1. Hearing screening systems The transition and the present situation of worldwide and domestic newborn hearing screening are reported. The incidence of bilateral hearing loss was about 0.7∼1.5 in 1,000 infants. About 0.4∼0.5 and 4.8∼22 in 1,000 low-risk and high-risk infants had bilateral hearing loss, respectively. According to the survey questionnaire that was sent to all domestic department of otolaryngology which were recommended by the Otorhinolaryngological Society of Japan, 75% of the infants referred to the department of otolaryngology accepted audiologic and medical evaluations before 3 months of age and 15.6% of them had early intervention services. On the other hand, 14.3% of the infants who passed newborn hearing screening but were referred to the department of otolaryngology had hearing loss. The transition and the actual situation regarding hearing screening for three-year-old children which is legislated in Japan are also reported. Eight hundred and forty-eight thousand, two hundred and eighteen children underwent hearing screening in 2007. Six hundred and twenty-seven of them (0.07%) had hearing loss. The number of children who had bilateral sensorineural hearing loss in 2007 was the same as it was in 1997. Both reports clarified that hearing rescreening systems subsequent to newborn hearing screening should be provided. 2. Audiological and medical evaluation for infants, toddlers and preschoolers Audiological and medical evaluation for infants, toddlers and preschoolers is quite different from such evaluation in adults. As for audiological evaluation, developmental assessment is also necessary to arrive at appropriate measures. The procedures and points in mind for behavioral audiometry (behavioral observation audiometry, conditioned orientation response audiometry, peep show test and play audiometry), auditory brainstem response, auditory steady state response and otoacoustic emissions are explained. The medical evaluation is as follows: physical examination, clinical history, family history of childhood and onset of permanent hearing loss, identification of syndromes associated with early- or late-onset permanent hearing loss and radiologic and laboratory studies. Knowledge of pediatric hearing loss is mandatory so that otolaryngologists can evaluate synthetic and comprehensive hearing loss in children. 3. Rehabilitation of children with hearing loss Although the current situation regarding rehabilitation of children with hearing loss in Japan still presents some problems, these children and their families now have more options for a possible institute and/or method for rehabilitation than before. Moreover, rehabilitation goal setting has been gradually changing, moving away from the idea of "working towards obtaining normal hearing" and rather approaching the viewpoint of "living with being hard-of-hearing" so that affected children can actively seek various possibilities in their own lives.
Hyperacusis denotes increased sensitivity and unpleasant response to sounds at levels that would not disturb a normal individual. This auditory hypersensitivity may occur in patients with normal hearing. We analyzed 24 patients (eight male, sixteen female) who complained of hyperacusis in the absence of hearing disturbances. These patients underwent the SISI (short increment sensitivity index) test, MCL/UCL (most comfortable loudness level/uncomfortable loudness level) test, Bekesy audiometry, and Metz test, and the positivity rate of the recruitment phenomenon was examined. Despite normal hearing, 17 patients (71%) showed positive results in some of these tests. The positivity rates of the SISI test, MCL/UCL test, Bekesy audiometry and Metz test were 27%, 38%, 38% and 33%, respectively. Nine patients (37.5%) complained of unilateral hyperacusis and fifteen patients (62.5%) complained of bilateral hyperacusis. In the subjects with unilateral hyperacusis, the side showing positivity of the recruitment phenomenon did not completely correspond to the symptomatic side.
We investigated the actual status of enrollment of hearing-impaired young children with some other disabilities, or a tendency towards developmental disabilities (ADHD, PDD, etc.) which had not been diagnosed medically, in kindergarten schools for the deaf. According to the responses, about 31% of the young children had some disabilities in addition to hearing impairment, and about 19% of the young children had associated or suspected developmental disabilities. We also investigated the developmental characteristics of the hearing-impaired young children with, or suspected to have, ADHD. The results revealed that while no significant differences in the developmental characteristics were observed at the age of 3 years between the hearing-impaired young children without other disabilities and hearing-impaired young children with, or suspected to have, ADHD, significant differences between the two groups were found in the speech perception ability, language development, and use of communication media (spoken words, lip reading, written words) at the age of 4-5 years. Also, about 36% of hearing-impaired young children with, or suspected to have, ADHD showed delay of manual fine motor development, and about 10% of hearing-impaired young children with, or suspected to have, ADHD tics.
We investigated the reality status of children with hearing loss detected during school medical checkups in 86 elementary schools and 33 junior high schools in Joetsu region. Of these schools, we received responses to our questionnaire from the nurse-teachers at 74 elementary schools and 28 junior high schools. The number of children in the 102 schools was 22954. The number of children who were diagnosed as having hearing loss was 178. Fourteen of these children were using hearing aids, and 2 had undergone cochlear implantation. They had received some support during their school life. On the other hand, our results revealed that there were a number of children with hearing loss in elementary schools and junior high schools who were not using hearing aids and had not undergone cochlear implantation. The results of the school medical checkup in these children were only being notified to the home. Only about 75% of the nurse-teachers reported any case of hearing loss found during a school medical checkup to the otolaryngology doctor or a qualified consultant; the remaining cases went unreported to a qualified person. We wish to emphasize that it is important to explain and enlighten nurse-teachers at schools about the consequences of hearing loss and the need to support children with hearing loss.
The aim of this study was to evaluate the symptoms of inattention and hyperactivity/impulsivity using 18-item DSM-IV-oriented rating scales in 14 children (ranging in age from 23 to 72 months; mean 38.3±13.7 months) with profound hearing loss before and after cochlear implantation (observation period ranging from 6 to 36 months; mean 19.7±11.1 months). The average scores for hyperactive/impulsive symptoms were higher than those for inattentive symptoms during the period between pre-implant and 12 months post-implant. Furthermore, the average scores for both inattentive and hyperactive/impulsive symptoms decreased at 6 months post-implant, increased temporarily at 12 months post-implant, and decreased again thereafter. These findings suggest that in such children undergoing cochlear implantation adequate support should be ensured, because they show temporary deterioration of the symptoms of inattention and/or hyperactivity/impulsivity.