Rie Nagayasu

Better speech performance in cochlear implant patients with GJB2-related 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.

Language development of a multiply handicapped child after cochlear implantation

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 subjects cognitive delay had not changed upon 2-year follow-up. The cochlear implant can be credited to his improvement in language development.

Monosyllable speech perception of Japanese hearing aid users with prelingual hearing loss: implications for surgical indication of cochlear implant.

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.

Syntactic development in Japanese hearing-impaired children.

Objectives: This study examined syntactic development of auditory comprehension of sentences in Japanese-speaking school-age children with and without hearing impairment. Methods: In total, 592 preschool and school-age children (421 normal-hearing and 171 hearing-impaired) were included in this cross-sectional observation study conducted using the Syntactic Processing Test for Aphasia for Japanese language users. Linear regression analysis was used to determine the estimated age at which each syntactic structure was acquired. Results: Acquisition of syntactic structures was observed in hearing-impaired and normal-hearing children. Basic word order sentences of agent-object-verb and the goal benefactive construction were acquired at preschool age (earlier group), whereas reverse word order sentences of object-agent-verb, source benefactive construction, passive voice, and relative clauses were acquired at school age (later group). The results showed that many hearing-impaired children may not acquire Japanese grammatical structures until the age of 12 years. Conclusions: Adequate screening for language development for school-age hearing-impaired children is required for an effective intervention.

Subjective hearing-related quality-of-life is a major factor in the decision to continue using hearing aids among older persons

Abstract Conclusion: Audiological parameters alone do not determine the choice to use hearing aids (HA). Subjective hearing-related QoL is a major factor that determines whether or not an older person will continue to wear HA. Objective: This study aimed to identify which audiological parameters and quality-of-life (QoL) measures determine whether or not older persons will continue wearing HA. Methods: Charts of 157 patients aged ≥65 years who attended the HA service unit at the Otolaryngology Department were retrospectively reviewed. After HA fitting and a trial, the patients were divided into groups, depending upon whether or not they wanted to continue wearing the HA (users, 58.2%; non-users, 41.8%) and then audiological parameters were compared between them. At least 4 months after the HA fitting, the self-reported QoL questionnaire, Hearing Handicap Inventory for the Elderly (HHIE), was mailed to all 157 patients and HHIE scores were compared between HA users and non-users. Result: Speech discrimination score and dynamic range did not significantly differ between HA users and non-users. A difference in the average hearing threshold was marginally significant. The response rate to the HHIE was 65.2%. Total HHIE and emotional scores were higher (more impaired) among HA users than non-users.

Impact of early intervention on comprehensive language and academic achievement in Japanese hearing-impaired children with cochlear implants

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.

Cochlear Implantation for Children with Auditory Neuropathy Among Japanese Language Users

Among 80 prelingually deafened children who underwent cochlear implant at Okayama University Hospital, two auditory neuropathy/auditory dys-synchrony (AN/AD) cases with cochlear implant were identifi ed from review of medical records. These two cases fi rst demonstrated stable responses with distortion product otoacoustic emission, although later the response disappeared. In spite of the presence of AN/AD, the language development of these cases was quite satisfactory so far. Herein, we report the clinical course and language development of these auditory neuropathy cases with cochlear implant.

Relationship between pure-tone audiogram findings and speech perception among older Japanese persons

Abstract Objective: To clarify how the pure-tone threshold (PTT) on the PTA predicts speech perception (SP) in elderly Japanese persons. Methods: Data on PTT and SP were cross-sectionally analyzed in Japanese persons (656 ears in 353 patients, aged ≥65 years). Correlations of SP and average PTT in all tested frequencies were evaluated by Pearson’s correlation coefficient and simple linear regression. After adjusting for sex, laterality of ears, and age, the relationship of average and frequency-specific PTT with impaired SP ≤50% was estimated by logistic regression models. Results: SP correlated well (r = −0.699) with the average PTT of all tested frequencies. On the other hand, the correlation between patient age and SP was weak, especially among ≤85-year-old persons (r = −0.092). Linear regression showed that the average PTT corresponding to SP of 50% was 76.4 dB nHL. Odds ratios for impaired SP were highest for PTT at 2000 Hz. Odds ratios were higher for middle (500, 1000, 2000 Hz) and high frequencies (4000, 8000 Hz) than low frequencies (125, 250 Hz). Conclusion: The PTT on the pure-tone audiogram (PTA) is a good predictor of SP by speech audiometry among older persons, which could provide clinically important information for hearing aid fitting and cochlear implantation.

Pediatric central auditory processing disorder showing elevated threshold on pure tone audiogram

Central auditory processing disorder (CAPD) is a condition in which dysfunction in the central auditory system causes difficulty in listening to conversations, particularly under noisy conditions, despite normal peripheral auditory function. Central auditory testing is generally performed in patients with normal hearing on the pure tone audiogram (PTA). This report shows that diagnosis of CAPD is possible even in the presence of an elevated threshold on the PTA, provided that the normal function of the peripheral auditory pathway was verified by distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), and auditory steady state response (ASSR). Three pediatric cases (9- and 10-year-old girls and an 8-year-old boy) of CAPD with elevated thresholds on PTAs are presented. The chief complaint was difficulty in listening to conversations. PTA showed elevated thresholds, but the responses and thresholds for DPOAE, ABR, and ASSR were normal, showing that peripheral auditory function was normal. Significant findings of central auditory testing such as dichotic speech tests, time compression of speech signals, and binaural interaction tests confirmed the diagnosis of CAPD. These threshold shifts in PTA may provide a new concept of a clinical symptom due to central auditory dysfunction in CAPD.

Characteristics of audiogram configuration in multiple-system atrophy C and cortical cerebellar atrophy.

Abstract Conclusion: The prevalence of low-tone hearing loss (LTHL) is significantly high in spinocerebellar degeneration (SCD) with cerebellar predominance, including multiple-system atrophy C (MSA-C) and cortical cerebellar atrophy (CCA). Objective: This study aimed to test the hypothesis that SCD with cerebellar predominance, MSA-C and CCA may cause auditory symptoms. Methods: The shape and threshold of pure-tone audiograms were evaluated for MSA-C (n = 47; mean (± SD) age, 61.6 ± 8.9 years), CCA (n = 16; 62.8 ± 9.5 years), and age-matched controls (n = 169; 62.5 ± 10.7 years). To differentiate specific hearing loss for MSA-C and CCA from presbycusis, the shape of audiograms was examined based on previously established audiological criteria. Results: When audiogram shape was defined according to audiological criteria, the odds ratio for LTHL in SCD compared to controls was 2.492 (95% confidence interval (CI) = 1.208–5.139; p < 0.05, Pearson’s Chi-square test) in MSA-C and 2.194 (95% CI = 0.709–6.795) in CCA. When the selection of audiogram shape according to these criteria was verified by three certified audiologists, odds ratios for LTHL in MSA-C and CCA were 3.243 (95% CI = 1.320–7.969) and 3.692 (95% CI = 1.052–12.957), respectively, significantly higher than in controls.