Co-occurrence of acoustic neuroma with middle ear cholesteatoma

Background.

Acoustic neuroma is a rare slow-growing benign tumor of the cerebellopontine angle. Current treatment options include watch-and wait strategy, radiation therapy, and surgical treatment. The golden standard of diagnostics is contrast-enhanced magnetic resonance imaging of the brain. Middle ear cholesteatoma most commonly develops due to invasion of the epidermis resulting from retraction of the tympanic membrane and has an incidence of 9.2 cases per 100,000 population per year. The probability of the co-occurrence of these two diseases is rather low, so the treatment choice problem does not lose its relevance. In the foreign literature, only about 4 clinical cases of the co-occurrence have been described.

Clinical case description. This manuscript presents the case of unilateral acoustic neuroma and middle ear cholesteatoma in a 55-year-old female patient. She contacted our clinic in September 2021 with complaints of hearing loss and tinnitus in the right ear. This case is remarkable because most specialists recommend starting with a surgical treatment of the middle ear lesion and then managing the neuroma when middle ear cholesteatoma and acoustic neuroma are concomitant. The patient underwent radiotherapy – stereotactic Gamma Knife radiosurgery with possible underestimation of the middle ear state – and several years later, she noted deterioration of her hearing and increased tinnitus. An otorhinolaryngologist detected retraction of the tympanic membrane during otomicroscopy and the patient was referred to our clinic. The examination confirmed the presence of a middle ear cholesteatoma on the right. Surgical treatment with tympanoplasty was performed on the right ear. One month later, otomicroscopy of the right ear showed a complete epidermalization of the neo-membrane, the postoperative cavity partially filled by autocartilage, no retractions or perforations. Our recommendations to the patient included annual otomicroscopy, MRI of the temporal bones in non-EPI DWI mode for the next 3 years, and dynamic observation with a neurosurgeon.

Conclusion. This clinical case is of special interest due to the rare co-occurrence of these diseases and the difficulty in the choice of treatment tactics. In the presence of clear clinical and diagnostic signs of cholesteatoma and neuroma, the management should be chosen based on the analysis of benefits and risks of different treatment methods.

Key words: acoustic neuroma, middle ear cholesteatoma, unilateral acoustic neuroma and middle ear cholesteatoma, treatment tactics

Acknowledgements. The authors would like to thank the Otorhinolaryngology Department of the FSBEI CPE RMACPE MOH, Russia.
Funding. There was no funding for this study.
Conflicts of interest. The authors have no conflicts of interest to declare