Congenital cholesteatoma together with ossicular chain anomaly

2016 ◽  
Vol 274 (2) ◽  
pp. 1179-1182 ◽  
Author(s):  
Leyla Kansu ◽  
Levent N. Ozluoglu
Keyword(s):  
Ossicular Chain ◽  
Congenital Cholesteatoma

Bilateral congenital cholesteatoma in branchio-oto-renal syndrome

1999 ◽  
Vol 113 (9) ◽  
pp. 841-843 ◽  
Author(s):  
George A. Worley ◽  
Archana Vats ◽  
Jonathan Harcourt ◽  
David M. Albert
Keyword(s):  
Hearing Loss ◽  
Autosomal Dominant ◽  
Ossicular Chain ◽  
Branchial Arch ◽  
Congenital Cholesteatoma ◽  
Autosomal Dominant Condition ◽  
Dominant Condition

AbstractBranchio-oto-renal syndrome is a rare autosomal dominant condition characterized by hearing loss, branchial arch abnormalities and renal tract malformations. We present the first reported case of branchio-oto-renal syndrome associated with bilateral congenital cholesteatoma and ossicular chain abnormalities. The pathogenesis of this syndrome is described and the literature is reviewed.

Congenital cholesteatoma: 20 years' experience at The Children's Hospital of Philadelphia

2002 ◽  
Vol 126 (4) ◽  
pp. 409-414 ◽  
Author(s):  
William P. Potsic ◽  
S. Bruce Korman ◽  
Daniel S. Samadi ◽  
Ralph F. Wetmore
Keyword(s):  
Hearing Loss ◽  
Retrospective Review ◽  
Residual Disease ◽  
Recurrent Disease ◽  
Ossicular Chain ◽  
Predictors Of Outcome ◽  
Male Predominance ◽  
Congenital Cholesteatoma ◽  
Bilateral Disease ◽  
Second Look

OBJECTIVE: We report our experience with congenital cholesteatoma over a span of 20 years with an emphasis on presenting characteristics and predictors of outcome. METHODS: We conducted a retrospective review from 1981 through 2000. RESULTS: One hundred seventy-two congenital cases were identified in 167 patients. Five patients had bilateral disease. The majority (72%) were found in boys, with an average age of 5.0 years. Hearing loss was slight to moderate. When confined to 1 quadrant, cholesteatoma was anterosuperior in 82% of cases; 47% had cholesteatoma in 2 or more quadrants. Ossicular chain involvement was found in 43% of all cases, and mastoid extension was evident in 23%. The rate of recurrent disease was directly related to the extent and number of quadrants involved. CONCLUSION: To our knowledge, this is the largest series of congenital cholesteatomas to be reported. This review confirms the male predominance and predilection for the anterosuperior quadrant. The extent of cholesteatoma and its relation to residual disease should be used as a guide for planning a second-look procedure.

Congenital Cholesteatoma in a Case of Congenital Aural Atresia

2006 ◽  
Vol 27 (7) ◽  
pp. 934-936 ◽  
Author(s):  
Robert J. Caughey ◽  
Robert A. Jahrsdoerfer ◽  
Bradley W. Kesser
Keyword(s):  
Aural Atresia ◽  
Congenital Cholesteatoma ◽  
Congenital Aural Atresia

Ossicular chain reconstruction: endoscopic or microscopic?

2021 ◽  
pp. 1-7
Author(s):  
E A Guneri ◽  
A Cakir Cetin
Keyword(s):  
Word Recognition ◽  
Middle Ear ◽  
Risk Index ◽  
Ossicular Chain ◽  
Recognition Score ◽  
Reconstruction Surgery ◽  
Ossicular Chain Reconstruction ◽  
Operation Duration ◽  
Air Conduction

Abstract Objective To compare the results of endoscopic and microscopic ossicular chain reconstruction surgery. Methods Patients undergoing ossicular chain reconstruction surgery via an endoscopic (n = 31) or microscopic (n = 34) technique were analysed for age, gender, Middle Ear Risk Index, ossicular chain defect, incision type, ossicular chain reconstruction surgery material, mean air conduction threshold, air–bone gap, air–bone gap gain, word recognition score, mean operation duration and mean post-operative follow up. Results Post-operative air conduction, air–bone gap and word recognition score improved significantly in both groups (within-subject p < 0.001 for air conduction and air–bone gap, and 0.026 for word recognition score); differences between groups were not significant (between-subject p = 0.192 for air conduction, 0.102 for air–bone gap, and 0.709 for word recognition score). Other parameters were similar between groups, except for incision type. However, endoscopic ossicular chain reconstruction surgery was associated with a significantly shorter operation duration (p < 0.001). Conclusion Endoscopic ossicular chain reconstruction surgery can achieve comparable surgical and audiological outcomes to those of microscopic ossicular chain reconstruction surgery in a shorter time.

scholarly journals Endoscopic Surgery of Congenital Cholesteatoma in the Anterior Epitympanic Recess: A Case Report of a Rare Location

2020 ◽  
pp. 014556132096924
Author(s):  
Hong Chan Kim ◽  
Hyung Chae Yang ◽  
Hyong-Ho Cho
Keyword(s):  
Case Report ◽  
Minimally Invasive ◽  
Middle Ear ◽  
Endoscopic Surgery ◽  
Minimally Invasive Approach ◽  
Ear Surgery ◽  
Invasive Approach ◽  
Congenital Cholesteatoma ◽  
Rare Location ◽  
Histopathological Type

Congenital cholesteatoma is a whitish mass in the middle ear medial to an intact tympanic membrane. It is often without symptoms and therefore incidentally diagnosed. Pediatric congenital cholesteatoma generally starts as a small pearl-like mass in the middle ear cavity that eventually expands to involve the ossicles, epitympanum, and mastoid. The location, size, histopathological type, and extent of the mass must be evaluated to select the appropriate surgical method. Although microscopic ear surgery has traditionally been performed to remove congenital cholesteatoma, a recently introduced alternative is endoscopic surgery, which allows a minimally invasive approach and has better visualization. Here, we report the first known case of a patient with congenital cholesteatoma in the anterior epitympanic recess and discuss the utility of an endoscopic approach in the removal of a congenital cholesteatoma in the hidden area within the middle ear.

Ossicular Discontinuity and Exostoses in Proteus Syndrome: A Case Report

2005 ◽  
Vol 114 (3) ◽  
pp. 242-246
Author(s):  
Joni K. Doherty ◽  
Dennis R. Maceri
Keyword(s):  
Hearing Loss ◽  
High Frequency ◽  
Conductive Hearing Loss ◽  
Ossicular Chain ◽  
Typical Feature ◽  
Proteus Syndrome ◽  
Frequency Range ◽  
Pure Tone Average ◽  
Ossicular Chain Reconstruction ◽  
Conductive Hearing

Proteus syndrome (PS) is a rare hamartomatous disorder characterized by mosaic overgrowth of multiple tissues that manifests early in life and is progressive. The presence of unilateral external auditory canal exostoses in a patient who is not a swimmer or surfer is suggestive of PS. However, hearing loss is not a typical feature. Here, we describe exostoses and ossicular discontinuity with conductive hearing loss in a patient with PS. The treatment consisted of canalplasty and ossicular chain reconstruction. A postoperative reduction was demonstrated in the patient's air-bone gap, from 21 dB to 13 dB for the pure tone average (four frequencies) and from 41 dB to 15 dB in the high-frequency range (6,000 to 8,000 Hz). Causes of ossicular discontinuity are discussed. Routine annual audiometric and otolaryngological evaluation should be considered in all patients with temporal bone inyolvement of PS.

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