CONGENITAL MIDDLE-EAR CHOLESTEATOMA IN CHILDREN: A RETROSPECTIVE REVIEW
Background
Congenital cholesteatoma is defined as a middle-ear cholesteatoma located behind an intact tympanic membrane. Lesions typically occur in the anterior-superior quadrant (ASQ).1 Lesions in the posterior-superior quadrant (PSQ) constitute a rare variant that is sometimes found among older individuals.2 Posterior lesions have been observed among Asian populations3. The purpose of this retrospective study was to present the clinical features of congenital cholesteatoma in children treated at our institute.
Methods
We reviewed the clinical and surgical records of 15 patients who underwent primary surgery for congenital cho-lesteatomas at Kobe University Hospital between 1990 and 2011. The patients were followed up for at least one year after their last operation. The criteria for the diagnosis of congenital cholesteatoma included the presence of a white mass behind an intact tympanic membrane with no perforation and no history of otologic surgeries. The four-stage system proposed by Potsic et al.4 was used (Table 1). Audiometric analysis was performed according to the guidelines recommended by the Japan Otological Society. The pure-tone average (PTA) was calculated from a three-tone average of 0.5, 1, and 2 kHz. Selected important characteristics for each event are summarized in Table 2.
Results
The median age at the primary surgery was 5.0 years (range 3–15 years). There were 13 male patients and two female patients. The left side was involved in four cases (27%); the right side was involved in 11 cases (73%). No case of bilateral congenital cholesteatoma was observed.
Hearing loss was the main presenting symptom and was observed in eight cases (53%). Among the 15 cases included in the study, five (33%) involved patients who complained of otalgia, one (7%) involved ear fullness, and one (7%) involved facial palsy.
The anatomic sites of cholesteatoma involvement at the time of the initial procedure are summarized in Table 3. The PSQ was affected most often, in nine cases (60%). The ASQ was affected in only six of 15 ears (40%).
Regarding the disease stage at the time of surgery, two cases were classified as surgical stage 1 (13%), two cases as stage 2 (13%), seven cases as stage 3 (47%), and four cases as stage 4 (27%). The surgical method used most frequently was canal-wall-up tympanomastoidectomy; this procedure was used in ten ears (67%) to eradicate the disease. A canal-wall-down procedure was required in five ears (33%). Ossiculoplasty with a columella on the stapes was performed in six (40%) patients, ossiculoplasty with a columella on the footplate in six (40%) patients, and the Wullstein type-I method in three (20%) patients. The operation procedures and ossiculoplasty protocol are summarized in Table 4.
Table 1. Staging system for congenital cholesteatoma (Potsic et al., 2002).
Stage 1 | Single quadrant: no ossicular involvement or mastoid extension |
Stage 2 | Multiple quadrant: no ossicular involvement or mastoid extension |
Stage 3 | Ossicular involvement: includes ossicle erosion and surgical removal to eradicate disease; no mastoid involvement |
Stage 4 | Mastoid extension (regardless of findings elsewhere) |
Table 2. Characteristics of 15 ears in 15 patients.
Table 3. Anatomical involvement.
N0.(%) | |
Anterior-superior quadrant | 6 (40) |
Posterior-superior quadrant | 9 (60) |
Anterior-inferior quadrant | 5 (33) |
Posterior-inferior quadrant | 4 (27) |
Mastoid region | 4 (27) |
Pre-operative hearing data were available for nine of the 15 ears. The initial mean (± SD) hearing loss was 31.5 ± 14.8 dB. Post-operative hearing data were available for nine ears, and the mean (± SD) hearing loss was 23.0 ± 11.4 dB. The residual rate was 0% in stage 1, 50% in stage 2, 57% in stage 3, and 50% in stage 4. The residual rate and the pre-operative and post-operative PTAs are shown in Table 5.
Table 4. Operation procedure a
Table 5. Residual rate and hearing data. Residual Rate (%)
Congenital cholesteatoma is thought to start most often in the ASQ, grow gradually into the posterior quadrant and the attic, and then finally invade the mastoid.2 On the other hand, Inokuchi et al. reported that posterior-type congenital cholesteatoma was seen more often in Asian patients.3 In this study, posterior-type cholesteatoma was dominant in Japanese patients, which is consistent with previous reports.3,5,6
References
1.Nelson M, et al. Congenital cholesteatoma: classification, management, and outcome. Arch Otolaryngol Head neck Surg 128:810–814, 2002
2.Koltai PJ, Nelson M, Castellon RJ, Garabedian EN, Triglia JM, Roman S, Roger G. The natural history of congenital cholesteatoma. Arch Otolaryngol Head Neck Surg 128(7):804–809, 2002
3.Inokuchi G, Okuno T, Hata Y, Baba M, Sugiyama D. Congenital cholesteatoma: posterior lesions and the staging system. Ann Otol Rhinol Laryngol 119(7):490–494, 2010
4.Potsic WP, Samadi DS, Marsh RR, Wetmore RF. A staging system for congenital cholesteatoma. Arch Otolaryngol Head Neck Surg 128(9):1009–1012, 2002
5.Kikuchi M, et al. Clinical evaluation of congenital cholesteatoma of the middle ear [in Japanese]. Nippon Jibiinkoka Gakkai Kaiho 106:797–807, 2003
6.Kojima H, Tanaka Y, Shiwa M, Sakurai Y, Moriyama H. Congenital cholesteatoma clinical features and surgical results. Am J Otolaryngol 27:299–305, 2006
Address for correspondence: Takeshi Fujita, MD, PhD, Department of Otolaryngology-Head and Neck Surgery, Kobe University Graduate School of Medicine, 7–5-1 Kusunoki-cho, Chuo-ku, Kobe 650–0017, Japan. kcgaku@gmail.com
Cholesteatoma and Ear Surgery – An Update, pp. 383–385
Edited by Haruo Takahashi
2013 © Kugler Publications, Amsterdam, The Netherlands