Objective: The most common initial symptoms of the acoustic neuroma ar
e unilateral hearing loss that evolves gradually, tinnitus, and unstea
diness. However, atypical presentations may sometimes occur, more ofte
n with a small intracanalicular neuroma or with a large medial neuroma
placed in the cerebellopontine angle. Results: In our group of 51 pat
ients suffering from acoustic neuroma, atypical presentations were obs
erved in 9 cases (17.6%). Two patients had normal hearing function but
reported tinnitus; two patients reported sudden hearing loss, with pa
rtial recovery; two patients had a history of fluctuating hearing loss
; two patients reported neurologic symptoms (one reported trigeminal p
aresthesia and the other had a history of trigeminal paresthesia and r
ecurrent headache); and one patient reported profound hearing loss for
many years and the recent onset of unsteadiness. Conclusion: Patients
with these atypical presentations have to undergo a diagnostic evalua
tion for acoustic neuroma and must be evaluated with BAEPs and then wi
th gadolinium-enhanced MRI.