A 9-year-old boy was referred to an otolaryngologist for subjective right-sided hearing loss. A hearing test revealed mild conductive hearing loss at low and middle frequencies. Otoendoscopy showed a mass with the appearance of a small white pearl (arrow) behind the right eardrum and in contact with the malleus. On the basis of the clinical findings, congenital cholesteatoma was diagnosed. Cholesteatomas are abnormal collections of squamous epithelium and keratin debris that usually involve the middle ear and mastoid. Only about 2 to 4% of cholesteatomas are congenital; they are characterized by a white mass behind an intact eardrum in a child with no history of otitis. Although histologically benign, cholesteatomas can expand and destroy surrounding bone; thus, they require surgical excision. The patient underwent excision of the lesion. Nine months after treatment, he no longer reported hearing loss and had a normal hearing test.
