OBJECTIVE: To investigate the treatment time and efficiency of constricted ears of different severity after correction.
METHODS: We included the patients with constricted ear presented to our hospital for treatment between December 2021 and December 2023 in this retrospective analysis. The patients were divided into class I, II and III groups based on the severity of the constriction. Then we collected the data on classification of severity from each patient, together with sex, family history, age at initial correction, being informed upon diagnosis after birth, as well as utilization of auricle correction system. Logistic regression analysis was performed to identify the factors associated with the treatment time and efficiency.
RESULTS: The correction system yielded a high effective rate in the constricted ears. The treatment time in class II was significantly longer compared with those of class I after adjusting these parameters. Compared with the cases of class I, those with a class III showed significant attenuation in the symptoms and conditions (95 % CI: 0.034, 0.365; P < 0.001), after adjusting the age at initial correction, being informed upon diagnosis after birth, and utilization of auricle correction system. There were no statistical differences between class II and III in the treatment efficiency after correction.
CONCLUSIONS: The Amazing Ear Correction System was effective in treating constricted ear, yielding satisfactory treatment efficiency. Patients with class II constriction required longer treatment time compared with those of class I. The treatment outcome in the class I constriction was better than that of class III.

BACKGROUND: Auriculocondylar syndrome (ARCND) is an extremely rare autosomal dominant or recessive condition that typically manifests as question mark ears (QMEs), mandibular condyle hypoplasia, and micrognathia. Severe dental and maxillofacial malformations present considerable challenges in patients\' lives and clinical treatment. Currently, only a few ARCND cases have been reported worldwide, but most of them are related to genetic mutations, clinical symptoms, and ear correction; there are few reports concerning the treatment of dentofacial deformities.
METHODS: Here, we report a rare case of ARCND in a Chinese family. A novel insertional mutation in the guanine nucleotide-binding protein alpha-inhibiting activity polypeptide 3 (GNAI3) was identified in the patient and their brother using whole-exome sequencing. After a multidisciplinary consultation and examination, sequential orthodontic treatment and craniofacial surgery, including distraction osteogenesis and orthognathic surgery, were performed using three-dimensional (3D) digital technology to treat the patient\'s dentofacial deformity. A good prognosis was achieved at the 5-year follow-up, and the patient returned to normal life.
CONCLUSIONS: ARCND is a monogenic and rare condition that can be diagnosed based on its clinical triad of core features. Molecular diagnosis plays a crucial role in the diagnosis of patients with inconspicuous clinical features. We present a novel insertion variation in GNAI3, which was identified in exon 2 of chromosome 110116384 in a Chinese family. Sequential therapy with preoperative orthodontic treatment combined with distraction osteogenesis and orthognathic surgery guided by 3D digital technology may be a practical and effective method for treating ARCND.

耳鼻咽喉头颈外科的发展早期依赖手术创新，随着非手术治疗的普及，耳内科疾病成为研究热点。全球听力损失、耳鸣和眩晕患者数量庞大，这些内耳疾病具有突发性、致残性和致死性。本文分享了头晕、耳鸣、突聋等常见耳内科疾病的现状和未来研究方向，强调了对这些疾病的深入理解和治疗策略的重要性。同时，提出了这些疾病与全身因素的关联以及未来研究应关注耳与脑之间的关系。.

Precision and intelligence in evaluating the complexities of middle ear structures are required to diagnose auriculotemporal and ossicle-related diseases within otolaryngology. Due to the complexity of the anatomical details and the varied etiologies of illnesses such as trauma, chronic otitis media, and congenital anomalies, traditional diagnostic procedures may not yield accurate diagnoses. This research intends to enhance the diagnosis of diseases of the auriculotemporal region and ossicles by combining High-Resolution Spiral Computed Tomography (HRSCT) scanning with Deep Learning Techniques (DLT). This study employs a deep learning method, Convolutional Neural Network-UNet (CNN-UNet), to extract sub-pixel information from medical photos. This method equips doctors and researchers with cutting-edge resources, leading to groundbreaking discoveries and better patient healthcare. The research effort is the interaction between the CNN-UNet model and high-resolution Computed Tomography (CT) scans, automating activities including ossicle segmentation, fracture detection, and disruption cause classification, accelerating the diagnostic process and increasing clinical decision-making. The suggested HRSCT-DLT model represents the integration of high-resolution spiral CT scans with the CNN-UNet model, which has been fine-tuned to address the nuances of auriculotemporal and ossicular diseases. This novel combination improves diagnostic efficiency and our overall understanding of these intricate diseases. The results of this study highlight the promise of combining high-resolution CT scanning with the CNN-UNet model in otolaryngology, paving the way for more accurate diagnosis and more individualized treatment plans for patients experiencing auriculotemporal and ossicle-related disruptions.

BACKGROUND: Auriculocondylar syndrome (ARCND) is a rare congenital craniofacial developmental malformation syndrome of the first and second pharyngeal arches with external ear malformation at the junction between the lobe and helix, micromaxillary malformation, and mandibular condylar hypoplasia. Four subtypes of ARCND have been described so far, that is, ARCND1 (OMIM # 602483), ARCND2 (ARCND2A, OMIM # 614669; ARCND2B, OMIM # 620458), ARCND3 (OMIM # 615706), and ARCND4 (OMIM # 620457).
METHODS: This study reports a case of ARCND2 resulting from a novel pathogenic variant in the PLCB4 gene, and summarizes PLCB4 gene mutation sites and phenotypes of ARCND2.
RESULTS: The proband, a 5-day-old male neonate, was referred to our hospital for respiratory distress. Micrognathia, microstomia, distinctive question mark ears, as well as mandibular condyle hypoplasia were identified. Trio-based whole-exome sequencing identified a novel missense variant of NM_001377142.1:c.1928C>T (NP_001364071.1:p.Ser643Phe) in the PLCB4 gene, which was predicted to impair the local structural stability with a result that the protein function might be affected. From a review of the literature, only 36 patients with PLCB4 gene mutations were retrieved.
CONCLUSIONS: As with other studies examining familial cases of ARCND2, incomplete penetrance and variable expressivity were observed within different families\' heterozygous mutations in PLCB4 gene. Although, motor and intellectual development are in the normal range in the vast majority of patients with ARCND2, long-term follow-up and assessment are still required.

OBJECTIVE: To make surgeons aware of the differing types of preauricular sinuses (PAS), we summarize our experience with diagnosis and treatment of varying types of PAS.
METHODS: We retrospectively reviewed clinical data from patients who had undergone preauricular fistulectomy between March 2015 and March 2020. These patients were categorized into two groups according to locations of congenital fistula pit.
RESULTS: Twelve patients with variant PAS accounted for 6.8% (12/177) of all patients. The variant types of PAS could be classified into three types (from type 1 to type 3), based on the location of the fistula pit. Type 1 (seven patients; eight ears) patients had pits located on the ascending helix crus, whereas type 2 (four patients, four ears) and type 3 (one patient, one ear) patients had pits located on the external auditory canal (EAC) and lobule, respectively. Fistular tracts penetrated the cartilage of the helix crus in seven of the type 1 variant ears. Swelling and discharge were located at the ascending helix crus (in four ears), cavum concha (in two ears), and posterior to the auricle (in one ear). In four of the type 2 ears, the fistular tracts were located at the anterior margin of the ascending limb of the helix.
CONCLUSIONS: Fistula tracts where fistula pit occurred on the ascending helix crus were more likely to penetrates through the cartilage, and fistula tracts with fistula pits that occurred on the EAC were adjacent to the cartilage of the ascending helix and tragus. Meticulous dissection and complete removal of fistula tissue are critical to avoid postoperative recurrence.
METHODS: 4 Laryngoscope, 134:3839-3845, 2024.

BACKGROUND: Primrose syndrome is an autosomal dominant disorder characterized by craniofacial dysmorphism, mental retardation, developmental delay, progressive muscle atrophy and calcification of the earlobe due to a mutation in the ZBTB20.
METHODS: We reported a case of a Chinese boy with clinical symptoms resembling Primrose Syndrome, and performed genetic etiology analysis of the proband\'s family through Trio whole exome sequencing.
RESULTS: A novel missense variant c.1927T>A(p.F643I) in exon 14 of the ZBTB20 (NM_001348803) was identified in the proband. This is the first report case of primrose syndrome in China, and our case extends the variant spectrum of ZBTB20 and further strengthens the understanding of primrose syndrome.
CONCLUSIONS: However, there are no formal clinical guidelines for the management of this disease, and research on treatment and prognosis remains a challenge and focus in future.

OBJECTIVE: To evaluate the feasibility and safety of employing a Eustachian tube video endoscope with a supporting balloon as a viable treatment and examination option for patients with Eustachian tube dysfunction.
METHODS: A study involving nine fresh human cadaver heads was conducted to investigate the potential of balloon dilatation Eustachian tuboplasty using a Eustachian tube video endoscope and a supporting balloon catheter. The Eustachian tube cavity was examined with the Eustachian tube video endoscope during the procedure, which involved the dilatation of the cartilaginous portion of the Eustachian tube with the supporting balloon catheter.
RESULTS: The utilisation of the Eustachian tube video endoscope in conjunction with the supporting balloon catheter demonstrated technical ease during the procedure, with no observed damage to essential structures, particularly the Eustachian tube cavity.
CONCLUSIONS: This newly introduced method of dilatation and examination of the Eustachian tube cavity using a Eustachian tube video endoscope and the supporting balloon is a feasible, safe procedure.

Objective:To analyze the clinical diagnosis, treatment ，and surgical timing of otogenic intracranial complications. Methods:The clinical data of 11 patients with intracranial complications with ear symptoms as the first manifestation in Department of Otorhinolaryngology Head and Neck Surgery, Qilu Hospital of Shandong University（Qingdao） from December 2014 to June 2022 were collected, including 8 males and 3 females, aged from 4 to 69 years. All patients had complete otoendoscopy, audiology, imaging and etiology examination, and the diagnosis and treatment plan was jointly developed through multidisciplinary consultation according to the critical degree of clinical symptoms and imaging changes. Among the 11 patients, 5 cases were treated with intracranial lesions first in neurosurgery department and middle ear lesions later in otolaryngology, 3 cases of meningitis, were treated with middle ear surgery after intracranial infection control, 1 case was treated with middle ear lesions and intracranial infection simultaneously, and 2 cases were treated with sigmoid sinus and transverse sinus thrombosis conservatively. They were followed up for 1-6 years. Descriptive statistical methods were used for analysis. Results:All the 11 patients had ear varying symptoms, including ear pain, pus discharge and hearing loss, etc, and then fever appeared, headache, disturbance of consciousness, facial paralysis and other intracranial complication. Otoendoscopy showed perforation of the relaxation of the tympanic membrane in 5 cases, major perforation of the tension in 3 cases, neoplasia in the ear canal in 1 case, bulging of the tympanic membrane in 1 case, and turbidity of the tympanic membrane in 1 case. There were 4 cases of conductive hearing loss, 4 cases of mixed hearing loss and 3 cases of total deafness. Imaging examination showed cholesteatoma of the middle ear complicated with temporal lobe brain abscess in 4 cases, cerebellar abscess in 2 cases, cholesteatoma of the middle ear complicated with intracranial infection in 3 cases, and sigmoid sinus thrombophlebitis in 2 cases. In the etiological examination, 2 cases of Streptococcus pneumoniae were cultured in the pus of brain abscess and cerebrospinal fluid, and 1 case was cultured in streptococcus vestibularis, Bacteroides uniformis and Proteus mirabilis respectively. During the follow-up, 1 patient died of cardiovascular disease 3 years after discharge, and the remaining 10 patients survived. There was no recurrence of intracranial and middle ear lesions. Sigmoid sinus and transverse sinus thrombosis were significantly improved. Conclusion:Brain abscess, intracranial infection and thrombophlebitis are the most common otogenic intracranial complications, and cholesteatoma of middle ear is the most common primary disease. Timely diagnosis, multidisciplinary collaboration, accurate grasp of the timing in the treatment of primary focal and complications have improved the cure rate of the disease.
目的：分析耳源性颅内并发症的临床诊断、治疗方式及手术时机的选择。 方法：收集2014年12月—2022年6月就诊于山东大学齐鲁医院（青岛）耳鼻咽喉头颈外科以耳部症状为首发表现的颅内并发症共11例患者的临床资料，其中男8例，女3例，年龄4～69岁。所有患者均有完善的耳内镜检查、听力学检查、影像学检查及病原学检查。采取神经内科、神经外科、感染科、影像科及耳鼻咽喉科多学科协作模式，根据临床症状危急程度、影像学变化情况，共同制定抗感染药物治疗及手术治疗方案。11例患者中，有5例神经外科先处理颅内病变，耳鼻咽喉科后处理中耳病变；3例脑膜炎患者先控制急性期脑炎症状，后处理中耳病变，1例中耳病变和颅内感染灶同期处理，2例处理中耳病变后，乙状窦、横窦血栓行保守治疗。随访时间为1～6年。采用描述性统计学方法进行分析。 结果：11例患者均有时间不等的耳部症状，表现为耳痛、流脓、听力下降等，后出现发烧、头痛、意识障碍、面瘫等颅内并发症表现。耳内镜检查表现为鼓膜松弛部穿孔5例、紧张部大穿孔3例，耳道新生物1例，鼓膜膨出1例，鼓膜浑浊内陷1例。听力学检查传导性听力下降4例，混合性听力下降4例，全聋3例。影像学检查提示中耳胆脂瘤合并颞叶脑脓肿4例、小脑脓肿2例，中耳胆脂瘤合并脑膜炎3例，中耳感染合并乙状窦血栓性静脉炎2例。病原学检查中，脑脓肿脓液及脑脊液培养肺炎链球菌2例次，前庭链球菌、单形拟杆菌、奇异变形杆菌各1例次。随访过程中，1例出院后3年因合并心血管疾病去世，其余10例健在，定期复查颅内及中耳病变无复发。 结论：耳源性颅内并发症以脑脓肿、脑膜炎及乙状窦血栓性静脉炎最常见，中耳胆脂瘤为最常见的病因。及时诊断、多学科协作、原发灶及合并症处理时机的准确把握提高了本病的治愈率。.

OBJECTIVE: This study aims to compare the characteristics of Wideband Acoustic Immittance (WAI) in Chinese infants under three months of age, with either normal or abnormal middle ear function.
METHODS: We recruited 98 infants with either normal or abnormal middle ear function, and subsequently divided them into four groups based on their middle ear function and chronological age. The absorbances at tympanometric peak pressure (TPP) were collected across 1/3rd octave frequencies ranging from 226 to 8000 Hz.
RESULTS: Among infants with normal middle ear function, no significant differences were observed concerning ear laterality. However, significant differences were noted at 3364 Hz and 4000 Hz with respect to age. For infants with either normal or abnormal middle ear function, we found significant differences at the majority of frequencies. Additionally, the receiver operating characteristic (ROC) curves and maxima Youden index indicated that absorbances at 1682 Hz and 1297 Hz could be employed to evaluate the middle ear function of infants at 1 and 2 months of age.
CONCLUSIONS: This study demonstrates that WAI holds promise as a valuable tool for assessing the middle ear condition of infants at 1 and 2 months of age. Infants aged 1 and 2 years, having absorbance values equal to or greater than 0.7470 at 1682 Hz and 0.6775 at 1297 Hz respectively, may indicate normal middle ear function. Furthermore, it underscores the necessity of establishing ethnicity- and age-specific norms for WAI in infants under 3 months of age.