Congenital Middle Ear Research Articles

Selection of inner ear fenestration strategy and surgical effect of patients with oval window atresia accompanied by facial nerve aberration

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.

Distinct audiometric patterns in congenital round window atresia: A comparative study with common congenital middle ear anomalies

ObjectiveLittle 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). MethodsLiterature 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. ResultsAverage 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. ConclusionCRWA 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.

Diagnosis and Management of Unexplained Conductive Hearing Loss With Intact Tympanic Membrane: A Systematic Review.

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.

Treatment of congenital middle ear cholesteatoma in children using endoscopic and microscopic ear surgeries: a case series

IntroductionSurgical removal is widely employed in children with congenital middle ear cholesteatoma (CMEC). Here, we report the surgical outcomes of CMEC removal via endoscopic ear surgery (EES) and microscopic ear surgery (MES) in children.MethodsChildren with CMEC who underwent preoperative medical history inquiry, hearing test, endoscopic evaluation, and radiology imaging before receiving EES or MES were included. Postoperative audiological outcomes and recurrence rates were collected.ResultsSeventeen children (20 ears) with stage II-IV CMEC were included. Of those, 11 ears (55.0%) underwent EES, and 9 ears (45.0%) underwent MES. The follow-up time was 35 ± 13.5 months. One child in the EES group with stage III CMEC had a recurrence during the follow-up period. In the EES group, the average minimum diameter of the external auditory canal on the affected side was 5.8 mm (4.3–8.0 mm). No linear association was found between age and the minimum diameter of the external auditory canal.DiscussionEES is a promising treatment option for children with early-stage CMEC because of its low recurrence rate and minimally invasive nature. The minimum diameter of the external auditory canal on the affected side should be meticulously examined when performing EES in children.

Clinical features of congenitally enlarged bony portion of Eustachian tube

Objective:To investigate the clinical features of patients with congenitally enlarged bony portions of the Eustachian tube（ET）. Methods:The medical history, physical examination, hearing test, temporal bone high resolution computed tomography（HRCT） of six patients（nine ears） with congenitally enlarged bony portion of the ET were retrospectively analyzed. Results:Four patients were men and two were women. The minimum, maximum, and average ages were 5, 21, and（14.7±6.4） years, respectively. Three malformations were bilateral and three were left-sided. Three ears had conductive hearing loss（average bone and air conduction thresholds were 13.7 dB and 71.3 dB）, three had mixed hearing loss（average bone and air conduction thresholds were 27.7 dB and 83.7 dB）, and one had extremely severe sensorineural hearing loss. The average maximum length and width of the enlarged bony ET on temporal bone HRCT were（22.61±2.94） mm and（6.50±2.33） mm, respectively. The enlargement was combined with an external auditory canal malformation in six ears, narrow tympanic cavity in six, tympanic antrum malformation in five, ossicular chain malformation in seven, cochlear malformation in six, helicotrema malformation in three, vestibule widening in two, semicircular canal malformation in three, vestibular window malformation in six, facial nerve abnormality in five, internal auditory meatus malformation in two, low middle cranial fossa in eight, and severe internal carotid artery malformation in one. Conclusion:Bony ET enlargement is a rare congenital middle ear malformation which could combined with other ear malformations. Patients can have no ET dysfunction but different patterns of hearing loss. The defect is usually found unintentionally during imaging, and the HRCT of temporal bone is significant.

Expert consensus on the application of bone-conducted hearing device in patients with congenital middle and external ear malformations

Expert consensus on the application of bone-conducted hearing device in patients with congenital middle and external ear malformations

Fusion computed tomography-magnetic resonance imaging scans for pre-operative staging of congenital middle-ear cholesteatoma.

To evaluate if fusion computed tomography-diffusion-weighted magnetic resonance imaging may have a role in the pre-operative assessment of congenital middle-ear cholesteatoma. A retrospective chart review of surgically treated congenital middle-ear cholesteatoma patients over a 2-year timespan was conducted. Pre-operative staging was performed on computed tomography and fusion computed tomography-diffusion-weighted magnetic resonance imaging based on extension of the disease according to the ChOLE classification system and the Potsic classification system. Intra-operative staging was compared to imaging findings to evaluate accuracy of the two imaging modalities in predicting congenital middle-ear cholesteatoma extent. Computed tomography was able to correctly predict congenital middle-ear cholesteatoma extent in three out of six cases according to the ChOLE classification system, all of which were staged as Ch1a and Ch1b on pre-operative computed tomography. Cases in which computed tomography was not able correctly to determine congenital middle-ear cholesteatoma extent were staged as Ch3 on pre-operative computed tomography. Fusion scans correctly determined congenital middle-ear cholesteatoma extent in all cases according to the ChOLE classification. Fusion computed tomography-diffusion-weighted magnetic resonance imaging may be helpful in cases of congenital middle-ear cholesteatoma where pre-operative computed tomography shows mastoid and antrum opacification, in which computed tomography alone may overestimate cholesteatoma extension beyond the level of the lateral semi-circular canal.

Diagnosis and management of bilateral lipoma of the middle ear.

Middle ear lipomas are rare, accounting for less than 1% of all middle ear tumours. To our knowledge there have been only 16 reported patients with middle ear lipoma. Only one of these had bilateral congenital middle ear lipomas. The aim of this review is to comprehensive overview the diagnosis and management of congenital lipomas of the middle ear. A literature review was performed searching multiple data bases, using the key words "middle ear", AND "lipoma", AND "congenital". A total of 116 articles were identified. One hundred were excluded on various criteria, leaving 16 articles for detailed review. Additionally, we report the second case of bilateral congenital middle ear lipoma. Based on this case report and literature review, the incidence of middle ear lipomas is greater in paediatric patients than in adult patients. Middle ear lipomas are distributed evenly between males and females. Three patients had recognised genetic syndromes, while five had prior ventilation tube placement. Clinically patients present with conductive hearing loss and middle ear effusions. In some cases, presence of a white or yellow mass behind the anterior aspect of the tympanic membrane is recognised. The most common location of the masses is in the anterosuperior quadrant of the middle ear, the site of predilection for small congenital cholesteatomas. An MRI scan is most useful in distinguishing between lipoma and cholesteatoma. All cases reported surgical excision of the middle ear lipoma. There was no evidence of recurrence noted up to 4 years follow up. Though rare, congenital lipomas of the middle ear should be a differential diagnosis of a middle ear masses. Early identification can allow surgical lipoma resection with minimal disruption to the ossicular chain. Though there is currently no consensus regarding management, surgical removal and appropriate reconstruction of the ossicular chain if needed, is appropriate.

Surgical results of 29 ears with congenital middle ear anomalies; Microscopic vs. endoscopic ear surgery

ObjectiveAlthough congenital middle ear anomalies include various types of ossicular anomalies, all of these can be treated by ossiculoplasty or stapes surgery. Transcanal endoscopic ear surgery (TEES) is a minimally invasive surgical method for middle ear disease with an excellent surgical view that has been widely adopted worldwide. To determine the efficacy of TEES for middle ear anomalies, we describe the surgical results and compare the hearing outcomes between patients treated by TEES and microscopic ear surgery (MES). MethodsA total of 39 ears with congenital middle ear anomalies were treated surgically at the University Hospital of Kochi Medical School between January 2011 and December 2021. In total, 29 ears of 23 patients were included in the study. Demographics, type of anomaly, surgical methods, pre- and postoperative hearing thresholds, and surgical complications were investigated by retrospective chart review. ResultsOf the 29 ears, 11 were treated by MES and 18 were treated by TEES. There were no differences in sex, age, preoperative hearing thresholds, or rate of stapes surgery between the two groups. The mean air-bone gap improvement was 20.6 dB in the MES group and 28.8 dB in the TEES group; these values were not significantly different. The median operation time was not significantly different between the MES and TEES groups (125 and 130 min, respectively). The improvements of air conduction in class 1 and 2 (stapes surgery) and class 3 (ossiculoplasty) cases were also not different between the groups. ConclusionsTEES achieved comparable hearing outcomes to MES without postauricular or endaural incisions. Further, class 1 and 2 anomalies demonstrated hearing improvement similar to class 3 without major complications. Since the surgical field limited around the ossicular chain, coupled with the fact that the middle ear anomaly itself does not exhibit inflammation leading to unfavorable bleeding, TEES is a feasible surgical procedure for all types of congenital ossicular anomalies.

Bifid Intratemporal facial nerves in an adult without other congenital anomalies.

To describe a case of bifid intratemporal facial nerves without associated middle or inner ear abnormalities encountered on computed tomography (CT) imaging during preoperative preparation for unilateral cochlear implant placement in an adult male with profound sensorineural hearing loss (SNHL). A rare case of bilateral bifid intratemporal facial nerves in an adult male is presented. The finding's impact on approach to safe cochlear implantation is discussed. Bifurcation of the intratemporal facial nerve is rarely seen and is usually associated with congenital middle or inner ear anomalies. A unique case of bilateral bifid intratemporal facial nerves without other middle or inner ear abnormalities was encountered incidentally on CT imaging during preparation for unilateral cochlear implant placement in an adult male with profound SNHL. The nerve was bifid along the mastoid segment with a nerve branch traversing through the facial recess precluding safe traditional approach to cochlear implant placement. Accessory stylomastoid foramina were noted bilaterally. Unilateral subtotal petrosectomy was performed with successful implantation and excellent hearing outcome. No additional clinical or radiographic otologic abnormalities were noted. Abnormal bifurcation of the facial nerve may occur in adults without other middle or inner ear anomalies. This case highlights the importance of independent imaging review by the surgeon and vigilance to potential rare anatomic aberrations of the facial nerve during cochlear implantation. IV.

Etiology analysis and diagnosis of noninflammatory conductive hearing loss in children

Objective:To investigate the etiology, diagnosis and treatment of noninflammatory conductive hearing loss in children. Methods:The clinical data of children patients admitted to the Eye & ENT Hospital of Fudan University from January 2019 to November 2022 were retrospectively analyzed. Results:A total of 179 cases（189 ears） were analyzed. The main symptoms from high to low were: ear tightness, hearing loss, earache, and facial paralysis. The degree of hearing loss was mild in 34 ears（19.5%）, moderate in 70 ears（40.2%）, moderate-severe in 52 ears（29.9%）, severe in 18 ears（10.3%）. The mean hearing threshold of otosclerosis was the highest（63.5±7.8） dB HL, and the mean air-bone gap of ossicular chain malformation was the largest（35.4±9.8） dB HL. The mean hearing threshold of the affected ear was（50.4±14.5） dB HL, and the mean air bone gap was（30.3±10.4） dB HL. After operation, the results were（36.1± 14.5） dB HL and（20.0±8.6） dB HL, respectively. Distribution of surgical methods for ossicular chain reconstruction: 88 ears（46.6%） of TORP, 49 ears（25.9%） of PORP, 8（4.2%） ears of Piston, 9 ears（4.8%） of autogenous ossicular reconstruction, and 35 ears（18.5%） of ossicular chain relaxation. CT diagnostic rate showed more sensitivity to malleus and incus abnormalities, the diagnosis rate of congenital middle ear cholesteatoma was the highest. The mean duration time of diagnosis was（2.2±2.9） years, while the diagnosis of ossicular chain malformation（[5.2±4.2]years）, otosclerosis（[4.4±4.1]years）, tympanosclerosis（[5.4±0.9]years） took longer. Conclusion:In the diagnosis and treatment of noninflammatory conductive hearing loss in children, the combination of detailed medical history, specialized examination and imaging examination can maximize the accuracy of diagnosis and achieve the purpose of personalized comprehensive treatment. Surgical intervention with appropriate timing is important to remove lesions and improve hearing.

Transcanal endoscopic management of isolated congenital middle ear malformations

Background A few studies have reported transcanal endoscopic management of isolated congenital middle ear malformations (CMEMs). Objective The purpose of this study is to describe our surgical experience in endoscopic ear surgery for isolated CMEMs and evaluate the surgical effect of hearing reconstruction. Methods From January 2017 to January 2022, a retrospective study was performed on 36 patients (37 ears) with isolated CMEMs who all underwent endoscopic surgery. Demographic data, high-resolution computed tomography (HRCT) findings, intraoperative findings, surgical management and audiometric data were recorded. Results Anomalies were categorized according to the Teunissen and Cremers classification system: 8 ears were categorized as class I, 8 ears as class II, 19 ears as class III and 2 ears as class IV. The air conduction pure tone average (AC-PTA) of 37 cases was 61.5 ± 8.6 dB preoperatively and 29.6 ± 6.9 dB postoperatively (p < 0.001). The mean preoperative air-bone gap (ABG) significantly decreased from 43.1 ± 8.7 dB to 12.8 ± 5.5 dB postoperatively. 36 of 37 cases (97%) met the criteria for successful operation. Conclusion Isolated CMEMs are mainly manifested as aplasia of the stapes’ superstructure and dysplasia of the long process of the incus. Transcanal endoscopic surgery seems a safe technique for the management of isolated CMEMs.

先天性耳小骨奇形の手術

Transcanal endoscopic ear surgery (TEES) is a minimally invasive surgical treatment technique for middle ear diseases and is recently becoming popular around the world. TEES enables accurate surgical manipulation under a wide magnified, and clear surgical view. We describe the results of surgical treatment for congenital middle ear anomalies by comparing the hearing outcomes between patients treated by TEES and those treated by microscopic ear surgery (MES). Congenital middle ear anomalies were safely treated by TEES, with comparable hearing outcome to those after MES. The surgical field is limited in the tympanic cavity, inflammation is usually absent, and there is little bleeding during the operation; therefore, TEES is a feasible surgical approach for the treatment of congenital middle ear anomalies. However, as middle ear anomalies are diverse, preoperative diagnosis is sometimes difficult, and the surgeon must be adequately prepared in advance for various types of procedures.

Transcanal endoscopic treatment for congenital middle ear cholesteatoma in children.

To investigate the feasibility and efficacy of transcanal endoscopic treatment for congenital middle ear cholesteatoma in children.Eleven children diagnosed with congenital middle ear cholesteatoma, who underwent total ear endoscopic surgery under general anesthesia, were included from the Huazhong University of Science and Technology Union Shenzhen Hospital between January 2016 and December 2020. We retrospectively analyzed their operation process and surgical complications through the surgical video; moreover, we compared the pre- and postoperative hearing outcomes.One child underwent a planned second operation to reconstruct the ossicular chain. At 6 postoperative months, all 11 children underwent reexamination. There was no significant change and a significant decrease in the mean bone and air conduction hearing thresholds, respectively (P > .05 and P < .05); moreover, there was a significant reduction in the air–bone conduction difference (P < .05). Further, the air–bone conduction difference was reduced to >20 dB and >10 dB in 11 and 7 children, respectively. Follow-up of the children did not reveal sensorineural deafness, facial paralysis, and other serious complications; further, there were no cases of recurrence.Transcanal endoscopic treatment for congenital middle ear cholesteatoma in children is feasible, minimally invasive, and functional.

Immunohistochemical localization of d-β-aspartic acid in congenital and acquired middle ear cholesteatoma.

Objective/HypothesisMiddle ear cholesteatoma is characterized by abnormal growth of the keratinizing squamous epithelium of the temporal bone. d‐β‐aspartic acid is the major isomer of d‐aspartic acid found in elderly tissue. We assessed the immunoreactivity to k‐β‐aspartic acid of congenital and acquired middle ear cholesteatomas.Study DesignCase–control studies.Material and MethodsTissue samples were collected from 21 patients comprising 21 ears with congenital middle ear cholesteatoma and 26 patients comprising 29 ears with acquired type. Their clinical and histopathological features were investigated. We divided the middle ear cholesteatoma samples into three layers: the perimatrix, matrix, and cystic contents. The patterns of immunoreactivity to d‐β‐aspartic acid expression were then assessed immunohistochemically.ResultsTwo patterns of immunoreactivity to d‐β‐aspartic acid were detected in middle ear cholesteatoma: infiltrative and diffuse. In congenital middle ear cholesteatoma, d‐β‐aspartic acid expression was observed throughout all the layers (perimatrix, matrix, and cystic contents), and immunoreactivity to d‐β‐aspartic acid was dramatically strong in all layers. The expression levels of d‐β‐aspartic acid to the cystic content and perimatrix were significantly higher in congenital middle ear cholesteatoma than in the acquired type.ConclusionsThis study showed the expression levels of d‐β‐aspartic acid in middle ear cholesteatoma to differ significantly between congenital and acquired middle ear cholesteatoma. Our results indicate that overexpression of d‐β‐aspartic acid is likely to be involved in the pathogenesis of cholesteatoma, and we speculate that d‐β‐aspartic acid could be a novel biomarker for, and a therapeutic target in, congenital and acquired middle ear cholesteatoma.Level of Evidence4

Clinical Effect of Ear Endoscopic Intervention on CMEC Patients and Analysis of the Relationship between ROS, P-Akt, and HIF-1α Expression and the Degree of Bone Destruction.

The clinical efficacy of ear endoscopic intervention in patients with congenital middle ear cholesteatoma (CMEC) is explored, and the relationship between the expression of reactive oxygen species (ROS), phosphorylated protein kinase B (P-Akt), hypoxia-inducible factor-1 α (HIF-1α) and the degree of bone damage are analyzed. A total of 72 CMEC patients admitted to the otolaryngology department of our hospital from 2019 to January 2021 for surgical treatment are selected. According to the different intervention methods, the microscope group and the otolaryngology intervention group are established, respectively, with 36 patients in each group. The patients in the microscope group are treated with a microscope for middle ear cholesteatoma surgery, and the patients in the otoscope intervention group are treated with an otoscope for middle ear cholesteatoma surgery. The experimental results show that ear endoscopic intervention has better clinical efficacy for CMEC patients, which can effectively shorten the operation time, reduce the incidence of postoperative complications, and effectively improve the hearing of patients.

Multislice Spiral Computer Tomography Findings of Simple Congenital Middle Ear Malformations.

Background Simple congenital middle ear malformations (SCMEMs) are a group of congenital ear malformations. The study aims to analyze the multi-slice spiral computer tomography (MSCT) manifestations of normal ears and SCMEM ears. Objectives This study aimed to investigate the MSCT manifestations of normal ears and SCMEM ears and to evaluate the relationship between the SCMEM and the tympanic segment of the FNC pathway. Methods This was a retrospective case-control study. Patients who were diagnosed with SCMEM were included in the SCMEM group. Patients with vertigo, pulsatile tinnitus, or other symptoms were included in the control group. MSCT examination and image processing of the ossicular chain, facial nerve canal, and related structures were performed and compared between the two groups. Results There were 94 cases in the SCMEM group (125 ears) and 97 cases in the control group (190 ears). Sixty-three cases (67.0%) were unilateral malformations (36 right ears and 27 left ears). MSCT showed congenital stapes malformation in 107 ears (85.6%) and incus long process malformation in 84 ears (67.2%). Among these, simple stapes malformations were found in 41 ears (32.8%), simple incus malformation in 18 ears (14.4%), and stapes malformation complicated with incus malformation in 66 ears (52.8%). The distance between the facial nerve and lateral semicircular canal (FNC-LSC) in the SCMEM group was 1.30 ± 0.64 mm compared to 0.79 ± 0.11 mm in the control group (P < 0.001), and the distance between facial nerve canal and oval window (FNC-OW) was 0.67 ± 0.53 mm in the SCMEM group and 1.13 ± 0.18 mm in the control group (P < 0.001). Conclusion SCMEM occurred mainly in ossicular chain abnormalities. MSCT clearly showed the slight structural changes in the middle ear and provided an accurate basis for preoperative diagnosis.

Right Incus Osteoma in a Child: A Differential Diagnosis of Middle Ear Malformations.

Osteomas of the temporal bone, especially those involving the incus, are rare, unilateral, benign osseous tumors. The clinical presentation is usually isolated conductive hearing loss, and the diagnosis is confirmed by a temporal computed tomography scan. Osteomas of the incus represent a differential diagnosis of congenital middle ear malformations in children, which are the most frequent cause of conductive hearing loss with a normal eardrum in the pediatric population. In case of disabling symptomatology, surgery seems to be a safe way to recover normal hearing.

The Role of Otoscopy in the Diagnosis of Rare Diseases in Pediatrics. Congenital Middle Ear Cholesteatoma in the Child: Clinical Case

The clinical recommendations “Acute respiratory viral infection (ARVI) in children" indicate that otoscopy should be a part of routine pediatric examination of each patient along with auscultation, percussion, etc. Nowadays, there are no legal regulations on which specialists can perform otoscopy. Thus, there is significant pediatricians’ interest in otoscopy, especially in diagnosis of acute otitis media (AOM) during primary examination for timely antibacterial management. Moreover, pediatricians could reveal such rare and very aggressive middle ear disease as cholesteatoma, its early diagnosis can prevent the development of any complications and determines the range and quality of rehabilitation actions.

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

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.