Case Study 4

Petrous apex cholesteatoma & XII VII Anastomosis

A 51-year-old male first presented to the office 12 years ago with a recurrent left sided cholesteatoma for which had previously undergone 5 separate surgeries for. By the time he was first seen in our office, he had a left sided complete facial paralysis (6/6 House Brackmann scale) and a severe to profound sensorineural hearing loss. Over the subsequent 10 years, he underwent 3 different skull base surgeries (most recently in 2010) for recurrent left sided petrous apex cholesteatoma using a combination of transcochlear, translabyrinthine, and infralabyrinthine approaches to the left petrous apex. He had extensive cholesteatoma adherent to the dura, internal carotid artery, jugular bulb, facial nerve, and eroding through the otic capsule, necessitating closure of his external auditory canal. He was routinely followed with imaging and subsequent procedures were done based on his symptoms and growth of the cholesteatoma on imaging. His facial movement never returned and reconstructive options were discussed over the course of his care.

The patient continued to be bothered by his complete left sided facial paralysis. EMG testing confirmed non-functional motor end plates. After a detailed discussion of the different reconstructive options, the patient underwent a left hypoglossal facial nerve anastomosis, sural nerve graft, and tensor fascia lata sling to reconstruct the left side of his face in July 2012. This procedure involved connecting part of the 12th cranial nerve to the facial nerve stump via a cable graft nerve, which was the sural nerve in this patient’s case. We also harvested the fascia overlying the thigh muscle to pull up the sagging side of his left lower face. The goal of the surgery was to restore facial tone and ultimately symmetry to his face. He was discharged home several days after the operation. He had some left lower leg paresthesias from the sural nerve donor site, which resolved over the following 2 months. He is currently 3 months out from his reconstructive surgery and already has increased left sided facial tone. The final results from the surgery take anywhere from 9 to 12 months to allow for nerve ingrowth and reinnervation of the facial muscles.

figure 1

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The most recent CT scan of the temporal bone in July 2012 showed residual cholesteatoma along the tympanic segment of the facial nerve and a second mass at the level of the jugular foramen with erosion of the carotid canal (Fig. 1-2). The patient remains asymptomatic from the residual cholesteatoma; he is undergoing close observation and monitoring with periodic CT scans given the adherence of the cholesteatoma to critical structures.

Cholesteatoma is a collection of skin cells which continue to grow and erode middle ear structures. Patients with cholesteatomas tend to have recurrent ear drainage from infections as well as hearing loss. In rare cases such as this, cholesteatomas can grow outside the confines of the middle ear to affect critical structures of the skull base, including the dura, internal carotid artery, jugular bulb and facial nerve. These cases require expert neuro-otologic care given the need for extensive skull base surgery. Hypoglossal facial nerve anastomosis is one way to help restore tone and symmetry to the face when the facial nerve is no longer functioning from this disease.