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Objectives: Early hearing detection and intervention (EHDI) is critical for achievement of age-appropriate speech perception and language development in hearing-impaired children. It has been 15 years since newborn hearing screening (NHS) was introduced in Japan, and its effectiveness for language development in hearing-impaired children has been extensively studied. Moreover, after over 20 years of cochlear implantation in Japan, many of the prelingual cochlear implant (CI) users have reached school age, and the effect of CI on language development have also been assessed. To identify prognostic factors for language development, audiological/language test scores and demographic factors were compared among prelingual severe-to-profound hearing-impaired children with CI divided into subgroups according to age at first hearing aid (HA) use and whether they received NHS., Methods: Prelingual severe-to-profound deafened children from the Research on Sensory and Communicative Disorders (RSCD) project who met the inclusion criteria were divided into groups according to the age (in months) of HA commencement (before 6 months: group A, after 7 months: group B), and the presence or absence of NHS (groups C and D). Language development and socio-economic data were obtained from audiological/language tests and a questionnaire completed by caregivers, respectively., Results: In total, 210 children from the RSCD project participated in this study. Group A (n=49) showed significantly higher scores on comprehensive vocabulary and academic achievement (p<0.05) than group B (n=161), with no difference in demographics except for significantly older age in group B. No differences in language scores were observed between group C (n=71) and group D (n=129), although participants of group D was significantly older and had used CIs longer (p<0.05)., Conclusions: Early use of HAs until the CI operation may result in better language perception and academic achievement among CI users with prelingual deafness. A long-term follow-up is required to assess the usefulness of NHS for language development., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Aim: Using proteomics, we aimed to identify the proteins differentially regulated by dexamethasone in the mouse cochlea based on mass-spectrometry data., Background: Glucocorticoid therapy is widely used for many forms of sensorineural hearing loss; however, the molecular mechanism of its action in the cochlea remains poorly understood., Methods: Dexamethasone or control saline was intratympanically applied to the cochleae of mice. Twelve hours after application, proteins differentially regulated by dexamethasone in the cochlea were analyzed by isobaric Tag for Relative and Absolute Quantitation (iTRAQ)-mass spectrometry. Next, dexamethasone-dependent regulation of these proteins was verified in the cochleae of mice with noise-induced hearing loss (NIHL) and systemic administration of dexamethasone by western blotting. Immunolocalizations of these proteins were examined in cochleae with NIHL., Results: A total of 247 proteins with a greater than 95% confidence interval of protein identification were found, and 11 differentially expressed proteins by dexamethasone were identified by the iTRAQ-mass spectrometry. One protein, myelin protein zero (Mpz), was upregulated (1.870 ± 0.201-fold change, p < 0.01) at 6 hours post-systemic dexamethasone and noise exposure in a mouse model of NIHL. Heat shock protein 70 (Hsp70) was downregulated (0.511 ± 0.274-fold change, p < 0.05) at 12 hours post-systemic dexamethasone. Immunohistochemistry confirmed Mpz localization to the efferent and afferent processes of the spiral neurons, whereas Hsp70 showed a more ubiquitous expression pattern in the cochlea., Conclusion: Both Mpz and Hsp70 have been reported to be closely associated with sensorineural hearing loss in humans. Dexamethasone significantly modulated the expression levels of these proteins in the cochleae of mice.

Conclusion: Performance in consonant-vowel (CV) monosyllable speech perception after cochlear implantation (CI) in the elderly (≥ 65 years) is equivalent to that of young adults (18-64 years). Present data in the Japanese language supported the indication for CI in the elderly. Word recognition after CI was significantly lower in the elderly than young adults., Objective: This study compared outcomes of monosyllable perception and word recognition after CI between elderly patients and young adults with post-lingual deafness. Age-related differences in CV monosyllable speech perception in Japanese were examined., Methods: A retrospective chart review of 137 patients with post-lingual deafness who underwent CI at Okayama University Hospital during 1992-2014 [young adults aged 18-64 years (n = 96) and elderly aged ≥ 65 years (n = 41) at implantation] was conducted. CV monosyllable speech perception post-CI was compared among age groups (18-29, 30-39, 40-49, 50-59, 60-69, 70-79, 80-89). CV monosyllable perception and word recognition scores post-CI were compared between the elderly and young adults., Results: There was no significant difference in CV monosyllable speech perception among age groups. CV monosyllable speech perception in the elderly (61.4 ± 25.5%) did not differ from that of young adults (65.9 ± 24.8%). Word recognition scores were significantly lower in the elderly (64.3 ± 28.1%) than young adults (80.4 ± 25.9%) (p < 0.05).

The spread of neonatal hearing screening has made the identification of hearing loss in early childhood possible, and most of the children with hearing loss are diagnosed and provided with early intervention within several months after their birth. However, they have to wait to receive the Special Child Dependent's Allowance until their hearing levels are evaluated with audiometers. Under the Special Child Dependent's Allowance Act now in force, a delay of several years occurs for those children before they can receive the allowance after diagnosis. We retrospectively reviewed hearing test results of children who had not reacted at 90dBnHL bilaterally in ABR within 12 months after birth, and examined when those results were certain enough to determine their allowance eligibility. We found that the eligibility in children with hearing loss alone could be determined at 1 year of age; as for children who had other disorders, since it was mostly difficult to test them with an audiometer, the diagnosis of hearing loss must always be made carefully but it was also possible to determine the eligibility for the allowance at 1 year of age.

Objective: While investigators have reported that patients with GJB2-associated deafness and cochlear implants have preferable language development, the mechanisms of this phenomenon remains unknown. The goal of the present study was to assess higher brain functions of patients with GJB2-related and GJB2-unrelated deafness as a method of evaluating language development., Methods: Eight children with cochlear implants were subjected to genetic testing for GJB2 and underwent the Raven colored progressive matrices test, Rey's auditory verbal learning test, Rey's complex figure test, the standardized language test for aphasia, the picture vocabulary test, and the standardized comprehension test for abstract words., Results: Three children were diagnosed with GJB2-related deafness, and five children were diagnosed with GJB2-unrelated deafness. All three GJB2-related cases demonstrated normal range higher brain functions and fair language development. By contrast, one GJB2-unrelated case showed a semantic disorder, another demonstrated a visual cognitive disorder with dyslexia, and another had attention deficit-hyperactivity disorder., Conclusions: Children with GJB2-unrelated deafness showed a high frequency of heterogeneous disorders that can affect proper language development. This difference between children with GJB2-related and GJB2-unrelated deafness may account for the improved language development in children with GJB2-related deafness and cochlear implants. Further, genetic diagnosis of the non-syndromic hearing loss represents a useful tool for the preoperative prediction of outcomes following a cochlear implant procedure.

Background: Cochleosaccular dysplasia is the most common pathologic finding seen in children with profound congenital sensorineural hearing loss. There has been no quantitative study on the peripheral vestibular system in cochleosaccular dysplasia., Objective: To investigate quantitatively the extent of pathologic changes of the vestibular sensory epithelium in cochleosaccular dysplasia., Subjects and Methods: Thirteen temporal bones with congenital deafness from 10 individuals were selected for this study from the temporal bone collection of University of Minnesota that showed suitable pathologic findings for the histopathologic criteria of cochleosaccular dysplasia. Age-matched normal control temporal bones were also selected. The vestibular hair cells including types I and II hair cells were counted separately in the saccular macula, utricular macula, and three cristae of the semicircular canals using Nomarski microscopy., Results: The hair cell densities of types I and II hair cells in the macula of the saccule in cochleosaccular dysplasia were significantly decreased compared with the data of normal subjects. Both types I and II hair cells in the utricular macula and the cristae of the three semicircular canals in cochleosaccular dysplasia were well preserved, and no significant difference was observed between findings of cochleosaccular dysplasia and normal controls in the utricle and the three semicircular canals., Conclusions: In cases with cochleosaccular dysplasia, the neurosensorial hair cells of the saccule were affected; however, the osseous labyrinth, the membranous utricle, and the semicircular canals were normal. Further studies should be performed to establish the pathogenesis of cochleosaccular dysplasia in humans.

Objective: The monosyllable speech perception ability after years of educational intervention was compared between prelingually deafened pediatric hearing aid users and their cochlear implant counterparts., Design: An open-set monosyllabic speech perception test was conducted on all subjects. The test required subjects to indicate a corresponding Japanese character to that spoken by the examiner. Fifty-two subjects with prelingual hearing impairment (47 hearing aid users and 5 cochlear implant users) were examined., Results: Hearing aid users with average pure-tone thresholds less than 90 dB HL demonstrated generally better monosyllable perception than 70%, which was equivalent or better performance than that of the cochlear implant group. Widely dispersed speech perception was observed within the 90-99 dB HL hearing-aid user group with most subjects demonstrating less than 50% speech perception. In the cluster of >100 dB HL, few cases demonstrated more than 50% in speech perception. The perception ability of the vowel part of each mora within the cochlear implant group was 100% and corresponding to that of hearing aid users with moderate and severe hearing loss., Conclusion: Hearing ability among cochlear implant users can be comparable with that of hearing aid users with average unaided pure-tone thresholds of 90 dB HL, after monosyllabic speech perception testing was performed.

Objectives: Mutations in connexin26 (GJB2) are one of the most frequent causes of prelingual hearing impairment. Several different types of one-base deletions in exon2 were the most common type of GJB2 mutation regardless of ethnicity, including 35delG in American-European populations, 235delC in Japanese population and 167delT in Ashkenazi Jewish population. Various types of one-base substitutions were also considered to be causative mutations of GJB2 associated hearing impairment. This article describes a rapid and high-throughput screening procedure for the detection of one-base deletion/substitution in GJB2 with less invasive sampling procedure in the implication for the clinical application., Methods: 53 hearing-impaired children and 50 healthy controls were admitted to take part in this study program. DNA samples obtained from buccal swab were used to amplify the exon2 of GJB2, and single run with an automated sequencer was used to identify the one-base deletion. Single-base substitutions were also screened by primer-extension procedure with dye terminators. The presence of both types of mutations was confirmed by direct sequence of the GJB2 exon2., Results: Two of 50 controls (4%) included one-base deletion in GJB2 as heterozygote. 14 of 53 hearing impaired cases (26.4%) contained deletion in GJB2 either as homozygote (five cases) or heterozygote (nine cases) form. Sequencing analysis of whole exon2 of GJB2 identified all these deletions as 235delC. Primer-extension analysis revealed additional mutations with single base substitutions in three cases with compound heterozygote with 235delC., Conclusions: Rapid screening procedure of GJB2 can be potentially useful for the identification of prelingual deafness.

Objective: We applied mutation screening in seven cochlear implant users to identify those persons with GJB2-related deafness to determine whether etiology of deafness was predictive of speech performance after implantation., Methods: Direct sequence of GJB2 was conducted over seven cochlear implant users with prelingual hearing impairment and their speech, language and cognitive performance was examined., Results: The three persons with GJB2-related deafness had a mean vocabulary of 1243 words compared to a mean vocabulary of 195 words in the four children with GJB2-unrelated deafness, although the number of patients examined here was limited. The developmental quotient (DQ) of cognitive ability also was higher in those children with GJB2-related deafness., Conclusions: These preliminary results suggest that better speech performance after cochlear implantation may be observed in persons with GJB2-related deafness. In the future, detailed phenotypic studies and mutation screening for non-syndromic hearing loss may play an important role in the preoperative assessment of prelingually-deafened children.

Conclusion:Significant expression of macrophage migration inhibitory factor and its receptor (CD74) was observed in both the middle ear and inner ear in experimental otitis media in mice. Modulation of macrophage migration inhibitory factor and its signaling pathway might be useful in the management of inner ear inflammation due to otitis media. Objectives:Inner ear dysfunction secondary to otitis media has been reported. However, the specific mechanisms involved are not clearly understood. The aim of this study is to investigate the expression of macrophage migration inhibitory factor and CD74 in the middle ear and inner ear in lipopolysaccharide-induced otitis media. Method:BALB/c mice received a transtympanic injection of either lipopolysaccharide or phosphate-buffered saline (PBS). The mice were sacrificed 24 h after injection, and temporal bones were processed for polymerase chain reaction (PCR) analysis, histologic examination, and immunohistochemistry. Results:PCR examination revealed that the lipopolysaccharide-injected mice showed a significant up-regulation of macrophage migration inhibitory factor in both the middle ear and inner ear as compared with the PBS-injected control mice. The immunohistochemical study showed positive reactions for macrophage migration inhibitory factor and CD74 in infiltrating inflammatory cells, middle ear mucosa, and inner ear in the lipopolysaccharide-injected mice. [ABSTRACT FROM PUBLISHER]

Conclusions: The microarray analysis identified 39 genes up- or down-regulated by dexamethasone in the cultured tissue of mice cochlea. Of the eight genes most highly affected, several are suggested to have protective effects in the traumatized inner ear ( Fkbp5, Glucocorticoid-induced leucine zipper (Gilz), glutathione peroxidase 3) and for others, a plausible mechanism of action can be offered (c laudin 10, glutamate-ammonia ligase). The present data may support the use of dexamethasone to treat acute sensorineural hearing loss. It is warrantable to test these results in the in vivo cochlea. Objectives: To identify genes whose expression is markedly up- or down-regulated by dexamethasone in the cochlear tissue. Methods: Murine cochlear tissue was cultured with or without dexamethasone for 48 h in vitro. The gene expression profiles were compared between the dexamethasone-treated and untreated cochlear tissue using a microarray that covers 33 696 transcripts (24 878 genes) of mice and quantitative real-time RT-PCR. Results: The microarray analysis identified 39 genes that are up- or down-regulated by more than twofold in the presence of dexamethasone in the cochlear culture. Genes up- or down-regulated by at least threefold include Fkbp5, Gilz, glutathione peroxidase 3, claudin 10, glutamate-ammonia ligase, proteoglycan 1, integrin beta-like 1, and alpha subunit of glycoprotein hormone. [ABSTRACT FROM AUTHOR]

Objective: To investigate whether a novel laser-assisted stapedotomy technique is effective in treating congenital stapes fixation. Methods: With this surgical method, the suprastructure of the stapes is vaporized continuously from the superior half of the anterior crus to the inferior part of the posterior crus using a potassium-titanyl-phosphate (KTP) laser. The midmost point of the vaporization is the head of the stapes. This method was used to treat three patients with congenital stapes fixation (total of four ears). Results: The average hearing gain using this method was 32.2 dB, which lasted for at least 1 year. No complications were observed. Conclusions: This novel laser-assisted stapedotomy technique is an excellent method for the treatment of congenital stapes fixation. [Copyright &y& Elsevier]

Auditory Brainstem Response (ABR) is the most reliable and most frequently used procedure to evaluate audiological conditions in early infancy. However, several reports have demonstrated that developmental change in the central nervous system may affect the results of ABR in audiological evaluations. We examined statistically the reliability of ABR for the diagnosis of profound deafness in early childhood according to our experience over the past 12 years of follow-up in our facility. Subjects included 371 children among 1,041 children who were admitted to Kanariya-Gakuen (institute for pre-school deaf children) from April 1985 to March 1997. These children were examined with ABR to determine their hearing levels. In five cases with an abnormal hearing threshold determined by ABR and other audiological tests, repeated examinations carried out during a 5- to 6-month follow-up period revealed that they had normal hearing. Three of these children had been diagnosed previously with mental retardation and the remaining two were infants <5 months old. The specificity and sensitivity of ABR were calculated as 97.3% and 100%, respectively. The predictive value of a positive result was 94.7%. There remains the possibility of a false negative for such cases, although the rate seems to be very low (<0.2%). [ABSTRACT FROM AUTHOR]

Recently, the effectiveness of cochlear implantation for hereditary deafness has been reported. We performed cochlear implantation for two patients with symptomatic hereditary deafness. Deafness in one patient was thought to be a result of albinism-deafness syndrome and in the other patient, a result of chronic progressive external ophthalmoplegia syndrome. Since their speech perception abilities improved dramatically, we believe that cochlear implantation should be actively performed for these two syndromes. [ABSTRACT FROM AUTHOR]

The post-operative complications and hearing results of surgical intervention for congenital aural atresia were investigated. Seventy-five ears with congenital aural atresia were operated on for hearing loss from 1982 to 1996. Tympanoplasty was performed on 37 ears and canaloplasty on the remaining 38. Hearing improvement rates were 70.3% and 31.6%, respectively. The post-operative complications included stenosis of the created auditory canal (29.3%), deterioration of the improved hearing (21.3%) and infection (12.0%). Reoperations were performed on 6 cases for restoring hearing, 16 for stenosis and 6 for infection. Reoperative findings demonstrated that scar formation in the canal caused the stenosis of the newly formed external canal and that lateralization of the new tympanic membrane and subsequent detachment from the ossicles caused the deterioration of improved hearing. Using cartilage and pedicled skin flap prevented stenosis at the orifice of the newly formed external canal. Maintaining hearing improvement requires creating a wide ear canal using as many pedicle flaps as possible and attaching a new tympanic membrane firmly to the ossicles. [ABSTRACT FROM AUTHOR]

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Transcanal endoscopic ear surgery (TEES) will become a very useful therapeutic option. A perilymphatic fistula (PLF) is defined as sudden sensorineural hearing loss and/or vertigo caused by leakage of the perilymph through a fistula from the oval window and/or round window. We report a case of PLF after electric acoustic stimulation (EAS), a kind of cochlear implant, successfully treated by TEES. A 38-year-old man presented to our hospital with vertigo and hearing loss (HL). His vertigo was induced by Valsalva maneuvers. Eight months ago, he underwent EAS for his right ear for congenital sensorineural HL. Although he maintained his hearing level after EAS, his pure tone audiogram this time showed deterioration of hearing at low frequencies in his right ear. A diagnosis of right PLF was made. After confirming the non-effectiveness of oral prednisolone treatment, PLF repair surgery to patch the oval and round windows by TEES was performed. His vertigo did not recur after the surgery. To the best of our knowledge, this is the first report of PLF repair surgery by TEES without a microscope. The wide-field view of the middle ear by TEES was useful to prevent electrode damage in a PLF patient with a cochlear implant. [ABSTRACT FROM AUTHOR]

Abstract: Learning disability combined with hearing impairment (LDHI) is a poor prognostic factor for the language development of hearing impaired children after educational intervention. A typical example of a child with LDHI and effective interventions provided by cochlear implants are presented in this report. A case of congenital cytomegaloviral infection that showed dysgraphia as well as profound deafness was reported and an underlying visual processing problem diagnosed in the present case caused the patient''s dysgraphia. The dysgraphia could be circumvented by the use of auditory memory fairly established by a cochlear implant. [Copyright &y& Elsevier]

The presence of additional handicaps in hearing-impaired children makes the prediction of language ability after cochlear implantation unreliable. Only limited follow-up data on developmental improvement after implantation among multiply handicapped children is available. The present study reports the course of development (audiological and linguistic) after cochlear implantation in one subject with moderate mental retardation. Preoperatively, his language development showed 34 months delay when compared to chronological age. The difference had shortened to 23 months by 2 years post-surgery. The subject''s cognitive delay had not changed upon 2-year follow-up. The cochlear implant can be credited to his improvement in language development. [Copyright &y& Elsevier]

Abstract: We describe the histopathological temporal bone findings of metastatic rhabdomyosarcoma in the patient with hearing loss and facial nerve paralysis. A 45-year-old female was admitted to the hospital with a mass of the left tibia. Surgical operation was performed and the pathological diagnosis was alveolar rhabdomyosarcoma. She showed left facial palsy and bilateral hearing loss. She was pronounced dead because of respiratory arrest caused by multiple metastases including meningeal metastasis after 14 months of total clinical course. Histological examination of the temporal bone revealed that the VII and VIII cranial nerves were involved by tumor cells in the internal auditory canal, and that the organ of Corti was damaged. In addition, the membranous labyrinth showed labyrinthitis. No remarkable bony destruction was observed in the internal auditory canal. Histopathological examination revealed that hearing loss and facial nerve paralysis of the patient was due to temporal bone metastasis of rhabdomyosarcoma. [Copyright &y& Elsevier]