Objective: This study aimed to report the hearing results of endolymphatic
sac occlusion in patients with enlarged vestibular aqueduct syndrome.
Study Design: The study design was a multiinstitutional retrospective case
series.
Setting: The study was conducted at tertiary otologic referral centers.
Patients: The study included 10 previously unreported patients with progres
sive sensorineural hearing loss and vestibular aqueducts greater than 1.5 m
m in diameter on computerized tomography.
Intervention: Occlusion of the enlarged vestibular aqueduct was performed b
y means of a transmastoid surgical approach. Either intraluminal endolympha
tic sac obliteration (five patients) or extraluminal extradural endolymphat
ic sac obliteration (five patients) was accomplished with temporalis fascia
.
Main Outcome Measures: The postoperative pure tone average (PTA) and speech
discrimination scores were compared with the preoperative levels using con
ventional audiometry.
Results: Nine of 10 patients experienced some degree of sensorineural heari
ng loss. The median change in PTA was a loss of 21 decibels (dB), and 50% o
f the patients experienced a sensorineural hearing loss greater than 25 dB.
Postoperative change in PTA ranged from +10 dB to -59 dB. The median chang
e in speech discrimination score was a loss of 27.5%. Only one patient had
an improvement in both speech discrimination score and pure tone averages a
fter surgery. Patients who underwent extraluminal occlusion had a median PT
A loss of 12 dB, and patients who underwent open snc occlusion had a median
PTA loss of 34 dB. These were not statistically different.
Conclusion: In this series of 10 patients, 5 had a greater than 25 dB decre
ase in hearing after occlusion of the enlarged vestibular aqueduct. Surgica
l occlusion of the enlarged vestibular aqueduct showed no significant benef
it in hearing preservation The otologic surgeon is alerted to the potential
for severe sensiorineural hearing loss after occlusion of the enlarged ves
tibular aqueduct.