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PubMed1997

Speech perception by prelingually deaf children and postlingually deaf adults with cochlear implant.

We review recent data from the University of Iowa obtained from prelingually deaf children over 3 years using feature-extraction versions of the Nucleus cochlear implant, and from postlingually deaf adults using the compressed-analog Ineraid, a feature-extraction version of Nucleus, and a continuous interleaved sampling version of the Clarion cochlear implant. Both adults and children were followed over a 3-year period. Average results are shown where each patient is represented at each point in time. Results for the children show continued improvement over the 3-year period, with some children obtaining maximum scores possible on the easier closed-set tests. Substantial improvements in audiovisual enhancement were provided by the cochlear implant. Results from the adults show maximum gains within the first year of implant use, although some patients show continued improvements after 2 years of implant use. Adults who have been deaf for several years and who receive their implant when they are older tend not to perform as well as adults who have been deaf for only a few years or receive their implant when they are younger.

Adult↗
PubMed2006

Cochlear alterations in deaf and unaffected subjects carrying the deafness-associated A1555G mutation in the mitochondrial 12S rRNA gene.

The A1555G mutation in the mitochondrial small ribosomal RNA gene (12S rRNA) has been associated with aminoglycoside-induced, nonsyndromic hearing loss. However, the clinical phenotype of A1555G carriers is extremely variable. In the present study, we have performed an audiological evaluation of a group of deaf patients and hearing carriers of mutation A1555G with the aim to assess the prevalence of the mutation and determine the associated cochlear alterations. Fifty-four patients affected of nonsyndromic hearing loss were screened for the presence of the A1555G mitochondrial mutation. Nine of the familial cases (21%) carried the A1555G mutation, whereas the mutation was not found in any of the sporadic cases. The positive cases and some of their family members underwent a clinical study consisting in a clinical evaluation and audiological testing. The phenotype of A1555G patients varied in age of onset and severity of hearing loss, ranging from profound deafness to completely normal hearing. The audiometric alterations showed bilateral hearing loss, being more severe at high frequencies. Otoacoustic emissions were absent in deaf A1555G carriers, and auditory brainstem response indicated a prolonged Wave I, suggesting a cochlear dysfunction without any effect of the auditory nerve. Moreover, all hearing carriers of A1555G also presented alterations in cochlear physiology. In conclusion, the A1555G mitochondrial mutation causes a cochlear form of deafness, characterized by a more severe loss of hearing at high frequencies. Although the expression of the mutation is variable, cochlear alterations are present in all carriers of mutation A1555G.

Adult↗
PubMed1997

Learning to look in the right place: a comparison of attentional behavior in deaf children with deaf and hearing mothers.

Eleven 18-month-old children with profound prelingual hearing loss were video-recorded in a free-play session with their mothers. Five of the mothers were profoundly deaf and fluent users of British Sign Language (BSL) or Auslan. The other six were hearing and had enrolled in a signing program. Ten-minute segments from each session were analyzed to determine the number of switches in attention shown by each child. Switches in attention were subdivided into three categories: spontaneous (where the child spontaneously looked to the mother); responsive (where the child responded to some maternal action such as moving an object); and elicited (where the mother made a direct attempt to gain the child's attention. Failed attempts to gain attention were also noted. A comparison of deaf and hearing mothers revealed no difference in the proportion of spontaneous or responsive switches in attention shown by their children. Responsive switches were by far the most frequent category for both groups, but these most commonly focused on objects and did not provide an opportunity for maternal signing. Successful perception of signing typically followed from spontaneous or elicited attentional switches. Deaf mothers were generally more insistent on their children turning to look at them, and they were more successful in eliciting attentional switches although they also had more failed attempts. These overall differences between the two groups were overshadowed by large individual differences within the groups. Within the sample there were both deaf and hearing mothers who achieved successful signed communication with their children.

Journal Article↗
PubMed1998

Studies of neural processing in deaf signers: toward a neurocognitive model of language processing in the deaf.

The ability to comprehend and produce language stands as a defining characteristic of human cognition and enables the transfer of knowledge and culture within human society. A proper characterization of the human capacity for language is required for the development of interventions that may be used to assist those individuals who have failed to achieve, or who have lost competence in, language behaviors. For signed languages, models of competent language use are lacking. This lack of knowledge hampers the development of effective assessment measures for deaf children who may be experiencing learning problems beyond those confronting the normal deaf child. I discuss two research avenues that have begun to provide a window into the neural systems involved in sign language processing: studies of language disruptions in adult deaf signers who have suffered brain injury, and studies of functional brain imaging in normal deaf signers. This research provides a basis for the development of a comprehensive neurocognitive model of sign language processing.

Journal Article↗
PubMed2006

Does universal newborn hearing screening identify all children with GJB2 (Connexin 26) deafness? Penetrance of GJB2 deafness.

OBJECTIVE: Deafness is the most common neurosensory defect at birth, and GJB2 (connexin 26) mutations are the most frequent genetic cause of hearing loss in many populations. The hearing loss caused by GJB2 mutations is usually congenital in onset and moderate to profound in degree. Considerable phenotypic variation has been noted however, including two anecdotal cases of apparent non penetrance at birth. The objective of this study is to document nine additional children with two pathogenic GJB2 mutations who had non penetrance of hearing loss at birth. DESIGN: Subjects were identified through a national repository which includes deaf probands ascertained primarily from the United States through the Annual Survey of Deaf and Hard of Hearing Children and Youth conducted at the Research Institute at Gallaudet University. The hearing of each of these children had been screened at birth using standard audiologic techniques. Parents were interviewed and available medical records were reviewed. Testing for GJB2 mutations was performed by PCR and sequencing of the entire coding exon in all nine individuals. RESULTS: Using parent interviews and medical records, we documented that all nine children passed newborn audiologic hearing screening. The age at which the hearing loss was subsequently identified in these nine children ranged from 12-60 mo. Of these nine children, 3 were compound heterozygotes and six were homozygous for the 35delG mutation in the GJB2 gene. CONCLUSION: These nine cases demonstrate that current newborn hearing screening does not identify all infants with two GJB2 mutations. These cases suggest that the frequency of non penetrance at birth is approximately 3.8% or higher. It is important to consider connexin deafness in any child with recessive nonsyndromic hearing loss as well as simplex cases with no history of other affected family members even when the newborn hearing screening results were within the normal range.

Audiometry, Evoked Response↗
PubMed2005

Deaf people in Hitler's Europe: conducting oral history interviews with deaf Holocaust survivors.

Deaf people living in Europe between 1933 and 1945 were mistreated, forcibly sterilized, incarcerated, and murdered by the Nazis. Their stories have been overlooked or underappreciated because of the complexities of communication and the difficulties historians face gaining access to those communities. This article describes the challenges faced by two United States historians when they interviewed deaf Holocaust survivors in Budapest, Hungary and during a conference, "Deaf People in Hitler's Europe," co-sponsored by the United States Holocaust Memorial Museum and Gallaudet University. It also raises general questions of adapting methodologies to facilitate "oral" history interviews for deaf informants.

Europe↗
PubMed2003

An intersubjective developmental perspective on interactions between deaf and hearing mothers and their deaf infants.

Most research into interactions between mothers and their infants with hearing impairments focuses on mothers' and infants' behaviors separately, speculating about the interplay among these behaviors and their effects on child development. In the present article, an intersubjective developmental theory focusing on the development of the "interworld" between deaf and hearing mothers and their deaf infants is used to integrate and interpret the seemingly incoherent research on early mother-deaf child interaction. Inspired by Stern's work (e.g., Stern, 1985), the intersubjective developmental theory distinguishes four stages in the development of intersubjectivity: emerging (birth-2 months), physical (2-8 months), existential (8-13 months), and symbolic (13 months and older), each characterized by a different type of mother-infant interaction. The integration of research findings on early mother-deaf child interaction into these four developmental stages offers new perspectives that can advance research and resolve certain early-intervention issues.

Communication↗
PubMed1992

Phenotypic evidence for a common pathogenesis in X-linked deafness pedigrees and in Xq13-q21 deletion related deafness.

A structural cochlear abnormality has been observed by high resolution CT scanning in some families where X-linked deafness is segregating. We now present evidence that the same abnormality is present in a deaf patient who has a deletion within Xq21. This observation provides phenotypic evidence that the genotypic basis of deafness is the same in both patient groups. It is also likely that the perilymphatic fluid "gusher" abnormality may be common to both.

Child↗
PubMed1997

mdfw: a deafness susceptibility locus that interacts with deaf waddler (dfw).

The deaf waddler (dfw) mutation is a model system to study the biology of neuroepithelial hearing defects in mice. Here we describe the identification and characterization of a new allele of deaf waddler (dfw2J) and present evidence for a hearing susceptibility locus (mdfw) that interacts with dfw. We found that CBy-dfw2J/dfw2J homozygotes exhibit no discernible auditory brainstem responses (ABR) to sound pressure level stimuli up to 100 dB, indicating a profound deafness. Interestingly, the ABR in CBy-dfw2J/+ heterozygotes is also abnormal, showing age-dependent elevated thresholds characteristic of a progressive hearing loss. When outcrossed onto the CAST/Ei strain, only 24% of the F2 CBy/CAST-dfw2J/ + heterozygotes displayed increased ABR thresholds, suggesting that a second locus, controlling hearing function in dfw2J/+ heterozygotes, was segregating in the CBy/CAST-dfw2J intercross. By linkage analysis, we localized this locus (mdfw) to Chromosome 10, between markers D10Mit127 and D10Mit185, within a 4.0 +/- 1.1 cM genetic interval. All CBy/CAST-dfw2J/+ heterozygotes that develop hearing loss are homozygous for the CBy-derived recessive allele (mdfwC). In contrast, CBy/ CAST-dfw2J/+ heterozygotes expressing even a single copy of the CAST/Ei-derived mdfw allele (Mdfw) retain their normal hearing function. Our results reveal an epistatic relationship between the mdfw and the dfw genes and provide a model system to study nonsyndromic hearing loss in mice.

Alleles↗
PubMed1983

Childhood deafness in southern Africa. An aetiological survey of 3,064 deaf children.

We have completed a survey of the causes of deafness in 3,064 children with defective hearing who attend special schools in Southern Africa. Specific genetic or multifactorial syndromes were diagnosed in 7 per cent, familial undifferentiated deafness was recognized in 11 per cent, while in 25 per cent the deafness was acquired.

Africa, Southern↗
PubMed1979

Are social workers turning a deaf ear? A study of social services to the deaf.

This study assessed the quality and quantity of social services received by deaf persons residing in Salt Lake County, Utah. It was found that although a majority of human service agencies in the county have had some contact with deaf clients, only those agencies that have made an effort to develop programs especially for the deaf and employ someone who is skilled in sign language could deliver services effectively to this population.

Adult↗
PubMed1998

Lipomatous myelomeningocele, athyrotic hypothyroidism, and sensorineural deafness: a new form of syndromal deafness?

This case report describes a 4 year old boy with the unique triad of lipomatous myelomeningocele, congenital hypothyroidism secondary to thyroid agenesis, and sensorineural deafness. While associations between deafness and abnormal thyroid function and deafness and sacral lipoma have previously been described, the constellation of findings in this patient has not been reported.

Abnormalities, Multiple↗
PubMed1999

Removing barriers and building bridges: American deaf interns teaching Chinese deaf children.

Two deaf American student-teaching M.A. candidates did an internship at a school for the Deaf in China. This was the first such internship ever done in China. Critical procedures for establishing international internships are described; cultural and educational experiences and the accommodations made to guarantee the success of such internships are analyzed, including issues related to communication modality, educational philosophy, expectations for deaf learners, and teaching methods. The views of both interns, and those of two teacher educators one American and one Chinese, are included.

Adult↗
PubMed1997

Deaf-mutia. Responses to alienation by the deaf in the mid-nineteenth century.

The development of the deaf community in the mid-nineteenth century was intimately linked to schooling. Although a matrix of variables contributed, a major factor underlying the community's enduring strength was the alienation suffered by deaf persons in society. To counter this alienation, proposals emerged throughout the nineteenth century for the formation of a separate deaf state. The most significant of these was the plan of Flournoy in the late 1850s.

Attitude↗
PubMed1995

Strategies deaf mothers use when reading to their young deaf or hard of hearing children.

The purpose of the study was to examine behaviors utilized by deaf mothers when reading to their deaf or hard of hearing children aged three to five years. Six deaf mothers were video-taped while reading the same book. The videotapes were transcribed and coded by two observers. Data analysis revealed six specific strategies that the mothers used while reading. These included sign placement, text paired with signed demonstration, real world connection between text and child's experience, attention maintenance, physical demonstrations of character changes, and non-manual signals as questions.

Attention↗
PubMed2003

Deaf people at work: assessment of communication among deaf and hearing persons in work settings.

This paper focuses on the assessment of communication among deaf and hearing persons within work settings. The paper is organized into two sections. In the first section, findings are presented from an ethnographic study of deaf professionals who have achieved relative success in their employment. The results from this study provide the foundation for a model of communication in work settings, presented in the second section of the paper. This model uses an ecological approach, focusing on assessment of communication at the individual level as it occurs within the broader context of organizational culture, influenced by national trends regarding legal rights, technological advances, and social awareness/attitudes towards deafness.

Adaptation, Psychological↗
PubMed2002

Health care utilization and adults who are deaf: relationship with age at onset of deafness.

OBJECTIVES: To evaluate the health care utilization of a nationally representative sample of U.S. deaf adults while accounting for the age at onset of deafness, an indicator of linguistic and sociocultural group affiliation. DATA SOURCES/STUDY SETTING: Data from the 1990 to 1991 National Health Interview Surveys the most recent years the Hearing Supplement was administered. The data were collected during in-home interviews of a sample of the U.S. civilian noninstitutionalized population. STUDY DESIGN: Cross-sectional analyses comparing health-related measures of adults deafened before (prelingually) and after (postlingually) the age of 3 and those of a representative sample of the general population, adjusting for sociodemographics and health status. Key measures were physician visits and preventive health care services utilization. PRINCIPAL FINDINGS: Compared with the general population, prelingually deafened adults had fewer physician visits and were less likely to have visited a physician in the preceding 2 years, whereas postlingually deafened adults had more physician visits and were more likely to have visited a physician in the preceding 2 years. Postlingually deafened women were less likely to have had a mammogram within the previous 2 years. CONCLUSIONS: In terms of health care utilization, the deaf population is heterogeneous. Prelingually deafened adults' use of health care is similar to that of other language minority groups. Postlingually deafened adults' use of health care services appears similar to people with chronic illness. Future studies must distinguish different groups of people with hearing loss in order to identify barriers and monitor improvements in health care services access.

Adolescent↗
PubMedDate not supplied

[Surgically implanted electronic apparatus for the rehabilitation of total deafness and deaf-mutism].

An electronic device for multichannel cochlear implant is now available in case of total deafness and deaf-muteness. Any surgical technic of electrodes implantation may use this prosthesis. It consists in two parts, an outside package and an implanted receptor. The external device cut the sound in eight channels, whose frequencies correspond to those of the telephon bands. These eight sinusoidal waves are changed in pulses whose frequency and duration depend on the intensity of the initial signal. All these informations are multiplexed and injected in a high frequency wave to an antenna. Electromagnetic induction supplies through the skin the implanted receptor with power and informations. This receptor is preoperatively connected to the electrodes. With this device and with phoniatric training some speech recognition, and in case of deaf-mute patients voice improvement, may be obtained in some months.

Cochlear Nerve↗