R. NICKBRYANMDPATRICIA A.HUDGINSMD
Congenital cholesteatoma was first described by House1 in 1953. The diagnostic criteria should include a normal tympanic membrane in a patient with no history of ear surgery, trauma, or perforation.2 Congenital cholesteatoma may be divided into the following 5 groups based on anatomical origin: external ear canal, middle ear, mastoid cavity, petrous apex, and cerebellopontine angle.3 The usual clinical presentations of congenital cholesteatoma vary according to the site. For example, in congenital cholesteatoma of the external ear, the common finding is squamous debris within the external ear canal.4 If congenital cholesteatoma arises within the middle ear, it may manifest with hearing impairment in childhood because of its effect on the ossicular chain. If it originates within the mastoid cavity, it may present with pain over the mastoid area or ear discharge through a fistula from the mastoid to the external ear canal.5 In the petrous apex, congenital cholesteatoma may initially present with hearing loss or facial nerve paralysis.6 When it arises in the cerebellopontine angle, the most common primary symptom at presentation relates to vestibulocochlear dysfunction, such as hearing loss, imbalance, or vertigo.7 Congenital cholesteatoma arising in the middle ear is rare, with a reported incidence ranging from 2% to 24% of all cholesteatomas of the middle ear.8 The present case of congenital cholesteatoma presented like a vascular tumor of the middle ear and manifested the symptoms of acute otitis media, with hemorrhaging from the middle ear.
Radiology Quiz Case 3—Diagnosis. Arch Otolaryngol Head Neck Surg. 2004;130(9):1125-1126. doi:10.1001/archotol.130.9.1125