Purpose The aim of the study was to assess whether sequential cochlear implantation (CI) with a prolonged interimplant interval ( M = 15.2 years) between the first and second CIs benefited speech recognition and health-related quality of life. Method This prospective study included 14 prelingually deafened participants who received their second CI after a prolonged interimplant interval ( M = 15.2 years). Additionally, speech recognition ability over a 12-month period of bilateral implant use was investigated. The results of the speech recognition test in both quiet and noisy conditions were statistically analyzed for each CI alone and both CIs together. Nijmegen Cochlear Implant Questionnaire scores were also collected at activation and at 12 months after activation. Results Improvements in speech recognition ability were observed following the use of the first implant alone and with the use of both implants together; however, progress was much slower with the use of the second implant alone, following its introduction. Furthermore, a significant difference in the trajectory of speech recognition ability was observed between the first and the second implanted ear. According to Nijmegen Cochlear Implant Questionnaire scores, all participants benefitted from bilateral CI after 12 months. Conclusions Prolonged interimplant intervals resulted in asymmetrical speech recognition abilities. A significant improvement in the speech recognition scores was observed with the first implanted ear, and much slower progress was observed with the second implanted ear. However, the “poorer” second implanted ear could provide a considerable beneficial effect on the improved speech recognition and health-related quality of life with the bilateral CI. Supplemental Material https://doi.org/10.23641/asha.12861152
Background:The Protein tyrosine phosphatase receptor Q (PTPRQ) gene encodes a member of the type III receptor-like protein tyrosine phosphatase family found in the stereocilium. Mutations in PTPRQ are mostly associated with deafness, autosomal recessive type 84 (DFNB 84), which usually results in progressive familial hearing loss. Methods: A 25-year-old woman and her sister, both with postlingual-delayed progressive sensorineural hearing loss, were examined. They were from a nonconsanguineous marriage and had no family history of hearing loss. New compound heterozygous PTPRQ gene mutations, nonsense (c.90C > A, p.Y30X) and splice (c.5426 + 1G > A) mutations in two PTPRQ alleles, were identified in the two sisters and were presumably autosomal recessive. The c.90C > A (p.Y30X) mutation was mapped to exon 2 of PTPRQ (NM_001145026). Results: The c.90C > A mutation leads to a premature stop codon and a truncated protein. The c.5426 + 1G > A mutation leads to a truncated protein lacking the extracellular domain. Hence, both mutations were predicted to be pathogenic, leading to a deficiency of the extracellular, transmembrane, and phosphatase domains because of nonsense-mediated mRNA degradation.Conclusions: This study increases the spectrum of PTPRQ gene mutations that might be involved in delayed progressive autosomal recessive non-syndromic hearing loss.
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