title: Rehabilitation and outcome of severe profound deafness in a group of 16 infants affected by congenital cytomegalovirus infection.
abstract: The aim of the study was to characterize the audiological consequences of congenital cytomegalovirus infection (CMV) and to evaluate the outcome of rehabilitation with hearing aids and/or cochlear implant (CI), associated with an adequate speech-language therapy. A retrospective review of data was made from a total of 16 infants, affected by severe to profound hearing loss from congenital CMV infection, referred to a tertiary audiological center for rehabilitation. Audiological evaluation was performed using behavioral audiometry, auditory brainstem responses (ABR) and/or electrocochleography (ECochG). Of the 16 children (median age at diagnosis of hearing loss: 21.33 +/- 0.7 months) with CMV hearing loss, 14 were affected by profound bilateral hearing loss and received a CI, while 2 were affected by bilateral severe hearing loss and received hearing aids. Cochlear implants can provide useful speech comprehension to patients with CMV-related deafness, even if language development is lower when compared to a group of Connexin (Cx) 26+ cochlear-implanted children (eight subjects), matched for age. Congenital CMV infection still represents a serious clinical condition, as well as an important cause of hearing loss in children. More studies have claimed to identify the pathophysiological mechanisms of damage and thus to ensure a better therapeutic approach. Nonetheless, in cases of CMV-deafened babies, the overall outcome of cochlear implantation is good.
authors.affiliation: Audiology Department, University Hospital of Ferrara, Ferrara, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Ciorba
authors.forename: Roberto
authors.initials: R
authors.lastname: Bovo
authors.forename: Patrizia
authors.initials: P
authors.lastname: Trevisi
authors.forename: Chiara
authors.initials: C
authors.lastname: Bianchini
authors.forename: Rosa
authors.initials: R
authors.lastname: Arboretti
authors.forename: Alessandro
authors.initials: A
authors.lastname: Martini
mesh_terms: D001300:Audiometry, Evoked Response; D001933:Brain Stem / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D019929:Cochlear Implantation; D003586:Cytomegalovirus Infections / Q000151:congenital*; D003638:Deafness / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology / Q000534:rehabilitation*; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D006310:Hearing Aids; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007805:Language Development Disorders / Q000175:diagnosis / Q000503:physiopathology / Q000534:rehabilitation; D008297:Male; D010348:Patient Care Team; D013069:Speech Reception Threshold Test