Primary intralabyrinthine schwannomas were first identified by Wanamaker1 in 1972. Since that time, approximately 34 cases have been reported in the literature. While our patient had no history of von Recklinghausen disease, there is an association between intralabyrinthine schwannomas and neurofibromatosis.
Intralabyrinthine schwannomas originate in the labyrinth; they are not cerebellopontine angle tumors with lateral extension. Intracochlear schwannomas are most common in the basal turn of the cochlea and scala tympani and may arise from either division of the eighth nerve, most commonly from the cochlear division.2,3 Almost all these cases have been incidentally diagnosed during labyrinthectomy for Ménière disease or during autopsy. In our case, the tumor was seen on an MRI scan and extracted intraoperatively for histopathologic diagnosis.
Imaging Quiz Case 2. Arch Otolaryngol Head Neck Surg. 1999;125(1):106-109. doi: