In previous reports, hypothyroidism, hypopituitrism, and hypogonadism were common endocrine causes of SCFE, but this is the first time that congenital adrenal hyperplasia has been observed. As such, patients who have undergone long-term endocrine treatment for congenital adrenal hyperplasia could potentially be subjected to a higher risk for SCFE.

UNASSIGNED: Surgical 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.
UNASSIGNED: Children 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.
UNASSIGNED: Seventeen 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.
UNASSIGNED: EES 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.

OBJECTIVE: Sigmoid sinus wall anomalies (SSWA) are closely linked to venous pulsatile tinnitus (PT). This study aims to demonstrate that SSWA develops progressively rather than being congenital.
METHODS: We retrospectively analyzed 42 PT patients with SSWA who had at least two non-operative CT scans at our clinic. CT images were longitudinally assessed to track SSWA progression, while MRI and Doppler ultrasound evaluated transverse sinus stenosis and venous hemodynamics. Changes in PT perception were tracked using the tinnitus handicap inventory (THI) questionnaire.
RESULTS: Among the 42 SSWA patients, 12 (28.6%) exhibited progression. Anastomosis between diploic vein and diverticulum was significantly higher compared to the dehiscence cohort (p < 0.01). Within the diverticulum group, seven individuals (30.4%) experienced enlargement, with a mean diverticular wall expansion of 5.9% ± 11.4%. Progressive erosion was observed in two cases (12.5%) in the dehiscence cohort, with a mean sigmoid plate erosion of 3.8% ± 10.1%. In cases progressing from dehiscence to diverticulum, three subjects transitioned, with a mean sigmoid sinus wall length expansion of 43.8% ± 31.9%. SSWA progression showed a significant negative correlation with QBILATERAL (r = -0.857, p = 0.014), and there was a significant difference between initial and revisit THI scores (p < 0.01).
CONCLUSIONS: SSWA can undergo morphological progression, indicating it is a progressive clinical condition rather than congenital.
METHODS: 4 Laryngoscope, 2024.

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.
目的：探讨先天性咽鼓管骨部异常扩大畸形的临床特征。 方法：回顾性分析6例（9耳）确诊为先天性咽鼓管骨部异常扩大患者的病史、体格检查、听力检查、颞骨高分辨CT（high-resolution computed tomography，HRCT）检查等结果。 结果：6例先天性咽鼓管骨部异常扩大患者中3例双侧咽鼓管扩大，3例左侧咽鼓管扩大。7耳听力结果提示，3耳为传导性聋，平均骨导13.7 dB，平均气导71.3 dB；3耳为混合性聋，平均骨导27.7 dB，平均气导83.7 dB；1耳为极重度感音神经性聋。颞骨HRCT上骨性咽鼓管异常扩大典型表现为咽鼓管骨部增长、增宽，水平位上测量最大长度和最大宽度分别为（22.61±2.94） mm和（6.50±2.33） mm。此外，合并外耳道畸形6耳，鼓室狭小6耳，鼓窦畸形5耳，听骨链畸形7耳，耳蜗畸形6耳，蜗孔畸形3耳，前庭增宽2耳，半规管畸形3耳，前庭窗畸形6耳，面神经走形异常5耳，内听道畸形2耳，颅中窝低位8耳，颈内动脉畸形1耳。 结论：咽鼓管骨部异常扩大属于罕见的先天性中耳畸形，可以合并外耳、其他中耳结构以及内耳畸形。患者可以无咽鼓管异常开放症状而仅表现为不同程度听力下降，通常在影像学检查时被无意发现，因此颞骨HRCT具有重要提示意义。.

OBJECTIVE: To investigate the alterations in extraocular muscles (EOMs) by magnetic resonance imaging (MRI) among patients diagnosed with Duane retraction yndrome (DRS) and congenital fibrosis of the extraocular muscles (CFEOM), who present with various cranial nerve anomalies in an attempt to enhance the clinical diagnostic process.
METHODS: A case-control study was conducted to evaluate 27 patients with DRS and 14 patients with CFEOM. All patients underwent MRI scans of the brainstem and orbital examination. Neurodevelopmental assessments were conducted through MRI, and maximum cross-sectional area and volumes of EOMs were obtained. Three types of models were constructed using machine learning decision tree algorithms based on EOMs to predict disease diagnosis, cranial nerve abnormalities, and clinical subtypes.
RESULTS: Patients with bilateral CN VI abnormalities had smaller volumes of LR, MR, and IR muscles compared to those with unilateral involvement (P < 0.05). Similarly, patients with CFEOM and unilateral third cranial nerve abnormalities had a smaller maximum cross-section of the affected eye\'s SR compared to the contralateral eye (P < 0.05). In patients with both CN III and CN VI abnormalities, the volume of SR was smaller than in patients with CN III abnormalities alone (P < 0.05). The prediction model using EOMs volume showed a diagnostic precision of 82.5% for clinical cases and 60.1% for predicting cranial nerve abnormalities. Nonetheless, the precision for identifying clinical subtypes was relatively modest, at only 41.7%.
CONCLUSIONS: The distinctive volumetric alterations in EOMs among individuals exhibiting distinct cranial nerve anomalies associated with DRS or CFEOM provide valuable diagnostic insights into to Congenital Cranial Neurodevelopmental Disorders (CCDDs). MRI analysis of EOMs should thus be regarded as a crucial diagnostic modality.

Lipoma of the tongue is a rare benign tumor that accounts for approximately 1% to 5% of all oral cavity tumors while 0.3% of tumors are of the tongue. Notably, it is rarer in children. In this article, we report the case of a 4-year-old girl with lipomas of the tongue. The lipomas were found at age 1 year by her parents, located on the tip, ventro, and dorsum of the tongue, and presenting with a trend to increase gradually. At the time of presentation to the hospital at age 4 years, the articulatory function of the patient was partially affected, and surgical excision was performed. The surgery was uneventful, and no evidence of recurrence was noted at 3 month follow-up.

OBJECTIVE: To observe the efficacy of EarWell ear orthosis in treating children with different types of ear deformities.
METHODS: We selected 80 children aged <6 weeks with ear deformities (110 ears: 15, 30, 21, 25, and 19 ears with prominent ear, lop ear, cup ear, cryptotia, and helical rim deformity, respectively). Differences in effectiveness rate, treatment time, and incidence of complications among children with different types of auricular deformities were compared. Recurrence rates at 1 and 3 months after the treatment were compared.
RESULTS: The overall success rate was 92.73 %, and the treatment effectiveness rate did not differ significantly among the children with different types of auricular malformations (P > 0.05). The correction time of the helical rim deformity was the shortest, and the correction times of the prominent and cup ears were significantly longer than those of the other groups (P < 0.05). The incidence of complications associated with helical rim deformity and lop ear was lower, and the incidence of prominent and cup ear complications was significantly higher than that in the other groups (P < 0.05). The recurrence rate in children with prominent and cup ears was higher at 1 and 3 months after correction, and children with a lop ear and cryptotia showed no recurrence at 1 and 3 months after treatment, which correlated with the correction time, incidence of complications, and recurrence rate (P < 0.05) CONCLUSION: The EarWell auricle orthosis is an effective treatment in children with auricular morphological malformations. Correction time, complication rate, and recurrence rate were related to the malformation type.

OBJECTIVE: Clinical presentations encompass respiratory, feeding issues, nasopharyngeal mass, meningitis, CSF leakage, craniofacial anomalies, and endocrine problems. Surgery is the primary treatment, transitioning from frontal craniotomy to endoscopic methods, offering improved outcomes. Yet, more studies are needed. A comprehensive review on trans-sellar trans-sphenoidal encephalocele (TSTSE) is missing. Our study aims to fill this gap, offering a comprehensive perspective for physicians.
METHODS: This review adhered to the PRISMA guideline. Eligible studies focused on human subjects, specifically trans-sellar encephaloceles, and provided comprehensive treatment details. English language articles published up to April 11th, 2023, were considered. Two trained researchers conducted article screening using consistent criteria. Data extraction covered various aspects, including clinical presentation, surgical methods, and outcomes, with results presented descriptively in two tables. Due to the rarity of this congenital anomaly, meta-analysis and publication bias assessment were not feasible. Data extraction was independently conducted by two reviewers, with subsequent cross-verification.
RESULTS: A total of 36 patients were identified from 14 studies, the most frequently observed clinical presentation was dyspnea (41.67%) and the most frequently observed accompanying anomaly was cleft lip/palate (55.56%). CT and MRI were adopted in nearly all the cases, and trans-nasal approach was the most often used surgical approach (57.14%) with the \'soft material combination\' the most commonly used method for cranial base repairment (35.71%). A total of two deaths occurred and diabetes insipidus was the most common perioperative complication which occurred in six surgery patients (21.43%).
CONCLUSIONS: TSTSE predominantly affects males and presents with dyspnea, visual deficits, pituitary insufficiency, and cranial base-related symptoms. Early diagnosis is critical, with advanced imaging playing a key role. Endocrine assessment is vital for hormone management. Surgery offers symptom relief but entails risks, including reported fatalities and complications. The choice between surgery and conservative management requires careful deliberation. The trans-nasal approach is favored for its reduced trauma, yet further research is necessary to validate this preference.

Localized congenital cutis verticis gyrate (CVG) is rare and potentially risks skull involvement. A 23-year-old woman presented with a congenital scalp mass in the occipital region. Local thickening of her left occipital scalp with ridges and furrows was observed on examination. Head computed tomography scan showed a lytic area underneath the same area of the occipital calvarium. The mass was surgically removed due to the skull erosion and cosmetic reasons. Pathologic evaluation established CVG. Surgical excision is best for localized congenital CVG with skull erosion due to cosmetic reasons. Surgical excision was rewarding to the patient it allowed her to style her hair.

OBJECTIVE: The occurrence and predictors of symptomatic subdural hygroma (SSH) subsequent to the fenestration of pediatric intracranial arachnoid cysts (IACs) are unclear. In this study, the authors aimed to investigate the likelihood of an SSH following IAC fenestration and the impact on operative efficacy with the ultimate goal of constructing a nomogram.
METHODS: The medical records of 1782 consecutive patients who underwent surgical treatment at the Xin Hua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine were reviewed. Among these patients, a training cohort (n = 1214) underwent surgery during an earlier period and was used for the development of a nomogram. The remaining patients formed the validation cohort (n = 568) and were used to confirm the performance of the developed model. The development of the nomogram involved the use of potential predictors, while internal validation was conducted using a bootstrap-resampling approach.
RESULTS: SSH was detected in 13.2% (160 of 1214) of patients in the training cohort and in 11.1% (63 of 568) of patients in the validation cohort. Through multivariate analysis, several factors including Galassi type, IAC distance to the basal cisterns, temporal bulge, midline shift, IAC shape in the coronal view, area of the stoma, and artery location near the stoma were identified as independent predictors of SSH. These 7 predictors were used to construct a nomogram, which exhibited a concordance statistic (C-statistic) of 0.826 and demonstrated good calibration. Following internal validation, the nomogram maintained good calibration and discrimination with a C-statistic of 0.799 (95% CI 0.665-0.841). Patients who had nomogram scores < 30 or ≥ 30 were considered to be at low and high risk of SSH occurrence, respectively.
CONCLUSIONS: The predictive model and derived nomogram achieved satisfactory preoperative prediction of SSH. Using this nomogram, the risk for an individual patient can be estimated, and the appropriate surgery can be performed in high-risk patients.