Effect of malleostapedotomy procedure on 17 cases of congenital middle ear malformation

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.

To compare preoperative temporal bone high-resolution computed tomography (HRCT) readings to intraoperative findings during exploratory tympanotomy for suspected cases of isolated congenital middle ear malformations (CMEMs) and summarize the malformations that can and cannot be diagnosed with HRCT. A retrospective study was conducted. All cases were confirmed as isolated CMEMs during surgery. Detailed clinical records were reviewed, with a focus on imaging and surgical findings. One hundred and thirty-two patients and 145 ears were reviewed. Ninety cases (62.1%) could be identified as isolated CMEMs and at least one as middle ear anomaly using preoperative HRCT. Fifty-five cases (37.9%) were reported to be completely normal and the patients underwent exploratory tympanotomy to determine the final diagnosis. Stapes fixation, either alone or associated with other ossicular chain anomalies, contributed to 53.1% of the cases. Most cases of aplasia or dysplasia of the ossicular chain, for example, aplasia/dysplasia of the long process of the incus, aplasia of the stapes' superstructure, and atresia of the oval window were easily identified in preoperative HRCT. However, fixation of the ossicular chain can be elusive in HRCT, and exploratory tympanotomy is needed for a definitive diagnosis. HRCT provides helpful preoperative clinical information in CMEM and may obviate the need for middle ear exploration in some cases. The negative findings (anomalies that are difficult to identify through preoperative HRCT) and the positive findings (anomalies that are relatively easy to identify through preoperative HRCT) were summarized.

To describe the operative findings and surgical results of unilateral congenital middle ear malformations with intact external ear. A retrospective review was performed on 64 patients with unilateral congenital middle ear malformations and intact external ear who underwent exploratory tympanotomy from 2011 to 2016. Demographic data, clinical data, high-resolution computed tomography findings, audiometric data and intraoperative findings were collected. Audiological evaluations before and 6months after surgery were analyzed in 47 patients. The most common malformation were mobile stapes with missing incus long process and stapes suprastructure. The air conduction pure tone average was 58.9 ± 10.5dB HL (range 34.4-78.1dB HL) preoperatively and 28.8 ± 10.6dB HL (range 9.4-55.6dB HL) postoperatively (P = 0.000). Twenty-five cases (53.2%) acquired an air conduction hearing gain exceeding 30dB. Mean air-bone gap (ABG) was 44.5 ± 9.4dB (range 22.5-66.4dB HL) before surgery and 15.6 ± 9.3dB (range 0-35.6dB) after surgery (P = 0.000) for an average gain of 28.8 ± 11.5dB. Thirty-four cases (72.3%) showed a postoperative ABG of less than 20dB, 15 had an ABG within 10dB, and 4 had 0dB ABG after operation. No significant difference was observed for air conduction hearing gain regarding age (P = 0.261) or types of malformations (mobile stapes footplate with or without a suprastructure anomaly, P = 0.058). Unilateral congenital middle ear malformations with intact external ear can be complex and diverse. Functional ossiculoplasty for patients with unilateral congenital middle ear malformations can achieve good hearing outcomes.

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.

To evaluate hearing outcomes and postoperative complications among patients with middle and external ear malformations undergoing active middle ear implantation with Vibrant Soundbridge® (VSB). Review of the literature. Studies published in English, Portuguese, or Spanish at the following databases: PubMed, MEDLINE, Scopus, Web of Science, EMBASE, and Cochrane Library were searched. The search strategy yielded a total of 141 potentially relevant studies. Of these, ten were included in this analysis. The mean preoperative air conduction threshold was 66.7 ± 6.2 dB. The mean air-bone gap was 46 ± 7.7 dB. VSB implantation resulted in mean hearing gain of 40.5 ± 7.1 dB in the air-conduction thresholds among the evaluated frequencies. The speech recognition index if the Floating Mass Transducer (FMT) was placed in the short process was 86.0% ± 9.6%, with significant difference when compared to long process coupling (p = 0.035) and the round window coupling (p = 0.048). Bone conduction thresholds did not worsen in any of the studies included in the present review. VSB implantation resulted in a mean hearing gain of 40 dB at air conduction thresholds.

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.

ObjectivesExtended high frequency (EHF) audiometry is the recommended method for monitoring oxotoxic hearing loss in children. This study aims to provide high quality reference audiological data for the EHF range in healthy children. MethodsParticipants were 126 healthy schoolchildren between 6 and 14 years of age. All participants were term born with normal birthweight, had not suffered severe neonatal illness and had no history of middle ear disease. ResultsThe averaged mean (SD) hearing threshold for the EHF 9, 10, 11.2, 12.5, 14 and 16 kHz was −0.4 (6.0) dB HL. The lowest mean hearing thresholds were observed at 14 kHz with −4.2 (8.7) dB and at 16 kHz with −6.4 (12.1) dB HL. We found significantly lower thresholds at 16 kHz for children aged 6–9 years (−8.7 dB HL) compared to age 10–14 years (−3.9 db HL), p 0.042. For both age groups the inter-subject variability increased in the highest frequencies. We found no significant differences in mean hearing thresholds between right and left ears at any frequency, and no gender differences in the EHF range. ConclusionOur findings support that decreased hearing sensitivity in the EHF's may start around or even before the age of 10 years. In order to use EHF audiometry for ototoxic monitoring in children, we suggest to establish an international reference standard for hearing levels in children under the age of 18. Specific references for different age groups are needed as hearing in the EHF range appears to gradually deteriorate from an early age. Clinical trial registrationNCT03253614.

The clinical data of 47 children (52 ears) with middle ear cholesteatoma at Eye & ENT Hospital, Fudan University from January 2021 to February 2024 were prospectively collected. There were 30 males and 17 females, with a mean age of (8.5±2.8) years. Surgical procedures included endoscopic tympanoplasty (29 ears), combined endoscopic/microscopic tympanoplasty (8 ears) and microscopic tympanoplasty (15 ears). After endoscopic surgeries, the mean air conduction hearing threshold, mean bone conduction hearing threshold and air bone gap were (28.4±10.4) dB HL, (12.4±5.9) dB HL and (16.0±6.3) dB HL, respectively, which were significantly improved compared with preoperative indicators [(40.2±14.0) dB HL, (16.3±6.8) dB HL and (23.9±9.8) dB HL, respectively] (all P<0.05). There were statistically significant differences in the postoperative mean air conduction hearing threshold and air bone gap among three surgical groups, and the value of endoscope group was lower than those of microscope group and endoscope combined with microscope group (all P<0.05). After a follow-up of 5.6 (1.2, 11.5) months, no statistically significant differences were observed in the rate of successful tympanic membrane healing between endoscope group and microscope group (P=0.747). One ear in the endoscope group experienced recurrence three years after surgery, and no intracranial and extracranial complications occurred. The current study indicates that endoscopic surgery for pediatric cholesteatoma improves hearing with good results.

Objective: To investigate the clinical features of patients with congenital atresia of the oval window (CAOW). Methods: A retrospective analysis was conducted on 7 cases (8 ears) of surgically confirmed CAOW treated at our department from July 2018 to July 2024. Among the cases, 1 patient had bilateral CAOW, and 4 patients had unilateral CAOW combined with other types of ossicular chain malformations in the contralateral ear. We collected and analyzed the clinical data, audiological features, and temporal bone HRCT results of all patients. Results: The 7 patients were diagnosed at ages ranging from 8 to 19 years, with a mean age of (13.2±6.9) years. None of the patients exhibited significant auricular deformities. All presented with conductive hearing loss or mixed hearing loss predominantly of the conductive type, with an intact tympanic membrane. The diagnosis of CAOW was confirmed via endoscopic tympanotomy, revealing a concave oval window area on the medial wall of the tympanic cavity, sealed by a bony plate. All 8 ears exhibited additional ossicular chain deformities. Stapes absence was present in all 8 ears. Partial absence of the incus long process was observed in 3 ears, while, abnormal bony connections between the incus long process and the promontory were seen in 4 ears, 1 ear had a short malleolar handle, 1 ear had a smaller than normal malleus volume. In addition, facial nerve deformities were found in 6 ears, with 4 ears showing bifurcation of the facial nerve and 2 ears showing facial nerve obscuration of the oval window. Pure-tone audiometry revealed that 62.5% (5/8 ears) of patients had air conduction (AC) thresholds≥60 dB preoperatively, with a mean pure-tone average (PTA) of (69.0±11.8) dB HL and a mean air-bone gap (ABG) of (52.0±7.0) dB. The mean AC threshold and ABG were higher in the low-frequency (125-1 000 Hz) range compared to the high-frequency (2 000-8 000 Hz) range (both P<0.05). Preoperative HRCT showed abnormalities in all patients, with 7 ears being diagnosable as CAOW. Although the remaining 1 ear could not be diagnosed as CAOW, stapes and incus long process absence were detected. Conclusion: CAOW is rare in clinical, as the patients with non-progressive conductive hearing loss (AC≥60 dB, ABG≥50 dB) since childhood, intact tympanic membrane without malformations of auricle and external auditory canal, and thick bony plate covered the oval window of the HRCT imaging, CAOW should be highly suspected, which could be confirmed by the exploratory tympanotomy.

To standardize the information for families of children having functional surgery for middle ear malformations, we describe the audiometric results of the subgroup of patients with the most favorable anatomic conditions: viable auditory canal, intact tympanic membrane, mobile stapes, and corresponding to a Jahrsdoerfer score of 8 or higher. Case series, tertiary referral center. Charts of patients undergoing functional surgery for congenital middle ear malformations were reviewed for demographic data, preoperative Jahrsdoerfer score, ossicular chain status, type of ossiculoplasty, and audiometric data before and 6 months postsurgery. Eighteen consecutive interventions were performed on 13 patients (average age of 9 years, 8 girls and 5 boys) between 2004 and 2011. The ossiculoplasties performed were as follows: incus repositioning (4), double-layer tragal cartilage (5), intact native chain reconstruction (3), and partial ossicular prosthesis (6). Mean air bone gap (ABG) was 40.8 ± 12.4 dB preoperatively and 20.9 ± 12.9 dB postoperatively (p < 0.0001). Preoperative and postoperatively mean air conduction PTA thresholds were 49.9 ± 9.5 and 30.0 ± 14.1 dB, respectively (p < 0.0001). All ears operated on except one had air conduction improvement. There were no complications. Functional surgery for congenital middle ear malformations gives variable hearing outcomes. In this study, with the most favorable anatomic conditions, 12 ears (67%) of 18 had air conduction improvement below 30 dB.

View Video S1 Laryngoscope, 131:E961–E965, 2021

Background and ObjectivesZZThe aim of this study was to analyze the results of malleostapedotomy as primary surgical procedure in stapes fixation. Subjects and MethodZZThis study was a retrospective chart review of 12 patients who underwent primary malleostapedotomy for conductive hearing loss. The intraoperative findings, surgical outcomes including audiologic data and complications were analyzed. ResultsZZNine patients had ossicular fixation with ossicular anomalies and 3 patients had ossicular fixation alone. The median length of piston wire was 5.5 mm in total length. The preoperative mean bone and air-conduction thresholds were 57.5±8.8 (mean±SD) dB, 19.7±10.3 dB, respectively, and the mean air-bone gap (ABG) was 44.6±13.2 dB. After malleostapedotomy, hearings were improved and mean postoperative ABG was 11.1±11.3 dB. In eight patients (66.7%), ABG was reduced to 20 dB or less. There was no intraoperative or postoperative complication except for mild postoperative vertigo for 1 or 2 days. ConclusionZZMalleostapedotomy can be a safe and effective surgical procedure as an alternative of incus stapedotomy in certain cases of absence or anomalous incus long process, and/or immobile incus in patients with stapes fixation. Korean J Otorhinolaryngol-Head Neck Surg 2009;52:961-7 Key WordsZZStapes surgery·Stapes fixation·Conductive hearing loss.

Objective: To analyze the surgical efficacy of patients with mixed hearing loss and otosclerosis with different air bone gap (ABG) before surgery, and to provide reference for the prognosis evaluation of otosclerosis surgery. Methods: The clinical data of 108 cases(116 ears) of otosclerosis who had undergone stapes fenestration technique artificial stapes implantation in Xiangya Hospital of Central South University from November 2013 to May 2020 and had mixed hearing loss before surgery were collected, including 71 women(76 ears)and 37 men (40 ears), with an average age of 38.5 years. According to preoperative pure tone audiometry ABG, they were divided into three groups: group S, 15 dB≤ABG<31 dB, a total of 39 ears; group M, 31 dB≤ABG<46 dB, a total of 58 ears; and group L, ABG≥46 dB, 19 ears in total. The hearing outcomes of three groups of patients at 6-12 months after surgery were compared and analyzed using SPSS 24.0 statistical software. Results: A total of 3 patients (group S: 2 cases; group L: 1 case) experienced severe sensorineural hearing loss after surgery and were not included in the statistical analysis. After surgery, the pure tone hearing threshold of patients with otosclerosis in each group was significantly improved compared to before surgery, with an average air conduction threshold improvement of(21.6±13.4) dB. The difference between before and after surgery was statistically significant(t=17.13, P<0.01). The average bone conduction threshold improved by(3.7±7.6) dB, and the difference was statistically significant before and after surgery(t=5.20, P<0.01). The postoperative ABG was(18.3±9.3) dB, which was significantly reduced compared to preoperative(36.2±8.6)dB. Among the three groups of patients, the L group had the highest improvement in air conduction threshold[(29.9±10.8)dB], while the S group had the lowest improvement[(15.7±11.4)dB]. There was no statistically significant difference in post operative pure tone hearing thresholds between the three groups(P>0.05). The postoperative ABG in group S was the smallest[(16.5±9.0)dB], while in group L, the postoperative ABG was the largest[(20.5±10.0)dB]. Compared with group S, group M and group L still had a large residual ABG at 2 000 Hz after surgery. The bone conduction threshold of both S and M groups improved to some extent after surgery compared to before (P<0.01). Conclusions: Surgery can benefit patients with mixed hearing loss and otosclerosis with different preoperative ABG. Patients with small preoperative ABG have better surgical results and ideal ABG closure at all frequencies after surgery. Patients with large preoperative ABG can significantly increase the gas conduction threshold during surgery, but certain frequencies of ABG may still be left behind after surgery. The improvement effect of surgery on bone conduction threshold is not significant. Patients should be informed of treatment methods such as hearing aids based on their actual situation for selection.

To investigate hearing outcomes after stapes surgery in children with stapes fixation. Retrospective study. Tertiary referral center. Forty-nine patients (66 ears) aged less than 15 years who received stapes surgery. Stapes surgery. Preoperative symptoms, bilateral involvement, pure-tone hearing levels, and perioperative complications were analyzed using paired t test and Mann-Whitney U test. The chief complaint of all patients was hearing disturbance. Sixteen ears were diagnosed with stapes fixation and an additional congenital ossicular anomaly and 50 ears had only stapes fixation. Preoperative mean bone conduction and air conduction thresholds were 12.0 ± 5.8 dB and 60.9 ± 10.9 dB, respectively. The mean air-bone gap (ABG) was 48.9 ± 12.0 dB in patients with stapes fixation and an ossicular anomaly. The postoperative mean ABG was 23.6 ± 14.5 dB, and the ABG closure was 25.3 ± 18.2 dB. In patients with stapes fixation only, the preoperative mean bone conduction and air conduction thresholds were 14.3 ± 7.5 dB and 49.6 ± 9.5 dB, respectively, and the mean ABG was 35.5 ± 9.6 dB. The postoperative mean ABG was 14.4 ± 10.3 dB, and the ABG closure was 16.2 ± 16.1 dB. The successful results (ABG <20 dB) were 75.8% overall, 56.3% for fixation and an ossicular anomaly, and 82.0% for fixation only. In children with stapes fixation, hearing loss was worse when the fixation was combined with an ossicular anomaly. Ossicular continuity, especially of the incus, is the most important factor for successful stapes surgery. Appropriate diagnosis and surgical intervention can lead to good results for children with stapes fixation.

Objectives: To assess hearing gain after successful myringoplasty in relation to the size of tympanic membrane perforation.&#x0D; Methods: This cross-sectional study was done in the department of otolaryngology and head neck surgery, BSMMU, Sahbag, Dhaka during the period of January 2009 to December 2010.A total of 60 patients were under went myringoplasty operation after taking detailed history, clinical examination and investigation. Preoperative and postoperative hearing assessment was done. Analysed data presented by various tables, graphics and figures.&#x0D; Results: In case of small size perforation preoperative mean bone conduction threshold was 7.66 dB, mean air conduction threshold was 34.14 dB and mean air bone gap was 26.48 dB. In case of medium size perforation preoperative mean bone conduction threshold was 9.61 dB, mean air conduction threshold was 44.48 dB. Mean air bone gap was 34.87 dB. In case of large size perforation preoperative mean bone conduction threshold was 13.12 dB, mean air conduction threshold was 59 dB, and mean air bone gap was 45.88 dB. Hearing loss increases with increasing size of perforation. Ahmed and Rahim (1979) showed in the study that hearing loss increases with increasing the size of the perforation which was relevant in the study. After myringoplasty post-operative mean air bone gap was 21.24 dB in small size, 21.74 dB in medium sized and 24 dB in large size. From the record improvement of mean air bone gap or hearing gain was 5.24 dB in small size perforation respectively. The different of air bone gap closure between small and medium size perforation was statistically significant by unpaired’ test.&#x0D; Conclusion: Hearing gain after myringoplasty is better in large size perforation.&#x0D; Bangladesh J Otorhinolaryngol; April 2019; 25(1): 54-59