Objective:To retrospectively analyze the clinical features and surgical efficacy of congenital cholesteatoma （CC） and acquired cholesteatoma （AC） in children. Methods:Clinical data of 169 children with middle ear cholesteatoma were reviewed in the Department of Otorhinolaryngology Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University from January 2010 to July 2020. The clinical characteristics, stages, surgical methods, and postoperative recurrence rates were analyzed and summarized. Results:The age distribution of enrolled children ranged from 2 to 14 years. The mean age of the CC group was （5.60±2.48） years compared with （6.45±2.48） years in the AC group, and the difference was statistically significant （P<0.05）. Preoperative hearing in the CC group was （40.06±13.52） dB HL, which was better than in the AC group at （48.40±13.84） dB HL （P<0.05）. The proportion of stage Ⅰ in the CC group was lower than that in the AC group according to EAONO/JOS staging （P<0.05）. The recurrence rate after primary surgery was 19.23% （10/52） in the CC group compared with 36.29% （45/124） in the AC group （P<0.05）. The mastoid retention rates after all operations were 28.85% （15/52） in the CC group and 5.65% （7/124） in the AC group （P<0.05）. Conclusion:Compared with congenital cholesteatoma, acquired cholesteatoma in children is more aggressive and has more complications, higher postoperative recurrence rate, and less possibility of mastoid retention. Early clinical detection and treatment are required, and canal wall-down tympanoplasty should be considered in surgery.
Humans ; Cholesteatoma, Middle Ear/congenital* ; Child ; Retrospective Studies ; Child, Preschool ; Adolescent ; Male ; Female ; Recurrence ; Cholesteatoma/congenital* ; Tympanoplasty ; Treatment Outcome

OBJECTIVETo analyze the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane, and summarize the key diagnostic points, differential diagnosis and observe the effects of surgical treatment.

METHODSWe reviewed data from 82 patients with unilateral conductive hearing loss with intact tympanic membranes who accepted the exploratory tympanotomy from April 2011 to September 2013. There were 41 males and 41 females, aged from 7 to 66( averaged 26.5±13.7)years, with a history of one month to 50 years. The history, clinical symptoms, audiological evaluation, high resolution temporal bone CT, the results of surgical exploration and hearing reconstruction were analyzed.

RESULTSThe exploratory tympanotomy revealed 43 cases of congenital middle ear malformations (52.4%), 22 cases of otosclerosis (26.8%), eight cases of congenital cholesteatoma (9.8%), six cases of trauma induced conductive hearing loss (7.3%), three cases of congenital ossicular malformations with congenital cholesteatoma (3.7%). Progressive hearing loss was common in patients with otosclerosis and congenital cholesteatoma, and patients with congenital middle ear malformations described their hearing loss since childhood. High resolution temporal bone CT of congenital middle ear malformation, trauma induced conductive hearing loss, congenital cholesteatoma diagnosis rate was 40.0%, 50.0%, and 83.3% respectively. The preoperative air-conductive threshold of patients with absence of the oval window were increased to (66.9±1.1)dBHL, the preoperative bone-conductive threshold achieved (28.3±10.4)dBHL at 2 000 Hz. While patients with stapes fixation and that with ossicular chain discontinuity were (27.2±9.7)dBHL and (17.8±8.8)dBHL(P=0.000)respectively. Through the tympanic exploration with endaural incision under the microscope, different hearing reconstruction were applied according to different lesions. After the operation, the hearing level of 52 patients with return visit were improved, the mean air-conductive threshold were decreased from (60.0±11.4)dBHL to (32.2±12.1)dBHL(P=0.000); and the mean ABG were decreased from (43.2±12.0)dB to (16.3±9.4)dB(P=0.000).

CONCLUSIONSCongenital middle ear malformations, otosclerosis, congenital cholesteatoma are the most common causes in unilateral conductive hearing loss with an intact tympanic membrane. The diagnosis rate can be improved by analyzing the clinical features. Through exploratory tympanotomy and hearing reconstruction, we can clarify the diagnosis and achieve a satisfying hearing recover.

Adolescent ; Adult ; Aged ; Audiometry ; Child ; Cholesteatoma ; congenital ; pathology ; Diagnosis, Differential ; Ear Ossicles ; pathology ; Ear, Middle ; abnormalities ; Female ; Hearing Loss, Conductive ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Middle Ear Ventilation ; Otosclerosis ; pathology ; Tympanic Membrane ; Young Adult

OBJECTIVE: To investigate the location, staging, clinical symptoms, imaging features, and surgical treatment of the congenital cholesteatoma of middle ear (CCME). METHOD: This was a retrospective review of 20 CCME cases. RESULT: Of 20 cases with CCME, 2 cases were classified as Postic stage I, 0 as stage II, 13 as stage III, 5 as stage IV. Conductive hearing loss was the most common clinical symptom. The mean preoperative PTA was 54.1 dB, and the mean ABG was 41.7 dB. One case underwent a modified mastoidectomy and a second-stage ossicular reconstruction; 2 cases experienced a radical mastoidectomy without ossicular reconsturction for extensive cholesteatoma; 5 cases underwent modified mastoidectomy and a one-stage tympanoplasty, with one case diagnosed as congenital malformation of ossicular chain (stapes hypoplasia); other 12 cases underwent a one-stage tympanoplasty. The cholesteatoma localized to the posterior-epitympanum or mesotympanum in all patients, mainly located in the incudostapedial joint. The mean postoperative PTA from 16 cases was 35.3 dB, and A-B gap was 20.2 dB. All patients were followed-up for at least 1 year after operation and recurrence was found in 2 cases. Three cases were accompanied with congenital malformation of ossicular chain. CONCLUSION CCME is a rare entity and diagnosis is usually delayed in clinical practice due to the silent nature of disease in its early stage. The prognosis of CCME mainly depended on the stage of the lesions.
Adolescent ; Adult ; Child ; Child, Preschool ; Cholesteatoma ; classification ; congenital ; pathology ; surgery ; Cholesteatoma, Middle Ear ; classification ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Male ; Retrospective Studies ; Young Adult

OBJECTIVE: To investigate the clinical features and operation treatment of the congenital middle ear cholesteatoma. METHOD: A retrospective review of clinical and surgical records of 10 patients with congenital middle ear cholesteatoma were performed. All patients were treated by surgeries,5 of 10 cases deal with one-stage tympanoplasty after drum exploration by external auditory meatus, 3 cases dealed with closed mastoidotympanectomy and tympanomastoidectomy, 2 cases dealed with open mastoidotympanectomy and tympanomastoidectomy. RESULT: The cholesteatomas were located at or around the posterior tympanum or mesotympanum in 5 patients, confined to the tympanic cavity and attic in 3 patients, advanced cholesteatoma that extended from the tympanic cavity into the mastoid antrum was seen in 2 patients. The mean postoperative PTA was 30 dB HL, the mean ABG was within 20 dB, after six months. No residual or recurrence of cholesteatoma was found. CONCLUSION The congenital cholesteatoma often originates from the posterior or anterior of the middle ear, with hiding lesion, thus result in severe conductive hearing loss. Imaging examination plays an important role in diagnosing and treating of congenital cholesteatoma. Early stage surgical treatment can obtain a good hearing reconstruction effect.
Adolescent ; Adult ; Child ; Child, Preschool ; Cholesteatoma ; congenital ; surgery ; Cholesteatoma, Middle Ear ; surgery ; Ear, Middle ; physiopathology ; Female ; Follow-Up Studies ; Humans ; Male ; Retrospective Studies ; Tympanoplasty ; methods ; Young Adult

OBJECTIVE: To investigate the location, clinical symptom, image features, treatment and prognosis of the congenital middle ear cholesteatoma. METHOD: Retrospective review of 9 patients treated at Department of Otolaryngology Head and Neck Surgery, the affiliated Hospital of the first Strasbourg University, France. RESULT: The mean age was 6.1 years. Including 7 boys and 2 girls with hearing loss, and 7 of them suffered conductive hearing loss. Typical cholesteatomas were found behind posterior tympanic membrane according the otoscopic examinations in 6 cases. CT were completed in 8 of 9 cases, spheroid or ovoid masses were found in the tympanic cavities, most of them located in the posterior region. All of the patients underwent the surgical treatment, 5 of 9 cases had the ossicular erosion. Four of 9 cases had the residual diseases. All of the cases were followed-up at the mean period of 5.3 years, the preoperative hearing threshold is 28 dB compared with 26 dB of postoperative hearing threshold. CONCLUSION Congenital middle ear cholesteatoma is a rare and hiding lesion. The symptoms are more typical in children than that in adults, which is unilateral conductive hearing loss. Likewise,the preoperative CT scanning is important. The rate of residual lesions and prognosis of congenital cholesteatoma is mainly depended on the extension and the methods of the surgery, revision surgery as well.
Auditory Threshold ; Child ; Child, Preschool ; Cholesteatoma, Middle Ear ; congenital ; diagnosis ; Female ; Hearing Loss, Conductive ; etiology ; Humans ; Male ; Prognosis ; Retrospective Studies

Congenital middle ear cholesteatoma (CMEC) is a rare disease entity in otolaryngology. However, we try to assess the characteristic features and recurrences of CMEC in pediatric patients according to stages, and to determine the value of preoperative computed tomography (CT) scan. Retrospective review of 35 cases of CMEC under the age of 15 yr that had been treated at the tertiary referral center from 1995 through 2006. The main outcome measures were CT findings, surgical findings, recurrence rate and hearing assessment. Preoperative CT scan accurately predicted the extent of the cholesteatoma seen during surgery in 30/35 (85.7%). The recurrence rate of CMEC was 5.7% (2/35) and all of recurred cases were stage IV. In recurred cases, cholesteatomas were extended to sinus tympani and facial recess at revisional operation as well as initial operation. So we concluded that preoperative CT scan is essential in defining the extent of existing pathology. The intraoperative CMEC extension and location influence the outcome of surgery. In the higher stages, careful eradication of disease, particularly in the region of sinus tympani and facial recess is recommended.
Adolescent ; Audiometry, Pure-Tone ; Child ; Child, Preschool ; Cholesteatoma, Middle Ear/*congenital/*diagnosis/surgery ; Female ; Humans ; Male ; Recurrence ; Retrospective Studies ; Severity of Illness Index ; Tomography, X-Ray Computed

As the auricle is a very complex structure covered with thin, sensitive skin, the best reconstruction requires a great deal of skill and the use of diverse approaches. Otolaryngologists should give greater consideration to otoplasty because patients do not want their self-confidence impaired by auricular deformities. When treating external auditory canal (EAC) cholesteatoma caused by EAC stenosis, the blood circulation may be deleteriously affected if the retroauricular incision is made first, and thus the otoplasty must be first carried out. We report two cases of otoplasty performed simultaneously with meatoplasty without the aid of plastic surgeons. The first case involved a 33-year-old woman with cholesteatoma due to congenital auricular atresia and microtia. The second case was a 64-year-old man with traumatic auricular amputation and EAC stenosis due to scar contracture. Simultaneous otoplasty and meatoplasty should be of benefit to patients because it reduces the number of operations and has a high success rate.
Adult ; Aminocaproic Acids ; Amputation ; Blood Circulation ; Cholesteatoma ; Cicatrix ; Congenital Abnormalities ; Constriction, Pathologic ; Contracture ; Ear ; Ear Canal ; Female ; Humans ; Middle Aged ; Skin

Adolescent ; Adult ; Aged ; Cholesteatoma, Middle Ear ; congenital ; pathology ; Female ; Humans ; Male ; Melanocytes ; Middle Aged ; Young Adult

Adolescent ; Cholesteatoma ; congenital ; Cholesteatoma, Middle Ear ; congenital ; Humans ; Male

Adult ; Cholesteatoma ; congenital ; Female ; Humans ; Temporal Bone