Early Cytomegalovirus Testing Can Detect Sensorineural Hearing Loss in Very Young Children

What is the feasibility and cost effectiveness of incorporating cytomegalovirus (CMV) testing to determine the etiology of pediatric hearing loss?

Background: CMV is the most common infectious cause of congenital sensorineural hearing loss (SNHL). It is estimated that 40,000 congenitally infected neonates are born annually, and CMV is estimated to be the cause of 20% or more of SNHL in young children; however, there have been few studies evaluating the yield of CMV testing for the hearing-impaired child.

Study design: Retrospective study of 111 children aged 3 years or younger presenting with SNHL from May 2008 to September 2013.

Setting: Division of Otolaryngology–Head and Neck Surgery, University of Utah, Salt Lake City.

Synopsis: Of the 111 patients, 45 had profound SNHL, 82 had bilateral SNHL, and eight had auditory neuropathy spectrum disorder. A genetic etiology was the most common cause, usually from an undefined familial predisposition to hearing loss or Down syndrome; other conditions included branchio-otorenal, Pendred, Alport, Waardenburg, and CHARGE syndromes. Acquired SNHL causes included a complication from MMR vaccination, a temporal bone fracture, meningitis, and chemotherapy and prematurity complications. Eighty-three children underwent CMV testing, imaging, and a geneticist evaluation. Those with confirmed or probable CMV-induced SNHL made up 30% of all children tested. Sixteen children were diagnosed with confirmed CMV, and nine children were diagnosed with probable CMV. Those without a known etiology were the largest group at 39%. An enlarged vestibular aqueduct was the most common abnormality identified, followed by cochlear nerve deficiency and cochleovestibular dysplasia. Cost calculation was based on the sum for institutional cost for GJB2 screening ($611), imaging ($1,591), and CMV polymerase chain reaction testing ($66); dried blood spot testing would increase the cost by another $66. The major limitation was the inability to obtain audiologic, clinical notes, imaging, and laboratory results for all patients studied.

Bottom line: The relatively high incidence of CMV-induced SNHL, the low cost for this assay, and the indirect benefits from early diagnosis support the role of early CMV testing for SNHL patients.

Citation: Park AH, Duval M, McVicar S, Bale JF, Hohler N, Carey JC. A diagnostic paradigm including cytomegalovirus testing for idiopathic pediatric sensorineural hearing loss. Laryngoscope. 2014;124:2518-2525.

—Reviewed by Amy Eckner