Comparison of rehabilitation results in deaf patients with and without genetically related hearing loss

Abstract

The introduction of prognostic tools to evaluate rehabilitation progress in cochlear implant patients (CI patients) is of great importance. The authors attempted to verify whether the identified 35delG mutation in the GJB2 gene can serve as a valuable indicator for rehabilitation progress of CI patients. A group of 51 subjects was studied. Molecular analysis was based on the identification of 35delG in GJB2. Logopedic assessment was performed with a non-verbal test of seven sounds, evaluating detection, discrimination and identification of the sounds during the first, third and sixth months after implantation. Results indicated that patients with GJB2-related deafness (DFNB1)s achieve better results in rehabilitation, but only at the early stages of rehabilitation. Prolonged rehabilitation equalised differences, which, subsequently, excluded this marker as an indicator for rehabilitation evaluation.

Prediction of cochlear implant performance by genetic mutation: The spiral ganglion hypothesis

“… a GJB2 references include: (Angeli et al, 2011; Arndt et al, 2010; Bauer et al, 2003; Chora et al, 2010; Choung et al, 2008; Connell et al, 2007; Cullen et al, 2004; Dahl et al, 2003; Dalamon et al, 2009; Daneshi et al, 2011; Fukushima et al, 2002; Gerard et al, 2010; Green et al, 2002; Karamert et al, 2011; Lustig et al, 2004; Matsushiro et al, 2002; Reinert, Honegger & Gurtler, 2010; Sinnathuray et al, 2004a; Sinnathuray et al, 2004b; Taitelbaum-Swead et al, 2006; Weegerink et al, 2011a; Wrobel et al, 2008; Wu et al, 2008; Wu et al, 2011a; Yoshikawa et al, 2011. …”

Is deafness etiology important for prediction of functional outcomes in pediatric cochlear implantation?

“…They depend mainly on the native language features and experiences in CI programs in the given geographic region. The tests we have chosen for follow-up are in good correlation with the patient's overall Different authors also assessed the hearing outcomes for dissimilar time periods elapsing from CI, ranging from up to 6 months to over 5 years and thus making the comparisons or meta-analyses even more difficult [7,15]. In particular, the short evaluation periods may lead to ambiguous conclusions.…”

“…Patients carrying causal biallelic mutations in either of the two genes seem to strongly benefit from CI [6]. However, the impact of connexin 26 gene on CI outcomes, when compared to other deafness etiologies, is rather controversial [7,8].…”