Objective: To summarize the HRCT and MRI appearances of stapical footplate fistula related to inner ear malformation (SFF-Re-IEM). Methods: The HRCT and MRI materials of 48 cases (53 ears) SFF-Re-IEM were retrospectively analyzed. Among them, 25 SFF-Re-IEM ears were confirmed by surgery. Their CT and MRI findings including associated IEM type, internal auditory canal (IAC) malformation, tympanic fluid, its density and signal features, and accompanied labyrinthitis were recorded. Results: Among 48 cases (53 ears) with SFF-Re-IEM, 17 ears with incomplete partition type Ⅰ, accounting for 32.1%, 13 ears with common cavity for 24.5%, 13 ears with cochlear aplasia for 24.5%, 7 ears with cochlear dysplasia Ⅱ for 13.2%, and 3 ears with Mondini for 5.7%,were found respectively. 94.3% of them were associated with a defect or dysplasia in the found of the IAC. They were divided into 4 types according to the intact of the stapical footplate and accompanied CSF otorrhea: 22 ears were diagnosed as the stapical footplate leaking, of them, 2 ears might come from the stapical footplate bony defect, 6 ears were from the stapical footplate hernia. 1 ear belonged to the peristapical footplate leaking. 30 ears with the isolated the stapical footplate hernia were another found. The bony defect in 2 ears with the stapical footplate bony defect were not presented on CT and MRI.The focal bony defect of the affected stapical footplate of 36 ears with the stapical footplate hernia were demonstrated, which presented the hemispherical protruding into the tympana, the soft-tissue density on CT, and CSF-like signal on the MR heaved-T2WI images. Among 22 ears with the stapical footplate leaking, their imaging appearances varied from the different amount of the leaking CSF. Besides the focal bony defects of the affected stapical footplates, there were much more CSF-like density or signal in the ipsilateral tympanic cavity in 17 affected ears connecting with the vestibule through the defect area. In the CSF leaking ears with less CSF leaking in 5 ears, the CSF-like cysts like SFH were shown on the stapical footplate defect area, but their outer edges were irregular, and the CSF-like signal scattering in the tympanic cavity did not connect with the protruding cysts at the stapical area. Conclusion: The variable appearances of the SFF-Re-IEM ears based on the different subtypes are its characteristic HRCT and MRI appearances. This is helpful for the SFF-Re-IEM diagnosing to grasp its imaging features.
目的： 总结内耳畸形相关性镫骨底板瘘（stapical footplate fistula related to inner ear malformation，SFF-Re-IEM）的CT和MRI表现特征。 方法： 回顾性分析山东省第二人民医院2014年8月至2023年10月期间48例（53耳）SFF-Re-IEM患者的CT和MRI资料，其中男35例、女13例，年龄1~55岁；53耳中有25耳经手术证实。记录患耳伴发内耳畸形的类型、内听道发育情况、镫骨底板及鼓岬完整性、鼓室内积液的密度、信号特征等。 结果： 53耳SFF-Re-IEM中伴发内耳畸形不全分隔Ⅰ型17耳（32.1%，17/53）、共同腔畸形13耳（24.5%，13/53）、耳蜗未发育13耳（24.5%，13/53）、耳蜗发育不良7耳（13.2%，7/53）、Mondini畸形3耳（5.7%，3/53）。94.3%（50/53）患耳伴发内听道底部发育不良或缺损。根据镫骨底板骨质完整性及伴发脑脊液耳漏情况分为4类：镫骨底板骨质缺损、镫骨底板疝、镫骨底板漏和镫骨底板旁漏。镫骨底板漏22耳，其中2耳可能由镫骨底板骨质缺损发展而来，6耳由镫骨底板疝发展而来；镫骨底板旁漏1耳；孤立性镫骨底板疝30耳。2耳镫骨底板骨质缺损患耳缺损区在CT和MRI上不能显示。36耳镫骨底板疝在CT上表现为镫骨底板局部骨质缺损、缺损区见半球形软组织密度影，其在MRI水成像序列上呈脑脊液样信号影，外缘光整，鼓室内无积液信号。22耳镫骨底板漏中，患侧镫骨底板除在CT上显示局限性骨质缺损外，患侧鼓室内显示多少不一脑脊液样密度或信号影；17耳大量漏出患耳显示鼓室内高信号经缺损区与畸形内耳内高信号相连续，5耳少量脑脊液漏出患耳表现为患侧足弓间隙内囊状脑脊液信号影，与鼓室内少量积液不连续。1耳镫骨底板旁漏表现患侧镫骨底板骨质完整，其后下方鼓岬骨质局限性缺损，鼓室内脑脊液样信号通过缺损区与内耳内高信号相连续。 结论： SFF-Re-IEM因类型不同其影像学表现具有多样性，了解其影像学表现特征，有助于其诊断。.

OBJECTIVE: Little is known about the hearing characteristics in patients with congenital round window atresia (CRWA). This study aimed to investigate hearing characteristics in patients with CRWA by comparing them with two relatively common congenital middle ear anomalies: congenital stapedial fixation (CSF) and congenital ossicular discontinuity (COD).
METHODS: Literature searches yielded five patients with surgically confirmed CRWA (seven ears), who were included in the CRWA group, along with one of our patients. Air and bone conduction thresholds; air-bone gap (ABG); and presence and depth of the Carhart notch were analyzed. These audiometric variables in the CRWA group were compared with those in the CSF (n = 15) and COD (n = 22) groups, comprising patients identified from our institution\'s medical database.
RESULTS: Average bone and air conduction thresholds in the CRWA group were 16.4 (standard deviation [SD]: 2.9; 95 % confidence interval [CI]: 14.6-18.3) and 44.6 (SD: 3.5; 95 % CI: 42.6-47.3) dB hearing level (HL). Bone conduction thresholds at high frequencies (≥2 kHz) were higher than those at low frequencies (<2 kHz), while air conduction thresholds at high frequencies were lower than those at low frequencies: ABGs at high frequencies were significantly smaller than those at low frequencies (2 kHz vs. 0.5 kHz, p = 0.027; 2 kHz vs. 1 kHz, p = 0.041; 4 kHz vs. 0.5 kHz, p = 0.042; 4 kHz vs. 1 kHz, p = 0.027). There were no between-group differences in incidence and depth of the Carhart notch.
CONCLUSIONS: CRWA could manifest as a distinct audiometric pattern with poorer bone conduction and better air conduction at ≥2 kHz, resulting in significantly smaller ABGs at higher frequencies than that at lower frequencies. Our findings indicated that this pattern differed from that of CSF and COD. The unique beer bottle-shaped audiogram associated with CRWA might facilitate its early diagnosis in patients with congenital conductive hearing loss.

The auditory ossicles amplify and transmit sound from the environment to the inner ear. The distribution of bone mineral density is crucial for the proper functioning of sound transmission as the ossicles are suspended in an air-filled chamber. However, little is known about the distribution of bone mineral density along the human ossicular chain and within individual ossicles. To investigate this, we analyzed fresh-frozen human specimens using synchrotron-based phase-contrast microtomography. In addition, we analyzed the volume and porosity of the ossicles. The porosity for the auditory ossicles lies, on average, between 1.92% and 9.85%. The average volume for the mallei is 13.85 ± 2.15 mm3, for the incudes 17.62 ± 4.05 mm3 and 1.24 ± 0.29 mm3 for the stapedes. The bone density distribution showed a similar pattern through all samples. In particular, we found high bone mineralization spots on the anterior crus of the stapes, its footplate, and along areas that are crucial for the transmission of sound. We could also see a correlation between low bone mineral density and holey areas where the bone is only very thin or missing. Our study identified a similar pattern of bone density distribution within all samples: regions exposed to lower forces generally show higher bone density. Further, we observed that the stapes shows high bone mineral density along the anterior crus and its footplate, which may indicate its importance in transmitting sound waves to the inner ear.

OBJECTIVE: To describe the new semisynthetic total ossicular replacement prosthesis (New-SSTORP) and to evaluate the New-SSTORP ossiculoplasty results both with the presence and absence of the stapes superstructure.
METHODS: Prospective study.
METHODS: Tertiary referral center.
METHODS: From April 2023 to May 2023, 18 New-SSTORP ossiculoplasties were performed by the first author. In all patients, the New-SSTORP was interposed between the footplate and the eardrum. The study group was divided into two groups (group A and group B). Group A included 13 patients with the absence of stapes superstructure. Group B included five patients with the presence of stapes superstructure. A successful reconstruction was defined as a postoperative air-bone gap ABG ≤20 dB. For all patients of groups A and B, the last audiometric control considered was performed in January 2024. The mean follow-up was 8½ months. The χ2 test was used to compare results. p < 0.05 was considered significant.
METHODS: Mean postoperative ABG ≤20 dB.
RESULTS: At the end of follow-up, the overall success rate (ABG ≤20 dB) of New-SSTORP ossiculoplasty was obtained in 88.8% (n = 16 of 18) of cases. In group A, the success rate of New-SSTORP ossiculoplasty occurred in 84.6% (n = 11 of 13) of cases, and in group B, the success rate of New-SSTORP ossiculoplasty occurred in 100% (n = 5 of 5) of cases. There was no audiological statistically significant difference between groups A and B (Fisher value is 1; p < 0.05). In all cases, the time for positioning of New-SSTORP was about 5 minutes.
CONCLUSIONS: The New-SSTORP has a minimal technical challenge for building and placement. The New-SSTORP ossiculoplasty results are very good both with the presence and absence of SS.

Sound transmission to the human inner ear by bone conduction pathway with an implant attached to the otic capsule is a specific case where the cochlear response depends on the direction of the stimulating force. A finite element model of the temporal bone with the inner ear, no middle and outer ear structures, and an immobilized stapes footplate was used to assess the directional sensitivity of the cochlea. A concentrated mass represented the bone conduction implant. The harmonic analysis included seventeen frequencies within the hearing range and a full range of excitation directions. Two assessment criteria included: (1) bone vibrations of the round window edge in the direction perpendicular to its surface and (2) the fluid volume displacement of the round window membrane. The direction of maximum bone vibration at the round window edge was perpendicular to the round window. The maximum fluid volume displacement direction was nearly perpendicular to the modiolus axis, almost tangent to the stapes footplate, and inclined slightly to the round window. The direction perpendicular to the stapes footplate resulted in small cochlear responses for both criteria. A key factor responsible for directional sensitivity was the small distance of the excitation point from the cochlea.

OBJECTIVE: Revision stapes surgery is a challenging procedure performed in relatively small numbers compared to other middle ear procedures. Despite numerous data on hearing results of different middle ear surgeries, the audiological standards for successful outcome of this procedure are still not clarified. On the basis of well-documented data, we wanted to determine what the expected audiological results and complications are after revision stapes surgery in order to set a realistic threshold for surgical success.
METHODS: After the protocol registration in the PROSPERO database, a systematic review was performed in multiple databases (PubMed, Cochrane, Web of Science, Scopus, ScienceOpen, ClinicalTrials.gov, Google Scholar) according to PRISMA guidelines. Twelve articles were reviewed according to the inclusion criteria. A total of 1032 cases were obtained for evaluation. A modified version of Newcastle-Ottawa Scale (NOS) was used to assess publication quality.
RESULTS: Average air-bone gap (ABG) gain was 17.3 dB, average air conduction (AC) gain was 17.5 dB. The average postoperative air-bone gap was 11.1 dB. The postoperative ABG distribution was the following 0-10 dB: 53.3%, > 10-20 dB: 28.2%, > 20 dB: 18.5%. SNHL as a surgical complication was described in a total of 17 cases (1.6%), no equilibrium disorder was reported.
CONCLUSIONS: The pooled data suggest that revision stapes surgery is an effective solution after failure of previous stapes surgery. However, the results are clearly inferior to those of primary stapedotomies. Hence, we need to apply different expectations and use different standards in the indication and evaluation of this type of surgery.

The current work investigated the effects of mass-loading the eardrum on wideband absorbance in humans. A non-invasive approach to mass-loading the eardrum was utilized in which water was placed on the eardrum via ear canal access. The mass-loaded absorbance was compared to absorbance measured for two alternative middle ear states: normal and stiffened. To stiffen the ear, subjects pressurized the middle ear through either exsufflation or insufflation concurrent with Eustachian tube opening. Mass-loading the eardrum was hypothesized to reduce high-frequency absorbance, whereas pressurizing the middle ear was hypothesized to reduce low- to mid-frequency absorbance. Discriminant linear analysis classification was performed to evaluate the utility of absorbance in differentiating between conditions. Water on the eardrum reduced absorbance over the 0.7- to 6-kHz frequency range and increased absorbance at frequencies below approximately 0.5 kHz; these changes approximated the pattern of changes reported in both hearing thresholds and stapes motion upon mass-loading the eardrum. Pressurizing the middle ear reduced the absorbance over the 0.125- to 4-kHz frequency range. Several classification models based on the absorbance in two- or three-frequency bands had accuracy exceeding 88%.

OBJECTIVE: During an initial diagnostic assessment of an ear with normal otoscopic exam, it can be difficult to determine the specific pathology if there is a mechanical lesion. The audiogram can inform of a conductive hearing loss but not the underlying cause. For example, audiograms can be similar between the inner-ear condition superior canal dehiscence (SCD) and the middle-ear lesion stapes fixation (SF), despite differences in pathologies and sites of lesion. To gain mechanical information, wideband tympanometry (WBT) can be easily performed noninvasively. Absorbance , the most common WBT metric, is related to the absorbed sound energy and can provide information about specific mechanical pathologies. However, absorbance measurements are challenging to analyze and interpret. This study develops a prototype classification method to automate diagnostic estimates. Three predictive models are considered: one to identify ears with SCD versus SF, another to identify SCD versus normal, and finally, a three-way classification model to differentiate among SCD, SF, and normal ears.
METHODS: Absorbance was measured in ears with SCD and SF as well as normal ears at both tympanometric peak pressure (TPP) and 0 daPa. Characteristic impedance was estimated by two methods: the conventional method (based on a constant ear-canal area) and the surge method, which estimates ear-canal area acoustically.Classification models using multivariate logistic regression predicted the probability of each condition. To quantify expected performance, the condition with the highest probability was selected as the likely diagnosis. Model features included: absorbance-only, air-bone gap (ABG)-only, and absorbance+ABG. Absorbance was transformed into principal components of absorbance to reduce the dimensionality of the data and avoid collinearity. To minimize overfitting, regularization, controlled by a parameter lambda, was introduced into the regression. Average ABG across multiple frequencies was a single feature.Model performance was optimized by adjusting the number of principal components, the magnitude of lambda, and the frequencies included in the ABG average. Finally, model performances using absorbance at TPP versus 0 daPa, and using the surge method versus constant ear-canal area were compared. To estimate model performance on a population unknown by the model, the regression model was repeatedly trained on 70% of the data and validated on the remaining 30%. Cross-validation with randomized training/validation splits was repeated 1000 times.
RESULTS: The model differentiating between SCD and SF based on absorbance-only feature resulted in sensitivities of 77% for SCD and 82% for SF. Combining absorbance+ABG improved sensitivities to 96% and 97%. Differentiating between SCD and normal using absorbance-only provided SCD sensitivity of 40%, which improved to 89% by absorbance+ABG. A three-way model using absorbance-only correctly classified 31% of SCD, 20% of SF and 81% of normal ears. Absorbance+ABG improved sensitivities to 82% for SCD, 97% for SF and 98% for normal. In general, classification performance was better using absorbance at TPP than at 0 daPa.
CONCLUSIONS: The combination of wideband absorbance and ABG as features for a multivariate logistic regression model can provide good diagnostic estimates for mechanical ear pathologies at initial assessment. Such diagnostic automation can enable faster workup and increase efficiency of resources.

The cause of sudden conductive loss in the absences of other vestibulo-audiologic abnormalities can be isolated to specific subsites of the ossicular chain using audiometric testing. In the absence of such abnormalities, a rare etiology may be the cause after an inciting trauma. Laryngoscope, 2024.

OBJECTIVE: To examine the clinical features and surgical outcomes in patients with congenital absence of the oval window (CAOW), and to investigate the potential factors that affect audiologic results.
METHODS: A retrospective chart review.
METHODS: A tertiary academic center.
METHODS: A total of 17 ears among 16 patients were confirmed to have CAOW. Among them, 13 ears underwent vestibulotomy for hearing reconstruction. Clinical parameters associated with the hearing outcomes were analyzed.
METHODS: A mean air-bone gap (ABG) after 6-month and long-term follow-up was compared with preoperative measurements.
RESULTS: Intraoperative findings showed that anomalies of the malleus or incus were observed in 11 ears (64.7%), stapes anomalies were present in all ears (100%), and facial nerve anomalies were present in 10 ears (58.8%). Because of unfavorable facial nerve anomalies, hearing reconstruction was aborted in four cases (23.5%). In the hearing reconstruction group, the mean ABG at 6 months postoperation was significantly reduced after compared with the preoperative value (44.0 ± 8.4 dB versus 58.8 ± 9.1 dB, p = 0.006). After dividing ears into a success subgroup (ABG ≤ 30 dB, seven ears) and non-success subgroup (ABG > 30 dB, six ears), the use of a drill during vestibulotomy was significantly related to a poor hearing outcome (100% versus 16.7%, p = 0.015). The long-term follow-up result (mean, 60 mo) revealed no deterioration compared with the 6-month postoperative result. Five ears (29.4%) underwent revision surgery, and three of them showed ABG improvements. No serious complications were reported.
CONCLUSIONS: Vestibulotomy is an effective and safe option for hearing restoration in patients with CAOW, particularly when the use of a drill is not required. The long-term audiologic outcome is also reliable.