• Korean Journal of Otorhinolaryngology-Head and Neck Surgery
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• v.61 no.12
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• pp.650-657
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• 2018

Background and Objectives The present study aimed to compare receptive and expressive language development in children who have undergone simultaneous bilateral cochlear implantation (SCI) and those who have undergone bimodal stimulation (unilateral CI+ hearing aid). Subjects and Method In a retrospective analysis of clinical data, 15 pediatric patients who have received SCI and nine patients who have received bimodal stimulation (BM group) were enrolled. CI was performed for all patients at 24 months of age. Category of Auditory Performance (CAP) scores, Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) scores, and developmental quotients (DQ) for expressive and receptive language were compared between the groups at 12 month of follow-up. The Percentage of Consonants Correct (PCC) of children evaluated at 4 years old was also compared. Results At 12 months of follow-up, significantly greater improvements in CAP scores (${\Delta}4.25{\pm}0.5$) were noted in the SCI group compared to the BM group (${\Delta}3.56{\pm}0.88$, p=0.041). Significantly greater improvements in IT-MAIS scores were also noted in the SCI group (${\Delta}36.17{\pm}4.09$) than in the BM group (${\Delta}30.17{\pm}2.91$, p=0.004). The DQ of receptive language was higher in the SCI group than in the BM group ($87.6{\pm}15.4%$ vs. $75.5{\pm}12.0%$, p=0.023) at 12 months of follow-up. Moreover, early SCI was associated with better receptive language skills. PCC index of children at 4 years old was higher in the SCI group than in the BM group ($88.5{\pm}13.2%$ vs. $62{\pm}15.8%$, p=0.014). Earlier SCI was associated with even greater improvements. Conclusion Bilateral SCI is associated with significant improvements in language development when compared with bimodal stimulation. Earlier SCI was associated with better outcomes.

• Journal of Audiology & Otology
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• v.23 no.4
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• pp.193-196
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• 2019

Background and Objectives: Facial nerve stimulation (FNS) is a complication of cochlear implantation (CI). This study compared the thickness and density of the bone separating the upper basal turn of the cochlea (UBTC) and the labyrinthine segment of the facial nerve (LSFN) on preoperative computed tomography (CT) in patients with and without FNS after CI. Subjects and Methods: Adult patients who underwent CI from January 2011 to February 2017 with preoperative CT at a tertiary referral hospital were considered for this retrospective case-control study. Patients were divided into two groups: with FNS (n=4) and without FNS (n=53). The density and thickness of the bone between the LSFN and UBTC were measured on preoperative CT. Charts were reviewed for other parameters. Results: A statistically significant difference was seen in the thickness (p=0.007) but not in the density (p=0.125) of the bone between the UBTC and LSFN. Four patients had FNS at the mid-range electrode arrays, and one of them additionally had FNS at the basal arrays. Conclusions: Decreased thickness of the bone between the UBTC and LSFN can explain postoperative FNS, confirming the histologic and radiologic findings in previous studies, which indicated that the thickness of the temporal bone between the LSFN and UBTC is less in patients who experience FNS. While the density in this region was also less, it was not statistically significant.

• Korean Journal of Audiology
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• v.23 no.4
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• pp.193-196
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• 2019

Background and Objectives: Facial nerve stimulation (FNS) is a complication of cochlear implantation (CI). This study compared the thickness and density of the bone separating the upper basal turn of the cochlea (UBTC) and the labyrinthine segment of the facial nerve (LSFN) on preoperative computed tomography (CT) in patients with and without FNS after CI. Subjects and Methods: Adult patients who underwent CI from January 2011 to February 2017 with preoperative CT at a tertiary referral hospital were considered for this retrospective case-control study. Patients were divided into two groups: with FNS (n=4) and without FNS (n=53). The density and thickness of the bone between the LSFN and UBTC were measured on preoperative CT. Charts were reviewed for other parameters. Results: A statistically significant difference was seen in the thickness (p=0.007) but not in the density (p=0.125) of the bone between the UBTC and LSFN. Four patients had FNS at the mid-range electrode arrays, and one of them additionally had FNS at the basal arrays. Conclusions: Decreased thickness of the bone between the UBTC and LSFN can explain postoperative FNS, confirming the histologic and radiologic findings in previous studies, which indicated that the thickness of the temporal bone between the LSFN and UBTC is less in patients who experience FNS. While the density in this region was also less, it was not statistically significant.

• Journal of Preventive Medicine and Public Health
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• v.37 no.4
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• pp.353-358
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• 2004

Objective : To determine the quality of life and cost consequences for deaf adults who received a cochlear implant. Methods : The data from 11 patients, post-lingual deaf adults who received cochlear implants from 1990 to 2002, underwent cost-utility analysis. The average age of the participants was 49.6 years. The main outcomes were direct cost per quality-adjusted life-year (QALY) using the visual analog scale (VAS), health utility index (HUI), EuroQol (EQ-5D), and quality well-being (QWB), with costs and utilities being discounted 3% annually. Results : Recipients had an average of 5.6 years of implant use. Mean VAS scores increased by 0.33, from 0.27 before implantation to 0.60 at survey. HUI scores increased by 0.36, from 0.29 to 0.65, EQ-5D scores increased by 0.26, from 0.52 to 0.78, and QWB scores increased by 0.16, from 0.45 to 0.61. Discounted direct costs were $22,320, yielding $19,223/QALY using VAS, $17,387/QALY using HUI, $24,604/QALY using EQ-5D, and $40,474/QALY using QWB. Cost-utility ratios using VAS, HUI, and EQ-5D were all below $25,000 per QALY, except using QWB. Conclusion : Cochlear implants in post-lingual deaf adult have a positive effect on quality of life at reasonable direct costs and appear to produce a net saving to society.

• Phonetics and Speech Sciences
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• v.6 no.4
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• pp.171-179
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• 2014

Purposes: Speech acceptability means the subjective judgement of listeners regarding the naturalness and normality of the speech. The purpose of this study was to determine the predicting variables for speech acceptabilities of children with cochlear implants. Methods: Twenty seven children with CI participated. They had profound pre-lingual hearing loss without any additional disabilities. The mean of chronological ages was 8;9, and mean of age of implantation was 2;11. Speech samples of reading and spontaneous speech were recorded separately. Twenty college students who were not familiar to the speech of deaf children evaluated the speech acceptabilities using visual analog scale. 1 segmental (articulation) and 6 suprasegmental features (pitch, loudness, quality, resonance, intonation, and speaking rate) of speech were perceptually evaluated by 3 SLPs. Correlation and multiple regression analysis were performed to evaluate the predicting variables. Results: The means of speech acceptability for reading and spontaneous speech were 73.47 and 71.96, respectively. Speech acceptability of reading was predicated by the severity of intonation and articulation. Speech acceptability of spontaneous speech was predicated by the severity of intonation and loudness. Discussion and conclusion: Severity of intonation was the most effective variable to predict the speech acceptabilities of both reading and spontaneous speech. A further study would be necessary to generalize the result and to apply this result to intervention in clinical settings.

• Korean Journal of Audiology
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• v.25 no.2
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• pp.110-114
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• 2021

We report a case of hearing rehabilitation following combined cochlear implantation and ossiculoplasty. A 71-year-old patient visited the clinic for right-sided mixed hearing loss. We targeted neural and conductive components, performing two different operations simultaneously. At two months post-operative, the patient showed satisfactory results with respect to hearing threshold and speech comprehension. Our experience suggests that careful evaluation of patients and consideration of the diverse array of available treatment strategies can be used to provide personalized rehabilitation with maximal effectiveness. To the best of our knowledge, this is the first report to take such an integrated approach to treat hearing impairment and is thus likely to have clinical importance for otologists.

• Journal of Audiology & Otology
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• v.25 no.2
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• pp.110-114
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• 2021

We report a case of hearing rehabilitation following combined cochlear implantation and ossiculoplasty. A 71-year-old patient visited the clinic for right-sided mixed hearing loss. We targeted neural and conductive components, performing two different operations simultaneously. At two months post-operative, the patient showed satisfactory results with respect to hearing threshold and speech comprehension. Our experience suggests that careful evaluation of patients and consideration of the diverse array of available treatment strategies can be used to provide personalized rehabilitation with maximal effectiveness. To the best of our knowledge, this is the first report to take such an integrated approach to treat hearing impairment and is thus likely to have clinical importance for otologists.

• Molecules and Cells
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• v.38 no.9
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• pp.781-788
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• 2015

Mutations of MYO15A are generally known to cause severe to profound hearing loss throughout all frequencies. Here, we found two novel MYO15A mutations, c.3871C>T (p.L1291F) and c.5835T>G (p.Y1945X) in an affected individual carrying congenital profound sensorineural hearing loss (SNHL) through targeted resequencing of 134 known deafness genes. The variant, p.L1291F and p.Y1945X, resided in the myosin motor and IQ2 domains, respectively. The p.L1291F variant was predicted to affect the structure of the actin-binding site from three-dimensional protein modeling, thereby interfering with the correct interaction between actin and myosin. From the literature analysis, mutations in the N-terminal domain were more frequently associated with residual hearing at low frequencies than mutations in the other regions of this gene. Therefore we suggest a hypothetical genotype-phenotype correlation whereby MYO15A mutations that affect domains other than the N-terminal domain, lead to profound SNHL throughout all frequencies and mutations that affect the N-terminal domain, result in residual hearing at low frequencies. This genotype-phenotype correlation suggests that preservation of residual hearing during auditory rehabilitation like cochlear implantation should be intended for those who carry mutations in the N-terminal domain and that individuals with mutations elsewhere in MYO15A require early cochlear implantation to timely initiate speech development.

• Journal of Audiology & Otology
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• v.23 no.3
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• pp.153-159
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• 2019

Background and Objectives: We aim to explore the effects of residual auditory steady state response (ASSR) on cochlear implantation (CI) outcomes in children lacking auditory brainstem responses (ABRs). Subjects and Methods: We retrospectively reviewed the data of child CI recipients lacking ABRs. All ears were divided into two groups: with residual ASSR and without ASSR. For each frequency, the T- and C-levels and the electrical dynamic ranges of postoperative 3-month and 1-year mappings were compared between the groups. To evaluate speech perception, patients who received simultaneous bilateral CIs were divided into two groups: group 1 exhibited responses at all frequencies in both ears; in group 2, at least one ear evidenced no response. The Categories of Auditory Perception (CAP) and Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) scores were compared between the groups. Results: We enrolled 16 patients. At 2 kHz, the postoperative 3-month and 1-year T-levels of patients with residual hearing were lower than those of hearing loss group (p=0.001, p=0.035). In residual hearing group, the ASSR threshold correlated positively with the postoperative 1-year T-level (p=0.012, R²=0.276) and C-level (p=0.002, R²=0.374). Of 10 simultaneous bilateral CI recipients, 5 exhibited ASSRs at all frequencies and the other 5 showed no response at ≥1 frequency. The latter had higher CAP scores at the postoperative 1-year (p=0.018). Conclusions: In children exhibiting hearing loss in ABR testing, residual hearing at 2 kHz ASSR correlated positively with the post-CI T-level. Those with ASSRs at all frequencies had significantly lower CAP scores at the postoperative 1year. CI should not be delayed when marginal residual hearing is evident in ASSR.

• Korean Journal of Audiology
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• v.23 no.3
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• pp.153-159
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• 2019

Background and Objectives: We aim to explore the effects of residual auditory steady state response (ASSR) on cochlear implantation (CI) outcomes in children lacking auditory brainstem responses (ABRs). Subjects and Methods: We retrospectively reviewed the data of child CI recipients lacking ABRs. All ears were divided into two groups: with residual ASSR and without ASSR. For each frequency, the T- and C-levels and the electrical dynamic ranges of postoperative 3-month and 1-year mappings were compared between the groups. To evaluate speech perception, patients who received simultaneous bilateral CIs were divided into two groups: group 1 exhibited responses at all frequencies in both ears; in group 2, at least one ear evidenced no response. The Categories of Auditory Perception (CAP) and Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) scores were compared between the groups. Results: We enrolled 16 patients. At 2 kHz, the postoperative 3-month and 1-year T-levels of patients with residual hearing were lower than those of hearing loss group (p=0.001, p=0.035). In residual hearing group, the ASSR threshold correlated positively with the postoperative 1-year T-level (p=0.012, R²=0.276) and C-level (p=0.002, R²=0.374). Of 10 simultaneous bilateral CI recipients, 5 exhibited ASSRs at all frequencies and the other 5 showed no response at ≥1 frequency. The latter had higher CAP scores at the postoperative 1-year (p=0.018). Conclusions: In children exhibiting hearing loss in ABR testing, residual hearing at 2 kHz ASSR correlated positively with the post-CI T-level. Those with ASSRs at all frequencies had significantly lower CAP scores at the postoperative 1year. CI should not be delayed when marginal residual hearing is evident in ASSR.