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.

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.

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 Usher syndrome Type I in an adult Nepalese male: a rare case report

2018 ◽  
Vol 9 (2) ◽  
pp. 203-205
Author(s):  
Sabin Sahu ◽  
Sanjay Kumar Singh
Keyword(s):  
Hearing Loss ◽  
Retinitis Pigmentosa ◽  
Genetic Disorder ◽  
Usher Syndrome ◽  
Type I ◽  
Syndrome Type ◽  
Profound Hearing Loss ◽  
Rare Case Report ◽  
Different Populations ◽  
Usher Syndrome Type

Usher syndrome, also known as retinitis pigmentosa-dysacusis syndrome, is an extremely rare genetic disorder, characterized by retinitis pigmentosa (RP) and congenital sensorineural hearing loss. It has been estimated to account for 3-6% of the congenitally deaf population, upto 8-33% of individuals with RP and half of all cases with combined deafness and blindness (Vernon M,1969; Boughman JA et al,1983). The prevalence of Usher syndrome have been reported to range from 3.5 to 6.2 per 100,000 in different populations (Vernon M,1969; Boughman JA et al,1983; Yan D et al, 2010).We report a case of Usher syndrome type I in an adult Nepalese male with typical congenital profound hearing loss, and night blindness secondary to retinitis pigmentosa.

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.

What Do We Want Them To Be When They Grow Up?

2010 ◽  
Vol 20 (1) ◽  
pp. 27-31
Author(s):  
Lyn Robertson
Keyword(s):  
Hearing Loss ◽  
Social Development ◽  
Young Women ◽  
Hearing Aids ◽  
Test Scores ◽  
Spoken Language ◽  
Language Therapy ◽  
Profound Hearing Loss ◽  
Children With Hearing Loss ◽  
Learning To Listen

Abstract Learning to listen and speak are well-established preludes for reading, writing, and succeeding in mainstream educational settings. Intangibles beyond the ubiquitous test scores that typically serve as markers for progress in children with hearing loss are embedded in descriptions of the educational and social development of four young women. All were diagnosed with severe-to-profound or profound hearing loss as toddlers, and all were fitted with hearing aids and given listening and spoken language therapy. Compiling stories across the life span provides insights into what we can be doing in the lives of young children with hearing loss.

scholarly journals Perception and learning of relative pitch by musicians using the vibrotactile mode

2021 ◽  
pp. 102986492110152
Author(s):  
Carl Hopkins ◽  
Saúl Maté-Cid ◽  
Robert Fulford ◽  
Gary Seiffert ◽  
Jane Ginsborg
Keyword(s):  
Hearing Loss ◽  
Music Education ◽  
Success Rate ◽  
Normal Hearing ◽  
Pitch Discrimination ◽  
Professional Musicians ◽  
Profound Hearing Loss ◽  
Relative Pitch ◽  
Significant Difference ◽  
Schools For The Deaf

This study investigated the perception and learning of relative pitch using vibrotactile stimuli by musicians with and without a hearing impairment. Notes from C3 to B4 were presented to the fingertip and forefoot. Pre- and post-training tests in which 420 pairs of notes were presented randomly were carried out without any feedback to participants. After the pre-training test, 16 short training sessions were carried out over six weeks with 72 pairs of notes per session and participants told whether their answers were correct. For amateur and professional musicians with normal hearing and professional musicians with a severe or profound hearing loss, larger pitch intervals were easier to identify correctly than smaller intervals. Musicians with normal hearing had a high success rate for relative pitch discrimination as shown by pre- and post-training tests, and when using the fingertips, there was no significant difference between amateur and professional musicians. After training, median scores on the tests in which stimuli were presented to the fingertip and forefoot were >70% for intervals of 3–12 semitones. Training sessions reduced the variability in the responses of amateur and professional musicians with normal hearing and improved their overall ability. There was no significant difference between the relative pitch discrimination abilities between one and 11 semitones, as shown by the pre-training test, of professional musicians with and without a severe/profound hearing loss. These findings indicate that there is potential for vibration to be used to facilitate group musical performance and music education in schools for the deaf.

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