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.

Bone-anchored hearing aids in patients with acquired and congenital unilateral inner ear deafness (Baha CROS): clinical evaluation of 56 cases.

OBJECTIVES: We performed an evaluation of the audiological and subjective benefits of the bone-anchored hearing aid (Baha) as a device for transcranial routing of sound (Baha CROS) in 56 patients with unilateral inner ear deafness. METHODS: We performed a prospective clinical follow-up study in a tertiary referral center. Previously reported results of 29 patients were supplemented with a second series of 30 patients with unilateral inner ear deafness; 3 patients dropped out during the evaluation. Audiometric measurements were taken before and after Baha CROS fitting. Subjective benefits were quantified with 4 different patient questionnaires. RESULTS: The sound localization results in a well-structured test setting were not differentiable from chance. The 5 patients with congenital hearing loss showed better scores in the unaided sound localization measurements. Overall, most patients reported some subjective improvement in their capacity to localize sounds with the Baha CROS in daily life. The main effect of the Baha CROS was to alleviate the head shadow effect during the speech-in-noise test. CONCLUSIONS: Poor sound localization in this larger series of patients confirms the findings of previous studies. Improvements in the speech-in-noise scores corroborated the efficacy of the Baha CROS in alleviating the head shadow effect. The 4 different patient questionnaires revealed subjective benefit and satisfaction in various domains.

Pilot study on the effectiveness of the conventional CROS, the transcranial CROS and the BAHA transcranial CROS in adults with unilateral inner ear deafness.

The objective of the present pilot study is to evaluate the effectiveness of three conventional contralateral routing of sound (CROS) hearing aids in adults with unilateral inner ear deafness. The study included tertiary referral center. Ten patients with unilateral inner ear deafness and normal hearing in the contralateral ear were selected to evaluate three different methods of amplification: the CROS hearing aid, the completely in the canal hearing aid and the bone-anchored hearing aid CROS (BAHA). Each of the three hearing aids was tried in a random order for a period of 8 weeks. Audiometric performance, including speech-in-noise, directional hearing and subjective benefit were measured after each trial period, using the APHAB, SSQ and single-sided deafness questionnaire. Sound localization performance was essentially at chance level in all four conditions. Mixed results were seen on the other patient outcome measures that alternated in favor of one of the three CROS devices. After the trial, three patients chose to be fitted with the BAHA CROS and one with the conventional CROS. In conclusion, most of the patients experienced some degree of benefit with each of the three hearing aids. Preference for one of the three hearing aids was independent of the order in which they were tried. It would be worthwhile to formulate selection criteria; still, we recommend that all patients with unilateral inner ear deafness should be offered a trial with at least the BAHA CROS.

Subjective benefit after BAHA system application in patients with congenital unilateral conductive hearing impairment.

OBJECTIVE: To study whether unilateral Bone-anchored Hearing Aid (BAHA) fitting led to subjective hearing benefit in patients with congenital unilateral conductive hearing impairment. STUDY DESIGN: Prospective evaluation on 20 patients. SETTING: Tertiary referral center. PATIENTS: Ten adults and 10 children with congenital unilateral conductive hearing impairment, with a mean air-bone gap of 50 dB, were included. METHODS: Subjective bilateral hearing benefit after BAHA fitting was measured using 2 disability-specific questionnaires: Chung and Stephens and the Speech, Spatial and Qualities of hearing profile (children's version in the patients aged <18 yr). The Glasgow children's benefit inventory was also used to measure patient's health benefit after BAHA fitting. RESULTS: Chung and Stephens' questionnaire showed an overall preference for the BAHA in several specific hearing situations. The Glasgow children's benefit inventory demonstrated an overall mean improvement of +34, which was the most prominent in the learning domain. The 10 adults showed an already good score on the Speech, Spatial and Qualities of hearing scale in the unaided situation. CONCLUSION: The BAHA was well accepted by most of the patients with congenital unilateral conductive hearing impairment. A preoperative trial of the BAHA system with the BAHA on a headband is part of the preoperative procedure. In children with unilateral conductive hearing loss, with regard to possible childs' development and communication difficulties, intervention with BAHA can be considered as an option.

Bone-anchored hearing aid system application for unilateral congenital conductive hearing impairment: audiometric results.

OBJECTIVE: To study the audiologic outcome of bone-anchored hearing aid (BAHA) application in patients with congenital unilateral conductive hearing impairment. STUDY DESIGN: Prospective audiometric evaluation on 20 patients. SETTING: Tertiary referral center. PATIENTS: The experimental group comprised 20 consecutive patients with congenital unilateral conductive hearing impairment, with a mean air-bone gap of 50 dB. METHODS: Aided and unaided hearing was assessed using sound localization and speech recognition-in-noise tests. RESULTS: Aided hearing thresholds and aided speech perception thresholds were measured to verify the effect of the BAHA system on the hearing acuity. All patients fulfilled the criteria that the aided speech reception thresholds or the mean aided sound field thresholds were 25 dB or better in the aided situation. Most patients were still using the BAHA almost every day. Sound localization scores varied widely in the unaided and aided situations. Many patients showed unexpectedly good unaided performance. However, nonsignificant improvements of 3.0 (500 Hz) and 6.9 degrees (3,000 Hz) were observed in favor of the BAHA. Speech recognition in noise with spatially separated speech and noise sources also improved after BAHA implantation, but not significantly. CONCLUSION: Some patients with congenital unilateral conductive hearing impairment had such good directional hearing and speech-in-noise scores in the unaided situation that no overall significant improvement occurred after BAHA fitting in our setup. Of the 18 patients with a complete data set, 6 did not show any significant improvement at all. However, compliance with BAHA use in this patient group was remarkably high. Observations of consistent use of the device are highly suggestive of patient benefit. Further research is recommended to get more insight into these findings.

Bone-anchored hearing aid in patients with moderate mental retardation: impact and benefit assessment.

OBJECTIVE: To assess the impact and the subjective benefit of Bone-anchored Hearing Aid (BAHA) implementation in patients with hearing impairment combined with moderate mental retardation. STUDY DESIGN: Case control study using two validated patient-oriented instruments. SETTING: Tertiary referral center. PATIENTS: Twenty-two patients with moderate mental retardation and conductive or mixed hearing loss. INTERVENTION: Rehabilitative. MAIN OUTCOME MEASURES: Subjective benefit, listening and learning capabilities. RESULTS: BAHA implementation in patients with moderate mental retardation, by using the Glasgow Children's Benefit Inventory and the Listening Inventory for Education, showed a subjective benefit, which was comparable with that of the control group and was consistent with the results of earlier studies. CONCLUSION: The use of BAHA proved beneficial in most patients with hearing impairment and moderate mental retardation. Extending the indications for BAHA application to this special patient group shows to be a very valuable option.

Rehabilitation of patients with conductive hearing loss and moderate mental retardation by means of a bone-anchored hearing aid.

OBJECTIVE: To evaluate whether the bone-anchored hearing aid (BAHA) can be applied successfully to patients with conductive hearing loss and moderate mental retardation. STUDY DESIGN: Retrospective clinical evaluation. SETTING: Tertiary referral center. PATIENTS: Twenty-two patients with congenital moderate mental retardation and conductive or mixed hearing loss were selected to receive a BAHA at the University Medical Centre Nijmegen, the Netherlands. Four of them were fitted despite a limited air-bone gap. INTERVENTION: Rehabilitative BAHA application. MAIN OUTCOME MEASURES: Implantation results, skin reactions, and audiological data were evaluated during a mean follow-up of 36 months. RESULTS: All the patients were still using the BAHA 7 days a week and for more than 8 hours a day after a follow-up period between 5 and 96 months. Two implants (9%) were lost due to insufficient integration but were reimplanted successfully. With the BAHA, mean free-field thresholds showed a clear mean improvement of 9 dB compared with the previous hearing aid. Considerable improvements in daily activities were seen in at least five patients. CONCLUSION: Moderate mental retardation should no longer be considered as a contraindication for BAHA application. Although implant loss was low, extra attention may be required from the personal care providers to maintain the percutaneous implant. The BAHA was well-accepted by the patients with moderate mental retardation and was being used for most of the day. Implementation of the BAHA as hearing aid treatment in patients with moderate mental retardation proved to be sufficiently effective and may have strongly positive effects on activities at school or at work.