[Treating age-related hearing loss: hearing aids are not very popular]

Item does not contain fulltextPresbycusis means the deterioration of hearing resulting from the ageing process. Presbycusis can greatly affect one's quality of life; impaired hearing restricts communication and untreated presbycusis could result in social isolation and even depression. Nevertheless, only a minority of elderly with impaired hearing use a hearing aid. It is preferable to propose a hearing-aid fitting in the earliest stage possible, provided the current reimbursement criteria are met. The Dutch consensus model ('Veldnorm Hoortoestelverstrekking 2009') is the most important guideline in this regard

Genetic Hearing Loss Affects Cochlear Processing

The relationship between speech recognition and hereditary hearing loss is not straightforward. Underlying genetic defects might determine an impaired cochlear processing of sound. We obtained data from nine groups of patients with a specific type of genetic hearing loss. For each group, the affected cochlear site-of-lesion was determined based on previously published animal studies. Retrospectively obtained speech recognition scores in noise were related to several aspects of supra-threshold cochlear processing as assessed by psychophysical measurements. The differences in speech perception in noise between these patient groups could be explained by these factors and partially by the hypothesized affected structure of the cochlea, suggesting that speech recognition in noise was associated with a genetics-related malfunctioning of the cochlea. In particular, regression models indicate that loudness growth and spectral resolution best describe the cochlear distortions and are thus a good biomarker for speech understanding in noise

Audiometric characteristics of USH2a patients.

Item does not contain fulltextTemporal processing, frequency discrimination and frequency resolution, three basic mechanisms involved in speech perception, were studied in 11 USH2a patients from 10 Dutch families. Measurements included loudness scaling, gap detection, determination of auditory filter shapes and difference limen for frequency. The results were compared to values obtained from listeners with normal hearing, DFNA8/12 patients, DFNA13 patients and unselected patients with presbyacusis. Overall, the results for USH2a were most similar to those obtained in patients with sensorineural hearing loss caused by hair cell defects. To our knowledge, this is the first study that presents results on audiometric measurements combined with psychophysical measurements in a group of USH2a patients

The bone-anchored hearing aid for children: recent developments.

Contains fulltext : 70824.pdf (publisher's version ) (Closed access)In 1984 the Bone-Anchored Hearing Aid, or BAHA, system was introduced. Its transducer is coupled directly to the skull percutaneously to form a highly effective bone-conduction hearing device. Clinical studies on adults with conductive hearing loss have shown that the BAHA system outperforms conventional bone-conduction hearing aids. Therefore, the next step was to apply the BAHA system in children with congenital or acquired conductive hearing loss. Reviewed data showed that, on average, such children benefited significantly more from the BAHA than from reconstructive surgery. Thus, BAHA application appears to be the best option to achieve normal communication and speech and language development in children with bilateral conductive hearing loss. However, in children under the age of three to four years, a conventional solution must be applied, e.g. a bone conductor with a transcutaneous coupling, because they are too young to undergo BAHA implant surgery. In the case of unilateral congenital conductive hearing loss, there is no convincing evidence in the clinical literature for early intervention. In summary, the BAHA system can be considered a new, indispensable tool for children with bilateral conductive hearing loss

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

Contains fulltext : 70914.pdf (publisher's version ) (Closed access)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.

Contains fulltext : 70417.pdf (publisher's version ) (Closed access)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

Age-related use and benefit of the bone-anchored hearing aid compact.

Contains fulltext : 79913.pdf (publisher's version ) (Closed access)OBJECTIVE: To study age-related patient satisfaction with the bone-anchored hearing aid (BAHA) compact. METHODS: A retrospective postal questionnaire, the International Outcome Inventory for Hearing Aids (IOI-HA), was sent to 211 BAHA Compact users. Questionnaire responses from 135 BAHA users were analyzed related to age, sex, years of BAHA experience, and the hearing thresholds (pure-tone average) at the aided side. Age ranged from 18 to 77 years. RESULTS: The IOI-HA showed that the BAHA Compact was greatly appreciated by almost all of the users: most patients stated that they were using the device for most of the day; it helped them to hear better and it reduced the number of situations in which hearing impairment was problematical. The cumulative score on the questionnaire was negatively influenced by age (rho = -0.191, p = 0.05). Furthermore, increase in sensorineural hearing loss (SNHL) component was associated with decrease in total IOI-HA scores (Spearman rho = -0.193, p < 0.05). A significant correlation was found between age and the SNHL component (Spearman rho = 0.525, p < 0.001).There were no significant differences in the levels of difficulty with placing the BAHA on the implant or with handling the BAHA between the age groups. Cleaning the skin around the implant causes the most difficulties in the youngest age group (p < 0.02). CONCLUSION: The BAHA Compact enhances participation in various domains of communication. Differences in patients' satisfaction seemed to be correlated with the SNHL component rather than age

Audiometric Characteristics of a Dutch Family with a New Mutation in GATA3 Causing HDR Syndrome.

Item does not contain fulltextWe present the case of a Dutch family with a new mutation (c523_528dup) in GATA3 causing HDR syndrome. HDR syndrome is characterised by hypoparathyroidism, deafness and renal defects. In this study, we describe the audiometric characteristics of 5 patients from this family. Their hearing impairment was congenital, bilateral and symmetric. Audiograms showed mild-to-moderate hearing impairment with a flat audiogram configuration. Higher frequencies tended to be affected more strongly. Cross-sectional analyses showed no progression, and a mean audiogram was established. Psychophysical measurements in 3 HDR patients - including speech reception in noise, loudness scaling, gap detection and difference limen for frequency - were obtained to assess hearing function in greater detail. Overall, the results of the psychophysical measurements indicated characteristics of outer hair cell loss. CT scanning showed no anomalies in 3 of the HDR patients. Although 2 patients displayed vestibular symptoms, no anomalies in the vestibular system were found by vestibulo-ocular examination. Our results are in agreement with the theory that outer hair cell malfunctioning can play a major role in HDR syndrome. (c) 2014 S. Karger AG, Basel

Audiometric characteristics of a Dutch family with Muckle-Wells syndrome.

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Audiometric Characteristics of a Dutch DFNA10 Family With Mid-Frequency Hearing Impairment

OBJECTIVES: Mutations in EYA4 can cause nonsyndromic autosomal dominant sensorineural hearing impairment (DFNA10) or a syndromic variant with hearing impairment and dilated cardiomyopathy. A mutation in EYA4 was found in a Dutch family, causing DFNA10. This study is focused on characterizing the hearing impairment in this family. DESIGN: Whole exome sequencing was performed in the proband. In addition, peripheral blood samples were collected from 23 family members, and segregation analyses were performed. All participants underwent otorhinolaryngological examinations and pure-tone audiometry, and 12 participants underwent speech audiometry. In addition, an extended set of audiometric measurements was performed in five family members to evaluate the functional status of the cochlea. Vestibular testing was performed in three family members. Two individuals underwent echocardiography to evaluate the nonsyndromic phenotype. RESULTS: The authors present a Dutch family with a truncating mutation in EYA4 causing a mid-frequency hearing impairment. This mutation (c.464del) leads to a frameshift and a premature stop codon (p.Pro155fsX). This mutation is the most N-terminal mutation in EYA4 found to date. In addition, a missense mutation, predicted to be deleterious, was found in EYA4 in two family members. Echocardiography in two family members revealed no signs of dilated cardiomyopathy. Results of caloric and velocity step tests in three family members showed no abnormalities. Hearing impairment was found to be symmetric and progressive, beginning as a mid-frequency hearing impairment in childhood and developing into a high-frequency, moderate hearing impairment later in life. Furthermore, an extended set of audiometric measurements was performed in five family members. The results were comparable to those obtained in patients with other sensory types of hearing impairments, such as patients with Usher syndrome type IIA and presbyacusis, and not to those obtained in patients with (cochlear) conductive types of hearing impairment, such as DFNA8/12 and DFNA13. CONCLUSIONS: The mid-frequency hearing impairment in the present family was found to be symmetric and progressive, with a predominantly childhood onset. The results of psychophysical measurements revealed similarities to other conditions involving a sensory type of hearing impairment, such as Usher syndrome type IIA and presbyacusis. The study results suggest that EYA4 is expressed in the sensory cells of the cochlea. This phenotypic description will facilitate counseling for hearing impairment in DFNA10 patients