Κυριακή 10 Ιανουαρίου 2016

20Q: Hearing Aids - The Future Ain't What it Used to Be!

Back in May of 1994, I wrote an article titled, Update On Programmable Hearing Aids (With An Assist From Yogi Berra). At the time, there were a lot of questions surrounding the use of programmable hearing aids, and I thought it might be appropriate to use several Yogi-isms to help sort out the confusion. With the passing of Yogi this past September, it only seems appropriate to revisit that theme today to look at current issues regarding hearing aid selection and fitting. In a way, it's like "déjà vu all over again."

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20Q: Hearing Aids - The Future Ain't What it Used to Be!

Back in May of 1994, I wrote an article titled, Update On Programmable Hearing Aids (With An Assist From Yogi Berra). At the time, there were a lot of questions surrounding the use of programmable hearing aids, and I thought it might be appropriate to use several Yogi-isms to help sort out the confusion. With the passing of Yogi this past September, it only seems appropriate to revisit that theme today to look at current issues regarding hearing aid selection and fitting. In a way, it's like "déjà vu all over again."

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20Q: Hearing Aids - The Future Ain't What it Used to Be!

Back in May of 1994, I wrote an article titled, Update On Programmable Hearing Aids (With An Assist From Yogi Berra). At the time, there were a lot of questions surrounding the use of programmable hearing aids, and I thought it might be appropriate to use several Yogi-isms to help sort out the confusion. With the passing of Yogi this past September, it only seems appropriate to revisit that theme today to look at current issues regarding hearing aid selection and fitting. In a way, it's like "déjà vu all over again."

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Quality of life outcomes for children with hearing impairment in Singapore.

Quality of life outcomes for children with hearing impairment in Singapore.

Int J Pediatr Otorhinolaryngol. 2016 Jan;80:88-100

Authors: Looi V, Lee ZZ, Loo JH

Abstract
OBJECTIVES: This study aimed to investigate the hearing-related quality of life (hearing-QOL) of children with hearing loss in Singapore using hearing aids (HAs) and/or cochlear implants (CIs). Their health-related QOL (health-QOL) as well as their families' health-QOL were compared with normally hearing (NH) children and their families.
METHODS: This cross-sectional study recruited families (i.e., children aged 2-18 years and their parents) of NH children (n=44), children wearing HAs (n=22) and children wearing CIs (n=14). Hearing-QOL was assessed using the parent-reported Children Using Hearing Devices QOL questionnaire. General health-QOL was assessed using parent and child-reported measures from the PedsQL inventory consisting of the Generic Core Scales, General Well-being Scale and Family Impact Module. The questionnaires were self-administered for children aged 8-18 years, and interviewer-administered for children aged 5-7 years.
RESULTS: The NH children and their families had significantly higher general health-QOL scores compared to the children with hearing loss and their families. This indicates that hearing loss significantly impacts on the well-being of children and their families. Congruence between parent and child reports was only observed in the NH group. Parents of children wearing HAs rated their child's overall hearing-QOL significantly higher than parents of children wearing CIs. Family household income was the only significant predictor of child hearing-QOL scores with parents from the middle income families rating their child's hearing-QOL significantly poorer than parents from high income families.
CONCLUSION: Overall, NH children and their families have higher self-rated general health-QOL than children with hearing loss and their families, with children using HAs providing higher hearing-QOL than those using CIs. Evaluating both general health and hearing specific QOL from both the child and their parent/caregiver is worthwhile, allowing a more holistic measure of real-life outcomes and better individualised clinical care.

PMID: 26746619 [PubMed - in process]



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Identification of a nonsense mutation in the STRC gene in a Korean family with moderate hearing loss.

Identification of a nonsense mutation in the STRC gene in a Korean family with moderate hearing loss.

Int J Pediatr Otorhinolaryngol. 2016 Jan;80:78-81

Authors: Sagong B, Baek JI, Bok J, Lee KY, Kim UK

Abstract
Hereditary hearing loss is a heterogeneous disorder that results in a common sensorineural disorder. To date, more than 150 loci and 89 genes have been reported for non-syndromic hearing loss. Next generation sequencing has recently been developed as a powerful genetic strategy for identifying pathogenic mutations in heterogeneous disorders with various causative genes. In this study, we performed targeted sequencing to identify the causative mutation in a Korean family that had moderate hearing loss. We targeted 64 genes associated with non-syndromic hearing loss and sorted the homozygous variations according to the autosomal recessive inheritance pattern of the family. Implementing a bioinformatic platform for filtering and detecting variations allowed for the identification of two variations within different genes (c.650G>A in TRIOBP and c.4057C>T in STRC). These variants were selected for further analysis. Among these, c.4057C>T (p.Q1353X) was a divergent sequence variation between the STRC gene and the STRC pseudogene. This was the critical difference that resulted in loss of the protein-coding ability of the pseudogene. Therefore, we hypothesized that the p.Q1353X variation in the STRC gene is the causative mutation for hearing loss. This result suggests that application of targeted sequencing will be valuable for the diagnosis of heterogeneous disorders.

PMID: 26746617 [PubMed - in process]



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Otorhinolaryngological, audiovestibular and swallowing manifestations of patients with Niemann-Pick disease Type C.

Otorhinolaryngological, audiovestibular and swallowing manifestations of patients with Niemann-Pick disease Type C.

Int J Pediatr Otorhinolaryngol. 2016 Jan;80:1-4

Authors: Senirli RT, Kuşçu O, Akyol U, Topçu M, Yiğit Ö, Aksoy S, Demir N

Abstract
OBJECTIVES: The aim of this study is to evaluate audiovestibular and swallowing impairment of patients with NPC.
METHODS: Audiovestibular and swallowing evaluation were performed on patients with Niemann-Pick disease type C (NPC) at Hacettepe University between 20013 and 2015 prospectively. Pure-tone audiometry (PTA), Auditory Brain stem response (ABR), Flexible endoscopic evaluation of swallowing (FEES) test and posturography were done. Hearing, swallowing and balance states were measured.
RESULTS: There were 16 patients (5 male and 11 female, with a median age of 6.5 years old). The most common ABR abnormalities observed were absent waves I and III (%70 absent I waves, %43.75 absent III waves). Twelve of sixteen patients (%75) had an ABR abnormality in at least one ear, of these, four patients had normal hearing and three of them had periferal hearing loss. 12 (75%) patients had complaint of postural imbalance. 11(69%) of patients had peripheral and one (6%) patient had central impairment. Nine of sixteen patients (56.25%) show some degree of dysphagia (either penetration or aspiration). Two patients (12.5%) showed aspiration both liquid and viscous nutrition. Three patients (18.75%) showed aspiration primarily in liquids and two of them had penetration with viscous nutrition. Three patients (18.75%) had penetration with no aspiration neither liquid nor viscous nutrition (PEN-ASP score was 3, 3, 5, respectively).
CONCLUSION: There is no curative treatment for this devastating and fatal disorder and hearing impairment, balance and swallowing disorders can be seen especially late onset form of disease.

PMID: 26746602 [PubMed - in process]



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Otorhinolaryngological, audiovestibular and swallowing manifestations of patients with Niemann-Pick disease Type C.

Otorhinolaryngological, audiovestibular and swallowing manifestations of patients with Niemann-Pick disease Type C.

Int J Pediatr Otorhinolaryngol. 2016 Jan;80:1-4

Authors: Senirli RT, Kuşçu O, Akyol U, Topçu M, Yiğit Ö, Aksoy S, Demir N

Abstract
OBJECTIVES: The aim of this study is to evaluate audiovestibular and swallowing impairment of patients with NPC.
METHODS: Audiovestibular and swallowing evaluation were performed on patients with Niemann-Pick disease type C (NPC) at Hacettepe University between 20013 and 2015 prospectively. Pure-tone audiometry (PTA), Auditory Brain stem response (ABR), Flexible endoscopic evaluation of swallowing (FEES) test and posturography were done. Hearing, swallowing and balance states were measured.
RESULTS: There were 16 patients (5 male and 11 female, with a median age of 6.5 years old). The most common ABR abnormalities observed were absent waves I and III (%70 absent I waves, %43.75 absent III waves). Twelve of sixteen patients (%75) had an ABR abnormality in at least one ear, of these, four patients had normal hearing and three of them had periferal hearing loss. 12 (75%) patients had complaint of postural imbalance. 11(69%) of patients had peripheral and one (6%) patient had central impairment. Nine of sixteen patients (56.25%) show some degree of dysphagia (either penetration or aspiration). Two patients (12.5%) showed aspiration both liquid and viscous nutrition. Three patients (18.75%) showed aspiration primarily in liquids and two of them had penetration with viscous nutrition. Three patients (18.75%) had penetration with no aspiration neither liquid nor viscous nutrition (PEN-ASP score was 3, 3, 5, respectively).
CONCLUSION: There is no curative treatment for this devastating and fatal disorder and hearing impairment, balance and swallowing disorders can be seen especially late onset form of disease.

PMID: 26746602 [PubMed - in process]



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