Unlike the acoustic neuroma that occupies the internal acoustic meatus and extends into the cerebellopontine angle, the true intralabyrinthine neuroma is an extremely uncommon finding. The majority of the published cases were found at autopsy or during destructive labyrinthectomy for uncontrollable Meniere's syndrome. To the best of our knowledge, we present the smallest exclusively intrautricular neuroma that has ever been diagnosed preoperatively. Detailed magnetic resonance imaging studies allowed the detection of this tumor, measuring only 3.5 mm in diameter. The tumor was removed via atticomastoidectomy and partial labyrinthectomy. The diagnostic workup, radiographic characteristics, and surgical treatment are discussed, together with a review of the literature.
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