Imaging analysis of dural exposure in cholesteatomas with a skull base defect.

PURPOSE: Dural exposure during cholesteatoma surgery can pose a risk of cerebrospinal fluid leakage or residual disease. Therefore, delicate handling of the area surrounding the bone defect in the cranial fossa is required. However, in small-sized defects, preoperative prediction of dural exposure can be challenging. This study aimed to evaluate the diagnostic value of computed tomography (CT) for preoperative prediction of cholesteatoma-related dural exposure in bone discontinuities in the skull base. METHODS: We evaluated serial high-resolution CT images showing bone density discontinuities in the middle cranial fossa (MCF) requiring mastoidectomy for cholesteatoma. The CT and intraoperative findings were analyzed retrospectively. We evaluated the length between the superior margins of the bone density discontinuities using coronal CT planes. Receiver operating characteristic (ROC) curves were constructed to determine the optimal cut-off values. RESULTS: We extracted data from 107 bone density discontinuities, among which 54 (50.5%) showed dural exposure intraoperatively. Discontinuities with dural exposure (n = 54) had significantly greater lengths than did those without (n = 53) (p < 0.001, Wilcoxon rank-sum test). The area under the curve was 0.9780 according to the ROC analysis, and the optimal cut-off value was determined to be 2.99 mm (sensitivity 92.59%; specificity 94.34%). CONCLUSION: A bone density discontinuity length of > 2.99 mm in the MCF on coronal CT plane is a reliable diagnostic marker for cholesteatoma-related dural exposure. Thus, preoperative high-resolution CT analysis can inform optimal surgical preparation and planning before manipulating the area surrounding the osteolytic lesion in the MCF.

Radiological and audiological predictors of stapes destruction in adherent pars tensa.

PURPOSE: Progressive adherent pars tensa occasionally induces ossicular erosion. Specifically, stapes discontinuity adversely affects postoperative hearing. However, this irretrievable sequela is challenging to prove preoperatively, partly because perimatrix inflammation on the pars tensa can obscure the visibility of the ossicles or the partial volume effect of computed tomography (CT) imaging can hamper detailed ossicular visualization. Therefore, there is no consensus regarding the ideal timing for switching from a wait-and-see approach to a surgical one. Herein, we aimed to explore the potential predictors of stapes superstructure destruction in adherent pars tensa. METHODS: This retrospective cohort study enrolled consecutive patients who underwent primary tympanoplasty for adherent pars tensa categorized as grade IV on Sadé's grading scale between April 2016 and September 2021. The impact of features on otoscopy and CT and air-bone gap (ABG) on stapes superstructure destruction was assessed using uni- and multivariable logistic regression analyses. RESULTS: Sixty-four ears were included. Multivariate analysis revealed the presence of debris on the adherent pars tensa (odds ratio [OR] [95% confidence interval {CI}]): 4.799 [1.063-21.668], p = 0.0415), presence of soft-tissue density occupying the oval window (OR [95% CI]: 13.876 [3.084-62.437], p = 0.0006), and a ≥ 20-dB preoperative ABG at 3 kHz (OR [95% CI]: 7.595 [1.596-36.132], p = 0.0108) as independent predictors for stapes superstructure destruction. CONCLUSION: High preoperative awareness of the possibility of destruction of the stapes superstructure would enable the surgeon to make a timely decision to provide surgical intervention before progression to severe stapes destruction, thereby maintaining long-term satisfactory hearing.

Radiological and audiological prediction for hearing outcome in cholesteatoma recidivism surgery.

PURPOSE: In cholesteatoma recidivism, achieving satisfactory hearing outcome after revision surgery remains challenging. The presence of concomitant recidivism pathology or related anatomical abnormalities can impact revision reconstruction of the sound transmission system. The current study aimed to identify prognostic factors affecting hearing outcomes after surgery for cholesteatoma recidivism. METHODS: This retrospective cohort study included consecutive patients whose ears required initial surgery for recidivism between January 2016 and December 2021. Patients followed up for < 6 months and those not indicated for ossiculoplasty were excluded. The impact of preoperative otoscopic findings, computed tomography (CT) features, and hearing levels on the prediction of satisfactory hearing (postoperative air-bone gap [ABG] ≤ 20 dB) was evaluated using univariate and multivariate logistic regression analyses. RESULTS: Overall, 102 patients were included, with a mean follow-up of 24.8 months. Multivariate logistic regression analysis revealed the following independent predictive factors for satisfactory hearing: presence of aeration in the tympanic cavity (odds ratio [OR] [95% confidence interval {CI}]: 13.287 [1.113-158.604], p = 0.0409), absence of soft-tissue density occupying the oval window (OR [95% CI]: 13.445 [3.178-56.887], p = 0.0040), and ≤ 22.5 dB preoperative ABG in four-frequency average (OR [95% CI]: 9.339 [2.026-43.050], p = 0.0042). CONCLUSIONS: For cholesteatoma recidivism, reliable preoperative prediction based on CT and ABG would facilitate decision-making regarding the probability of efficient revision ossiculoplasty or appropriate preoperative counseling, including early hearing rehabilitation using hearing aids or implementation of simultaneous implantable hearing equipment during surgery for recidivism.

Detailed clinical features and genotype-phenotype correlation in an OTOF-related hearing loss cohort in Japan.

Mutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). Although it is reported that most of the patients with OTOF mutations have stable, congenital or prelingual onset severe-to-profound hearing loss, some patients show atypical clinical phenotypes, and the genotype-phenotype correlation in patients with OTOF mutations is not yet fully understood. In this study, we aimed to reveal detailed clinical characteristics of OTOF-related hearing loss patients and the genotype-phenotype correlation. Detailed clinical information was available for 64 patients in our database who were diagnosed with OTOF-related hearing loss. As reported previously, most of the patients (90.6%) showed a "typical" phenotype; prelingual and severe-to-profound hearing loss. Forty-seven patients (73.4%) underwent cochlear implantation surgery and showed successful outcomes; approximately 85-90% of the patients showed a hearing level of 20-39 dB with cochlear implant and a Categories of Auditory Performance (CAP) scale level 6 or better. Although truncating mutations and p.Arg1939Gln were clearly related to severe phenotype, almost half of the patients with one or more non-truncating mutations showed mild-to-moderate hearing loss. Notably, patients with p.His513Arg, p.Ile1573Thr and p.Glu1910Lys showed "true" auditory neuropathy-like clinical characteristics. In this study, we have clarified genotype-phenotype correlation and efficacy of cochlear implantation for OTOF-related hearing loss patients in the biggest cohort studied to date. We believe that the clinical characteristics and genotype-phenotype correlation found in this study will support preoperative counseling and appropriate intervention for OTOF-related hearing loss patients.

Retrograde mastoidectomy with canal wall reconstruction versus intact canal wall tympanomastoidectomy for cholesteatoma with minimal mastoid extension.

PURPOSE: Appropriate reconstruction of the canal wall or maintenance of the middle ear pressure in cholesteatoma may help in preventing recurrence. Retrograde mastoidectomy with canal wall reconstruction (RMR) can overcome the challenge of a wider canal wall defect or temporal bone immaturity, which possibly increases the recurrence risk. This study compared the outcomes of RMR and intact canal wall tympanomastoidectomy (ICW) for cholesteatomas with minimal mastoid extension and quantitatively evaluate the relationship between anatomical features and recurrence. METHODS: This single-center retrospective cohort study included patients who had undergone primary ICW or RMR for pars flaccida cholesteatoma with minimal mastoid extension from 2009 to 2019. The main outcome measures were anatomical measurements of the shortest distance between the cranial fossa and the upper canal wall (SCU), attic volume, and bony defect area of the canal wall (BDC) on computed tomography; recidivism; and postoperative air-bone gap (ABG). RESULTS: There were no significant differences in the preoperative anatomical factors, recidivism incidence, and postoperative ABG between the RMR (n = 20) and ICW (n = 60) groups. However, the median BDC was significantly greater in the RMR group (58.3 vs. 37.0 mm2). There was no significant difference in the SCU and attic volume between patients with and without recurrence. CONCLUSION: Selection of RMR or ICW may not affect recidivism and hearing outcomes in cholesteatoma with minimal mastoid extension. Bony defect size and attic narrowness were not associated with recurrence. Considering wider visualization and one-staged operation, RMR can be more adaptable than ICW for cholesteatoma with minimal mastoid extension.

Bilateral Deafness as a Complication of the Vaccination-A Case Report.

The vaccination has much profit for an infectious disease. On the other hand, there is little frequency, side effects may appear. It includes severe complication. We reported the case that resulted in bilateral acute profound hearing loss after mumps alone and measles and rubella (MR) vaccination. The case was a 5 years old girl. She inoculated mumps alone and MR vaccine. After 18days later, both sides profound hearing loss occurred in her. The hearing loss was not improved by the intravenous feeding of the steroid. Three months later, cochlea implantation was carried out to her right ear. She got hearing again. As for the hearing loss, mumps vaccine was considered as a cause from a latency period until the onset. The bilateral profound hearing loss that was a very rare complication was occurred by vaccination. The care of the hearing is important, but the mental care of an affected child and the parent is important, too.

The patients associated with TMPRSS3 mutations are good candidates for electric acoustic stimulation.

OBJECTIVES: To clarify the frequency of TMPRSS3 mutations in the hearing loss population, genetic analysis was performed, and detailed clinical characteristics were collected. Optical intervention for patients with TMPRSS3 mutations was also discussed. METHODS: Massively parallel DNA sequencing (MPS) was applied for the target exon-sequencing of 63 deafness genes in a population of 1120 Japanese hearing loss patients. RESULTS: Hearing loss in 5 patients was found to be caused by compound heterozygous TMPRSS3 mutations, and their detailed clinical features were collected and analyzed. Typically, all of the patients showed ski slope type audiograms and progressive hearing loss. Three of the 5 patients received electric acoustic stimulation (EAS), which showed good results. Further, the onset age was found to vary, and there were some correlations between genotype and phenotype (onset age). CONCLUSIONS: MPS is a powerful tool for the identification of rare causative deafness genes, such as TMPRSS3. The present clinical characteristics not only confirmed the findings from previous studies but also provided clinical evidence that EAS is beneficial for patients possessing TMPRSS3 mutations.

Time Analysis of Recidivism in Acquired Cholesteatoma Using the Kaplan-Meier Method.

OBJECTIVE: To analyze the time trends of recidivism of acquired cholesteatoma using the Kaplan-Meier method. STUDY DESIGN: We conducted a retrospective, observational study of 256 patients having their first cholesteatoma surgery. The cumulative recidivism-free rate was calculated using Kaplan-Meier survival analysis related to the follow-up period, pathophysiology, the extent of the disease, and recidivism pathologies. RESULTS: Pars flacida cholesteatoma with tympanic cavity progression had a high likelihood of recurrence disease. Pars tensa cholesteatoma led to more recurrence of the disease than the residual disease. In both pars flacida and pars tensa cholesteatoma, the incidence of disease recurrence increased even 3 years after surgery. On the contrary, the incidence of residual disease peaked within 3 years after surgery, and thereafter, the incidence of residual disease tended to be small. In particular, pars flacida cholesteatoma extending into the mastoid cavity or tympanic cavity tended to recur up to 5 years postoperatively. CONCLUSIONS: We calculated the cumulative recidivism-free rates of 256 patients with cholesteatoma using Kaplan-Meier survival analysis. These results can lead to better estimates of the length of the follow-up period. LEVEL OF EVIDENCE: Level IV evidence from case-control studies.

Impact of Sclerotic Lesions on the Surgical Outcome of Cholesteatoma.

OBJECTIVE: Cholesteatoma is occasionally accompanied by excessive calcification and presumably has different osteogenic dynamics compared to noncholesteatomous chronic otitis media. Cholesteatoma-related sclerotic lesions through the temporal bone can pose manipulatory difficulties, possibly leading to worse surgical outcomes or complications. Therefore, we compared surgical outcomes of cholesteatomas with and without sclerotic lesions. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary academic medical center. PATIENTS: Consecutive ears with acquired cholesteatomas requiring primary resection between January 2009 and December 2019. The ears followed up for <1 year were excluded. INTERVENTIONS: Tympanoplasty with/without mastoidectomy. MAIN OUTCOME MEASURES: Prevalence and location of sclerotic lesion, comorbid fistulae, postoperative air-bone gap, recidivism, and complications. RESULTS: Overall, 19 (6.4%) of 299 ears had sclerotic lesions. More than two-thirds (68.4%) of sclerotic lesions were located adjacent to the lateral semicircular canal (LSC). Among ears with sclerotic lesions adjacent to the LSC, 46.2% had a comorbid fistula in the LSC. The prevalence of labyrinth fistula was significantly greater in ears with sclerotic lesions (42.1%) than in ears without sclerotic lesions (5.0%) (p < 0.0001, Fisher's exact test). Sensorineural hearing deterioration was significantly higher in ears with sclerotic lesions than in ears without (p = 0.0004, Fisher's exact test). Multivariate logistic regression analysis demonstrated that the presence of sclerotic lesions was a significant prognostic factor for residual disease (odds ratio [95% confidence interval]: 6.820 [2.055-22.633], p = 0.0017). CONCLUSIONS: Surgeons should be conscious of preoperative identification of sclerotic lesions adjacent to one of the semicircular canals, possibly leading to postoperative sensorineural hearing deterioration or residual cholesteatoma.

Radiological and Audiological Prediction for Ossicular Fixation in Chronic Otitis Media and Tympanic Membrane Perforation.

OBJECTIVES: Recurrent middle-ear infection can lead to ossicular fixation, adversely affecting post-tympanoplasty hearing outcomes. Preoperative prediction of ossicular fixation remains challenging. We aimed to investigate potential predictors of ossicular fixation in patients with chronic otitis media. STUDY DESIGN: Retrospective. SETTING: Tertiary academic medical center. PATIENTS: Patients with noncholesteatomatous chronic otitis media and tympanic membrane perforation, without ossicular discontinuities. INTERVENTIONS: Diagnostic. MAIN OUTCOME MEASURES: The fixation of each ossicle was assessed during tympanoplasty. The impact of preoperative otoscopic findings, computed tomography (CT) features, and hearing levels on the prediction of ossicular fixation was evaluated using uni- and multivariable logistic regression analyses. RESULTS: One hundred thirty-five patients were included. Soft-tissue density between the malleus head and the anterior wall (odds ratio, 3.789 [95% confidence interval, 1.177-12.196]; p = 0.0255) and poor development of mastoid cells (16.826 [2.015-134.520]; p = 0.0078) were independent predictors of malleus fixation. In addition, ≥50% tympanic membrane perforation (5.412 [1.908-15.353]; p = 0.0015), poor development of mastoid cells (3.386 [1.039-11.034]; p = 0.0431), and a ≥40-dB preoperative air-bone gap (ABG) at 500 Hz (4.970 [1.732-14.261]; p = 0.0029) were independent predictors of incus fixation. Soft-tissue density surrounding the stapes (18.833 [1.856-191.104]; p = 0.0119) and a ≥40-dB preoperative ABG at 500 Hz (13.452 [1.640-∞]; p = 0.0138) were correlated with stapes fixation. CONCLUSIONS: The accurate prediction of ossicular fixation in patients with chronic otitis media based on CT features and the ABG may facilitate decision-making regarding the need for ossiculoplasty, possibly avoiding unnecessary manipulation or overlooking of fixation.

Non-echoplanar diffusion weighed imaging and T1-weighted imaging for cholesteatoma mastoid extension.

OBJECTIVES: A broad mastoid extension limits cholesteatoma resection via a transmeatal approach including endoscopic ear surgery. Therefore, a preoperative diagnosis of mastoid extension is a the most critical factor to determine whether to perform mastoidectomy. The purpose of this study was to assess the efficacy of non-echoplanar diffusion-weighted imaging (non-EPI DWI) and T1-weighted imaging in the evaluation of mastoid extension in cholesteatomas of the middle ear. METHODS: Patients who underwent magnetic resonance imaging (MRI) for pretreatment evaluation before primary surgery for pars flaccida or tensa cholesteatoma, which revealed a high-signal intensity in the mastoid on T2-weighed imaging were retrospectively evaluated. Two board-certified radiologists retrospectively evaluated the extent of cholesteatomas on MRI with non-EPI DWI, non-EPI DWI- and T1-weighted axial imaging. The presence of a high signal intensity on non-EPI DWI or low or high signal intensity on T1-weighted imaging in the mastoid was evaluated. All cases were subclassified as M+ (surgically mastoid extension-positive) or M- (surgically mastoid extension-negative). RESULTS: A total of 59 patients with middle ear cholesteatoma were evaluated. There were 37 M+ cases and 22 M- cases. High-signal intensity on non-EPI DWI exhibited a sensitivity of 0.89 and specificity of 0.82, whereas partial low-signal intensity on T1-weighted imaging exhibited a sensitivity of 0.84 and specificity of 0.91 for detecting mastoid involvement. Complete high-signal intensity on T1-weighted imaging exhibited a sensitivity of 0.73 and specificity of 0.89 for detecting non-involvement of the mastoid. The sensitivity (0.92) and specificity (0.96) of combined non-EPI DWI and T1-weighted imaging evaluation were higher than those of with non-EPI DWI or T1-weighted imaging alone. The interobserver agreement for the presence of high-signal intensity in the mastoid cavity on non-EPI DWI was very good at 0.82, that of a partial low-signal intensity area in the mastoid cavity lesions on T1-weighted imaging was good, at 0.76 and that of complete high-signal intensity in the mastoid cavity lesions on T1-weighted imaging was good, at 0.67. CONCLUSIONS: The signal intensity on non-EPI DWI and T1-weighted imaging of the mastoid could be used to accurately assess the extent of middle ear cholesteatoma, which could facilitate surgical treatment planning.

Preoperative predictive criteria for mastoid extension in pars flaccida cholesteatoma in assessments using temporal bone high-resolution computed tomography.

OBJECTIVES: We aimed to clarify the usefulness of high-resolution computed tomography (HRCT) and establish HRCT criteria for presurgical assessment of the mastoid extension in pars flaccida cholesteatomas of the middle ear. METHODS: Retrospective observational study. Patients who underwent primary surgery for pars flaccida cholesteatoma and those who underwent temporal bone HRCT for pretreatment evaluation were reviewed. The distance in the anterior-most portion of the mastoid sinus on HRCT was measured, and the presence of surgically verified mastoid extension of cholesteatoma was evaluated. All cases were subclassified as M+ (surgically mastoid extension-positive) or M- (surgically mastoid extension-negative). RESULTS: A total of 107 patients with pars flaccida cholesteatoma were included. The distance in the M+ cases was significantly longer than that in the M- cases, and the cutoff value was 3.6 mm. The difference between the ipsilateral/diseased-side distance and the contralateral/evaluable side (difference value) in M+ cases was larger than that in M- cases, with a cutoff value of 0.6 mm. The inter-rater reliability of this distance measurement was excellent, regardless of imaging experience. CONCLUSIONS: The cutoff values of the distance and the difference value can be used for pretreatment HRCT evaluation of mastoid extension in middle ear cholesteatoma with relatively high accuracy, regardless of the experience and skill levels of the evaluator.

Imaging Analysis for Cholesteatoma Extension to the Perilymphatic Space in Labyrinth Fistulae.

OBJECTIVES/HYPOTHESIS: Disturbed perilymph behind a labyrinth fistula can lead to hearing deterioration; thus, delicate manipulation is required during surgery for cholesteatomatous fistulae with matrix extension to the perilymphatic space (EPS). However, it remains challenging to identify the EPS preoperatively. This study aimed to evaluate the diagnostic value of computed tomography (CT) for preoperative prediction of the EPS of cholesteatomatous fistulae. STUDY DESIGN: Retrospective study. METHODS: We included serial high-resolution CT images showing a cholesteatomatous bone defect in the lateral semicircular canal (LSC) requiring mastoidectomy. CT and intraoperative findings were analyzed retrospectively. Using axial CT planes, we evaluated the length and angle between the margins of bone defects. Receiver operating characteristic (ROC) curves were constructed to determine the cutoff points. RESULTS: We extracted data from 30 bone defects, of which six (20.0%) showed EPS intraoperatively. Bone defects with EPS (n = 6) had significantly greater length and angle values than those without EPS (n = 24) (P < .001 for both, Wilcoxon rank sum test). For length and angle, the area under the curve was 0.944 (95% confidence interval [CI]: 0.858-1.000) and 0.951 (95% CI: 0.875-1.000), respectively, according to the ROC analysis, and the optimal cutoff values were 3.65 mm and 71.6°, respectively, with 100% sensitivity and 91.67% specificity for both. CONCLUSIONS: Results demonstrated that a length >3.65 mm and an angle >71.6° for LSC bone defects on axial CT images are reliable diagnostic markers of EPS. Preoperative high-resolution CT analysis can provide surgeons with a more conscientious preparation for handling deeper labyrinth fistulae. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E1301-E1307, 2021.

The impact of middle ear aeration on surgical outcome after intact canal wall tympanoplasty for cholesteatoma.

OBJECTIVE: The aeration status of the middle ear is presumed to be one of the factors affecting the outcome in acquired cholesteatoma. The present study investigated the impact of postoperative middle ear aeration on hearing and recurrence after intact canal wall tympanoplasty. METHODS: We conducted a retrospective chart review of 127 pars flaccida cholesteatoma patients who underwent primary intact canal wall tympanoplasty at a tertiary academic medical center. We collected data on clinical characteristics, including extent of cholesteatoma, surgical procedure, pre- and postoperative middle ear aeration, hearing level, and recurrence. The aeration was measured before and at one year after operation on two axial computed tomography (CT) planes: at the lateral semicircular canal (mastoid cavity) and at the oval window (tympanic cavity). Based on the postoperative air-bone gap (ABG), patients were categorized into two groups: the successful hearing (ABG ≤15 dB) group and the unsuccessful hearing (ABG >15 dB) group. We used aeration ratio to assess the relationship between postoperative aeration improvement and hearing outcome or recurrence. Multivariate logistic regression analysis was used to identify the factors associated with hearing outcome. RESULTS: At one year after operation, aeration ratio in both mastoid and tympanic cavities was significantly improved than the preoperative status (p < 0.001, p < 0.001, respectively, Wilcoxon signed-rank test). The positive correlation was found between postoperative aeration ratios of mastoid cavity and tympanic cavity (r = 0.348, p < 0.001, Spearman's rank-correlation coefficient). In mastoid and tympanic cavities, the postoperative aeration ratio in successful hearing group (n = 57) was significantly higher than that in the unsuccessful hearing group (n = 45) at one year after operation (p < 0.001, p = 0.028, respectively, Mann-Whitney U test). Multivariate logistic regression analysis demonstrated that postoperative aeration ratio in mastoid cavity and preoperative ABG were significant independent prognostic factors for successful hearing (odds ratio [95% confidence interval]: 2.630 [0.985 - 7.024], p = 0.045; 0.891[0.840 - 0.944], p < 0.001, respectively). However, postoperative aeration ratios did not significantly differ between with (n = 14) and without recurrence (n = 113) groups in both cavities. CONCLUSION: Our results suggest that better postoperative aeration in mastoid cavity is independently associated with satisfactory hearing outcome after intact canal wall tympanoplasty in pars flaccida cholesteatoma. However, no significant differences were observed between middle ear aeration at one year after operation and recurrence.

Prevalence and clinical features of hearing loss caused by EYA4 variants.

Variants in the EYA4 gene are known to lead to autosomal dominant non-syndromic hereditary hearing loss, DFNA10. To date, 30 variants have been shown to be responsible for hearing loss in a diverse set of nationalities. To better understand the clinical characteristics and prevalence of DFNA10, we performed genetic screening for EYA4 mutations in a large cohort of Japanese hearing loss patients. We selected 1,336 autosomal dominant hearing loss patients among 7,408 unrelated Japanese hearing loss probands and performed targeted genome enrichment and massively parallel sequencing of 68 target genes for all patients. Clinical information of cases with mutations in EYA4 was gathered and analyzed from medical charts. Eleven novel EYA4 variants (three frameshift variants, three missense variants, two nonsense variants, one splicing variant, and two single-copy number losses) and two previously reported variants were found in 12 probands (0.90%) among the 1,336 autosomal dominant hearing loss families. The audiometric configuration of truncating variants tends to deteriorate for all frequencies, whereas that of non-truncating variants tends to show high-frequency hearing loss, suggesting a new correlation between genotype and phenotype in DFNA10. The rate of hearing loss progression caused by EYA4 variants was considered to be 0.63 dB/year, as found in this study and previous reports.

Clinical Characteristics of Pars Tensa Cholesteatoma: A Comparative Study of Area-Based Classification Systems Proposed by the Japanese Otological Society and the European Academy of Otology - Neuro-Otology.

OBJECTIVES: To assess the clinical characteristics of extent patterns in pars tensa cholesteatoma. MATERIALS AND METHODS: This was a retrospective chart review. Forty-four patients with pars tensa cholesteatoma who underwent primary surgery at a tertiary academic medical center were included. The main outcomes measured were sex, age, clinical background, and stage classification of pars tensa cholesteatoma (including the extent of cholesteatoma and involvement of the sinus tympani) according to two staging classifications: criteria advocated by the Japanese Otological Society (JOS) and those advocated by the European Academy of Otology and Neuro-Otology (EAONO)/JOS joint consensus statements. RESULTS: The mean patient age ± standard deviation was 38.4±19.6 years. The patients comprised 19 men and 25 women. According to the JOS classification, 18 ears (40.9%) were classified as stage I, 22 (50.0%) as stage II, and 4 (9.1%) as stage III. According to the EAONO/JOS joint consensus statements, 14 ears (31.8%) were classified as stage I, 26 (59.1%) as stage II, and 4 (9.1%) as stage III. Fourteen ears (31.8%) demonstrated involvement of the sinus tympani. Four ears (9.1%) that were originally categorized as stage I cholesteatoma by the JOS criteria showed sinus tympani invasion and were subsequently categorized as stage II according to the EAONO/JOS criteria. CONCLUSION: We determined the clinical characteristics of pars tensa cholesteatoma based on the novel and well-defined classification criteria. Further studies including long-term outcomes are necessary to demonstrate the clinical relevance of the discrepancy between the two criteria with respect to involvement of the sinus tympani.

OTOF mutation analysis with massively parallel DNA sequencing in 2,265 Japanese sensorineural hearing loss patients.

The OTOF gene (Locus: DFNB9), encoding otoferlin, is reported to be one of the major causes of non-syndromic recessive sensorineural hearing loss, and is also reported to be the most common cause of non-syndromic recessive auditory neuropathy spectrum disorder (ANSD). In the present study, we performed OTOF mutation analysis using massively parallel DNA sequencing (MPS). The purpose of this study was to reveal the frequency and precise genetic and clinical background of OTOF-related hearing loss in a large hearing loss population. A total of 2,265 Japanese sensorineural hearing loss (SNHL) patients compatible with autosomal recessive inheritance (including sporadic cases) from 53 otorhinolaryngology departments nationwide participated in this study. The mutation analysis of 68 genes, including the OTOF gene, reported to cause non-syndromic hearing loss was performed using MPS. Thirty-nine out of the 2,265 patients (1.72%) carried homozygous or compound heterozygous mutations in the OTOF gene. It is assumed that the frequency of hearing loss associated with OTOF mutations is about 1.72% of autosomal recessive or sporadic SNHL cases. Hearing level information was available for 32 of 39 patients with biallelic OTOF mutations; 24 of them (75.0%) showed profound hearing loss, 7 (21.9%) showed severe hearing loss and 1 (3.1%) showed mild hearing loss. The hearing level of patients with biallelic OTOF mutations in this study was mostly severe to profound, which is consistent with the results of past reports. Eleven of the 39 patients with biallelic OTOF mutations had been diagnosed with ANSD. The genetic diagnosis of OTOF mutations has significant benefits in terms of clinical decision-making. Patients with OTOF mutations would be good candidates for cochlear implantation; therefore, the detection of OTOF mutations is quite beneficial for patients, especially for those with ANSD.

Practical analysis of pars flaccida cholesteatoma with classification and staging system proposed by Japan Otological Society: a comparative study.

BACKGROUND: The Japan Otology Society (JOS) proposed the classification and staging system for middle ear cholesteatoma. However, there was little analysis of the pathology of cholesteatoma using this staging system. AIMS/OBJECTIVES: To analyze the pathology of pars flaccida cholesteatoma using the staging system by JOS. MATERIAL AND METHODS: A total of 183 cases of fresh pars flaccida cholesteatoma treated between January 2009 and December 2015 were included. We used the staging system recommended by JOS (2015). The association of the following variables in each stage of pars flaccida cholesteatoma was examined: age, gender, preoperative hearing level, staging, statuses of mastoid cell growth and stapes, tympanic sinus invasion. RESULTS: Stage II disease showed the highest degree of progression, and peak incidence was observed in the third and fourth decades of life. Stage III significantly increased after the age of 40 years. The progression of the disease stage was significantly associated with deterioration of hearing level. Cholesteatoma invasion to tympanic sinus was recognized in 14.2% of cases. The state of the stapes is increasingly likely be to fracture as stage progresses. CONCLUSIONS: The JOS Staging System appropriately reflects the disease state, and it was found to be clinically meaningful in this study.

Effectiveness of setting numerical targets in the surgical training of residents: a trial to achieve an optimal balance.

During the past 10 years, residency training in otorhinolaryngology-head and neck surgery (ORL-HNS) in Japan, especially at university hospitals, has emphasized subspecialization, resulting in insufficiencies in basic surgical techniques with an extreme bias toward acquiring subspecialty surgical case experience. To address this problem, we developed a target-oriented program intended to achieve a more balanced approach to surgical training and performed a 1-year trial of the program at the Jikei University School of Medicine. Fourteen residents with 1 to 4 years of ORL-HNS experience completed the trial. Each resident's competencies in six basic surgical procedures were assessed on the basis of the number of cases handled by the resident, and each resident's case selection bias after implementation of the target-oriented training was examined. The case selection bias in the trial group residents was reduced and their balance in case experience was shown to be improved in comparison with that in control group residents who were trained in the conventional way. In addition, opinion surveys of the participants and supervising otorhinolaryngologists (trainers) indicated that they felt that the new training system had been effective in improving the balance in case experience and improving motivation, and creating greater awareness of training goals and progress.