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

Abstract

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.

Figure 1:

Preoperative external auditory canal (EAC) endoscopy, left (A) and right (B) and intraoperative views of the external fistula tracts, left (C) and right (D). EAC cholesteatoma can be seen bilaterally just lateral to the inferior-anterior annulus, with type I first branchial cleft fistulae can be seen lateral to the cholesteatoma site separated from the cholesteatoma by a healthy bridge of tissue (A & B). Surgical exploration revealed significant bony erosion below the granulation tissue in both ears. The fistula tract in the left ear contained purulent drainage (A) that was contiguous with external postauricular drainage. Left fistula tract exiting the preauricular crease posterior to the lobule. Inset shows a more detailed view of this region. The fistula tract was cannulated intraoperatively and determined to tract into the external auditory EAC (C). Intraoperative view of the cannulated right sinus tract. An 0-0 Prolene suture was used to probe the right EAC fistula, which revealed a blind pouch that did not track through to the postauricular skin (D).

Figure 2:

Preoperative non-contrasted CT scan of the temporal bones, coronal views. Right ear with bony erosion of the lateral external auditory canal (EAC) with overlying soft tissue debris (A) and left ear with soft-tissue debris in the EAC (B).

Figure 3:

Selected slides from pathologic evaluation of submitted specimens. Upper show specimens from the initial surgery on the right ear, with keratinized squamous epithelium and the underlying granulation tissue representing the usual findings in a classic cholesteatoma (A), and squamous lined fistulous tract mucosa with chronic inflammation, fibrosis and focal granulation tissue formation (B). Submitted specimens from the second procedure are shown in the lower panels with the medially situated cholesteatoma keratin eroding the underlying bone of the external canal (C), and the laterally situated specimen with squamous lined sinus tract extending from the first branchial cleft cyst (D).