Active inhibition of the first over the second ear implanted sequentially.

OBJECTIVE: To identify the influence of the first implanted ear on the performance of the delayed sequentially implanted ear in pediatric patients. STUDY DESIGN: Retrospective case series review. SETTING: Outpatient Cochlear Implant (CI) center. PATIENTS: Congenitally deaf children who underwent unilateral implant at various ages followed by sequential implant of the contralateral ear with various delays were enrolled in the study. Children were divided into two groups based on the retention of the first implant or its removal, creating a sequential unilateral user group (USG) and a sequential bilateral user group (BSG). Both groups reflect differing durations of auditory deprivation in each ear, being relatively longer for the USG group for each ear than for the BSG group. INTERVENTION: Rehabilitative. MAIN OUTCOME MEASURES: All patients underwent periodic speech recognition testing for two years after the second surgery. The results for each implanted ear in both groups were evaluated and an index score representing the Difference Between the Ears, (DBE), was calculated. RESULTS: A statistically significant difference results was obtained between the groups. Both groups reached interaural equivalence (i.e. DBE=0%), after two years post-sequential implant. The USG patients achieved interaural equivalence at a faster rate than the BSG patients with 85% USG subjects demonstrating it at 6months compared to the majority of BSG patients demonstrating equivalence at one-year post-sequential implant. CONCLUSIONS: The performance of the sequentially implanted ear depends to an extent on a negative influence, which could be considered as a type of inhibition, of the first implanted ear over the second ear. It appears that the role of the unilateral auditory deprivation is not the primary influencing factor. These findings have driven us clinically to redefine some important considerations for rehabilitation management of the deaf children in the cases of delayed sequential implant.

Analysis of the impact of professional involvement in evidence generation for the HTA Process, subproject "cochlear implants": methodology, results and recommendations.

The aim of Health Technology Assessment (HTA) is to provide decision-makers, distributors and recipients with information on the effectiveness, cost and impact of health technologies. The present study constitutes a subproject within the wider project "Analysis of the impact of professional involvement in evidence generation for the HTA process", which is part of the strategic programme "Transfer of the results of the research in clinical practice and organisation of healthcare services", coordinated by Laziosanità - Agency of Public Healthcare of the Lazio Region and AgeNaS (National Agency for Regional Healthcare Services). The objectives of the present subproject (cochlear implants) are as follows: a) to produce a report regarding the health impact of cochlear implants (CI) on their recipients, through a systematic review of literature and extensive selection of relative studies, combining the outcomes with metanalytical techniques. Output: report on the indications of usage in the groups of population for which benefits are controversial; b) to create a registry of patients using cochlear implants. The registry should contain a selection of anagraphic and clinical information relative to patient follow-up in order to assess factors associated with safety and impact on cochlear implant users. This source of information is essential for future observational studies. This was divided into 4 phases: 1(st) phase: definition of key participants in the assessment process; 2(nd) phase: definition of methods and timing of "Aims" (definition of the objective); 3(rd) phase: definition of the methods and times of the "assessment process", 4(th) phase: production of the final report. From the analysis of systematic reviews and italian and international guidelines, the Working Group members approved recommendations on the following topics: results after CI in children in relation to age at implantation, bilateral CI in children, CI in deaf children with associated disabilities, CI in adults with advanced age, bilateral CI in adults and CI in adults with pre-lingual deafness. These recommendations have also been evaluated by the Consulting Committee members and approved with minimal suggestions.

Systematic review of the literature on the clinical effectiveness of the cochlear implant procedure in paediatric patients.

The aim of this systematic review of the literature was to summarize the results of scientific publications on the clinical effectiveness of the cochlear implant (CI) procedure in children. The members of the Working Group first examined existing national and international literature and the principal international guidelines on the procedure. They considered as universally-accepted the usefulness/effectiveness of unilateral cochlear implantation in severely-profoundly deaf children. Accordingly, they focused attention on systematic reviews addressing clinical effectiveness and cost/efficacy of the CI procedure, with particular regard to the most controversial issues for which international consensus is lacking. The following aspects were evaluated: post-CI outcomes linked to precocity of CI; bilateral (simultaneous/ sequential) CI vs. unilateral CI and vs. bimodal stimulation; benefits derived from CI in deaf children with associated disabilities. With regard to the outcomes after implantation linked to precocity of intervention, there are few studies comparing post-CI outcomes in children implanted within the first year of life with those of children implanted in the second year. The selected studies suggest that children implanted within the first year of life present hearing and communicative outcomes that are better than those of children implanted after 12 months of age. Concerning children implanted after the first year of life, all studies confirm an advantage with respect to implant precocity, and many document an advantage in children who received cochlear implants under 18 months of age compared to those implanted at a later stage. With regard to bilateral CI, the studies demonstrate that compared to unilateral CI, bilateral CI offers advantages in terms of hearing in noise, sound localization and during hearing in a silent environment. There is, however, a wide range of variability. The studies also document the advantages after sequential bilateral CI. In these cases, a short interval between interventions, precocity of the first CI and precocity of the second CI are considered positive prognostic factors. In deaf children with associated disabilities, the studies analyzed evidence that the CI procedure is also suitable for children with disabilities associated with deafness, and that even these children may benefit from the procedure, even if these may be slower and inferior to those in children with isolated deafness, especially in terms of high communicative and perceptive skills.

A registry for the collection of data in cochlear implant patients.

The need to optimize the use of all the information that modern technological tools have made available to the physician ENT/audiologist has increasingly emerged within the Italian scientific community. Towards this purpose, it is necessary to create a registry of the patients using cochlear implants (CIs). This registry will include a homogeneous summary of the information deriving from multiple sources related to daily clinical practice, in order to assess auditory benefits, safety and reliability in patients with cochlear implants, and organization over the national territory. The primary objectives relative to the above-mentioned analysis are to assess the impact of the use of cochlear implants on patient health, to ensure traceability of the devices currently used, monitoring their safety and reliability over time, to guarantee access of the technique in clinical and organizational conditions that can allow the best possible benefits. The aspects concerning implementation of the registry were discussed extensively during the first meetings of the Working Group (WG). In particular, owing to the complexity and high costs related mainly to the development of the technological aspects and the need to involve technological partners external to the WG, and to respect current privacy laws, the WG members decided that the project should be limited to proposal of a paper registry to be implemented at a later stage, possibly within the framework of successive research projects. During meetings, the WG members discussed various aspects of implementation of the registry, and in particular the scientific features connected to objectives, inclusion criteria, and structure of the forms needed for data collection and organizational aspects. A registry is proposed herein.

Auditory cortical responses in patients with cochlear implants.

Currently, the most commonly used electrophysiological tests for cochlear implant evaluation are Averaged Electrical Voltages (AEV), Electrical Advisory Brainstem Responses (EABR) and Neural Response Telemetry (NRT). The present paper focuses on the study of acoustic auditory cortical responses, or slow vertex responses, which are not widely used due to the difficulty in recording, especially in young children. Aims of this study were validation of slow vertex responses and their possible applications in monitoring postimplant results, particularly restoration of hearing and auditory maturation. In practice, the use of tone-bursts, also through hearing aids or cochlear implants, as in slow vertex responses, allows many more frequencies to be investigated and louder intensities to be reached than with other tests based on a click as stimulus. Study design focused on latencies of N1 and P2 slow vertex response peaks in cochlear implants. The study population comprised 45 implant recipients (aged 2 to 70 years), divided into 5 different homogeneous groups according to chronological age, age at onset of deafness, and age at implantation. For each subject, slow vertex responses and free-field auditory responses (PTAS) were recorded for tone-bursts at 500 and 2000 Hz before cochlear implant surgery (using hearing aid amplification) and during scheduled sessions at 3rd and 12th month after implant activation. Results showed that N1 and P2 latencies decreased in all groups starting from 3rd through 12th month after activation. Subjects implanted before school age or at least before age 8 yrs showed the widest latency changes. All subjects showed a reduction in the gap between subjective thresholds (obtained with free field auditory responses) and objective thresholds (obtained with slow vertex responses), obtained in presurgery stage and after cochlear implant. In conclusion, a natural evolution of neurophysiological cortical activities of the auditory pathway, over time, was found especially in young children with prelingual deafness and implanted in preschool age. Cochlear implantation appears to provide hearing restoration, demonstrated by the sharp reduction of the gap between subjective free field auditory responses and slow vertex responses threshold obtained with hearing aids vs. cochlear implant.

Normative findings of electrically evoked compound action potential measurements using the neural response telemetry of the Nucleus CI24M cochlear implant system.

One hundred and forty-seven adult recipients of the Nucleus 24 cochlear implant system, from 13 different European countries, were tested using neural response telemetry to measure the electrically evoked compound action potential (ECAP), according to a standardised postoperative measurement procedure. Recordings were obtained in 96% of these subjects with this standardised procedure. The group results are presented in terms of peak amplitude and latency, slope of the amplitude growth function and ECAP threshold. The effects of aetiological factors and the duration of deafness on the ECAP were also studied. While large intersubject variability and intrasubject variability (across electrodes) were found, results fell within a consistent pattern and a normative range of peak amplitudes and latencies was established. The aetiological factors had little effect on the ECAP characteristics. However, age affected ECAP amplitude and slope of the amplitude growth function significantly; i.e., the amplitude is higher in the lowest age category (15-30 years). Principal component analysis of the ECAP thresholds shows that the thresholds across 5 electrodes can be described by two factors accounting for 92% of the total variance. The two factors represent the overall level of the threshold profiles ('shift') and their slopes across the electrode array ('tilt'). Correlation between these two factors and the same factors describing the T- and C-levels appeared to be moderate, in the range of 0.5-0.6.

Occurrence of del(GIB6-D13S1830) mutation in Italian non-syndromic hearing loss patients carrying a single GJB2 mutated allele.

Molecular screening for GJB2 (connexin 26) mutations represents the standard diagnostic approach for the genotype definition of non-syndromic deafness. Nevertheless, a single GJB2 pathogenic mutation is detectable in a relevant number of cases, therefore failing to explain the phenotype. We aimed at assessing the occurrence of the recently described del(GIB6-D13S1830) mutation, occurring in the connexin 30 gene, in a group of Italian hearing-impaired patients carrying a single GJB2 mutated allele. A total of 59 non-syndromic hearing loss (NSHL) patients were screened for GJB2 mutations. Among these, nine NSHL patients were found to be heterozygous for a single GJB2 mutation. These patients, heterozygotes for different GJB2 mutated alleles (35delG, L90P, M34T, V153I), together with 11 additional 35delG/neg cases previously described, were studied for the presence of the del(GIB6-D13S1830) mutation. Two double heterozygotes del(GIB6-D13S1830)/35delG were identified. In both cases the degree of hearing loss was profound. Furthermore, GJB2 molecular screening led to the identification of a novel change (T55G) occurring in compound heterozygosity with the V37I mutation. In conclusion, our data suggest a significant frequency of del(GIB6-D13S1830) mutation in Italian hearing-impaired subjects (10% of unexplained GJB2 heterozygotes) similar to that reported in other European countries.

Exploring the clinical and epidemiological complexity of GJB2-linked deafness.

GJB2 mutation analysis was performed in 179 unrelated subjects with sporadic or familial hearing loss (HL). Among 57 families, 18 showed a vertical transmission of HL, the disease being present in two or three generations. Besides 155 nonsyndromic cases, 24 patients presenting with extra-auditory clinical signs were included in the molecular study. GJB2 mutation analysis was also performed in 19 subjects with an anamnestic history of perinatal risks factors for acquired HL. The 35delG mutation accounted for 22.1% of analyzed chromosomes in sporadic cases and 39.4% in familial cases; 35delG prevalence reached 41% in autosomal recessive and 44.4% in pseudodominant pedigrees. Two novel GJB2 mutations were identified in compound heterozygosity with 35delG allele (D159V, 284ins/dup[CACGT]). Two 35delG homozygous subjects were identified among HL cases classified as environmental in origin. Four patients 35delG heterozygous (35delG/V95M, 35delG/L90P, 35delG/167delT, and 35delG/?) and two homozygous presented with extra-auditory clinical signs involving different organs (skin, vascular system, hemopoietic lineages, and thyroid). In a high proportion of 35delG heterozygous HL patients (52%), no second GJB2 mutation was detected. The reported data highlight the complexity of the genetic epidemiology of GJB2-linked deafness, further enlarging the spectrum of situations in which GJB2 mutation analysis should be performed. The presence of extra-auditory signs in a significant portion of GJB2-mutated patients suggests the possibility that GJB2 loss of function could contribute to clinical phenotypes presenting in association with deafness. This hypothesis deserves further investigation. The failure to identify a presumed partnering GJB2 mutation in a high proportion of deaf patients remains a challenging problem to be clarified.

[Temporal bone metastasis of adenocarcinoma of the breast: personal observations and a review of the literature]. / Metastasi temporale da adenocarcinoma mammario: osservazione personale e revisione della letteratura.

A single case of temporal bone metastases from breast carcinoma is reported and its clinical features and main instrumental diagnostic characteristics are described. Although this is quite rare the possibility cannot be neglected when making diagnosis should the clinical features and test results suggest such an expansive process of the temporal bone. In fact, although extremely limited, treatment is strictly linked to proper and early diagnosis.

[Evoked potentials of the brain stem in normal and pathologic conditions: experience with 692 adult subjects]. / I potenziali evocati del tronco encefalico nel normale e nel patologico: esperienza su 692 soggetti adulti.

The main purpose of the study of evoked potentials has always been to objectively evaluate hearing thresholds in uncooperative subjects and it has become increasingly important in recent years with the introduction of auditory brainstem responses (ABR). Reading the ABR is relatively simple in terms of "audiometry" since it is based on the absence, or presence, of a marker peak wave V. Besides the strictly audiometric uses, study of the bioelectric brainstem complex even makes it possible to obtain important information regarding retrocochlear dysfunction. However, reading the response is not so simple for the otoneurologist since it is not based only on the observation of the absence or presence in order to compare the normal values obtained in homogeneous groups. For the above reason auditory brainstem response testing was performed on 692 subjects in order to define the normal values as well as any characteristic patterns for various audiological pathologies. For all subjects auditory brainstem responses were recorded mono-aurally with clicks of alternating polarity, 11 and 31 pps, at an intensity of 120 dB SPLpe. Use was made of ipsilateral stimulus derivations. Only for the right ear was absolute wave V latency measured at 100 and at 80 dB SPLpe. The amplifier of the bioelectric signal was set at a sensitivity of 20 microvolts with passing filters of 100 and 2000 Hz. Absolute latency, conduction time, symmetry indexes (IT I-V and ITV) and the shift in latency at the two stimulation cadences were statistically processed for homogeneous groups. Thus standards for normality were obtained. The results have shown that the following biological variables are to be taken into consideration: sex, age for males and type of deafness. The indexes most reliable in identifying a retrocochlear dysfunction proved to be: morphological completeness, central I-V conduction times and the two symmetry indexes IT I-V and ITV which are considered complementary. Again in terms of identifying a retrocochlear dysfunction, and in case of an incomplete response, the test can be interpreted thanks to the so-called minor responses which only take into account the absolute wave V latency. However, wave V latency behaviour is rather similar to what has been found in conductive hearing-loss. Finally, in cochlear deafness it was found that response did not vary with a variation in degree of deafness since the results produced were similar to those of normal hearing subjects except in those cases where there was a delay in the appearance of wave I.

[Stereophonic apparatus: our hearing aid test]. / Appareillages stéréophoniques : notre test audioprothétique.

The authors wanted to verify the advantages produced by binaural amplification in the loudness and in the speech identification. To value the improvement in speech identification they used the Jerger's test (1976); and to value the loudness improvement they measured, gain characteristics of the aid with monoaural and binaural amplification. They executed the test on 50 hearing aid users, chosen among 10 and 84 years old, suffering from sensorineural bilateral hearing loss between 40 and 80 dB. It results that : 1. The gain obtained by the use of binaural amplification is 5 dB lower than the one obtained by monoaural. 2. The binaural amplification permits a remarkable advantage in speech identification, the advantage is directly proportionated to deafness. The authors conclude that the described test is valid, simple and that it is possible to apply it by cheap instruments.