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Nature Communications Oct 2023The middle ear ossicles in modern mammals are repurposed from postdentary bones in non-mammalian cynodonts. Recent discoveries by palaeontological and embryonic studies...
The middle ear ossicles in modern mammals are repurposed from postdentary bones in non-mammalian cynodonts. Recent discoveries by palaeontological and embryonic studies have developed different models for the middle ear evolution in mammaliaforms. However, little is known about the evolutionary scenario of the middle ear in early therians. Here we report a detached middle ear preserved in a new eutherian mammal from the Early Cretaceous Jehol Biota. The well-preserved articulation of the malleus and incus suggest that the saddle-shaped incudomallear joint is a major apomorphy of Early Cretaceous eutherians. By contrast to the distinct saddle-like incudomallear articulation in therians, differences between the overlapping versus the half-overlapping incudomallear joints in monotremes and stem mammals would be relatively minor. The middle ear belongs to the microtype by definition, indicating its adaptation to high-frequency hearing. Current evidence indicates that significant evolutionary innovations of the middle ear in modern therians evolved in Early Cretaceous.
Topics: Animals; Biological Evolution; Phylogeny; Eutheria; Mammals; Ear, Middle; Fossils
PubMed: 37884521
DOI: 10.1038/s41467-023-42606-7 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... Oct 2023For tympanosclerosis patients with ossicular chain fixation, we use ossicular chain bypass technique and evaluate its long-term effects. From June 2017 to June 2019, 147...
For tympanosclerosis patients with ossicular chain fixation, we use ossicular chain bypass technique and evaluate its long-term effects. From June 2017 to June 2019, 147 patients with tympanosclerosis who underwent middle ear surgery with otoscopy in Yinchuan First People's Hospital were reviewed. The subjects were divided into three groups according to the implemented operation plan, 51 cases in the ossicular chain mobilization group(OCM), 56 cases in the ossicular chain bypass reconstruction group(OCB), and 40 cases in the malleus-incus complex resection reconstruction group(MICR). Through a three-year follow-up, the medium and long-term effects of different operation plans were compared and analyzed. There was no significant difference among the three groups in the incidence of tympanic membrane perforation, delayed facial nerve palsy, and the dispatch and displacement of PORP. The incidence of tympanic membrane retraction pocket or cholesteatoma after operation in OCB group(0) was significantly lower than that in OCM group(11.76%) and MICR group(7.5%)(<0.05). At 12 months after operation, ΔABG of OCB group and MICR group were better than that in the OCM group(<0.05). At 36 months after operation, ΔABG of OCB group was better than that in the OCM group(<0.05), and there was no significant difference between OCB group and MICR group. The audiological performance of patients with epitympanic sclerosis(ETS) at 12, 24 and 36 months after operation was better than that of patients with posterior tympanosclerosis(PTS) and total tympanosclerosis(TTS)(<0.05). Compared with patients undergoing ossicular chain mobilization and malleus-incus complex resection for ossicular chain reconstruction, patients with tympanosclerosis undergoing bypass technique have better and stable hearing prognosis in medium and long term. This technique can effectively prevent the formation of retracted pocket and cholesteatoma in patients with tympanosclerosis after operation.
Topics: Humans; Tympanosclerosis; Ear Ossicles; Ear, Middle; Malleus; Cholesteatoma; Retrospective Studies; Ossicular Prosthesis; Treatment Outcome
PubMed: 37828886
DOI: 10.13201/j.issn.2096-7993.2023.10.010 -
Molecular Genetics & Genomic Medicine Jan 2024Kabuki syndrome 1 (KS1; OMIM:147920), which is characterized by distinctive dysmorphic facial features (such as arched eyebrows, long palpebral fissures with eversion of...
BACKGROUND
Kabuki syndrome 1 (KS1; OMIM:147920), which is characterized by distinctive dysmorphic facial features (such as arched eyebrows, long palpebral fissures with eversion of the lower lid, and large protuberant ears), intellectual disability, short stature, and dermatoglyphic and skeletal abnormalities, is brought on by pathogenic variants in KMT2D (OMIM:602113). In this work, three individuals with novel pathogenic KMT2D gene variants had their longitudinal audiological manifestations and ear structural characteristics outlined.
METHODS
The longitudinal audiological data from neonatal hearing screening and a battery of several hearing tests were evaluated. The battery of hearing tests included tympanometry, distortion product otoacoustic emission (DPOAE), click-evoked air-conduction auditory brain-stem response (AC-ABR), click-evoked bone-conduction auditory brain-stem response (BC-ABR), narrow band CE-chirp auditory steady-state response (NB CE-chirp ASSR), and pure-tone audiometry (PTA). Phenotype identification and whole exome sequencing (WES) were performed on recruited individuals.
RESULTS
All three patients (two females and on male; last evaluations at 14 months, 11 months, and 5.7 years, respectively) failed the newborn hearing screening, and the audiological follow-up data revealed mild to profound fluctuating hearing loss, which was directly influenced by the incidence and severity of otitis media with effusion (OME). When OME occurred, the AC-ABR thresholds increased from 30-75 dBnHL to 45-90 dBnHL. The threshold for the BC-ABR and BC-PTA was between 25 and 50 dBnHL, indicating mild to moderate sensorineural hearing loss (SNHL). The high-resolution computed tomography (HRCT) pictures indicated that all three patients had middle and inner ear abnormalities. Middle ear anomalies showed as diminished mastoid gasification and ossicle dysplasia. Cochlear dysplasia, a dilated vestibule, fusion of the vestibule with the horizontal semicircular canals, and a short and thick horizontal semicircular canal were visible on images of the inner ear. This study recruited three individuals with three novel pathogenic variants (c.5104C>T, c.10205delA, and c.12840delC) of KMT2D who were identified at ages 27 days, 2 months, and 5.5 years.
CONCLUSIONS
Hearing characteristics of three individuals with three novel pathogenic variants of KMT2D range from mild to profound fluctuating hearing loss with mild to moderate SNHL. HRCT scans showed that all three individuals had anatomical middle and inner ear abnormalities. KS 1 patients must get clinical therapy for OME, frequent auditory monitoring, and prompt intervention.
Topics: Infant, Newborn; Female; Humans; Male; Hearing; Hearing Tests; Abnormalities, Multiple; Hearing Loss; Face; Hematologic Diseases; Vestibular Diseases
PubMed: 37921229
DOI: 10.1002/mgg3.2306 -
Journal of the Belgian Society of... 2024Tophaceous gout can rarely present in the middle ear as a mass-like lesion, causing conductive hearing loss. Noncontrast high-resolution computed tomography (HRCT) of...
Tophaceous gout can rarely present in the middle ear as a mass-like lesion, causing conductive hearing loss. Noncontrast high-resolution computed tomography (HRCT) of the temporal bone plays a significant role in the diagnosis. Awareness of this condition among radiologists is important since it presents a distinctive appearance on HRCT. We present a case of tophaceous gout of the middle ear diagnosed with photon-counting computed tomography (PCCT). The presence of a partially calcified mass with a semolina-like appearance within the middle ear is highly suggestive of tophaceous gout, even in the presence of normal serum uric acid levels.
PubMed: 38371367
DOI: 10.5334/jbsr.3421 -
Journal of Visualized Experiments : JoVE Dec 2023Congenital cholesteatoma accounts for 25% of cholesteatoma cases in children. Transcanal Endoscopic Ear Surgery (TEES) is ideal for these patients because it offers a...
Congenital cholesteatoma accounts for 25% of cholesteatoma cases in children. Transcanal Endoscopic Ear Surgery (TEES) is ideal for these patients because it offers a wide endoscopic view of the middle ear and a minimally invasive approach. The two main limitations are the loss of one operative hand and a narrow external auditory canal in younger children. Here, we present the case of a 3-year-old patient with a Potsic stage III congenital cholesteatoma adherent to the incus and branches of the stapes. A robotic-assisted TEES procedure was performed, during which a robotic arm with 6 degrees of freedom held a 0°, 2.9 mm wide endoscope, enabling the surgeon to work in a narrow environment with both hands. The procedure's duration was 2 h and 9 min, including 16 min for the installation and draping of the robotic arm. After a trans-canal approach, the cholesteatoma was dissected from the ossicles using both a needle (or sickle knife) and suction to stabilize the ossicles and limit the risk of hearing trauma. The cholesteatoma was debulked to reduce its size, allowing it to be pushed under the malleus anteriorly and then separated from other adherences before removal. A tragal cartilage graft was used to reinforce the tympanic membrane.
Topics: Child, Preschool; Humans; Cholesteatoma, Middle Ear; Retrospective Studies; Robotic Surgical Procedures; Robotics; Treatment Outcome
PubMed: 38163263
DOI: 10.3791/64861 -
The Laryngoscope Mar 2024We describe an unusual case of glomangiopericytoma presenting as a mass filling the middle ear, enveloping the ossicles, and extending into the mastoid antrum without...
We describe an unusual case of glomangiopericytoma presenting as a mass filling the middle ear, enveloping the ossicles, and extending into the mastoid antrum without bony destruction. Management involved three surgeries and stereotactic radiosurgery, which achieved short-term local control with no evidence of disease on MRI imaging 12 months after radiation. Facial nerve function and hearing were preserved. This is the first report to our knowledge of a glomangiopericytoma presenting as a primary temporal bone lesion. Treatment with surgery and stereotactic radiosurgery for residual or recurrent disease is a reasonable approach to achieve local control and functional preservation. Laryngoscope, 134:1426-1430, 2024.
Topics: Humans; Ear Diseases; Hearing; Radiosurgery; Head and Neck Neoplasms; Ear, Middle; Treatment Outcome
PubMed: 37615366
DOI: 10.1002/lary.30987 -
Lin Chuang Er Bi Yan Hou Tou Jing Wai... May 2024To explore the clinical manifestations and imaging characteristics, and to clarify the imaging value in the diagnosis of facial nerve schwannomas. Retrospectively...
To explore the clinical manifestations and imaging characteristics, and to clarify the imaging value in the diagnosis of facial nerve schwannomas. Retrospectively analyze the data of 23 patients with facial nerve schwannomas confirmed by surgery and pathology in the Department of Otorhinolaryngology of the First Affiliated Hospital of the Air Force Military Medical University from September 2020 to September 2022, including 8 males and 15 females, aged 18-66 years old. Summarize and analyze their clinical symptoms, specialized examinations, and imaging findings. The clinical manifestations were facial nerve paralysis in 15 cases(2 cases of HB Ⅳ, 6 cases of HB Ⅴ, 7 cases of HB Ⅵ), hearing loss in 14 cases(5 cases of conductive deafness, 2 cases of mixed deafness, and 7 cases of severe sensorineural hearing loss), 8 cases tinnitus, 7 cases ear pain, 4 cases dizziness, 4 cases headache, 2 cases ear pus, and parotid gland tumors in 6 cases presenting as local masses. Endoscopic examination revealed 8 cases of external ear canal tumors and 3 cases of intratympanic tumors. Combining temporal bone HRCT, MRI enhanced scanning, and CPR imaging techniques, 1 case involved the internal auditory canal segment, 2 cases in the tympanic segment, 6 cases in the parotid gland area. A total of 14 cases involved two or more segments of the internal auditory canal segment, the labyrinthine segment, geniculate ganglion, the tympanic segment, and the mastoid segment. When the tumors were large, adjacent structures were involved. It was found that 8 cases invaded the external auditory canal and tympanic cavity, ossicles were displaced or bony destruction; 3 cases invaded the jugular foramen area, and 1 case grew to the middle cranial fossa region with temporal lobe brain parenchymal compression. The clinical manifestations of facial nerve schwannomas are diverse. The combination of various imaging techniques will be conducive to topical and qualitative diagnosis and provide an important basis for treatment strategies.
Topics: Humans; Male; Female; Middle Aged; Adult; Neurilemmoma; Aged; Adolescent; Magnetic Resonance Imaging; Young Adult; Retrospective Studies; Facial Nerve; Tomography, X-Ray Computed; Cranial Nerve Neoplasms
PubMed: 38686473
DOI: 10.13201/j.issn.2096-7993.2024.05.006 -
Ear, Nose, & Throat Journal Nov 2023During the postoperative period, most patients with otosclerosis report vertigo and/or nausea caused by interventions within the inner ear. The aim of this study was to...
OBJECTIVE
During the postoperative period, most patients with otosclerosis report vertigo and/or nausea caused by interventions within the inner ear. The aim of this study was to evaluate both early and late vertigo associated with hearing improvement after stapes surgery for otosclerosis.
METHODS
The analysis included 170 patients admitted to the hospital undergoing their first surgery for otosclerosis. Audiological diagnostics, surgical techniques, and symptoms reported by the patients were all analyzed.
RESULTS
A statistical correlation and an unfavorable influence of late, undesired symptoms, such as vertigo, nausea/vomiting, and nystagmus, on final hearing improvement after surgical treatment of otosclerosis were found. Prostheses that were too long or placed too deep within the inner ear space were the most frequent cause of both vertigo and lack of hearing improvement observed after stapedotomy.
CONCLUSIONS
A significant negative influence on bone conduction thresholds, particularly at 2000 Hz, was associated with vestibular symptoms persisting for 7 days after the surgery. Symptoms of impaired bony labyrinth function after stapedotomy, persisting for more than 1 year, were associated with insufficient reduction of the air-bone gap and worse improvement in bone conduction thresholds at 1000 and 2000 Hz. The cause of both problems was related to a prosthesis that was too long or placed too deep in the inner ear during stapedotomy.
Topics: Humans; Otosclerosis; Hearing; Stapes Surgery; Bone Conduction; Vertigo; Nausea; Treatment Outcome; Retrospective Studies; Stapes
PubMed: 34176331
DOI: 10.1177/01455613211023014 -
BMC Surgery May 2024Transcanal endoscopic ear surgery (TEES) reportedly requires a long learning curve and may be associated with more complications and longer operative times than...
BACKGROUND
Transcanal endoscopic ear surgery (TEES) reportedly requires a long learning curve and may be associated with more complications and longer operative times than microscopic ear surgery (MES). In this study, we aimed to examine the usefulness and validity of TEES for ossicular chain disruption in the early stages of its introduction in our institution.
METHODS
TEES was performed on 11 ears (10 with congenital ossicular chain discontinuity and 1 with traumatic ossicular chain dislocation), and MES was performed with a retroauricular incision on 18 ears (6 with congenital ossicular chain discontinuity and 12 with traumatic ossicular chain dislocation) in a tertiary referral center. Postoperative hearing results, operative times, and postoperative hospital length of stay were retrospectively reviewed. The Mann-Whitney U test and Fisher's exact test was performed to compare variables between the TEES and MES groups. Pre- and postoperative air- and bone-conduction thresholds and the air-bone gap of each group were compared using the Wilcoxon signed-rank test. The Mann-Whitney U test and Wilcoxon signed-rank was performed to compare the pre- and postoperative air-bone gaps between the diagnoses.
RESULTS
No significant differences in the postoperative air-conduction thresholds, bone-conduction thresholds, air-bone gaps, or incidence of air-bone gap ≤ 20 dB were observed between the TEES and MES groups. The air-conduction thresholds and air-bone gaps of the TEES group significantly improved postoperatively. The air-conduction thresholds and air-bone gaps of the MES group also significantly improved postoperatively. No significant difference was observed in the operative times between the groups (TEES group: median, 80 min; MES group: median, 85.5 min). The TEES group had a significantly shorter postoperative hospital stay (median, 2 days) than the MES group (median, 7.5 days).
CONCLUSIONS
TEES was considered appropriate for the treatment of ossicular chain disruption, even immediately after its introduction at our institution. For expert microscopic ear surgeons, ossicular chain disruption may be considered a suitable indication for the introduction of TEES.
Topics: Humans; Ear Ossicles; Male; Female; Retrospective Studies; Adult; Adolescent; Endoscopy; Child; Middle Aged; Young Adult; Treatment Outcome; Operative Time; Otologic Surgical Procedures
PubMed: 38745291
DOI: 10.1186/s12893-024-02445-9 -
European Archives of... Nov 2023To describe the surgical procedure of exclusive endoscopic ossiculoplasty (EEO) with autologous grafts and evaluate audiological results, focusing on the advantages or...
PURPOSE
To describe the surgical procedure of exclusive endoscopic ossiculoplasty (EEO) with autologous grafts and evaluate audiological results, focusing on the advantages or drawbacks compared to the corresponding microscopic technique.
METHODS
A retrospective review of consecutive adult and pediatric patients affected by chronic otitis media (COM) with or without cholesteatoma who underwent EEO was conducted. Only autologous ossiculoplasty was included in the study. The procedure was performed by experienced surgeons of our institution between November 2014 and September 2019. Hearing outcomes were evaluated using postoperative air-bone gap (ABG) and success rates in different subgroups of patients and different types of ossiculoplasty (OPL) were analyzed. Our results were finally compared with the existing literature regarding both microscopic and endoscopic ossicular chain reconstruction.
RESULTS
In total, 74 endoscopic ossicular chain repair procedures performed within the study period met the inclusion criteria. Of these, 21 were pediatric patients (28%) and 53 were adults (72%). Surgical reconstruction procedures included 43 partial ossicular reconstructions (POR) and 31 total ossicular reconstructions (TOR). The postoperative ABG improved significantly compared to preoperative measurements, and the mean ABG closure was 7.85 dB HL (p = 0.00064). No statistically significant differences in audiological outcomes between TOR/POR techniques and pediatric/adult groups were found in our study cohort, with p values of 0.10 and 0.88, respectively.
CONCLUSIONS
At present, EEO can be considered a valid surgical option for re-establishing a functioning ossicular chain with acceptable hearing restoration in children and adults. Further reports in wider case series are required to confirm these results.
Topics: Adult; Humans; Child; Ossicular Prosthesis; Ossicular Replacement; Treatment Outcome; Tympanoplasty; Ear Ossicles; Retrospective Studies
PubMed: 37160464
DOI: 10.1007/s00405-023-08005-w