[Subsidized project of hearing aid purchase for mild-moderate hearing impaired children in Akita prefecture].

The dissemination of newborn hearing screening has detected children with mild-moderate hearing impairment at an early age. However, there is no nation-wide welfare system for children with mild-moderate hearing impairment in Japan. Under these kinds of social conditions, a subsidized project of hearing aid purchase for mild-moderate hearing impaired children has come into force from April 2010 in Akita prefecture. All 18 candidates who applied for this project were subsidized in Akita prefecture. Eighteen children purchased their hearing aids using this subsidy. The feature of this project was that every child could have access to subsidies as long as their doctor recognized the effectiveness of hearing aids because children with hearing impairment need to learn language. They contacted the hospital, prefectural government and institutes related to hearing loss before this project come into force. We recognized parents who are raising a child with mild-moderate hearing impairment have high interest in this project. Hearing aids can represent a considerable expense for young parents who are raising a child. We encountered some children who had to give up the idea of hearing aids due to their parents' economic circumstances before this project become effective. These situations were completely avoided after this project came into being. This administrative purpose was of demonstrated value in children with mild-moderate hearing impairment.

Outcomes of regional-based newborn hearing screening for 35,461 newborns for 5 years in Akita, Japan.

OBJECTIVES: Newborn hearing screening (NHS) has been actively performed since 2001 in Akita, Japan. The NHS coverage rate has increased yearly, and performance has been consistently >90% since 2012. The purpose of this study was to summarize NHS outcomes in the Akita prefecture of Japan and to obtain new insights for from our summarized data for the future. METHODS: A total of 35,461 newborns in hospitals and clinics where hearing screening was performed in Akita from 2012 to 2016 were included. The outcome data of NHS were collected for analysis. RESULTS: The overall screening coverage rate for hearing loss was 94.7%. Of the screened infants, 0.53% received a referral on the 2-stage automated auditory brainstem response (ABR), and 80.4% of referred infants had a check-up at the hospital to receive a diagnostic hearing examination. Finally, the prevalence of bilateral congenital hearing loss was 0.14%, that of bilateral moderate to profound hearing loss was 0.12%, and that of unilateral congenital hearing loss was 0.10%. Furthermore, the average consultation period in infants with risk factors was significantly later than that in infants without risk factors (p = 0.0015). Follow-up for infants diagnosed with normal hearing after diagnostic hearing examination revealed that 4.7% suffered bilateral moderate to profound hearing loss later. This percentage is significantly higher than that of the general group (p < 0.001). CONCLUSION: The prevalence of bilateral congenital hearing loss was 0.14% in Akita and 0.12% of infants were diagnosed with bilateral moderate to severe hearing loss. Medical personnel should be enlightened regarding the importance of performing hearing diagnostic examinations until 3 months of age. Even if infants were diagnosed with normal hearing after a diagnostic examination, we strongly suggest continuing follow-up until they are able to perform pure tone audiometry with accuracy.

Leaflets and continual educational offerings led to increased coverage rate of newborn hearing screening in Akita.

OBJECTIVE: Newborn hearing screening (NHS) has been actively performed in Japan since 2001. The NHS coverage rate has increased each year in Akita Prefecture. We analyzed the details of the NHS program and how the Akita leaflets and the many educational offerings about the importance of NHS led to the high NHS coverage rate. METHODS: A retrospective study was conducted in liveborn newborns in hospitals and in clinics where hearing screening was performed from the program's beginning in 2001 through the end of 2015. We describe the chronological history of NHS. The outcome data of NHS were collected from our department and analyzed. RESULTS: From the founding of the program in 2001 to 2015, the live birth rate in Akita continually declined. Nevertheless, the number of infants receiving NHS rose each year. Since 2012, the coverage rate of NHS has been over 90%. From 2001 to 2015, 75,331 newborns constituted the eligible population for the NHS program. Since 2012, the number of NHS tests has stabilized. We prepared educational leaflets for Akita Prefecture early in 2002. We also provided many educational classes about the importance of NHS for not only pregnant women but also professionals including obstetricians and gynecologists, pediatricians and municipal staff members. The NHS program received the complete endorsement of the Akita Association of Obstetricians and Gynecologists in 2010. The largest increase in the NHS coverage rate occurred from 2001 to 2002, and the second largest increase occurred from 2009 to 2010. The number of participating institutions increased the coverage rate. The coverage rate is strongly correlated with the number of participating institutions (rs=0.843, p<0.001, Spearman's rank correlation coefficient). Comparing the coverage rate for 5 years before and after the Akita Association of Obstetricians and Gynecologists reached their consensus on the importance of NHS, the coverage rate after 2010 was significantly higher than before 2010 (p<0.001, paired sample t-test). CONCLUSION: The NHS coverage rate ultimately reached 95.4% without need for legislation or subsidization. The number of participating institutions increased each year, and the number of NHS tests and the coverage rate increased proportionately. The number of participating institutions statistically has a strong correlation with the number of NHS tests and the coverage rate. Our research indicates that the Akita leaflets and the provision of educational sessions about the importance of NHS were the most significant factors in establishing the high NHS coverage rate.

Parkinsonism Improved With Levodopa After Endoscopic Third Ventriculostomy in Shunted Hydrocephalus Due to Aqueductal Stenosis.

INTRODUCTION: Levodopa-responsive parkinsonism has been reported following ventriculoperitoneal (VP) shunt in patients with obstructive hydrocephalus due to aqueductal stenosis. It has been thought to arise from injury to the global rostral midbrain including the nigrostriatal pathway by a transtentorial pressure gradient. We present a similar patient, but his parkinsonism resisted levodopa administration during the initial therapy. CASE REPORT: A 51-year-old man suffered from hydrocephalus due to secondary aqueductal stenosis presumably attributed to massive bleeding during surgery for a fourth ventricle hemangioblastoma. After resolution of the hydrocephalus with VP shunt, he developed severe parkinsonism, Parinaud syndrome, and hyperreflexia, suggesting global rostral midbrain dysfunction, but high-dosage levodopa therapy was not effective. An inverted transtentorial pressure gradient suggested by his unilateral slit-like ventricle was assumed to be the cause of the levodopa resistance. Also based on an assumption that the absorption of cerebrospinal fluid was impaired due to the intraoperative bleeding, a lumbar peritoneal shunt was added to the preexisting VP shunt, but it failed to control the ventricular size. Instead, endoscopic third ventriculostomy stabilized it, characteristically inducing levodopa responsiveness in our patient. An increase of the levodopa dosage led to clinical improvement, which needed a maintenance dosage because of dependency. CONCLUSION: The details of this patient suggest that a transtentorial pressure gradient may have impaired more distal basal ganglia connections over a global rostral midbrain including the nigrostriatal pathway, and that aggressive levodopa therapy after endoscopic third ventriculostomy can be effective for refractory parkinsonism.

Hearing screening for Japanese children and young adults using the automated auditory brainstem response.

OBJECTIVE: Hearing screening programs in schools are particularly important for children because they can enable early detection of hearing problems and early intervention. However, there has been little research on this topic. The MB11BERAphone(®) is a novel, accurate and efficient Automated Auditory Brainstem Response device for hearing screening in infants. The aim of this study was to investigate the validity of the MB11BERAphone(®) as a hearing screening device for pre-school, school-age and young-adult individuals. METHODS: Between January 2010 and March 2012, 163 normal and hearing impaired individuals, corresponding to 321 ears from subjects aged 3-22 years 11 months at Luther Aiji Kindergarten and Nakadori General Hospital in Akita, Japan, underwent primarily conditional play audiometry or conventional audiometry followed by the Automated Auditory Brainstem Response by MB11 BERAphone(®). The statistical analysis was performed with the Predictive Analytics Software (PASW Statistics 18) and presented as the mean, standard deviation (SD) and frequency distribution. The sensitivity, specificity and false-positive and false-negative rates were estimated to analyze the validity of the MB11 BERAphone(®) test over audiometry. RESULTS: Among the normal and hearing impaired ears, 140 were scored as "REFER" and 181 were scored as "PASS" during the hearing screening examination conducted using the MB11BERAphone(®) device. The specificity was 95.1%, and the sensitivity was 96.3%. The false positive rate was 5%, and the false negative rate was 4%. The overall timing without precise measurement was less than 7min. The individuals were divided into groups: pre-schoolers (3-5 years), school-age (6-17 years) and young-adults (18-22 years). When the audiometry and MB11BERAphone(®) results were compared, no statistically significant differences (p=ns) were detected among general (pre-schoolers+school-age+young-adult), pre-school, school-age, and young-adult groups. CONCLUSION: The results suggest that the MB11BERAphone(®) is not only useful for newborn hearing screening but also for hearing screening in older individuals, due to the low cost of the integrated electrodes and the speed with which the examination can be performed.