Management of Hearing Loss in a Case of Vestibular Aqueduct Syndrome
Georgia, an 11-year-old girl, was identified with left unilateral hearing loss following a failed newborn hearing screen. She has received pediatric audiology care in the same medical center for the past 11 years. This case study reviews the comprehensive audiologic, otologic, and educational follow-up needed when an infant is diagnosed with unilateral hearing loss.
Georgia was born at 37 weeks’ gestation following an uncomplicated pregnancy and delivery. She weighed 8.8 lbs at birth and had Apgar scores of 8 and 9. She passed her initial newborn hearing screen with automated auditory brainstem response (AABR) in the right ear but did not pass in the left. A similar result was obtained at the time of an outpatient re-screen 1 month later. A diagnostic auditory brainstem response (ABR) evaluation at 2 months of age was consistent with normal hearing sensitivity in the right ear and a moderate sensorineural hearing loss in the left ear. The results were discussed with her family and the implications of unilateral hearing loss and potential benefits and limitations of hearing aid use for the left ear were discussed. Georgia’s parents indicated they preferred to delay hearing aid fitting until they had an opportunity to see how her speech and language developed. A return appointment was scheduled for otologic examination, and with the family’s permission, a referral was made for early intervention services. Two weeks later, an otologic examination was completed and magnetic resonance imagining (MRI) and electrocardiogram (EKG) tests were ordered. In addition, cytomegalovirus (CMV) and Connexin 26 and 30 tests were performed using the blood sample obtained to screen for metabolic disorders in the newborn period (Guthrie’s test). The EKG, CMV, and Connexin tests all came back negative. At 3 months of age, Georgia was enrolled in the state’s birth-to-three early intervention program and a teacher of the deaf and hard of hearing provided monthly in-home visits.