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.