Sage Journals: Discover world-class research

Abstract

Objective

To evaluate the speech perception and language development with cochlear implants (CI) of DFNB1 children in comparison with non-DFNB1 deaf children.

Study Design

Retrospective case series.

Setting

Academic tertiary center.

Results

Thirty-one congenitally deafened children, screened for GJB2 allele variants, were followed for an average 32 months after CI. With the use of age-appropriate testing, 75% of DFNB1 and 53% of non-DFNB1 children achieved open set word recognition (speech perception category [SPC] level 6). Multivariate analysis showed that SPC was primarily dependent on duration of CI use, but not on the cause of hearing loss. In Reynell language tests, DFNB1 children showed more consistent and quicker gains than non-DFNB1 children.

Conclusion

Although children with CI with DFNB1 show faster gains in Reynell scores, duration of CI use appears to have a greater effect on speech perception than DFNB1 status. SIGNIFICANCE: Identification of DFNB1 children is useful in counseling of CI outcomes.

Get full access to this article

View all access options for this article.

References

Estivill

Fortina

Surrey

, et al. Connexin-26 mutations in sporadic and inherited sensorineural deafness Lancet. 1998 351 394–8. (Grade B).

Guilford

Ben

Arab S

Blanchard

A nonsyndromic form of neurosensory recessive deafness maps to pericentromeric region of chromosome 13q

Nat Genet 1994 6(1) 24–8. (Grade C).

Forge

Becker

Casalotti

, et al. Gap junctions and connexin expression in the inner ear Novartis Found Symp 1999 219 134–56. (Grade D).

Denoyelle

Marlin

Weil

Clinical features of the prevalent form of childhood deafness, DFNB1, due to a connexin-26 gene defect: implication for genetic counseling

Lancet 1999 353 1298–303. (Grade B).

Kikuchi

Kimura

Paul

, et al. Gap junctions in the rat cochlea: immunohistochemical and ultrastructural analysis Anat Embryol (Berlin) 1995 191(2)101–18. (Grade D).

Jun

McGuirt

Hinojosa

, et al. Temporal bone histopathology in connexin 26-related hearing loss Laryngoscope 2000 110 269–75. (Grade C).

Bauer

Geers

Brenner

The effect of GJB2 allele variants on performance after cochlear implantation

Laryngoscope 2003 113 2135–40. (Grade B).

Pyman

Blamey

Lacy

, et al. The development of speech perception in children using cochlear implants: effects of etiologic factors and delayed milestones Am J Otol 2000 21 57–61. (Grade C).

Francis

Pulsifer

Chinnici

, et al. Effects of central nervous system residua on cochlear implant results in children deafened by meningitis Arch Otolaryngol Head Neck Surg 2004 130 604–11. (Grade B).

10.

Matsushiro

Doi

Fuse

, et al. Successful cochlear implantation in prelingual profound deafness resulting from common 233delC mutation of the GJB2 gene in the Japanese Laryngoscope 2002 112 255–61. (Grade C).

11.

Sinnathuray

Toner

Clarke-Lyttle

, et al. Connexin 26(GJB2) gene-related deafness and speech intelligibility after cochlear implantation Otol Neurotol 2004 25 935–42. (Grade B).

12.

Sinnathuray

Toner

Geddis

, et al. Auditory perception and speech discrimination after cochlear implantation in patients with connexin 26(GJB2) gene-related deafness Otol Neurotol 2004 25 930–4. (Grade B).

13.

Taitelbaum-Swead

Brownstein

Muchnik

, et al. Connexin-associated deafness and speech perception outcome of cochlear implantation Arch Otolaryngol Head Neck Surg 2006 132 495–500. (Grade B).

14.

Dahl

Wake

Sarant

, et al. Language and speech perception outcomes in hearing-impaired children with and without connexin 26 mutations Audiol Neurootol 2003 8 263–8. (Grade B).

15.

Geers

Moog

Predicting spoken language acquisition on profoundly hearing-impaired children

J Speech Hear Disord 1987 52 84–94. (Grade D).

16.

Cheng

Niparko

Analyzing the effects of early implantation and results with different causes of deafness. In: Niparko

, ed. Cochlear implants: principles and practices. Baltimore: Lippincott Williams & Wilkins; 2000. p. 259–64. (Grade C).

17.

Sobe

Vreugde

Shahin

, et al. The prevalence and expression of inherited connexin 26 mutations associated with non-syndromic hearing loss in the Israeli population Hum Genet 2000 106 50–7. (Grade C).

18.

Houseman

Ellis

Pagnamenta

, et al. Genetic analysis of the connexin-26 M34T variant: identification of genotype M34T/M34T segregating with mild-moderate non-syndromic sensorineural hearing loss J Med Genet 2001 38 20–5. (Grade B).

19.

Kenna

Lip

, et al. Use of a multiplex PCR/sequencing strategy to detect both connexin 30 (GJB6) 342 kb deletion and connexin 26 (GJB2) mutations in cases of childhood deafness Am J Med Genet 2003 121(2)102–8. (Grade C).

20.

Geers

Brenner

Davidson

Factors associated with development of speech perception skills in children implanted by age five

Ear Hear 2003 24 24–35. (Grade C).

Performance after cochlear implantation in DFNB1 patients

Abstract

Objective

Study Design

Setting

Results

Conclusion

Get full access to this article

References