Kristianna Mey1, Lone Percy-Smith2,3, Maria Hallstrøm3, Matilde Sandvej2, Per Cayé-Thomasen1,4. 1. Department of Oto-Rhino-Laryngology, Head and Neck Surgery and Audiology, Rigshospitalet/Gentofte Hospital, Copenhagen University Hospital, Copenhagen, Denmark. 2. East Danish Cochlear Implant Centre and Department of Oto-Rhino-Laryngology, Head and Neck Surgery and Audiology, Copenhagen University Hospital Rigshospitalet/Gentofte Hospital, Copenhagen, Denmark. 3. Patient Organisation Decibel, Hellerup, Denmark. 4. Faculty of Health and Medical Sciences, Copenhagen University, Copenhagen, Denmark.
Abstract
Background: Children with Pendred syndrome (PS) and non-syndromic enlarged vestibular aqueduct (NSEVA) represent a group of pre-lingual hearing-impaired individuals with rehabilitation challenges.Objective: To evaluate receptive language capabilities in a pediatric cohort with PS and NSEVA.Materials and methods: Twenty-four (24) children diagnosed with either PS or NSEVA, were examined using the Peabody Picture Vocabulary Test (PPVT) and compared to a cohort of 55 Danish children with normal hearing, as well as to a mixed group of 29 children with hearing impairment of congenital and hereditary non-syndromal type. In addition, test results were compared to normative data (PPVT-4 US standard scores). Results: PS/NSEVA children's PPVT-4 test scores fall within the normative data for the PPVT-4 (US) but proved to be significantly lower statistically, when compared to Danish children with normal hearing (p<.0001) or to children with mixed non-syndromic hereditary hearing impairment (p=.006). Implantation age was significantly later for the PS/NSEVA group (median = 43 months), compared to the mixed non-syndromic hereditary congenial hearing impairment group (median = 11 months).Conclusions and significance: Children with PS/NSEVA perform below age equivalent for receptive vocabulary outcome when compared to both children with normal hearing, and children with non-syndromic mixed hereditary congenital hearing impairment who receive cochlear implants earlier.
Background: Children with Pendred syndrome (PS) and non-syndromic enlarged vestibular aqueduct (NSEVA) represent a group of pre-lingual hearing-impaired individuals with rehabilitation challenges.Objective: To evaluate receptive language capabilities in a pediatric cohort with PS and NSEVA.Materials and methods: Twenty-four (24) children diagnosed with either PS or NSEVA, were examined using the Peabody Picture Vocabulary Test (PPVT) and compared to a cohort of 55 Danish children with normal hearing, as well as to a mixed group of 29 children with hearing impairment of congenital and hereditary non-syndromal type. In addition, test results were compared to normative data (PPVT-4 US standard scores). Results:PS/NSEVAchildren's PPVT-4 test scores fall within the normative data for the PPVT-4 (US) but proved to be significantly lower statistically, when compared to Danish children with normal hearing (p<.0001) or to children with mixed non-syndromic hereditary hearing impairment (p=.006). Implantation age was significantly later for the PS/NSEVA group (median = 43 months), compared to the mixed non-syndromic hereditary congenial hearing impairment group (median = 11 months).Conclusions and significance: Children with PS/NSEVA perform below age equivalent for receptive vocabulary outcome when compared to both children with normal hearing, and children with non-syndromic mixed hereditary congenital hearing impairment who receive cochlear implants earlier.
Authors: Jiri Skrivan; Michal Jurovcik; Zdenka Aksenovova; Jaromír Astl; Radka Kremlikova Pourova; Petra Dytrych; Tomas Sieger Journal: Balkan Med J Date: 2021-07 Impact factor: 2.021