Unilateral acoustic neuroma in childhood

Three cases of unilateral acoustic neuroma in childhood that are associated with neither neurofibromatosis type 1 nor type 2 were reported. All three cases had a hearing disorder as an initial symptom. Two of them had a large neuroma and had considerable abnormal findings in neurootological examinations, and one case with an intracanalicular tumor showed a unilateral progressive sensorineural hearing loss that had no response to steroid administration. Surgical removal of the tumor was carried out for these cases. Different approaches were used in each case; suboccipital approach, one-stage suboccipital and middle fossa approach, and middle fossa approach. Although the facial nerve functions were fairly well maintained, hearing preservation could not be attained in all. Papers dealing with this tumor were reviewed, and certain characteristics of cases with acoustic neuroma in childhood were discussed.