Bilateral Ear Canal Cholesteatoma with Underlying Type I First Branchial Cleft Anomalies

Ann Otol Rhinol Laryngol. 2019 Apr;128(4):360-364. doi: 10.1177/0003489418821700. Epub 2019 Jan 4.


Objectives:: To describe a case of bilateral ear canal cholesteatomas in the setting of underlying first branchial cleft cyst anomalies and to review the pathophysiology underlying the development of external auditory canal cholesteatomas from branchial cleft cyst abnormalities.

Methods and results:: We present a case study of a 61-year-old man who presented with chronic right-sided hearing loss and left-sided postauricular drainage. Clinical evaluation, radiographic work-up, and pathologic analysis confirmed a diagnosis of bilateral ear canal cholesteatoma in the setting of underlying first branchial cleft cyst anomalies. The patient's clinical course, surgical treatment, and management considerations are discussed here.

Conclusion:: Ear canal cholesteatoma represents a rare clinical disease entity deserving a thorough initial assessment. Careful consideration of underlying diseases that result in chronic inflammation, such as branchial cleft lesions, should be included in the differential diagnosis of idiopathic canal cholesteatoma in the absence of prior otologic surgery or trauma.

Keywords: branchial cleft cyst; cholesteatoma; chronic infection; external auditory canal; temporal bone pathology.

Publication types

  • Case Reports

MeSH terms

  • Branchial Region / abnormalities*
  • Branchial Region / surgery
  • Cholesteatoma* / diagnosis
  • Cholesteatoma* / etiology
  • Cholesteatoma* / physiopathology
  • Cholesteatoma* / surgery
  • Craniofacial Abnormalities* / complications
  • Craniofacial Abnormalities* / diagnosis
  • Craniofacial Abnormalities* / surgery
  • Ear Canal* / diagnostic imaging
  • Ear Canal* / pathology
  • Hearing Loss, Unilateral* / diagnosis
  • Hearing Loss, Unilateral* / etiology
  • Humans
  • Male
  • Middle Aged
  • Otologic Surgical Procedures / methods*
  • Pharyngeal Diseases* / complications
  • Pharyngeal Diseases* / diagnosis
  • Pharyngeal Diseases* / surgery
  • Tomography, X-Ray Computed / methods
  • Treatment Outcome

Supplementary concepts

  • Branchial Cleft Anomalies