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.

Risk factors associated with severity of eosinophilic otitis media.

OBJECTIVE: Eosinophilic otitis media (EOM) is an intractable otitis media associated with bronchial asthma. Clinical characteristics of EOM are apparent, but severe EOM, which is unresponsive to ongoing treatments, can occur. The present study aimed to investigate potential risk factors associated with the severity of EOM. METHODS: We scored the EOM severity of 26 patients according to quantity of middle ear effusion (MEE), thickness of the middle ear mucosa, use of topical and oral corticosteroids, and use of antibiotics, all measured over a 3-month period. The scores for four 3-month periods (1 year) were averaged. We analyzed the prevalence of clinical variables by partial regression: sex, age, body mass index (BMI), duration of bronchial asthma, association of aspirin-intolerant asthma, Lund-Mackay score for sinusitis, mastoid pneumatization, width of the bony Eustachian tube at the tympanic orifice, percentage of eosinophils and immunoglobulin E in peripheral blood, and association of allergic rhinitis. Duration of bronchial asthma was defined as the period from onset of bronchial asthma to the age of first consultation at our hospital. Samples of MEE were taken for bacterial culture. RESULTS: The average severity score was 6.6 (out of 16). The severity score in the pathogen-positive MEE group was significantly higher than that in the pathogen-negative MEE group (p<0.05). The score was not significantly different between the seasons. Linear multiple regression analysis showed that BMI and the duration of bronchial asthma significantly affected the EOM severity score (p<0.05). The presence of aspirin intolerant asthma tended to be correlated with the severity score. The Lund-Mackay score tended to be negatively correlated with it. CONCLUSIONS: There is a significant association between the severity of EOM and obesity, as well as with the duration of bronchial asthma.

A comparison of preoperative characteristics of chronic otitis media in Down and non-Down syndrome.

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.

Effect of omalizumab on biomarkers in middle ear effusion in patients with eosinophilic otitis media.

CONCLUSIONS: Eosinophil cationic protein (ECP) concentrations in middle ear effusion (MEE) in patients with eosinophilic otitis media (EOM) were significantly decreased at 3 months after the administration of omalizumab from the baseline level (p < 0.05). This study provides new evidence that omalizumab reduces eosinophilic inflammation in the middle ear and that the reduction of ECP may not be caused by suppression of interleukin (IL)-5 production in the middle ear mucosa. OBJECTIVE: EOM is an intractable otitis media characterized by a highly viscous effusion containing eosinophils. We recently reported that anti-IgE therapy using omalizumab was efficacious in the treatment of EOM. To clarify the underlying mechanism, we determined changes in biomarkers in MEE related to eosinophilic inflammation after therapy. METHODS: Nine patients with EOM received the anti-IgE agent omalizumab for 3 months. Among them, five patients continued anti-IgE therapy for longer than 1 year. Eight EOM patients without administration of omalizumab were also included in the study as controls. The concentrations of eosinophilic inflammatory markers such as ECP, IgE, IL-4, and IL-5 in MEE were measured before and after the administration of omalizumab. RESULTS: After 3 months of omalizumab therapy, the ECP concentration in MEE was significantly reduced from the baseline level (p < 0.05), while no significant change of ECP in the serum was observed. The concentrations of IL-4 and IL-5 in MEE showed no significant change before and after the therapy in EOM patients treated with omalizumab.

Fallopian canal dehiscence at pediatric cholesteatoma surgery.

The objectives of the study were to investigate the characteristics of ears with dehiscence of the fallopian canal at the time of cholesteatoma surgery and the relationship between dehiscence and age, and to consider the reasons why the fallopian canal tends to be preserved in pediatric patients. This study included 37 ears with cholesteatoma in pediatric patients (mean age 9.2 years, age range 4-14 years) and 273 ears with cholesteatoma in non-pediatric patients (mean age 45 years, age range 15-84 years). Patients were treated between January 2006 and April 2012. All patients had undergone prior tympanoplasty under general anesthesia at our institution. Facial canal dehiscence was evaluated by inspection and through palpation by blunt picking after the pathological tissues had been removed. The size of fallopian canal dehiscence was not investigated in this study. The frequency of dehiscence of the fallopian canal according to the type of cholesteatoma and coexisting pathological conditions, including destruction of the stapes, presence of a labyrinthine fistula, and dural exposure, were compared between the pediatric and non-pediatric groups. The frequency of dehiscence in cases with destruction of the stapes was also compared between the pediatric and non-pediatric groups. Dehiscence of the fallopian canal occurred in 6 of 37 ears (16.8 %) in the pediatric group and 91 of 273 ears (33.3 %) in the non-pediatric group (p < 0.05). In congenital cholesteatoma, the frequency of dehiscence was lower in the pediatric group than in the non-pediatric group (p < 0.05). However, in other types of cholesteatoma there was no statistically difference between the two types of cholesteatoma. The frequency of the destruction of the stapes was higher in the pediatric group than in the non-pediatric group (43.2 vs. 16.5 %, p < 0.001). In patients with severe destruction of the stapes, the fallopian canal was preserved more frequently in the pediatric group than in the non-pediatric group (p < 0.05). The frequency of dehiscence of the fallopian canal at the time of cholesteatoma surgery was lower in the ears of pediatric patients than in the ears of non-pediatric patients. This is probably due to the difference in types of cholesteatoma between the two groups and other unknown mechanisms.

Reversible cochlear function with ANCA-associated vasculitis initially diagnosed by otologic symptoms.

OBJECTIVE: To present 8 cases with hearing loss as an initial symptom of antineural cytoplasmic antibody (ANCA)-associated vasculitis (AAV) involving granulomatosis with polyangiitis (GPA) and to discuss the treatment and mechanisms of hearing outcomes after immunosuppressive therapy. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Eight patients were referred to our university hospital between 2004 and 2012 for intractable otitis media with acute progressive mixed (conductive and sensorineural) hearing loss and facial palsy. INTERVENTION(S): Diagnostics and treatment. MAIN OUTCOME MEASURES: Otologic symptoms as initial manifestations of otitis media with AAV and cochlear function after treatment. RESULTS: Eight cases (6 female and 2 male subjects; aged 54-73 yr; 6 MPO [myeloperoxidase]-ANCA-positive and 2 PR3 [proteinase 3]-ANCA-positive cases) were included. Progressive hearing loss was present in all patients, and facial palsy was present in 5 of 8 patients total. Patients with hearing levels better than 95 dB improved with good speech discrimination after immunosuppressive therapy, but the completely deaf could not be recovered. All patients have been successfully controlled for 1 to 8 years without any systemic disorders. CONCLUSION: This study showed the difficulty of diagnosing localized AAV and the effectiveness of immunosuppressive therapy for hearing loss in the early stage. Based on these results, early-stage AAV would influence the stria vascularis in the cochlea. Otitis media with ANCA-associated vasculitis is a new entity among the causes of intractable otitis media and progressive hearing loss.

Differences in clinical characteristics of fallopian canal dehiscence associated with pars flaccida and pars tensa cholesteatomas.

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.

A comparison of patterns of disease extension in keratosis obturans and external auditory canal cholesteatoma.

OBJECTIVE: To investigate the different pathways of progression to the middle ear in keratosis obturans (KO) and external auditory canal cholesteatoma (EACC). STUDY DESIGN: Retrospective case review. SETTING: Referral hospital otolaryngology department. PATIENTS: 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. MAIN OUTCOME MEASURES: Intraoperative findings of the middle ear cavity were investigated in KO and EACC groups. RESULTS: 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. CONCLUSION: 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.

Case of high-risk epithelioid hemangioendothelioma of the neck.

BACKGROUND: Epithelioid hemangioendotheliomas (EHEs) of the head and neck region are uncommon malignant neoplasms that exhibit various biologic behaviors characteristic of both low- and high-grade malignancy. A subgroup of EHEs identified as "high-risk" EHEs because of their size and mitotic activity is associated with an unfavorable clinical course and poor prognosis. MATERIALS AND RESULTS: We describe the treatment of the first and, in terms of size, largest case of high-risk EHE arising from the neck. Despite wide excision, recurrence occurred 9 months after surgery, as had been expected. However, the tumor was found to express both vascular endothelial growth factor (VEGF) and VEGF receptor 2, indicating the potential of anti-VEGF therapy in the treatment of such cases. CONCLUSION: The finding that a high-risk EHE arising from the head and neck region is characterized by expression of VEGF and its receptor provides further support for the development of targeted molecular therapies.

Clinical efficacy of anti-IgE therapy for eosinophilic otitis media.

OBJECTIVE: Eosinophilic 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 efficacious in the treatment of EOM. STUDY DESIGN: Prospective study. SETTING: Tertiary referral center. PATIENTS AND METHODS: Eight patients with EOM received the anti-IgE agent omalizumab for at least 3 months, in addition to ordinary treatments for EOM. They were evaluated by a questionnaire for ear and respiratory symptoms, clinical scores, surrogate markers in the blood, and hearing acuity before and after the anti-IgE therapy. Nine EOM patients without anti-IgE therapy were included as controls. RESULTS: The ear symptom scores and clinical scores gradually decreased during the therapy. In particular, 5 patients who were treated for more than 1 year showed improvement of their clinical scores with resolution of the MEE. The total serum IgE level was significantly elevated after 3 months of therapy (p < 0.01). Deterioration of the bone conduction hearing levels was more frequently found in the control group than in the omalizumab group. CONCLUSION: This pilot study provides new evidence establishing that long-term anti-IgE therapy improved the clinical ear symptoms of EOM and bone conduction hearing levels were mostly preserved. Therefore, long-term anti-IgE therapy can be effective for EOM to inhibit eosinophilic inflammation in the middle ear.

Clinical characteristics and surgical benefits and problems of chronic otitis media and middle ear cholesteatoma in elderly patients older than 70 years.

OBJECTIVE: To investigate the benefits and problems of tympanoplasty in elderly patients older than 70 years. STUDY DESIGN: Retrospective case review. SETTING: Referral hospital otolaryngology department. PATIENTS: Among 1,014 patients who underwent tympanoplasty for chronic otitis media from 2006 to 2011, those aged over 70 years were eligible for inclusion. MAIN OUTCOME MEASURES: 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. RESULTS: 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 (p < 0.001). 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. CONCLUSION: Tympanoplasty in elderly patients older than 70 years seems to be as safe as when performed in younger patients.

Differences in middle ear ventilation disorders between pars flaccida and pars tensa cholesteatoma in sonotubometry and patterns of tympanic and mastoid pneumatization.

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.

Two cases of spontaneous regression of congenital cholesteatomas.

Congenital cholesteatoma arises behind a normal, intact tympanic membrane and is most plausibly explained by the persistence of fetal epidermoid formation. It is generally accepted that cholesteatoma in children has greater aggressive growth activity than that in adults. The congenital type of cholesteatoma starts as a small pearl in the middle ear and eventually grows to involve the ossicles and then the attic and mastoid, causing varying degrees of destruction and functional impairment. However, here we report the cases of two children with congenital cholesteatoma which disappeared spontaneously during the follow-up period. To the best of our knowledge, this is the first report on the spontaneous regression of congenital cholesteatoma. On the basis of the two cases, we could take a "wait and watch" attitude for congenital cholesteatoma in the absence of severe infection, hearing loss, or bone destruction in the middle ear.

Relationship between postoperative aeration around the stapes and postoperative hearing outcome after canal wall down tympanoplasty with canal reconstruction for cholesteatoma.

OBJECTIVES: To evaluate the correlation between postoperative aeration around the stapes and hearing outcome after canal wall down tympanoplasty with canal reconstruction for cholesteatoma. STUDY DESIGN: Retrospective case review. SETTING: University hospital otolaryngology department. PATIENTS: Seventy ears of 65 patients with middle ear cholesteatoma were included. Patients who had fixed or poorly mobile ossicular chain was excluded. INTERVENTIONS: They were underwent canal wall down tympanoplasty with canal reconstruction. Canal wall was reconstructed with the tragal or conchal cartilage and the cortical bone plate. MAIN OUTCOME MEASURES: We measured aeration around the stapes on coronal and axial computed tomographic sections at 1 year after ossiculoplasty and investigated the correlation between postoperative aeration around the stapes and postoperative air-bone gap (using the mean of 0.5-, 1-, and 2-kHz threshold values) at 1 year after ossiculoplasty. We also investigated it for each of Wullstein type and for each of 0.25-, 0.5-, 1-, 2-, and 4-kHz thresholds. RESULTS: Aeration around the stapes was negatively correlated with postoperative air-bone gap (correlation coefficient, -0.53; p < 0.05). Types I and IV tympanoplasty had a higher correlation with postoperative air-bone gap than type III tympanoplasty. The 0.5-KHz frequency had a higher correlation with postoperative air bone gap than other frequencies. CONCLUSION: Measurement of postoperative aeration around the stapes is an effective method for evaluating the importance of middle ear aeration. Aeration around the stapes contributes to better hearing outcome.

A case of multiple abnormalities with eustachian tube obstruction by a protruded internal carotid artery.

We report a case of multiple abnormalities with eustachian tube obstruction by a protruded internal carotid artery. A 10-year-old male presented with multiple abnormalities including anomalous pinna, poor eyesight, facial palsy, moderate conductive deafness, and otitis media with effusion. Temporal bone computed tomography demonstrated obstruction of the right eustachian tube by a protruded internal carotid artery. Insertion of a tympanostomy tube did not improve his hearing, indicating a possible ossicular chain anomaly. Although tympanoplasty is necessary to improve the patients' hearing, the poor drainage function makes this difficult. Knowledge of this vascular anomaly is important when performing myringotomy or tympanoplasty.