Otolaryngol Clin N Am

39 (2006) 1143–1159

The Pathophysiology of Cholesteatoma

Maroun T. Semaan, MD, Cliff A. Megerian, MD*
Department of Otolaryngology and Head and Neck Surgery, University Hospitals
of Cleveland, Case Western Reserve University, LKS 4500, 11100 Euclid Avenue,
Cleveland, OH 44106, USA

Cholesteatoma is a cystic lesion formed from keratinizing stratified squa-

mous epithelium, the matrix of which is composed of epithelium that rests
on a stroma of varying thickness, the perimatrix. The resulting hyperkerato-
sis and shedding of keratin debris usually results in a cystic mass with a
surrounding inflammatory reaction. It may present extradurally and intra-
durally. Extradurally, cholesteatoma most commonly involves the middle
ear cleft but can occur in all portions of the petrous bone including the
mastoid, petrous apex, and external auditory canal. Intradurally, cholestea-
toma, also known as epidermoid, have been described in a variety of
anatomic locations, the most common being the cerebellopontine angle.
The history of cholesteatoma has been reviewed recently [1] and is sum-
marized briefly. In 1683, Duverney [2] published the first description of what
might correspond to a cholesteatoma. He described an abscess of the bone
originating from the auditory canal that opened behind the auricle, forming
a fistula above the mastoid process, shedding the small sheets composed of
what he describes as scales. The abscess described was accompanied by
a bad odor and gave rise to what was described as grave accidents. He
also mentioned that the same process easily enters the middle ear cleft
through the auditory canal, destroying its contents and resulting in deafness.
Nearly a century and a half after Duverney’s original description, Cruveilh-
ier [3] provided in 1829 a detailed description of what he thought was an
avascular tumor originating from the cells of the subarachnoid space. Inde-
pendently, Müller [4] in 1838 used the term cholesteatoma as he became
aware of the presence of cholesterin and fat in what he believed to be a tu-
mor. Although, he noted the resemblance between the squamae of choles-
teatoma and the cells of the stratum corneum he did not postulate the
epidermal origin of these lesions. In 1855, Virchow [5] classified

* Corresponding author.
E-mail address: Cliff.Megerian@uhhs.com (C.A. Megerian).

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