A 5-year-old boy is brought to the clinic due to persistent ear discharge. The patient began to have purulent right ear drainage a month ago. Topical antibiotic drops and oral antibiotics have not improved his symptoms. He has had no fever, ear pain, dizziness, or tinnitus. The patient was born with a cleft palate that was repaired in infancy. Due to bilateral persistent middle ear effusions, tympanostomy tubes were also placed. Three years ago, the tympanostomy tubes were removed, after which audiometry was normal. He has no other medical conditions. Temperature is 37.2 C (99 F). There is scant, yellow, malodorous discharge in the right ear canal. The right tympanic membrane (TM) is immobile with insufflation and appears intact. There is retraction of the superior portion of the TM and a pearly white mass. The left TM is mobile and normal in appearance. Audiometry reveals conductive hearing loss on the right side. Gait is normal. Which of the following is most likely responsible for this patient's presentation?

Question

A 5-year-old boy is brought to the clinic due to persistent ear discharge.  The patient began to have purulent right ear drainage a month ago.  Topical antibiotic drops and oral antibiotics have not improved his symptoms.  He has had no fever, ear pain, dizziness, or tinnitus.  The patient was born with a cleft palate that was repaired in infancy.  Due to bilateral persistent middle ear effusions, tympanostomy tubes were also placed.  Three years ago, the tympanostomy tubes were removed, after which audiometry was normal.  He has no other medical conditions.  Temperature is 37.2 C (99 F).  There is scant, yellow, malodorous discharge in the right ear canal.  The right tympanic membrane (TM) is immobile with insufflation and appears intact.  There is retraction of the superior portion of the TM and a pearly white mass.  The left TM is mobile and normal in appearance.  Audiometry reveals conductive hearing loss on the right side.  Gait is normal.  Which of the following is most likely responsible for this patient's presentation?

Quizplus · Accepted Answer

11ec25c8_d652_578c_bc81_cfa66170e9d1_MD0003_00 Cholesteatoma is a benign growth of the squamous epithelium and accumulation of keratin debris within the middle ear. Most cases are acquired (not congenital) and unilateral. Risk factors for development of cholesteatomas include a history of recurrent acute otitis media (AOM), chronic middle ear effusion, and tympanostomy tube placement. Due to the increased risk of middle ear disease with cleft palate, these patients are at increased risk of developing cholesteatoma, as seen in this case. Pathogenesis involves the development of retraction pockets (invaginations) of the tympanic membrane (TM) due to chronic middle ear disease. The resultant pouch traps epithelium and debris, leading to chronic otorrhea if infected or conductive hearing loss if growth expands to the nearby ossicles. Examination reveals a pearly white mass, most commonly in the anterosuperior quadrant of the TM, or a visible retraction pocket with draining debris. Treatment is surgical excision. (Choice A) AOM is characterized by TM bulging or middle ear effusion with signs of TM inflammation (eg, erythema). Drainage associated with AOM is suggestive of TM perforation. This patient's TM is intact, nonbulging, and without inflammation. (Choice B) Foreign bodies of the external ear canal are often asymptomatic but may present with pain, drainage, and decreased hearing. If there have been prior attempts at removal, abrasions or lacerations may also be seen. This patient with a history of tympanostomy tubes has a pearly mass within the TM, a finding specific for cholesteatoma. (Choice C) Otitis externa is characterized by external ear canal maceration and inflammation and is most commonly infectious. This patient has not responded to appropriate therapy of topical antibiotics and has a distinct mass on examination suggestive of cholesteatoma. (Choice D) Patients who have a vestibular schwannoma with neurofibromatosis type 2 may have sensorineural hearing loss and tinnitus due to cochlear nerve compression. Vestibular schwannomas typically develop in adulthood, and this patient has no dermatologic (eg, cutaneous tumors) or ophthalmologic (eg, cataracts) findings suggestive of neurofibromatosis. Educational objective: Cholesteatomas result from the accumulation of keratin debris and squamous epithelial cells within a tympanic membrane retraction pocket. Symptoms include persistent otorrhea and conductive hearing loss, and examination often reveals a pearly white mass behind an intact tympanic membrane. References: Cholesteatoma definition and classification: a literature review. (http://www.ncbi.nlm.nih.gov/pubmed/28274903) Middle ear, cholesteatoma. (http://www.ncbi.nlm.nih.gov/pubmed/28846338)

A 5-Year-Old Boy Is Brought to the Clinic Due to Persistent