Comparisons of Auditory Performance and Speech Intelligibility after Cochlear Implant Reimplantation in Mandarin-Speaking Users.

Objectives. We evaluated the causes, hearing, and speech performance before and after cochlear implant reimplantation in Mandarin-speaking users. Methods. In total, 589 patients who underwent cochlear implantation in our medical center between 1999 and 2014 were reviewed retrospectively. Data related to demographics, etiologies, implant-related information, complications, and hearing and speech performance were collected. Results. In total, 22 (3.74%) cases were found to have major complications. Infection (n = 12) and hard failure of the device (n = 8) were the most common major complications. Among them, 13 were reimplanted in our hospital. The mean scores of the Categorical Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR) obtained before and after reimplantation were 5.5 versus 5.8 and 3.7 versus 4.3, respectively. The SIR score after reimplantation was significantly better than preoperation. Conclusions. Cochlear implantation is a safe procedure with low rates of postsurgical revisions and device failures. The Mandarin-speaking patients in this study who received reimplantation had restored auditory performance and speech intelligibility after surgery. Device soft failure was rare in our series, calling attention to Mandarin-speaking CI users requiring revision of their implants due to undesirable symptoms or decreasing performance of uncertain cause.

Long-Term Cochlear Implant Outcomes in Children with GJB2 and SLC26A4 Mutations.

OBJECTIVES: To investigate speech and language outcomes in children with cochlear implants (CIs) who had mutations in common deafness genes and to compare their performances with those without mutations. STUDY DESIGN: Prospective study. METHODS: Patients who received CIs before 18 years of age and had used CIs for more than 3 years were enrolled in this study. All patients underwent mutation screening of three common deafness genes: GJB2, SLC26A4 and the mitochondrial 12S rRNA gene. The outcomes with CIs were assessed at post-implant years 3 and 5 using the Categories of Auditory Performance (CAP) scale, Speech Intelligibility Rating (SIR) scale, speech perception tests and language skill tests. RESULTS: Forty-eight patients were found to have confirmative mutations in GJB2 or SLC26A4, and 123 without detected mutations were ascertained for comparison. Among children who received CIs before 3.5 years of age, patients with GJB2 or SLC26A4 mutations showed significantly higher CAP/SIR scores than those without mutations at post-implant year 3 (p = 0.001 for CAP; p = 0.004 for SIR) and year 5 (p = 0.035 for CAP; p = 0.038 for SIR). By contrast, among children who received CIs after age 3.5, no significant differences were noted in post-implant outcomes between patients with and without mutations (all p > 0.05). CONCLUSION: GJB2 and SLC26A4 mutations are associated with good post-implant outcomes. However, their effects on CI outcomes may be modulated by the age at implantation: the association between mutations and CI outcomes is observed in young recipients who received CIs before age 3.5 years but not in older recipients.

Written Language Ability in Mandarin-Speaking Children with Cochlear Implants.

Objectives. To examine narrative writing in cochlear implant (CI) children and understand the factors associated with unfavorable outcomes. Materials and Methods. Forty-five CI children in grades 2-6 participated in this study. They received CIs at 4.1 ± 2.1 years of age and had used them for 6.5 ± 2.7 years. A story-writing test was conducted and scored on 4 subscales: Total Number of Words, Words per Sentence, Morphosyntax, and Semantics. Scores more than 1.5 SD lower than the mean of the normal-hearing normative sample were considered problematic. Language and speech skills were examined. Results. Significantly more implanted students were problematic on "Total Number of Words" (p < 0.001), "Words per Sentence" (p = 0.049), and "Semantics" (p < 0.001). Poorer receptive language and auditory performance were independently associated with problematic "Total Number of Words" (R (2) = 0.489) and "Semantics" (R (2) = 0.213), respectively. "Semantics" problem was more common in lower graders (grades 2-4) than in higher graders (grades 5-6; p = 0.016). Conclusion. Implanted children tend to write stories that are shorter, worse-organized, and without a plot, while formulating morphosyntactically correct sentences. Special attention is required on their auditory and language performances, which could lead to written language problems.

Comorbid alcohol dependence disorder may be related to aldehyde dehydrogenase 2 (ALDH2) and alcohol dehydrogenase 1B (ADH1B) in bipolar II disorder, but only to ALDH2 in bipolar I disorder, in Han Chinese.

OBJECTIVES: A high prevalence rate of bipolar disorder (BP) comorbid with alcohol dependence (AD) (BP+AD) in Western patients with BP has been reported, but whether this is true for Han Chinese with BP is uncertain. We explored the prevalence of BP+AD in a Han Chinese population with BP, and investigated the effect of alcohol-metabolizing genotypes on bipolar I disorder (BP-I) + AD and bipolar II disorder (BP-II) + AD. METHODS: Healthy controls (HCs) (n = 672) and 18- to 65-year-old patients with BP (BP-I: n = 530; BP-II: n = 788) were recruited. Patients with any other major or minor mental illnesses, neurological disorders, or organic mental disorders were excluded. A polymerase chain reaction and restriction fragment length polymorphism analysis was used to determine genotypes for alcohol dehydrogenase 1B (ADH1B) and aldehyde dehydrogenase 2 (ALDH2), two alcohol-metabolizing enzymes. RESULTS: AD comorbidity rates were 11.7% with BP-I and 17.1% with BP-II. Significantly fewer patients with BP not comorbid with AD (BP-AD) carried the AHD1B*1 allele than did the HCs. Logistic regression analysis showed a main effect of ALDH2*1/*1 only in the BP-I-AD group. In BP+AD patients, logistic regression analysis showed main effects of ALDH2*1/*1 and ADH1B*1/*1 only in the BP-II+AD group. CONCLUSIONS: Having BP-II+AD may be related to ALDH2 and ADH1B, but having BP-I+AD may be related only to ALDH2. We conclude that ALDH2 and ADH1B have different effects in Han Chinese patients with BP-I+AD and BP-II+AD.

Auditory performance and speech intelligibility of Mandarin-speaking children implanted before age 5.

OBJECTIVES: (1) To report the auditory performance and speech intelligibility of 84 Mandarin-speaking prelingually deaf children after using cochlear implants (CIs) for one, two, three, four, and five years to understand how many years of implant use were needed for them to reach a plateau-level performance; (2) to investigate the relation between subjective rating scales and objective measurements (i.e., speech perception tests); (3) to understand the effect of age at implantation on auditory and speech development. METHODS: Eighty-four children with CIs participated in this study. Their auditory performance and speech intelligibility were rated using the Categorical Auditory Performance (CAP) and the Speech Intelligibility Rating (SIR) scales, respectively. The evaluations were made before implantation and six months, one, two, three, four, and five years after implantation. At the fifth year after implantation, monosyllabic-word, easy-sentence, and difficult-sentence perception tests were administered. RESULTS: The median CAP score reached a plateau at category 6 after three years of implant use. The median SIR arrived at the highest level after five years of use. With five years of CI experiences, 86% of the subjects understood conversation without lip-reading, and 58% were fully intelligible to all listeners. The three speech perception tests had a moderate-to-strong correlation with the CAP and SIR scores. The children implanted before the age of three years had significantly better CAP and monosyllabic word perception test scores. CONCLUSIONS: Five years of follow-up are needed for assessing the post-implantation development of communication ability of prelingually deafened children. It is recommended that hearing-impaired children receive cochlear implantation at a younger age to acquire better auditory ability for developing language skills. Constant postoperative aural-verbal rehabilitation and speech and language therapy are most likely required for the patients to reach the highest level on the CAP and SIR scales.

Timing of surgical intervention with cochlear implant in patients with large vestibular aqueduct syndrome.

OBJECTIVES: (1) To report the speech perception and intelligibility results of Mandarin-speaking patients with large vestibular aqueduct syndrome (LVAS) after cochlear implantation (CI); (2) to compare their performance with a group of CI users without LVAS; (3) to understand the effects of age at implantation and duration of implant use on the CI outcomes. The obtained data may be used to guide decisions about CI candidacy and surgical timing. METHODS: Forty-two patients with LVAS participating in this study were divided into two groups: the early group received CI before 5 years of age and the late group after 5. Open-set speech perception tests (on Mandarin tones, words and sentences) were administered one year after implantation and at the most recent follow-up visit. Categories of auditory perception (CAP) and Speech Intelligibility Rating (SIR) scale scores were also obtained. RESULTS: The patients with LVAS with more than 5 years of implant use (18 cases) achieved a mean score higher than 80% on the most recent speech perception tests and reached the highest level on the CAP/SIR scales. The early group developed speech perception and intelligibility steadily over time, while the late group had a rapid improvement during the first year after implantation. The two groups, regardless of their age at implantation, reached a similar performance level at the most recent follow-up visit. CONCLUSION: High levels of speech performance are reached after 5 years of implant use in patients with LVAS. These patients do not necessarily need to wait until their hearing thresholds are higher than 90 dB HL or PB word score lower than 40% to receive CI. They can do it "earlier" when their speech perception and/or speech intelligibility do not reach the performance level suggested in this study.

Genetic variants of the BDNF and DRD3 genes in bipolar disorder comorbid with anxiety disorder.

BACKGROUND: The high comorbidity rate between bipolar disorder (BP) and anxiety disorder (AD) has been studied in depth. This comorbidity is not as high in Han Chinese in Taiwan. Therefore, we explored the genetic effects BP comorbid with AD. METHODS: We recruited 1316 participants: 286 with BP-I, 681 with BP-II, and 349 healthy Controls. Genotypes of the BDNF Val66Met and DRD3 Ser9Gly polymorphisms were determined using polymerase chain reactions plus restriction fragment length polymorphism analysis. RESULTS: The DRD3 Ser9Gly polymorphism was associated with BP-II comorbid with AD (BPII(+AD)), and the BDNF Val66Met polymorphism was associated with BP-I comorbid with AD (BPI(+AD)). An interaction between the Val/Val genotype of the BDNF Val66Met and Gly/Gly polymorphism of the DRD3 Ser9Gly was found in BPII(+AD), but not in BP-II not comorbid with AD (BPI(-AD)) compared with healthy Controls. LIMITATION: The low comorbidity rate of AD in both BP subtypes, especially BP-I, limit generalizing our findings. CONCLUSION: The involvement of the dopaminergic pathway in AD was confirmed, particularly with BP-II rather than BP-I. Because the Val/Val genotype of the BDNF Val66Met polymorphism, rather than the other two polymorphisms, has been associated with anxiety, it seems to affect BP-I comorbid with AD without the involvement of the DRD3 Seg9Gly polymorphism, but may modify the involvement of DRD3 Gly/Gly in BP-II comorbid with AD.

Low anxiety disorder comorbidity rate in bipolar disorders in Han Chinese in Taiwan.

OBJECTIVES: Studies report high comorbidity of lifetime anxiety disorders with bipolar disorders in Western patients, but it is unclear in Taiwan. The authors explored the comorbidity of anxiety disorders in different bipolar disorder subtypes in Han Chinese in Taiwan. METHODS: Three hundred twenty-five patients with bipolar disorder (bipolar I: 120; bipolar II: 205) disorder were recruited from two general medical outpatient services. They were evaluated and their diagnoses confirmed by a psychiatrist using the Chinese version of the Modified Schedule of Affective Disorder and Schizophrenia-Lifetime. The exclusion criteria were: any DSM-IV-TR Axis I diagnosis, other than bipolar disorder, being outside the 18-65-year-old age range, any other major and minor mental illnesses except anxiety disorder, any neurological disorders or organic mental disorders. RESULTS: Thirty-two (26.7%) of patients were comorbid with lifetime anxiety disorder and bipolar I, 80 (39.0%) with lifetime anxiety disorder and bipolar II, 7 (5.8%) were comorbid with two or more anxiety disorders and bipolar I, and 27 (13.2%) with two or more anxiety disorders and bipolar II. CONCLUSION: That more than twice as many bipolar II than bipolar I patients reported two or more anxiety disorders implies that the complication is more prevalent in bipolar II patients.

Etiologic features in patients with unilateral vocal fold paralysis in Taiwan.

BACKGROUND: To develop an updated screening, treatment and follow-up strategy for acquired unilateral vocal fold paralysis (UVFP), this Taiwan study investigated concurrent etiologies in patients treated in a tertiary medical center. METHODS: This retrospective study was conducted at the voice centers of Chang Gung Memorial Hospital, Keelung, and Chang Gung Memorial Hospital, Linkuo Medical Center, Taiwan. Outpatient and inpatient medical records of patients with UVFP treated between January, 2002 and March, 2006 were retrospectively reviewed. Patients with laryngeal/hypopharyngeal malignancies, those with incomplete examination and follow-up data and those with congenital UVFP were excluded from the study. RESULTS: One hundred sixty-one patients, including 104 females and 57 males, with ages ranging from 15 to 85 years, met the inclusion criteria. One hundred ten patients (68%) had left side UVFP. The causes were iatrogenic in 48%, subclinical tumor in 12%, neck trauma in 7%, radiation effect in 6% and other causes in 5%. Thyroidectomy was the most common surgical cause of UVFP (n = 51). The most common origins of subclinical tumors were the thyroid (n = 8) and lung (n = 6). In patients younger than 30 years, neck trauma was a major cause of UVFP. CONCLUSIONS: A tailored management strategy for UVFP is needed because vocal fold immobilization is an important sign of various underlying diseases. Extended follow-up is crucial in idiopathic UVFP due to the risk of undiagnosed subclinical tumor.