoma (p < 0.01 and p < 0.05, respectively). CONCLUSION Significantly different tubal function and mastoid pneumatization patterns suggest differences in ventilation disorders and cause between ears with pars flaccida and pars tensa cholesteatoma.

Objective Eosinophilic otitis media (EOM) is a newly recognized intractable middle ear disease, characterised by the accumulation of eosinophils in middle ear effusion and middle ear mucosa. Since EOM patients show gradual or sudden deterioration of hearing, it is important to properly diagnose EOM and to start adequate treatment for EOM. We aimed to investigate the clinical risk factors of EOM and to establish the diagnostic criteria of EOM. Patients and methods We reviewed 138 patients with EOM and 134 age-matched patients with the common type of otitis media with effusion or chronic otitis media as controls. We analyzed the incidence of the following clinical variables in both groups: bilaterality of otitis media, viscosity of middle ear effusion, formation of granulation tissue in the middle ear, response to the treatment for otitis media, deterioration of bone conduction hearing level, and association with other diseases such as bronchial asthma, chronic rhinosinusitis, nasal polyposis, and allergic rhinitis. Results A high odds ratio was obtained from an association with bronchial asthma (584.5), resistance to conventional treatment for otitis media (232.2), viscous middle ear effusion (201.6), association with nasal polyposis (42.17), association with chronic rhinosinusitis (26.49), bilaterality (12.93), and granulation tissue formation (12.62). The percentage of patients with EOM who were positive for two or more among the highest four items was 98.55%. Conclusion A patient who shows otitis media with effusion or chronic otitis media with eosinophil-dominant effusion (major criterion) and with two or more among the highest four items (minor criteria), can be diagnosed as having EOM. Patients with ear symptoms should have the proper diagnosis of EOM using the proposed diagnostic criteria, and then can receive adequate treatment, resulting in prevention of deterioration of hearing and quality of life.

Objective:Small cell carcinoma has the worst prognosis of all laryngeal neoplasms. In order to further characterise this tumour, with a view to development of new therapeutic approaches, we report the results of KIT gene and platelet-derived growth factor receptor α gene expression analysis, for two extremely rare cases of primary small cell carcinoma of the larynx.Method:Case reports, including immunohistochemical study, and review of the literature.Results:We present two patients with laryngeal small cell carcinoma, who died from tumour metastasis to the lungs and brain despite aggressive treatment. Immunohistochemical studies revealed positive reactions for KIT gene expression and platelet-derived growth factor α gene expression in patient one, and for KIT gene expression in patient two. Molecular genetic analysis, using polymerase chain reaction direct sequencing, identified no mutations of the KIT or platelet-derived growth factor receptor α genes.Conclusion:Although further investigation is necessary regarding KIT gene expression and platelet-derived growth factor receptor α gene expression in laryngeal small cell carcinoma, the reported results suggest that these genes may be significant in the development of molecular targeted therapy.

Objective Eosinophilic otitis media (EOM) is characterized by the extensive accumulation of eosinophils in the middle ear mucosa and middle ear effusion and is usually associated with bronchial asthma. Eosinophilic otitis media patients show gradual or sudden deterioration of hearing. In our previous study, we reported that high-tone loss was more frequently found and more severe in EOM patients than in control patients with chronic otitis media. These findings suggest that not only bacterial infection but also eosinophilic inflammation in the middle ear may damage the inner ear. The present study was performed to determine whether eosinophilic inflammation is indeed related to deterioration of bone-conduction hearing level (BCHL). Patients Fifty-five ears of 28 patients with EOM associated with bronchial asthma were included in this study. Middle ear effusion (MEE) samples were collected from all the patients, and the concentrations of eosinophilic cationic protein (ECP) and immunoglobulin E (IgE) were measured by fluorescence enzyme immunoassay. The BCHLs at 2 and 4 kHz for the worse-hearing ear of each patient were correlated with the concentrations of ECP and IgE. Results The concentration of IgE in MEE significantly and positively correlated with BCHL at 2 and 4 kHz. The ears with a higher concentration of ECP in MEE also tended to show deterioration of BCHL at 4 kHz. Other clinical risk factors for BCHL deterioration were male sex, long duration of EOM, association with bacterial infection, severe inflammatory changes of the middle ear mucosa, and high serum IgE concentration. Conclusion Eosinophilic-inflammation-related substances such as ECP and IgE are closely related to the deterioration of BCHL at high frequencies. Particularly, IgE concentration in MEE is a good indicator of BCHL elevation. We should always pay attention to the hearing acuity of EOM patients with the risk factors.

We report two cases of otitis media positive for antimyeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) associated with facial palsy. Case 1: A 73-year-old man treated for 3 months for bilateral otitis media with effusion had left facial nerve palsy and deteriorated bone conduction hearing in both ears. Blood analysis showed elevated MPO-ANCA to 134 EU. Case 2: A 66-year-old woman treated for about one year for bilateral otitis media with effusion and fluctuating mixed hearing loss had bilateral facial nerve palsy and a blood test positive for MPO-ANCA at 67 EU. Both were diagnosed with otitis media caused by ANCA-related vasculitis. After prednisolone and cyclophosphamide administration for half a year, blood test results were negative for MPO-ANCA. Both recovered almost completely from facial nerve palsy and bone conductive hearing loss partially improved except in one hearing-impaired ear. ANCA-related vasculitis of the temporal bone should thus be considered in those with intractable otitis media and deteriorated bone conduction hearing before the occurrence of facial palsy.