Spontaneous Regression of Congenital Cholesteatoma: A Report of Two Cases with Differing Ossicle Involvement (original) (raw)

Congenital cholesteatoma of the middle ear - uncommon clinical presentation

Vojnosanitetski pregled, 2014

Introduction. Congenital cholesteatoma of the middle ear is un uncommon and yet not well-defined disease. Only few cases of cholesteatoma in the fossa ovalis with unusual clinical presentation have been reported in medical literature. Case report. We reported a 16-year-old girl with congenital cholesteatoma in the fossa ovalis with minimal clinical presentation. A small mass was found occluding the fossa ovalis and mimicking otosclerotic process within tympanic cavity. The operation started as stapedotomy, and when the process was confirmed it converted to mastoidectomy via the retroauricular approach. Conclusion. The diagnosis of congenital cholesteatoma in children should always be considered, even if the clinical symptoms imitate other ear disorders, in our case otosclerosis.

Unusual cases of congenital cholesteatoma of the ear

The Journal of Laryngology & Otology, 1999

Congenital cholesteatoma may originate at various sites in the temporal bone. For example, in the petrous apex, the cerebellopontine angle, the middle ear cavity, the mastoid process or the external auditory canal. The least common site being the mastoid process. We present two cases of congenital cholesteatoma of the mastoid process, each presenting with different symptoms and at different ages. Both patients underwent surgical treatment, which confirmed the diagnosis and radiological findings.

A Unified Classification of Middle Ear and Petrous Bone Congenital Cholesteatomas

The Journal of International Advanced Otology

BACKGROUND: The aim of this study was to classify congenital cholesteatoma along an entire spectrum of involvement ranging from the middle ear to petrous apex. METHODS: A total of 131 patients (85 adults and 46 children) underwent operations for congenital cholesteatoma over the duration of 27 years. RESULTS: For most cases, middle ear mucosa was normal, the first ossicle eroded by the mass was the stapes, and the mastoid air cell system was well-pneumatized on intraoperative and radiographic views. Totally 34% of patients presented with facial nerve weakness and 45% of these cholesteatomas arose from the supralabyrinthine area (32.8%) and from the petrous apex (12.2%). CONCLUSION: In this unified classification system, the otologist sees congenital cholesteatoma as a continuum, with facial nerve involvement and anacusis as part of the picture. This system of congenital cholesteatoma accommodates the supralabyrinthine and petrous bone locations of the disease.

Atypical presentation of congenital cholesteatoma in an adult case with good hearing result

Annals of Medicine and Surgery, 2015

Introduction: Congenital cholesteatoma is thought to be caused by inadequate folding of the epidermoid formation inside the middle ear cleft. During development of the middle ear mucosa, stratified squamous epithelium accumulates in the embryonic life. Its typical appearance is a "pearl" beneath the anterosuperior quadrant of the tympanic membrane. Presentation of case: We report 28 years-old case with congenital cholesteatoma in the posterosuperior quadrant of middle ear cavity. The main complaint was the hearing loss which had developed slowly over several years. Discussion: The case was surgically treated. Postoperative hearing result was satisfactory. Conclusion: Congenital cholesteatoma may occur in atypical locations and ages. Many authors prefer canal wall down tympanomastoidectomy. But it can also be treated successfully by intact canal wall tympanomastoidectomy with good hearing results.

Cholesteatoma: Congenital and Acquired

Military Medicine, 1978

CHOLESTEATOMA, a progressively enlarging accumulation of keratinizing squamous epithelium within the temporal bone, is a disease process which is of common interest to the pediatrician, family practitioner, and otologist. These lesions may be classified into congenital and acquired varieties,1,4,9 as noted in Table I. While the acquired types are relatively common, congenital cholesteatomas remain asymptomatic until erosion into important structures in the temporal bone, or secondary infection arouses suspicion of their presence. Those arising in the middle ear can frequently be diagnosed by otoscopy prior to the onset of significant destruction. Four cases of congenital cholesteatoma are reported. The diagnosis, pathophysiology, and management of this condition is reviewed in an effort to stimulate renewed awareness of congenital cholesteatoma, and facilitate early diagnosis and treatment prior to the development of extensive otologic disease. R~port of Cases Case J. A two year old male was referred for evaluation of recurrent episodes of bilateral, acute otitis media. There was no past history of otorrhea or hearing loss. Examination showed bilateral pearly white masses in the anterior-superior quadrant of each mesotympanum (Fig. 1). The tympanic membranes were intact, translucent and slightly retracted. Surgical exploration of the right middle ear disclosed an easily removable three to four mm spherical mass attached to the anterior portion of the neck of the malleus (Figs. 2 and 3). Six weeks later, surgical exploration of the left middle ear showed a similar lesion. Histological examination of both masses was compatible with the diagnosis of cholesteatoma. Case 2. A 26 month old male was referred because of an abnormal otologic examination. There were no symptoms of otologic disease. Examination revealed a white mass in the anterior-superior quadrant of the right mesotympanum. Surgical exploration disclosed a three to four mm mass attached to the undersurface of the tympanic membrane which was removed without difficulty. By histologic examination, the diagnosis was cholesteatoma. Case 3. A five year old male was referred in 1971 for an evaluation of serous otitis media. The tympanic membranes were intact, dull, and retracted. Pure tone audiometry showed a 25dB conductive loss in the right ear and 15dB loss in the left. Myringotomies were performed. Ten months later, hearing in the left ear deteriorated to a 53dB conductive loss. The tympanic membranes were opaque and tympanosclerotic, and myringotomies were again performed. Two years later, due to persistant conductive loss and "thickening" of the posterior-superior quadrant of the left tympanic mem-From the

Radiological Features of Acquired and Congenital Cholesteatoma

2021

This chapter provides a detailed summary of middle ear cholesteatoma. Clinical and imaging differences between acquired and congenital forms are highlighted, and potential differential diagnoses are explored. Quick summary of surgically relevant reporting points is provided as well.

Transcanal Endoscopic Ear Surgery for Congenital Cholesteatoma: A Preliminary Report

Archives of Otorhinolaryngology-Head & Neck Surgery, 2017

Objectives: Transcanal endoscopic ear surgery (TEES) has become one of the most influential methods for the treatment of cholesteatoma. The present study aimed to discuss the feasibility of transcanal endoscopic management of congenital cholesteatoma in pediatric patients. Methods: Two children with a diagnosis of congenital cholesteatoma underwent transcanal endoscopic tympanotomy and extended atticotomy to completely remove a cholesteatoma. The evaluations of these cases included surgical time, hearing gain, post-operative complications, and mastoid function reservation. Results: There were no complications after the surgeries, and the patients were followed up for at least 6 months; excellent clinical appearance and hearing improvement were noted. Conclusion: These cases demonstrated that TEES could be a satisfying alternative to traditional microscopic surgery for the management of congenital cholesteatoma, even in pediatric patients. However, one-handed surgery demands greater skill and requires more practice to achieve a good outcome.

Problems in diagnosis and treatment of cholesteatoma in children

International Journal of Pediatric Otorhinolaryngology, 1991

Our clinical study includes 56 cases of cholesteatoma in children aged 3-14 years, treated in a 6-year period. Cholesteatoma was localized in attic (6 cases), in middle ear (6 cases), in attic plus middle ear (16 cases) and in attic + middle ear + mastoid (28 cases or 50% of the total number). In the surgical treatment combined approach tympanoplasty (intact canal wall technique) was used in 76.8%, radical tympanomastoidectomy in 16.1%, and other techniques in 7.1%. Intact ossicular chain was found in 25%. Reconstruction of the ossicular chain (including autograft, homograft and allograft material) was done in 59%, and the remaining 16% were treated by classic radical surgery. Hearing results: unchanged, 48%; improved, 45%; slightly worsened, 7%; and no dead ear. Recurrency in 31% is considerably higher as compared to 15% in adults found in another comparable study by us.