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PubMed1983

[Results of 10 years of deafness screening and diagnosis in children and its implications. Early education of the young deaf child].

In ten years, we have tested more than 1 200 children of 0 to 4 years in this way: at birth, within the maternity hospital, and then at 2, 6 and 9 months-old, doubtful cases with the Veit-Bizaguet audiometer and also Mrs Borel-Maisonny's sound toys: we have thus obtained the child's hearing test. Later on, according to age, we use R.O.C.--Peep-Show, tonal audiometry, and vocal audiometry with pictures. We also resort to objective means: systematically, tympanometry and Stape Reflex, and in difficult, doubtful cases, psychomotor retardation and autistic behaviour, we make B.E.R.A. We also search for hereditary, iatrogen and meningitic deafness. Such screening methods, at an early stage, to trace down severe and profound deafness enabled a demutization and early education of deaf children who will be equipped with stereophonic hearing aids; we use only an oral method with some cued speech. The children will thus be given a good school and social integration within the world of normal people. Then the children are in possession of a more structural language, of a voice of better quality and can develop all their abilities.

Age Factors↗
PubMed2006

Language development in deaf children's interactions with deaf and hearing adults: a Dutch longitudinal study.

The language development of two deaf girls and four deaf boys in Sign Language of the Netherlands (SLN) and spoken Dutch was investigated longitudinally. At the start, the mean age of the children was 3;5. All data were collected in video-recorded semistructured conversations between individual children and deaf and hearing adults. We investigated the lexical richness and syntactic complexity of the children's utterances in SLN and spoken Dutch, as well as language dominance and interactional participation. Richness and complexity increase over time, as well as children's participation. An important outcome is that syntactic complexity is higher in utterances with both sign and speech. SLN does not have higher outcomes on richness or complexity, but is dominant in terms of frequency of use.

Adult↗
PubMed1984

[Conduction deafness and collapse of the auditory meatus. Value and limitations of the stapedial reflex in conduction deafness].

Collapse of the auditory meatus constitutes an audiometric trap provoking a false transmission deafness. It can also permanently affect audition, whether it be primary in origin or post-mastoidectomy. The association of conservation of the stapedial reflex and transmission deafness is noticed immediately, but this is in fact compatible with an interruption in the chain by incus luxation. These notions must be known in order to avoid operation in false transmission deafness, and inversely to decide when to explore the cavity in certain post-mastoidectomy sequelae with meatal collapse.

Adolescent↗
PubMed1982

Cochlear nerve fiber populations in 25 patients with profound deafness due to Menière's disease or sudden deafness.

Surgical biopsy specimens of the cochlear nerve from 19 patients with Menière's disease and six patients with sudden deafness were studied. The total number of myelinated nerve fibres in the transverse fascicular area of each nerve were counted and the results were correlated with the clinical and preoperative audiological data. The total number of cochlear neurons in patients with Menière's disease ranged from 5,760 to 27,722 and in patients with sudden deafness from 8,500 to 22,714. The long duration of the disease or absent speech discrimination appeared not to be connected with lower numbers of neurons than preserved speech discrimination or short history of the disease.

Adult↗
PubMed2000

"Deaf hearing": unacknowledged detection of auditory stimuli in a patient with cerebral deafness.

We describe a patient with the rare disorder of total deafness caused by a bilateral lesion in the temporal lobes and lesions in the central pontine area. Although she displayed no voluntary ability to detect or localize or identify sounds and denied hearing them when asked in writing, she retained some ability to respond reflexively to sounds. When attempts were made to restore awareness of sounds and/or voluntary responses to sounds by drawing her attention to her appropriate orienting head movements her performance improved and she began to respond successfully in a "forced-choice" paradigm. However, even when she became confident at detecting and localizing sounds she remained densely agnosic to their meaning. Her condition of deaf-hearing bears many similarities to that of blindsight.

Acoustic Stimulation↗
PubMed2003

Claudin 14 knockout mice, a model for autosomal recessive deafness DFNB29, are deaf due to cochlear hair cell degeneration.

Tight junctions (TJs) create ion-selective paracellular permeability barriers between extracellular compartments. In the organ of Corti of the inner ear, TJs of the reticular lamina separate K(+)-rich endolymph and Na(+)-rich perilymph. In humans, mutations of the gene encoding claudin 14 TJ protein cause profound deafness but the underlying pathogenesis is unknown. To explore the role of claudin 14 in the inner ear and in other tissues we created a mouse model by a targeted deletion of Cldn14. In the targeted allele a lacZ cassette is expressed under the Cldn14 promoter. In Cldn14-lacZ heterozygous mice beta-galactosidase activity was detected in cochlear inner and outer hair cells and supporting cells, in the collecting ducts of the kidney, and around the lobules of the liver. Cldn14-null mice have a normal endocochlear potential but are deaf due to rapid degeneration of cochlear outer hair cells, followed by slower degeneration of the inner hair cells, during the first 3 weeks of life. Monolayers of MDCK cells expressing claudin 14 show a 6-fold increase in the transepithelial electrical resistance by decreasing paracellular permeability for cations. In wild type mice, claudin 14 was immunolocalized at hair cell and supporting cell TJs. Our data suggest that the TJ complex at the apex of the reticular lamina requires claudin 14 as a cation-restrictive barrier to maintain the proper ionic composition of the fluid surrounding the basolateral surface of outer hair cells.

Animals↗
PubMed2002

An analysis of teacher preparation in deaf education: programs approved by the Council on Education of the Deaf.

An analysis of the most recent self-study reports of the 46 teacher preparation programs approved by the Council on Education of the Deaf yielded data on program demographics, curriculum and content, faculty, practicum, students, and graduates. Only 4 of the 46 teacher preparation programs had been established since 1980. Thirty-nine offered comprehensive teacher preparation, five offered auditory/oral, and two offered preparation in bilingual-bicultural education. Thirty-three offered two or more specializations, of which elementary (96%) and secondary (52%) were the most common. Students' practicum experiences often did not coincide with employment after graduation. The study reported a median of 2 full-time faculty per program, who taught 75% of the courses. Although adjuncts taught 25% of the courses in the deaf education program, they made up 75% of the faculty. The study's results indicate extreme diversity among the programs and great breadth and complexity within them. Compared with a 1986 survey, the number and level of the programs appears to be declining while the number of specializations and program length are increasing.

Deafness↗
PubMed1995

Technical report: service provision under the Individuals with Disabilities Education Act-Part H, as Amended (IDEA-Part H) to children who are deaf and hard of hearing ages birth to 36 months. Joint Committee of American Speech-Language-Hearing Association and the Council on Education of the Deaf.

At the request of the Council on Education of the Deaf (CED) and the American Speech-Language-Hearing Association (ASHA), the Annals is publishing in its entirety and verbatim the report of a joint CED/ASHA committee designed to provide information to professionals about the services to deaf and hard of hearing children below three years of age and their families mandated by the Individuals with Disabilities Education Act-Part H, as Amended. This information should be of benefit to all professionals providing services through an individual family service plan. The report has also been published in the August, 1994 issue of the ASHA Journal.

American Speech-Language-Hearing Association↗
PubMed1977

[Treatment of acute inner ear deafness (sudden deafness and acoustic trauma) (author's transl)].

The principal facts concerning therapeutic measures in cases of sudden deafness and acoustic trauma are outlined. Experiment data concerning the pharmacology of inner ear circulation are presented. There is sufficient evidence for a critical evaluation of a few treatments whose effect seems to be proven (experimental increase of inner ear circulation in normal animals) or at least possible (in normal animals no or dubious increase; effective under pathological conditions only?). Experimental and clinical experiences concerning the dextran treatment of sudden deafness and acoustic trauma are reported.

Acute Disease↗
PubMed1985

[The role of cytomegalovirus in infantile deafness and deafness of unknown origin].

It is widely accepted that the cause of congenital deafness is genetic in one third of cases roughly, is due to acquired affections during pregnancy or delivery in another third and remains unknown in the last third. It is possible that the cytomegalovirus (CMV) plays an important role in the latter group. The CMV is thought to be involved in 10 to 30% of cases of auditory sequelae from fetal infection, either severe neonatal CMV-induced disease, which is rare, or the frequent subclinical infections affecting an average of 1% of newborn infants. The only certain way to determine the importance of the role of CMV in deafness of unknown etiology is large-scale neonatal biologic screening followed by long-term audiologic surveillance: currently available documented data suggest that this role is very important.

Antibodies, Viral↗
PubMedDate not supplied

Deafness in childhood: 1. Diagnosis and treatment of deaf babies and children.

Recent technological advances make it possible to detect hearing impairments in very young babies. Babies as young as a few weeks old can now be helped with hearing aids and early language therapy. The earlier the diagnosis and start of treatment, the better the later performance in speech. Nurses and midwives, together with the parents, are often the first to suspect a baby has a hearing loss. Bear in mind the risk factors--low birth weight, neonatal jaundice, family history of deafness etc-and if in doubt, always refer. There are different degrees of hearing loss and it is possible to be misled into thinking a child with partial hearing can hear normally. Again, if in doubt, refer. Hearing aids are essential for almost all children with sensori-neural hearing loss and can also be helpful for moderate conductive hearing loss. Remember that while some parents are the first to suspect deafness and seek treatment, others may deny their suspicions and even refuse to accept a diagnosis of hearing loss. They want to be able to believe that their child is normal.

Child, Preschool↗
PubMed2006

Extremely low penetrance of deafness associated with the mitochondrial 12S rRNA mutation in 16 Chinese families: implication for early detection and prevention of deafness.

Mutations in mitochondrial DNA (mtDNA) have been found to be associated with sensorineural hearing loss. We report here the clinical, genetic, and molecular characterization of 16 Chinese pedigrees (a total of 246 matrilineal relatives) with aminoglycoside-induced impairment. Clinical evaluation revealed the variable phenotype of hearing impairment including audiometric configuration in these subjects, although these subjects share some common features: being bilateral and sensorineural hearing impairment. Strikingly, these Chinese pedigrees exhibited extremely low penetrance of hearing loss, ranging from 4% to 18%, with an average of 8%. In particular, nineteen of 246 matrilineal relatives in these pedigrees had aminoglycoside-induced hearing loss. Mutational analysis of the mtDNA in these pedigrees showed the presence of homoplasmic 12S rRNA A1555G mutation, which has been associated with hearing impairment in many families worldwide. The extremely low penetrance of hearing loss in these Chinese families carrying the A1555G mutation strongly supports the notion that the A1555G mutation itself is not sufficient to produce the clinical phenotype. Children carrying the A1555G mutation are susceptible to the exposure of aminoglycosides, thereby inducing or worsening hearing impairment, as in the case of these Chinese families. Using those genetic and molecular approaches, we are able to diagnose whether children carry the ototoxic mtDNA mutation. Therefore, these data have been providing valuable information and technology to predict which individuals are at risk for ototoxicity, to improve the safety of aminoglycoside therapy, and eventually to decrease the incidence of deafness.

Adolescent↗
PubMed2001

[Central and peripheral deafness among autistic and deaf children].

Deafness concerns periphericals etiology but also central and psychics etiologies. Knowing the level and the mechanism is difficult with the best ORL exams. Amplification is a possibility to diagnose the reality of the lack of hearing with caution and repetition. It prepares to the use of a hearing aid which can obtain the diagnosis.

Auditory Diseases, Central↗
PubMed1984

Etiology of deafness in Robarts School for the Deaf.

A survey of the children at the Robarts School for the Deaf was performed in order to determine the etiology of hearing loss, the reading ability as related to the degree of hearing loss, and the incidence of middle ear disease. The largest category for etiology was idiopathic at 31%, followed by hereditary at 22.5%, and maternal rubella at 19%. The incidence of serous otitis media was found to be 11%.

Adolescent↗
PubMed1998

[Perform vestibular test among all small deaf children! Early detection of Usher syndrome improves the possibilities of communication in the event of later deaf-blindness].

Although deaf children depend upon sight for communication, retinitis pigmentosa is present in 6 per cent of those with a profound hearing deficit. Usher syndrome type 1 (USH-1) is an autosomal recessive disease characterised by profound hearing impairment, absent vestibular function, and progressive loss of vision due to retinitis pigmentosa. Owing to the severe handicap of the combined disorders, early diagnosis of USH-1 is of crucial importance. The profound hearing deficit is often detected at hearing tests in infancy, but as the sight problems do not develop until later diagnosis is often delayed. The diagnosis of Usher's syndrome is established by electroretinography (ERG), as confirmation of the retinitis pigmentosa is a prerequisite. As the progressive deterioration of sight hinders the use of sign language, a cochlear implant constitutes an invaluable aid at an early age. Early diagnosis is thus of both medical and educative importance. To avoid delay in diagnosis past the age when optimal results may be expected from a cochlear implant, a screening test was sought for infants with profound hearing loss to identify those at increased risk of USH-1, for whom ERG is a priority. Although conventional vestibular testing methods are not well tolerated by infants, with recently developed equipment for video or CCI) (charge-coupled device) camera inspection of eye movements, vestibular screening can now be performed in the office. In our experience it is possible to screen most infants, given a careful approach by the investigator. Children with clinically diagnosed USH-1, without any vestibulo-ocular reflex, manifested no vestibular responses during 'video-nystagmoscopy' when rotated on the parent's lap, preliminary findings strongly suggest that vestibular function should be tested in all children with a profound congenital hearing deficit, as it is important for children with USH-1 to be provided with a cochlear implant permitting aural reception of communication from the environment at an early age, because of the progressive deterioration of vision and the ultimate double handicap.

Blindness↗