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Congenital Cholesteatoma Theories

Congenital Cholesteatoma Theories

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Congenital Cholesteatoma: Theories,
Facts, and 53 Patients
Marc Bennett, MD*, Frank Warren, MD,
Gary C. Jackson, MD, David Kaylie, MD

The Otology Group, Otolaryngology Head & Neck Surgery,
Vanderbilt University, 300 20th Avenue North,
Suite 502, Nashville, TN 37203, USA

Although the \ufb01rst written report of congenital cholesteatoma (CC) was over 100 years ago in 1885, its true incidence, etiology, and pathogenesis still remains a controversial. CC of the temporal bone can be found intradural, most commonly at the cerebropontine angle, or extradural in the middle ear or mastoid. Congenital cholesteatoma of the middle ear was \ufb01rst described by Howard House[1] in 1953. Later, Derlacki and Clemis[2] described six cases of CC and established the clinical criteria for the diagnosis. These in- clude a pearly white mass medial to an intact tympanic membrane, a normal pars tensa and \ufb02accida, and no history of otorrhea, perforation, or previous otologic procedure. Levenson revised the criteria by adding that previous bouts of otitis media or e\ufb00usion should not be exclusion criteria.

Once thought to be relatively rare, CC of the middle ear is now thought to be on the rise, and accounts for 2% to 5% of all cholesteatomas[3]. There are multiple theories to the pathophysiology of congenital cholesteatomas of the middle ear. Proposed mechanisms are inclusion, migration, or invasion of squamous epithelium, epithelial rests from faulty embryogenesis, or meta- plasia of normal epithelium. Patients with CC may present with a variety of complaints including conductive hearing loss, but the most common presen- tation is an asymptomatic white mass medial to an intact tympanic membrane. Early detection of CC is critical, limiting the size of the retro- tympanic mass and reducing the risks and complications from surgery. Treatment remains surgical removal. This study will discuss the diagnosis, classi\ufb01cation, treatment, and theories of pathogenesis, as well as report the 53 cases of CC treated at our institution.

* Corresponding author.
E-mail address:marc.bennett@vanderbilt.edu(M. Bennett).
0030-6665/06/$ - see front matter\u00d3 2006 Elsevier Inc. All rights reserved.
Otolaryngol Clin N Am
39 (2006) 1081\u20131094

The true incidence of CC is di\ufb03cult to determine. Initially thought to be rare, the incidence seems to be on the rise[4]. The incidence of CC of the mid- dle ear is estimated to be between 1% to 5% of all cholesteatomas in most published series[4\u20136]. Earlier treatment of otitis media and allergies is reduc- ing the number of acquired cholesteatomas, and consequently increasing the percentage of CC. There are also many reasons that the number of reported cases of CC has increased over the last 30 years. Heightened awareness of the condition by pediatricians and otolaryngologists has lead to earlier diagnosis and intervention, avoiding the tympanic membrane perforation, which would eventually occur as the natural progression of the disease[7]. This would preclude the diagnosis of CC. Routine audiologic screening has also identi\ufb01ed children with conductive hearing losses at early ages. Workup of these children with better otoscopic equipment may identify masses medial to the tympanic membrane that previously would have been unseen. In ad- dition, incidental masses are occasionally seen on CT scans of the temporal bone ordered in children with a conductive hearing loss.

Presentation and growth

The clinical presentation of any ear mass depends on its size, location, and histology. Earlier diagnosis decreases the overall size of the CC and re- duces the likelihood of ossicular erosion. The most common presentation of a CC is a white retrotympanic middle ear mass[5], although they may be discovered incidentally during the time of routine otologic evaluation or during a myringotomy. Improved American health care and preventive medicine for children has lead to a substantially earlier diagnosis of CC at an average age of 4 to 5 years old[5,7].

The most common location of CCs is the anterior superior quadrant of
the tympanic membrane, followed by the posterior\u2013superior quadrant
[5,7]. Lesions discovered at later ages are more likely to be located in the

posterior mesotympanum, as continued growth of the cholesteatoma is gen- erally in a posterior direction of spread. The growth follows a natural course as described by Koltai[8]. Although growth can proceed inferiorly toward the hypotympanum, it more commonly extends posteriorly along the medial surface of the ossicles. Once in the posterior superior mesotympanum, the expansion can involve the incudostapedial joint or stapes superstructure, but usually spares the footplate. Continued expansion proceeds toward the facial recess, sinus tympani, and eventually the mastoid air cells. It is rare for the cholesteatoma to invade the otic capsule bone or labyrinth.

As growth continues, symptoms become more common. Both large ante- rior and posterior lesions present with conductive hearing loss for di\ufb00erent lesions. Posterior located lesions a\ufb00ect the ossicles much more frequently, causing a conductive hearing loss from ossicular movement impairment or


discontinuity. Anterior lesions may compromise the function of the Eusta- chian tube and cause a conductive hearing loss through middle ear e\ufb00usion. Otalgia and otorrhea are rare, but nearly 50% of patients describe episodes of previous otitis media[9]. If cholesteatoma extends to invade the laby- rinth, patients may su\ufb00er from vertigo or sensorineural hearing loss. Despite early reports describing a high percentage of facial nerve palsy at presenta- tion, facial nerve dysfunction is relatively uncommon from middle ear CC

[10]. In fact, facial paresis mandates exclusion of malignancies, metastases,
facial nerve neuromas, and other diagnoses.

CC or epidermoid cyst is a strati\ufb01ed squamous epithelial lined cyst \ufb01lled with keratin debris. Like acquired cholesteatoma, the cyst forms as the result of progressive desquamation of the epithelium. The congenital form of choles- teatoma is indistinguishable by histology from the acquired form; therefore, it is the clinical picture that is important in distinguishing the two entities.


Pediatric patients with conductive hearing loss and a normal otomicro- scopic examination require radiologic evaluation to evaluate for the presence of middle ear anomalies like CC. Because plain radiographs are nonspeci\ufb01c, high-resolution CT and MRI are the most commonly used imaging modalities

[11]. CT is generally used as the \ufb01rst imaging modality because of its superior

bony de\ufb01nition. CT cannot only con\ufb01rmthe location of a middle ear mass, but can accurately determine the size of the lesion. CC is usually seen as a hypo- dense expansile lesion, round to oval in shape, with well-de\ufb01ned margins that do not enhance with contrast. The lack of enhancement helps distinguish the cholesteatoma from other lesions like neuromas, glomus tumors, sarco- mas, or meningiomas. Unlike patients with chronic ear disease, the mastoid air cells are usually well aerated and nonsclerotic[12].

MRI further enhances the evaluation by distinguishing the cholesteatoma from other middle ear masses such as neuromas, adenomas, schwannomas, or metastasis. On T1 weighted images, CC appears as a homogenous lesion that is hypointense to brain, but can also appear isointense[12]. On T2 weighted images, signal intensity is usually high like cerebral spinal \ufb02uid (CSF). There is usually no enhancement with gadolinium. Di\ufb00usion weighted imaging can be used to help distinguish cholesteatoma from other cystic masses as well[11].

CC of the middle ear is often staged by its location and relationship with
surrounding structures. Derlacki and Clemis[13] are credited with the \ufb01rst

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