Cochlear implantation in children with Waardenburg syndrome

2005 ◽  
Vol 119 (9) ◽  
pp. 719-723 ◽  
Author(s):  
A Daneshi ◽  
S Hassanzadeh ◽  
M Farhadi
Keyword(s):  
Speech Perception ◽  
Speech Production ◽  
Cochlear Implantation ◽  
Auditory Perception ◽  
Speech Intelligibility ◽  
Hearing Impaired ◽  
Type I ◽  
Waardenburg Syndrome ◽  
Retrospective Case ◽  
One Year

Waardenburg syndrome is an autosomal-dominant trait resulting from mutations occurring in different genes. It is often characterized by varying degrees of: congenital hearing loss; dystopia canthorum; synophrys; broad nasal root; depigmentation of hair (white forelock), skin or both; and heterochromic or hypochromic irides.A retrospective case study was done to assess speech perception, speech production, general intelligence and educational setting in six profoundly hearing-impaired children with Waardenburg syndrome (four with type I, one with type II and one with type III) ranging in age from two years to 14 years, seven months (mean = six years, six months). None of the patients had malformation of the cochlea and were implanted using Nucleus 22/24 and Med-el combi40+. Five out of the six cases were of average intelligence and one had a borderline intelligence quotient. The follow-up period ranged from one year, 10 months to six years, six months (mean = three years, six months) after implantation. The evaluation of auditory perception in patients was accomplished using the Persian Auditory Perception Test for the Hearing-Impaired, a Persian Spondee wordstest and the Categories of Auditory Performance Index. The Speech Intelligibility Rating test was used to evaluate speech production ability. All the patients’ speech perception and speech intelligibility capabilities improved considerably after receiving the implants, and they were able to be placed in regular educational settings. Patients used their cochlear-implant devices whenever awake, implying that they benefitted from the devices. We suggest that any further expansion of cochlear-implantation criteria in children include those with Waardenburg syndrome.

Prevalence of GJB2-associated deafness and outcomes of cochlear implantation in Iran

2011 ◽  
Vol 125 (5) ◽  
pp. 455-459 ◽  
Author(s):  
A Daneshi ◽  
S Hassanzadeh ◽  
H Emamdjomeh ◽  
S H Mohammadi ◽  
S Arzhangi ◽  
...  
Keyword(s):  
Speech Perception ◽  
Speech Production ◽  
Cochlear Implantation ◽  
Speech Intelligibility ◽  
Rating Scales ◽  
Direct Sequencing ◽  
Gene Mutations ◽  
Deaf Children ◽  
Nested Polymerase Chain Reaction ◽  
Iranian Children

AbstractObjectives:To investigate the prevalence of mutations in the coding exon of the GJB2 gene in Iranian children with cochlear implants, and to compare the outcomes of auditory perception and speech production in cochlear-implanted children with and without GJB2 mutation.Materials and methods:One hundred and sixty-six prelingually deaf children who had undergone cochlear implantation at the Iranian Cochlear Implant Center, Tehran, were selected from a pool of 428 implanted children. The prevalence of GJB2 gene mutations was assessed using nested polymerase chain reaction and direct sequencing. To enable comparisons, we also identified 36 implanted children with non-GJB2 deafness. Patients' speech perception and speech production were assessed using the Categorization of Auditory Performance and Speech Intelligibility Rating scales.Results:Thirty-three of 166 probands (19.9 per cent) were found to have GJB2 deafness-causing allele variants and were diagnosed with DFNB1 deafness. Results also indicated a significant improvement in speech perception and production scores in both GJB2 and non-GJB2 patients over time.Conclusion:Children with GJB2-related deafness benefit from cochlear implantation to the same extent as those with non-GJB2-related deafness.

The Relationship Between Speech Production and Speech Perception Deficits in Parkinson's Disease

2016 ◽  
Vol 59 (5) ◽  
pp. 915-931 ◽  
Author(s):  
Kim De Keyser ◽  
Patrick Santens ◽  
Annelies Bockstael ◽  
Dick Botteldooren ◽  
Durk Talsma ◽  
...  
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Speech Perception ◽  
Speech Production ◽  
Auditory Perception ◽  
Speech Intelligibility ◽  
Acoustic Analysis ◽  
Estimation Task ◽  
Intensity Measurements ◽  
Intensity Perception

PurposeThis study investigated the possible relationship between hypokinetic speech production and speech intensity perception in patients with Parkinson's disease (PD).MethodParticipants included 14 patients with idiopathic PD and 14 matched healthy controls (HCs) with normal hearing and cognition. First, speech production was objectified through a standardized speech intelligibility assessment, acoustic analysis, and speech intensity measurements. Second, an overall estimation task and an intensity estimation task were addressed to evaluate overall speech perception and speech intensity perception, respectively. Finally, correlation analysis was performed between the speech characteristics of the overall estimation task and the corresponding acoustic analysis. The interaction between speech production and speech intensity perception was investigated by an intensity imitation task.ResultsAcoustic analysis and speech intensity measurements demonstrated significant differences in speech production between patients with PD and the HCs. A different pattern in the auditory perception of speech and speech intensity was found in the PD group.ConclusionsAuditory perceptual deficits may influence speech production in patients with PD. The present results suggest a disturbed auditory perception related to an automatic monitoring deficit in PD.

Evaluation of speech in patients after cochlear implantation – Motol Speech Scale

2021 ◽  
Vol 70 (3) ◽  
pp. 156-160
Author(s):  
Zdenka Aksenovová ◽  
Miroslav Okluský ◽  
Jiří Skřivan
Keyword(s):  
Speech Perception ◽  
Speech Production ◽  
Cochlear Implantation ◽  
Auditory Perception ◽  
Public Key ◽  
Spearman Correlation ◽  
New Classification ◽  
Statistical Software ◽  
The Public ◽  
Spearman Correlation Test

Summary Introduction: The aim of this study was to create a new classification scale for the evaluation of speech in patients after cochlear implantation. Our newly created Motol Speech Scale (MSS) independently evaluates both speech components – perception and expression. Material and methods: We processed a set of 416 cochlear implant users. It was evaluated patients’ auditory perception level and speech status according to the internationally used Nottingham scale – Categories of Auditory Performation (CAP) and, at the same time, according to the Motol Speech Scale (MSS). The data obtained on both scales were compared and evaluated using the GraphPad statistical software. Results: The Spearman correlation test demonstrated the existence of a positive correlation between the CAP and MSS scales. Conclusion: Our newly created MSS scale is a suitable tool for classifying speech after cochlear implantation. It is understandable for CI users, parents, professionals, and the public. Key words cochlear implantation – speech perception – speech production – classification scale

Cochlear Implantation in Children with Postlingual Progressive Steeply Sloping High-Frequency Hearing Loss

2017 ◽  
Vol 28 (10) ◽  
pp. 913-919 ◽  
Author(s):  
Margaret A. Meredith ◽  
Jay T. Rubinstein ◽  
Kathleen C. Y. Sie ◽  
Susan J. Norton
Keyword(s):  
Hearing Loss ◽  
Speech Perception ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
High Frequency ◽  
Case Series ◽  
Hearing Preservation ◽  
Rapid Improvement ◽  
Retrospective Case ◽  
Monosyllabic Word

Background: Children with steeply sloping sensorineural hearing loss (SNHL) lack access to critical high-frequency cues despite the use of advanced hearing aid technology. In addition, their auditory-only aided speech perception abilities often meet Food and Drug Administration criteria for cochlear implantation. Purpose: The objective of this study was to describe hearing preservation and speech perception outcomes in a group of young children with steeply sloping SNHL who received a cochlear implant (CI). Research Design: Retrospective case series. Study Sample: Eight children with steeply sloping postlingual progressive SNHL who received a unilateral traditional CI at Seattle Children’s Hospital between 2009 and 2013 and had follow-up data available up to 24 mo postimplant were included. Data Collection and Analysis: A retrospective chart review was completed. Medical records were reviewed for demographic information, preoperative and postoperative behavioral hearing thresholds, and speech perception scores. Paired t tests were used to analyze speech perception data. Hearing preservation results are reported. Results: Rapid improvement of speech perception scores was observed within the first month postimplant for all participants. Mean monosyllabic word scores were 76% and mean phoneme scores were 86.7% at 1-mo postactivation compared to mean preimplant scores of 19.5% and 31.0%, respectively. Hearing preservation was observed in five participants out to 24-mo postactivation. Two participants lost hearing in both the implanted and unimplanted ear, and received a sequential bilateral CI in the other ear after progression of the hearing loss. One participant had a total loss of hearing in only the implanted ear. Results reported in this article are from the ear implanted first. Bilateral outcomes are not reported. Conclusions: CIs provided benefit for children with steeply sloping bilateral hearing loss for whom hearing aids did not provide adequate auditory access. In our cohort, significant improvements in speech understanding occurred rapidly postactivation. Preservation of residual hearing in children with a traditional CI electrode is possible.

Noise and acoustic conditions of premises for hearing-impaired people in Korea

2021 ◽  
Vol 69 (1) ◽  
pp. 77-85
Author(s):  
Cheol-Ho Jeong ◽  
Wan-Ho Cho ◽  
Ji-Ho Chang ◽  
Sung-Hyun Lee ◽  
Chang-Wook Kang ◽  
...  
Keyword(s):  
Speech Perception ◽  
Speech Intelligibility ◽  
Hearing Impaired ◽  
Noise Levels ◽  
Listening Effort ◽  
Subjective Data ◽  
Impaired People ◽  
The Republic ◽  
Primary School Pupils ◽  
Acoustic Conditions

Hearing-impaired people need more stringent acoustic and noise requirements than normal-hearing people in terms of speech intelligibility and listening effort. Multiple guidelines recommend a maximum reverberation time of 0.4 s in classrooms, signal-to-noise ratios (SNRs) greater than 15 dB, and ambient noise levels lower than 35 dBA. We measured noise levels and room acoustic parameters of 12 classrooms in two schools for hearing-impaired pupils, a dormitory apartment for the hearing-impaired, and a church mainly for the hearing-impaired in the Republic of Korea. Additionally, subjective speech clarity and quality of verbal communication were evaluated through questionnaires and interviews with hearing-impaired students in one school. Large differences in subjective speech perception were found between younger primary school pupils and older pupils. Subjective data from the questionnaire and interview were inconsistent; major challenges in obtaining reliable subjective speech perception and limitations of the results are discussed.

Cochlear implantation in prelingually deaf persons with additional disability

2006 ◽  
Vol 121 (7) ◽  
pp. 635-638 ◽  
Author(s):  
A Daneshi ◽  
S Hassanzadeh
Keyword(s):  
Attention Deficit Hyperactivity Disorder ◽  
Cerebral Palsy ◽  
Mental Retardation ◽  
Learning Disability ◽  
Cochlear Implantation ◽  
Auditory Perception ◽  
Hyperactivity Disorder ◽  
One Year ◽  
Deaf Persons ◽  
Prelingually Deaf

Objectives: We aimed to identify the frequency with which the following conditions were present as a second disability in cochlear-implanted, prelingually deaf persons: mild and moderate mental retardation; learning disability; attention deficit/hyperactivity disorder; cerebral palsy; congenital blindness; and autism. We also aimed to document the development of auditory perception in patients having one of these additional disabilities.Study design: A retrospective study was designed to pursue the above aims.Methods: We examined the records of 398 cochlear-implanted, prelingually deaf patients who had received a cochlear implant at least one year previously. Patients were selected who showed a delay in motor, cognitive or emotional development. The selected cases were referred for psychological evaluation in order to identify patients with additional disabilities. We then compared these patients' auditory perception prior to and one year following cochlear implantation.Results: A total of 60 (15 per cent) cochlear-implanted, prelingually deaf patients were diagnosed with additional disabilities. These were classified as: mild mental retardation in eight cases (13.33 per cent); moderate mental retardation in five (8.33 per cent); learning disability in 20 (33.33 per cent); attention deficit/hyperactivity disorder in 15 (25 per cent); cerebral palsy in five (8.33); congenital blindness in three (5 per cent); and autism in four (6.66 per cent). All patients showed significant development in speech perception, except for autistic and congenitally deaf-blind patients.Conclusion: Although cochlear implantation is not contraindicated in prelingually deaf persons with additional disabilities, congenitally deaf-blind and autistic patients showed limited development in auditory perception as a main outcome of cochlear implantation. These patients require unique rehabilitation in order to achieve more auditory development.

scholarly journals A Systematic Review to Define the Speech and Language Benefit of Early (<12 Months) Pediatric Cochlear Implantation

2016 ◽  
Vol 21 (2) ◽  
pp. 113-126 ◽  
Author(s):  
Hanneke Bruijnzeel ◽  
Fuat Ziylan ◽  
Inge Stegeman ◽  
Vedat Topsakal ◽  
Wilko Grolman
Keyword(s):  
Speech Perception ◽  
Speech Production ◽  
Cochlear Implantation ◽  
Receptive Language ◽  
Additional Benefit ◽  
Speech And Language ◽  
Language Outcome ◽  
Auditory Performance ◽  
Auditory Integration

Objective: This review aimed to evaluate the additional benefit of pediatric cochlear implantation before 12 months of age considering improved speech and language development and auditory performance. Materials and Methods: We conducted a search in PubMed, EMBASE and CINAHL databases and included studies comparing groups with different ages at implantation and assessing speech perception and speech production, receptive language and/or auditory performance. We included studies with a high directness of evidence (DoE). Results: We retrieved 3,360 articles. Ten studies with a high DoE were included. Four articles with medium DoE were discussed in addition. Six studies compared infants implanted before 12 months with children implanted between 12 and 24 months. Follow-up ranged from 6 months to 9 years. Cochlear implantation before the age of 2 years is beneficial according to one speech perception score (phonetically balanced kindergarten combined with consonant-nucleus-consonant) but not on Glendonald auditory screening procedure scores. Implantation before 12 months resulted in better speech production (diagnostic evaluation of articulation and phonology and infant-toddler meaningful auditory integration scale), auditory performance (Categories of Auditory Performance-II score) and receptive language scores (2 out of 5; Preschool Language Scale combined with oral and written language skills and Peabody Picture Vocabulary Test). Conclusions: The current best evidence lacks level 1 evidence studies and consists mainly of cohort studies with a moderate to high risk of bias. Included studies showed consistent evidence that cochlear implantation should be performed early in life, but evidence is inconsistent on all speech and language outcome measures regarding the additional benefit of implantation before the age of 12 months. Long-term follow-up studies are necessary to provide insight on additional benefits of early pediatric cochlear implantation.

The Effects of Preoperative Audiovisual Speech Perception on the Audiologic Outcomes of Cochlear Implantation in Patients with Postlingual Deafness

2020 ◽  
pp. 1-8
Author(s):  
Hyun Jin Lee ◽  
Jeon Mi Lee ◽  
Jae Young Choi ◽  
Jinsei Jung
Keyword(s):  
Speech Perception ◽  
Cochlear Implantation ◽  
Visual Information ◽  
Significant Negative Correlation ◽  
Retrospective Case ◽  
Audiovisual Speech Perception ◽  
Auditory Rehabilitation ◽  
Before And After ◽  
Case Comparison ◽  
Perception Score

<b><i>Introduction:</i></b> Patients with postlingual deafness usually depend on visual information for communication, and their lipreading ability could influence cochlear implantation (CI) outcomes. However, it is unclear whether preoperative visual dependency in postlingual deafness positively or negatively affects auditory rehabilitation after CI. Herein, we investigated the influence of preoperative audiovisual per­ception on CI outcomes. <b><i>Method:</i></b> In this retrospective case-comparison study, 118 patients with postlingual deafness who underwent unilateral CI were enrolled. Evaluation of speech perception was performed under both audiovisual (AV) and audio-only (AO) conditions before and after CI. Before CI, the speech perception test was performed under hearing aid (HA)-assisted conditions. After CI, the speech perception test was performed under the CI-only condition. Only patients with a 10% or less preoperative AO speech perception score were included. <b><i>Results:</i></b> Multivariable regression analysis showed that age, gender, residual hearing, operation side, education level, and HA usage were not correlated with either postoperative AV (pAV) or AO (pAO) speech perception. However, duration of deafness showed a significant negative correlation with both pAO (<i>p</i> = 0.003) and pAV (<i>p</i> = 0.015) speech perceptions. Notably, the preoperative AV speech perception score was not correlated with pAO speech perception (<i>R</i><sup>2</sup> = 0.00134, <i>p</i> = 0.693) but was positively associated with pAV speech perception (<i>R</i><sup>2</sup> = 0.0731, <i>p</i> = 0.003). <b><i>Conclusion:</i></b> Preoperative dependency on audiovisual information may positively influence pAV speech perception in patients with postlingual deafness.

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