Transcanal Endoscopic Ear Surgery for Congenital Cholesteatoma: A Multi-institutional Series

Objective To assess outcomes of transcanal endoscopic ear surgery (TEES) for congenital cholesteatoma. Study Design Case series with chart review of children who underwent TEES for congenital cholesteatoma over a 10-year period. Setting Three tertiary referral centers. Methods Cholesteatoma extent was classified according to Potsic stage; cases with mastoid extension (Potsic IV) were excluded. Disease characteristics, surgical approach, and outcomes were compared among stages. Outcomes measures included residual or recurrent cholesteatoma and audiometric data. Results Sixty-five cases of congenital cholesteatoma were included. The mean age was 6.5 years (range, 1.2-16), and the mean follow-up was 3.9 years (range, 0.75-9.1). There were 19 cases (29%) of Potsic stage I disease, 10 (15%) stage II, and 36 (55%) stage III. Overall, 24 (37%) patients underwent a second-stage procedure, including 1 with Potsic stage II disease (10%) and 21 (58%) with Potsic stage III disease. Eight cases (12%) of residual cholesteatoma occurred. One patient (2%) developed retraction-type (“recurrent”) cholesteatoma. Recidivism occurred only among Potsic stage III cases. Postoperative air conduction hearing thresholds were normal (<25 dB HL) in 93% of Potsic stage I, 88% of stage II, and 36% of stage III cases. Conclusion TEES is feasible and effective for removal of congenital cholesteatoma not extending into the mastoid. Recidivism rates were lower with the TEES approach in this large series than in previously reported studies. Advanced-stage disease was the primary risk factor for recidivism and worse hearing result. As minimally invasive TEES is possible in the youngest cases, children benefit from early identification and intervention.

A case report of congenital cholesteatoma in adult patient mimicking as Bell palsy and proposed follow-up schedule for Bell palsy

Background Making the diagnosis of congenital cholesteatoma in adult patients still pose a challenge even among experienced otologists. Case presentation We are discussing a case of a young adult who was initially treated as Bell palsy in a different center. Two years later, she presented with marked unilateral hearing loss in addition to her ipsilateral non-resolving facial asymmetry. Conclusions Considering the delayed pattern of otological presentation in congenital cholesteatoma, as seen in our case, we proposed monthly follow up in patients diagnosed with Bell palsy for at least 6 months, and those who do not show noticeable recovery after 3 months warrant referral to neurologist or otorhinolaryngologist for further investigations. Contrast-enhanced, high-resolution computed tomography (HRCT) of the temporal bone complemented by magnetic resonance imaging (MRI) supported our diagnosis. Mastoid exploration and complete removal of the disease was imperative.

Congenital Cholesteatoma in Adult: Is it Still Possible?

Congenital cholesteatoma is a mass of squamous epithelium located medial to an intact tympanic membrane without previous history of tympanic membrane perforation, otorrhoea or otological surgery. We described a 24 year old gentleman with a left postauricular discharging fistula for 3 years with recent history of gradual hearing loss, tinnitus and recurrent episodes of positional vertigo. Clinical examination noted left postauricular fistula opening and otoscopy showed a whitish mass medial to a bulging intact tympanic membrane. High-resolution computed tomography of temporal bone was suggestive of cholesteatoma. Left modified radical mastoidectomy was done and he recovered with resolution of symptoms. Bangladesh J Otorhinolaryngol; April 2021; 27(1): 96-99

Association of Matrix Metalloproteinase-2 mRNA Expression with Subtypes of Pediatric Cholesteatoma

Objective. Cholesteatoma is a clinically heterogeneous disease, with some patients showing spontaneous regression, while others experiencing an aggressive, lethal disease. Cholesteatoma in children can be divided into two types: congenital and acquired. Identifying good prognostic markers is needed to help select patients who will require immediate surgical intervention. Matrix metalloproteinase-2 (MMP2) was previously reported to play an important role in cholesteatoma progression, by promoting bone destruction and keratinocyte infiltration. Herein, we analyzed MMP2 mRNA expression level in cholesteatoma using RNA-in situ hybridization in formalin-fixed, paraffin-embedded (FFPE) tissue samples. Methods. Sixty patients with cholesteatoma under 15 years old, who underwent their primary surgery at Aichi Medical University’s Otolaryngology Department, were analyzed for MMP2 expression level, using RNA-in situ hybridization. Results. There were no significant differences in MMP2 mRNA expression level between congenital cholesteatoma and acquired cholesteatomas. In congenital cholesteatoma, higher MMP2 signals were observed in the open type than in the closed type ( p < 0.001 ). In acquired cholesteatoma, higher MMP2 signals were observed in the pars tensa than in the pars flaccida ( p < 0.001 ). MMP2 mRNA expression level was almost exclusively found in the fibroblasts or in the inflammatory cells in the stroma, but not in the epithelium. Conclusion. Our study reveals that MMP2 mRNA expression level is strongly associated with the subtypes of cholesteatoma. The findings suggest that the level of expression of MMP2 mRNA may be related to the pathogenesis and aggressive features of cholesteatoma.