OBJECTIVES: To clarify the actual state of pediatric otitis media (OM) and to examine several points of issue in the guideline of OM and OM.
SUBJECT: 3,500 children under the age of 15 with OM who visited my clinic from September 2005 to May 2010 and 1,624 first grade school children in 2012 and 2013.
METHODS: Examination of clinical course in 3,500 children with OM and survey by questionnaire taken from their parents of 1,624 first grade school children.
RESULTS: Ten out of 20 children suffered from OM at least one time by their first grade, and three out of 10 children had only one episode of OM which was not repeated. Three out of 10 children had severe OM. Nearly 95% of children with OM were free of disease by the first grade.
CONCLUSION: The clinical course obtained by this report was similar to those reported since the 1970's. The author pointed out several problems concerning OM and OM guideline.

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Staphylococcus aureus causes 50% of chronic otitis media (COM) cases. We postulated that population structure of S. aureus is based on geographical location or diseases such as impetigo and atopic dermatitis. We studied whether a lineage causing COM reflected a population structure of S. aureus in the research area or specific to the disease. Between Jan. 2010 and July 2012, 35 strains of S. aureus, including 11 MRSA isolates were collected from patients with COM following tympanoplasty. Phylogenetic analysis was performed using multilocus sequence typing (MLST). In COM, the most prevalent clonal complex (CC; lineages sharing: at least 5 of 7 house-keeping genes in MLST) was CC5 (23%), followed by CC8 (17%). Prevalence of CC5, which included MRSA isolates, in COM was significantly greater than in healthy volunteers (P<0.001, Chi-square for independence test). Prevalence of CC59, CC75, and CC121, which are minor strains in nasal cavities, was also greater in COM patients. In contrast, prevalence of CC188 and CC508, which are major strains in nasal cavities, were lower in COM patients. This study demonstrated that S. aureus types were disease specific and genotypes from COM patients did not correspond to those of nares from healthy volunteers.

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Recently an increasing number of studies concerning of usefulness of preoperative diffusion-weighted magnetic resonance imaging for detection of primary or recurrent cholesteatoma have been reported. In order to assess the usefulness of diffusion-weighted imaging (DWI), we conducted the pre-operative periodically rotated overlapping parallel lines with enhanced reconstruction (PROPELLER) -DWI using 1.5T or 3TMR units for 63 patients with chronic otitis media. Patients suspected of having or requiring re-operation of middle ear cholesteatoma who underwent preoperative MRI and CT for cholesteatoma surgery between March 2011 and September 2012 were investigated.
There were 34 males and 29 females, with an average age of 48 (range: 6-88) years. 39 cases were receiving their first surgery and the remaining 24 cases were postoperative.
Final diagnosis was surgically confirmed.
We assessed the sensitivity, specificity, and positive and negative predictive value of preoperative DWI and CT for detecting cholesteatoma.
Overall (n=63), the sensitivity, specificity, and positive and negative predictive value of DWI were 95.3%, 75%, 89.1% and 88.2%, those of CT 90.6%, 70%, 86.7% and 77.8% respectively.
In the 39 cases receiving their first surgery, the sensitivity, specificity, and positive and negative predictive value of DWI were 93.9%, 50%, 91.1% and 60% and those of CT 96.9%, 33.3%, 88.8% and 66.6% respectively. In the 24 postoperative cases, the sensitivity, specificity, and positive and negative predictive value of DWI were 100%, 85.7%, 83.3% and 100%, and those of CT 70%, 85.7%, 77.7% and 80% respectively.
One of these cases was negative by preoperative diagnosis (DWI) but positive by preoperative CT. A cholesteatoma was confirmed by surgery but it was found to be desiccated.
Both CT and MRI showed high sensitivity for preoperative evaluation of chronic otitis media with cholesteatoma.
The drawback of DWI is that a false-negative result can be obtained if a cholesteatoma is present but desiccated.
If CT findings provide information about invasion to mastoid cells or the auditory ossicles, the possibility of chronic otitis media with cholesteatoma should be considered even if DWI is negative. DWI was thought to be an useful reference for follow-up of postoperative cases with cholesteatoma in which bone shapes showed deformities.

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We encountered 2 cases of bilateral acute otitis media presenting with moderate mixed hearing loss in bilateral ears. The first case was a 37-year-old man who had a cold and bilateral ear fullness. He was diagnosed with acute otitis media, with mixed hearing loss in the right and left ears (65.0dB and 52.5dB, respectively). After myringotomy, antibiotic administration and intravenous drip infusion of steroids, the hearing loss in the both ears improved to 28.8dB and 30.0dB, respectively. The second case was a 48-year-old woman. She presented with fever and bilateral ear fullness, and further had mixed hearing loss in the right and left ears (70.0dB and 55.0dB, respectively). After myringotomy, antibiotic administration and intravenous drip infusion of steroids, the hearing loss improved to 21.3 dB and 15.0 dB in the right and left ears, respectively.
The incidence of inner ear disorder caused by acute otitis media ranges from 1.5% to 1.6%, with 9.6% of the cases showing a bilateral hearing loss. In Japan, 17 cases of acute otitis media have been reported in patients aged 30-50 years. It is believed that the inflammation spreads to the inner ear through the round and oval window, and via microfissures in the bone around the inner ear window. No cases of reiterate sensorineural hearing loss have been reported. We supposed that this is due to the thickness of the window membrane as a result of repeated chronic inflammation.

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We experienced rare case of temporal bone spontaneous leakage of cerebrospinal fluid (CSF) without prior history.
A 55-year-old male patient with hearing loss, headache and vertigo, was diagnosed of otitis media with effusion and received repeated myringotomy. The patient visited our hospital because of no improvement by previous treatments.
Computed tomography (CT) scan images of the temporal bone showed a bony defect in floor of the middle cranial fossa. In the same region, enhanced MRI showed a small soft tissue density which was thought as brain herniation. We performed surgical treatment with transmastoid approach. Sever CSF leakage was found in the region detected by CT scan image and MRI. And there was also identified a brain herniation without dura matter.
Brain herniation was coagulated by bipolar and the bone defect was repaired by autologous bone pate and temporal fascia.
One and half years after the operation, the CSF leakage was succseefully managed and there were no complaints and recurrence.

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We reported a case of congenital cholesteatoma localized in the mastoid cavity.
A right sided conductive hearing loss was found in a patient during the preschool medical checkup and later the diagnosis was confirmed by computed tomography. The causes of conductive hearing loss were multiple ossicular chain anomalies, such as absence of the incus and the anterior crus of stapes, and displacement of the posterior crus of stapes. We performed tympanoplasty type IVi.
Most cases of congenital cholesteatomas are less likely to be diagnosed as the ossicular chain anomaly, but the present case was showing the ossicular chain anomaly. Because there was no anatomical relation between the location of cholesteatoma and lesion of ossicular chain.

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Usher syndrome (US) is a genetic disorder resulting in sensorineural hearing loss and retinitis pigmentosa (RP). We performed hearing tests, balance tests, and genetic tests for 5 patients with bilateral sensorineural hearing loss of unknown cause complicated by RP. Two patients with severe hearing loss and bilateral vestibular hypofunction in balance tests were considered as US type 1. Two patients presented with congenital moderate sensorineural hearing loss associated with RP were classified as US type 2. One patient showing progressive hearing loss from middle age was considered as US type 3. In genetic tests, MYO7A mutation was identified in 2 patients with US type 1. and this result was consistent with their clinical symptoms. One of these 2 patients had undergone cochlear implant surgery and later diagnosed with RP. As shown in these cases, the first symptom of Usher syndrome appears as impaired hearing, and the symptoms of RP are manifesting later. Therefore, it is necessary to pay attention to the initial symptoms of RP, such as night blindness and visual field constriction, in patients with congenital sensorineural hearing loss. Genetic tests are considered useful in establishing the definitive diagnosis.

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Eosinophlic otitis media (EOM) is well known as an intractable otitis media with eosinophil enriched middle ear effusion, and carries a high risk of sensory neural hearing loss. In the present study, we performed a histological investigation to elucidate the inner ear damage in an animal model of EOM which we newly constructed recently. Guinea pigs were sensitized with ovalbumine (OVA) by intraperitoneal injection, and stimulated with OVA by intratympanic injection to middle ear.
The number of eosinophils infiltrated in the perilymphtic space of the cochlea increased, as the periods of intratympanic injection of OVA to the middle ear became longer. Moreover severe morphological damage of the organ of Corti, spiral ligament, and the stria vascularis was also observed in the 28-day OVA injection animals. These results indicated that eosinophilic inflammation in the inner ear caused profound hearing loss.

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Meniere's disease is a common inner ear disease with an incidence of 15-50 per 100,000 population. Since Meniere's disease is thought to be triggered by an immune insult to inner ear, we examined intra-endolymphatic sac application of large doses of steroids as de novo treatment for intractable Meniere's disease.
The technical details of endolymphatic sac drainage with steroid-instillation are as follows: A simple mastoidectomy was performed, clearly exposing the endolymphatic sac in the area between the sigmoid sinus and the inferior margin of the posterior semicircular canal. If possible, the sac was exposed including the rugose portion. The sac was opened with an L- (right ear) or backward L- (left ear) shaped incision made along the posterior and distal margins of the lateral wall. Then the sac was filled with a mass of 20 mg of prednisolone. While dissolving the mass in the sac, we prepared a bundle of absorbable gelatin films (ca. 4×20×0.7mm×5sheets) with fan- and stick-shaped ends. These films were tied to each other with biochemical adhesive (human thrombin combined with human fibrinogen) at the stick-shaped end. The fan-shaped end was then inserted into the sac and small pieces of absorbable gelatin sponge soaked in a high concentration of dexamethasone (32mg/4ml) were placed inside and outside the sac lumen expanded with the bundle. The sponges containing dexamethasone placed outside the sac were coated with the adhesive so that dexamethasone was slowly delivered into the sac over a long period of time as a natural sustained-release vehicle. The stickshaped end extending out of the sac was fixed to the front edge of the mastoid cavity with the same adhesive so that the incision into the sac was also expanded as long as possible. The mastoid cavity was filled with relatively large pieces of absorbable gelatin sponge dipped in steroid antibiotic solution, after which the wound was closed with skin sutures.

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Antineutrophil cytoplasmic antibody (ANCA) - associated vasculitis is characterized by systemic necrotizing vasculitis. It is known that otitis media with ANCA associated vasculitis (OMAAV) is one of the main etiology of intractable otitis media. The diagnostic criteria of OMAAV has been proposed by 32 patients initially diagnosed by ear symptoms such a hearing loss, otorrhea, ear pain in 2013. To evaluate the validity of this diagnostic criteria, this study consisted of 90 patients with OMAAV or highly suspicious for OMAAV diagnosed and treated by the OMAAV working group containing 10 medical institutions in Japan. Serum PR3- or MPO-ANCA were detected in 67 (74.4%) patients. 89 (98.9%) patients had hearing loss and 83 (93.2%) patients had a rapid progression of hearing loss. Facial nerve paly, hypertrophic pachmeningitis developed in 25 (27.8%), 18 (20.5%) patients during the course of therapy, respectively. After checking the detail of the clinical features of 90 patients, we proposed the revised version of diagnostic criteria of OMAAV.

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