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.

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.

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 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.

Progressive Hearing Impairment in Children with Congenital Cytomegalovirus Infection

1979 ◽  
Vol 44 (2) ◽  
pp. 220-229 ◽  
Author(s):  
Arthur J. Dahle ◽  
Faye P. McCollister ◽  
Sergio Stagno ◽  
David W. Reynolds ◽  
Holly E. Hoffman
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Cytomegalovirus Infection ◽  
Sensorineural Hearing ◽  
Hearing Impairments ◽  
Congenital Cytomegalovirus Infection ◽  
Progressive Hearing Loss ◽  
Congenital Cytomegalovirus

Audiological assessment of 86 children with congenital cytomegalovirus infection revealed progressive hearing loss in four of 12 subjects with sensorineural hearing impairments. Case descriptions are presented documenting the progression of the hearing loss. In view of the findings, children with congenital cytomegalovirus should be monitored closely to insure detection of possible delayed or progressive hearing impairment and delivery of appropriate habilitative services.

The Development of Constant Items for Speech Discrimination Testing

1968 ◽  
Vol 11 (3) ◽  
pp. 656-667 ◽  
Author(s):  
Elmer Owens ◽  
Earl D. Schubert
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Speech Discrimination ◽  
Discrimination Test ◽  
Closed Set ◽  
Response System ◽  
Speech Discrimination Test ◽  
Discrimination Testing ◽  
English Speaking ◽  
Manner Of Articulation

Subjects were English-speaking adults with hearing impairment. Etiology of hearing loss did not enter into selection. Consonant errors were observed on speech discrimination test lists employing a closed-set response system. Fifteen subjects were employed for the first list and 20 each for the remaining four lists, with an occasional subject serving in more than one group. Confusions between unvoiced and voiced consonants rarely occurred; the /r/ and /l/ were seldom confused with other phonemes; and nasals were seldom confused with non-nasals. Discrimination difficulty was related to both place and manner of articulation.

Impairment Tutorial: Hearing Impairment

2003 ◽  
Vol 8 (2) ◽  
pp. 11-11
Author(s):  
Kathryn Mueller ◽  
Christopher R. Brigham
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Extended Period ◽  
Loud Noise ◽  
Speech Discrimination ◽  
Recent Exposure ◽  
Whole Person ◽  
The Us ◽  
Record Data ◽  
Rating Sheet

Abstract A 1999 study of adults showed hearing loss was the fifth most common disability in the US population, and almost 50% of workers in carpentry, plumbing, and mining had hearing impairment. Determining hearing impairment according to the AMA Guides to the Evaluation of Permanent Impairment (AMA Guides), Fifth Edition, Section 11.2a, Criteria for Rating Impairment Due to Hearing Loss, is straightforward, if limited. Examiners should be aware that hearing can be temporarily impaired by recent exposure to loud noise and should test only after an extended period without such exposure. Audiometers should be properly calibrated, and technicians must be appropriately trained to obtain accurate measurements. The evaluator should separately test both of the individual's ears at 500 Hz, 1000 Hz, 2000 Hz, and 3000 Hz (the representative or test frequencies) and then identify the total worst ear decibel level using the AMA Guides Table 11-1 or 11-2. The evaluator can use Tale 11-3 to convert hearing impairment to whole person loss. Tinnitus also can be rated if a hearing loss in that ear affects speech discrimination; in such instances, the tinnitus rating is limited to a 5% loss. The article includes a Hearing Impairment Rating Sheet that can be used to record data from the hearing impairment evaluation.

Effect of speechreading in presbycusis: Do we have a third ear?

2017 ◽  
Vol 71 (6) ◽  
pp. 38-44
Author(s):  
Luis Roque Reis ◽  
Pedro Escada
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Spoken Word Recognition ◽  
Cross Sectional Study ◽  
Spoken Word ◽  
Speech Discrimination ◽  
Cross Sectional ◽  
Age Related ◽  
Bilateral Sensorineural Hearing Loss ◽  
Age Related Hearing Loss

Introduction: Evidence regarding the effect of speechreading is lacking in age-related hearing loss (presbycusis). Thus, in individuals with presbycusis, this study determined whether speechreading would improve word intelligibility. Moreover, the study investigated the effect of speechreading on word intelligibility depending on hearing impairment severity. Materials and methods: This analytical, cross-sectional study involved two groups of individuals aged > 65 years that were enrolled by convenience sampling: 29 individuals with bilateral sensorineural hearing loss compatible with presbycusis (n=58 ears; mean age, 74.1±9.4 years) and 10 controls with at-most-mild hearing loss (n=58 ears; mean age, 73.8±8.5 years). All participants underwent a comprehensive medical and audiological evaluation, which included speech audiometry with and without observation of the audiologist’s face, i.e. speechreading. Within each group, the effect of speechreading was determined as a change in the speech reception threshold. For all statistical analyses, p < 0.05 was considered significant. Results: Both in individuals with presbycusis and controls, speechreading significantly improved speech discrimination (p<.001<.05); however, compared to controls, this effect of speechreading on speech discrimination was more pronounced in individuals with presbycusis (p<.001). Discussion: Individuals with presbycusis or hearing impairment displayed improved spoken-word intelligibility when spoken-word recognition was coupled with speechreading. Thus, speechreading may serve as a “third ear”.

Rating Cognitive Impairment, Part 2: Objective and Evidence-Based Integration of Neuropsychology Testing

2017 ◽  
Vol 22 (2) ◽  
pp. 6-12
Author(s):  
Robert J. Barth ◽  
John E. Meyers
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Binaural Hearing ◽  
Speech Discrimination ◽  
Sixth Edition ◽  
Age Related ◽  
Whole Person ◽  
The Individual ◽  
Loss Of Hearing ◽  
Relationship Of

Abstract Hearing impairment rating determination is described in the AMA Guides to the Evaluation of Permanent Impairment (AMA Guides), Sixth Edition, Section 11.2a, Criteria for Rating Impairment Due to Hearing Loss. A hearing impairment evaluation for adults who have acquired language skills is derived from a pure-tone audiogram and always is based on the functioning of both ears even though hearing loss may be present in only one ear. Audiometers should be properly calibrated, and technicians should be appropriately trained to obtain accurate measurements. Audiograms typically are obtained at four frequencies (test frequencies): 500, 1000, 2000, and 3000 Hz, which are considered to be representative of everyday auditory speech ranges. The evaluator tests the individual's right and left ears at the test frequencies and adds the decibel levels for each of these frequencies for each ear separately; consults Table 11-2, Computation of Binaural Hearing Impairment; and finally consults Table 11-3, Relationship of Binaural Hearing Impairment to Impairment of the Whole Person. Tinnitus can be rated if the individual experiences hearing loss in the ear and this loss affects speech discrimination; loss is limited to a maximum of 5% loss. The AMA Guides provides no correction in the hearing section for age-related loss of hearing, although the latter may be apportionable. A table presents a model hearing impairment report.

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