[The application of endoscope and microscope in the stapes surgeries].

Objective:To compare the application of endoscope and microscope in all kinds of stapes surgeries. Methods:Fifty-nine stapes surgeries have been collected from April 2020 to May 2023 in Senior Department of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Chinese PLA Medical School. Hearing level, hospital stay post-operation, times of hospital visit post-operation, etc. have been compared between the endoscopic group and microscopic group. Patients who were failed to place the stapes prosthesis because of the poor exposure of the oval window have been analyzed. Results:Otosclerosis was the most common diagnosis in both groups. There was 1（1/23） middle ear malformation in the endoscopic group and 5（5/36） middle ear malformations in the microscopic group. There were 2 Van Der Hover syndromes and 4 Treacher Collins syndromes in the microscopic group. In the endoscopic group ABG of 10 ears（43.5%） ≤ 10 dB, and ABG of 21 ears（91.3%） ≤20 dB.In the microscopic group ABG of 13 ears（41.9%） ≤ 10 dB, and ABG of 28 ears（90.3%） ≤ 20 dB. There was no statistic difference between 2 groups. Times of hospital visit post-operation in the endoscopic group was less than in the microscopic group（P<0.01）. There was no facial palsy, tympanic perforation or profound sensorineural hearing loss in both groups. Conclusion:Endoscope is more suitable for patients who are evaluated with no severe stapes malformation, or less manipulation of drilling the bone. It could also reduce the hospital visit post-operation. Patients with narrow ear canal or severe middle ear malformation are recommended to perform the surgery with microscope, because it provides the chance of manipulation with 2-hands of surgeons.

A clinical epidemiological study on congenital ear malformation (CEM).

BACKGROUND: There is no report about the definition, classification and clinical epidemiological study of congenital ear malformation (CEM).Aims/Objectives: To investigate the definition, clinical classification and distribution of a large number of CEM cases, along with the clinical and epidemiological characteristics associated with congenital malformation of the middle and outer ear (CMMOE). MATERIAL AND METHODS: A total of 3231 cases (4714 ears) with CEM and related malformations were retrospectively analyzed, including 2,658 cases (4,064 ears) CEM and 573 cases (650 ears) preauricular fistulas and accessory ears, specifically related malformations to CEM. RESULTS: Among the 2,658 cases (4,064 ears) CEM, 64.9% cases were male and 35.1% were female. 52.9% cases with bilateral, 29.1% with right and 18.0% with left CEM. A subgroup of 1,050 cases (1,331 ears) was identified as CMMOE out of 1,090 cases (1,379 ears) microtia. In the CMMOE subgroup, 74.0% cases were male, 46.3% on the right side, 26.8% showed bilateral involvement. Out of the CMMOE with available images, there were 947 cases (1,105 ears). Additionally, related malformations including preauricular fistulas and accessory ears, were solely occured in 573 cases (650 ears), except for their occurrence simultaneously with CEM. The classification and distribution of CEM are as follows:1. 2658 cases of CEM (1) 69.9% of them were confined to the ear, including: ① the outer ear 2.6%, the middle ear 17.0% and the inner ear 32.4%, ② two parts simultaneous malformations of the outer ear, the middle ear and the inner ear 17.2%, ③ three parts simultaneous malformations of the outer ear, the middle ear and the inner ear 0.6%, (2) Multiple malformations including in CEM 29.1%; (3) syndromes related to CEM 1.0%.2. 1050 cases of CMMOE (1) 36.3% of them were confined to the middle and outer ears; (2) Multiple malformations associated with CMMOE 61.4%, including hemifacial microsomia 50.5%, accessory ears 9.8%, preauricular fistulas 9.2%, heart malformation 7.1%, and kidney malformation 2.0%; and (3) syndromes related to CMMOE 2.3%, (4) Complications of the ear 16.9%, mainly including otitis media 7.9%, outer ear canal cholesteatoma 6.3%, retroauricular abscess 1.1%, and nasal and pharyngeal complications 18.6%. The proportions of microcia grades I-Ⅴ based on the images of 1105 ears CMMOE were 5.2%, 10.5%, 74.1%, 6.9%, and 3.3%, respectively. Among the 1331 ears CMMOE, the incidence rates of outer ear canal atresia, stenosis, normal, and ossicles malformation were 86.5%, 9.9%, 3.6%, and 98.3%, respectively.Conclusion and Significance: Approximately 69.9% of CEM cases were confined to the ear itself, with the highest proportion being inner ear malformation alone. Multiple malformations were predominantly associated with CMMOE. Syndromes related to CEM were relatively rare. Among cases of microtia, 96.3% were classified as CMMOE. These cases were predominantly observed in males, right ear and exhibited characteristics such as grade III microtia, atresia of the outer ear canal, ossicles malformation, and multiple malformations. The multiple malformations frequently included hemifacial microsomia, accessory ear, preauricular fistula, as well as heart and kidney deformities. CMMOE often coexisted with ear infections, cholesteatoma, and complications in the nasal and pharyngeal regions.

Congenital ear malformation (CEM).

BACKGROUND: At present, there are not international unified standards and reports on Congenital Ear Malformation (CEM) in the world, which makes it difficult to transfer information and compare the literature. AIMS/OBJECTIVES: Through the statistical analysis of a large sample of CEM, a unified standard of all aspects of CEM is proposed and the data are provided for reference, which is convenient for the international work and literature comparison in this field. MATERIALS AND METHODS: Based on the author's 30 years of clinical and scientific research work on CEM and the relevant cases of 3231 (4714 ears) in our hospital, and combined with literature, statistical analysis was made. RESULTS: This paper summarizes the classification, definition, epidemiology, embryonic development, pathogenic factors of CEM and elaborates on the clinical manifestations, examination and sequence therapy of representative Congenital Malformation of the Middle and Outer Ear (CMMOE). We also introduce malformation of the auricle and inner ear, so as to cover the outer, middle and inner ear. At the same time, we introduce our achievements and contributions in this field. CONCLUSIONS AND SIGNIFICANCE: This study provides reference to the international unified standard and treatment principle of the CEM.

Persistent Stapedial Artery, Oval Window Atresia and Congenital Stapes Agenesis-Case Report.

Background: The persistent stapedial artery (PSA) is a rare congenital vascular malformation involving the middle ear. It is usually associated with pulsatile tinnitus and/or conductive hearing loss and can account for multiple risks during middle ear surgery. Case Report: we present a case of a 9-year-old male child with conductive hearing loss and persistent stapedial artery in his right ear, who was admitted to our ENT Department for hearing loss. During surgery, we discovered PSA along with congenital stapes agenesis and oval window atresia, as well as an abnormal trajectory of the mastoid segment of the facial nerve. After ossicular reconstruction (transcanal total ossicular replacement prosthesis) with cochleostomy, no surgical complications were recorded and hearing improvement was monitored by pre- and postoperative audiometry. Conclusion: Stapedial artery is a rare anatomical middle ear abnormality that can prevent proper surgical hearing restoration and can be associated with other simultaneous temporal bone malformations.

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.

[Hearing rehabilitation with the Vibrant Soundbridge in patients with congenital middle ear malformation]. / Die Vibrant Soundbridge zur Versorgung von Patienten mit kongenitalen Mittelohrfehlbildungen.

BACKGROUND: Congenital aural atresia, which is usually unilateral, causes hearing loss and aesthetic impairment. Besides tympanoplasty with/without canalplasty and bone conduction devices, active middle ear implants are also available for functional rehabilitation. OBJECTIVE: This article aims to present a contemporary review on the treatment possibilities for middle ear malformations, with a focus on audiological rehabilitation with the Vibrant Soundbridge. MATERIALS AND METHODS: A selective literature search for treatment possibilities was performed in PubMed up to October 2020, and personal clinical experiences are reported. RESULTS: The Vibrant Soundbridge, which is approved for children ≥ 5 years, is suitable for treatment of middle ear malformations with a Jahrsdoerfer score ≥ 5. Although implantation of a Vibrant Soundbridge is surgically more demanding than implantation of a bone conduction device, the method is safe, delivers good auditory results (superior to bone conduction devices in terms of speech understanding and spatial hearing), does not involve intensive postsurgical care, and rarely requires revision surgery. The Vibrant Soundbridge can be coupled to (remnants of) the ossicular chain or the round window. CONCLUSION: The Vibrant Soundbridge is an appropriate treatment method in patients with middle ear malformations who have suitable anatomical preconditions.

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.

Developmental Disruptions of the Human Stapes.

OBJECTIVE: To evaluate and classify developmental malformations of the human stapes. METHODS: Twenty-five temporal bone specimens from 18 patients with congenital stapes malformations were identified in the Mass Eye and Ear temporal bone collection. Serial sections stained with hematoxylin and eosin were examined by light microscopy and the morphology of the stapes was compared to age-matched controls. RESULTS: Each case of stapes malformation could be classified into one of four malformation types based on our current understanding of the embryologic origin of the subunits of the stapes and timing of development. Twenty-seven percent of stapes malformations had a Type I morphology characterized by a hypoplastic or absent inner footplate and hypoplastic to absent mesoderm footplate or oval window. The crura and capitulum may be absent, monopodal or dysmorphic. Eleven percent expressed a Type II malformation with dysmorphic or monopodal capitulum and crura and a fixed footplate. Twenty-seven percent were of Type III with a dysmorphic or monopodal capitulum and or crura. The footplate, and thereby oval window is present and without fixation. The most common malformation, Type IV, was isolated footplate fixation observed in 33% of cases. CONCLUSIONS: Malformations of the human stapes follow consistent patterns of early or late disruptions of the stapes subunits of mesodermal and/or neural crest origin. While the molecular events, including temporal coordination, that lead to a normally formed stapes are not yet fully understood, the observed patterns of human stapes malformation can be consistently classified into one of four patterns of developmental disruption.

Congenital malformations of the external and middle ear accompanied by temporal bone anomaly in a calf.

A 7-month-old female Holstein calf presented with bilateral microtia and absent external acoustic meatus. The real-time polymerase chain reaction test was negative for bovine viral diarrhea virus and bovine leukemia virus. The calf's dam had a normal reproductive history. Computed tomography confirmed bilateral atresia of external auditory canals, aplasia of tympanic cavities and the ossicular chain, and temporomandibular joint abnormality. Necropsy revealed a severe malformation of the temporal bone. In the tympanic region, the external acoustic pore, tympanic bulla, and muscular process were absent bilaterally. The bilateral inner ear structure was normal. Based on these findings, we diagnosed the present case as congenital malformations of the external and middle ear accompanied by temporal bone anomaly.

Otopathologic Abnormalities in CHARGE Syndrome.

OBJECTIVE: To perform an otopathologic analysis of temporal bones (TBs) with CHARGE syndrome. STUDY DESIGN: Otopathologic study of human TB specimens. SETTING: Otopathology laboratories. METHODS: From the otopathology laboratories at the University of Minnesota and Massachusetts Eye and Ear Infirmary, we selected TBs from donors with CHARGE syndrome. These TBs were serially sectioned at a thickness of 20 µm, and every 10th section was stained with hematoxylin and eosin. We performed otopathologic analyses of the external ear, middle ear (middle ear cleft, mucosal lining, ossicles, mastoid, and facial nerve), and inner ear (cochlea, vestibule, internal auditory canal, and cochlear and vestibular nerves). The gathered data were statistically analyzed. RESULTS: Our study included 12 TBs from 6 donors. We found a high prevalence of abnormalities affecting the ears. The most frequent findings were stapes malformation (100%), aberrant course of the facial nerve (100%) with narrow facial recess (50%), sclerotic and hypodeveloped mastoids (50%), cochlear (100%) and vestibular (83.3%) hypoplasia with aplasia of the semicircular canals, hypoplasia and aplasia of the cochlear (66.6%) and vestibular (91.6%) nerves, and narrowing of the bony canal of the cochlear nerve (66.6%). The number of spiral ganglion and Scarpa's ganglion neurons were decreased in all specimens (versus normative data). CONCLUSIONS: In our study, CHARGE syndrome was associated with multiple TB abnormalities that may severely affect audiovestibular function and rehabilitation.

Congenital Aural Atresia: What the Radiologist Needs to Know?

Congenital aural atresia (CAA) is characterized by hypoplasia or aplasia of external auditory canal associated with auricular deformity. It also embodies a spectrum of temporal bone abnormalities including anomalies of middle ear, ossicles, facial nerve, oval window, round window, mastoid pneumatization, temporomandibular joint and occasionally inner ear and vascular anomalies. The management in such patients is variable and consists of rehabilitative hearing aids or surgical correction depending on various patient factors like associated temporal bone abnormalities, degree of hearing impairment and laterality. Surgical management if planned, depends on the extent of associated temporal bone anomalies and hence necessitates comprehensive knowledge about the normal and abnormal anatomy of these structures. High resolution computed tomography (HRCT) is the optimal investigation which provides required anatomical information and plays a pivotal role in deciding the management strategy. This pictorial review aims to review the spectrum of anomalies involving critical structures in CAA on HRCT along with their clinical significance.

Multicenter Study of Congenital Middle Ear Anomalies. Report on 246 Ears.

OBJECTIVES/HYPOTHESIS: Congenital middle ear anomalies represent a relatively rare condition. This study aimed to describe the characteristics and the surgical outcomes for patients with middle ear anomalies. METHODS: A multicenter study was conducted of consecutive patients with congenital middle ear anomalies who underwent primary surgical treatment between January 2008 and December 2017. Demographics, surgical procedures, and audiometric data were registered into the institutional database. Hearing changes and postoperative air-bone gap (ABG) were evaluated 1 year after surgery. RESULTS: A total of 246 patients (246 ears) (median age: 14 years, range: 4-75 years old) were included in this study. Anomalies were subdivided using the Teunissen and Cremers classification: 53 ears (22%) were categorized as class I, comprising only stapes ankylosis; 35 ears (14%) as class II, having ossicular chain anomalies with stapes ankylosis; 139 ears (57%) as class III, having ossicular chain anomalies with a mobile stapes-footplate; and 19 ears (8%) as class IV, with aplasia of the oval window. Evaluation of hearing outcomes for 198 ears with more than 1 year of follow-up revealed that good postoperative ABG (≤20 dB) was achieved in 82% of class I, 68% of class II, 74% of class III, and 23% of class IV anomalies. The postoperative ABG in class IV was significantly worse than in class I (P < .001) or class III (P < .01). CONCLUSIONS: This study demonstrated that class III anomalies comprised the majority of middle ear anomalies and surgical outcomes for class IV anomalies are unfavorable. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E2323-E2328, 2021.

Prenatal development of the human tympanic ring: a morphometric study with clinical correlations.

PURPOSE: To establish normal reference values for the human Tympanic Ring (TR) during prenatal development, and to describe and interpret its growth dynamics. METHODS: Fifty spontaneously aborted human fetuses aged 12-37 weeks with normal external characteristics were evaluated. The parameters measured in the TR were the cephalocaudal and dorsoventral axes, total area, thickness, height, and length and angle of the notch of Rivinus (NR). Data were subjected to statistical analysis. RESULTS: The following values were obtained at the end of fetal development: cephalocaudal and dorsoventral axes, 10.03 and 8.3 mm, respectively; ratio between the two axes, 120%; total area, 65.63 mm2; height and thickness, 0.88 mm and 1.10 mm, respectively; and length and angle of the NR, 4.66 mm and 26.2 degrees, respectively. There were variations in the length of the dorsoventral axis throughout fetal development that affected all other parameters, except for the cephalocaudal axis. There were no sex-based differences in TR size. CONCLUSION: The prenatal development of the TR is dynamic as evidenced by the size variations noted throughout fetal development. Notwithstanding, this structure is a reliable and sensitive marker of developmental abnormalities of the external and middle ear.

Fetal Bovine Collagen Grafts for Repair of Tegmen Defects and Encephaloceles Via Middle Cranial Fossa Approach.

To evaluate the use of commercially available allogenic dural graft materials made of fetal bovine collagen, we present an analysis of our case series with use of autologous and allogenic graft materials. Patients who underwent surgical repair of a tegmen tympani defect associated with ipsilateral conductive hearing loss and cerebrospinal fluid (CSF) otorrhea using a middle cranial fossa (MCF) approach from 2004 to 2018 at Loyola University Medical Center were included. Resolution of CSF otorrhea, audiologic outcomes, facial nerve preservation, and surgical complications was analyzed. Thirty-three patients with an average age of 55.3 years (range: 21-78, standard deviation [SD]: 12.9) and body mass index of 34.4 (range: 22-51, SD: 7.4) underwent an MCF repair of a tegmen and dural defect. All patients presented with CSF otorrhea and conductive hearing loss ipsilateral to the defect. Repairs were made with combinations of allograft and autograft in 17 cases, allograft only in 15 cases, and autograft only in 5 cases. Improvement in hearing was noted in 33 cases, and resolution of CSF otorrhea was noted in 36 cases; one patient required repeat surgery which resolved CSF otorrhea. Three patients had minor complications; all these were in the autograft group. The MCF approach coupled with the use of fetal bovine collagen grafts is a safe and viable method to repair tegmen tympani and associated dural defects with salutary outcomes and low morbidity.

Getting Started with Endoscopic Ear Surgery.

Because endoscopic technology has matured over the past several decades, ear surgeons have increasingly used endoscopy to address some of the limitations of operative microscopy. The wide field of view and high-resolution images provided by endoscopes allow for improved visualization of the tympanic cavity using minimally invasive surgical portals compared with the standard operative binocular microscope. The endoscope is becoming an essential tool in the otologist's armamentarium. In this article, the authors discuss rationale for endoscopic ear surgery, terminology and classification, surgical indications, essential equipment, surgical ergonomics, and practical steps to incorporate endoscopic ear surgery into practice.

Numerical analysis of the effects of ossicular chain malformations on bone conduction stimulation.

To assess the effects of ossicular chain malformations on the performance of bone conduction hearing aids, a human ear finite-element model that includes an ear canal, a middle ear, and a spiral cochlea incorporating the third windows was established. This finite element model was built based on micro-computed tomography scanning and reverse modelling techniques, and the reliability of the finite element model was verified by comparison with reported experimental data. Based on this model, two main types of ossicular chain malformations, i.e., the incudostapedial disconnection and the ossicles fixation, were simulated, and their influences on bone conduction were analyzed by comparing the trans-cochlear-partition differential pressures. The results indicate that the incudostapedial disconnection mainly deteriorates the bone conduction response at mid frequencies. The stapes fixation has the largest effect among the ossicles fixation with the bone conduction stimulation, which also mainly decreases the mid-frequency response of the bone conduction, especially at 2 kHz. As the speech intelligibility has the most important frequency range at the range between 1 kHz and 2.5 kHz, the mid-frequency deterioration caused by ossicular chain malformations should be compensated in optimizing the design of the bone conduction hearing aids. For treating patients with the ossicular chain malformations, especially for the patients who suffer from the stapes fixation, the output of bone conduction hearing aids' actuator in the middle frequency band should be improved.

Unfavourable prognostic factors in patients treated surgically for otosclerosis.

Backgrounds: Otosclerosis is the cause of between 5% and 9% of all deafness cases and between 18% and 22% of conductive hearing loss cases. Neurosensory deafness develops in 30% of patients with otosclerosis.Aims/Objectives: The aim was to seek a correlation that would reflect the dependence of the results of middle ear surgery on the type of abnormalities atypical of otosclerosis but found during the stapedotomy surgery.Materials and Methods: The analysis included 140 patients who underwent surgery for otosclerosis. The hearing of all patients was assessed using an audiometric test.Results: In the assessment of changes in the mean bone conduction values, statistically significant differences between the reference group and the subgroup of patients on whom a myringoplasty was performed, as well as in patients with adhesions present in the middle ear spaces, were found only for the 500 Hz frequency.Conclusion and Significance: The removal of abnormalities, such as the loss of the eardrum (iatrogenic), changes to the lining and adhesions other than those typical of otosclerosis, restores middle ear mechanics after a stapedotomy on the inner ear and leads to a measurable improvement in mean bone conduction values.

A Rare Case of Bifurcated Chorda Tympani.

Preservation of the chorda tympani is important in middle ear surgery to prevent dysgeusia postoperatively. However, determining the exact course of the chorda tympani before surgery is not always possible, especially in cases with accompanying malformations. In this report, we presented an extremely rare case of bifurcation of the chorda tympani in a 15-year-old male patient. We performed tympanoplasty for a middle ear malformation with conductive hearing loss. During the operation, we noticed and carefully preserved the bifurcated chorda tympani. The patient did not develop dysgeusia postoperatively. Appropriate handling and understanding of the anomalous chorda tympani preserved the patient's sense of taste and hence quality of life.