Middle-ear cholesteatoma co-existing with labyrinthine fistula and vestibular schwannoma

Background Many conditions, among them vestibular schwannoma and middle ear cholesteatoma with lateral semicircular canal destruction, may be associated with asymmetrical sensorineural hearing loss (SNHL) and vertigo. However, the probability that these two distinct disease entities causing the same symptoms occur in a single patient is very low, approximately 1 per 28 billion per 1 year. Methods We present the case of a 40-year-old male admitted to our clinic because of chronic middle ear inflammation with concomitant tinnitus vertigo, and deafness in the right ear. The patient was diagnosed with lateral semicircular canal fistula caused by middle-ear cholesteatoma and concomitant vestibular schwannoma. Canal wall-down surgery was carried out to remove the cholesteatoma, followed by gamma knife radiosurgery for the vestibular schwannoma. Results Vertigo and tinnitus resolved within 3 days after the ear surgery, and gamma knife treatment resulted in the complete involution of the vestibular schwannoma. The patient presented with completely dry middle-ear cavity and no recurrence of the cholesteatoma was observed during a 3-year follow-up. Conclusion As the hereby reported condition is very rare, the results cannot be compared with any similar report published previously. Nevertheless, based on the outcome, the treatment strategy seems to be both reasonable and effective.