Vestibular Dysfunction in Pediatric Patients With Congenital Cytomegalovirus Infection and Hearing Loss: Occurrence and Characteristics.

Objective: To explore the occurrence of vestibular dysfunction in pediatric patients with congenital cytomegalovirus (CMV) infection and define the characteristics of vestibular loss in this population.

Study design: Retrospective study with controls.

Setting: Tertiary pediatric referral center.

Patients: Pediatric patients with hearing loss associated with congenital CMV infection and GJB2 mutation(s).

Interventions: Balance and vestibular evaluation.

Main outcome measures: Normal versus abnormal results of laboratory vestibular testing.

Results: A total of 50 pediatric patients with congenital CMV infection, average age = 3.4 years (ranging from 7 mo to 12 yr, SD = 2.9 yr), underwent vestibular workup, including Videonystagmography, rotary chair test, and cervical vestibular evoked myogenic potential test. Of these children with CMV infection, 35 (70%) had bilateral vestibular loss, 7 (14%) had unilateral loss, and only 8 (16%) had a normal vestibular workup. In contrast, among the 48 pediatric patients without a history of CMV infection and with hearing loss associated with GJB2 mutation(s), only 9 (19%) had bilateral vestibular loss, 2 (4%) had unilateral loss, while 37 (77%) had a normal vestibular workup. Developmental/motor delay was documented in 35 (70%) patients with congenital CMV infection and hearing loss.

Conclusions: Children with congenital CMV infection are at high risk for vestibular loss, which is frequently bilateral, impacting the semicircular canals and otolith organs, not just the well-known hearing loss. A comprehensive balance and vestibular workup is warranted for children with congenital CMV infection with the goal of early identification of vestibular dysfunction and earlier management.