Congenital cholesteatoma and cochlear implantation: Implications for management

Abstract

The 0.25% incidence of congenital cholesteatoma in our population of CI patients is higher than expected of this rare condition. It is surprisingly common given the absence of any cases of primary acquired cholesteatoma, which is considerably more common even in the pediatric population. Both patients likely had an inherited form of hearing loss and a genetic contribution to the presence of congenital cholesteatoma cannot be excluded. The presence of congenital cholesteatoma has implications for the algorithm currently employed for the assessment of CI. We consider that surgery should be staged to ensure complete removal of the cholesteatoma before implantation. Thus bilateral CI should be provided sequentially rather than simultaneously in the presence of unilateral cholesteatoma.

Role of High Resolution Computed Tomography of Mastoids in Planning Surgery for Chronic Suppurative Otitis Media

“…Our study confirmed that CT is substantially reliable in the determination of the status of the ossicular chain. These results are in concordance with other studies [8,11,14,15,19,21]. Pre-operative knowledge of the status of the ossicular chain would allow the surgeon to be ready for ossicular chain reconstruction and to better advise the patient on the degree of hearing attainable after surgery.…”

“…The exact role of CT in preoperative assessment of patients with chronic otitis media is controversial [10]. Some authors have reported a high degree of accuracy in pathological diagnosis of ossicular chain and inner ear conditions [11] and others have concluded that CT has poor ability to diagnose cholesteatoma and should not be relied on to visualize abnormalities of previously mentioned structures [12]. The results of this study suggest that cholesteatoma can be accurately diagnosed by computed tomography.…”

“…CT-scan imaging allows a comprehensive preoperative evaluation of the anatomic variations and bone details of the middle ear as well as ossicular chain and soft tissue [15][16][17]. HRCT is most valuable for detection of early erosive changes in the ossicles, particularly in smaller parts, as well as in detection of non-dependent soft tissue opacification suggestive of cholesteatoma, usually made on otologic examination [11].…”

Cochlear implant and congenital cholesteatoma

“…Except for paper of Chung et al, to date, there has been no literature published on the incidence of congenital cholesteatoma found prior to implantation [ 4 ]. The authors found this incidence (0,25 %) much higher than expected of this rare condition in general population (0.00012 %).…”

“…The incidence of congenital cholesteatoma in the overall population is 0.00012 % and 1–3 % of childhood cholesteatomas are congenital [ 2 , 3 ]. Chung et al reported that congenital cholesteatoma was identified in 2 out of 794 pediatric CI patients during their pre-operative evaluations for CI (incidence, 0.25 %) [ 4 ]. The authors suggest that the incidence was much higher than expected of this rare condition.…”