Henryk Skarżyński

Factors Affecting Auditory Performance of Postlinguistically Deaf Adults Using Cochlear Implants: An Update with 2251 Patients

Objective: To update a 15-year-old study of 800 postlinguistically deaf adult patients showing how duration of severe to profound hearing loss, age at cochlear implantation (CI), age at onset of severe to profound hearing loss, etiology and CI experience affected CI outcome. Study Design: Retrospective multicenter study. Methods: Data from 2251 adult patients implanted since 2003 in 15 international centers were collected and speech scores in quiet were converted to percentile ranks to remove differences between centers. Results: The negative effect of long duration of severe to profound hearing loss was less important in the new data than in 1996; the effects of age at CI and age at onset of severe to profound hearing loss were delayed until older ages; etiology had a smaller effect, and the effect of CI experience was greater with a steeper learning curve. Patients with longer durations of severe to profound hearing loss were less likely to improve with CI experience than patients with shorter duration of severe to profound hearing loss. Conclusions: The factors that were relevant in 1996 were still relevant in 2011, although their relative importance had changed. Relaxed patient selection criteria, improved clinical management of hearing loss, modifications of surgical practice, and improved devices may explain the differences.

Preservation of low frequency hearing in partial deafness cochlear implantation (PDCI) using the round window surgical approach

Conclusion. Successful hearing preservation is possible in individuals with excellent low frequency hearing. This is possible due to the partial insertion of an atraumatic electrode using an atraumatic round window surgical technique. Objectives. This paper describes the round window surgical technique used to preserve excellent low frequency hearing in patients receiving partially inserted MED-EL cochlear implant electrodes. Results of preserved low frequency hearing in partial deafness cochlear implantation (PDCI) are reported. Patients and methods. The surgical approach is described in detail. Ten subjects received a partial insertion of a standard electrode, using the round window approach. Pure tone audiometry was conducted in the implanted and non-implanted ear preoperatively, at implant fitting and then at 1, 3, 6 and 12 months after initial device fitting. Results. Results show hearing preservation in 9 of the 10 subjects. One subject lost all hearing 2 weeks after cochlear implantation. Hearing has remained essentially stable up to the 1 year postoperative period. Eight of the nine subjects use the cochlear implant together with their natural low frequency hearing; one subject uses a hearing aid in the implanted ear to amplify the low frequencies.

Pre-, Per- and Postoperative Factors Affecting Performance of Postlinguistically Deaf Adults Using Cochlear Implants: A New Conceptual Model over Time

Objective To test the influence of multiple factors on cochlear implant (CI) speech performance in quiet and in noise for postlinguistically deaf adults, and to design a model of predicted auditory performance with a CI as a function of the significant factors. Study Design Retrospective multi-centre study. Methods Data from 2251 patients implanted since 2003 in 15 international centres were collected. Speech scores in quiet and in noise were converted into percentile ranks to remove differences between centres. The influence of 15 pre-, per- and postoperative factors, such as the duration of moderate hearing loss (mHL), the surgical approach (cochleostomy or round window approach), the angle of insertion, the percentage of active electrodes, and the brand of device were tested. The usual factors, duration of profound HL (pHL), age, etiology, duration of CI experience, that are already known to have an influence, were included in the statistical analyses. Results The significant factors were: the pure tone average threshold of the better ear, the brand of device, the percentage of active electrodes, the use of hearing aids (HAs) during the period of pHL, and the duration of mHL. Conclusions A new model was designed showing a decrease of performance that started during the period of mHL, and became faster during the period of pHL. The use of bilateral HAs slowed down the related central reorganization that is the likely cause of the decreased performance.

Preservation of Residual Hearing in Children and Post-Lingually Deafened Adults after Cochlear Implantation: An Initial Study

Objective: To investigate whether the residual hearing of severely hearing-impaired children and adults could be preserved using the soft surgery approach. Patients and Methods: This project employed a prospective study design. All testing and surgery took place in the Institute of Physiology and Pathology of Hearing, Warsaw, Poland. Twenty-six patients (7 children and 19 post-lingually deafened adults) with residual hearing were assessed. Subjects were assessed using conventional pure-tone audiometry at least 1 month prior to surgery. Cochlear implant surgery with a Med-El Combi 40/40+ standard electrode array was conducted, using the soft surgery approach. Pure-tone audiometry thresholds were re-assessed at least 1 month after surgery. The researchers assessed change in auditory thresholds using pure-tone audiometry to determine preservation of residual hearing. Results: Sixteen of 26 patients (62%) retained their residual hearing within 5 dB HL of pre-operative scores. Only 5 of 26 patients (19%) lost all measurable residual hearing after cochlear implantation. This suggests that surgeons are often able to preserve residual hearing during cochlear implant surgery using the soft surgery technique. Conclusions: Preservation of residual hearing is an important consideration in cochlear implantation in the light of changing selection criteria for cochlear implant candidates, and as younger children are receiving implants. This is important, as we do not know yet the long-term effects of inner ear damage due to traumatic insertions of electrodes. This finding suggests a good prognosis for future possibilities of re-implantation.

M34T and V37I mutations in GJB2 associated hearing impairment: Evidence for pathogenicity and reduced penetrance

Despite research the role of the M34T and V37I variants of GJB2 in causing hearing impairment (HI) remains controversial. Our purpose was to test a hypothesis that M34T and V37I are pathogenic but have distinct features resulting in a reduced penetrance. We screened for known GJB2/GJB6 mutations 233 Polish consecutive unrelated subjects with non‐syndromic, sensorineural HI who were previously found to carry 35delG mutation on one chromosome. The most frequent mutations were also analyzed in ∼1,000 controls. We found that M34T and V37I were significantly (P ≪ 10−6) overrepresented among patients, but their penetrance was estimated as 1/10 relative to mutations of undisputed pathogenicity. This finding apparently could not be explained by low degree of HI associated with M34T and V37I since another mutation causing comparably mild HI (L90P) did not have reduced penetrance. Subsequent analyses showed that the patients with M34T/35delG and V37I/35delG had significantly later onset of HI than patients with other genotypes (P < 10−6) including the L90P/35delG (P = 0.006). Also, among these patients (but not others) a strong correlation between the degree of HI and its duration was found (r = 0.79, P < 10−5). We tentatively suggest that M34T and V37I might cause mild HI characterized by relatively late onset and progression.

Partial deafness cochlear implantation provides benefit to a new population of individuals with hearing loss

Conclusion. Partial deafness cochlear implantation (PDCI) is a feasible means of treating individuals who have good low frequency hearing, but a severe to profound hearing loss in the mid to high frequencies. The individuals have benefit in noise and show significant benefit in a number of difficult listening conditions, when compared with their acoustic-only hearing prior to implantation. This benefit is maintained over time. Objectives. PDCI using the round window surgical technique is one means of treating individuals with a ‘ski-slope’ hearing loss, who gain minimal benefit from amplification with conventional hearing instruments. This paper aims to demonstrate the benefit that PDCI provides these individuals. Patients and methods. Ten subjects received a partial insertion of a standard MED-EL electrode, using the round window approach. Pure tone audiometry and monosyllable testing in quiet and noise were conducted preoperatively, at implant fitting and then at 1, 3, 6 and 12 months after initial device fitting. The APHAB questionnaire was completed by subjects preoperatively and then at 6 and 12 months after receiving their cochlear implant. Results. Hearing was preserved in 9 of 10 cases. One subject uses a hearing aid to amplify low frequency hearing, the remainder use natural low frequency hearing. Improvements in monosyllabic scores over time in both quiet and noise were significant, particularly within the first 3 months of PDCI use.

Cochlear implantation with hearing preservation yields significant benefit for speech recognition in complex listening environments

Objective: The aim of this study was to assess the benefit of having preserved acoustic hearing in the implanted ear for speech recognition in complex listening environments. Design: The present study included a within-subjects, repeated-measures design including 21 English-speaking and 17 Polish-speaking cochlear implant (CI) recipients with preserved acoustic hearing in the implanted ear. The patients were implanted with electrodes that varied in insertion depth from 10 to 31 mm. Mean preoperative low-frequency thresholds (average of 125, 250, and 500 Hz) in the implanted ear were 39.3 and 23.4 dB HL for the English- and Polish-speaking participants, respectively. In one condition, speech perception was assessed in an eight-loudspeaker environment in which the speech signals were presented from one loudspeaker and restaurant noise was presented from all loudspeakers. In another condition, the signals were presented in a simulation of a reverberant environment with a reverberation time of 0.6 sec. The response measures included speech reception thresholds (SRTs) and percent correct sentence understanding for two test conditions: CI plus low-frequency hearing in the contralateral ear (bimodal condition) and CI plus low-frequency hearing in both ears (best-aided condition). A subset of six English-speaking listeners were also assessed on measures of interaural time difference thresholds for a 250-Hz signal. Results: Small, but significant, improvements in performance (1.7–2.1 dB and 6–10 percentage points) were found for the best-aided condition versus the bimodal condition. Postoperative thresholds in the implanted ear were correlated with the degree of electric and acoustic stimulation (EAS) benefit for speech recognition in diffuse noise. There was no reliable relationship among measures of audiometric threshold in the implanted ear nor elevation in threshold after surgery and improvement in speech understanding in reverberation. There was a significant correlation between interaural time difference threshold at 250 Hz and EAS-related benefit for the adaptive speech reception threshold. Conclusions: The findings of this study suggest that (1) preserved low-frequency hearing improves speech understanding for CI recipients, (2) testing in complex listening environments, in which binaural timing cues differ for signal and noise, may best demonstrate the value of having two ears with low-frequency acoustic hearing, and (3) preservation of binaural timing cues, although poorer than observed for individuals with normal hearing, is possible after unilateral cochlear implantation with hearing preservation and is associated with EAS benefit. The results of this study demonstrate significant communicative benefit for hearing preservation in the implanted ear and provide support for the expansion of CI criteria to include individuals with low-frequency thresholds in even the normal to near-normal range.

Outcomes of treatment of partial deafness with cochlear implantation: a DUET study.

Objectives: To compare speech test performance of adults with partial deafness cochlear implantation (PDCI) with that of adults with cochlear implant (CI). Based on the results, our objective is to determine the efficacy of the two applications of cochlear implantation, the first characterized by a shallow electrode insertion and preservation of low‐frequency natural hearing for partial deafness, and the second characterized by a very deep electrode insertion used in subjects with severe to profound deafness. All the PDCI participants in this study were fitted with a recently upgraded DUET Hearing System from Med‐El Corporation, Innsbruck, Austria.

Partial Deafness Treatment with the Nucleus Straight Research Array Cochlear Implant

Introduction: The Nucleus Straight Research Array (SRA) cochlear implant has a new 25-mm electrode carrier designed to minimize insertion trauma, in particular allowing easy insertion via the round window. The aims of this study were to measure preoperative to postoperative benefit in terms of speech recognition in quiet and in noise in three groups of patients (electrical complement, EC; electrical stimulation, ES; electro-acoustic stimulation, EAS) with varying levels of low-frequency hearing, and to evaluate the preservation of residual hearing after implantation with the SRA cochlear implant. Methods: The study design was prospective with sequential enrolment and within-subject comparisons: 23 adult cochlear implant candidates were divided into three groups according to their level of preoperative residual hearing at 500 Hz (EC ≤50 dB; 50 dB < EAS < 80 dB; ES ≧80 dB). Monosyllabic word recognition using the SRA cochlear implant in combination with residual low-frequency hearing was assessed at 4 and 13 months after implantation. Hearing threshold levels were also monitored over time. Results: Subjects across all three groups had significant improvements in speech recognition scores (i.e. >20 percentage points) both for listening in quiet (71% of subjects) and in noise (100% of subjects). The average score at 4 months after operation for words presented in quiet was 61.7%, and in 10 dB SNR noise 46.5%, compared to 34.4 and 10.6% preoperatively (p < 0.001). All subjects retained measurable hearing at 500 Hz in the implanted ear at 4 months after the operation; mean increases were 19, 29 and 1 dB for the EC, EAS and ES groups (n = 21). Across frequencies of 125–1000 Hz, the median increase in thresholds was 15 dB up to 13 months postoperatively (n = 15). Conclusions: Speech recognition performance of subjects with various levels of residual low-frequency hearing was significantly improved with the SRA cochlear implant. A high level and rate of hearing preservation was achieved with the SRA implanted using a round window surgical technique. Subjects with preoperative low-frequency hearing levels between 50 and 80 dB HL (EAS group) tended to lose more hearing than those with either better or worse hearing.

Results of Partial Deafness Cochlear Implantation Using Various Electrode Designs

Nineteen adults and 9 children who received a unilateral cochlear implant between 2002 and 2007 were included in the study. All subjects were preoperatively diagnosed with significant residual hearing in low frequencies, termed as ‘partial deafness’, and were implanted according to a 6-step round window surgical technique for partial deafness cochlear implantation. Hearing was preserved to a great extent in the partial deafness cochlear implantation (PDCI) group. After a short period following activation of the cochlear implant, highly significant improvement in the recognition of monosyllabic words was observed. With a developed round window surgical procedure and limited electrode insertion, hearing can be preserved in the majority of patients with partial deafness. PDCI is a feasible means of treating individuals who have good low-frequency hearing but severe to profound hearing loss in the mid to high frequencies.