Rescue from Hearing Loss in Usher's Syndrome

2013 ◽  
Vol 369 (18) ◽  
pp. 1758-1760 ◽  
Author(s):  
Karen B. Avraham
Keyword(s):  
Hearing Loss ◽  
Usher’S Syndrome

Hearing Loss Associated with Retinitis Pigmentosa

1985 ◽  
Vol 79 (9) ◽  
pp. 404-405 ◽  
Author(s):  
Adrienne Karp
Keyword(s):  
Hearing Loss ◽  
Retinitis Pigmentosa ◽  
Progressive Hearing Loss ◽  
Rehabilitation Programs ◽  
Usher’S Syndrome

Clients with retinitis pigmentosa and a mild, moderate, or progressive hearing loss may be unrecognized and unserved by our diagnostic and rehabilitation programs. They are often treated separately for their visual and hearing dysfunctions, with no dialogue between managing agencies. The purpose of this article is to offer professionals a description of this particular variation of Usher's syndrome, and appropriate guidelines for its identification.

Demographic Characteristics and Impairments of Louisiana Students with Usher's Syndrome

1987 ◽  
Vol 81 (3) ◽  
pp. 106-109
Author(s):  
S.C. Brown
Keyword(s):  
Hearing Loss ◽  
Retinitis Pigmentosa ◽  
Visual Impairment ◽  
Demographic Characteristics ◽  
Genetic Condition ◽  
Profound Hearing Loss ◽  
Usher’S Syndrome

Usher's syndrome is a genetic condition that is characterized by hearing loss and progressive blindness from retinitis pigmentosa. This article reports on a study of the impairments and demographic characteristics of 60 Louisiana students with Usher's syndrome that were analyzed to identify their interrelationships. The research found that although a substantial majority of the students had a manifested visual impairment and profound hearing loss, some of the students had less-than-profound hearing loss and no manifested visual impairment—a finding that corroborated the findings of previous studies. Moreover, the severity of these two impairments seems to be interrelated.

Progressive Hearing Loss in Usher's Syndrome

1989 ◽  
Vol 98 (11) ◽  
pp. 863-866 ◽  
Author(s):  
Seppo Karjalainen ◽  
Leena Pakarinen ◽  
Helena Kääriäinen ◽  
Markku Teräsvirta ◽  
Eero Vartiainen
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Auditory Threshold ◽  
Speech Discrimination ◽  
Threshold Values ◽  
Progressive Hearing Loss ◽  
Usher’S Syndrome

In 18 patients with Usher's syndrome, progressive hearing loss was verified audiologically in eight cases. Despite poor auditory threshold values and low speech discrimination scores, there was only one patient who could not communicate with speech. The possibility of hearing impairment being mainly progressive in Usher's syndrome is discussed.

Considerations in Diagnosing Usher's Syndrome: RP and Hearing Loss

1982 ◽  
Vol 76 (7) ◽  
pp. 258-261 ◽  
Author(s):  
Mccay Vernon ◽  
Joann A. Boughman ◽  
Linda Annala
Keyword(s):  
Hearing Loss ◽  
Retinitis Pigmentosa ◽  
Hearing Impairment ◽  
Clinical Problems ◽  
Usher’S Syndrome

The association of hearing loss and retinitis pigmentosa has been generally recognized as Usher's Syndrome, although variations in the syndrome have not been clearly delineated. The diagnosis of a progressive visual disease in a person with severe hearing impairment has devastating implications for the individual's future. This article reviews findings of this syndrome and suggests strategies for dealing with some of the clinical problems displayed by Ushers Syndrome patients.

scholarly journals Stable and Progressive Hearing Loss in Type 2A Usher's Syndrome

1996 ◽  
Vol 105 (12) ◽  
pp. 962-967 ◽  
Author(s):  
Annelies van Aarem ◽  
Alfred J. L. G. Pinckers ◽  
William J. Kimberling ◽  
Patrick L. M. Huygen ◽  
Elisabeth M. Bleeker-Wagemakers ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Pure Tone ◽  
Regression Coefficient ◽  
Significant Heterogeneity ◽  
Progressive Hearing Loss ◽  
Chromosome 1Q ◽  
Pure Tone Average ◽  
Significant Regression ◽  
Usher’S Syndrome

Audiograms were traced or additionally performed on 23 Usher's syndrome patients in 10 Dutch multi-affected families, all linked to chromosome 1q (USH2A locus). Serial audiograms, available in 13 patients, were used for a regression analysis of binaural pure tone average on age (follow-up, 9 to 32 years) to test for “significant progression,” ie, a significant regression coefficient, here called the “annual threshold increase” (ATI, expressed in decibels per year). A significant ATI (>1 dB/y) was observed in 3 patients. Analysis of variance of ATI demonstrated significant heterogeneity; hearing loss was either stable or progressive. This implies a significant clinical heterogeneity. A similar analysis performed on our progressive USH2A cases and “type III” cases previously reported by others (ATI of 1 to 5 dB/y), some of which were recently linked to chromosome 3q (USH3 locus), failed to show any significant heterogeneity in the progression of hearing loss.

Cochlear Implants in Young Children with Usher'S Syndrome

1995 ◽  
Vol 104 (9_suppl2) ◽  
pp. 342-345 ◽  
Author(s):  
N. M. Young ◽  
J. C. Johnson ◽  
M. B. Mets ◽  
T. C. Hain
Keyword(s):  
Hearing Loss ◽  
Early Diagnosis ◽  
Cochlear Implant ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Autosomal Recessive Disorder ◽  
Sensorineural Hearing ◽  
Special Importance ◽  
Sensory Deficits ◽  
Usher’S Syndrome

Usher's syndrome (US) is an autosomal recessive disorder characterized by congenital sensorineural hearing loss and retinitis pigmentosa. The majority of affected individuals have severe to profound sensorineural hearing loss and are therefore potential cochlear implant candidates. A goal of our otology program has been early identification and implantation of children with US. At our center, early diagnosis of US has been achieved by comprehensive ophthalmologic evaluation including electroretinography. Four of our 19 pediatric cochlear implant recipients with congenital sensorineural hearing loss were identified with US and implanted prior to the onset of signs or symptoms of visual loss. All children have received measurable benefit from cochlear implantation. In light of the dual sensory deficits present in US, the need for early diagnosis and cochlear implantation is of special importance in this population.

Retinitis Pigmentosa and Progressive Hearing Loss

1983 ◽  
Vol 48 (3) ◽  
pp. 308-314 ◽  
Author(s):  
Adrienne Karp ◽  
Frances Santore
Keyword(s):  
Hearing Loss ◽  
Retinitis Pigmentosa ◽  
Hearing Impairment ◽  
Case Histories ◽  
Progressive Hearing Loss ◽  
Pathologic Condition ◽  
Number Of Patients ◽  
Prelingual Deafness ◽  
Usher’S Syndrome

Much material has been written about the deaf-blind patient diagnosed as having Usher's syndrome, a pathologic condition involving hearing impairment and retinitis pigmentosa. Contrary to the accepted pattern of prelingual deafness in such cases, however, there are a number of patients who report a progressive, postlingual hearing loss associated with retinitis pigmentosa. These patients may suffer from a variation of classical Usher's syndrome. An attempt is made to verify this statement through presentation of case histories and audiologic findings. In addition, diagnostic and rehabilitative techniques employed in assisting patients with these dual impairments are offered.

scholarly journals Retinitis pigmentosa and deafness

1987 ◽  
Vol 80 (1) ◽  
pp. 17-20 ◽  
Author(s):  
R P Mills ◽  
D M Calver
Keyword(s):  
Hearing Loss ◽  
Retinitis Pigmentosa ◽  
Auditory System ◽  
The Other ◽  
Different Parts ◽  
Usher’S Syndrome

Seventeen patients with retinitis pigmentosa (RP) have been investigated audiologically. Of 9 found to have a significant hearing loss, 6 were examples of Usher's syndrome; these patients had a cochlear pattern of hearing loss. The other 3 were examples of Senior's syndrome, Kearne-Sayre syndrome and Lawrence-Moon-Biedle syndrome respectively. Two of these patients had absent stapedius reflexes. It is suggested that patients with different RP-deafness syndromes may have lesions in different parts of the auditory system.

Fluorocitrate Neural Dystrophy of the Guinea Pig Cochlea

1975 ◽  
Vol 33 ◽  
pp. 368-369
Author(s):  
G.J. Spector ◽  
C.D. Carr ◽  
I. Kaufman Arenberg ◽  
R.H. Maisel
Keyword(s):  
Hearing Loss ◽  
Hair Cells ◽  
Sodium Phosphate ◽  
Sensory Cells ◽  
Barium Salt ◽  
Perfusion Medium ◽  
Cochlear Hair Cells ◽  
Neural Degeneration ◽  
Sodium Phosphate Buffer ◽  
Cochlear Neurons

All studies on primary neural degeneration in the cochlea have evaluated the end stages of degeneration or the indiscriminate destruction of both sensory cells and cochlear neurons. We have developed a model which selectively simulates the dystrophic changes denoting cochlear neural degeneration while sparing the cochlear hair cells. Such a model can be used to define more precisely the mechanism of presbycusis or the hearing loss in aging man.Twenty-two pigmented guinea pigs (200-250 gm) were perfused by the perilymphatic route as live preparations using fluorocitrate in various concentrations (15-250 ug/cc) and at different incubation times (5-150 minutes). The barium salt of DL fluorocitrate, (C6H4O7F)2Ba3, was reacted with 1.0N sulfuric acid to precipitate the barium as a sulfate. The perfusion medium was prepared, just prior to use, as follows: sodium phosphate buffer 0.2M, pH 7.4 = 9cc; fluorocitrate = 15-200 mg/cc; and sucrose = 0.2M.

Teacher Identification of Elementary School Children with Hearing Loss

1978 ◽  
Vol 9 (1) ◽  
pp. 24-28 ◽  
Author(s):  
Richard H. Nodar
Keyword(s):  
Elementary School ◽  
Hearing Loss ◽  
School Children ◽  
Elementary School Children ◽  
Total Sample ◽  
Hearing Impaired ◽  
Teacher Interviews ◽  
Teacher Screening ◽  
Children With Hearing Loss ◽  
Hearing Problems

The teachers of 2231 elementary school children were asked to identify those with known or suspected hearing problems. Following screening, the data were compared. Teachers identified 5% of the children as hearing-impaired, while screening identified only 3%. There was agreement between the two procedures on 1%. Subsequent to the teacher interviews, rescreening and tympanometry were conducted. These procedures indicated that teacher screening and tympanometry were in agreement on 2% of the total sample or 50% of the hearing-loss group. It was concluded that teachers could supplement audiometry, particularly when otoscopy and typanometry are not available.

Sign in / Sign up

Export Citation Format

Share Document