Surgical management of secondary acquired cholesteatoma depends on its characteristics.

OBJECTIVE: Cholesteatoma secondary to tympanic perforation, known as "secondary acquired cholesteatoma" may progress slower than a retraction pocket cholesteatoma, with less bone destruction and fewer intracranial complications. However, complete surgical removal remains difficult because the pathological epithelium on the marginal side of the extension is not covered by the subepithelial layer of the cholesteatoma matrix, making the boundary with the middle ear mucosa difficult to identify. Therefore, considering the pathophysiology of secondary acquired cholesteatoma, suitable preoperative evaluation and surgical techniques are required. In this study, we aimed to evaluate (i) the extension of secondary acquired cholesteatoma according to the size and location of tympanic membrane perforation; and (ii) the microscopic surgical outcomes, including the rate of residual cholesteatoma, requirement for specialized surgical management, and changes in hearing. METHODS: This retrospective study included data of cases with secondary acquired cholesteatoma (n = 66; 66 ears of 64 patients, 2 patients had bilateral ear involvement), including those who underwent a staged operation (n = 25). RESULTS: The perforation level of the tympanic membrane was associated with the cholesteatoma extension. When the cholesteatoma extended around the stapes, staged operation was chosen. Six cases of spontaneous resolution of stapes lesions at the time of staged surgery were observed. A significant postoperative improvement in hearing was observed; however, five cases experienced sensorineural hearing loss. CONCLUSION: Surgery, including staged surgery of the stapes lesions, along with careful observation of the perforation, is required in secondary acquired cholesteatoma. For lesions that are visible yet challenging to remove, it is imperative to exercise prudent judgment, taking into account the possibility of spontaneous resolution.

Risk factors for occurrence and progression of external auditory canal cholesteatoma.

OBJECTIVE: External auditory canal cholesteatoma (EACC) is characterized by retained squamous debris within the external canal and variable amounts of localized bone destruction. The etiology of primary EACC remains incompletely understood. This study was conducted to analyze the clinical features and backgrounds of patients with primary EACC and to clarify the risk factors for the occurrence and progression of EACC. METHODS: Sixty-nine ears of 62 patients diagnosed with primary EACC were included in this study (EACC group). Additionally, 74 ears of 60 patients with chronic otitis media (COM) with perforation who underwent tympanoplasty or myringoplasty were included as controls (COM group). We retrospectively investigated the clinical features, life history, and medical history of the patients in both groups. In addition, to investigate the risk factors for progression of EACC, we compared the clinical features and medical history of patients with stage IV (advanced) disease versus stage I + II (mild) disease. RESULTS: The inferior wall of the bony canal was the main structure affected in patients with primary EACC of all stages. The following factors were significantly more common in the EACC than COM group: older age, female sex, left-sided disease, osteoporosis, renal dysfunction, anemia, and treatment with bisphosphonates. Among these, the most significant factor associated with EACC was renal dysfunction (odds ratio, 11.4; 95 % confidence interval, 2.32-55.9). The significant factors observed in patients with stage IV disease were younger age, male sex, posterior wall involvement, and otorrhea. Surgical treatment was required for more than half of the patients with stage III and IV EACC. CONCLUSION: Patients with renal dysfunction are at risk of primary EACC. In particular, younger patients and relatively younger elderly patients with posterior wall involvement have a risk of progression to advanced-stage EACC. Canalplasty should be considered in patients with EACC who have these risk factors to prevent progression to advanced-stage disease.

Myringoplasty for Eosinophilic Otitis Media.

OBJECTIVE: Surgical intervention of eosinophilic otitis media (EOM) has been considered contraindicated because middle ear surgery is associated with a risk of deafness. Myringoplasty is believed to be less invasive. Therefore, we analyzed the surgical results of myringoplasty for perforated eardrums in patients with EOM treated by biological drugs (biologics). STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Nine ears of seven patients with EOM with eardrum perforation associated with bronchial asthma were treated with add-on biologics; myringoplasty was then performed. The controls comprised 17 ears of 11 patients with EOM treated by myringoplasty without biologics. INTERVENTIONS: The EOM status of each patient of both groups was assessed using severity scores, hearing acuity, and temporal bone computed tomography scores. MAIN OUTCOME MEASURES: Preoperative and postoperative changes in severity scores and hearing acuity, postoperative closure of the perforation, and relapse of EOM. RESULTS: Severity scores significantly decreased after the use of biologics but did not change after myringoplasty. One patient developed postoperative relapse of middle ear effusion (MEE); in the control group, however, 10 ears developed recurrence of MEE. Significant improvement of the air conduction hearing level was obtained in the biologics group. No patients showed deterioration of the bone conduction hearing level. CONCLUSIONS: This is the first report to describe successful surgical interventions with add-on biologics for patients with EOM. In the era of biologics, surgical interventions such as myringoplasty will be indicated to improve hearing and to avoid recurrence of MEE in patients with EOM with perforated eardrums, with the use of biologics.

Eosinophilic otitis media; state-of-the-art diagnosis and treatment.

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.

Clinical practice guidelines for the diagnosis and management of otitis media with effusion (OME) in children in Japan - 2022 update.

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. METHOD: 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. RESULTS: 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. CONCLUSION: 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.

Clinical characteristics of atelectatic eardrums and adhesive otitis media in children.

OBJECTIVES: 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. PATIENTS AND METHODS: 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. RESULTS: 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). CONCLUSION: 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.

[SUCCESSFUL TREATMENT FOR OTITIS MEDIA AND SINUSITIS ASSOCIATED WITH EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS USING ANTI-IL-5 RECEPTOR MONOCLONAL ANTIBODY BENRALIZUMAB].

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.

Pharmacotherapy focusing on for the management of otitis media with effusion in children: Systematic review and meta-analysis.

OBJECTIVE: To examine the evidence for treating children with otitis media with effusion with pharmacotherapy. DATA SOURCES: 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). STUDY SELECTION: Articles addressing pharmacotherapy for the management of otitis media with effusion in children were selected in English. DATA EXTRACTION: 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. DATA SYNTHESIS: 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. CONCLUSIONS: We found evidence of benefit from treatment of OME in children with intranasal steroids and S-carboxymethylcysteine at longer-term follow-up.

Comparative study on adhesive otitis media and pars tensa cholesteatoma in children.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Effectiveness of myringoplasty in patients with eosinophilic otitis media.

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.

Dupilumab therapy for patients with refractory eosinophilic otitis media associated with bronchial asthma.

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.

Risk factors for bacterial infection to cause sensorineural hearing loss in eosinophilic otitis media.

OBJECTIVE: 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. METHODS: 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. RESULTS: Two risk factors for SNHL in EOM were middle ear mucosal thickening (p <0.01) and infection (p <0.05). Compared to the group with <40% neutrophils in otorrhea/MEE samples, groups with 40-70% and ≥70% neutrophils had a significantly higher bone conduction hearing level (p <0.01, p <0.05, respectively). Two risk factors associated with the occurrence of infection in EOM were tympanic membrane (TM) perforation (p <0.01) and the coincidence of otorrhea/MEE and rhinorrhea in bacterial culture test results (p <0.001). A positive correlation was observed between TM perforation and infection (p <0.001). Our analysis of the relationship between the frequency of intratympanic corticosteroids administration and the time-period until the occurrence of TM perforation showed that >4 intratympanic administrations/year significantly increased the risk of perforation (p<0.001). 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. CONCLUSION: 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.

Clinical characteristics, the diagnostic criteria and management recommendation of otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) proposed by Japan Otological Society.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a form of necrotizing vasculitis with few or no immune deposits. It primarily affects small and medium blood vessels. AAV is classified into three categories, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangitis (EGPA), and two major ANCAs, proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA are involved in their pathogenesis. Intractable otitis media frequently occurs in patients with GPA, MPA or EGPA, although all patients show similar clinical features, regardless of the type of AAV. Furthermore, approximately 15% patients with otitis media caused by AAV do not show ANCA positivity, histopathological evidence, or any other AAV-related lesions at the initial visit; therefore, these patients do not fulfill the ordinary diagnostic criteria for systemic AAV. Thus, we first proposed that this condition could be categorized as "otitis media with AAV (OMAAV)". Subsequently, the Japanese Otological Society (JOS) conducted a nationwide survey between December 2013 and February 2014 and identified 297 patients with OMAAV. The survey revealed that OMAAV is a disease that initially occurs in the middle ear and subsequently spreads to other organs such as the lungs and kidneys, with eventual involvement of all body organs. Severe sequelae such as facial palsy, hypertrophic pachymeningitis, complete deafness, and subarachnoid hemorrhage resulting in death can also occur. In this review, we introduce the clinical features, diagnostic criteria, and treatment strategies recommended by JOS for early diagnosis and treatment of OMAAV.

Clinical efficacy of anti-IL-5 monoclonal antibody mepolizumab in the treatment of eosinophilic otitis media.

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.

Proposal of a Treatment Strategy for Eosinophilic Otitis Media Based on Middle Ear Condition.

OBJECTIVE: 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. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: 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. INTERVENTIONS: Diagnostics and treatment. MAIN OUTCOME MEASURES: Treatment responses to intratympanic instillation of triamcinolone acetonide (TA) and surgery. RESULTS: 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). CONCLUSIONS: 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.

Clinical Characteristics of Chronic Perforated Otitis Media in Different Age Groups.

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<0.05). The mean mastoid pneumatization index was significantly lower in the elderly group (p<0.05). Preoperative air-conduction hearing and bone-conduction hearing differed significantly among age groups (p<0.05 for both). There were no significant differences in the air-bone gap among the three groups. The mean patch effect was significantly better in the younger group than in middle or elderly groups (p<0.05). CONCLUSION: The less pneumatized mastoid and ossicular diseases in elderly patients with chronic perforated otitis media suggest that they had longer lasting and more severe childhood middle ear pathologies than did younger patients.

Clinical practice guidelines for the diagnosis and management of otitis media with effusion (OME) in children in Japan, 2015.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Attic cholesteatoma with closure of the entrance to pars flaccida retraction pocket.

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.

Clinical features and treatment outcomes of otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV): A retrospective analysis of 235 patients from a nationwide survey in Japan.

OBJECTIVE: 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). METHODS: 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. RESULTS: 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). CONCLUSION: Since OMAAV has novel clinical features, the disease may be categorized as a subentity for the classification of AAV.