CONGENITAL CHOLESTEATOMA OF THE MIDDLE EAR: A REPORT OF 54 CASES

Katsumi Doi1, Mitsuo Sato1, Mie Miyashita1, Kazuya Saito1, Michio Isono1, Kyoichi Terao1, Yasuhiro Osaki2, Tetsuo Morihana2, Yumi Ohta2

1Department of Otolaryngology, Kinki University Gradutae School of Medicine, Osaka, Japan; 2Department of Otolaryngology, Osaka University Graduate School of Medicine, Osaka, Japan

Introduction

Congenital cholesteatoma (CC) of the middle ear, first reported by House (1953), is a rare clinical entity that classically presents as a white mass situated in the anterior-superior quadrant of the middle ear behind an intact tympanic membrane (TM) associated with an ipsilateral conductive and/or sensorineural hearing loss.1 Derlacki and Clemis established the diagnostic criteria for CC:2 1) A pearly white mass medial to an intact TM; 2) Normal pars tensa and pars flaccida; 3) No history of otorrhea, perforation or previous otologic procedures. Later Levenson et al. insisted the past history of acute otitis media should not be precluded from the diagnostic criteria.3 CC is seen far more frequently in children, but House and Sheehy remarked adult patients with cholesteatoma behind an intact TM fit the criteria.4

Materials and methods

A retrospective analysis was conducted of the clinical charts of all patients with CC in both children (n = 50) and adults (n = 4) at Osaka and Kinki University Hospitals from 1992 to 2012. The male/female ratio was 36:18. CCs of the petrous apex (n = 9) were excluded. The age ranged from 2 to 40 years, with an average of ten years and a median of seven years. During 1992–2012, 1212 cases of acquired and congenital cho-lesteatomas were treated by the senior author. The prevalence of CC, therefore, should be 4.5% (54/1212).

Results

Histopathological studies demonstrate two distinct pathological types of CC. A ‘closed’ cyst in the anterior mesotympanum, which is easily removed. An ‘open’ infiltrative type in which the matrix is in direct continuity with the middle-ear mucosa. Surgical removal is difficult for this type and it is more likely to be associated with residual disease. We found 22 closed-type CCs and 34 open-type CCs (both pathologies were co-existent in two cases). Potsic et al.5 proposed a staging system for CC: Stage I: single quadrant, no ossicular involvement or mastoid extension; Stage II: multiple quadrants, no ossicular involvement or mastoid extension; Stage III: ossicular involvement, no mastoid extension; Stage IV: mastoid extension. Based upon this system, 54 patients were classified in stages I-IV according to the surgical findings: eight cases in stage I, six in stage II, 22 in stage III, and 18 in stage IV (Fig. 1A). According to the growth pattern confirmed at the surgery, it was suggested that these CCs could be derived from the epidermoid formation (EF)6 in 47 cases.

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Fig. 1. Staging and location of CC.

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Fig. 2. Techniques used in the treatment of CC.

Regarding the classification of CC according to the location at the surgery, the CC lesion was divided into the anterior (A; n = 7), posterior (P; n = 7), and anterior-posterior (A-P; n = 40), using the malleus handle as the reference point (Fig. 1B). The favored position of the CC in the anterior-superior region of the middle ear in early cases be explained by its origin from the only cell rests (EF).

All patients were scheduled for tympanoplasty with or without mastoidectomy and all surgeries were performed with the post-auricular approach and lateral graft technique (Fig. 2A). A planned two-staged surgery was conducted in 48 cases (89%), while one-stage surgery was adopted in six cases (11%) including five cases of stage I (Fig. 2B). Three cases received multiple surgeries due to the residual and recurrent CCs several years after.

The residual cholesteatoma at the time of second-stage surgery was detected in 18 out of 43 cases (42%). The most common residual sites were at the oval window (n = 7), followed by attic (n = 6) and promontory (n = 2). The recurrence was found in two cases. Hearing assessment was done in 48 cases: success in 39 cases (81%), moderate in eight cases, and failure in one. The success rate was 100% both in type-1 and type-3 tympanoplasty, but went down to 70% in type-4 tympanoplasty.

Discussion

One thousand two hundred-twelve cases of acquired and congenital cholesteatomas were treated by the senior author during 1992–2012. The prevalence of CCs, therefore, should be 54/1212 (4.5%). About half of the cases were referred on the basis of abnormal otoscopic findings by outside physicians, others were referred for hearing loss. A planned two-staged surgery was conducted in most of our cases (89%), while one-stage surgery was adopted in six cases (11%). The rate of residual CC at the time of second surgery is very diverse (8–81%) in the literatures. In our cases, the residual cholesteatoma at the time of second stage surgery was detected in 18 out of 43 cases (42%).

Derlacki et al. advocate the use of a transcanal extended anterior tympano-meatal flap for limited anterior quadrant CCs and transcanal atticotomy for CCs with superior extension.2 Levenson et al. recommend a transcanal anterior-superior tympanomeatal flap for CCs located antero-superiorly in the middle ear,3 while House and Sheehy prefer the post-auricular incision and lateral graft tympanoplasty.4 They recommend two-stage tympanoplasty, in which replacement of the TM with a fascia graft, and removal of any attic or mastoid extension via mastoidectomy could be done. Only very limited, encapsulated CC is handled in one-stage. Every attempt should be made to preserve the posterior canal wall, but it must be taken down if there is a significant mastoid extension of CCs. At present, we are following the same technique.

As the stage of CC advanced, the area of its invasion could be enlarged, which should result in a higher risk of the CC residual. Considering that CC is usually discovered in its advanced stages (stages 3 and 4), the establishment of a screening program including otoscopic and CT examinations and hearing tests for early CC diagnosis should be required.

References

1.House HP. An apparent primary cholesteatoma: case report. Laryngoscope 63:712–713, 1953

2.Derlacki EL Clemis JD. Congenital cholesteatoma of the middle ear and mastoid. Ann Otol Rhinol Laryngol 74:706–727, 1965

3.Levensone MJ, Michaels L, et al. Congenital cholesteatomas in children: an embryologic correlation. Laryngoscope 98:949–955, 1988

4.House JW, Sheehy JL. Cholesteatoma with intact tympanic membrane: a report of 41 cases. Laryngoscope 90:70–76, 1968

5.Potsic WP, Samadi DS, et al. A staging system for congenital cholesteatoma. Arch Otolarngol Head Neck Surg 128:1009–1012, 2002

6.McGill TJ, Merchant S, et al. Congenital cholesteatoma of the middle ear in children: a clinical and histopathological report. Laryngoscope 101:606–613, 1991


Address for correspondence: Katsumi Doi, MD/PhD, 377–2, Oono-Higashi, Osaka-Sayama, 589–8511, Osaka, Japan. kdoi@med.kindai.ac.jp

Cholesteatoma and Ear Surgery – An Update, pp. 143–145

Edited by Haruo Takahashi

2013 © Kugler Publications, Amsterdam, The Netherlands