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Objective: An intact cochlear nerve is necessary for successful cochlear implantation (CI). Although the promontory stimulation test (PST) using a promontory stimulator (PS) and a transtympanic needle electrode is invasive, it is still commonly used to verify cochlear nerve function. PSs are currently unavailable because they are no longer manufactured; however, considering that PST continues to be beneficial in certain situations, alternative equipment is needed. The PNS-7000® (PNS) was developed as a neurologic instrument for stimulating the peripheral nerves. This study investigated the usefulness of the ear canal stimulation test (ECST) using PNS with a silver ball ear canal electrode, which is a new noninvasive alternative technique to the PST., Methods: ECST was performed from November 2013 to December 2018 using PS and PNS for patients with severe to profound sensorineural hearing loss. The electrical threshold, most comfortable loudness level, uncomfortable loudness level, dynamic range, and gap detection were measured in the ECST. The results of these measured PNS items were compared with PS., Results: ECST was performed in 61 ears of 35 patients (age, 59.9 ± 20.1 years) using PS and PNS. The sound sensation was elicited in 51 (83.6%) and 52 (85.2%) ears with PS and PNS, respectively. All items excluding GAP were measured in 46 (75%) and 43 (70%) ears at 50 and 100 Hz, respectively. GAP was measured in 33 ears by the ascending and descending methods using PS and PNS. Spearman's rank-order correlation coefficient revealed a significant positive linear correlation between the PS and PNS results in all measurements. No significant difference was found between the PS and PNS thresholds in all measured items., Conclusions: PNS is a useful instrument for performing ECST as a new alternative to PS. ECST using a silver ball electrode is a less invasive and easier test than PST., Competing Interests: Declaration of Competing Interest The authors have no financial conflicts of interest to declare., (Copyright © 2023. Published by Elsevier B.V.)

Objective: In Japan, the 57S and 67S single syllable lists have been used to test speech perception in cochlear implant (CI) users. However, due to advances in implants and processors, these lists have become too simple for CI users. In 2019, the Japan Otological Society created a new list, referred to as the iCI2004. The objective of this study was to compare the performance of these lists., Methods: The Japanese single syllable speech perception tests, iCI2004, 57S, and 67S, were administered to 70 patients with CI users. The effects of single syllable characteristics on the test scores were examined. The type and number of single syllables used in each list were different. Therefore, we compared the scores for normal lists, shared single syllables, and non-shared single syllables., Results: The average test results were 52% for iCI2004, 64% for 57S, and 77% for 67S; 67S performed the best, followed by 57S and iCI2004. The test results were significantly different. In a comparison of shared single syllables, the average scores were 58% for iCI2004 and 63% for 57S (45 pieces). A comparison of iCI2004 and 67S (17 pieces) showed that the average scores were 63% for iCI2004 and 75% for 67S. A comparison of 57S and 67S (20 pieces) showed that the average score for 57S was 70% and the average scored for 67S was 77%. No significant differences were detected under all conditions. Based on these results, the type of single syllable adopted affected the result., Conclusion: The data gives no clear indication that the selection of the word table or presentation of sound pressure affects listening to sound for CI users in Japan. Based on the type and number of single syllables used, iCI2004 seems appropriate for evaluation of hearing in patients using CI., Competing Interests: Declaration of Competing Interest The authors have no financial conflicts of interest to declare., (Copyright © 2021. Published by Elsevier B.V.)

Objective: This study was aimed to determine the characteristics of middle ear cholesteatoma and to investigate short-term outcomes regarding the rates of residual and recurrent cholesteatoma and the postoperative hearing results in Japan, via a nationwide survey using staging and classification criteria for middle ear cholesteatoma, as proposed by the Japan Otological Society (JOS)., Methods: The first-round survey was conducted in 2016. The target was patients with middle ear cholesteatoma who were surgically treated in Japan between January and December 2015. Medical information on the patients was anonymized. The questionnaire entries were age, sex, cholesteatoma classification and stage, preoperative hearing level, mastoid development, status of the stapes, and surgical method. There were a total of 1,787 registered patients from 74 facilities from all over Japan. The second survey was conducted in January 2018 and received 1,456 responses from 49 facilities in Japan. Of the 1,456 cases, 1,060 were conducted in the postoperative hearing survey and 1,084 in the residual recurrence survey., Results: The most common cholesteatoma type was pars flaccida cholesteatoma (63.3%), followed by pars tensa cholesteatoma (13.0%), congenital cholesteatoma (12.9%), and cholesteatoma secondary to chronic tensa perforation (5.6%). Cholesteatoma of uncertain origin accounted for 5.0% (90 cases). Stage II was predominant in pars flaccida and pars tensa cholesteatoma, which frequently involves the mastoid, whereas about half of cases of cholesteatoma secondary to chronic tensa perforation and congenital cholesteatoma were classified as stage I. One hundred fifty-two of 1,084 cases (14.0%) had recurrent cholesteatoma, residual cholesteatoma, or both following first surgeries. The postoperative rates of hearing success rate was 63.3%., Conclusion: We were able to clarify not only the current epidemiological status of middle ear cholesteatoma but also the current trends of cholesteatoma surgery in Japan. The development of a staging system by the JOS Committee serving an epidemiological database for international or time-dependent comparison. It is possible to use this staging system with reasonable reliability., Competing Interests: Declaration of Competing Interest All authors declare that there are no conflicts of interest regarding the publication of this paper., (Copyright © 2020. Published by Elsevier B.V.)

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a form of necrotizing vasculitis with few or no immune deposits. It primarily affects small and medium blood vessels. AAV is classified into three categories, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangitis (EGPA), and two major ANCAs, proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA are involved in their pathogenesis. Intractable otitis media frequently occurs in patients with GPA, MPA or EGPA, although all patients show similar clinical features, regardless of the type of AAV. Furthermore, approximately 15% patients with otitis media caused by AAV do not show ANCA positivity, histopathological evidence, or any other AAV-related lesions at the initial visit; therefore, these patients do not fulfill the ordinary diagnostic criteria for systemic AAV. Thus, we first proposed that this condition could be categorized as "otitis media with AAV (OMAAV)". Subsequently, the Japanese Otological Society (JOS) conducted a nationwide survey between December 2013 and February 2014 and identified 297 patients with OMAAV. The survey revealed that OMAAV is a disease that initially occurs in the middle ear and subsequently spreads to other organs such as the lungs and kidneys, with eventual involvement of all body organs. Severe sequelae such as facial palsy, hypertrophic pachymeningitis, complete deafness, and subarachnoid hemorrhage resulting in death can also occur. In this review, we introduce the clinical features, diagnostic criteria, and treatment strategies recommended by JOS for early diagnosis and treatment of OMAAV., Competing Interests: Declaration of Competing Interest All authors require neither any financial support nor other benefits from commercial sources for the work, nor any other financial interests that any of the authors may have, which could create a potential conflict of interest or the appearance of a conflict of interest with regard to the work., (Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.)

Objective: This study was undertaken to determine the characteristics of congenital cholesteatoma (CC) in Japan, via a nationwide survey using staging and classification criteria for middle ear cholesteatoma, as proposed by the Japan Otological Society (JOS)., Methods: A nationwide survey regarding middle ear cholesteatoma treated in 2015 was performed. There were 1787 registrations from 74 facilities, among which, CC accounted for 12.9% (231 cases) of all middle ear cholesteatoma cases. The extent of the disease was classified according to the classification and staging of cholesteatoma proposed by JOS in 2015., Results: The age of the patients ranged from 1 to 55 years (mean, 8.2 years; median, 6 years; 149 ears of males; 82 ears of females). Among these cases, 105 (45.5%) were classified as stage I, 121 (52.4%) as stage II, and 5 (2.1%) as stage III, with no cases in stage IV. In the sub classification of stage I, 35 (33%), 43 (41%) and 27 (26%) ears were classified as stages I-a, I-b and I-c, respectively. Stage I-b (cholesteatoma confined to the posterior half of the tympanic cavity) was more frequent than stage I-a (the anterior half of tympanic cavity). Pre-operative hearing level of air-conduction was 20.5dB for stage I-a, 34.5dB for stage I-b, 30.5dB for stage I-c, 38.6dB for stage II, and 59.0dB for stage III. The rate of missing stapes superstructure increased as the disease progressed. In the same way, the mastoid development lowered as the stage of the disease progressed. There were significant differences in the age at operation, pre-operative hearing level, status of stapes, and major ossiculoplasty, between stage l-a and l-b., Conclusions: The current condition of CC in Japan was been evaluated; in particular, it was observed that the cases limited to the posterior part of tympanic cavity were not rare. The JOS stage classification based on the progression of the disease seemed simple and efficient., (Copyright © 2018. Published by Elsevier B.V.)

Objective: The Objective of this study was to assess the practicality of the cholesteatoma staging system proposed by the Japan Otological Society (JOS) for acquired middle ear cholesteatoma (the 2010 JOS staging system)., Methods: Between 2009 and 2010, 446 ears with retraction pocket cholesteatoma underwent primary surgery at 6 institutions in Japan. The extent of cholesteatoma was surgically confirmed, and classified into three stages., Results: The cholesteatoma affected the pars flaccida in 325 ears (73%), the pars tensa in 100 ears (22%), and both regions combined in 21 ears (5%). The hearing outcome (postoperative air-bone gaps dB) worsened as follows (Stage I, II, III): 84%, 68%, 53% in pars flaccida; 71%, 62%, 30% in pars tensa, and 42% at Stage II, and 50% at Stage III in the combined group. The incidence of residual cholesteatoma increased as follows (Stage I, II, III): 2%, 12%, 23% in pars flaccida; 7%, 30%, 21% in the pars tensa group. The severity of disease was reflected in postoperative hearing and increasing incidence of recurrence rate., Conclusion: The 2010 JOS staging system is suitable for evaluating initial pathology. It is particularly practical for standardizing reporting of retraction pocket cholesteatoma and for adjusting for the severity of the condition during outcome evaluations. It may also provide information that is useful for counseling patients., (Copyright © 2017 Elsevier B.V. All rights reserved.)

In order to provide a basis for meaningful exchange of information among those treating cholesteatoma, the Committee on Nomenclature of the Japan Otological Society (JOS) was appointed in 2004 to create a cholesteatoma staging system as simple as possible to use in clinical practice in Japan. Following the announcement of preliminary criteria for the staging of pars flaccida (attic) cholesteatoma in 2008, we proposed the 2010 JOS staging system for two major types of retraction pocket cholesteatoma, pars flaccida and pars tensa cholesteatoma. Since then, the JOS staging system has been widely used in clinical studies of cholesteatoma in Japan, allowing standardization in reporting of surgical outcomes based on the respective stages of cholesteatoma. We have recently expanded the range of cholesteatoma by adding cholesteatoma secondary to a tensa perforation and congenital cholesteatoma as the 2015 JOS staging system for middle ear cholesteatoma. Although further revisions may be required for universal acceptance of these criteria, we hope our staging system will open the way for international consensus on staging and classification of middle ear cholesteatoma in the near future., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Objective: To analyze the difficulty of discriminating Japanese nonsense monosyllables in each of several grades of high-frequency hearing loss and to evaluate the limitations of amplification., Methods: We collected retrospective data on the discrimination of Japanese nonsense monosyllables by patients with three grades of high-frequency hearing loss who fulfilled or nearly fulfilled the Japanese criteria for EAS. Discrimination of the twenty monosyllables included in the 67-S speech audiometric test, which is approved by the Japan Audiological Society, was evaluated under quiet conditions., Results: One hundred and five ears of ninety-one adults with high-frequency hearing loss were tested. We classified the ears according to hearing threshold at 1000 Hz; Group 1: <45 dB; Group 2: ≥45 dB and <70 dB; Group 3: ≥70 dB. Under the best conditions, the best speech discrimination scores were 72.3 ± 18.6% (mean ± SD, N=11), 56.9 ± 19.9% (N=57) and 38.1 ± 22.6% (N=37) in Group 1, Group 2 and Group 3, respectively. For most of the monosyllables, discrimination score declined gradually as high-frequency hearing loss became more severe. The high incidence in the Japanese language of [k], an easy consonant to distinguish, may be an advantage for patients with high-frequency hearing loss who use hearing aids. By employing a new confusion matrix that displays consonants and the following vowels separately, we were able to reveal the interactions of those two components. We observed that discrimination of preceding nasal consonants and that of the following vowels were not independent in patients with high-frequency hearing loss., Conclusion: Our classification based on threshold at 1000 Hz was useful to predict the effectiveness and limitations of amplification in high-frequency hearing loss. Threshold at 1000 Hz can be an index enabling us to refine the indications of EAS for native Japanese speakers to maximize its effectiveness against high-frequency hearing loss., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Epstein syndrome is a rare autosomal dominant platelet disorder characterized by thrombocytopenia, giant platelets, and sensorineural hearing loss. It is included among four overlapping syndromes, the others being May-Hegglin anomaly, Fechtner syndrome, and Sebastian syndrome. It is now established that all four disorders are caused by mutations in the MYH9 gene. We report the case of a patient with Epstein syndrome in whom bilateral profound hearing impairment developed and cochlear implantation was carried out. A cochlear implant was successfully used with a speech discrimination score of 100% on a Japanese sentence recognition test. This report offers the second description of the performance of a cochlear implant in a patient with Epstein syndrome. This case study may offer hope for patients and their family members with this kind of mutation., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Objective: This study evaluated the usefulness of diffusion-weighted magnetic resonance imaging (DWI) in the diagnosis of middle ear cholesteatoma., Methods: We performed DWI on 73 patients suspected of having middle ear cholesteatoma, including 21 revision cases. Magnetic resonance imaging was performed with 1.5T units using diffusion-weighted spin-echo-type echo planar imaging (DWI)., Results: Of 73 subjects, 59 had cholesteatoma that consisted of 41 primary acquired cholesteatoma, 13 had residual and/or recurrent cholesteatoma, four had congenital cholesteatoma, and one had iatrogenic cholesteatoma. Positive DWI findings were observed in 42 subjects and negative findings in 31 subjects. The sensitivity, specificity, and positive and negative predictive values of DWI for cholesteatoma were 69.4%, 92.8%, 97.5%, and 41.9%, respectively. In the case of 34 patients who were positive for cholesteatoma on both otoscopic and CT examinations, 33 were diagnosed with cholesteatoma. Of the remaining 39 subjects with one or both negative results for cholesteatoma, the sensitivity, specificity, positive predictive value, and negative predictive value of DWI were 57.6%, 92.3%, 93.7%, and 52.1%, respectively. Cholesteatoma mass diameters were less than 5mm in 10 out of 18 subjects with both cholesteatoma and negative DWI findings. Of the 21 subjects who received revision surgery, the sensitivity, specificity, and positive and negative predictive values of DWI for residual or recurrent acquired cholesteatoma were 71.4%, 100%, 100%, and 63.6%, respectively., Conclusions: Since DWI clearly showed high specificity and positive predictive value, it is useful for diagnosing middle ear cholesteatoma, including postoperative recurrent cholesteatoma of 5mm diameter or larger. DWI could sufficiently detect cholesteatoma with one or both negative results on otoscopic and CT examinations, but it was difficult to detect cholesteatoma of less than 5mm diameter using DWI owing to the tiny mass and small volume of debris., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Objective: To evaluate the function of the postoperative auditory nerve preserved after translabyrinthine (TL) vestibular schwannoma (VS) removal., Methods: Fifteen patients, who underwent unilateral VS resection via a TL approach, were preserved auditory nerve anatomically. The size and location of VS were measured on MRI preoperatively. After surgery, the electrical stimulation test (EST) or electrically evoked auditory brainstem response (EABR) was performed., Results: Four cases (27%) out of fifteen patients were retained the functional integrity of the auditory nerve after surgery. The maximum tumor size in the group with a positive response to EST or EABR was significantly smaller than that in the group with a negative response to EST. It seems to be difficult to preserve auditory nerve function in cases where a tumor extends to the fundus of internal auditory canal., Conclusion: These results suggest that size of tumor and/or extension of tumor to the fundus might be important factors in preserving the auditory nerve function even if using a TL approach.

Objective: To determine the extent of audiometric correlation with CT findings., Methods: Forty-four patients (82 ears) with surgically confirmed otosclerosis underwent preoperative CT examination. Based on the computed tomography (CT) findings, the ears were classified into five groups as follows: Group A, the group with no pathological CT findings; Group B1, the group with demineralization localized in the region of the fissula antefenestram; Group B2, the group with demineralization extending towards the cochleariform process from the anterior region of the oval window; Group B3, the group with extensive demineralization surrounding the cochlea; and Group C, the group with thick anterior and posterior calcified plaques., Results: There were 32 ears (39.0%) in Group A, 21 ears (25.6%) in Group B1, 16 ears (19.5%) in Group B2, 7 ears (8.5%) in Group B3, and 6 ears (7.3%) in Group C. The mean bone conduction levels were greater in the order of the extent of demineralization: Groups A, B1-B3 suggesting positive relationship between the cochlear function and the degree of labyrhinthine otosclerosis., Conclusion: A good correlation between the preoperative CT findings and audiometry findings suggests that CT with a slice intervals between 0.5 and 1mm could provide useful informations in assuming the extent of otosclerosis in the inner ear.

Objectives: In order to evaluate the effect of a medical administration for the sudden deafness patients, single-drug treatment for idiopathic sudden sensorineural hearing loss (ISSHL) was assessed at multi-centers participating in the Acute Severe Hearing Loss Study Group sponsored by the Ministry of Health, Labor and Welfare of Japan., Methods: The subjects consisted of ISSHL patients who were (1) 20 years of age or older, (2) diagnosed within 2 weeks after the onset of hearing loss, (3) showing a mean hearing level of 40-90 dB at five frequencies from 250 to 4000 Hz, (4) previously untreated, and (5) with normal for age in hearing of the opposite ear. The drugs used in this study were ATP, alprostadil, hydrocortisone and amidotrizoate, which were administered intravenously, and beraprost sodium and betamethasone, which were given orally. Two drugs were assigned to each center, one of which was selected according to the code hidden in envelopes and administered for 1 week. The treatment after the single-drug administration was conducted at the discretion of each center. The hearing gain and recovery rate at 1 week after the initiation of single-drug treatment and at 1 month or over when the hearing level was fixed, were evaluated based on the criteria for hearing recovery prepared by the Acute Severe Hearing Loss Study Group., Results: There was no statistically significant difference in the recovery rate among drugs either at 1 week after the initiation of single-drug treatment or at the time of fixed hearing level. At the time when the hearing level was fixed, a statistically significant difference in the complete recovery rate was detected only between amidotrizoate and beraprost sodium., Conclusion: From these results, we could not find any specific drugs recommended for ISSNHL. In evaluating the effect of the drugs, however, several problems in the clinical trial for ISSHL should be considered.

A new technique of laryngoplasty using the function of the suprahyoid muscles (hyoid transposition laryngoplasty) was reported. Total laryngectomy is carried out as usual and the tracheal stump is anastomosed directly to the hypopharynx. The hyoid bone is cut medially, then transpositioned and sutured to the posterolateral margins of the tracheal stump. With this procedure, the new glottis is pressed strongly to the tongue base during swallowing and aspiration is avoided. Postoperative swallowing and speech in the cases operated on with this method were satisfactory, and even nasal respiration was possible in some cases.

A new conception of the pathogenesis of cholesteatoma is proposed based on clinical results of anterior tympanotomy, a procedure developed in order to prevent recurrence of cholesteatoma following retraction pocket formation after canal-up operation. In the cholesteatomatous ear, there is always a long, thick bony septum between the epitympanum and the supratubal recess. This septum is called the anterior attic bony plate, forming the tympanic diaphragm together with the tensor tympani mucosal fold. In anterior tympanotomy, this bony plate is removed finishing intact canal wall mastoidectomy, making a new ventilating route to the eustachian tube. With this technique, the rate of recurrence was reduced from 17 to 5%. Based on these results it is proposed that the real pathogenesis of cholesteatoma is not tubal dysfunction, but the osseous tympanic diaphragm.

The condition of the mastoidectomy cavity following intact canal wall tympanoplasty (ICWT) is always of great interest to the otosurgeon. We evaluated the status of postoperative aeration in a stable ear following ICWT for various types of chronic otitis media using high-resolution computed tomography (CT). The mastoidectomy cavity in all the cases of simple suppurative otitis is totally aerated and that in over 60% of the cases of adhesive otitis, attic type cholesteatoma and adhesive type cholesteatoma is obliterated by a soft tissue density mass. The size of aerated spaces in the postoperative middle ear cavity not only depends on the type of chronic otitis media but also tends to correlate with the degree of mastoid pneumatization in the opposite ear. CT is useful for the postoperative assessment of middle ear aeration.