1 | INTRODUCTION
Enlarged vestibular aqueduct was a common kind of cochlear malformation
in the temporal bone, which could induce hearing loss(1). Large
vestibular aqueduct syndrome(LVAS) could be diagnosed with the classical
hearing loss and a diameter >1.5 mm at the midpoint of
vestibular aqueduct according to the valvassori criterion(1,2). So far,
LVAS has been increasingly accepted as the most common cause of
congenital progressive sensorineural hearing loss in early childhood(1).
Apart from hearing loss, vestibular dysfunction has been reported
associated with LVAS(3,4). About 87% of LVAS patients marked vestibular
weakness exhibited by vestibular function tests in Berrettini’s study
and 89% of children with LVAS had at least one abnormal vestibular test
results in Christina’s reports (5,6). Disequilibrium or vertigo as the
typically vestibular symptoms were frequently appeared in LVAS patients
(1,7,8). In a retrospective study, vestibular symptoms (imbalance,
vertigo, and/or motor delay) were approximately 46% and 48% in adult
and pediatric patients with LVAS, respectively(9). Delayed ambulation
and poor coordination were also reported in children with LVAS(8,10,11).
In the early stages of life, auditory input and communication were
essential for cognition, behavior and social development(12-14) .
Developmental delay has been reported in children with hearing loss(14).
For the patients with LVAS, as a special group of hearing loss with the
characteristics of both hearing impairments and vestibular problems, we
were used to paying them special attention in the clinical practice. In
addition, the great body balance ability and skilled movement were
important prerequisite for the child to interact with the external world
to promote outstanding and all-round development (15-18). Yet, would
vestibular disorders of LVAS children affect their motor development?
were their comprehensive development levels worse than other deaf
children? And would the factor of LVAS extra affect the developments of
children? The above questions should be addressed in order to answer
clinicians’ and parents’ doubts and give clinical guidance.
However, until to now, little study has been devoted to evaluated the
development of children with LVAS particularly. In the present study,
firstly, we investigated the comprehensive developments in children with
LVAS and without LVAS separately, then compared their performance, and
lastly analyzed the risk factors for developments of children with LVAS.