Bilateral Congenital Cholesteatoma Research Articles

Bilateral congenital cholesteatoma: a case report

BackgroundCholesteatoma is regarded as a critical health issue in the world. Nevertheless, congenital cholesteatoma (CC) is an unusual problem, and bilateral CC is extremely rare.Case presentationThe present work reports a young boy with conductive hearing loss because of CC. The left side was operated using a canal-wall-down mastoidectomy method for immense destruction of the middle ear structures and mastoid air cells, and endoscopic ear surgery was performed on the second side.ConclusionAlthough CC is very rare, it should be considered in the differential diagnosis of hearing loss in children. Moreover, it should be carefully followed up because it can probably occur on the second side.

A Case of Bilateral Congenital Cholesteatoma Treated by Transcanal Endoscopic Ear Surgery

A Case of Bilateral Congenital Cholesteatoma Treated by Transcanal Endoscopic Ear Surgery

Bilateral Congenital Cholesteatoma.

To report the first large case series of extremely rare bilateral congenital cholesteatoma (CC). A retrospective cohort study. University hospital otology referral clinic. Six hundred four children with surgically confirmed CC. The bilateral CCs were compared with the unilateral CCs. The incidence of bilateral CC was 3.6% (22/615) per case or 1.8% (11/604) per child. Bilateral CC did not differ from unilateral CC regarding its demographics, and invasiveness by the proportion of advanced CC as 31.8% (7/22) versus 28.2% (167/594). But the invasiveness or location randomly differed between the ears, that advanced CC per child was higher as 45.4% (5/11). Bilateral exploration was attempted with a concern for hearing loss, which featured a combination of laser myringotomy to treat early CC and endaural laser-assisted single-stage inside-out cholesteatoma surgery to treat advanced CC. Bilateral exploration was difficult in three children with initially negative otoendoscopy. Among the seven advanced CC, proportion of anterior type was 71.4% (5/7), who all exhibited more than 20 dB HL, but two posterior type retained normal hearing. Therefore, bilateral advanced CCs of anterior origin showed poorest hearing outcome as bilateral more than 20 dB HL, which were in two children. Six second-look operations and one third-look operation were required to treat six residual CCs (30%) in four children (40%), including bilateral residual CC in two (20%); such reoperations were significantly more frequent than in unilateral CC. The diagnosis of bilateral CC required high index of suspicion from TBCT, and early bilateral exploration.

A rare case of bilateral congenital posterior mesotympanic cholesteatoma

Bilateral congenital mesotympanic cholesteatoma is a very rare disease. It can present differently from ordinary congenital cholesteatoma. We report a case of bilateral congenital cholesteatoma diagnosed at age of 22 years old. She presented with bilateral intermittent ear discharge since 10 years old that worsening two weeks prior to her presentation to our clinic and associated with bilateral reduced hearing. Clinically there was intact tympanic membrane with retraction of the mesotympanic area with present of mass medial to tympanic membrane. CT scan imaging showed there was soft tissue in the bilateral middle ear cavity with intact scutum and ossicles. Patient undergone canal wall down procedure and the diagnosis of congenital mesotympanic cholesteatoma was confirmed with present of cholesteatoma sac at the posterosuperior part, as opposed to anterosuperior quadrant, where the common site for congenital cholesteatoma.Bangladesh Journal of Medical Science Vol.17(2) 2018 p.307-310

Bilateral congenital cholesteatoma: Surgical treatment and considerations

ObjectivesTo describe a multicenter study regarding surgical management of bilateral congenital cholesteatoma (BCC) and underline the importance of endoscopes in the management of this condition. In BCC, hearing preservation is more crucial than in unilateral cases. The endoscopic approach allows complete removal of cholesteatoma via a minimally invasive technique offering low residual disease rates while preserving the normal physiology of the middle ear and possibly the ossicular chain. Study designRetrospective chart and surgical video review of patients with BCC who underwent surgery at Otolaryngology Department of Modena and Verona University Hospitals and the Hospital for Sick Children, Toronto. MethodsFrom 2002 to November 2016, six patients were identified with bilateral congenital cholesteatoma and included in this study. Pre-operative assessments, surgical treatments and outcomes were collected and described. ResultsThe median age at presentation was 4 years (range 2–7 years). A microscopic post auricular tympanoplasty was performed in two ears, four underwent a canal wall up mastoidectomy procedure and in the other six a transcanal endoscopic approach (TEA) was used. No intra- or post-operative complications were observed in any patients. The mean follow up period was 54.5 months. ConclusionsWhen both ears are involved with congenital cholesteatoma, it is particularly important to use a minimally invasive technique that preserves normal ossicular and mastoid structure and function whenever possible. In many cases this can be achieved with TEA, even in young children. In addition the endoscope allows good surgical control of cholesteatoma removal from hidden recesses.

Soft-wall reconstruction of the canal wall with retrograde bone work for pediatric cholesteatoma: Long-term results

ObjectiveTo retrospectively evaluate the long-term results of surgery for retrograde bone work, using soft-wall reconstruction of the canal wall, for pediatric cholesteatoma. MethodsWe retrospectively evaluated a series of 25 consecutive ears of 24 patients who were ≤16 years of age. All children underwent cholesteatoma surgery between October 2002 and August 2008. The type of cholesteatoma, the length of follow-up, the incidence of residual and recurrent cholesteatoma, postoperative hearing results, and the form of the reconstructed external canal wall and tympanic membrane were assessed. ResultsThere were 21 males and 3 females. The procedure was performed on both ears of one patient who had bilateral congenital cholesteatoma (CC). At the initial surgery, 16 cases (64%) had CC and nine (36%) had acquired cholesteatoma (AC). The mean age at surgery was 8.2 years and 10.4 years for CC and AC cases, respectively. The mean postoperative follow-up period after the initial surgery was 90 months for CC cases and 108 months for AC cases. Cholesteatoma recurrence occurred for 6% and 56% of cases with CC and AC, respectively. Successful serviceable hearing was achieved for 93.8% with CC and 100% with AC. Cases with inadequate hearing after surgery were characterized by disease extension to the mastoid and the protympanum. The long-term forms of the reconstructed external canal wall changed depending on their middle ear aeration. Some cases of tympanic membrane perforation and otitis media with effusion were occurred during the follow-up period. ConclusionOverall, the retrograde approach with soft-wall reconstruction of the canal wall achieved a low recurrence rate for cholesteatoma and good hearing outcomes during long-term follow-up for the pediatric case. However, in cases with eustachian tube dysfunction and/or cholesteatoma involving the protympanum, the hearing outcomes were less favorable.

Adult-Onset Labyrinthine Fistula as a Sign of Undiagnosed Bilateral Congenital Cholesteatoma

Objective. Bilateral congenital cholesteatoma is a very rare condition. We describe the first case of an adult patient presenting with bilateral congenital cholesteatoma with labyrinthine fistula. Case report. We present a 34-year-old woman of bilateral congenital cholesteatoma with labyrinthine fistula, inducing left- sided mixed conductive-sensorineural hearing loss and vertigo, subsequently accompanied by right-sided conductive hearing loss. Otoscopic examination and computed tomography scan revealed bilateral congenital cholesteatoma with a fistula in the left lateral semicircular canal. Tympanomastoidectomy was performed, and both congenital cholesteatoma were completely removed, and membranous fistula of the left lateral semicircular canal was covered with a graft. Conclusion. Congenital cholesteatoma is usually detected in childhood not but in adulthood. With delay in diagnosis, the risk of cholesteatoma complications increases. Greater attention should be paid to complications such as labyrinthine fistula resulting from long-term exposure to congenital cholesteatoma in adults.

Occult contralateral congenital cholesteatoma: is the epidermoid formation theory enough?

Bilateral congenital cholesteatomas are rare entities. Nine cases have been previously described in the literature. Many different etiologies for the development of congenital cholesteatoma have been proposed. The case of a five-year-old boy with bilateral congenital cholesteatoma is discussed. A lesion of the left ear was apparent clinically. However, the right-sided lesion was silent and was demonstrated only by radiologic exam. Theories of pathogenesis are reviewed.

Bilateral congenital cholesteatoma in branchio-oto-renal syndrome

Branchio-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.

Two cases of bilateral congenital cholesteatoma — usual and unusual presentations

Bilateral congenital cholesteatomas are rare. We present the 8th and 9th case of this entity in the literature. One of these cases is especially unusual. In that patient an ear that had been documented as normal, both radiographically and clinically, was found to have a large cholesteatoma 18 months after the contralateral ear had been treated for the same disease. The theories of the pathogenesis of this entity are also reviewed. Our cases support several of these theories. In fact, in one of the patients, it is possible that a different etiology was involved in each ear. Incidental note is made of the fact that three of the nine reported cases of bilateral congenital cholesteatoma have occurred in the same geographic locale.

Bilateral congenital cholesteatoma

We report a case of bilateral congenital cholesteatoma in a 6-year-old boy. Cholesteatoma was present in both ears around the tympanic isthmus (the only open passage from the tympanic cavity to the attic), extending to behind the horizontal portion of the facial nerve. This patient underwent a total of three canal wall up operations on each side to remove the cholesteatoma completely and improve hearing. This case fulfills the criteria proposed by Derlacki and Clemis in 1965. Bilateral congenital cholesteatoma is a rare condition, but the incidence of congenital cholesteatoma in children has increased recently for the following reasons: Reassessment of the criteria, the introduction of computed tomography, and increased awareness of congenital cholesteatoma. When operating on patients with bilateral cholesteatoma, the best method for preservation of hearing should be chosen. We discuss these problems in the present report.

CONGENITAL MIDDLE EAR CHOLESTEATOMA

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.