Children with mild to moderate hearing loss react partially to sounds and voices and show inadequate development of speech and language skills; therefore, the hearing loss in these patients tends to be discovered late, causing a delay in rehabilitation and hearing development that eventually results in issues in language skills as well as emotional problems. However, as screening of neonates for hearing loss has been widely practiced in recent years, it is possible to detect hearing loss very early during infancy, and some patients have shown favorable growth and development. On the other hand, many children are not subjected to screening, and in many cases, condition occurs in conjunction with late-onset hearing loss/multiple handicaps; therefore, suitable tests as well as various ways of dealing with each situation need to be further developed. In addition, we expect an increase in the use of hearing aids as well as use of hearing aids at an earlier stage; simultaneously, we provide a detailed account of the financial burden posed on young parents who have to buy hearing aids for their children, provision of public assistance by local governments, and active interventions in terms of treatment and rehabilitation.
This study was conducted to examine the challenges in the medical care and education of children with hearing loss and the current status of support for these children. The subjects were 20 children who underwent the speech perception test and the language test at the Department of Otolaryngology, Yamaguchi University Hospital from April 2011 to April 2012. The processes of identifying hearing loss and conducting hearing tests, as well as the educational backgrounds of the children were investigated, while measuring speech perception and the hearing-aid or cochlear-implant thresholds of the children and performing the Wechsler intelligence test. Furthermore, the children's communication modes in their nurseries or elementary schools were evaluated using the observation method. The extracted education-related challenges included: delayed identification of hearing loss, delayed wearing of hearing aids in everyday life, and the progression of hearing loss. Among the 17 children who underwent the WISC-IV Intelligence Test, 5 showed verbal comprehension index scores of less than 80. Although the remaining 12 showed verbal comprehension index scores of 80 or more, the verbal comprehension index scores were significantly lower than the perceptual reasoning index scores in 5 of the patients. The results suggest that speech therapy should be provided on a continuing basis for children with delayed speech learning.
We evaluated the results of aided threshold, speech discrimination, language development, speech intelligibility and communication mode in 8 children with congenital cytomegalovirus infection (CMV) who underwent cochlear implantation (CI) at our hospital. Four of the 8 children had mental retardation (MR), one had pervasive developmental disorder (PDD) and another had suspected PDD. All the children exhibited good aided thresholds. Two of the 8 children who did not have any psycho-neurological disorders showed good results in all the examinations. The speech discrimination scores of the four children with MR were good and these children showed progress in language development, although at a slow pace. Their speech intelligibility scores varied widely from clear to unintelligible. One of the children used spoken language, while the other three used total communication. In contrast, the child diagnosed as having PDD and the one with suspected PDD exhibited the poorest performances in all the postoperative evaluations. However, they showed improvement of the reaction to sound, recognized their names, and their vocalization in social interactions increased. Both the benefits and limitations of CI should be explained to the parents before deciding the surgical indications in pediatric CI candidates with congenital CMV infection and psycho-neurological disorders.
With the suburbs of Ibaraki Medical Center (Mito city where the centre is located, and six towns adjacent to Mito city) as the target region, a survey was conducted concerning children aged under six with hearing impairment, who were asked by the center to use hearing aids. Of the 46,914 people born in the target region between 2000 and 2007, 78 had hearing impairment ; thus, the proportion of children with hearing impairment was 0.166%. Of this, about 75% had sensorineural hearing loss. With regard to the degree of hearing impairment, there were 41 (52.6%) children with light-to-moderate hearing impairment, and 37 (47.4%) children with severe hearing impairment, that is, 0.087% and 0.079% of births respectively. There were 46 cases (59.0%) with no risk factors of hearing impairment and 32 cases (41.0%) with one or more risk factors. Thus, the number of cases with no risk factors were higher, that is, 0.098% of births. Of those cases that underwent newborn hearing screening, about 20% had passed.
We investigated 12 students with hearing impairment (nine girls, and three boys) who were changed to a school for the deaf from the mainstream, and clarified the features of the students and the problems in the support for these children in the mainstream. The average age at diagnosis was 5 years four months and the average hearing level at the time of diagnosis was 43dB. Nine students had progressive hearing loss and six of them were changed to a school for the deaf after the hearing level exceeded 70dB. Ten students had concomitant developmental disorder. The children had problems such as difficulties of learning and long absences from school because of the limited support available to them in the mainstream. Moreover, there is little information available with the teacher about the support available for such children at schools for the deaf. The features of the students who were changed to schools for the deaf were mainly those with delayed diagnosis of the hearing loss, with progressive hearing loss, and with concomitant developmental disorder. The major problem was the limited support available to deal with the difficulties of students with hearing impairement in the mainstream. Networking among related institutions with hearing-impaired students is required for their support.
We developed the “Auditory Matrix Test” to evaluate the speech recognition of hearing-impaired children within a short time. The test is a closed-set speech perception test. The purpose of this study was to evaluate the educational effectiveness of the Auditory Matrix Test. We examined the Auditory Matrix Test performance of 50 (147 cases) prelinguistic hearing-impaired children. The results were as follows: there was no clear correlation between the scores on the aided HTL and the Auditory Matrix Test. There was a correlation between the scores on the Auditory Matrix Test and the open-set speech perception test, the Japanese speech recognition test battery “CI-2004”. Therefore, use of CI-2004 or other open-set speech perception tests is recommended when the Auditory Matrix Test score is higher than or equal to 35/40. The Auditory Matrix Test is effective for evaluation of a child's perceptual abilities, especially when it is used in combination with other batteries.
A 33-year-old woman with proteinuria was admitted to our hospital for a renal biopsy. She had suffered from slight hearing impairment since she was 15 years old. Pure-tone audiometry revealed bilateral mild sensorineural hearing loss. Although renal biopsy revealed evidence of IgA nephropathy, the clinical features of this patient were not typical of this disease, as the proteinuria was not associated with hematuria. Genetic analysis of mitochondrial DNA revealed the A3243G mutation. Electron-microscopic observation of the kidney biopsy specimen revealed abnormal structures of the mitochondria. When we see patients with sensorineural hearing loss associated with unexplained proteinuria, this type of mitochondrial disease should be considered in the differential diagnosis, even in the absence of a history of diabetes mellitus.