Clinical case of a large congenital cholesteatoma of the temporal bone pyramid

Relevance. Petrous bone cholesteatoma is a rare benign ear pathology, that leads to a deterioration in the quality of life of patients and also causes severe life-threatening intra- and extracranial complications. The frequency of its occurrence among all pathological processes of the petrous bone is 4–9%. A feature of the pathology is a long asymptomatic course. The surgical treatment of such patients is still associated with a residual process; risk of injury to the facial nerve, internal carotid artery, jugular vein bulb, etc; insufficient visibility of hidden areas and high recurrence rate despite the development of surgical approaches. Clinical case description. Patient G., 31 years old, was admitted for treatment to the Department of Ear and Skull Base Diseases of the Federal State Budgetary Institution of the National Medical Research Center of Otorhinolaryngology of the FMBA of the Russian Federation in February 2019 with complaints on profound hearing loss in the left ear (to deafness), discomfort in the left ear, recurrent spasms of m. orbicularis oculi, m. orbicularis oris. Diagnosis: Massive petrous bone cholesteatoma. Surgical treatment was performed: removal of cholesteatoma using a combined micro-endoscopic transotic approach. The decrease of the facial muscles function (V degree according to House-Brackmann grade scale) was revealed in the postoperative period. The rehabilitation of the facial nerve function was carried out: comprehensive home exercise program, self-massage, supportive drug therapy, face-taping, botulinum therapy. Twelve months after the surgery, the function of the mimic muscles recovered to grade I according to House-Brackmann. According to MRI in DWI mode, no recurrence of cholesteatoma was detected 12 months after surgery. Conclusion: Surgical treatment of patients with the petrous bone cholesteatoma is a complex and urgent problem, that requires good surgical preparation and the possibility of the simultaneous use of micro- and endoscopic techniques. Rehabilitation is necessary to maximize facial nerve recovery, improve the quality of life of the patients and the results of the surgical treatment (facial nerve preservation or reconstruction). Long-term dynamic observation and MRI in DWI mode are mandatory.

Key words: petrous bone cholesteatoma, facial nerve, internal carotid artery, internal auditory canal

Conflicts of interest. The authors have no conflicts of interest to declare. Funding. There was no funding for this study.