数字助听器算法开发平台pDHA的构建和测试

数字助听器是目前广大聋人康复的唯一有效手段,因此数字助听算法的研究日益重要.为便于进行助听算法的开发测试,本论文设计实现了一款基于可编程DSP的全数字助听算法开发平台pDHA.该平台具有计算功能强大、实时便携、扩展能力丰富等特点.本论文还针对此类平台的独特应用需求和相关标准,制定了一套完整的测试流程.参数测试结果表明,该系统具备足够的能力进行高级数字助听算法的开发和测试.     

Exploring the perceptual characteristics of voice-hallucinations in deaf people

Introduction. Previous research has not taken account of the possibility that deaf people will show greater heterogeneity in how they experience voice-hallucinations due to individual differences in experience with language and residual hearing. This study aims to explore how deaf participants perceive voice-hallucinations and whether the perceptual characteristics reported reflect individual experience with language and sensory input.

Method. A statement-sorting task generated data about perceptual characteristics of voice-hallucinations for exploratory factor analysis. The sample included 27 deaf participants with experience of voice-hallucinations, and a range of hearing loss and language backgrounds.

Results. Perceptual characteristics of voice-hallucinations map closely onto individual auditory experience. People born profoundly deaf loaded onto nonauditory factors. Deaf people with experience of hearing speech, through residual hearing, hearing aids, or predeafness experience, reported auditory features or uncertainty about mode of perception.

Conclusions. This is the first study to systematically explore voice-hallucinations in deaf people and to advance a model of subvocal articulation to account for such counterintuitive phenomena.     

Fitness Among Individuals with Early Childhood Deafness: Studies in Alumni Families from Gallaudet University

The genetic fitness of an individual is influenced by their phenotype, genotype and family and social structure of the population in which they live. It is likely that the fitness of deaf individuals was quite low in the Western European population during the Middle Ages. The establishment of residential schools for deaf individuals nearly 400 years ago resulted in relaxed genetic selection against deaf individuals which contributed to the improved fitness of deaf individuals in recent times. As part of a study of deaf probands from Gallaudet University, we collected pedigree data, including the mating type and the number and hearing status of the children of 686 deaf adults and 602 of their hearing siblings. Most of these individuals had an onset of severe to profound hearing loss by early childhood. Marital rates of deaf adults were similar to their hearing siblings (0.83 vs. 0.85). Among married individuals, the fertility of deaf individuals is lower than their hearing siblings (2.06 vs. 2.26, p = 0.005). The fitness of deaf individuals was reduced (p = 0.002). Analysis of fertility rates after stratification by mating type reveals that matings between two deaf individuals produced more children (2.11) than matings of a deaf and hearing individual (1.85), suggesting that fertility among deaf individuals is influenced by multiple factors.     

Clinical evaluation and etiologic diagnosis of hearing loss: A clinical practice resource of the American College of Medical Genetics and Genomics (ACMG)

Hearing loss is a common and complex condition that can occur at any age, can be inherited or acquired, and is associated with a remarkably wide array of etiologies. The diverse causes of hearing loss, combined with the highly variable and often overlapping presentations of different forms of hearing loss, challenge the ability of traditional clinical evaluations to arrive at an etiologic diagnosis for many deaf and hard-of-hearing individuals. However, identifying the etiology of hearing loss may affect clinical management, improve prognostic accuracy, and refine genetic counseling and assessment of the likelihood of recurrence for relatives of deaf and hard-of-hearing individuals. Linguistic and cultural identities associated with being deaf or hard-of-hearing can complicate access to and the effectiveness of clinical care. These concerns can be minimized when genetic and other health care services are provided in a linguistically and culturally sensitive manner. This clinical practice resource offers information about the frequency, causes, and presentations of hearing loss and suggests approaches to the clinical and genetic evaluation of deaf and hard-of-hearing individuals aimed at identifying an etiologic diagnosis and providing informative and effective patient education and genetic counseling.     

American College of Medical Genetics and Genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss

Hearing loss is a common and complex condition that can occur at any age, can be inherited or acquired, and is associated with a remarkably wide array of etiologies. The diverse causes of hearing loss, combined with the highly variable and often overlapping presentations of different forms of hearing loss, challenge the ability of traditional clinical evaluations to arrive at an etiologic diagnosis for many deaf and hard-of-hearing individuals. However, identifying the etiology of a hearing loss may affect clinical management, improve prognostic accuracy, and refine genetic counseling and assessment of the likelihood of recurrence for relatives of deaf and hard-of-hearing individuals. Linguistic and cultural identities associated with being deaf or hard of hearing can complicate access to and the effectiveness of clinical care. These concerns can be minimized when genetic and other health-care services are provided in a linguistically and culturally sensitive manner. This guideline offers information about the frequency, causes, and presentations of hearing loss and suggests approaches to the clinical evaluation of deaf and hard-of-hearing individuals aimed at identifying an etiologic diagnosis and providing informative and effective patient education and genetic counseling.Genet Med 2014:16(4):347–355.     

Visual memory for shapes in deaf signers and nonsigners and in hearing signers and nonsigners: atypical lateralization and enhancement

Deaf and hearing individuals who either used sign language (signers) or not (nonsigners) were tested on visual memory for objects and shapes that were difficult to describe verbally with a same/different matching paradigm. The use of 4 groups was designed to permit a separation of effects related to sign language use (signers vs. nonsigners) and effects related to auditory deprivation (deaf vs. hearing). Forty deaf native signers and nonsigners and 51 hearing signers and nonsigners participated in the study. Signing individuals (both deaf and hearing) were more accurate than nonsigning individuals (deaf and hearing) at memorizing shapes. For the shape memory task but not the object task, deaf signers and nonsigners displayed right hemisphere (RH) advantage over the left hemisphere (LH). Conversely, both hearing groups displayed a memory advantage for shapes in the LH over the RH. Results indicate that enhanced memory performance for shapes in signers (deaf and hearing) stems from the visual skills acquired through sign language use and that deafness, irrespective of language background, leads to the use of a visually based strategy for memory of difficult-to-describe items.     

The role of auditory feedback in the vocalizations of cats

Summary The vocalizations of deaf cats were compared with those of littermate hearing controls at 30 days, 50 days, 1 year and 3 years of age. At all ages, deaf cats called more loudly than hearing animals. At 30 days, 50 days, and 3 years, deaf cats called about twice as loudly as hearing animals while at 1 year the calls of the deaf animals were approximately 6 times louder than those of the hearing littermates. Analysis of variance revealed significant differences in call loudness between deaf and hearing animals at 30 days, 1, and 3 years. Deaf and hearing animals did not differ in rate of calling or in the duration of individual vocalizations at 30 days, 50 days, and 1 year. At 3 years, the calls of the deaf animal were shorter than those of the hearing control. The calls of deaf animals were less variable than those of hearing animals at 30 days, 50 days, and 3 years. There was a tendency for the fundamental frequency of the calls of deaf animals to be higher than that of hearing animals at 30 days, 50 days, and 1 year. These results document the importance of auditory feedback in the regulation of feline vocalization.     

Deafness in adults—screening in general practice

A random sample of 1,083 people, drawn from the lists of two practices in Leeds, was used to estimate the prevalence of deafness among adults. A test for deafness (defined as failure to hear 35 decibels at 1000 Hz in one or both ears) using a portable audiometer proved simple to operate with little observer variation. Eight per cent of adults were found to be deaf. The prevalence of deafness increased with age and was lowest in social classes I and II; there was no significant difference between the sexes. Audiometry showed that 17 per cent of those who thought their hearing was abnormal had no recorded loss of hearing using the stated test and that 18 per cent of those who are deaf would be overlooked if the question “Do you think your hearing is normal?” was used for initial screening in general practice. Less than 20 per cent knew of any services or aids for the deaf apart from those available through general practitioners.     

聋人婚前耳聋基因检测及婚配生育情况调查分析

目的了解婚前聋人基因检测及婚配生育情况,为预防耳聋提供依据。方法对自愿接受基因检测的情侣耳聋基因突变进行检测。结果聋人婚配模式是15对聋人与聋人婚配的9对占60％;聋人与健听人婚配占26．67％;聋人与重听人结婚的占13．33％。其中9对聋与聋在婚前进行遗传咨询占60．O％,接受致聋基因检测的仅有3对占20．0％。生育正常12例后代,1例听力正常的女孩为GJB2235delc杂合突变携带者,1例男婴,重度耳聋为SLC26A4IVS7—2A〉G杂合突变。结论婚前进行常见耳聋基因检测,是对耳聋预防与出生缺陷干预的有效措施。     

A proposal for comprehensive newborn hearing screening to improve identification of deaf and hard-of-hearing children

Early intervention for newborns who are deaf or hard-of-hearing leads to improved language, communication, and social–emotional outcomes. Universal physiologic newborn hearing screening has been widely implemented across the United States with the goal of identifying newborns who are deaf or hard-of-hearing, thereby reducing time to diagnosis and intervention. The current physiologic newborn hearing screen is generally successful in accomplishing its goals but improvements could be made. In the past ten years, genetic testing has emerged as the most important etiological diagnostic test for evaluation of children with deafness and congenital cytomegalovirus has been recognized as a major cause of childhood deafness that may be treatable. A comprehensive newborn hearing screen that includes physiologic, genetic, and cytomegalovirus testing would have multiple benefits, including (1) identifying newborns with deafness missed by the current physiologic screen, (2) providing etiologic information, and (3) possibly decreasing the number of children lost to follow up. We present a framework for integrating limited genetic testing and cytomegalovirus screening into the current physiologic newborn hearing screening. We identify needed areas of research and include an overview of genome sequencing, which we believe will become available over the next decade as a complement to universal physiologic newborn hearing screening.     

Methylthioadenosine phosphorylase (MTAP) in hearing: gene disruption by chromosomal rearrangement in a hearing impaired individual and model organism analysis

Genes with a role in the auditory system have been mapped by genetic linkage analysis of families with heritable deafness and then cloned through positional candidate gene approaches. Another positional method for gene discovery is to ascertain deaf individuals with balanced chromosomal translocations and identify disrupted or disregulated genes at the site(s) of rearrangement. We report herein the use of fluorescence in situ hybridization (FISH) to map the breakpoint regions on each derivative chromosome of a de novo apparently balanced translocation, t(8;9)(q12.1;p21.3)dn, in a deaf individual. Chromosomal breakpoints were assigned initially by GTG-banding of metaphase chromosomes and then BAC probes chosen to map precisely the breakpoints by FISH experiments. To facilitate cloning of the breakpoint sequences, further refinement of the breakpoints was performed by FISH experiments using PCR products and by Southern blot analysis. The chromosome 9 breakpoint disrupts methylthioadenosine phosphorylase (MTAP); no known or predicted genes are present at the chromosome 8 breakpoint. Disruption of MTAP is hypothesized to lead to deafness due to the role of MTAP in metabolizing an inhibitor of polyamine synthesis. Drosophila deficient for the MTAP ortholog, CG4,802, were created and their hearing assessed; no hearing loss phenotype was observed. A knockout mouse model for MTAP deficiency was also created and no significant hearing loss was detected in heterozygotes for Mtap. Homozygous Mtap-deficient mice were embryonic lethal.     

聋童与正常儿童智力比较研究

取聋童和正常听力儿童各３２人，年龄１０～１２岁，按年龄、性别、城乡、父母教育程度、职业进行１：１匹配。智力测验采用聋人智力量表（ＴｈｅＩｎｔｅｌｌｉｇｅｎｃｅＳｃａｌｅｆｏｒｔｈｅＤｅａｆ，简称ＩＳＦＤ）。结果显示聋童智商为１０７，低于正常儿童（ＩＱ＝１１５），但差异不显著；在分测验中，聋童的分类、接龙和编码成绩显著低于正常儿童，其它分测验两组无显著差异。结果提示：（１）聋童的非言语智力水平与正常儿童相近；（２）聋童在抽象思维、眼—手协调和运动速度方面不及正常儿童；（３）城乡差别、父母教育程度等对聋童智力的发展有明显的影响。     

Analysis of the factors influencing the response of the skin to audio signals

Skin-hearing aid is a new type of electronic product, which can improve hearing for deaf patients. It is different from audiphones and cochlear implant. The instrument makes use of the effect of the skin response to audio signals. The working process of the instrument is as following. Firstly, the sound signal is converted to audio signal by microphone, then through the power amplifier and booster. Then the signal is transmitted to the brain via skin by electrodes. And finally the hearing is formed. As skin-hearing aid transmits signals through the skin by the electrodes, the intensity of the skin resistance becomes the main factor influencing the response of the skin to audio signal. Skin resistance depends mainly upon the stratum corneum. This article aims to discuss the factors affecting the skin resistance, such as the thickness of the stratum corneum, hydration level of stratum corneum, the relation of audio frequency and skin resistance, and the skin resistance of acupuncture points.     

Maternally inherited nonsyndromic hearing loss.

In this study we characterized clinically and evaluated molecularly a large family with maternally inherited hearing impairment. Relatives were evaluated audiologically and clinically, the most likely pattern of inheritance was deduced, and molecular DNA analysis for the known mitochondrial mutations associated with hearing impairment was performed. Clinical examination of several relatives showed a normal general state of health, but in 14 of the members tested variable degrees of sensorineural hearing loss were noted. The pedigree was established and demonstrated a clear pattern of maternal inheritance, with 34 of 38 offspring of deaf mothers being hearing impaired, but none of 22 offspring of deaf fathers having any hearing impairment. Since by far the most likely explanation of such a maternal inheritance pattern is a mitochondrial mutation, molecular testing for the three known mitochondrial mutations, A1555G, A7445G, and Cins7472, was performed on 27 of the relatives. All of the individuals tested had the normal sequence at the sites tested. This family with nonsyndromic sensorineural hearing loss has an inheritance pattern strongly suggestive of a mitochondrial mutation. However, molecular testing for the three known mitochondrial mutations associated with nonsyndromic hearing impairment was negative, implying that additional molecular defects can lead to the same phenotype. The search for this novel molecular defect is underway.     

A workshop for medical students on deafness and hearing impairments.

There is a current need to improve health care delivery to deaf and hearing-impaired persons. The author designed an educational workshop for medical students and others as an initial step to address this need. The workshop was offered electively during 1997 and 1998 to first-year and second-year medical students at Dalhousie University, Nova Scotia, Canada. The workshop involved a broad, multidisciplinary scope, may have been the first of its kind in Canada, and is still one of the few documented ways to approach medical education about deafness and hearing impairments. Attendees explored general information on hearing impairments, communication between the hearing-impaired patient and his or her physician, and multicultural, technological, and ethical aspects of caring for hearing-impaired patients. There was an initial questionnaire, group exercises, lectures, student interviews of volunteer deaf "patients," discussions, and a "hands-on" materials display. The workshop was a low-cost and easily reproducible method of educating medical students about hearing impairments. If found to be educationally effective through future research, this type of workshop may foster better care to deaf and hearing-impaired persons by inclusion into medical school and continuing education curricula.     

Response speed advantage for vision does not extend to touch in early deaf adults

Early deaf adults typically respond faster than hearing controls when performing a speeded simple detection on visual targets. Whether this response time advantage can generalise to another intact modality (touch) or it is instead specific to visual processing remained unexplored. We tested eight early deaf adults and twelve hearing controls in a simple detection task, with visual or tactile targets delivered on the arms and occupying the same locations in external space. Catch trials were included in the experimental paradigm. Results revealed a response time advantage in deaf adults compared to hearing controls, selectively for visual targets. This advantage did not extend to touch. The number of anticipation errors was negligible and comparable in both groups. The present findings strengthen the notion that response time advantage in deaf adults emerges as a consequence of changes specific to visual processing. They also exclude the involvement of sensory-unspecific cognitive mechanisms in this improvement (e.g. increased impulsivity in initiation of response, longer-lasting sustained attention or higher motivation to perform the task). Finally, they provide initial evidence that the intact sensory modalities can reorganise independently from each other following early auditory deprivation.     

A multi-channel hearing prosthesis for profound-to-total hearing loss

A multi-channel cochlear implant hearing prosthesis providing 22 separate channels of stimulation has been developed. The electronics for the implantable receiver-stimulator have been incorporated on a single chip, using digital circuits and employing CMOS technology. The chip is enclosed in a titanium capsule with platinum/ceramic electrode feed-throughs. A pocket-sized speech processor and directional microphone extract the following speech parameters: signal amplitude, fundamental frequency and formant frequency. The fundamental frequency is coded as electric pulse rate, and formant frequency by electrode position. The speech processor has been realized using hybrid circuits and CMOS gate arrays. The multi-channel prosthesis has undergone a clinical trial on four postlingually deaf patients with profound-total hearing losses. The speech perception results indicate that they were able to obtain open-set speech recognition scores for phonetically balanced words, CID sentences and spondees. In all cases the tests showed significant improvements when using the cochlear prosthesis combined with lipreading compared to lipreading alone.     

Four years of experience with cochlear prostheses

Within the last four years, clinical as well as engineering experience has been gathered with 12 deaf persons who received several versions of an auditory prosthesis. Two years ago we settled on a system consisting of a versatile, passive 4-channel implant driving a scala tympani electrode, and a small external speech-processor. The main characteristics of the prosthetic hearing which can thus be established are: dynamic ranges vary between 12 and 20 dB, the number of discriminable amplitude steps within the dynamic range is comparable to that of a normal-hearing person; subjective pitch increases with stimulation frequency up to at least 1,000 Hz. Thresholds and dynamic ranges as well as a number of other characteristics remain stable over long periods of time. After an initial big step to good open speech understanding by one of our patients it could now be shown, that this patient is not an exemption. Several deaf patients participating in a series of speech tests have demonstrated that it is possible to reestablish some understanding of open speech through the use of the cochlear prosthesis without additional lipreading. A program has been established which aims at achieving experience with this prosthesis in a larger number of deaf people. The program includes: implant candidate selection, implantation, adjustment of speech-processor, hearing and speech rehabilitation, and the collection of comparative "psychoelectric" characteristics.     

Maternally inherited nonsyndromic hearing loss

In this study we characterized clinically and evaluated molecularly a large family with maternally inherited hearing impairment. Relatives were evaluated audiologically and clinically, the most likely pattern of inheritance was deduced, and molecular DNA analysis for the known mitochondrial mutations associated with hearing impairment was performed. Clinical examination of several relatives showed a normal general state of health, but in 14 of the members tested variable degrees of sensorineural hearing loss were noted. The pedigree was established and demonstrated a clear pattern of maternal inheritance, with 34 of 38 offspring of deaf mothers being hearing impaired, but none of 22 offspring of deaf fathers having any hearing impairment. Since by far the most likely explanation of such a maternal inheritance pattern is a mitochondrial mutation, molecular testing for the three known mitochondrial mutations, A1555G, A7445G, and Cins7472, was performed on 27 of the relatives. All of the individuals tested had the normal sequence at the sites tested. This family with nonsyndromic sensorineural hearing loss has an inheritance pattern strongly suggestive of a mitochondrial mutation. However, molecular testing for the three known mitochondrial mutations associated with nonsyndromic hearing impairment was negative, implying that additional molecular defects can lead to the same phenotype. The search for this novel molecular defect is underway. Am. J. Med. Genet. 84:369–372, 1999. © 1999 Wiley-Liss, Inc.     

Auditory deprivation affects biases of visuospatial attention as measured by line bisection

In this study, we investigated whether early deafness affects the typical pattern of hemispheric lateralization [i.e., right hemisphere (RH) dominance] in the control of spatial attention. To this aim, deaf signers, deaf non-signers, hearing signers, and hearing non-signers were required to bisect a series of centrally presented visual lines. The directional bisection bias was found to be significantly different between hearing and deaf participants, irrespective of sign language use. Hearing participants (both signers and non-signers) showed a consistent leftward bias, reflecting RH dominance. Conversely, we observed no evidence of a clear directional bias in deaf signers or non-signers (deaf participants overall showing a non-significant tendency to deviate rightward), suggesting that deafness may be associated to a more bilateral hemispheric engagement in visuospatial tasks.