Top

European Archives of Oto-Rhino-Laryngology

Published in:

22-09-2023 | Cholesteatoma | Case Report

Monozygotic twins and cholesteatomas: nature or nuture?

Authors: Sabrina Brar, Dennis M. Wolf, Morad Faoury, Julian Barwell, Anand Saggar, Hamid Daya

Published in: European Archives of Oto-Rhino-Laryngology | Issue 12/2023

Abstract

Purpose

Cholesteatoma is a rare middle ear pathology. It can be classified into acquired and congenital forms. Although benign, cholesteatomas can cause significant morbidity including hearing loss, infection, facial palsy and thrombosis. Congenital cholesteatomas are incredibly rare and bilateral disease has not commonly been published in the literature.

Method

We describe the case of female identical (monozygotic, monochorionic, diamniotic) twins who both developed congenital cholesteatomas. In this report, we review the aetiology, treatment, embryology and pathology of cholesteatoma.

Results

The patients have been followed up 15 years after their initial surgery with promising results - pure-tone audiometry and repeat scans have not illustrated any disease recurrence.

Conclusion

This paper presents one of the only cases of female monozygotic twins presenting with unilateral and bilateral cholesteatomas.

Jennings BA, Prinsley C, Philpott C, Willis G, Bhutta MF (2018) The genetics of cholesteatoma. A systematic review using narrative synthesis. Clin Otolaryngol. 43:55–67CrossRefPubMed

Udayabhanu HN, Lauda L, Causo A, Sanna M (2018) Differential presentation of congenital cholesteatoma in twins with atypical location. Ann Otol Neurotol ISO 1:115–119CrossRef

Castle JT (2018) Cholesteatoma pearls: practical points and update. Head Neck Pathol 12(3):419–429CrossRefPubMedPubMedCentral

Graham GE, Allanson JE (1999) Congenital cholesteatoma and malformations of the facial nerve: rare manifestations of the BOR syndrome. Am J Med Genet 86(1):20–26CrossRefPubMed

Shaoul R et al (1998) Congenital cholesteatoma in a child carrying a gene mutation for adenomatous polyposis coli. J Pediatr Gastroenterol Nutr. 28(1):100–3CrossRef

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

Derlacki EL, Clemis JD (1965) Congenital cholesteatoma of the middles ear and mastoid. Ann Otol Rhinol Laryngol 74(3):706–27CrossRefPubMed

Michaels L (1986) An epidermoid formation in the developing middle ear: possible source of cholesteatoma. J Otolaryngol 15(3):169–74PubMed

Rohlfind ML, Sukys JM, Poe D, Grundfast KM (2018) Bilateral congenital cholesteatoma: A case report and review of the literature. Int J Pediatr Otorhinolaryngol 107:25–30CrossRef

10.

Kuo CL et al (2015) Updates and knowledge gaps in cholesteatoma research. Biomed Res Int. https://doi.org/10.1155/2015/854024CrossRefPubMedPubMedCentral

Title: Monozygotic twins and cholesteatomas: nature or nuture?
Authors: Sabrina Brar
Dennis M. Wolf
Morad Faoury
Julian Barwell
Anand Saggar
Hamid Daya
Publication date: 22-09-2023
Publisher: Springer Berlin Heidelberg
Keywords: Cholesteatoma
Familial Adenomatous Polyposis
Published in: European Archives of Oto-Rhino-Laryngology / Issue 12/2023
Print ISSN: 0937-4477
Electronic ISSN: 1434-4726
DOI: https://doi.org/10.1007/s00405-023-08239-8

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Monozygotic twins and cholesteatomas: nature or nuture?

Abstract

Purpose

Method

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Method

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 12/2023

T-tube implantation combined with cricoid cartilage reconstruction in the treatment of congenital laryngeal webs with subglottic stenosis

Endoscopic posterior ventricular cordectomy with contact diode laser: how I do it

Self-reported dizziness, falls, and self-rated health in a rural population in Denmark

Effect of congenital inner ear malformations (IEMs) on electrically evoked compound action potential (ECAP) responses in cochlear implant children

Porcine model for tracheostomy training: evaluation of the content and construct validity

Are smartphones and low-cost external microphones comparable for measuring time-domain acoustic parameters?