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.

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.

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.

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.

Hearing Outcome of Stapes Surgery in NIENT, Bangladesh

2020 ◽  
Vol 26 (1) ◽  
pp. 31-36
Author(s):  
Md Zakaria Sarkar ◽  
AHM Ferdows Nur ◽  
Utpal Kumar Dutta ◽  
Muhammad Rafiqul Islam ◽  
Debabrota Roy ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cross Sectional Study ◽  
Surgical Trauma ◽  
Group 14 ◽  
Progressive Hearing Loss ◽  
Cross Sectional ◽  
Hearing Gain ◽  
Hearing Outcome

Objective: The aim of this study was to evaluate hearing outcome after stapedotomy in patients with Otosclerosis. Methods: This cross sectional study was carried out from July 2017 to January 2019 in National Institute of ENT, Unit V. About 22 patients with Otosclerosis were included in this study. Diagnosis of Otosclerosis was based on the history, medical status with Otoscopy, Tuning fork tests and Audiometric tests. We compiled data on the pre and post operative air-bone gap (ABG) at 0.5, 1, 2 KHZ. The ABG was Calculated using AC and BC thresholds on the same audiogram. Post operative hearing gain was then Calculated from the ABG before the operation minus the ABG of the last follow up examination Results: In this study most of the cases were age group 14-30 years (72.7%), female (54.5%). Most common symptoms was progressive hearing loss, tinnitus (77.8%).The average preoperative hearing loss in this study was (AC) was 48.31±7.68. The average post opt. hearing (AC) at follow up was 28.95±10.30 with an average hearing gain of 15.40±8.53 dB which was significant. The average pre-operative ABG was 28.99 dB ± 8.10. The average post opt. ABG was analyzed at 1 follow up showed ABG 13.18±8.09 dB which was found to be significant. Conclusion: Stapedotomy is an effective surgical procedure for the treatment of otosclerosis which leads to improvement in patient’s quality of life. A favorable hearing outcome can be obtained by the combination of experienced hands with minimal surgical trauma and appropriate surgical technique. Bangladesh J Otorhinolaryngol; April 2020; 26(1): 31-36

scholarly journals Associations Between Hearing Loss, Loneliness, and Social Isolation: A Systematic Review

2020 ◽  
Vol 4 (Supplement_1) ◽  
pp. 318-318
Author(s):  
Jonathan Suen ◽  
Aishwarya Shukla ◽  
Adele Goman ◽  
Carrie Price ◽  
Frank Lin ◽  
...  
Keyword(s):  
Older Adults ◽  
Hearing Loss ◽  
Social Isolation ◽  
Psychosocial Health ◽  
Cochrane Library ◽  
Cross Sectional ◽  
Design Assessment ◽  
Aural Rehabilitation ◽  
Rehabilitation Programs ◽  
Underlying Mechanisms

Abstract Hearing loss is highly prevalent among older adults, as is occurrences of loneliness and social isolation. Both loneliness and social isolation are also associated with insidious outcomes such as earlier mortality from all-causes and higher prevalence of chronic comorbidities. The purpose of this review is to synthesize published investigations that report on the associations between hearing loss with loneliness and social isolation. A systematic search through PubMed, Embase, CINAHL Plus, PsycINFO, and the Cochrane Library identified an initial total of 2495 references. Two independent reviewers screened articles for inclusion, with a third reviewer adjudicating. Studies published in English of older adults with hearing loss that also assessed loneliness and/or social isolation using a validated measure were included. Investigators used a modified Newcastle-Ottawa Scale (NOS) to appraise study quality. A final total of 14 articles were included in the review. The majority (12/14) were cross-sectional in design. Assessment methods were varied across hearing status, loneliness, and social isolation. Despite this heterogeneity, most multivariable adjusted investigations revealed that hearing loss was significantly associated with higher risks for both phenomena. Several studies also revealed this association to vary across gender, with women showing a stronger association than men. Our findings indicate that hearing loss is associated with both loneliness and social isolation, which have important implications for the cognitive and psychosocial health of older adults. Future investigations should examine possible underlying mechanisms of these relationships, as well as the efficacy of interventions through aural rehabilitation programs in addressing loneliness and social isolation.

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