[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.

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

Objective/Hypothesis: Middle ear cholesteatoma is characterized by abnormal growth of the keratinizing squamous epithelium of the temporal bone. d-ß-aspartic acid is the major isomer of d-aspartic acid found in elderly tissue. We assessed the immunoreactivity to k-ß-aspartic acid of congenital and acquired middle ear cholesteatomas. Study Design: Case-control studies. Material and Methods: Tissue 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-ß-aspartic acid expression were then assessed immunohistochemically. Results: Two patterns of immunoreactivity to d-ß-aspartic acid were detected in middle ear cholesteatoma: infiltrative and diffuse. In congenital middle ear cholesteatoma, d-ß-aspartic acid expression was observed throughout all the layers (perimatrix, matrix, and cystic contents), and immunoreactivity to d-ß-aspartic acid was dramatically strong in all layers. The expression levels of d-ß-aspartic acid to the cystic content and perimatrix were significantly higher in congenital middle ear cholesteatoma than in the acquired type. Conclusions: This study showed the expression levels of d-ß-aspartic acid in middle ear cholesteatoma to differ significantly between congenital and acquired middle ear cholesteatoma. Our results indicate that overexpression of d-ß-aspartic acid is likely to be involved in the pathogenesis of cholesteatoma, and we speculate that d-ß-aspartic acid could be a novel biomarker for, and a therapeutic target in, congenital and acquired middle ear cholesteatoma. Level of Evidence: 4.

Suggestion of a Modified Classification for Congenital Middle Ear Cholesteatoma: Based on the Clinical Characteristics and Staging of Fifty-Seven Patients.

Objective: To explore more refined classification methods of congenital middle ear cholesteatoma (CMEC) based on two existing staging systems. Subjects and Methods: This study involved a retrospective data review of 57 patients (61 ears involved) with CMEC requiring the surgical treatment. Patients were classified into different stages according to Nelson, Potsic, and Modified Nelson staging system. Preoperative data and intraoperative findings were recorded. Results: The mean age at operation was 15 ± 15.04 years with a median of 10 years. The main clinical manifestation was hearing loss (72.13%). CMEC mass was mainly located in the posterior portion of the tympanic cavity (65.57%). No patient was classified into Potsic stage II. The erosion of incus happened in all cases. Patients with Nelson type 2 and type 3 had erosions to the structures out of middle ear, such as dura mater, lateral semicircle canal, and facial canal. Postoperative follow-up time was more than 24 months. Recurrence occurred in four patients (6.56%), all of them in Nelson type 2, who had received canal wall down mastoidectomy (three cases) and canal wall up mastoidectomy (one case). Conclusions: Nelson staging system was more suitable for advanced CMEC patients than Potsic staging system. The rare case of Potsic stage II restricted the application of Potsic staging system. Moreover, since both of two staging systems do not distinguish the type of involved ossicles, the authors recommended to subdivide Nelson type 2 into type 2a and type 2b based on the erosion of the ossicular chain, as well as subdivide Nelson type 3 into type 3a and 3b based on the erosion of structures out of middle ear, which was named as Modified Nelson staging system.