Regenerative therapy for closure of tympanic membrane (TM) perforation using basic fibroblast growth factor and atelocollagen/silicone bilayer was conducted at the outpatient clinic of Ehime University Hospital. In total, 140 cases (45 males and 95 females; mean age, 64.8 years; range, 13-90 years) had undergone a day surgery from July 2009 to August 2011.
The complete closure rate was 83.6% in 3 months after surgery and 67.1% > 1 year after surgery. The complete closure rate according to the original size of the perforation was 71.6% for a small perforation, 68.6% for a mid-sized perforation and 50.0% for a large perforation. Additionally, the mean number of trials until complete closure in 94 cases was 1.38 times.
Closure of TM perforation was significantly harder to conduct regeneration therapy when the entire perforation edge was invisible because of bulging of the external auditory meatus, when eardrum calcification was remarkable, and when the perforation edge was close to the tympanic annulus.
Eighteen of 140 (12.9%) cases that showed aural discharge after surgery were thought to be due to postoperative infection. Postoperative epithelial pearls were found in 7 (5.0%) cases, which was a similar rate to the incidence of postoperative epithelial pearls following conventional myringoplasty. In a case in which atelocollagen soaked with bFGF was depressed into the middle ear cavity, the regenerated eardrum adhered to the middle ear muca and inflamed fibrous tissue was observed in the adhesion area. As the safety of bFGF on the middle ear mucosa has not been established, preparation for regenerative ear drum therapy; thus, it should be used not to flow the middle ear cavity.

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Sonotubometry is used in diagnosis of the patulous Eustachian tube (PET).
When using the apparatus, either one of the following parameters is used as a positive sign for the PET, i.e., the extension of the duration of ET opening upon swallowing and the decrease in the test tone sound pressure level applied to the nostril. However, these parameters are known to include considerable rates of false-positive and cut-off values for these parameters in diagnosing the PET are not yet standardized. In this report, we performed sonotubometry in 102 ears of 51 cases with unilateral sensorineural hearing loss (SNHL) without PET, in order to evaluate the specificity of sonotubometry in the diagnosis of the PET.
Either ‘the extension of the duration of ET opening upon swallowing’ or ‘the decrease in the test tone sound pressure level applied to the nostril’ was found in 66.7% of the SNHL cases without PET, showing the low specificity of these parameters in the diagnosis of the PET. On the other hand, one distinct pattern of sonotubometry recording (plateau type), representing continuous opening of the ET after swallowing, was never found in SNHL cases without PET, and therefore thought to be specific for PET.

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Objective: To investigate the benefits and problems of tympanoplasty in elderly patients over 70 years of age.
Patients and Methods: Among 1014 patients who underwent tympanoplasty for chronic otitis media from 2006 to 2011, elderly patients over 70 years of age were eligible for inclusion. Clinical characteristics including clinical symptoms, tympanosclerosis, preoperative severe complications, postoperative ear condition, and hearing outcomes were retrospectively investigated. The mean bone conduction hearing threshold at 500-, 1-, and 2-kHz frequencies was also estimated as postoperative inner ear damage.
Results: Ninety-seven ears of 83 patients were included. Otorrhea and hearing loss were major symptoms and vertigo was specific in chronic otitis media with cholesteatoma. Eighteen ears had obvious findings of tympanosclerosis (18 / 97 ears=18.6%). In chronic otitis media without cholesteatoma (52 ears / 47 patients), no preoperative complications were noted. In chronic otitis media with cholesteatoma (42 ears / 33 patients), we noted preoperative severe complications including labyrinthine fistula (7 / 97 ears=7.22%), widely exposed dura (1 / 97 ears=1.03%), and facial palsy (1 / 97 ears=1.03%). Most cases became carefree ear (94 / 97 ears=96.9%). The postoperative inner ear damage including statistically significantly increased bone conduction hearing thresholds and persistent vertigo was not found.
Conclusions: Tympanoplasty in elderly patients over 70 years seems to be safe and effective to improve their quality of life.

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A 68-year-old woman who had bilateral mixed hearing loss with right cholesteatoma and left tympanosclerosis underwent a round window vibroplasty of middle ear implant, Vibrant Soundbridge (VSB) and the results were compared with hearing aid. She underwent tymapanoplasties for the right cholesteatoma at her age of 55, 66 and 67 years. She had started wearing hearing aid on her left ear since age 57. The hearing aid evaluation test revealed that the effect of hearing aid has not been satisfactry. Maximum speech discrimination score in the right was much poor compared with that in the left ear. We performed the round window vibroplasty with VSB for her left ear and tried to use the RW-couple because of otosclerosis of round window.
Speech discrimination scores in quiet and noisy environment, functional gain, and questionnaire with APHAB test (except for aversiveness Scale) in the VSB were better than those in the conventional hearing aid after VSB fitting in the audio processor for 1 month.

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A thirty-year-old female with Bezold's abscess as well as untreated diabetes presented with a right otalgia and otorrhea at the end of December 2011. However, she did not seek medical consult because she thought she couldn't pay the medical fees. She had been an unemployed person and she had no health insurance or welfare for over 10 years. Three months later, she developed pain and a swelling in the right neck and fever. On ENT examinations, her right ear canal was stenotic and the audiogram showed a mixed hearing loss in the right ear. CT scan showed a right neck abscess and it seemed to develop through a bone defect of the posterior-inferior part of the mastoid tip, which was compatible with Bezold's abscess. A bone defect of the sigmoid sinus was also observed. She had no symptoms of acute mastoiditis like otorrhea, standing auricle and otalgia. We diagnosed Bezold's abscess caused by the masked mastoiditis. Further, she was diagnosed to have untreated diabetes (HbA1c was over10%).
After admission, she got intravenous antibiotic therapy and treatment for diabetes in collaboration with the internal medicine specialists. The next day after admission, she underwent mastoidectomy and abscess drainage under the condition of controlling high blood glucose level by insulin. After the operation, her neck pain became alleviated and her hearing level got better.
Six months after the surgery, there has been no sign of recurrence. However, her diabetes has not been well treated, because she missed periodical examination.
With the advent of advanced antibiotic therapy, the complications of otitis media such as Bezold's abscess had been rare. However, the abscess can be made and get larger without any sign in such a case like masked mastoiditis. Therefore, we should include this disorder in a differential diagnosis.

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We encountered a rare case of herniation of temporomandibular joint contents into the external auditory canal, possibly attributable to a bone defect of the canal associated with the foramen of Huschke. The patient was a 64-year-old woman who visited our hospital with a complaint of left otalgia persisting for 1 week. She was diagnosed as having pain due to temporomandibular joint disorder and external otitis. Approximately 10 days later, she revisited our hospital, complaining that the otalgia had worsened after eating dried squid. In the left external auditory canal, there was a hemispherical swelling that retracted with her mouth open and protruded with her mouth closed. Computed tomography revealed a bone defect of the external auditory canal, through which a soft-tissue density mass protruded into the external auditory canal. A biopsy ruled out the presence of a tumor. After initiation of splint therapy for temporomandibular joint disorder, the mass gradually became less noticeable.

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We reported a case of cholesterol granuloma due to Eustachian tube malformation.
There has been no similar case report, therefore this is the first report of the case.
A 50-year-old woman complained of intermittent otorrhea for 40 years. Pure tone audiometry revealed bilateral mixed hearing loss. CT showed soft tissue density from the mastoid cavity to tympanic cavity with normal ossicular chain and expansive bone erosion of Eustachian tube bilaterally. In order to comfirmation of the diagnosis, control of otorrhea and improvement of hearing, we performed tympanoplasty of the left ear and found granulation tissue occupied from the mastoid cavity to tympanic cavity, and there found collection of cholesterol crystal in the expanded Eustachian tube. Histological examination showed the typical features of cholesterol granuloma, with needle-like cholesterol clefts, hemosiderin deposits, and erythrocytes in non-specific granulation tissue.
Re-evaluation of the CT images with three-dimensional reconstruction to define the etiology showed that osteophyte arose from below and between bony portion and cartilaginous portion of Eustachian tube bilaterally. The osteophyte occurred from sphenoid bone. Therefore, we considered that the osteophyte obstructed Eustachian tube and cholesterol granuloma has developed.
At the revision surgery was performed because granulation developed again, fiberscopic finding in Eustachian tube revealed complete occlusion, and the tubal inflation from the pharyngeal orifice of Eustachian tube was impossible.
As a conclusion, we considered that cholesterol granuloma was produced by Eustachian tube obstruction due to the osteophyte.
The Eustachian tube was hardly evaluated with conventional CT scan, but three dimentional CT enable us to image along the Eustachian tube. Further investigation may show that Eustachian tube obstruction is caused not only by functional disorders, but also by organic disorders.

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The 3243 A to G mutation of the mitochondrial DNA (m.3243A>G) is responsible for mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS), and maternally inherited diabetes and deafness (MIDD). However, this mutation cannot be necessarily shown as typical phenotypes, and therefore it can cause isolated diabetes and hearing loss (HL).
A 48-year-old female, who had realized bilateral HL since her childhood, visited our department at the age of 18, received left tympanoplasty and was diagnosed as having left ossicular malformation. She returned to our department at the age of 33 because of the deterioration of her left HL. In her family history, maternal grandfather had HL, mother had diabetes, and maternal aunt had HL and diabetes, not suggesting of maternally inherited hereditary HL. After the second surgery, her left HL recovered. At the age of 44, m.3243A>G was detected in the patient as a result of an extensive screening for 29 major mitochondrial DNA mutation. At the same time, she realized the deterioration of her left HL, and the audiogram showed the increase of air-bone gaps at the lower frequencies tones with the slight elevation of bone conduction thresholds at the higher frequencies. She received an explanation that m.3243A>G can cause both the progression of HL and diabetes. She made a decision on using a hearing aid but not receiving third surgery. The patient was diagnosed as having early stage diabetes and started to receive dietary and exercise therapy. The m.3243A>G was a common mutation among the general population and can cause atypical features. Therefore, we should pay an attention for the existence of the mutation in a case whose family members have hearing loss and/or diabetes.

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Prostaglandin E1 (PGE1) is frequently used for the clinical treatment of acute sensorineural hearing loss. However, the mechanisms underlying the effects of PGE1 in the inner ear have not yet been elucidated. The physiological effects of PGE1 are mediated by four distinct E-prostanoidreceptor (EP) subtypes namely EP1, EP2, EP3, and EP4. Signal transduction pathway of EP subtypes has been studied. EP1 couples to Gq protein resulting in the elevation of the free Ca⁺ concentration. EP2 and EP4 receptors couple to Gs and mediate increases in intracellular cAMP levels. In contrast, EP3 couples to Gi for signaling resulting in decreased cAMP concentration. Furthermore, the ligand binding specificities of mouse prostanoid receptors have been examined. The rank order of affinity of PGE1 for four receptors was EP3≥EP4>EP2>EP1. In the current study, we examined EP subtypes expression in the mouse inner ear using the reverse transcription-polymerase chain reaction (RT-PCR) and immunohistochemistry in order to elucidate the mechanisms underlying the effects of PGE1 in the inner ear.
Male C57BL/6 mice at 8 weeks of age were used for the present studies. The results of RT-PCR demonstrated EP subtypes mRNA expression in the mouse cochleae. Immunohistochemistry for the cochleae demonstrated EP1 expression in the hair cells (HCs) and supporting cells (SCs), EP2 and EP4 expression occurred in the HCs, SCs, spiral ligament, stria vasculis, and spiral ganglion cells. EP3 expression occurred in the HCs, SCs, spiral ligament, and spiral ganglion cells. Immunohistochemistry for the vestibule and semicircular canals demonstrated four EP subtypes expression in the sensory epithelia of the vestibule and semicircular canals.
Previous studies have indicated the involvement of EP4 in the physiopathology of cochleae and the therapeutic capability of EP4 agonists for noise-induced hearing loss. Considering binding specificities of PGE1 to four EP subtypes and localization of EP subtypes in the inner ear, EP2 and EP4 may be involved in therapeutic effects of PGE1 in the cochlea, and the stimulation of EP3 by PGE1 may contradict effects of EP2 and EP4 stimulation by PGE1.

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Understanding the clinical anatomy of the round window membrane (RWM) is important in the era of implantable hearing devices. Cone-beam computed tomography (CBCT) is used for the evaluation of the temporal bone. The quality of the image obtained by CBCT is almost equal to that of high resolution computed tomography. The purpose of this study was to confirm the efficacy of CBCT for the measurement and evaluation of the round window membrane.
Fifty ears from 37 patients that underwent CBCT in Kagoshima City Hospital, which had well pneumatized mesotympanum, were selected. The width of the RWM was measured in an axial view. Angle A and B were defined in order to evaluate the surgical approach to the round window niche (RWN) and RWM. A tangent to the facial canal through the roof of RWN, a line through the floor of RWN and the base of suprameatal spine, and a tangent to the RWM were defined to be lines a, b, and c, respectively. The acute angle between line a and line b was angle A, and that between line b and line c was angle B.
The diameter of the RWM, angle A, and angle B were approximately 1.55 mm, 25.7 degrees, and 31.5 degrees, respectively. A statistical analysis showed that none of these data had any correlation with the age or gender.
These data were consistent with that reported in the literature, in which the distance was measured by histological procedures. The figure showed that angle A indicated the range for the opening the facial recess by a posterior tympanotomy and angle B indicated the range of the direction to the RWM. The data indicated that the direction from which the surgeon could completely identify the RWN and RWM was relatively limited and the RWM could not be observed from an oblique direction. Preoperative CBCT provided the surgeon with useful information for ear surgery, especially for placing a cochlear implant.

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OBJECTIVE: An aberrant internal carotid artery (ICA) in the middle ear is a rare congenital finding. We here report on the case of a successful cochlear implantation (CI) in a patient with an aberrant ICA in the middle ear.
CASE DESCRIPTION: The patient was a girl with hearing loss diagnosed by newborn screening. She had a history of truncusarteriosuscommunis (TAC) as a complex heart disease and repaired TAC at the age of 2months. Preoperative computed tomography (CT) and magnetic resonance imaging (MRI) suggested cochlear nerve hypoplasia in the right ear. In the left ear, therewas no inner ear malformation norcochlear nerve hypoplasia. There was, however, an aberrant ICA running near the promontory and round window in the middle ear. CIin her left ear was performed at 31months. The aberrant ICA in the middle ear wasconfirmedby microscope observation. Theelectrode array was inserted without arterial injury. Intraoperative, acoustic reflex threshold (ART) and electrically evoked auditory brainstem responses (EABR) were satisfactory.
CONCLUSIONS: To the best of our knowledge, this is the first case report of a successful cochlear implantation in a patient with an aberrant ICA in the middle ear.

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In recent years there has been an increase in reports of patients with refractory otitis media due to ANCA associated vasculitis. We have studied the clinical analysis of ANCA associated vasculitis with otitis media which we have experienced recently in our department.
The objects were those who had refractory otitis media and rise in PR3-ANCA or MPO-ANCA was observed during the course of otitis media or both were negative cases those that were strongly suspected ANCA related disease by multiple organ failures.
The age ranged from 55 to 81, 1 ear of 1 male and 15 ear of 8 females. Seven patients had affected bilateral ears and 2 had unilateral. Diagnosis at the first visit was otitis media with effusion in all cases, and showed an increase in bone conduction hearing threshold after 2.9 months average from the onset. Six cases of 10 ear could be evaluated their hearing after treatment. Six ears had improved remarkably, but 1 ear was only mild improvement and other 3 ears became deaf. For the 3 cases of 4 ears which had poor hearing prognosis, 2 cases of 3 ears showed a contrast effect of the cochlea or the internal auditory canal dura mater on MRI. In addition, urine occult blood was positive in 3 cases. On the other hand, the cases of hearing improvement had not shown these findings. From the above it have been suggested that urine occult blood and inner ear MRI findings will be hearing prognostic factors.

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Vasculitis is defined as the infiltration of inflammatory cells and necrosis of the vessel wall. Vasculitis related to anti-neutrophil cytoplasm antibody (ANCA) is called “ANCA-associated vasculitis (AAV)”, which is consisted of microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) (formerly Wegener's granulomatosis), and Churg-Strauss syndrome. It is clinically important that AAV is a life-threatening and /or debilitating disease and also serious organ dysfunction have been associated not only with vasculitis but also with treatment for vasculitis. In addition, the diagnosis will be experienced a variety of difficult problems. For this reason, the process of exclusion diagnosis is important, such as for infectious diseases, malignant tumors, and rheumatic diseases. As for AAV is systemic disease, it is very important to cooperate with doctors from many clinical fields.

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We report 2 cases of Otitis Media with ANCA-Associated Vasculitis (OMAAV). Case 1 was a 36-year-old man who presented bilateral otorrhea and vertigo with serum PR3-ANCA positive. He had bilateral mixed hearing loss. Ear symptoms were reduced by the treatment with prednisolone and cyclophosphamide. However, pachymeningitis was complicated subsequentlyand relieved by treatment. Case 2 was a 64-year-old woman who presented bilateral otorrhea and bilateral hearing loss without serum ANCA positive. She had bilateral mixed hearing loss. Ear symptoms were reduced by the treatment with prednisolone and cyclophosphamide. However, pachymeningitis was complicated subsequently, and she had a definitediagnosisof granulomatosis with polyangitisby open biopsy of the pachymeningitis. Pachymeningitis relieved by treatment. In OMAAV, pachymeningitis and neuropathy were easy to be complicated than an ANCA associated vasculitis without otitis media.

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Antineutrophil cytoplasmic antibody (ANCA) - associated vasculitis (AAV) is characterized by systemic necrotizing vasculitis. AAV comprise Granulomatosis with polyangitis (GPA), microscopic polyangitis (MPA) defined as necrotizing vasculitis of small vessels, including its renal limited subset designated as idiopathic necrotizing crescentic glomerulonephritis (iNCGN) and Churg-Strauss syndrome. The elevation of proteinase-ANCA (PRO-ANCA) and / or myeloperoxidase - ANCA (MPO-ANCA) titers are one of the criteria for diagnosis of ANCA associated vasculitis. AAV commonly manifests as a rapidly progressive glomerular nephritis (RPGN), with necrotizing glomerular tufts, alveolar hemorrhage, or interstitial pneumonia. The clinical features of PR3- positive and MPO-ANCA positive cases which diagnosed by the hearing loss as initial symptoms are presented.
Eight cases (six females, two males; age from 54 to 73 years; six MPO (myeloperoxidase)-ANCA positive, two PR3 (proteinase 3)-ANCA positive cases) were included in this study. Progressive hearing loss (100%) and hemi/bilateral facial palsy (62.5%) were present in the patients before diagnosis. The characteristic features of hearing were as follows: 1) Rapid hearing loss within less than 2 months 2) completely deaf could not be recovered after immunosuppression therapy 3) hearing levels better than 95 dB were improved with good speech discrimination even at the high frequency.
This study showed the diagnosis of localized ANCA associated vasculitis and the effectiveness of immunosuppressive therapy for hearing loss at an early stage. The immunosuppression therapy would be necessary for hearing disturbance as soon as detecting the elevation of PR3-ANCA/MPO-ANCA titer.

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Granulomatosis with polyangitis (GPA) is difficult to diagnose in an early stage and when limited to the head and neck legion. Although PR3-ANCA is a useful biomarker for diagnosis, a negative value could not neglect the disease. Recently, it has been shown that a capture technique for analysis of ANCA can detect some ELISA and IIF negative GPA patients. In the present study, we examined the usefulness of capture ELISA for the diagnosis of GPA. We also described 2 cases of otits media without ANCA elevation, but strongly suspected GPA from the clinical course.
Case 1 is 75-year-old man who complained of right otorrhea and otalgia. Treatment for otitis media with systemic antibiotics was not effective and mastoidectomy was then performed. Histological examinations of granulomatous tissue in the mastoid showed non-specific granulomatous inflammation. One month after operation, the patient developed paralysis of the abducens nerve (VI), glossopharyngeal (IX), hypoglossal (XII) nerves on the right side and bilatelar hearing loss. We clinically diagnosed the case as a limited form (E alone) of probable GPA with the middle ear as the primary site.
Case 2 is 63 year-old female who complained of bilateral hearing loss and otorrhea. Treatment with systemic antibiotics was not effective. The patient developed left facial palsy. An ear CT showed that both upper tympanic cavity filled with soft tissue density. MRI revealed the presence of meningeal thickening. We clinically diagnosed the case as a limited form (E alone) of probable GPA.
Plasma samples from 9 patients who receive maintenance drug therapy in out-patient clinic were tested for the presence of PR3-ANCA with two detection methods (direct-ELISA and capture ELISA). One of 9 patients (11.1%) was PR3-ANCA positive in direct-ELISA, while 3 of 9 patients (33.3%) was positive in capture ELISA. This finding suggested that capture ELISA technique might become a useful test for the diagnosis of GPA.

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It is known that chronic otitis media due to ANCA (antineutrophil cytoplasmic antibody)-associated vasculitis. This study consisted of 32 patients with otitis media with ANCA-associated vasculitis (OMAAV). All patients complained initially ear symptoms such as hearing loss, ear pain and/or otorrhea, which were not improved by treatments with antibiotics and tympanic tube. At the initial visit, granulomatous lesions in tympanic cavity and/or mastoid cavity were seen in all patients. Serum PR3- or MPO-ANCAs were detected in 25 (78%) patients. Thirty (94%) patients had rapid progression of sensory hearing loss. Facial nerve palsy, hypertrophic pachymeningitis and lung/kidney lesions developed in 14 (44%), 8 (25%) and 10 (31%) patients, respectively. Two (6%) patients died because of subarachnoid hemorrhage due to vasculitis of basial artery. We proposed the diagnostic criteria of OMAAV from these findings.

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Otitis media associated with ANCA vasculitis (OMAAV) is an intractable otitis media and difficult to diagnose. However, ANCA vasculitis is life threatening and early intervention is needed. There are several intractable otitis media which should be differentiated from OMAAV; eosinophilic otitis media, cholesterol granuloma, otitis media tuberculosa and so on. Here we propose the algorithm of differential diagnosis for OMAAV and the diagnostic tools such as serological test, cytology and radiological imaging.

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