Document Detail

Development of canal cholesteatoma in a patient with prenatal isotretinoin exposure.
MedLine Citation:
PMID:  20638138     Owner:  NLM     Status:  MEDLINE    
PURPOSE: To describe the clinical and radiologic findings in a case of isotretinoin embryopathy-like syndrome and discuss management of hearing loss, congenital external auditory canal (EAC) stenosis, and EAC cholesteatoma.
METHODS: Review of medical, audiological, and radiological records.
RESULTS: An 8 year old female presented with bilateral moderate conductive hearing loss, bilateral microtia, left EAC stenosis, and right EAC atresia, secondary to prenatal isotretinoin exposure. Comorbidities included developmental delay, ventricular septal defect, hypotonia, and retinal maldevelopment. The left EAC was sharply upsloping with a 2mm-diameter meatus. Computed tomography (CT) scan of the temporal bone demonstrated normal middle and inner ears bilaterally; serial CT scans over 6 years demonstrated progressive development of left canal cholesteatoma. Implantation of a right BAHA system was performed, followed by left canalplasty and excision of cholesteatoma with facial nerve monitoring. An endaural incision was utilized to avoid compromising future microtia repair. Postoperative left-sided hearing improved to mild low-frequency conductive hearing loss rising to normal at 2000 Hz and above.
CONCLUSIONS: Despite extensive precautions for its use, isotretinoin remains a cause of major birth defects, including sensorineural, conductive or mixed hearing loss. Congenital EAC stenosis is much less common than congenital atresia or acquired stenosis; optimal surgical approaches vary depending on hearing status and facial nerve anatomy. Close monitoring for development of canal cholesteatoma is necessary.
Kathryn M Van Abel; Marc E Nelson; Ryan M Collar; Marci M Lesperance
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2010-07-17
Journal Detail:
Title:  International journal of pediatric otorhinolaryngology     Volume:  74     ISSN:  1872-8464     ISO Abbreviation:  Int. J. Pediatr. Otorhinolaryngol.     Publication Date:  2010 Sep 
Date Detail:
Created Date:  2010-08-16     Completed Date:  2010-12-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8003603     Medline TA:  Int J Pediatr Otorhinolaryngol     Country:  Ireland    
Other Details:
Languages:  eng     Pagination:  1082-4     Citation Subset:  IM    
Copyright Information:
Copyright 2010 Elsevier Ireland Ltd. All rights reserved.
Thomas Jefferson School of Medicine, USA.
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MeSH Terms
Abnormalities, Drug-Induced / etiology
Cholesteatoma / chemically induced*,  radiography,  surgery
Ear / abnormalities*
Ear Canal* / radiography
Ear Diseases / chemically induced*,  radiography,  surgery
Hearing Loss, Conductive
Isotretinoin / adverse effects*
Prenatal Exposure Delayed Effects*
Reg. No./Substance:
0/Teratogens; 4759-48-2/Isotretinoin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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