Dataset Information

Performance of Children With Donnai-Barrow Syndrome After Cochlear Implantation: A Case Report.

ABSTRACT: Donnai-Barrow syndrome (DBS) is a rare autosomal recessive hereditary disorder that affects a variety of body systems. One of the most common symptoms in DBS patients is severe bilateral sensorineural hearing loss. The objective of this report is to highlight the performance of such patients after receiving cochlear implants as a management of their hearing loss. We reviewed the medical records of two cousins diagnosed with DBS before and after cochlear implantation, with a particular focus on their auditory and language performance. After receiving the cochlear implant, both patients showed substantial progress in auditory and speech perception, as well as their intelligence quotients, allowing them to join mainstream schools. In conclusion, our findings showed that cochlear implantation can be considered an ideal approach for the management of DBS patients who suffer from bilateral sensorineural hearing loss.

SUBMITTER: Alyousef MY

PROVIDER: S-EPMC8826483 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature

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Performance of Children With Donnai-Barrow Syndrome After Cochlear Implantation: A Case Report.

Alyousef Mohammed Yousef MY Abuhaimed Alanoud A AA Alkelabi Dania M DM AlKahtani Maram M Yousef Einas M EM Yousef Medhat F MF

Cureus 20220109 1

Donnai-Barrow syndrome (DBS) is a rare autosomal recessive hereditary disorder that affects a variety of body systems. One of the most common symptoms in DBS patients is severe bilateral sensorineural hearing loss. The objective of this report is to highlight the performance of such patients after receiving cochlear implants as a management of their hearing loss. We reviewed the medical records of two cousins diagnosed with DBS before and after cochlear implantation, with a particular focus on t ...[more]

PMID: 35165538

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Project description:Cochlear implantation is a surgical alternative to traditional amplification (hearing aids) that can facilitate spoken language development in young children with severe to profound sensorineural hearing loss (SNHL).To prospectively assess spoken language acquisition following cochlear implantation in young children.Prospective, longitudinal, and multidimensional assessment of spoken language development over a 3-year period in children who underwent cochlear implantation before 5 years of age (n = 188) from 6 US centers and hearing children of similar ages (n = 97) from 2 preschools recruited between November 2002 and December 2004. Follow-up completed between November 2005 and May 2008.Performance on measures of spoken language comprehension and expression (Reynell Developmental Language Scales).Children undergoing cochlear implantation showed greater improvement in spoken language performance (10.4; 95% confidence interval [CI], 9.6-11.2 points per year in comprehension; 8.4; 95% CI, 7.8-9.0 in expression) than would be predicted by their preimplantation baseline scores (5.4; 95% CI, 4.1-6.7, comprehension; 5.8; 95% CI, 4.6-7.0, expression), although mean scores were not restored to age-appropriate levels after 3 years. Younger age at cochlear implantation was associated with significantly steeper rate increases in comprehension (1.1; 95% CI, 0.5-1.7 points per year younger) and expression (1.0; 95% CI, 0.6-1.5 points per year younger). Similarly, each 1-year shorter history of hearing deficit was associated with steeper rate increases in comprehension (0.8; 95% CI, 0.2-1.2 points per year shorter) and expression (0.6; 95% CI, 0.2-1.0 points per year shorter). In multivariable analyses, greater residual hearing prior to cochlear implantation, higher ratings of parent-child interactions, and higher socioeconomic status were associated with greater rates of improvement in comprehension and expression.The use of cochlear implants in young children was associated with better spoken language learning than would be predicted from their preimplantation scores.

Dataset Information

Performance of Children With Donnai-Barrow Syndrome After Cochlear Implantation: A Case Report.

Publications

Performance of Children With Donnai-Barrow Syndrome After Cochlear Implantation: A Case Report.

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