Cases reported "Ear Diseases"

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1/8. 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.
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2/8. 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.
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3/8. Aberrant jugular bulb presenting as a middle ear mass.

    case reports of 3 patients seen with a bluish mass behind the tympanic membrane are presented. The initial diagnosis was probable glomus tumor. In 1 patient, middle ear exploration confirmed the presence of a high-lying jugular bulb. In the other 2, venography demonstrated that the jugular bulb projected superiorly into the middle ear cavity. In all 3, polytomography demonstrated a dehiscence of the bony septum which normally separates the jugular bulb from the hypotympanum. When a bony dehiscence is seen in the absence of destructive changes, a diagnosis of aberrant jugular bulb is strongly suggested.
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4/8. 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.
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5/8. 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.
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6/8. 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.
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7/8. 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.
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8/8. Protruding jugular bulb presenting as a middle ear mass. A case report.

    Protrusion of the jugular bulb is an uncommon but important cause of a bluish-purple mass behind the tympanic membrane. A knowledge of this condition is necessary to prevent massive haemorrhage following tympanotomy or during temporal bone surgery. The diagnosis can be established by jugular venography, which demonstrates the protrusion of the characteristic knuckle of the jugular bulb into the middle ear cavity.
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