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PubMed2000

Intracranial extension of acquired aural cholesteatoma.

OBJECTIVE: Cholesteatoma of the petrous bone extending into the intracranial region is an unusual occurrence. Most cases have been attributed to secondary extension of a primary epidermal blastomatous malformation of the temporal bone into the middle or posterior fossae. Within the past two and a half decades, intracranial extension of acquired aural cholesteatoma has been recognized as a likely alternative to this mechanism. Recent literature has rejoined this observation by considering both primary and secondary cholesteatoma of the petrous bone as a single group, petrosal cholesteatoma. The present study is presented to analyze the clinical presentation, imaging findings, and surgical treatment of six patients with acquired aural cholesteatoma extending into the intracranial region. Findings in this study are compared with the extant literature on congenital and acquired cholesteatoma of the petrous bone. This study proposes that petrosal cholesteatoma is a valid anatomical construct; however, the pathogenesis of petrosal cholesteatoma is still important in understanding the clinical presentation and management of cholesteatoma that extends beyond the usual confines of the middle ear and mastoid. STUDY DESIGN: Retrospective case review conducted at a tertiary referral center. METHODS: From 1985 to 1999, 477 patients were surgically treated for acquired aural cholesteatoma. Patients with intracranial extension of cholesteatoma were studied. Clinical presentation, imaging studies, operative findings, surgical treatment, and postoperative results were evaluated. RESULTS: Six cases in a series of 477 patients with acquired aural cholesteatoma had intracranial extension of disease. In this series, the most frequent pathway for intracranial extension was supralabyrinthine through the supratubal recess into the middle cranial fossa. A less frequent pathway was via the retrofacial air cells into the posterior cranial fossa. Surgical access for removal of intracranial cholesteatoma was accomplished through several approaches including translabyrinthine, transcochlear, retrolabyrinthine, and middle cranial fossa. In two patients who had reoperation for possible residual disease, one was free of residual disease and one was found to have residual cholesteatoma in the region of the horizontal facial nerve. CONCLUSION: Acquired aural cholesteatoma can extend into either the middle or posterior cranial fossae. In this study, cholesteatoma extended into the middle fossa through the supratubal recess along the labyrinthine facial nerve and into or above the internal auditory canal. A less frequent path is through the retrofacial air cells into the posterior fossa. Intracranial acquired cholesteatoma is generally small and presents with complaints related to underlying otitis media rather than the neurological deficits that are often associated with primary petrous bone cholesteatoma. While computed tomography and magnetic resonance imaging are both required to differentiate congenital petrous cholesteatoma from other lesions of the petrous bone, computed tomography of the temporal bone is usually sufficient to diagnosis and define intracranial extension of acquired aural cholesteatoma. These lesions can be completely excised rather than exteriorized.

Aged↗
PubMed1998

Risk factors for recurrent and residual cholesteatoma in children determined by second stage operation.

OBJECTIVE: To clarify the risk factors for the development of recurrent and residual cholesteatoma in children. METHODS: We studied 84 ears of 83 children aged 10 years or younger who underwent a second stage operation 1 year after primary surgery with a canal wall reconstruction procedure, and analyzed the clinical risk factors for recurrent and residual cholesteatoma. RESULTS: Recurrent cholesteatoma was detected in 21 ears (25%) and residual cholesteatoma was noted in 35 (42%) of 84 ears. With respect to recurrent cholesteatoma, significant risk factors were determined to be male gender, pars flaccida type of cholesteatoma and the association of otitis media with effusion either in the side affected by cholesteatoma or on the opposite side. On the other hand, congenital type of cholesteatoma was a significant negative risk factor. With respect to residual cholesteatoma, the only risk factor was a posterosuperior type of cholesteatoma. Residual cholesteatoma was sometimes found even when the surgeon had declared complete removal of the cholesteatoma matrix at the time of primary surgery. CONCLUSIONS: High incidence of recurrent and residual cholesteatoma was noted at the second stage operation. Occurrence of recurrent cholesteatoma is closely related to eustachian tube dysfunction. Thin and highly proliferative cholesteatoma matrix in children may be responsible for high occurrence of residual cholesteatoma. Therefore, planned staged surgery is preferable to single stage surgery for the treatment of pediatric cholesteatoma.

Child↗
PubMed2002

Bilateral cholesteatoma and habitual sniffing.

OBJECTIVE: To examine the clinical findings of acquired bilateral cholesteatoma with special reference to incidence of habitual sniffing and sniff-related negative middle ear pressure. METHODS: Eighty-eight fresh cases of unilateral cholesteatoma and 33 fresh cases of bilateral cholesteatoma, which were operated on at Department of Otolaryngology, Hyogo College of Medicine, were examined in this study. Responses to a detailed questionnaire were obtained from the patients concerning about the habit of habitual sniffing to relieve aural symptoms such as aural fullness, autophonia or hyperacusis. The same questionnaire was obtained from unilateral cholesteatoma patients to compare the incidence of habitual sniffing with that of patients with bilateral cholesteatoma. We measured the negative middle ear pressure at the time of sniffing by using TTAG (tubo-tympano aerodynamic graphy, Nagashima Co. Ltd, Tokyo). We also compared the positive percentage of the sniff test in bilateral cholesteatoma with in unilateral cholesteatoma and normal controls. Sniff test was performed in 30 patients with bilateral cholesteatoma, 20 patients with unilateral cholesteatoma and 20 normal controls. RESULTS: In 33 patients with bilateral cholesteatoma (66 ears), 57 ears had the pars flaccida type (86.4%) and 9 ears had the pars tensa type (13.6%). Cholesteatoma of pars flaccida type were predominant in bilateral cholesteatoma. The rate of habitual sniffing of bilateral cholesteatoma (23/33, 69.7%) was significantly higher than that of unilateral cholesteatoma (21/88, 23.9%). The incidence of positive sniff test in bilateral cholesteatoma (19/30, 63.3%) was significantly higher than in unilateral one (6/20, 30%) and normal control (3/20, 15%). CONCLUSIONS: Habitual sniffing was closely related to the pathogenesis of bilateral cholesteatoma, especially in cases with bilateral pars flaccida type.

Adolescent↗
PubMed1994

[Surgical evaluation and clinical features of cholesteatoma in children].

There are two types of cholesteatoma in children, congenital and acquired. The pathogenesis of each type is thought to be different. The pathogenesis of congenital cholesteatoma is thought to be the embryonic inclusion of squamous epithelium, whereas acquired cholesteatoma is thought to develop from various factors including recurrent otitis media and tubal insufficiency. We reviewed a series of 23 patients with congenital cholesteatoma and 27 patients with acquired cholesteatoma, aged 2-15 years, who were operated on at our institution between January 1981 and December 1990. Planned staged surgery was performed in all patients. In the first-stage operation, the canal-wall-up technique was used in all cases. About 12 months after the first operation, the second-stage operation was performed to investigate the course of the disease. The presence of recurrent and residual cholesteatoma was evaluated at this time. In the congenital cholesteatoma series, 11 cases of residual cholesteatoma (48%) were detected at the second-stage operation. Since recurrent cholesteatoma was not observed in the series of congenital cholesteatoma cases, the prognosis of these cases was considered to be good. On the other hand, in the acquired cholesteatoma series, 9 cases (33%) of recurrent cholesteatoma and 13 (48%) of residual cholesteatoma were detected at the second-stage operation. For the second-stage operation, the canal-wall-up technique was performed on 18 patients with no recurrence, whereas the canal-wall-down technique was used on all 9 patients with recurrence. Additional mastoid obliteration was performed on 7 of these 9 patients. The prognosis of the patients who underwent obliteration was good, and there was no recurrence of cholesteatoma after the second operation. From these observations, it was concluded that planned staged tympanoplasty was useful for eliminating recurrent and residual cholesteatoma. It is also apparent that, in order to prevent recurrent cholesteatoma, it is necessary to reduce the air space to compensate for poor eustachian tube ventilation.

Child↗
PubMed1998

[Examination for prediction of residual cholesteatoma].

Residual cholesteatoma, recurrent cholesteatoma, and the open cavity problem still remain to be solved in the surgical treatment of middle ear cholesteatoma. To avoid residual cholesteatoma, complete removal of the cholesteatoma matrix is necessary, but it is sometimes difficult to completely remove it at the initial operation. It is important to estimate the amount of residual cholesteatoma and to completely remove it before its enlargement causes several complications. We established some criteria in order to estimate the residual cholesteatoma at the initial operation, and analyzed the relationship between the criteria and the actual residual cholesteatoma at the second operation. We divided the degree of expectation of residual cholesteatoma into 4 groups: 'none', 'low-rate', 'high-rate', and 'certain'. 'Certain' means that we intentionally left a small part of the matrix to avoid inner ear damage during the procedures. Of 200 cholesteatoma patients who had undergone tympanoplasty between 1985 and 1993, we reviewed 118 who had also undergone a second look or a second stage surgical procedure. Residual cholesteatoma had developed in 25 patients. In those patients, 5 had two pieces of residual cholesteatoma. The incidence of residuum was 0%, 16.5%, 30% and 83.6% in the 'none', 'low-rate', 'high-rate', and 'certain' groups, respectively. The criteria were statistically analyzed and are discussed from the viewpoint of several parameters which would be expected to influence the development of residual cholesteatoma. The analysis disclosed that only one factor influenced the development. The factor was whether the operator could or could not maintain the continuity of the cholesteatoma matrix during the procedure. According to the result, we revised the criterion. The new criterion was considered to be valuable when residual cholesteatoma was predicted, and when adaptation of an exploratory operation was decided.

Adolescent↗