Isolated congenital middle ear malformation (CMEM) contributes significantly to congenital hearing loss and growth problems. This study aims to compare 0.1-mm isotropic ultra-high-resolution CT (U-HRCT) and conventional high-resolution CT (HRCT) for assessing isolated CMEM, using surgical exploration as the standard. This single-center retrospective study included patients with surgically confirmed isolated CMEM who underwent U-HRCT or HRCT from January 2015 to April 2025. Middle ear abnormalities were identified based on operative outcomes and 4 subtypes were classified via the Teunissen standard. Two neuroradiologists blinded to surgical outcomes reviewed CT images for 10 subtle structural abnormalities and specific subtypes. The comparison of U-HRCT and HRCT in terms of interobserver and intraobserver agreement and detection of structural abnormalities and subtypes of CMEM were analyzed. The U-HRCT and HRCT groups included 61 patients (69 ears) and 37 patients (44 ears), respectively. U-HRCT exhibited significantly higher interobserver and intraobserver agreement and stronger concordance with surgical findings for all 10 abnormalities compared with HRCT. It also showed superior diagnostic sensitivity for CMEM (100.0% versus 90.9%; P = .013) and outperformed HRCT in differentiating clinical subtypes (0.774 versus 0.352; P<.001). U-HRCT achieved accuracies exceeding 0.85 in identifying all abnormalities and outperformed HRCT in detecting specific abnormalities including abnormal long process of the incus, lenticular process, abnormal stapes superstructure, stapes footplate fixation, and oval window atresia (P < .05). Isotropic 0.1-mm U-HRCT significantly outperforms conventional HRCT in diagnosing CMEM, differencing subtypes, and detecting subtle abnormalities, supporting its clinical superiority for precise preoperative evaluation.

Objective: To investigate the efficacy of malleostapedotomy (MS) in treating congenital ossicular malformations and analyze the influencing factors of postoperative hearing outcomes using ultra-high-resolution computed tomography (U-HRCT). Methods: Patients with ossicular malformation who underwent MS surgery in the Center of Otolaryngology Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University from January 2015 to August 2024 were retrospectively included. Based on U-HRCT, the thickness of the stapes footplate or the oval window atresia plate and the angle and depth of Piston inserted into the vestibule were measured, and their correlations with postoperative efficacy were analyzed. Results: A total of 25 patients (26 ears) aged 15 (9, 27) years were included, with 15 males and 10 females. The average pure tone postoperative air-bone gap (ABG) of the 26 ears was lower than preoperative ABG [(22.7±10.1) decibels hearing level (dB HL) vs (44.8±12.0) dB HL, P<0.001]. Among them, eight ears underwent U-HRCT after the surgery. The measurement results showed that there was no correlation between postoperative ABG and the depth of Piston insertion (r=-0.061, P=0.885). Postoperative ABG was not correlated with the angle of Piston insertion (r=0.239, P=0.569). There was no correlation between postoperative ABG and the thickness of the stapes footplate or the atresia plate (r=0.099, P=0.852). The preoperative and postoperative bone conduction threshold (BC) was positively correlated with the thickness of the stapes footplate or the atresia plate (r=0.903, P=0.014; r=0.907, P=0.013). The preoperative and postoperative air conduction threshold (AC) was not correlated with the thickness of the stapes footplate or the atresia plate (r=0.167, P=0.752; r=0.732, P=0.098). Conclusions: MS surgery has a safe and reliable curative effect on patients with congenital ossicular malformations. As long as the insertion depth and angle of Piston are within a certain reasonable range, their impact on postoperative hearing is not significant. There is a correlation between the thickness of the stapes footplate or the oval window atresia plateand the bone conduction threshold.

Oval window atresia (OWA) is a rare otologic condition often associated with a maximal conductive hearing loss, and variable ossicular and facial nerve canal (FNC) anomalies, which have contributed to suboptimal middle ear surgical outcomes. No grading scheme exists to detail the spectrum of associated temporal bone anomalies in OWA; therefore, our objectives were to complete an audiometric and radiographic review to characterize audiometric patterns of hearing loss, and refine the classification system for OWA to determine suitability for middle ear surgery. A retrospective audiometric and radiographic review was conducted at a pediatric tertiary care institution. Patients with OWA identified on temporal bone computerized tomography (CT) scans obtained from 01/2010 to 06/2024 were included. Audiological, radiological, and patient factors were analyzed. Thirty-one patients (48 ears) with OWA were identified. Across frequencies, the air-bone gap decreased significantly as frequency increased (ANOVA with pairwise comparisons, p < 0.001) due to a worsening of bone conduction thresholds and improvement in air conduction thresholds. The FNC was abnormal in 43/48 ears and was determined to overlay the oval window in 6 ears. Additional anomalies included inferiorly displaced, dehiscent, and duplicated canals. Ossicular anomalies were reported in 46/48 ears, and stapedial anomalies were most common. Our findings indicate OWA may manifest audiometrically with consistent and specific hearing loss characterized by a 60-80 dB ABG at lower frequencies that decreases above 2 kHz. CT findings of OWA show considerable variability. We propose a new classification system for OWA based on facial nerve position as this directly influences middle ear surgical feasibility.

Introduction: Middle ear malformations are considered the main causes of hearing loss, and both the origins and the correlation of middle ear structures are not yet fully understood. Objectives and Methods: description of a tomographic study of a case of bilateral hearing loss. Results and Discussions: The study shows the absence of stapes with bilateral oval window atresia, associated with facial nerves with anomalous paths, medialized and positioned adjacent to the expected site for the oval windows. Conclusion: This study demonstrated a possible relationship between the embryogenesis of the stapes and its positioning in the proper formation of the oval window, as well as the correlation of these structures with the anatomical path of the facial nerve through the topography of the middle ear.

Background In some rare cases of congenital aplasia of the oval window (OW), malformed facial nerve (FN) locations covering the most or entire OW present a challenge to hearing reconstruction, there is no a highly effective surgical hearing reconstruction methods. Aims/objectives To update a Scala tympani drill-out technique (SDT) for abnormal FN course covering the OW. Material and methods All patients of congenital atresia of the OW was recruited between August 2014 and July 2023 in a tertiary-care center. When it’s inability to perform the vestibulotomy with FN covering the OW, The SDT surgery was made with a titanium TORP between the tympanic membrane and scala tympani fenestration for hearing reconstruction. Air conduction (AC) thresholds, bone conduction (BC) thresholds, and air-bone gap (ABG) at 0.5, 1, 2, 4 kHz pure tone frequencies were compared before and 3 months after surgery. Results A total of 11 patients underwent SDT surgery during the study period. All cases showed no bone conduction (BC) hearing loss, facial paralysis, tinnitus, or dizziness. The ABG has decreased by 21.2 ± 10.8 dB 3 month after operation. In the long term, three cases had the same hearing as pre-operative, three cases felt their hearing gradually decreased but better than pre-operative. Conclusions and significance As a new and optional method, our results suggest an effective way to reconstruct hearing for middle ear deformities with FN occlusion, the short-term effect can be confirmed, while the long-term effect is mixed, and a large amount of clinical research is still needed.

Objective: To summarize the clinical features and postoperative efficacy of patients with oval window atresia accompanied by facial nerve aberration. Methods: The clinical data of patients with congenital middle ear malformation with facial nerve aberration admitted to our hospital from January 2015 to March 2023 were retrospectively analyzed. There were 97 cases (133 ears) in total. Among them, 39 patients (44 ears) had complete follow-up data, including 27 male patients and 12 females, aged 7-48 years old, with an average age of 17.8 years old. Of these, 14 cases (16 ears) were patients combined with facial nerve aberration, and 25 cases (28 ears) were without facial nerve aberration. The results of imaging examination, pure-tone audiometry, selection of surgical strategy, intraoperative findings and postoperative hearing improvement were summarized and analyzed. The malformations of malleus, incus, stapes, oval window and facial nerve were recorded. Prism 9 software was used to statistically analyze the mean bone conductance and air-bone gap of patients before and after surgery. Results: All the 14 patients (16 ears) with middle ear malformation accompanied by facial nerve aberration and oval window atresia showed poor hearing and no facial palsy since childhood. High resolution CT (HRCT) examination of temporal bone, pure tone audiometry and Gelle test were performed before surgery. The malformations of malleus, incus, stapes, oval window and facial nerve were recorded. Preoperative high-resolution CT (HRCT) examination of temporal bone found 12 ears with 4 or more deformities, accounting for 75.00%, in the group of patients with facial nerve malformation. The preoperative average bone conductive threshold was (15.3±10.4) dB and the average air-bone gap was (46.3±10.6) dB in pure-tone audiometry (0.5, 1, 2, 4kHz). According to the different degrees of facial nerve and ossicle malformation, we performed three different hearing reconstruction strategies for the 14 patients (16 ears) with facial nerve aberration and oval window atresia, including 7 ears of incus bypass artificial stape implantation, 7 ears of Malleostapedotomy (MS) and 2 ears of Malleus-cochlear-prothesis (MCP). After 3 months to 18 months of follow-up, all patients showed no facial paralysis. The postoperative mean bone conductive threshold was (15.7±7.9) dB and air-bone gap was (19.8±8.5) dB. There were significant differences in mean air-bone gap before and after operation (t=7.766, P<0.05), and there was no significant difference between the mean bone conductive threshold before and after surgery (t=0.225, P=0.824). There was no significant difference of mean reduction of air-bone gap between patients with and without facial nerve aberration (t=1.412, P=0.165). There was no significant difference between the three hearing reconstruction strategies. There was no significant displacement of the Piston examined by U-HRCT. Conclusion: For patients of middle ear malformation whose facial nerve cover the oval window partially, incus bypass artificial stape implantation or Malleostapedotomy (MS) can be selected according to the specific condition of auditory ossis malformation, and for patients whose facial nerve completely covers the oval window area, Malleus-cochlear-prothesis (MCP) can be selected. Three types of stapes surgery are safe and reliable for patients with oval window atresia accompanied by facial nerve aberration. There was no significant difference in efficacy between them. Preoperative HRCT assessment of middle ear malformation is effective. There is no significant difference of surgical effect with or without facial nerve aberration. The U-HRCT can be used to evaluate the middle ear malformation before surgery and the Piston implantation status after surgery. Due to the risks of surgery, those who do not want to undergo surgery can choose artificial hearing AIDS, such as hearing aid, vibrating soundbridge, bone bridge or bone-anchored hearing aid.

Objective: To analyze the etiology, diagnosis, and treatment of unexplained conductive hearing loss (UCHL) with intact tympanic membrane. Methods: A systematic review was conducted based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 642 articles were retrieved from databases such as PubMed, Embase, Web of Science, and Cochrane. Fifty-four research articles and 21 case reports were screened out according to the inclusion and exclusion criteria for analysis of the etiology of UCHL. Seven research articles with UCHL who underwent exploratory tympanotomy were selected for data extraction and analysis of clinical characteristics. Results: UCHL is a common manifestation of various diseases, including congenital ossicular anomalies (COA), otosclerosis (OTS), congenital middle ear cholesteatoma (CMEC), oval window atresia, superior semicircular-canal dehiscence, congenital stapedial footplate fixation, middle ear osteoma or adenoma, congenital ossification of stapedial tendon, and so on. A total of 522 patients were included in the 7 articles; among whom OTS showed a tendency to increase with age. The main symptoms were hearing loss, followed by tinnitus, dizziness, ear fullness, ear pain, facial paralysis. A total of 87.5% to 93.0% patients with COA manifested as nonprogressive deafness that occurred since childhood, with tinnitus incidence of 15.6% to 30.2%, and 86.4% to 96.4% patients with OTS presented with progressive hearing loss, with tinnitus incidence of 60.1% to 90.9%. The diagnosis positive rate of high-resolution computed tomography (HRCT) was 33.8% to 87.1%, and CMEC was higher than that of COA (83.3%-100% vs 28.6%-64%). All the articles reported good hearing recovery. The most common surgical complications included taste abnormalities, tinnitus, and dizziness. Conclusion: UCHL presents with similar clinical manifestations and poses challenges in preoperative diagnosis. Exploratory tympanotomy is the primary method for diagnosis and treatment, with good prognosis after removing the lesion and reconstructing hearing during the operation. Children can also safely undergo the surgery.

Department of Otolaryngology–Head and Neck Surgery, University of Iowa, Iowa City, Iowa Address correspondence and reprint requests to Alexander D. Claussen, M.D., Department of Otolaryngology–Head and Neck Surgery, University of Iowa, 200 Hawkins Drive, Iowa City, IA 52246; E-mail: [email protected] Financial disclosure: This project was supported in part by NIH-NIDCD grant no. 3T32DC000040 (MJK). Declaration of interest: The authors disclose no conflicts of interest.

"Cement Bridge Over Troubled Incus" Technique in Patients With Oval Window Atresia and Anomalous Incus: A Preliminary Report.

Objective: To conclude the clinical features and the postoperative efficacy of congenital middle ear malformation treated with Malleostapedotomy (MS), and to explore the security and effectiveness of MS surgery. Methods: The clinical data of 17 patients (18 ears) with congenital middle ear malformation undergoing MS procedure were analyzed. There were 10 males (11 ears) and 7 females (7 ears), aged from 7 to 48 years. The imaging examination, pure-tone audiometry, intraoperative findings and postoperative hearing improvement of these patients were analyzed and summarized, and software SPSS23.0 was used for statistical analysis. Rusults All the 17 patients (18 ears) presented with hearing loss since childhood on the affected sides. Preoperative high resolution CT (HRCT) of the temporal bone revealed definite malformations in 9 ears (6 ears with incus long process dysplasia and 3 ears with anterior and posterior crus dysplasia). Before surgery, the mean bone conductive hearing threshold at 500, 1 000, 2 000 and 4 000 Hz was (15.6±10.2) dB HL, the mean air conductive hearing threshold was (60.6±9.7) dB HL, and the mean air-bone gap was (45.0±8.9) dB. During the surgery, all 18 ears were found to be accompanied by absence or hypoplasia of incus long process. 12 ears had stapes fixation, 6 ears had oval window atresia. All patients were treated with MS procedure by using Piston. The patients were followed up for 3 months to 1 year. The mean bone conductive hearing threshold was (14.7±8.8) dB HL. The mean air conductive hearing threshold was (37.7±11.6) dB HL, and the mean air-bone gap was (23.0±8.0) dB. There were statistically significant differences in the mean air conductive hearing threshold and mean air-bone gap before and after surgery (P<0.05). While there were no statistically significant differences in the mean bone conductive hearing threshold before and after surgery (P=0.550). Conclusions: MS procedure is safe and reliable in patients with congenital middle ear malformation of incus long process dysplasia, stapes fixation or oval window atresia. HRCT is useful in evaluating the major deformity of ossicular chain and facial nerve deformity. However, it is not enough to evaluate the joint of incus-stapes and oval window atresia. MS surgery in middle ear malformation requires advanced surgical experience and skills. The hearing improvement can be significant, even though some air-bone gap after surgery exist.

Isolated oval window atresia (OWA) is a rare cause of congenital conductive middle ear deafness and may be overlooked owing to the normal appearance of the external ear. This anomaly has been previously described, although the published numbers with both imaging and surgical findings are few. Our aim is to correlate the imaging features of OWA with intraoperative findings. This is a single-centre retrospective evaluation of patients who were diagnosed with OWA and who received surgery from January 1999 to July 2006. No new case was diagnosed after 2006 to the time of preparation of this manuscript. High resolution computed tomography (HRCT) imaging of the temporal bones of the patients were retrospectively evaluated by 2 head and neck radiologists. Images were evaluated for the absence of the oval window, ossicular chain abnormalities, position of the facial nerve canal, and other malformations. Imaging findings were then correlated with surgical findings. A total of 9 ears in 7 patients (two of whom with bilateral lesions) had surgery for OWA. All patients had concomitant findings of absent stapes footplate with normal, deformed or absent stapes superstructure and an inferiorly displaced facial nerve canal. HRCT was sensitive in identifying OWA and associated ossicular chain and facial nerve abnormalities, which were documented surgically. OWA is a rare entity that can be diagnosed with certainty on HRCT, best visualised on coronal plane. Imaging findings of associated middle ear abnormalities, position of the facial nerve canal, which is invariably mal-positioned, and associated deformity of the incus are important for presurgical planning and consent.

Bilateral Congenital Oval Window Atresia: A Case Report

The purpose of this study is to categorize anomalous tympanic facial nerve (FN) on high-resolution computed tomography (HRCT) and to determinate the significance of associated temporal bone anomalies and congenital syndromes without microtia in patients with hearing loss. A retrospective analysis of HRCT findings in 30 temporal bones in 18 patients with anomalous FN was performed. Abnormalities of the tympanic FN were categorized as follows: category 1: FN medially positioned, but above the oval window; category 2: FN in the oval window niche; and category 3: FN below the oval window. Potential associated findings that were assessed included stapes abnormalities, oval window atresia, and inner ear anomalies, as well as the presence of a known congenital syndrome with hearing loss. The most common type of anomalous tympanic FN was category 1 (67%, n = 20), following by group 2 (20%, n = 6) and group 3 (13%, n = 4). Stapes anomalies were detected in 77% of temporal bones (n = 23), oval window atresia was detected in 43% of temporal bones (n = 13), and inner ear anomalies were detected in 70% of temporal bones (n = 21). Anomalous tympanic facial nerves in temporal bone with conductive hearing loss were often (60%) not associated with oval window atresia. The combination of aberrant tympanic FN and inner ear anomalies was significantly (P = .038) associated with a known congenital syndrome (6 patients), including CHARGE syndrome, oculo-auriculo-vertebral spectrum, Pierre-Robin sequences, and Down syndrome. Therefore, an anomalous tympanic FN in conjunction with inner ear anomalies appears to be a biomarker for certain congenital syndromes with hearing loss in the absence of microtia.

Background: Simple or non-syndromic types of oval window (OW) or round window (RW) atresia are relatively rare in clinical. Few studies have assessed bone conduction (BC) hearing in OW or RW atresia patients, with some reporting that BC hearing lies within the normal range, whereas others observing impaired BC hearing.Aims/Objectives: This study explored the effect of blocking the OW and RW during BC in cat models.Material and Methods: Twenty-four cats were randomly divided into three immobilization groups (OW blockage, RW blockage, and OW + RW blockage) and control group. Each immobilization group also had the initial control state before blockage. Medical adhesive and ear mould glue were used to immobilise the stapes footplate and RW, respectively. Comparisons were made of the auditory brainstem response (ABR) thresholds before and after immobilization for the three immobilization groups during three different stimuli [air conduction (AC) click, BC click, and BC pure tones].Results: The AC click thresholds increased after immobilisation in three experimental groups compared to the control group (p < .05). The AC click thresholds increased compared to their initial control state after all three immobilization groups (p < .05). With an increase in frequency from 2 to 8 kHz, there was a general decrease in the difference between pre- and post-immobilization BC hearing thresholds in all three immobilization groups. The BC click threshold and BC tone thresholds at 2–4 kHz in both OW blockage and OW + RW blockage groups exceeded those in RW blockage group (p < .05).Conclusions and Significance: The use of medical adhesive and ear mould glue for the blockages of OW and RW, respectively in cats was feasible. The effect of blocking the OW and RW in BC hearing was larger at low frequencies than high frequencies between 2 and 8 kHz. OW blockage had a greater effect than RW blockage on BC hearing at 2–4 kHz range.

Objective:To investigate the surgical effects of round window implantation of vibrant soundbridge(VSB)for patients with congenital oval window atresia.Method:Retrospectively analyze the clinical information of 9 cases of congenital oval window atresia as well as the pre-and post-operative hearing and speech results. Three of them had undergone or attempted the vestibulotomy but obtained poor results. All cases underwent round window implantation of VSB. Result:All patients obtained an improvement of 21-33 dBHL after implantation. In the speech recognition rate test, the average increase of disyllabic words was 62%, and 60% in the sentence test. During the follow-up of 51 months in average, 2 cases had a decline of auditory benefit and finally 1 case regained the improvement after reoperation. Conclusion:Round window implantation can get a fairly good result in congenital oval windows atresia cases.

∗Department of ENT-Head and Neck Surgery †Department of Radiology, Ng Teng Fong General Hospital, Singapore, Singapore Address correspondence and reprint requests to Jiaying Liu, M.B.B.S., M.R.C.S., M.Med., F.A.M.S., Department of ENT-Head & Neck Surgery, Ng Teng Fong General Hospital, 1 Jurong East Street 21, Singapore 609606, Singapore. E-mail: [email protected] All three authors disclose no conflicts of interest.

Intracranial vascular, bone, and brain abnormalities might be observed in oculo-auriculo-vertebral spectrum (OAVS) patients even though these structures do not derive embryologically from the first and second pharyngeal arches. This neuroimaging study investigated their type, frequency and phenotype correlations among OAVS patients. Thirty-five OAVS patients (mean-age 4.8±6.0years; age range 0.3-30, 14 females) underwent head CT (all patients) and brain MRI (32 patients); 19 patients had a more severe phenotype (Goldenhar syndrome). Internal carotid artery hypoplasia/agenesia and abnormal course, brain abnormalities, internal acoustic canal stenosis/aplasia, cochlear-vestibular malformations, facial nerve bony canal anomalies, and oval window atresia were recorded. Nine of 35 (26%) OAVS patients showed internal carotid artery anomalies; 18/32 (56%) had protean brain MRI abnormalities, ranging from tegmental cap anomaly to mild ventriculomegaly, which were associated with Goldenhar syndrome (p<0.001) and concomitant cranial nerve abnormalities (p=0.004); 11/35 (31%) disclosed cochlear-vestibular abnormalities including Michel deformity, common cavity, cochlear hypoplasia, and incomplete partition type-1 that were associated with Goldenhar syndrome (p=0.01) and ipsilateral VIII cranial nerve abnormalities (p<0.001); 16/35 (46%, 23 sides) presented facial nerve bony canal abnormalities that were associated with Goldenhar syndrome (p<0.001) and ipsilateral VII cranial nerve (p<0.001) and cochlear-vestibular (p<0.001) abnormalities; and 23/31 (74%, 31 sides) showed oval window atresia, always with concomitant ossicular chain dysplasia. Intracranial vascular, bone, and brain abnormalities in OAVS patients are strikingly common and heterogeneous. As their detection might impact significantly on clinical and surgical management of affected patients, accurate neuroimaging investigations should be included in the diagnostic work-up of OAVS.

Scala tympani drill-out technique for oval window atresia with malformed facial nerve: A report of three cases

Conclusion: Application of the Vibrant Soundbridge to the round window (RW) membrane can be utilized as an efficient therapy for congenital oval window (OW) atresia. Objective: To report the surgical technique and auditory outcome of an active middle ear implant (AMEI) system used in patients with congenital OW atresia. Methods: Nine subjects with congenital OW atresia (six males and three females, ranging in age from 5.5 to 25 years, average 12.5 years) were implanted with an AMEI (Vibrant Soundbridge) at the round window (RW-Vibroplasty). Five cases were diagnosed as having isolated congenital OW atresia while four patients presented with combined external/middle ear malformation. Results: An improvement of 30 dB in average pure-tone air conduction thresholds (0.5–4 kHz) was achieved, with the high frequencies showing greater results. The subjects achieved postoperative speech recognition scores of 80–100% on the Computerized Mandarin Speech Test System (CMSTS) sentence test. Bone conduction thresholds were confirmed as stable in all subjects postoperatively. Decline in auditory benefit was noticed in two subjects, who then underwent revision surgery. One of these revision surgery patients then experienced stable hearing recovery, while the other patient’s hearing declined.

The occurrence of oval window atresia is a rare anomaly with conductive hearing loss. Traditional atresia surgeries involve challenging surgical techniques with risks of irreversible inner ear damage. Recent reports on Bonebridge (Medel, Innsbruck, Austria), a novel implantable bone conduction hearing aid system, assert that the device is safe and effective for conductive hearing loss. We present a case of Bonebridge implantation in an eight-year-old girl with bilateral oval window atresia.