Most Congenital cholesteatoma (CC) which is associated with well-pneumatized temporal bone and good Eustachian tube function occurs in the mesotympanum. Therefore, CC is considered to be a good indication for transcanal endoscopic ear surgery (TEES) with preservation of the normal anatomy (i.e., the mastoid air cells). In this report, we present our CC cases treated by TEES and microscopic ear surgery (MES), and describe our therapeutic strategy for CC. Totally, we studied 29 ears of 28 patients of 19 male and 9 female patients who were operated on in our department. The age at operation raged from 1 to 13 years (median age, 3 years). The average follow-up period was 40.6 months. Of the 29 ears, 23 were operated on by TEES, and four of 23 ears underwent staged surgery. TEES group included 14 ears of stage Ia, one ear of stage Ib, three ears of stage Ic and five ears of stage II. Type I tympanoplasty for 18 ears, type III for three ears and type IV for two ears were performed. On the other hand, six ears were operated on by MES, of which all of six ears classified to stage II. All six ears underwent staged surgery (canal wall-up tympanoplasty). The postoperative hearing evaluation showed that 21 of 22 ears in TESS group were “success” by judging from the Criteria by Japan Otological Society 2010. In MES group, 5 of 6 ears showed “success.” No major complications occurred during intraoperative period. No recurrence developed among either the TEES or the MES group. Our experience suggested that CCs of stage I should be operated on by TEES and stage II CCs without extension into mastoid can also be operated using TEES. Introduction of TESS into CC will be possible to perform effective, safe and minimally invasive tympanoplasty.

In the electric-acoustic stimulation (EAS), hearing preservation, especially in the low-frequency, is the most important issue. To achieve this issue, several methods have been applied including a minimally invasive surgery using soft cochlear implant (CI) electrodes and corticosteroid treatment. However, hearing deterioration after electrode insertion is inevitable on average.

We have been investigating the protective effects of insulin-like growth factor 1 (IGF1) on hair cell death. This effect was observed in the drug- or noise-induced or ischemic hearing loss. Moreover, a local treatment of IGF1 using hydrogel showed positive effects on hearing recovery in our clinical trial on the idiopathic sudden sensorineural hearing loss.

Based on these studies, we assume that IGF1 may be effective on the hearing preservation during CI electrode insertion because of several reasons. 1. One of the causes of hearing impairment after CI electrode insertion is the hair cell loss. 2. Local treatment to the cochlea is easy during CI surgery through cochleostomy or the opening of a round window soon after the impairment. 3. IGF1 is able to prevent hair cell loss. 4. More immediate IGF1 treatment showed better outcomes in our clinical trial. In the current paper, we will present preliminary data on the effects of IGF1 on the cochlear structure during CI electrode insertion.

Recurrence of cholesteatoma is mainly caused by poor mucosal regeneration in the middle ear cavity and mastoid cavity. Conventional canal wall up tympanoplasty often results in a lack of mucosal regeneration in the resected area of the mastoid cavity. In particular, mucosal regeneration in a poorly pneumatized mastoid cavity is extremely difficult. If regeneration of the damaged middle ear mucosa were possible in the early postoperative period, it would be possible to prevent re-adhesion of the tympanic membrane and recurrence of adhesive otitis media. Additionally, regeneration of middle ear mucosa would prevent recurrence of cholesteatoma. To overcome these limitations, we developed a novel treatment method combining tympanoplasty and autologous nasal mucosal epithelial cell sheet transplantation for postoperative regeneration of the middle ear mucosa. Tissue-engineered autologous nasal mucosal epithelial cell sheets were fabricated by culturing the harvested cells in an aseptic environment in a good manufacturing practice-compliant cell processing facility. The cultivated cell sheets were transplanted, during tympanoplasty, onto the exposed bony surface of the middle ear cavity where the mucosa had been lost. We performed this procedure on four patients with middle ear cholesteatoma and one patient with adhesive otitis media. All patients showed favorable postoperative course with no adverse events or complications. To our knowledge, this clinical research is the world’s first-in-human study to transplant cultured cells into the human middle ear. This treatment simultaneously preserves the external ear canal morphology, as in standard canal wall up tympanoplasty, and incorporates autologous cell sheet transplantation, which enables prevention of recurrence of cholesteatoma. This study represents a great step forward in the development of a new surgical approach for treating adhesive otitis media and cholesteatoma.

Acute, low-tone, sensorineural hearing loss（ALHL）is generally considered to have a good prognosis; however, it can recur, and some cases progress to Meniere’s disease. Primary treatment of ALHL is important to prevent worsening of hearing loss and onset of Meniere’s disease. We administered Ninjinyoeitou or Goreisan, herbal medications, to patients with intractable ALHL. A 44.4% cure or improvement rate was achieved from among 27 cases administered Ninjinyoeitou. On the other hand, a 20.0% cure or improvement rate was achieved from among 25 cases administered Goreisan. The efficacy of Ninjinyoeitou was significantly better than that of Goreisan. The hearing thresholds at 125Hz and 250Hz in the Ninjinyoeitou group were significantly improved. Ninjinyoeitou has hematopoietic action and is used to treat anemia associated with cancer chemotherapy. We hypothesized that Ninjinyoeitou would improve the impaired blood supply to the stria vascularis and therefore be effective in patients with intractable ALHL.

We describe a patient who presented with otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) and eardrum perforation that occurred after a remission of myeloperoxidase (MPO)-ANCA glomerulonephritis; the patient’s symptoms fluctuated in accordance with the MPO-ANCA levels.

A 74-year-old woman was diagnosed as having MPO-ANCA nephritis. The MPO-ANCA level was as high as 184IU/ml. A steroid was administered for 4 months; the MPO-ANCA level (2.3IU/ml) and the kidney function recovered to normal ranges at the end of steroid administration. However, the patient developed left-sided otorrhea after the cessation of the steroid therapy; perforation of the left eardrum and otorrhea were detected during a visit to an outpatient ear, nose, and throat (ENT) clinic. The patient had not undergone myringotomy previously. The MPO-ANCA level was relatively high (10.6IU/ml). Conservative treatment using antibiotics and ear cleaning was continued for 10 months; however, no significant improvement was observed. Myringoplasty was performed under local anesthesia when otorrhea was no longer observed (MPO-ANCA level: 5.7IU/ml). However, 6 months postoperatively, the MPO-ANCA level increased to 7.1IU/ml, and otorrhea and a small perforation were observed again. After this recurrence, the condition of the middle ear fluctuated in accordance with the MPO-ANCA levels. As the MPO-ANCA level decreased, otorrhea improved. On the contrary, the kidney function remained within normal range from the end of steroid administration to the last follow-up.

This is the first case report describing OMAAV with eardrum perforation, and OMAAV occurred after the remission of MPO-ANCA nephritis.

Facial nerve schwannoma is one of the most common tumors among temporal bone tumors. Its diagnosis and treatment are often difficult. We herein report a case of recurrent facial nerve schwannoma in a 65-year-old man who complained of progressive left mixed hearing loss. He also had left facial nerve paralysis caused by a surgery for a parotid gland tumor that he underwent 15 years prior. Otomicroscopic examination revealed a protruded lesion at the left eardrum posterior border. Temporal bone computed tomography showed a temporal bone tumor with bone destruction in the posterior fossa dura mater region. Based on the clinical course and magnetic resonance (MR) imaging findings, a recurrent facial nerve schwannoma was suggested. Temporal bone tumor resection was performed using transmastoid and posterior cranial approaches. Intraoperative findings showed that the tumor was highly adhered to the cranial dura mater, sigmoid sinus, and jugular bulb. Difficulty in removing the tumor near the jugular foramen resulted in subtotal resection of the tumor. Postoperatively, cyber knife treatment was performed for the residual tumor. The present case suggests that MR imaging is useful for confirming the diagnosis of temporal bone tumors and that radiation therapy is one of the options for treatment of facial nerve schwannoma adhering to the jugular foramen.

We report 2 cases of isolated stapes superstructure fixation. Case 1 presented with bilateral conductive hearing loss similar to otosclerosis. Intraoperative findings showed that the stapes superstructure was fixed to the bony facial canal by a bony bar. Stapes mobility was improved by resection of the ectopic bar. Case 2 presented with unilateral conductive hearing loss. Intraoperative findings indicated that the stapes superstructure was fixed by an elongated pyramidal eminence. Resection of the eminence improved stapes mobility.

Isolated stapes superstructure fixation is a rare entity. According to a literature review, four lesions have been identified; an ossified stapedius tendon, an elongated pyramidal eminence, fixation to the facial canal, and fixation to the promontory. These fixations can be either congenital or acquired. In case 1, an ectopic bony bar, of non-inflammatory origin, between the facial canal and stapes superstructure resulted in progressive hearing loss. In case 2, overdevelopment of the anlage of the pyramidal eminence caused congenital fixation of the stapes superstructure.

We evaluated the effectiveness of transcanal endoscopic ear surgery (TEES) for congenital cholesteatoma (CC), based on residual rate and improvement in hearing ability. The stage of CC was determined using the Japan Otological Society staging system for middle ear cholesteatoma (2016). The TEES is especially effective for Stage I CC. Endoscopy-assisted Microscopic Ear Surgery is also effective for Stage II CC with mastoidectomy. Cholesteatoma matrix adhered to one region in 11 ears; in 10 ears (92%) it was adherent to tensor tympani and its surroundings; this region is supposed to be the commonest region of origin for CC. With TEES, tensor tympani lesions can be visualized clearly, although the tensor tympani is a blind spot under a microscope. In Stage I CC localized in the tympanic cavity, no residual cholesteatoma was observed (0%) and the hearing outcomes were excellent (100% success rate of hearing). On the other hand, in four ears with stapedial lesions, three had residual CC on the stapes footplate (recurrence rate: 14%). There was no residual CC except on the stapes footplate. After a two-handed operation, tracking the matrix by one hand and detaching it from the footplate by the other hand, no residual CC was found on the stapes footplate. Therefore, two-handed operation under microscope or three hands surgeries with an endoscope are recommended for lesions of the stapes footplate.

Pinholing of the tympanic membrane occurred in 5 out of 16 ears, which temporarily detached the tympanic membrane from the malleus handle after TEES. In all cases, the tympanic membrane pinhole perforation closed naturally within three months. It has been suggested that the area surrounding the malleus handle is a tympanic membrane regeneration center. Therefore, membrane regeneration was temporarily impaired by the detachment of the tympanic membrane from the malleus handle. This postoperative complication of tympanic membrane perforation is regarded as minor and considered acceptable.