The two major purposes of tympanoplasty are the removal of pathological middle ear tissues and ossicular chain reconstruction (ossiculoplasty) to improve hearing. We summed up the cases of primary acquired pars flaccida cholesteatoma experienced in this hospital, namely the extent of choleastoma observed during tympanoplasty, post-surgical hearing outcomes and recurrence rate. 120 cases of primary acquired pars flaccida cholesteatoma underwent tympanoplasty in Kurashiki Central Hospital from October 2004 to June 2011. Our basic procedure was to perform tympanoplasty with postauricular incision, canal wall up technique (CWU), and primary ossiculoplasty. Ossiculoplasty type III with columella (IIIc) was performed in 73.3% of all cases. The patients were followed-up for a median period of 61 months (range, 13 to 95 months). Subjects were classified according to the extent of cholesteatoma based on the staging criteria for middle ear cholesteatoma 2010 Japan. The total success rate of postoperative hearing outcomes was 91.1%. The postoperative hearing outcomes were influenced significantly by the extent of stapedial abnormality. The recurrence rate of cholesteatoma was 18.3%. Recently, sufficient scutumplasty tended to decrease the retraction pocket formation after tympanoplasty. In general, the canal wall up technique resulted in higher rates of residual cholesteatoma and retraction pocket compared with the canal wall down technique. Postoperatively, regular follow-up for at least 10 years is essential to identify the formation of cholesteatoma recurrence.

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We retrospectively reviewed 12 cases of revision stapes surgery for otosclerosis. Clinical history, hearing level, previous surgical treatment, intraoperative findings in the revision surgery and postoperative clinical course were noted. The pathological enlargement of the wire loop in 2 cases, necrosis of the long process of incus in 8 cases and bony closure of the fenestrated footplate in 4 cases were observed intraoperatively. In cases with necrosis of the incus, medial side of the long process was the most common part of the erosion. In 10 of 12 cases, successful hearing results were obtained after the revision surgery. In the primary surgery, adequate choice and proper adaptation of the artificial ossicle are necessary. To avoid excessive pressure of the piston loop to the incus, especially to the medial side of the long process is the most important point for preventing the incus necrosis. Furthermore preservation of mucosa around the incus was also recommended. Hearing result after the revision surgery in our series had favorable prognosis, however, the adequate select of the operative procedure for each pathogenesis is necessary.

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We performed blind sac closure on 11 ears. The etiology, purpose, preoperative hearing, operation time and postoperative course were evaluated. The most frequent etiology (8 ears) was the post-operative cavity problem after cholesteatoma surgery, and the most frequent purpose of surgery was prevention of recurrent cholesteatoma (7 ears). The preoperative hearing was severe (2 ears) to profound (8 ears) hearing loss, with one case whose hearing was not determined due to mental retardation accompanied by pervasive developmental disorder. Postoperatively, all ears were dry, and there was no complaint about the hearing loss on the operated ear. Blind sac closure surgery may be indicated for the ears with refractory infection and/or cholesteatoma when their hearings are non-usable with no surgical improvement expected. Postoperative hearing must be secured by using the opposite ear, hearing aid or cochlear implantation. This surgery can be performed with cholesteatoma removal or cochlear implantation simultaneously.

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Bone-anchored hearing aids (BAHAs) provide better wearing comfort and sound quality compared to air conduction hearing aids or conventional bone conduction hearing aids in some patients with congenital external auditory canal anomalies or a middle ear postoperative state. However, BAHAs can cause postoperative skin complications, including inflammatory skin reactions, skin overhang and implant extrusion. In the present study, five ears of four patients who underwent BAHA surgery at our hospital or other institutes, but who needed a re-operation for their severe skin complications were enrolled. The follow-up periods between BAHA surgeries and re-operations ranged from nine to 91 months (mean: 62.6±33.5 months). All the ears had grade 3 skin reactions, and four ears showed skin overhang more than one time after the BAHA surgeries. The remaining ear had repetitive grade 2 or 3 skin reactions nine months post-surgery. Subcutaneous tissue resection was performed for two ears, and a combination of infectious skin and subcutaneous tissue resections with split-thickness skin grafting was carried out in the other three ears. The findings of re-operations included granulation tissue around the implant flange and a gap between the implant flange and bone. Up to now, no ears have experienced more than a grade 1 skin reaction following the re-operation. Using a long abutment may therefore represent a treatment option if further surgery is needed.

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Both of advanced cochlear otosclerosis and van der Hoeve syndrome (osteogenesis imperfecta) show similar progressive profound deafness and demineralization of the temporal bone surrounding the cochlear capsule. It has recently been reported that the association with COL1A1 in these two diseases may cause similar demineralization. For those patients, cochlear implantation (CI) is the only promising treatment option. However, such Japanese patients are very rare and only a case study on CI insists of relatively poor speech perception result and complications of facial nerve stimulation or unstable T/C level.
Here we reported a long-term outcome in 12 patients with advanced cochlear otosclerosis and van der Hoeve syndrome. We evaluated the speech performance by using Fukuda VTR before 2003 and using by CI-2004 since 2004. As a result, the mean sentence perception score (91%) of 12 cases was better than that (78%) of CI cases with deafness by other causes. Facial nerve stimulation was elicited in only one of 12 cases. Therefore, we concluded that CI can be considered as a treatment option for deaf patients with advanced cochlear otosclerosis and van der Hoeve syndrome.
Furthermore, according to this conclusion, we proposed the strategy flow chart how to select the hearing aid, stapes surgery or CI for Japanese patients with advanced otosclerosis and van der Hoeve syndrome, based on the speech discrimination score, computed tomography classification and the air-bone gap.

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Congenital Cytomegalovirus (CMV) infection and mumps infection are major cause of unilateral sensorineural hearing loss (SNHL), but there were few reports showing the frequency of congenital CMV infection in children with unilateral SNHL. The present study investigated the prevalence of congenital CMV infection diagnosed by detection of CMV DNA in dried umbilical cord specimens from children with unilateral SNHL and other causes of unilateral SNHL.
This study evaluated 88 children with unilateral SNHL who were referred to the Department of Otolaryngology, Shinshu University School of Medicine from May 2008 to April 2012. DNA was extracted from the dried umbilical cords and CMV DNA was examined by quantitative PCR. CMV DNA was identified in 8 (9.1%) of 88 children with unilateral SNHL. Unilateral severe to profound SNHL and mild to moderate SNHL caused by congenital CMV infection were detected in 9.6% (7/73) and 6.7% (1/15), respectively. Genetic deafness mutations could not be found. In conclusion, congenital CMV infection plays a major role as a cause of unilateral SNHL in children.

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Chronic tinnitus, an auditory phantom sensation, is common and often causes sleep complaints and depression. In the present study, we analyzed the data from the National Institute for Longevity Sciences-Longitudinal Study of Aging (NILS-LSA) to evaluate the prevalence of tinnitus and its correlation with sleep complaints and depression in middle aged and elderly persons. A subjective sleep questionnaire survey and CES-D (Center for Epidemiologic Studies Depression) and pure tone audiometry were undertaken among 2,142 subjects aged 40-79 years living in Aichi prefecture, Japan. Persistent or intermittent tinnitus was reported by 416 subjects (38.5%) of the men and 346 subjects (32.6%) of the women. The prevalence of tinnitus was higher for males than females significantly (p=0.005). The multiple logistic regression analysis revealed significant association between tinnitus and difficulty in falling asleep (male: odds ratio (OR) 1.61, 95% confidence interval (95%CI) 1.19-2.18, p=0.002, female: OR 1.75, 95%CI 1.31-2.32, p=0.0001), interrupted sleep (male: OR 1.57, 95%CI 1.17-2.09, p=0.002, female: OR 1.68, 95%CI 1.27-2.32, p=0.0003), having dreams (male: OR 1.81, 95%CI 1.33-2.47, p=0.0002, female: OR 1.56, 95%CI 1.15-2.12, p=0.004), and depression (male: OR 1.90, 95%CI 1.30-2.75, p=0.0007, female: OR 1.79, 95%CI 1.24-2.59, p=0.002) in both sexes, and revealed inversely association with restorative sleep (male: OR 0.74, 95%CI 0.57-0.95, p=0.017, female: OR 0.62, 95%CI 0.47-0.82, p=0.0007). The analysis of general linear model revealed significant reduction in sleep duration in females with tinnitus (p=0.006). However in males, it showed no significant difference in sleep duration. These results suggested that the effect of tinnitus on sleep complaints and depression is severe in middle aged and elderly persons.

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Intracochlear schwannoma (ICS) is classified as one of the intralabyrinthine schwannomas (ILS). ICS is a rare tumor and its symptoms include unilateral hearing loss, tinnitus, and vertigo are similar to those in Ménière's disease. However, treatment for Ménière's disease is ineffective and delays appropriate management. We report two cases of ICS in the patients who were initially diagnosed and treated as Ménière's disease.
Case 1 was a 37-year-old male who presented with pulsatile tinnitus and hearing loss in the right ear. MRI showed no abnormal findings and he had been diagnosed and treated as Ménière's disease. Five years later, his hearing was deteriorated and MRI study was repeated. He was finally diagnosed as ICS after MRI findings. Case 2 was a 59-year-old female who presented with tinnitus and hearing loss in the right ear. She had been diagnosed and treated as Ménière's disease in another hospital, where she was already examined by MRI. Three years later, MRI was repeated again since her hearing was deteriorated. She was finally diagnosed as ICS after MRI findings. In both cases, MRI during the initial visit could not detect any abnormality because their tumors were presumably small. The tumors remained in the labyrinth, and the patients have been followed up by MRI every year. When hearing getting deteriorated, MRI should be performed again even if the previous MRI showed no abnormality. Further, the clinician's eyes should focused on the entire labyrinth, not only on internal auditory canal.

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Meningoencephaloceles are herniations of brain tissues through dehiscences of the skull base. These skull defects are either acquired (otologic infection, trauma, surgery, neoplasia) or spontaneous. We report a case of 12-year old boy who developed CSF otorrhea and repeated meningitis caused by a meningoencephalocele in the right temporal bone seven years after temporal bone fracture by a head injury. Myringotomy was performed under the diagnosis of otitis media with effusion, which caused watery aural discharge of 200ml/day for four days. The temporal bone CT revealed a fracture of the right middle skull base. The MRI showed a large cyst in the right temporal lobe and posterior lobe and herniation of the right temporal lobe in the region of the temporal bone fracture. From the above findings, meningoencephalocele in the right middle skull base was suspected and the closure of cerebrospinal fluid leakage was performed by a neurosurgeon. The meningoencephalocele was amputated and a large defect of dura mater was closed with a pericranial flap and fascia lata. Neither the recurrence of cerebrospinal otorrhea nor the relapse of meningitis has been seen after the operation.

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A 13-year-old male presented with left hearing loss related to a high jugular bulb (HJB). High resolution Computed tomography (CT) of the temporal bone revealed the left HJB eroding into the vestibular aqueduct and posterior semicircular canal. Subsequently, his left hearing loss fluctuated, and dizziness and sense of wobble occurred. The glycerol test was performed and showed a positive result. We speculate that the venous pressure of the HJB could induce the endolymphatic hydrops through a bony defect between the vestibular aqueduct and HJB. Oral steroid and oral isosorbide and glycerol infusion were effective.

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Granulomatosis with polyangiitis (GPA) is characterized by granulomatous inflammation of the upper and lower respiratory tract and necrotizing vasculitis affecting small- to medium-sized vessels. By contrast, hypertrophic pachymeningitis causes a fibrosing inflammatory thickening of the dura mater that induces headache and multiple cranial nerve dysfunction, and is attributable mainly to antineutrophil cytoplasmic antibody (ANCA)-related systemic vasculitis or other collagen diseases. We reported a case of GPA with hypertrophic pachymeningitis, where the patient's initial symptoms were vertigo and progressive sensorineural hearing loss. Neither the detection of ANCA nor histopathological examination provided a conclusive and definitive diagnosis, and a long-term follow-up was needed to establish the diagnosis. The administration of prednisolone and cyclophosphamide improved the pulmonary lesions but not sensorineural hearing loss, which followed a prolonged course. It should be noted that serological and histopathological examinations do not necessarily indicate a diagnosis of GPA, especially in less severe disease or in the early stage.

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The Eustachian tube is crucial for middle ear pathophysiology, but endoscopic approaches to it have not been performed sufficiently. In this study, the sialendoscopy was used for examination of the eustachian tube pathology. The endoscopy might make a breakthrough in diagnosis and treatment of the tubal disorders.

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We studied application of otoendoscope in perilymphatic fistula (PLF). In order to diagnose PLF, we tried to find the existence of fistula and/or leakage of perilymph. The small otoendoscope can detect both small fistula and at least 5-10 μl. In order to observe the oval window, the otoendoscope was preferred than microscope because of saving bony wall. We also described influence of heat production between diameter and light gain of otoendoscope.

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Application of the endoscopic ear surgery for middle ear congenital cholesteatoma was discussed. The most valuable merit of the endoscopic ear surgery was to have a wide and clear intraoperative view field. On the other hand, the endoscopic surgery forced single-hand procedures and limited manipulation field on the operator.
In about 60% of the cases with middle ear congenital cholesteatoma, location of the lesion was limited to the meso- and hypotympanum. Such cases were good candidates for the endoscopic middle ear surgery because the rate of residual cholesteatoma might be reduced with the prominent ability of the endoscopic procedure. At the present time, however, cases with extension of the cholestratoma to the mastoid cavity should be treated with the otomicrosugery by retroauricular approach.

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Endoscopic surgery is particularly suitable for small fenestra stapedectomy (stapedotomy). Endoscopic stapes surgery can even be done in patients with a curved and narrow external auditory canal. Endoscopic surgery is also suitable for education, because the surgical anatomy can be understood easily and because both the surgeon and assistants can observe the procedure on the same monitor. On the other hands, it should be performed only by experienced surgeons, because one-handed manipulation is required and stereoscopic vision is not available.

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In this review, we evaluate recent studies that have used next-generation sequencing (NGS) for the genetic diagnosis of hearing loss. Hereditary hearing loss is genetically heterogeneous and caused by a large number of deafness genes. Because of the extreme genetic heterogeneity, genetic diagnosis has been applied only to a subset of patients. NGS can perform parallel sequencing of billions of nucleotides at low cost and high speed, which makes it ideally suited for the comprehensive genetic testing of hereditary hearing loss. We are developing an original protocol based on NGS for the targeted genomic capture of all known deafness genes with the aim of establishing an efficient genetic test for deafness.

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