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 current study included a within subjects, repeated-measures design including 21 English speaking and 17 Polish speaking cochlear implant 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 8-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: cochlear implant (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 6 English-speaking listeners were also assessed on measures of interaural time difference (ITD) 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 vs. the bimodal condition. Postoperative thresholds in the implanted ear were correlated with the degree of 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 following surgery and improvement in speech understanding in reverberation. There was a significant correlation between ITD threshold at 250 Hz and EAS-related benefit for the adaptive SRT. Conclusions Our results suggest that (i) preserved low-frequency hearing improves speech understanding for CI recipients (ii) 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 (iii) preservation of binaural timing cues, albeit poorer than observed for individuals with normal hearing, is possible following unilateral cochlear implantation with hearing preservation and is associated with EAS benefit. Our results demonstrate significant communicative benefit for hearing preservation in the implanted ear and provide support for the expansion of cochlear implant criteria to include individuals with low-frequency thresholds in even the normal to near-normal range.

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The authors present the accepted strategy of Partial Deafness Treatment (PDT) based on long-term observation and results: 8-years long in adult patients and over 5-years long in children. In therapy, there are two fundamental modes of complementary stimulation in cases of moderate to severe hearing loss. One of them is the acoustic stimulation (AS), comprising patients who use amplification with hearing aid (HA) and/or middle ear implant (MEI). The other mode, presented by the authors in this study, is the electric stimulation using cochlear implant (PDCI). The entire material in this mode is divided into three groups: 1. Electrical Complement (EC); 2. Electric Acoustic Stimulation (EAS); and 3. Electric Stimulation (ES). Surgical approach in PDT is based on the 6-steps method, emphasizing round window approach to the scala tympani. The results obtained in the long-term follow-up shows the preservation of preoperative hearing in 97% of subjects. Overall, for all audiometric frequencies the differences in mean pre- and mean postoperative thresholds, measured before surgery and 3 months afterwards were not statistically significant (p>0.05). In all four groups we observed a significant increase in scores between pre-operative and 12 months after surgery both under quiet and noisy conditions. The presented concept, supported by the substantial material and long-term follow-up, allows the comprehensive approach to the treatment of partial deafness using different modes of stimulation.

The Hearing Preservation Classification System proposed herein fulfills the following necessary criteria: 1) classification is independent from users' initial hearing, 2) it is appropriate for all cochlear implant users with measurable pre-operative residual hearing, 3) it covers the whole range of pure tone average from 0 to 120 dB; 4) it is easy to use and easy to understand.

Remote programming sessions were successfully finished for 69 recipients. No significant differences between T and C levels obtained by local and remote programming were found. The audiologists and monitoring clinicians agreed that the remote programming system provided an acceptable level of performance after most sessions. More than 50 participating recipients considered remote programming an efficient alternative to face-to-face-programming.

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.

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.

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 approximately 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.