Patients with neurofibromatosis type 2 (NF2) develop bilateral acousti
c neuromas, but preservation of hearing while achieving tumor removal
may present several unique problems. Our philosophy regarding hearing
preservation attempts in these cases has evolved over several decades.
The outcome of hearing preservation surgery using the middle cranial
fossa approach for 13 procedures in 10 patients with NF2 is presented.
There were 12 total tumor removals and one partial tumor removal. Two
ears retained good postoperative hearing, three had serviceable heari
ng, four had measurable hearing, and four had no measurable hearing. B
oth cases with tumors 2 cm or larger retained no hearing. For the 12 t
otal tumor removals, the rate of hearing preservation was 67 percent.
Of four total removals with long-term follow-up, two retained good or
serviceable hearing. The rate of hearing preservation is similar to th
at found in our series of unilateral acoustic neuroma surgeries. Based
on these findings, hearing preservation surgery is recommended for pa
tients with NF2 who have acoustic neuromas 1.5 cm or smaller.