To promote social participation and interaction of the elderly, countermeasures against hearing impairment, which is common among the elderly, are extremely valuable. Evidence has been accumulated to suggest hearing impairment is a risk factor for various disadvantageous circumstances in the elderly. According to a report of the World Health Organization published in 2017, the employment situation of hearing-impaired people is a cause for concern, and at the Alzheimer's Association International Conference (AAIC) 2017, hearing impairment was cited as one of the nine potentially modifiable risk factors for dementia. In regard to the effects of hearing aids prescribed for coping with hearing impairment, studies with various research designs have conducted evaluations of different aspects in persons with hearing impairment, such as employment, income, depression, social isolation, cognitive function, etc. The results reported from various countries such as the United States, United Kingdom, France and Australia have not always been consistent. The conflicting reports suggest that perhaps long-term observation and a large number of subjects are needed to evaluate the effects of hearing aids on factors other than hearing. However, handling of hearing impairment and improving the utilization of hearing aids in the elderly are considered matters of high priority for the realization of high longevity and a healthy society in Japan, the country with the highest longevity in the world.
Usher syndrome (USH) is an autosomal recessive disorder characterized by retinitis pigmentosa (RP) and hearing loss (HL). USH type 1 (USH1) is characterized by congenital severe-to-profound HL and vestibular dysfunction. It is frequently caused by mutations of MYO7A and CDH23, which account for 70%-80% of all cases. Mutation analysis of MYO7A and CDH23 in seven Japanese patients from six families revealed seven probable pathogenic mutations (three in MYO7A and four in CDH23) in six patients from five families. All six patients were diagnosed as having hearing impairment at 1 to 3 years of age. Despite having been fitted with hearing aids immediately after the diagnosis, none of the patients developed speech ability and all use sign language/writing to communicate. In all the patients, the visual fields were symmetrically constricted and the pigmentary degeneration was typical of RP, with peripheral bone-spicule pigmentation. The patients, especially those with severely constricted visual fields and low visual acuity due to advanced RP, also have difficulties in communicating even through sign language/writing. They need social support to develop the ability to communicate through other modes, including Braille. In addition, early diagnosis of USH1 is essential so that cochlear implantation may be promptly undertaken in infancy to allow development of speech ability.
Objectives: In this study, to obtain basic data for clinical use of the evaluation scores on “The Questionnaire on Hearing 2002”, the changes in the scores recorded after hearing-aid fitting were examined.
Subjects: One hundred and sixty-three persons with mild or moderate hearing loss (69 men, 94 women; mean age, 64 y 5 m±15 y 8 m).
Methods: The evaluation scores recorded prior to and after hearing-aid fitting were compared retrospectively.
Results: Concerning the distribution of the evaluation scores, there was no significant differences among the 6 subscales prior to the hearing-aid fitting. After hearing-aid fitting, the evaluation scores were distributed as follows; on the 3 subscales of hearing disability, 80% showed a score of 1 or 2, on the 2 subscales of psychosocial effects, more than half showed a score of 1 or 2, and on the subscale of communication strategy, there was no change from the distribution prior to hearing-aid fitting.
Discussion: After hearing-aid fitting, the distribution of the evaluation scores showed different patterns among the subscales. This result suggests rehabilitative issues left even after hearing-aid fitting. Clinically, the profile drawn through linking the evalution scores on the 6 subscales serves as a visual indicator about the next issue that needs to be resolved.
In this study, we examined vocabulary development in the early stage of younger cochlear implantees who had undergone the implant surgery by the age of five. Subjects were divided into six groups according to the results of the Japanese Picture-Vocabulary-Test (PVT). Thirty-two out of 88 subjects (36.4%) showed a good vocabulary development index (VDI>85), comparable to that of their hearing age peers, while 56 (63.6%) showed only fair (VDI<84) or poor (VDI<49) vocabulary development. The average age at the time of surgery was significantly lower in the children in whom the VDI had exceeded 85 by the age of four. On the contrary, the age at the time of the first hearing aid trial and the hearing threshold with cochlear implant showed no relationship to the VDI. In regard to the communication mode, most of the children tried to enhance their oral communication skill before enrollment to primary school, however, after enrollment, some in the poorly vocabulary development group (VDI<49) started to use sign language. It is essential to support hearing-impaired children over the long term on the basis of the understanding that they need better vocabulary proficiency, reading and speaking abilities, and social skills in order to communicate with people in the society.
The present study was conducted to examine the relationship between the hearing ability and the results of evaluation by WISC-IV in 12 children wearing hearing aids. Assessment of the children's hearing ability was conducted by pure-tone audiometry, sound-field threshold measurements, speech recognition tests, speech discrimination tests, and speech discrimination tests in noise. We found no correlation between either the Verbal Comprehension Index (VCI) or the Perceptional Reasoning Index (PRI) and the hearing ability in any of the 12 children. There were significant differences between the VCI scores and PRI in five children, in all of whom the VCI was significantly lower than the PRI. Although the results showed that language acquisition had not progressed to the same extent as their perceptual ability, the VCI scores exceeded the lower limit of the average in all cases.