1/9. Congenital middle ear cholesteatoma: report of 3 cases.In this report, we presented 3 cases of congenital middle ear cholesteatoma which occurred in a 12-year-old girl, a 4-year-old boy, and a 6-year-old boy. In all 3 cases, there was a whitish mass behind a normal tympanic membrane. Congenital middle ear cholesteatoma is not a rare disease. In the early stage, it is asymptomatic. But when it progresses, this disorder can destroy conductive systems of the middle ear and cause many symptoms. One patient (Case 1) had a complaint of hearing impairment. She underwent mastoidectomy and tympanoplasty; however, the cholesteatoma recurred. The other 2 patients had no symptoms. The abnormal appearance of their tympanic membrane was found by chance at their local otologists. We performed tympanotomies and removed cholesteatomas without aftereffects. When otologists note an abnormal appearance behind a normal tympanic membrane, with or without symptoms, tympanotomy should be done due to the possibility of congenital middle ear cholesteatoma.- - - - - - - - - - ranking = 1keywords = behind (Clic here for more details about this article) |
2/9. Congenital cholesteatoma of the middle ear in children: a clinical and histopathological report.Forty-one children with congenital cholesteatoma of the middle ear seen from 1978 through 1989 are reviewed. The most common presentation was that of an asymptomatic white mass behind a normal intact tympanic membrane. Computed tomography (CT) scan was useful in documenting extension beyond the mesotympanum. Surgical removal was performed using an extended tympanotomy for lesions in the middle ear and tympanomastoidectomy for those that had extended into attic and mastoid air cells. observation over an average 3.1-year period indicated that 80% of children were free of disease after initial surgery. Residual disease that required further surgery was present in 20%. The importance of early diagnosis of congenital cholesteatoma is strongly advocated. The prognosis is better when the cholesteatoma is confined to the anterosuperior quadrant of the middle ear. Seventeen patients in this study had such a lesion, and extended tympanotomy allowed removal of an encapsulated closed cholesteatoma with normal postoperative hearing and no residual cholesteatoma. The average age was 2.3 years. temporal bone histopathological studies of three cases of congenital cholesteatoma demonstrate two distinct pathological types of congenital cholesteatoma. A "closed" keratotic cyst in the anterior mesotympanum, which is easily removed, and an "open" infiltrative type in which there is no containment of the keratotic debris and the cholesteatoma matrix is in direct continuity with middle ear mucosa. Surgical extirpation of the "open" type is difficult and more likely to be associated with residual disease.- - - - - - - - - - ranking = 0.5keywords = behind (Clic here for more details about this article) |
3/9. Intractable retroauricular abscess associated with microtia and aural atresia--some views in relation to the congenital cholesteatoma and microtia.Five microtic and aural atretic ears associated with intractable retroauricular abscess unresponsive to conservative treatment are reported. The pathogenesis of this abscess is primary cholesteatoma called "cholesteatoma auris congenita of atretic ear," and these lesions can be clearly explained by the embryological development of the external auditory meatus. Microtic ears with such lesions invariably have part of the cartilaginous canal patent but have an atrophic bony canal. Therefore, grade 3 microtia has never been affected theoretically. We also discuss the relationship between the retroauricular abscess and the development of the tympanic bone. The evaluation of this lesion with computed tomography is mandatory for the detection of an occult congenital cholesteatoma behind the atretic canal, and regular follow-up of the patients with microtia and aural atresia is necessary for plastic surgeons.- - - - - - - - - - ranking = 0.5keywords = behind (Clic here for more details about this article) |
4/9. Two cases of cholesteatoma behind intact tympanic membrane with embryogenetic anomalies.Two new interesting cases of cholesteatoma of the middle ear behind an intact tympanic membrane are presented in this report. In both cases, which would fulfill Derlacki's criteria for a congenital cholesteatoma, the presence of an embryogenetic anomaly in the affected ear was noted. This association is suggestive of the congenital hypothesis of the observed cases.- - - - - - - - - - ranking = 2.5keywords = behind (Clic here for more details about this article) |
5/9. Treatable sensorineural hearing loss.Sensorineural hearing loss is generally felt to be an untreatable medical condition. However, in some cases, prompt diagnosis and treatment of the underlying condition may reverse the deafness. This article summarizes various treatable forms of sensorineural hearing loss and provides illustrative cases histories of patients who have had sensorineural hearing losses that were improved by medical or surgical intervention. patients with reversible sensorineural deafness due to inadvertent aminoglycoside over-dosage, congenital cholesteatoma, Meniere's syndrome, blood coagulopathy, and perilymphatic fistula all had improvements in auditory function after medical or surgical intervention. Recent experimental studies on animals may explain the basic mechanisms behind hearing loss and recovery. Aminoglycoside ototoxicity appears to have an initial reversible step, followed by a permanent process. Early endolymphatic hydrops and fistulas may cause mechanical effects in the cochlea which can be corrected. Coagulopathy may cause hypoxia which reverses after anticoagulation. These observations reveal that animal experiments can be useful in explaining human auditory dysfunction of the reversible type.- - - - - - - - - - ranking = 0.5keywords = behind (Clic here for more details about this article) |
6/9. cholesteatoma auris congenita arising from microtia.The external auditory canal opens medial to lateral in embryonal development. It is obvious that a developmental arrest anywhere during this stage would produce an absence or discontinuity of the canal, that is, congenital atretic ear. Whether or not the obliterative tissue has a bony structure, cholesteatoma can be formed behind it. Microtia is usually associated with an atretic meatus, and it may involve the possibility of epithelial cyst or cholesteatoma. The diagnosis can usually be made by the finding of tender swelling in the deformed auricle or its surroundings. On occasions, it may become necessary to distinguish these conditions from secondary infection of an epidermal cyst on the auricular surface. Several recurrent painful swellings on the deformed auricle in spite of conservative treatment indicate the need for routine otologic radiographs.- - - - - - - - - - ranking = 0.5keywords = behind (Clic here for more details about this article) |
7/9. Congenital middle ear cholesteatoma: two unusual cases and a review of the literature.Forty-three patients with congenital middle ear cholesteatoma have been described since the first case in 1953. In these patients ten cholesteatomas were confined to the anterior mesotympanum in young children who had no ossicular damage. Involvement of the posterior mesotympanum (8 ears), meso and epitympanum (21 ears), and antrum and middle ear (7 ears) was associated with an 81% incidence of ossicular erosion in addition to other abnormalities. Anterior mesotympanic cholesteatomas were readily identified by the appearance of a white mass behind a translucent drum. The other congenital cholesteatomas had a more varied appearance with a mass and a whitish appearance each noted in about half. In almost half there was a bulging of the TM. Two new cases are reported: a 4-year-old with an extensive epi and mesotympanic cholesteatoma initially misdiagnosed as non-suppurative otitis media and a 23-year-old with the third reported case of bilateral congenital cholesteatoma.- - - - - - - - - - ranking = 0.5keywords = behind (Clic here for more details about this article) |
8/9. The sleeve autograft in congenital cholesteatoma.An epithelial cyst behind an intact tympanic membrane with no previous history of ear infections is an unusual but not infrequent finding in children. If it is recognized early and completely removed, a permanent cure is obtained. However, if it is unrecognized or poorly treated, there may be far-reaching complications. The conventional technique of creating a tympanomeatal flap to remove the congenital cholesteatoma is often unsatisfactory. This is because the cyst may be located in the anterior-superior quadrant and extend beneath the malleus. It therefore becomes impossible to accomplish a complete removal and still preserve auditory function. This leads to incomplete removal with recurrence and subsequent radical removal with loss of auditory function and alteration of normal anatomy. A technique is presented whereby the canal skin, malleus, and cholesteatoma are removed en bloc. Once outside the confines of the bony ear canal, the tympanic membrane is inverted and the epithelial cyst removed under direct vision. The eardrum-malleus-canal skin autograft is then replaced in anatomical position and the hearing reconstructed with a homograft notched incus. This technique has been utilized in nine cases over the past 10 years. case reports demonstrate the anatomical and functional results.- - - - - - - - - - ranking = 0.5keywords = behind (Clic here for more details about this article) |
9/9. Congenital cholesteatoma. Preservation of cochlear function after extensive labyrinthine destruction.A patient with a large congenital cholesteatoma of the left temporal bone is presented. The case is unique in that, although the cholestetoma invaded all three semicircular as well as the basal coil of the cochlea, the patient retained normal hearing. She had an almost complete left-sided facial paralysis, yet she retained sensori-neural hearing postoperatively, in spite of removal of the superior part of the labyrinth (semicircular canals and utricle). The possible mechanisms behind such an unusual event are discussed together with the possible function of the utriculo-endolymphatic valve.- - - - - - - - - - ranking = 0.5keywords = behind (Clic here for more details about this article) |