DOI: https://dx.doi.org/10.18203/2349-3933.ijam20222409
Published: 2022-09-23

Congenital cholesteatoma: a review

Santosh Kumar Swain

Abstract


Congenital cholesteatoma (CC) is a whitish mass lesion found in the middle ear cleft behind the intact tympanic membrane in early life. It is a relatively uncommon disease and shows its growth and extension over a period of time. CC is evidenced with no prior history of tympanic membrane perforation, otorrhea, previous otologic procedures, normal pars tensa, and pars flaccida. The etiopathogenesis of CC is still controversial; however, the epithelial cell rest theory is the most commonly accepted one. The most common sites of CC are the anterior-superior and posterior-superior quadrants of the tympanic cavity. The most common clinical presentation of CC is conductive hearing loss. Early detection and intervention are needed to avoid the complications of CC. The treatment of choice in CC is still surgical. The frequency of recurrence seems to be lower in patients with CC than in acquired cholesteatoma. Rapid progression of the CC may occur in older children and the recurrence has been associated with advanced progression of the cholesteatoma. There are very few reports for CC available in the medical literature This objective of this review article is to discuss the prevalence, etiopathology, clinical manifestations, diagnosis, and treatment.


Keywords


CC, Middle ear cleft, Conductive hearing loss, Bony erosion

Full Text:

PDF

References


Bennett M, Warren F, Jackson G, Kaylie D. Congenital cholesteatoma: theories, facts, and 53 patients. Otolaryngol Clin N Am. 2006;39:1081-94.

Persaud R, Hajioff D, Trinidade A, Khemani S, Bhattacharyya MN, Papadimitriou N et al. Evidence-based review of aetiopathogenic theories of congenital and acquired cholesteatoma. J Laryngol Otol. 2007;121(11):1013-9.

Swain SK, Agrawala R. Mastoid surgery: a high-risk aerosol generating surgical procedure in COVID-19 pandemic. Int J Otorhinolaryngol Head Neck Surg. 2020;6(10):1941-6.

Swain SK, Behera IC, Sahu MC. Role of Betadine irrigation in chronic suppurative otitis media: Our experiences in a tertiary care teaching hospital of East India. Int J Heal All Sci. 2019;8(1):29-32.

Kazahaya K, Potsic WP. Congenital cholesteatoma. Curr Opinion Otolaryngol Head Neck Surg. 2004;12(5):398-403.

Karmody CS, Byahatti SV, Blevins N, Valtonen H, Northrop C. The origin of congenital cholesteatoma. Am J Otol. 1998;19(3):292-7.

House HP. Management of congenital ear canal atresia. Laryngoscope. 1953;63(10):916-46.

Potsic WP, Korman SB, Samadi DS, Wetmore RF. Congenital cholesteatoma: 20 years’ experience at The Children’s Hospital of Philadelphia. Otolaryngol Head Neck Surg. 2002;126:409-14.

Levenson MJ, Parisier SC, Chute P, Wenig S, Juarbe C. A review of twenty congenital cholesteatomas of the middle ear in children. Otolaryngol Head Neck Surg. 1986 ;94(5):560-7.

Benhammou A, Nguyen DQ, El Makhloufi K, Charachon R, Reyt E, Schmerber S. Long term results of congenital middle ear cholesteatoma in children. Ann Otolaryngol Chir Cervicofac. 2005;122(3):113-9.

Levenson MJ, Michaels L, Parisier SC. Congenital cholesteatomas of the middle ear in children: origin and management. Otolaryngol Clin N Am. 1989;22(5):941-54.

Dornhoffer J. Cartilage tympanoplasty: indications, techniques, and outcomes in a 1,000-patient series. Laryngoscope. 2003;113:1844-56.

Aimi K. Role of the tympanic ring in the pathogenesis of congenital cholesteatoma. Laryngoscope. 1983;93:1140-6.

Persaud R, Hajioff D, Trinidade A. Evidence-based review of aetiopathogenic theories of congenital and acquired cholesteatoma. J Laryngol Otol. 2007;121:1013-9.

Tos M. A new pathogenesis of mesotympanic (congenital) cholesteatoma. Laryngoscope. 2000;110:1890-7.

Swain SK, Nahak B, Mohanty JN. Pediatric tympanoplasty: Our experiences in a tertiary care teaching hospital of Eastern India. Med J Dr. DY Patil Vidyapeeth. 2020;13(3):229-34.

Kojima H, Miyazaki H, Tanaka Y, Shiwa M, Honda Y, Moriyama H. Congenital Middle Ear Cholesteatoma Experience in 48 Cases. Nippon Jibiinkoka Gakkai Kaiho. 2003;106(9):856-65.

Huang TS, Lee FP. Congenital cholesteatoma: review of twelve cases. Am J Otol. 1994;15:276-81.

Nelson M, Roger G, Koltai PJ, Garabedian EN, Triglia JM, Roman S et al. Congenital cholesteatoma: classification, management, and outcome. Arch Otolaryngol Head Neck Surg. 2002;128(7):810-4.

Koltai PJ, Nelson M, Castellon RJ, Garabedian EN, Triglia JM, Roman S et al. The natural history of congenital cholesteatoma. Arch Otolaryngol Head Neck Surg. 2002;128(7):804-9.

El-Bitar MA, Choi SS, Emamian SA, Vezina LG. Congenital middle ear cholesteatoma: need for early recognition-role of computed tomography scan. Int J Pediatr Otorhinolaryngol. 2003;67(3):231-5.

Inokuchi G. Congenital cholesteatoma: posterior lesions and the staging system. Annal Otol Rhinol Laryngol. 2010;119:5.

Swain SK, Janardan S, Mohanty JN. Endoscopy guided eustachian tube balloon dilation: Our experiences. Iran J Otorhinolaryngol. 2020;32(112):287-94.

Swain SK, Behera IC, Sahu MC. Tinnitus among children–Our experiences in a tertiary care teaching hospital of eastern India. Pediatria Polska. 2017;92(5):513-7.

Gilberto N, Custódio S, Colaço T, Santos R, Sousa P, Escada P. Middle ear congenital cholesteatoma: systematic review, meta-analysis and insights on its pathogenesis. Eur Arch Oto-Rhino-Laryngol. 2020;277(4):987-98.

Rohlfing ML, Sukys JM, Poe D, Grundfast KM. Bilateral congenital cholesteatoma: a case report and review of the literature. Int J Pediatr Otorhinolaryngol. 2018;107:25-30.

Swain SK, Das A, Mohanty JN. Acute otitis media with facial nerve palsy: Our experiences at a tertiary care teaching hospital of eastern India. J Acute Dis. 2019;8(5):204-7.

Swain SK, Das A, Munjal S. A rare cause of bilateral facial nerve paralysis due to acute otitis media in a 52-year-old man. Med J Dr. DY Patil Vidyapeeth. 2020;13(6):688-91.

Lazard DS, Roger G, Denoyelle F, Chauvin P, Garabédian EN. Congenital cholesteatoma: risk factors for residual disease and retraction pockets-a report on 117 cases. Laryngoscope. 2007;117(4):634-7.

Denoyelle F, Simon F, Chang KW, Chan KH, Cheng AG, Cheng AT et al. International Pediatric Otolaryngology Group (IPOG) consensus recommendations: congenital cholesteatoma. Otol Neurotol. 2020;41(3):345-51.

Swain SK, Behera IC, Sahu MC. Role of Betadine irrigation in chronic suppurative otitis media: Our experiences in a tertiary care teaching hospital of East India. Int J Health Allied Sci. 2019;8(1):29-32.

Darrouzet V, Duclos JY, Portmann D, Bebear JP. Congenital middle ear cholesteatomas in children: our experience in 34 cases. Otolaryngol Head Neck Surg. 2002;126(1):34-40.

Sahu MC, Swain SK. Surveillance of antibiotic sensitivity pattern in chronic suppurative otitis media of an Indian teaching hospital. World J Otorhinolaryngol-Head Neck Surg. 2019;5(02):88-94.

Sahu MC, Swain SK, Kar SK. Genetically diversity of Pseudomonas aeruginosa isolated from chronic suppurative otitis media with respect to their antibiotic sensitivity pattern. Ind J Otolaryngol Head Neck Surg. 2019;71(2):1300-8.

Kojima H, Tanaka Y, Shiwa M, Sakurai Y, Moriyama H. Congenital cholesteatoma clinical features and surgical results. Am J Otolaryngol. 2006;27(5):299-305.

Mcgill TJ, Merchant S, Healy GB, Friedman EM. Congenital cholesteatoma of the middle ear in children: a clinical and histopathological report. Laryngoscope. 1991;101(6):606-13.

Aimi K. Rule of the tympanic ring in the pathogenesis of congenital cholesteatoma. Laryngoscope. 1983;93:1140- 6.

Mcgill TJ, Merchant S, Healy GB, Friedman EM. Congenital cholesteatoma of the middle ear in children: a clinical and histopathological report. Laryngoscope. 1991;101(6):606-13.

Anthwal N, Thompson H. The development of the mammalian outer and middle ear. J Anat. 2016;228(2):217-32.

Swain SK, Samal R, Pani SK. Effect of smoking on outcome of tympanoplasty. Indian J Otol. 2011;17(3):120-2.