We present the unusual case of a massive skull base cholesteatoma diagnosed in middle-aged woman, 26 years after initial canal overclosure. This case highlights the importance of radiographic surveillance following ear canal overclosure and demonstrates the complex management conundrum when exteriorization would create a deep, gravity dependent, chronically draining cavity in an otherwise asymptomatic patient.
Patient history was pertinent for cleft lip and palate repair in infancy and four sets of tympanostomy tubes throughout early childhood. At age 16, the patient developed left facial nerve paralysis due to a left ear cholesteoma for which she underwent surgical management with facial nerve grafting. Shortly thereafter, the patient was diagnosed with acinic cell carcinoma of the parotid gland again on the left side. This was treated via radical surgical resection including sacrifice of the facial nerve and resulted in complete hearing loss on the left. A decade later, the patient underwent facial reanimation surgery at our institution, including temporalis muscle transposition, gold weight implantation, temporoparietal flap, and right lateral arm free tissue transfer. She was lost to follow up but eventually evaluated by a colleague in 2009. At that time, she was noted to have a narrow right ear canal and a thickened tympanic membrane with a patent T-tube in the left anterior inferior quadrant.
The patient presented to our clinic again in 2016 for evaluation of worsening hearing and was found to have developed complete stenosis of the right ear canal. Imaging revealed right middle ear disease with possible cholesteatoma, as well as a massive cholesteatoma in the left over-closed ear eroding through the entire left bony labyrinth and the internal auditory canal, encasing petrous carotid and eroding into the clivus to the occipital condyle. Longstanding facial nerve paralysis and hearing loss from her distant radical parotidectomy had rendered the patient asymptomatic despite the progressive destruction of her skull base – a complication that would only be detectable with radiologic surveillance. Treatment options considered included exteriorization with the development of a large cavity versus repeated debridement with re-closure of the ear. Given the gravity dependent and inaccessible nature of the clival portion of the cholesteatoma which would make the cavity prone to chronic drainage, the degree of difficulty that the patient experienced with even simple debridement of the ear canal, and the need for lifelong water precautions, we elected to proceed with left translabyrinthine and transpetrous approach for resection of her massive skull base, infratemporal fossa, and clival cholesteatoma. Since her first operation in 2017, she has required 3 further extensive operations, both trans-labyrinthine and trans-petrous approaches. Intraoperative images show exposed petrous carotid in the operative field, previously encased by cholesteatoma. This case highlights the importance of careful radiologic surveillance of the overclosed ear, and the massive skull base erosion that can occur without close follow up.
