Congenital cholesteatoma of the middle ear-a report of 10 cases

Objectives To study clinical, imaging features and treatment outcomes of congenital cholesteatoma of middle ear (CCME). Methods This is a retrospective review of 10 CCME cases selected from 952 cholesteatoma cases treated between January 1995 and December 2005 at the Department of Otolaryngology-Head and Neck Surgery, Chinese PLA General Hospital. The main outcome measures were the site of origin, clinical features, surgical findings, imaging characteristics and hearing results. Results The mean age of the 10 patients was 16 years(ranged from 10 to 24 years), with 6 being older than 18 years. There were 7 males and 3 females. The average delay to diagnosis was longer than 2 years. The mean preoperative PTA was 55 dB HL, with a mean ABG of 45 dB. Typical cholesteatomas were seen behind the tympanic membrane in the superoposterior quadrant on otoscopy only in 2 patients. High resolution CT was completed in all patients. Most of the patients(8/10) were diagnosed with otosclerosis or ossicular abnormality before operation. All patients underwent a one-stage tympanoplasty following transmeatal explorative tympanotomy and complete cholesteatoma removal, except one, who underwent a CWU mastoidectomy due to extensive cholesteatoma involvement. The choleasteatoma lesion was confined to the superoposterior mesotympanum in all patients. The mean postoperative PTA was 20 dB HL. All patients were followed-up for at least 1.5 years postoperatively. Revision procedures were performed in 2 patients for hearing deterioration. No residual or recurrence of cholesteatoma was found. Conclusion CCME is a rare disease that often gets delayed diagnosis. Residual lesions and the prognosis mainly depend on the extent of the lesion.

Pediatric Middle Ear Congenital Cholesteatoma: A Case Report

Congenital cholesteatoma（CC）is a rarely seen benign tumor of the temporal bone. There are five general sites of extradural occurrence: the middle ear, external auditory meatus, mastoid, squamous portion and the petrous apex of the temporal bone. CC grows slowly and presents no symptoms at the early stage. Delayed and mis-diagnosis are common with this condition. Case report A 10-year-old boy presented with a 3-month history of hearing loss on right side. There was no history of otorrhea, facial palsy, previous otological procedures or trauma. Otoscopy revealed a bulging posterosuperior quadrant in the otherwise intact right tympanic membrane （Fig.1）. Pure tone audiometry showed an average threshold of 51 dB for 500, 1000, 2000 and 4000Hz, with a 40 dB air-bone gap, suggesting a moderate conductive hearing loss（Fig.4）. CT scan of the temporal bone showed an isolated soft tissue density lesion in the middle ear（Fig.2）.

Diagnosis of Middle Ear Diseases Based on Convolutional Neural Network

An otoscope is traditionally used to examine the eardrum and ear canal.A diagnosis of otitis media(OM)relies on the experience of clinicians.If an examiner lacks experience,the examination may be difficult and time-consuming.This paper presents an ear disease classification method using middle ear images based on a convolutional neural network(CNN).Especially the segmentation and classification networks are used to classify an otoscopic image into six classes:normal,acute otitis media(AOM),otitis media with effusion(OME),chronic otitis media(COM),congenital cholesteatoma(CC)and traumatic perforations(TMPs).The Mask R-CNN is utilized for the segmentation network to extract the region of interest(ROI)from otoscopic images.The extracted ROIs are used as guiding features for the classification.The classification is based on transfer learning with an ensemble of two CNN classifiers:EfficientNetB0 and Inception-V3.The proposed model was trained with a 5-fold cross-validation technique.The proposed method was evaluated and achieved a classification accuracy of 97.29%.