Piezoelectric Bone Conduction Hearing Implant: A Case Series of Audiological, Surgical and Patient-Reported Outcomes.

Objective: To examine the surgical, audiological and patient-reported outcomes of the Osia 2 implant. Methods: Data from 14 consecutive subjects undergoing implantation between April 2022 and November 2023 were reviewed. Ten subjects had conductive hearing loss, three had mixed hearing loss and one had single-sided deafness (SSD). Warble tone thresholds, Pure Tone Average (PTA4) and Speech Discrimination Score (SDS) in quiet and in noise were determined unaided and aided. The subjective outcome was determined from two standardized questionnaires: (1) International Outcome Inventory for Hearing Aids (IOI-HA) and (2) Speech, Spatial and Qualities of Hearing Scale 12 (SSQ12b). Results: Unexpected postoperative pain was found in four cases. The warble tone thresholds exhibited a consistent reduction across all frequencies, contributing to a mean decrease of 27 dB in the aided PTA4. SDS demonstrated notable improvements, with a 57.3% increase at 50 dB and a 55.6% increase at 65 dB. In noise, SDS exhibited a 43.9% improvement. The mean IOI-HA Score was 3.8, and the mean overall score for SSQ12b was 6.6, with consistent findings across the subgroups. Conclusions: The Osia device emerges as a promising recommendation for individuals with conductive or mixed hearing loss, possibly also for those with SSD. Its safety and efficacy profile aligns with the broader category of active transcutaneous devices, demonstrating a reduced risk of wound infection compared to percutaneous alternatives. Both audiological assessments and subjective evaluations revealed positive outcomes.

Electrocochleographic frequency-following responses as a potential marker of age-related cochlear neural degeneration.

Auditory nerve (AN) fibers that innervate inner hair cells in the cochlea degenerate with advancing age. It has been proposed that age-related reductions in brainstem frequency-following responses (FFR) to the carrier of low-frequency, high-intensity pure tones may partially reflect this neural loss in the cochlea (Märcher-Rørsted et al., 2022). If the loss of AN fibers is the primary factor contributing to age-related changes in the brainstem FFR, then the FFR could serve as an indicator of cochlear neural degeneration. In this study, we employed electrocochleography (ECochG) to investigate the effects of age on frequency-following neurophonic potentials, i.e., neural responses phase-locked to the carrier frequency of the tone stimulus. We compared these findings to the brainstem-generated FFRs obtained simultaneously using the same stimulation. We conducted recordings in young and older individuals with normal hearing. Responses to pure tones (250 ms, 516 and 1086 Hz, 85 dB SPL) and clicks were recorded using both ECochG at the tympanic membrane and traditional scalp electroencephalographic (EEG) recordings of the FFR. Distortion product otoacoustic emissions (DPOAE) were also collected. In the ECochG recordings, sustained AN neurophonic (ANN) responses to tonal stimulation, as well as the click-evoked compound action potential (CAP) of the AN, were significantly reduced in the older listeners compared to young controls, despite normal audiometric thresholds. In the EEG recordings, brainstem FFRs to the same tone stimulation were also diminished in the older participants. Unlike the reduced AN CAP response, the transient-evoked wave-V remained unaffected. These findings could indicate that a decreased number of AN fibers contributes to the response in the older participants. The results suggest that the scalp-recorded FFR, as opposed to the clinical standard wave-V of the auditory brainstem response, may serve as a more reliable indicator of age-related cochlear neural degeneration.

Cochlear Implantation in Sporadic Intralabyrinthine Schwannomas with Single-Sided Deafness: Implications for Binaural Hearing.

BACKGROUND AND OBJECTIVE: Intralabyrinthine schwannomas (ILSs) may have detrimental effects on ipsilateral hearing, commonly leading to single-sided deafness (SSD). Cochlear implantation in patients with ILSs is an option to partly restore ipsilateral hearing; however, the available literature fails to account for the binaural hearing benefits of a cochlear implant (CI) for ILSs. METHODS: We prospectively evaluated SSD patients with sporadic ILS undergoing cochlear implantation with simultaneous tumor resection (n = 10) or with tumor observation (n = 1). Patients completed the Speech, Spatial and Qualities Questionnaire (SSQ12) and the Nijmegen Cochlear Implant Questionnaire (NCIQ) pre- and postoperatively, as well as the Bern Benefit in Single-Sided Deafness (BBSSD) questionnaire postoperatively. Patients were also tested postoperatively with and without their CI to measure the effect on localization ability and binaural summation, squelch, and head shadow. RESULTS: Evaluation was completed for nine and six patients (subjective and objective data, respectively). The CI significantly improved the speech reception threshold (SRT) in the head shadow condition where the target signal was presented to the CI side and the noise to the front (SCIN0). On the other hand, the SRTs in the colocated condition (S0N0) and the condition where the target signal was presented to the front and the noise to the CI side (S0NCI) were unaffected by the CI. The mean localization error decreased significantly from 102° to 61° (p = 0.0031) with the addition of a CI. The scores from SSQ12 demonstrated nonsignificant changes. For NCIQ, the self-esteem and the social interaction domains increased significantly but insignificantly for the remaining domains. The BBSSD responses ranged from +0.5 to +3.5 points. CONCLUSION: After implantation, patients achieved significantly better scores across some of the patient-reported and objective parameters. In addition to reporting on a number of ILS cases where implantation was performed, the study is the first of its kind to document patient-reported and objective binaural hearing improvement after cochlear implantation in patients with ILS and, thereby, lends support to the active management of ILS.

Tinnitus suppression in a prospective cohort of 45 cochlear implant recipients: occurrence, degree and correlates.

OBJECTIVE: To determine tinnitus prevalence and severity in a cohort of unselected first-time cochlear implant (CI) recipients whose primary motive for CI was sensorineural hearing loss (SNHL), and to evaluate the effect of CI on tinnitus after cochlear implantation. METHODS: Prospective longitudinal study of 45 adult CI recipients with moderate to profound SNHL. Patients completed the Danish version of the Tinnitus Handicap Inventory (THI) and a visual analogue scale (VAS) for tinnitus burden before implantation, 4 months after implantation and 14 months after implantation. RESULTS: The study included 45 patients, of which 29 (64%) had pre-implant tinnitus. Median THI score (IQR) significantly decreased from 20 (34) to 12 (24) at first follow-up (p < 0.05) and to 6 points (17) at second follow-up (p < 0.001). Median VAS (IQR) for tinnitus burden decreased from 33 (62) to 17 (40; p = 0.228) and 12 (27, p < 0.05) at the first and second follow-ups, respectively. Tinnitus was totally suppressed in 19% of patients, improved in 48%, remained unchanged in 19% and worsened in 6%. 2 patients reported new tinnitus. At the second follow-up, 74% of patients had slight or no tinnitus handicap, 16% had mild handicaps, 6% had moderate handicaps, and 3% had severe handicaps. High pre-implant THI and VAS scores correlated with greater decrease in THI scores over time. CONCLUSION: 64% of the patients with SNHL had pre-implant tinnitus, which was decreased 4 and 14 months after implantation. Overall, 68% of patients with tinnitus improved their tinnitus handicap after CI. Patients with higher THI and VAS scores had a larger decline and the highest benefits in terms of tinnitus handicap improvement. The study findings demonstrate that the majority of patients with moderate to profound SNHL eligible for cochlear implantation benefit from complete or partial tinnitus suppression and improved quality of life after implantation.

Cochlear Implantation Improves Both Speech Perception and Patient-Reported Outcomes: A Prospective Follow-Up Study of Treatment Benefits among Adult Cochlear Implant Recipients.

Cochlear implantation is considered the best treatment option for patients with severe-to-profound sensorineural hearing loss for whom conventional hearing aids are insufficient. We used a repeated measures longitudinal approach to evaluate speech recognition and patient-reported outcomes after cochlear implantation in an unbiased cohort of Danish adult patients in a prospective cohort study. We assessed 39 recipients before and two times after implantation using a battery of tests that included Dantale I, the Danish Hearing in Noise Test, the Nijmegen Cochlear Implant Questionnaire, and the Speech, Spatial, and Qualities of Hearing Scale. The study group improved significantly on all outcome measures following implantation. On average, Dantale I scores improved by 29 percentage points and Hearing in Noise Test scores improved by 22 percentage points. Most notably, the average Dantale score improved from 26 to 70% in the CI in quiet condition and from 12 to 42% in the cochlear implantation in noise condition when tested monaurally. Dantale demonstrated a significant positive correlation with Nijmegen Cochlear Implant Questionnaire and Speech, Spatial, and Qualities of Hearing Scale scores, while Hearing in Noise Test had no significant correlation with the patient-reported outcome measures. Patients improved significantly at 4 months and marginally improved further at 14 months, indicating that they were approaching a plateau. Our study's use of audiometric and patient-reported outcome measures provides evidence of the treatment benefits of cochlear implantation in adults, which may help physicians advise patients on treatment decisions and align treatment benefit expectations, as well as serve as a foundation for the development of new cochlear implantation selection criteria.

Objective Vestibular Test Battery and Patient Reported Outcomes in Cochlear Implant Recipients.

OBJECTIVE: Cochlear implantation (CI) may have undesired effects on the vestibular apparatus. However, the literature holds no consensus on vestibular affection and the testing tools applied to test for vestibular dysfunction after cochlear implantation are inconsistent. We aimed to investigate the impact of CI on vestibular function by an extensive test battery including patient-reported outcomes. STUDY DESIGN: Prospective observational study. SETTING: University hospital. PATIENTS: Forty adult unilateral first-time CI recipients. INTERVENTION: Vestibular function was evaluated pre- and post-implantation with the video head impulse test (VHIT), the caloric test and cervical vestibular evoked myogenic potentials (cVEMPs), and the patient-reported dizziness handicap inventory (DHI). RESULTS: Mean VHIT gain decreased from preoperative 0.92 to 0.84 postoperative (p = 0.018); mean caloric unilateral weakness increased from 20.5% preoperative to 42.9% postoperative (p < 0.0001); cVEMP responses were present on 10 operated ears preoperative and five ears postoperative, and compared with non-implanted ears, cVEMP responses on implanted ears were impaired (p = 0.023). 50% of patients reported early postoperative dizziness, but the mean DHI score remained unchanged (p = 0.94). The DHI scores correlated poorly with the objective outcomes (rs = 0.19 and rs = -0.22). CONCLUSION: Vestibular function is significantly affected after cochlear implantation, but vestibular hypofunction varies with the test used. Although early dizziness after implantation is common, later DHI scores are not significantly higher than before the implantation, indicating that central compensation plays a major role for these patients.

[Auditory brainstem implantation].

In this review, we discuss the auditory brainstem implant (ABI), which is a neuroprosthetic device being an advanced hearing aid in cases of bilateral, profound or complete hearing impairment due to a non-functional or absent cochlear nerve, or an inner ear malformation precluding cochlear implantation. Originally indicated in patients with bilateral vestibular schwannomas caused by neurofibromatosis type 2, the ABI has in recent years seen an increase in the aural rehabilitation of congenitally deaf children. Outcomes for patients are mixed, but generally the device leads to a reasonable improvement in speech perception.

[Cochlear implantation for adults].

A cochlear implant is an electrode, which is implanted in the inner ear to establish or re-establish hearing. Based on estimates on the prevalence of undertreated bilateral profound hearing loss, the number of adult people receiving a cochlear implant is low. In addition, many people with residual acoustic hearing or people with single-sided deafness could benefit from a cochlear implant. Due to the recent expansions in the indications for cochlear implantation and the unmet need for hearing improvement among the adult population, more focus on referring these patients to audiological cochlear implantation workup is recommended, as argued in this review.

Nordic results of cochlear implantation in adults: speech perception and patient reported outcomes.

BACKGROUND: Although cochlear implantation (CI) is widely performed in postlingually hearing-impaired adults in the Nordic countries, the literature on hearing outcomes remains scarce. Aims/objectives: To evaluate and correlate hearing outcomes after implantation. METHODS: Prospective evaluation of 40 adult first-implantation recipients pre- and post-implantation with Dantale, the Danish Hearing in Noise Test (HINT), the Nijmegen Cochlear Implant Questionnaire (NCIQ) and the Speech, Spatial and Qualities of Hearing Scale (SSQ12). RESULTS: Dantalemean increased from 50% (95% CI [41.7,58.4])) to 73% (95% CI [66.6,80.1]) and 19% (95% CI [13.3,25.1]) to 40%(95% CI [32.1,46.7]) (quiet/noise). HINTwords increased from 71% (95% CI [58.8,83.8]) to 83% (95% CI [69.8,95.4]) and 59% (95% CI [46.2,70.6]) to 73% (95% CI [63.3,82.8]) (quiet/noise). NCIQmean changed from 277 (95% CI [252.4,301.6]) to 396 (95% CI [366.7,424.7]) and SSQ12mean from 27 (95% CI [21.3,31.8]) to 48 (95% CI [39.4,55.6]). Correlations coefficients were r s = 0.39-0.74 (p < .01) between Dantale/HINT, rs = 0.78 (p < .0001) between NCIQ/SSQ12, r s = 0.41-0.59 (p < .01) between NCIQ/SSQ12 and Dantale and r s = 0.24-0.41 (ns) between NCIQ/SSQ12 and HINT. CONCLUSION: The study documents a high auditory performance and patient-perceived improvement after implantation and advocate broader awareness of implantation as treatment option in the growing population of severe-to-profound hearing-impaired adults. Correlations between hearing measures may influence future evaluation practices.

Association of SLC26A4 mutations, morphology, and hearing in pendred syndrome and NSEVA.

OBJECTIVE: To investigate the relations of monoallelic (M1), biallelic (M2), or the absence of mutations (M0) in SLC26A4 to inner ear morphology and hearing levels in individuals with Pendred syndrome (PS) or nonsyndromic enlarged vestibular aqueduct (NSEVA) associated with hearing loss. METHODS: In a cohort of 139 PS/NSEVA individuals, 115 persons from 95 unrelated families had full genetic sequencing of SLC26A4, and 113 had retrievable images for re-assessment of inner ear morphology. The association between the number of mutant alleles in SLC26A4, inner ear morphology (including endolymphatic sac size and protein content on magnetic resonance imaging), and hearing level (pure tone average) was explored. RESULTS: Biallelic SLC26A4 mutations (M2) occurred in three-quarters of the cohort and was invariably associated with poor hearing; in 87%, it was associated with incomplete partition type II of the cochlea as well as enlarged endolymphatic sac and vestibular aqueduct. M1 or M0 individuals exhibited a greater variability in inner ear morphology. Endolymphatic sac size and presence of "high-protein" sac contents were significantly higher in M2 individuals compared to M1 and M0 individuals. CONCLUSION: The number of SLC26A4 mutations is associated with severity and variability of inner ear morphology and hearing level in individuals with PS or NSEVA. M2 individuals have poorer hearing and present largely incomplete partition type II of the cochleas with enlarged endolymphatic sacs, whereas individuals with M1 and no detectable SLC26A4 mutations have less severe hearing loss and more diverse inner ear morphology. LEVEL OF EVIDENCE: 4. Laryngoscope, 129:2574-2579, 2019.

The Natural History of Hearing Loss in Pendred Syndrome and Non-Syndromic Enlarged Vestibular Aqueduct.

INTRODUCTION: The aim was to investigate the progress of hearing loss over time in a cohort of pendred syndrome and non-syndromic enlarged vestibular aqueduct (PS/NSEVA) with one or two confirmed pathogenic variations in SLC26A4. STUDY DESIGN: Retrospective cohort study. SUBJECTS AND METHODS: At our tertiary referral center, a retrospective search of all patients with enlarged vestibular aqueduct, hearing loss and SLC26A4 mutations yielded 103 individuals by March 2017, 96 of whom had records of hearing levels; both an early audiometry and the latest between 3 and 668 months follow-up. Pure-tone average (PTA; average of thresholds at 0.5, 1, 2 and 4 kHz) was calculated for both ears at time 1 and time 2. Neonatal screening results were retrieved. RESULTS: Eighty-seven (87) individuals had biallelic (M2) and 16 had monoallelic alterations (M1) in their SLC26A4. On average, the PTA progressed to 80 dB HL by the age of 6 years for the entire cohort, and 3.2 years for the biallelic (M2) affected individuals. 25% of the cohort was screened in the neonatal screening program; of these 42% had "passed" at least monaurally. Audiometric profiles related to age show faster deterioration in high frequencies than in low frequencies. CONCLUSION: In patients with PS/NSEVA and SLC26A4 mutations, the average hearing loss progresses to 80 dB HL by the age of 6 years. For biallelic (M2) affected individuals it was 3.2 years. Although hearing levels reached severe to profound during childhood, almost 1/2 had passed neonatal hearing screening, at least monaurally, emphasizing the need for close follow-up.

Otoacoustic Emissions for Outcome Prediction in Postanoxic Brain Injury.

Background: Non-invasive, easy-to-use bedside tools to estimate prognosis in unresponsive patients with postanoxic brain injury are needed. We assessed the usefulness of otoacoustic emissions as outcome markers after cardiac arrest. Methods: Distortion product otoacoustic emissions (DPOAE) and transient evoked otoacoustic emissions (TEOAE) were measured in cardiac arrest patients whose prognosis was deemed to be poor following standard neurological assessment (n = 10). Ten patients with myocardial infarction without prior loss of consciousness served as controls. Results: Compared to controls with myocardial infarction, cardiac arrest patients with poor neurological prognosis had significantly less often preserved DPOAE (9.2 vs. 40.8% positive measurements; OR 0.15 (CI 0.07-0.30); p < 0.0001). Partially preserved DPOAE were noted in 4 cardiac arrest patients. TEOAE were not statistically different between the two groups. Conclusions: Despite their convenience, otoacoustic emissions cannot be used as reliable prognostic markers in cardiac arrest survivors. This is because we identified 4 cases with partially preserved otoacoustic emissions in a sample of 10 unresponsive post-cardiac arrest patients whose neurological condition was so poor that active treatment was withdrawn. However, we suggest that future research should address if decaying outer hair cell function over time may serve as a proxy for evolving ischemic brain damage.

[Cochlear implantation in children].

Children with severe hearing impairment or deafness are preferably recognised and treated with a cochlear implant (CI) before the age of one year, as early stimulation of the auditory sense is essential for the development of spoken language. Today, children with deafness are offered a bilateral CI, as it allows for improved speech perception in noise and sound localisation. However, the indications for cochlear implantation have been extended to include children with asymmetric hearing loss, in order to avoid the development of aural preference syndrome, which may limit the effect of a CI later.

Correction to: The CAPOS mutation in ATP1A3 alters Na/K-ATPase function and results in auditory neuropathy which has implications for management.

The following information was inadvertently omitted in the original publication.

The CAPOS mutation in ATP1A3 alters Na/K-ATPase function and results in auditory neuropathy which has implications for management.

Cerebellar ataxia, areflexia, pes cavus, optic atrophy and sensorineural hearing impairment (CAPOS) is a rare clinically distinct syndrome caused by a single dominant missense mutation, c.2452G>A, p.Glu818Lys, in ATP1A3, encoding the neuron-specific alpha subunit of the Na+/K+-ATPase α3. Allelic mutations cause the neurological diseases rapid dystonia Parkinsonism and alternating hemiplegia of childhood, disorders which do not encompass hearing or visual impairment. We present detailed clinical phenotypic information in 18 genetically confirmed patients from 11 families (10 previously unreported) from Denmark, Sweden, UK and Germany indicating a specific type of hearing impairment-auditory neuropathy (AN). All patients were clinically suspected of CAPOS and had hearing problems. In this retrospective analysis of audiological data, we show for the first time that cochlear outer hair cell activity was preserved as shown by the presence of otoacoustic emissions and cochlear microphonic potentials, but the auditory brainstem responses were grossly abnormal, likely reflecting neural dyssynchrony. Poor speech perception was observed, especially in noise, which was beyond the hearing level obtained in the pure tone audiograms in several of the patients presented here. Molecular modelling and in vitro electrophysiological studies of the specific CAPOS mutation were performed. Heterologous expression studies of α3 with the p.Glu818Lys mutation affects sodium binding to, and release from, the sodium-specific site in the pump, the third ion-binding site. Molecular dynamics simulations confirm that the structure of the C-terminal region is affected. In conclusion, we demonstrate for the first time evidence for auditory neuropathy in CAPOS syndrome, which may reflect impaired propagation of electrical impulses along the spiral ganglion neurons. This has implications for diagnosis and patient management. Auditory neuropathy is difficult to treat with conventional hearing aids, but preliminary improvement in speech perception in some patients suggests that cochlear implantation may be effective in CAPOS patients.

Cochlear implantation in Pendred syndrome and non-syndromic enlarged vestibular aqueduct - clinical challenges, surgical results, and complications.

OBJECTIVE: To explore specific clinical issues, surgical results, and complications of 80 cochlear implantations (CI) in 55 patients with Pendred syndrome (PS) or non-syndromic enlarged vestibular aqueduct (NSEVA). BACKGROUND: Previous studies have focused either on unselected case series or on populations with mixed cochlear malformations. PS/NSEVA accounts for up to 10% of congenital SNHL, rendering this a large group of cochlear implant candidates. The abnormal inner ear anatomy of these patients may be associated with a lower surgical success rate and a higher rate of complications. STUDY DESIGN: Retrospective review of patients' medical records and CT/MRI. SETTING: Tertiary referral center. MATERIALS AND METHODS: The medical records and CT/MRI images of 55 PS/NSEVA patients receiving 80 cochlear implantations from 1982-2014 were reviewed. Demographic data, surgical results, intra-operative incidents, and post-operative complications were retrieved. RESULTS: Complications occurred in 36% of implantations; 5% hereof major complications. Gushing/oozing from the cochleostoma occurred in 10% of implantations and was related to transient, but not prolonged post-operative vertigo. CONCLUSION: Intra-operative risks of gushing/oozing and post-operative vertigo are the primary clinical issues in PS/NSEVA patients regarding CI. Nonetheless, the surgical success rate is high and the major complication rate is low; similar to studies of unselected series of CI recipients.

Objective and Subjective Outcome of a New Transcutaneous Bone Conduction Hearing Device: Half-year Follow-up of the First 12 Nordic Implantations.

OBJECTIVE: To examine the objective and subjective outcome of a new transcutaneous bone conduction hearing device. STUDY DESIGN: Prospective, consecutive case series. PATIENTS: Twelve patients were implanted. Eight patients had a conductive/mixed (con/mix) hearing loss. Four had single sided deafness. MAIN OUTCOME MEASURES: At half-year follow-up, aided and unaided sound field hearing was evaluated by 1) warble tone thresholds, 2) pure-tone average (PTA4), 3) speech discrimination score (SDS) in quiet, and 4) speech reception threshold 50% at 70 dB SPL noise level (SRT50%). Subjective outcome was evaluated by three questionnaires: 1) International Outcome Inventory for Hearing Aids, 2) Speech, Spatial and Qualities of Hearing Scale 12, and 3) a questionnaire on frequency and duration of use. RESULTS: No major complications occurred. The mean aided PTA4 was lowered by 23dB. SDS was increased by 40% at 50dB, by 34% at 65dB, and by 12% at 80 dB SPL. SRT50% in noise improved 5.2 dB. 58% of the patients used the device daily and 83% at least 5 days a week. 50% used the device ≥ 8 hours and 75% ≥ 4  hours a day. Mean International Outcome Inventory for Hearing Aids score was 3.7, corresponding to beneficial outcome. In Speech, Spatial and Qualities of Hearing Scale 12, "quality of hearing" scored especially high. The con/mix hearing loss group showed larger benefit especially in SDS, SRT50% in noise and the subjective evaluations, whereas frequency and duration of use were similar. CONCLUSION: This study on the first 12 Nordic patients implanted with a new transcutaneous bone conduction hearing device demonstrates significant objective, as well as subjective hearing benefit. Patient satisfaction was high, as was the frequency of use.

Psychometric properties of a revised Danish translation of the international outcome inventory for hearing aids (IOI-HA).

OBJECTIVE: The original Danish translation of the international outcome inventory for hearing aids (IOI-HA) proved problematic as the wording of item 5 was not semantically clear, rendering the questionnaire internally inconsistent. The objective of this study was to examine data collected with a revised Danish translation of the IOI-HA in order to: (1) evaluate the effect of the revision, and (2) to examine if the psychometric properties of the revised translation of the IOI-HA are equivalent to those of previously validated translations. DESIGN: Psychometric properties were evaluated performing inter-item correlation analysis, principal component analysis, and item-total correlation. STUDY SAMPLE: Three hundred forty-one adult hearing-impaired participants-all of whom were voluntary hearing aid testers attached to the Global Audiology Group in GN ReSound A/S on a non-payment basis - were mailed a revised Danish IOI-HA questionnaire. RESULTS: Statistical analysis revealed good internal consistency along with a clear division of items into two distinct factors. CONCLUSIONS: The revised Danish translation of the IOI-HA proves internally consistent. Furthermore, it possesses psychometric properties equivalent to those reported in several corresponding studies of other translations. Data obtained from it can therefore validly be considered comparable to data obtained from previously validated translations of the IOI-HA.

Experience with cochlear implants in Greenlanders with profound hearing loss living in Greenland.

OBJECTIVE: Cochlear implant (CI) treatment was introduced to the world in the 1980s and has become a routine treatment for congenital or acquired severe-to-profound hearing loss. CI treatment requires access to a highly skilled team of ear, nose and throat specialists, audiologists and speech-language pathologists for evaluation, surgery and rehabilitation. In particular, children treated with CI are in need of long-term post-operative auditory training and other follow-up support. DESIGN: The study is retrospective with updated information on present performance. RESULTS: Since 2001, a total of 11 Greenlandic patients living in Greenland have been treated with CI, 7 children and 4 adults. Of these children, 4 use oral communication only and are full-time CI-users, 2 with full-time use of CI are still in progress with use of oral communication, and 1 has not acquired oral language yet, but has started auditory and speech training. Six children attend mainstream public school while one child is in kindergarten. Of the adults, only 1 has achieved good speech perception with full-time use of CI while 3 do not use the CI. DISCUSSION: From an epidemiological point of view, approximately 1-3 children below 6 years are in need of a CI every second year in Greenland often due to sequelae from meningitis, which may cause postinfectious deafness. Screening of new-borns for hearing has been started in Greenland establishing the basis for early diagnosis of congenital hearing impairment and subsequent intervention. The logistics and lack of availability of speech therapists in Greenland hampers possibilities for optimal language and speech therapy of CI patients in Greenland. This study aims at describing the results of CI treatment in Greenlanders and the outcome of the CI operations along with the auditory and speech/language outcomes. Finally, we present a suggestion for the future CI treatment and recommendations for an increased effort in the treatment and rehabilitation of implanted patients in Greenland.

Familial aggregation of pure tone hearing thresholds in an aging European population.

OBJECTIVE: To investigate the familial correlations and intraclass correlation of age-related hearing impairment (ARHI) in specific frequencies. In addition, heritability estimates were calculated. STUDY DESIGN: Multicenter survey in 8 European centers. SUBJECTS: One hundred ninety-eight families consisting of 952 family members, screened by otologic examination and structured interviews. Subjects with general conditions, known to affect hearing thresholds or known otologic cause were excluded from the study. RESULTS: We detected familial correlation coefficients of 0.36, 0.37, 0.36, and 0.30 for 0.25, 0.5, 1, and 2 kHz, respectively, and correlation coefficients of 0.20 and 0.18 for 4 and 8 kHz, respectively. Variance components analyses showed that the proportion of the total variance attributable to family differences was between 0.32 and 0.40 for 0.25, 0.5, 1, and 2 kHz and below 0.20 for 4 and 8 kHz. When testing for homogeneity between sib pair types, we observed a larger familial correlation between female than male subjects. Heritability estimates ranged between 0.79 and 0.36 across the frequencies. DISCUSSION: Our results indicate that there is a substantial shared familial effect in ARHI. We found that familial aggregation of ARHI is markedly higher in the low frequencies and that there is a trend toward higher familial aggregation in female compared with male subjects.