Influence of the pre- or postlingual status of cochlear implant recipients on behavioural T/C-levels.

INTRODUCTION: Previous auditory experience modifies the sensitivity of the auditory cortex to the afferent activity of the auditory pathways and may influence the threshold (T) and comfort (C) levels in patients receiving a cochlear implant (CI). Literature data on this particular topic is very scarce. OBJECTIVE: This study aimed to evaluate the differences in T/C-levels between pre- and postlingually implanted cochlear implant patients. METHODS: Retrospective case review in a quaternary otologic referral centre was performed. Data on the T/C-levels have been collected in 90 consecutive CI patients divided into 2 groups. Group 1 comprised 16 prelingually deaf children implanted between 8 months and 10 years of age. Group 2 comprised 74 postlingually deaf adults (average age of 62 years). All patients were users of the Nucleus 24RECA (Freedom, Contour Advance-of-Stylet electrode) cochlear implant. All measurements were performed at the fifth implant programming session at 4-6 months after surgery, when stable T/C thresholds have already been obtained. RESULTS: The behavioural C-levels present important and statistically significant differences between the pre- and postlingually implanted patients for all electrode contacts that could reach 30 CL. For the T-levels the observed differences were smaller and statistically insignificant for most electrode contacts. CONCLUSIONS: The previous auditory experience (pre- or postlingual deafness) seems to be an independent parameter influencing the T/C-levels in patients receiving a CI. Together with the electrode contact impedance and the contact position in the electrode array it can explain up to 37% of the variability in the definition of the C-levels. The fact that the stabilised C-levels measured 4-6 months postoperatively can be up to 30 CL higher in the prelingually deaf patients than in the postlingual ones results also in a much higher dynamic range observed in prelingual subjects. Therefore implant programming of the prelingual patients should be very cautious in order to avoid the risk of overstimulation.

Prediction of Behavioral T/C Levels in Cochlear Implant Patients Based Upon Analysis of Electrode Impedances.

BACKGROUND: Fitting cochlear implants in babies and noncooperative patients is cumbersome and time consuming. Therefore, objective parameters have been sought in order to predict the subjective threshold (T) and maximum comfort (C) levels. Measurements of the electrically evoked compound action potentials (ECAPs) have been widely used for this purpose, yet the correlation between these objective measures and the subjective T/C levels is weak to moderate. PURPOSE: This article aims (1) to evaluate correlations between the subjective parameters of the fitting maps such as thresholds (T level) and maximum comfort levels (C level), the impedance of the electrode contacts, and the ECAP thresholds, and (2) to compare the value of the electrode impedances and the ECAP measures for prediction of the T/C levels. RESEARCH DESIGN: Case review study in a quaternary otologic referral center. STUDY SAMPLE: Ninety-eight consecutive CI patients were enrolled. The average age of the patients was 49 years. All patients were users of the Nucleus 24RECA (Freedom, Contour Advance-of-Stylet electrode) cochlear implant. DATA COLLECTION AND ANALYSIS: Data on impedance of the electrode contacts and the behavioral T/C levels at the first fitting session (2-5 weeks after surgery) and at the 5th fitting session (4-6 months after surgery) have been retrospectively collected in 98 consecutive CI patients. Additionally, the intraoperative impedance values and the ECAP thresholds (tNRT) have been recorded. RESULTS: Impedances of electrode contacts show significant strong negative correlations with the stabilized T/C levels at 4 to 6 months after implantation and are an important predictor for the behavioral T/C levels. They can explain R 2 = 28 to 41% of the variability of the behavioral T/C levels. In multiple regression analysis electrode contact impedances can explain twice as much of the variability of the stabilized T/C levels than the tNRT values. The electrode impedances together with the tNRT values are able to explain R 2 = 37 to 40% of the global variability of the T/C levels while the tNRT thresholds solely are able to explain only R 2 = 5 to 14% of the T/C levels variability. CONCLUSION: Impedances of electrode contacts correlate strongly with the stabilized behavioral T/C levels and may be used as an objective measure for fitting of cochlear implants.

Prognostic significance of electrophysiological tests for facial nerve outcome in vestibular schwannoma surgery.

OBJECTIVE: To assess the prognostic significance of pre-operative electrophysiological tests for facial nerve outcome in vestibular schwannoma surgery. METHODOLOGY: Retrospective study design in a tertiary referral neurology unit. We studied a total of 123 patients with unilateral vestibular schwannoma who underwent microsurgical removal of the lesion. Nine patients were excluded because they had clinically abnormal pre-operative facial function. Pre-operative electrophysiological facial nerve function testing (EPhT) was performed. Short-term (1 month) and long-term (1 year) post-operative clinical facial nerve function were assessed. RESULTS: When pre-operative facial nerve function, evaluated by EPhT, was normal, the outcome from clinical follow-up at 1-month post-operatively was excellent in 78% (i.e. HB I-II) of patients, moderate in 11% (i.e. HB III-IV), and bad in 11% (i.e. HB V-VI). After 1 year, 86% had excellent outcomes, 13% had moderate outcomes, and 1% had bad outcomes. Of all patients with normal clinical facial nerve function, 22% had an abnormal EPhT result and 78% had a normal result. No statistically significant differences could be observed in short-term and long-term post-operative facial function between the groups. CONCLUSION: In this study, electrophysiological tests were not able to predict facial nerve outcome after vestibular schwannoma surgery. Tumour size remains the best pre-operative prognostic indicator of facial nerve function outcome, i.e. a better outcome in smaller lesions.

Transient depression of inner ear function after stapedotomy: skeeter versus CO(2) laser technique.

Performing stapes surgery for otosclerosis is known to be potentially irreversibly harmful to the inner ear function in about 1% of the cases. An early postoperative transient depression of the bone conduction thresholds is frequently detected after stapes surgery. The purpose of this study was to compare the evolution of bone conduction thresholds after primary stapedotomy with two different techniques: skeeter versus CO(2) laser stapedotomy. Audiological data of 336 otosclerosis operations performed by 2 surgeons between 1997 and 2003 were subjected to analysis. The calibrated hole in the footplate was performed randomly either with the skeeter drill or with the CO(2) laser. Preoperative bone conduction thresholds were compared with the postoperative levels (day 2-3, week 2, week 6 and month 6) in all patients. Evolution of the bone conduction was compared for the two studied subgroups (laser versus skeeter).

High resolution micro-CT scanning as an innovative tool for evaluation of the surgical positioning of cochlear implant electrodes.

X-ray microtomography (micro-CT) is a new technique allowing for visualization of the internal structure of opaque specimens with a quasi-histological quality. Among multiple potential applications, the use of this technique in otology is very promising. Micro-CT appears to be ideally suited for in vitro visualization of the inner ear tissues as well as for evaluation of the electrode damage and/or surgical insertion trauma during implantation of the cochlear implant electrodes. This technique can greatly aid in design and development of new cochlear implant electrodes and is applicable for temporal bone studies. The main advantage of micro-CT is the practically artefact-free preparation of the samples and the possibility of evaluation of the interesting parameters along the whole insertion depth of the electrode. This paper presents the results of the first application of micro-CT for visualization of the inner ear structures in human temporal bones and for evaluation of the surgical positioning of the cochlear implant electrodes relative to the intracochlear soft tissues.

Allograft tympanoplasty.

Tympano-ossicular allografts are human transplants of the tympanic membrane alone or with the ossicles included. The authors review its use in their department since the introduction of the technique 40 years ago by J. Marquet. The advantages of the technique (near normal anatomical and physiological reconstruction) are discussed, as well as its disadvantages (time consuming harvesting, risk of disease transmission). Some technical modifications for specific indications have been adapted in our department and are described in detail. The authors believe that the tympano-ossicular allograft is the material of choice in extensively diseased middle ears destroyed by chronic otitis media with or without cholesteatoma.

Basic fitting and evaluation parameters of a newly designed cochlear implant electrode.

OBJECTIVE: To validate a newly designed cochlear implant electrode (TRACE) in the standard monopolar mode and compare it to a patient group implanted with a standard Nucleus Contour cochlear implant electrode. The electrode contacts of the TRACE electrode have the same active surface area for stimulation, but the position in the scala tympani is different from that of the Nucleus Contour electrode. MATERIAL AND METHODS: The following parameters, used in cochlear implant fitting and evaluation procedures, were determined: the threshold and comfort stimulation current levels; the electrode impedances; and the phoneme discrimination and speech recognition scores using the ACE speech algorithm. CONCLUSION: The new electrode does not differ significantly from the standard Nucleus Contour electrode in terms of the investigated parameters within the test group.

Overview of safety imaging methods for newly designed cochlear implant electrodes.

Different individual test methods and protocols have been reported for the in-vitro evaluation of temporal bones implanted with newly designed cochlear implant electrodes, prior to human implantation. In practice, however, these methods may not always give the required information. In this study, a large battery of tests has been evaluated using the electrode as a fixed parameter. Standard clinical x-ray gave the best information to evaluate the electrode's position. Light microscopic evaluation and polishing technique studies proved to be the most valuable techniques to evaluate endocochlear damage.

Design, construction and mechanical optimisation process of electrode with radial current flow in the scala tympani.

A 48 contact cochlear implant electrode has been constructed for electrical stimulation of the auditory nerve. The stimulating contacts of this electrode are organised in two layers: 31 contacts on the upper surface directed towards the habenula perforata and 17 contacts connected together as one longitudinal contact on the underside. The design of the electrode carrier aims to make radial current flow possible in the cochlea. The mechanical structure of the newly designed electrode was optimised to obtain maximal insertion depth. Electrode insertion tests were performed in a transparent acrylic model of the human cochlea.

Results of 100 vestibular schwannoma operations.

Between 1991 and 2000, 154 cerebello-pontine angle (CPA) tumors were seen at the University ENT-department of the Sint-Augustinus Hospital, Antwerp. Amongst these, 127 were vestibular schwannomas detected by MR-imaging. Noteworthy is that in 5% of these, the ABR latencies were within normal limits. One hundred patients underwent tumor removal either by the translabyrinthine (66) or by the retrosigmoid (34) approach and all had a follow-up of at least two years. For large tumors (> 2 cm extension in the CPA) or in the case of poor hearing the translabyrinthine approach was used. For patients with tumor extension in the CPA ofless than 2 cm and with serviceable residual hearing on the affected side, (at least < 50 dB PTA, > 50% SSD) the retrosigmoid approach with endoscopic control was used. Most patients (96%) had a House-Brackmann grade 1 or 2 facial function before surgery. Although this group dropped to 76% 6 months after surgery it increased again to reach 84% within 2 years. Thus, 88% percent of patients with normal preoperative facial function achieved a Grade I or II after two years. The facial outcome is very much dependent on the size of the tumor. A good result (House-Brackmann Grade 1 or 2) is the rule (92%) for small tumors (< 10 mm extension in the CPA), still attainable (82%) for medium tumors (11-25 mm), but less apparent (56%) for large tumors (> 26 mm). It was possible to preserve hearing in 38% of the retrosigmoid interventions. Although unbalance and headache are rather frequent early postoperative symptoms (respectively 52% and 31%), these complaints decrease with time and are infrequent after two years (unbalance = 7%, headache = 4%). Our results were compared with three large multicentric studies. They are in line with data from the literature and compare favorably with the better results. Although good grading systems exist for facial nerve and hearing outcomes, the authors regret that a general consensus on tumor size measurement is still not yet available. It would facilitate data comparison between different centers and the choice between the therapeutical modalities.

Middle ear status in ears showing high admittance tympanograms. Clinical value of the use of the two-component 678 Hz oto-admittance tympanogram.

The present paper reports on the results of a retrospective study using 678 Hz susceptance-conductance tympanometry performed on abnormally high admittant middle ears. High admittance was proven to be caused by (partial) disruption or lysis of the ossicular chain. Retrospective analysis shows that 678 Hz tympanometry seems to be more accurate in the differential diagnosis of ossicular chain disruptions compared to CT-imaging of the ossicular chain (88% versus 81% correct identification respectively). Classic tympanometry using a probe frequency of 226 Hz was abnormal in only 43% of the cases.

Otosclerosis: a genetically heterogeneous disease involving at least three different genes.

Otosclerosis is caused by abnormal bone homeostasis of the otic capsule, resulting in hearing impairment in 0.3%-0.4% of the white population. The etiology of the disease remains unclear and environmental as well as genetic factors have been implicated. We localized the first autosomal-dominant locus to chromosome 15 in 1998 (OTSC1) in an Indian family and, recently, we reported the localization of a second gene for otosclerosis to a 16 cM interval on chromosome 7q (OTSC2). In this study, we recruited and analyzed nine additional families (seven Belgian and two Dutch families with 53 affected and 20 unaffected subjects) to investigate the importance of these loci in autosomal-dominant otosclerosis. We completed linkage analysis with three microsatellite markers of chromosome 15 (D15S652, D15S1004, D15S657) and five microsatellite markers of chromosome 7 (D7S495, D7S2560, D7S684, D7S2513, D7S2426). In two families, results compatible with linkage to OTSC2 were found, but in the seven remaining families OTSC1 and OTSC2 were excluded. Heterogeneity testing provided significant evidence for genetic heterogeneity, with an estimated 25% of families linked to OTSC2. These results indicate that, besides OTSC1 and OTSC2, there must be at least one additional otosclerosis locus.

A Two-stage bipodal screening model for universal neonatal hearing screening.

OBJECTIVE: A model is proposed for universal neonatal hearing screening. METHODS: The screening model is two-staged because it consists of a first test and, in case of failure (1.4% of the subjects), of a retest 3 weeks later. It is bipodal because it involves both the hospital audiologic department and a central Well Baby Organization. The idea is to have a maximal number of newborns tested at the maternity by trained audiologists and to have the Well Baby Organization trace and chase the missing subjects. The model has been evaluated during 1 calendar year (1999) in a maternity with 2,012 newborns. RESULT: The result is a coverage of 99.3%. Most newborns (97.3%) were tested at the maternity ward with a total time investment of less than 15 minutes per child. The actual test time is 2 minutes, 12 seconds (median value). The Well Baby Organization keeps track of all the results and has to test no more than 2% of the newborns. Sensitivity and specificity were not the primary outcomes of this evaluation, but they were similar to those of a previous study evaluating the screen procedure on a larger scale, giving a sensitivity of approximately 100% and a false alarm rate of 1/1,000. CONCLUSION: These figures demonstrate that universal neonatal hearing screening is feasible within the existing health care structure, with unprecedented coverage, sensitivity, and specificity.

Contralateral suppression of transient evoked otoacoustic emissions: normative data for a clinical test set-up.

HYPOTHESIS: Contralateral suppression of transient evoked otoacoustic emissions (TEOAEs) can be used in a clinical set-up using a procedure based on a unique and robust parameter to quantify the magnitude of suppression for a subject. BACKGROUND: TEOAEs can be suppressed by delivering contralateral white noise (WN). This suppression is thought to be mediated via the efferent nerve fibers that innervate the outer hair cells. The ipsilateral TEOAE-eliciting click stimulus level and the contralateral WN level have a strong impact on the recorded level of suppression. METHODS: TEOAEs were recorded using the nonlinear stimulation mode in two conditions (with and without contralateral WN). An optimal TEOAE-eliciting click stimulus level and contralateral WN level were defined to obtain a unique and robust parameter to quantify the magnitude of suppression. RESULTS: Suppression of TEOAEs with contralateral WN can be measured in a clinical set-up using nonlinear stimulation, and the level of suppression is of the same order of magnitude as measures using the linear stimulation recording mode. The level of suppression appears to be "locked" to the interaural difference between ipsilateral TEOAE-eliciting broadband click stimulus level and the contralateral WN level. CONCLUSIONS: A procedure is proposed to record contralateral suppression in a clinical set-up, and normative data are given for a normal-hearing population (n = 60).

A second gene for otosclerosis, OTSC2, maps to chromosome 7q34-36.

Otosclerosis due to abnormal bone homeostasis of the otic capsule is a frequent cause of hearing loss in adults. Usually, the hearing loss is conductive, resulting from fixation of the stapedial footplate, which prevents normal ossicular vibration in response to sound. An additional type of sensorineural hearing loss may be caused by otosclerotic damage to the cochlea. The etiology of the disease is unknown, and both environmental and genetic factors have been implicated. Autosomal dominant inheritance with reduced penetrance has been proposed, but large families are extremely rare. To elucidate the pathogenesis of the disease, identification of the responsible genes is essential. In this study, we completed linkage analysis in a Belgian family in which otosclerosis segregates as an autosomal dominant disease. After excluding linkage to a known locus on chromosome 15 (OTSC1), we found linkage on chromosome 7q, with a multipoint LOD score of 3.54. Analysis of key recombinant individuals maps this otosclerosis locus (OTSC2) to a 16-cM interval on chromosome 7q34-36 between markers D7S495 and D7S2426.

Neonatal hearing screening with transient evoked otoacoustic emissions: a learning curve.

The present paper reports on the implementation of a neonatal hearing screening programme in a private hospital in Belgium. A maternity-based neonatal hearing screening project with transient evoked otoacoustic emissions (TEOAEs) was started in 1993. The cost of the test was not covered by the public health insurance, so the parents had to pay the full cost for screening their child (approximately 30 Euro). Since 1993 the programme strategies have been changed on several occasions to improve the quality and efficacy. A retrospective analysis was performed on: (1) the test pass rate; (2) the coverage; and (3) the number of children who become 'Lost to follow-up' after failing the initial test. The data show a steady learning curve with a time course of several years. They also demonstrate that it is worthwhile and feasible to run a high-quality screening programme in a private establishment.

Audiological findings in large vestibular aqueduct syndrome.

An enlarged vestibular aqueduct is a congenital disorder causing early onset and progressive hearing loss in children. This paper presents the audiological findings at first presentation and the audiological evolution in 10 consecutive cases presenting with hearing loss and showing a large vestibular aqueduct on imaging. The reported onset of the hearing loss is within the first few years of life. Most of the cases (80%) showed bilateral involvement. The sex ratio was 1. Patients presented on average at age 5 with a median hearing loss of 62 dB at the speech frequencies. The hearing loss was essentially asymmetrical with an interaural difference, of 33 dB and it was a mixed type of hearing loss in 90% of the cases. The authors claim that the conductive component of this hearing loss is a pure cochlear conductive loss which may be pathognomonic for the disease. The presence of a conductive component in a child is easily misinterpreted as a middle ear ventilation problem or in case of good ventilation as an ossicular problem (type otosclerosis). In addition and in contrast to most literature data, the authors did not find evidence for stabilization of the hearing loss but they found a steady decrease of the hearing at an average rate of 4 dB/year.

Tympano-ossicular allografts and HIV transmission.

OBJECTIVE: To evaluate the potential risk of human immunodeficiency virus (HIV) transmission by tympano-ossicular allografts by studying the efficacy of standard preservation techniques to eliminate the presence of proviral HIV-1 DNA fragments in contaminated ossicles. STUDY DESIGN: Randomized single-blind prospective study on the ossicles of HIV-1 patients. MATERIAL: Ossicles of five patients who had died of acquired immune deficiency syndrome (AIDS) (HIV-1 infection) were taken within 6 hours postmortem and allocated randomly to a treatment and nontreatment group. Liver and skin biopsies were taken as positive control specimens. PROCESSING: The treatment group was processed with standard techniques (formaldehyde) for tympano-ossicular allograft preservation and the nontreatment group was only washed, dried, and stored in sterile tubes at -700 degrees without further processing. MAIN OUTCOME MEASURE: Proviral HIV-1 DNA was detected using polymerase chain reaction amplification techniques. RESULTS: No proviral HIV-1 DNA was detected in any of the treated ossicles, whereas three of five sets of untreated ossicles were positive. The positive control specimens of all treated and nontreated sets were positive for proviral HIV-1 DNA. CONCLUSIONS: These results suggest that the preservation technique for tympano-ossicular allografts is safe with regard to HIV-1 transmission.

A new autosomal-dominant locus (DFNA12) is responsible for a nonsyndromic, midfrequency, prelingual and nonprogressive sensorineural hearing loss.

OBJECTIVE: This study aimed to report on the audiologic findings of a nonsyndromic autosomal-dominant hearing loss of which the gene (DFNA 12) recently was found to map to chromosome 11q22-24. The study also aimed to propose and evaluate an algorithm based on the audiometric findings to discriminate between affected and unaffected family members before genetic linkage analysis. STUDY DESIGN: The study design was a retrospective analysis of the audiometric data of genetically affected and unaffected patients. SETTING: The study was conducted at a tertiary referral center. PATIENTS: A total of 17 genetically affected and 54 unaffected family members were studied. INTERVENTIONS: Pure-tone audiometry with air and bone conduction and construction and evaluation of an algorithm were performed. MAIN OUTCOME MEASURES: The type and degree of hearing loss as compared to age and gender-dependent values according to the International Organization for Standardization 7029 standard were measured. For this comparison, the variable "hearing standard deviations" (HSD) is introduced and is defined as the number of standard deviations that a hearing threshold is lying above the age and gender-related median at the given frequency. A description of the algorithm and an evaluation in terms of alpha- and beta-error also were measured. RESULTS: The hearing loss is nonsyndromic, sensorineural, moderate-to-moderately severe (pure-tone average, 51 dB at age 18 years), with an early onset (probably prelingual) and no progression. It affects all frequencies but mainly the midfrequencies (500, 1,000, and 2,000 Hz). The algorithm consists of an analysis of variance to determine the frequency that is most sensitive for the genetic trait under study and on the ranking of the family members according to their hearing loss (HSD) at this frequency. Individual persons are labeled as "affected" or "unaffected" according to this ranking.

The LAURA Pediatric Cochlear Implant Program in Antwerp: what have we learnt in 5 years?

The LAURA Cochlear Implant Program at the University of Antwerp was initiated 5 years ago. Up to now 50 children have received the LAURA cochlear implant (Philips Hearing Implants). Our accumulated experience in selecting the patients, counselling their parents, implanting and fitting the device allowed us to gradually modify the inclusion/exclusion criteria for cochlear implantation and achieve very rewarding results. The most important change was the gradual shift towards a younger age at implantation, our youngest implantee being 11 months old. In this paper we describe the rationale for this change, as well as the prerequisites for a successful pediatric cochlear implant program.