We report the case of a 6 y/o Japanese-speaking girl whose genetic diagnosis of deafness was useful to select an earlier intervention, including the electric-acoustic stimulation (EAS) operation. Her preoperative pure-tone audiogram showed residual hearing in the lower frequencies bilaterally. Audiological assessment with HA using the monosyllable test showed values of 5% in the right ear and 25% in the left ear. Genetic testing of the blood showed that she had a mitochondrial mutation, suggesting potentially progressive deterioration of the deafness. Her parents provided consent for an EAS-type cochlear implantation using PULSAR FLEX24 via the RW membrane in the right ear. Postoperative assessment after 7 months revealed a result of 100% in the word test for infants and a result of 90% in the test for school children in her right EAS. After 17 months, the mean deterioration threshold of the residual hearing in the low frequencies (125, 250, 500Hz) was only 6.8dB and the residual hearing in the low frequencies was preserved, reflecting the useful effects of EAS. This proved the safety of the EAS-type cochlear implantation in this case. In conclusion, genetic screening and molecular confirmation seem important to guide the treatment strategy, even in children who have passed the NHS. Proof of genetic mutation encourages parents to receive earlier interventions, including EAS. Furthermore, it also contributes to selection of the optimum electrode length.
The primary purpose of this study was to determine sentence perception ability in noise and the effectiveness of an FM system for unilateral hearing loss (UHL). Three school-aged children with UHL and six childrens with normal hearing (NH) participated in the study. All the subjects underwent the Japanese Hearing in Noise Test (HINT-J) and were asked about their hearing in everyday life. As a result, the HINT-J scores for the children with UHL were lower than those for the (childrens with NH) under any noise condition. In particular, 2 of the 3 children could not perceive sentences at a lower than 0dB SN ratio in the presence of noise relative to the normal hearing side. Furthermore, one subject underwent a trial hearing using an FM system, and the effectiveness of the FM system for UHL was investigated using the HINT-J, a self-assessment inventory of hearing. When the subject used the FM system, the subject's HINT-J scores increased under noise conditions on the hearing impaired and normal hearing sides. The scores of the self-assessment inventory also improved. Long-term support is important to improve psychosocial aspects, and we considered the welfare subsidy and the tasks required for the use of this system.
Auditory neuropathy spectrum disorder (ANSD) is a hearing disorder characterized by the absence of auditory brainstem response (ABR) and presence of otoacoustic emissions (OAE). Although in most patients, ANSD is bilateral, unilateral ANSD patients have also been rarely reported. Recently, OAE and automated ABR (AABR) have been used widely for screening of hearing in newborns. With the advent of these newborn hearing screening tests, we can identify hearing-impaired children early. ANSD cannot be detected by OAE screening alone, because children with ANSD may also have normal cochlear function and a neural hearing loss. Herein, we report the case of a 7 year-old girl who was diagnosed as having unilateral ANSD. Her school hearing screening test was followed by the comment,“refer,” and she was found by further examination to have hearing loss on the right side. Although ABR was absent in the right ear, OAE were present in both ears. At the time of birth, the child had undergone a newborn hearing screening test by OAE, however her hearing loss had been overlooked. From now on, careful follow-up of her hearing is considered to be important, because the hearing loss may vary over time.
We conducted a survey of all teachers teaching children with unilateral hearing loss (UHL) in public elementary schools in one prefecture in the metropolitan area. According to the teachers, children with UHL did not have significant problems with school performance, language development, communication, or participation in activities. The survey results suggested that UHL did not directly affect the school performance or language development in the children. Children with UHL who had problems with class adaptation tended to have other disorders in addition. In comparison to bilateral mild hearing loss, UHL had lesser impact on language development, and greater impact on the emotional state. Moreover, children with UHL tended not to tell their friends that they had hearing loss in one ear. The results suggested that the problems of children with UHL were qualitatively different from those of children with bilateral mild hearing loss. It is necessary to provide patients, their families and teachers with information on UHL, and to provide support in accordance with the individual needs.