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1.
Sci Rep ; 9(1): 1651, 2019 02 07.
Artigo em Inglês | MEDLINE | ID: mdl-30733597

RESUMO

Among European countries, optimal birth weight at which the mortality is minimal is shown to be different by country. We investigated this difference examining one geopolitical population, the U.S. term live births, born to the five groups of the same ethnic parents; White, Black, Hispanic, North Asian, and South Asian. North Asians and South Asians had more favorable maternal factors for birth weight. Yet, Whites had the highest mean birth weight and South Asians, the lowest. However, neonatal mortality rate in Whites was 0.78 per 1,000 live births, significantly higher than 0.36 and 0.72 per 1,000 live births in North Asians and South Asians, respectively. Other maternal factors hardly explained this ethnic disparity in birth weight or mortality. Optimal birth weight was greatest in Whites (3,890 g), and least in South Asians (3,491 g). However, neonatal mortality at optimal birth weight was significantly lower in North Asians. Adjustment of maternal factors except parental ethnicity changed little of this difference. Optimal birth weight and its mortality differ by ethnicity. On planning the best birth outcome in a population, one should consider the variable mortality risks by ethnicity.


Assuntos
Peso ao Nascer , Etnicidade/estatística & dados numéricos , Mortalidade Infantil/tendências , Nascido Vivo , Adulto , Feminino , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Masculino , Gravidez , Estados Unidos
2.
J Audiol Otol ; 21(2): 95-102, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28704896

RESUMO

BACKGROUND AND OBJECTIVES: The mutation of the gap junction protein beta 2 (GJB2) gene is the predominant cause of autosomal recessive non-syndromic hearing loss. The purpose of this study was to evaluate the speech perception outcome after cochlear implantation according to the presence of a GJB2 mutation. SUBJECTS AND METHODS: During the period from March 2004 to February 2005, 38 patients underwent cochlear implantation at Asan Medical Center. Genetic factors and speech perception were evaluated in all subjects, and the patients were grouped according to the presence of a GJB2 mutation. The two groups were carefully matched according to the age at cochlear implantation. We analyzed four mutations in the GJB2 gene: 35delG, 167delT, 235delC, and E114G. Speech perception outcomes were measured using the open set, 1 and 2 syllables, the comprehension test, the Meaningful Auditory Integration Scale, the categories of auditory performance, and the Speech Intelligibility Rating scores. The evaluations were performed before the operation, 6 and 12 months thereafter, and then annually up to nine years after cochlear implantation. RESULTS: Fifteen patients had bi-allelic GJB2 mutations (11 with E114G and 4 with 235delC), whereas the remaining 23 had wild type alleles. For the age-matched analysis, 14 patients were selected and divided into two groups of 7 subjects each: GJB2 mutation and no mutation (i.e., deafness of unknown origin). Overall, all patients showed improvement of speech perception outcome after cochlear implantation. There was no difference in the improvement between patients with and without GJB2 mutations at the 5-year and 9-year follow up. The pattern of improvement throughout the duration of the follow-up also showed no difference between the two groups. CONCLUSIONS: Similar outcomes of speech perception are expected after cochlear implantation in pediatric patients with or without GJB2 mutation.

3.
Clin Mol Hepatol ; 23(2): 160-169, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28506055

RESUMO

BACKGROUND/AIMS: Little is known about the treatment or outcomes of hepatocellular carcinoma (HCC) complicated with bile duct invasion. METHODS: A total of 247 consecutive HCC patients with bile duct invasion at initial diagnosis were retrospectively included. RESULTS: The majority of patients had Barcelona Clinic Liver Cancer (BCLC) stage C HCC (66.8%). Portal vein tumor thrombosis was present in 166 (67.2%) patients. Median survival was 4.1 months. Various modalities of treatment were initially employed including surgical resection (10.9%), repeated transarterial chemoembolization (TACE) (42.5%), and conservative management (42.9%). Among the patients with obstructive jaundice (n=88), successful biliary drainage was associated with better overall survival rate. Among the patients with BCLC stage C, overall survival differed depending on the initial treatment for HCC; surgical resection, TACE, systemic chemotherapy, and conservative management showed overall survival rates of 11.5, 6.0 ,2.4, and 1.6 months, respectively. After adjusting for confounders, surgical resection and repeated TACE were significant prognostic factors for HCC patients with bile duct invasion (hazard ratios 0.47 and 0.39, Ps <0.001, respectively). CONCLUSIONS: The survival of HCC patients with bile duct invasion at initial diagnosis is generally poor. However, aggressive treatments for HCC such as resection or biliary drainage may be beneficial therapeutic options for patients with preserved liver function.


Assuntos
Neoplasias dos Ductos Biliares/diagnóstico , Carcinoma Hepatocelular/diagnóstico , Neoplasias Hepáticas/diagnóstico , Idoso , Neoplasias dos Ductos Biliares/secundário , Ductos Biliares/patologia , Carcinoma Hepatocelular/mortalidade , Carcinoma Hepatocelular/patologia , Carcinoma Hepatocelular/terapia , Quimioembolização Terapêutica , Feminino , Humanos , Icterícia/etiologia , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/terapia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento , alfa-Fetoproteínas/análise
4.
J Korean Med Sci ; 31(12): 1949-1956, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27822934

RESUMO

Despite a remarkable increase in Asian births in the U.S., studies on their birth outcomes have been lacking. We investigated outcomes of births to Asian parents and biracial Asian/White parents in the U.S. From the U.S. birth data (1992-2012), we selected singleton births to Korean, Chinese, Japanese, Filipino, Asian Indian, and Vietnamese. These births were divided into three groups; births to White mother/Asian father, Asian mother/White father, and births to the both ethnic Asian parents. We compared birth outcomes of these 18 subgroups to those of the White mother/White father group. Mean birthweights of births to the Asian parents were significantly lower, ranging 18 g to 295 g less than to the White parents. Compared to the rates of low birthweight (LBW) (4.6%) and preterm birth (PTB) (8.5%) in births to the White parents, births to Filipino parents had the highest rates of LBW (8.0%) and PTB (11.3%), respectively, and births to Korean parents had the lowest rates of both LBW (3.7%) and PTB (5.5%). This pattern of outcomes had changed little with adjustments of maternal sociodemographic and health factors. This observation was similarly noted also in births to the biracial parents, but the impact of paternal or maternal race on birth outcome was different by race/ethnicity. Compared to births to White parents, birth outcomes from the Asian parents or biracial Asian/White parents differed depending on the ethnic origin of Asian parents. The race/ethnicity was the strongest factor for this difference while other parental characteristics hardly explained this difference.


Assuntos
Nascido Vivo/psicologia , Povo Asiático/etnologia , Peso ao Nascer , Bases de Dados Factuais , Humanos , Recém-Nascido de Baixo Peso , Razão de Chances , Pais , Nascimento Prematuro , Risco , Estados Unidos , População Branca/etnologia
5.
J Korean Med Sci ; 31(9): 1450-4, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27510390

RESUMO

Difference in crude neonatal and infant mortality rates (NMR and IMR) among different countries is due to the differences in its two determinants: birth weight distribution (BWD) and birth weight-specific mortality rates (BW-SMRs). We aimed to determine impact of BWD and BW-SMRs on differences in crude NMR and IMR among Korea, Japan, and the U.S. Our study used the live birth data of the period 2009 through 2010. Crude NMR/IMR are the lowest in Japan, 1.1/2.1, compared to 1.8/3.2, in Korea, and 4.1/6.2, in the U.S., respectively. Japanese had the best BW-SMRs of all birth weight groups compared to the Koreans and the U.S. The U.S. BWD was unfavorable with very low birth weight (< 1,500 g) rate of 1.4%, compared to 0.6% in Korea, and 0.8% in Japan. If Koreans and Japanese had the same BWD as in the U.S., their crude NMR/IMR would be 3.9/6.1 for the Koreans and 1.5/2.5 for the Japanese. If both Koreans and Japanese had the same BW-SMRs as in the U.S., the crude NMR/IMR would be 2.0/3.8 for the Koreans and 2.7/5.0 for the Japanese. In conclusion, compared to the U.S., lower crude NMR or IMR in Japan is mainly attributable to its better BW-SMRs. Koreans had lower crude NMR and IMR, primarily from its favorable BWD. Comparing crude NMR or IMR among different countries should include further exploration of its two determinants, BW-SMRs reflecting medical care, and BWD reflecting socio-demographic conditions.


Assuntos
Peso ao Nascer , Mortalidade Infantil , Bases de Dados Factuais , Humanos , Lactente , Mortalidade Infantil/etnologia , Recém-Nascido , Japão , República da Coreia , Estados Unidos
6.
Acta Otolaryngol ; 136(9): 912-8, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27151170

RESUMO

CONCLUSION: This study shows that, in cochlear implantation (CI) surgery, pre-operative caloric test results are not correlated with post-operative outcomes of dizziness or speech perception. OBJECTIVES: To determine the role of pre-operative caloric tests in CI. METHODS: The records of 95 patients who underwent unilateral CI were reviewed retrospectively. Patients were divided into four groups according to caloric response. Forty-six patients with normal caloric responses were classified as Group A, 19 patients who underwent CI in the ear with worse caloric responses as Group B, 18 patients with bilateral loss of caloric responses as Group C, and 12 patients who underwent CI in the ear with better caloric responses as Group D. Speech performance and post-operative dizziness were compared between the four groups. Speech perception was determined by evaluating consonant-vowel phoneme detection, closed-set word and open-set mono-syllabic and bi-syllabic word identification, and sentence comprehension test scores. RESULTS: The speech perception and aided pure-tone average (PTA) test results at 3 and 6 months and at 1, 2, and 3 years after implantation were not significantly different between Groups A, B, C, and D (p > 0.05). Eight patients (8.4%) reported post-operative dizziness, but there was no significant difference between the four groups (p = 0.627).


Assuntos
Testes Calóricos , Implante Coclear , Percepção da Fala , Adulto , Idoso , Idoso de 80 Anos ou mais , Tontura/etiologia , Feminino , Perda Auditiva/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/etiologia , Valor Preditivo dos Testes , Cuidados Pré-Operatórios , Estudos Retrospectivos , Adulto Jovem
7.
Otol Neurotol ; 36(1): e11-7, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-25514784

RESUMO

OBJECTIVE: To compare audiologic performance after cochlear implantation (CI) in children with incomplete partition (IP) Type I and age-matched children with normal cochleae. STUDY DESIGN: Retrospective chart review. SETTING: Academic center. PATIENTS: Twenty-three children (25 ears) with IP Type I and 230 age-matched deaf children (230 ears) with nonsyndromic normal inner ears who underwent CI between January 2000 and June 2013. INTERVENTION CI MAIN OUTCOME MEASURE: The Categories of Auditory Performance (CAP) Scale score and the Meaningful Auditory Integration Scale (MAIS) score. RESULTS: The mean age of IP Type I patients at the time of CI was 5.3 years (standard deviation, 5.4 yr; range, 0.9-17.7 yr). The mean duration of follow-up was 4.7 years (standard deviation, 3.5 yr; range, 1.1-11.2 yr). Fourteen of the 25 IP Type I ears (56%) had cerebrospinal fluid gusher during the cochleostomy. In the IP type ears, the number of inserted electrodes was 16.3 ± 3.2 (range, 11-22), and the insertion angle was 236.5 ± 41.2 degrees (range, 180-305 degrees). The cochlear nerve was assessed in 17 of the 25 IP type ears, and hypoplasia was present in nine (53%). Facial nerve stimulation occurred in 15 of the 25 IP type ears. IP Type I patients younger than 3 years at CI had significantly lower CAP Scale and MAIS scores than age-matched controls at 12 and 24 months after CI, but similar CAP Scale and MAIS scores as age-matched controls at 42 and 72 months after CI. IP Type I patients aged 3 to 18 years at CI had similar CAP Scale and MAIS scores as age-matched controls at all post-CI time points. CONCLUSION: Children with IP Type I who underwent CI performed as well as children with normal cochlea in the long-term.


Assuntos
Cóclea/anormalidades , Cóclea/cirurgia , Implante Coclear/métodos , Surdez/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Resultado do Tratamento
8.
Liver Int ; 35(1): 232-9, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24576055

RESUMO

BACKGROUND & AIMS: The aim of this study was to re-evaluate the diagnostic performance of alpha-foetoprotein (AFP) as a surveillance test for hepatocellular carcinoma (HCC) in patients with hepatitis B virus-related chronic liver disease who were treated with entecavir (ETV). METHODS: Between January 2007 and August 2012, we analysed 373 treatment-naïve patients with HBV-related chronic hepatitis (n = 229) or cirrhosis (n = 144) who were candidates for surveillance test, and were treated with ETV (0.5 mg/day) for longer than 12 months. To minimize the effect of AFP elevation caused by hepatitis activity, serum AFP levels were measured 12 months after the initiation of ETV treatment. RESULTS: Hepatocellular carcinoma developed in 28 patients (7.5%) during a median follow-up period of 48.0 months (IQR = 40.5-57.3 months). The area under the receiver operating characteristic curve for AFP was 0.71 (95% CI = 0.59-0.84). The optimal AFP cut-off value was 13 ng/ml, leading to a sensitivity of 50.0%, specificity of 98.8%, positive predictive value of 77.8% and negative predictive value of 96.1%. In multivariate Cox analysis, an older age, the presence of cirrhosis and AFP levels of ≥20 ng/ml at 12 months after treatment were found to be significantly associated with an increased incidence of HCC. CONCLUSIONS: The role of serum AFP as a surveillance test should be re-evaluated in patients with HBV-related chronic liver diseases who were treated with antiviral therapy.


Assuntos
Carcinoma Hepatocelular/diagnóstico , Guanina/análogos & derivados , Hepatite B/complicações , Hepatite B/tratamento farmacológico , Neoplasias Hepáticas/diagnóstico , alfa-Fetoproteínas/análise , Adulto , Área Sob a Curva , Carcinoma Hepatocelular/etiologia , Feminino , Guanina/uso terapêutico , Humanos , Neoplasias Hepáticas/etiologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Modelos de Riscos Proporcionais , Curva ROC , Sensibilidade e Especificidade
9.
Otol Neurotol ; 35(4): 662-6, 2014 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-24622020

RESUMO

OBJECTIVE: The aim of this article was to assess the prevalence and influence of anomalous facial nerves in patients undergoing surgery for congenital stapes anomalies. PATIENTS: The medical records of all patients who underwent surgery for congenital stapes fixation between January 1999 and December 2012 were retrospectively reviewed. An abnormal facial nerve was found in 7 ears in 5 patients (from a total of 62 ears). MAIN OUTCOME MEASURES: The patients' temporal bone computed tomography results, operation records, video clips, and preoperative and postoperative hearing tests were reviewed. RESULTS: Oval window atresia was found in all 7 ears, and the facial nerves in all 7 ears ran inferior medially over the atretic oval window niche. The atretic plate was fenestrated into the vestibule in the superior margin of the facial nerve. A piston wire was anchored to the long process of the incus in 3 ears, to the handle of the malleus in 3 ears, and attached to the tympanic membrane in 1 ear. The postoperative ABG at long-term follow-up was 13.1 dB (SD, 4.6 dB; range, 8.8-18.3 dB). No patient had postoperative facial weakness. CONCLUSION: An anomalous facial nerve was found in 11.2% of ears in patients undergoing surgery for congenital stapes fixation. Despite the anomalous course of facial nerves in these patients, vestibular fenestration was successful without facial nerve damage. The audiometric outcome at long-term follow-up was serviceable hearing for all cases.


Assuntos
Nervo Facial/anormalidades , Perda Auditiva Condutiva/etiologia , Perda Auditiva Condutiva/cirurgia , Mobilização do Estribo/métodos , Cirurgia do Estribo/métodos , Estribo/anormalidades , Adolescente , Adulto , Anestesia Geral , Audiometria , Condução Óssea/fisiologia , Criança , Pré-Escolar , Ossículos da Orelha/anormalidades , Ossículos da Orelha/cirurgia , Feminino , Seguimentos , Humanos , Masculino , Martelo/cirurgia , Janela do Vestíbulo/anatomia & histologia , Radiografia , Estudos Retrospectivos , Osso Temporal/diagnóstico por imagem , Resultado do Tratamento , Vestíbulo do Labirinto/anatomia & histologia
10.
Int J Pediatr Otorhinolaryngol ; 78(1): 55-9, 2014 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24287253

RESUMO

OBJECTIVES: The aims of this study were to review the causes of stapes fixation in children undergoing stapes surgery and to analyze the results of stapes surgery in children in the short term, at 1 year, and over the long term. METHODS: The medical records of 18 children (28 ears) who had undergone stapes surgery between January 1999 and December 2012 were retrospectively reviewed. The medical history, computed tomography results, intraoperative findings, video clips, and hearing outcomes of all patients were reported. RESULTS: The mean age of patients was 11.1 years (range, 5.9-15.3 years). Congenital stapes fixation (22/28 ears, 79%) and juvenile otosclerosis (6/28 ears, 21%) were responsible for all cases of stapes fixation. Intraoperatively, abnormal facial nerves that were downwardly displaced over the stapes footplate were noted in four ears. Incudostapedotomy was performed in 24 ears, malleostapedotomy in three, and partial stapedectomy in one. The early postoperative audiometric outcome was favorable in 21 ears (87.5%). There was no significant difference between early postoperative (87.5%), 1 year postoperative (91%), and long term (92.3%) favorable audiometric results. There was no significant difference in the postoperative hearing results between patients with congenital stapes fixation and juvenile otosclerosis. CONCLUSION: Congenital stapes fixation was diagnosed in 22 (79%) ears and juvenile otosclerosis in six (21%) ears from a series of 28 ears that were operated on for stapes fixation. Facial nerve anomalies were found in four of 22 ears (18%) that had congenital stapes fixation. There was no difference in the postoperative hearing results between patients with congenital stapes fixation and juvenile otosclerosis. Regardless of the cause of stapes fixation, stapedotomy is a safe and effective procedure for managing the condition.


Assuntos
Perda Auditiva Condutiva/cirurgia , Cirurgia do Estribo/métodos , Estribo/anormalidades , Adolescente , Audiometria , Criança , Pré-Escolar , Feminino , Perda Auditiva Condutiva/etiologia , Humanos , Masculino , República da Coreia , Estudos Retrospectivos , Resultado do Tratamento
11.
Laryngoscope ; 124(3): 746-50, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23794324

RESUMO

OBJECTIVES/HYPOTHESIS: Although labyrinth fistulae are caused mostly by cholesteatoma, they can occur in long-standing chronic otitis media (COM) without cholesteatoma. We aimed to compare the prevalence of radiologic SCD on computed tomography (CT) between normal ears and contralateral COM ears in patients with unilateral COM and to assess the prevalence of superior canal dehiscence (SCD) according to the age. STUDY DESIGN: Case series with comparison performed at a tertiary care academic referral center. METHODS: We retrospectively reviewed consecutive temporal bone CT scans of 759 patients with unilateral COM between 2009 and 2011. The mean (± standard deviation) age was 48 years (±14 years). Images were independently evaluated by two otologists, and the bone overlying the superior canal was characterized as normal, suspicious, or definite SCD. RESULTS: The prevalence (3.4%) of definite SCD in COM ears was significantly higher than that (0.3%) in normal ears. The prevalence (6.6%) of suspicious or definite SCD in COM ears was also higher than that (1.2%) in normal ears. There was no correlation between the prevalence of SCD and age in either normal or COM ears. All of the normal ears with suspicious or definite SCD also showed contralateral suspicious or definite SCD (bilateral involvement). CONCLUSIONS: Our present findings suggest that the COM is related to the presence of SCD. The roof of the temporal bone may become thin by the failure of postnatal bone development and susceptible to chronic brain pulsation and pressure exerted by the temporal lobe in COM ears.


Assuntos
Doenças do Labirinto/diagnóstico por imagem , Doenças do Labirinto/epidemiologia , Otite Média/diagnóstico por imagem , Otite Média/epidemiologia , Canais Semicirculares/diagnóstico por imagem , Osso Temporal/diagnóstico por imagem , Centros Médicos Acadêmicos , Adulto , Distribuição por Idade , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Causalidade , Doença Crônica , Comorbidade , Feminino , Humanos , Doenças do Labirinto/fisiopatologia , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Otite Média/fisiopatologia , Prevalência , Prognóstico , Valores de Referência , Estudos Retrospectivos , Medição de Risco , Canais Semicirculares/fisiopatologia , Distribuição por Sexo , Osso Temporal/fisiopatologia , Tomografia Computadorizada por Raios X/métodos
12.
Acta Otolaryngol ; 133(11): 1148-53, 2013 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-24125186

RESUMO

CONCLUSION: Regardless of the extent of inner ear anomalies and intellectual faculties, cochlear implantation (CI) with careful treatment planning can be a highly effective option for hearing rehabilitation in children with sensorineural hearing loss and CHARGE syndrome. OBJECTIVE: CHARGE is a mnemonic term for coloboma, heart defects, choanal atresia, retarded growth and development, genital abnormalities, and ear anomalies. CHARGE syndrome is one of the leading causes of congenital deafness and blindness in children. We evaluated the language performance of six pediatric patients with CHARGE syndrome. METHODS: Six patients with CHARGE syndrome underwent CI at the Asan Medical Center in Seoul between 2002 and 2012. The mean age of these CI patients was 4.9 years (range 2-9.8 years). All six patients had inner ear malformations; five children had an auricle anomaly and otitis media with effusion; three of these cases showed ossicle anomalies and four patients had downward displacement of the facial nerve (FN) toward the promontory; five patients showed delayed development; four of these children had cardiovascular malformations; three patients had cryptorchidism; two had choanal atresia; and there was one case of coloboma. RESULTS: In the initial operations, two patients each were implanted with a CI512, CI24R, or CI24RE device. One patient initially received a CI512 device in the right ear, followed by a CI24RE device in the left ear. Another patient (case 1) first received a CI24R and then CI24RST device in the right ear due to poor performance, but this was eventually removed due to exposure of the electrode from adhesive otitis media and continuing poor performance. Assessment of the meaningful auditory integration scale (MAIS) showed that four patients reached a score above 95% and one patient achieved 60%. The categories of auditory performance (CAP) score showed that one patient reached CAP 7 at 2 years postoperatively and two cases showed CAP 5.5 at 1 year after implantation, while the remaining two patients had CAP scores of 3 at 12 months and 4.5 at 4 years, respectively. The speech intelligibility rating (SIR) score at 18 months post-surgery was 4.5 in two patients, 2.5 in two patients, and 1.5 in one case. The remaining patient (case 1) who was explanted showed no meaningful speech improvement.


Assuntos
Síndrome CHARGE/cirurgia , Implante Coclear , Surdez/congênito , Síndrome CHARGE/complicações , Criança , Pré-Escolar , Surdez/cirurgia , Feminino , Testes Auditivos , Humanos , Masculino , Estudos Retrospectivos , Resultado do Tratamento
13.
Otol Neurotol ; 34(3): 544-8, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23470556

RESUMO

OBJECTIVE: To compare audiologic performances of cochlear implantation (CI) in children with X-linked deafness with those of CI in age- and sex-matched children with normal cochleae. PATIENTS: We identified 4 patients with X-linked deafness and selected 10 age- and sex-matched deaf patients with normal cochleae between April 1999 and April 2012. MAIN OUTCOME MEASURES: Auditory brainstem responses, Categories of Auditory Performance (CAP), and Meaningful Auditory Integration Scale (MAIS). RESULTS: The ages of patients with X-linked deafness at the time of implantation ranged between 1.3 and 13.6 years, with a mean age of 5.3 years. All patients were male subjects and showed severe-to-profound hearing loss. Patient 1 lacked ABR in both ears. Patients 2, 3, and 4 showed ABR in the contralateral ear, at 70, 70, and 90 dB nHL, respectively. All 4 patients had bilateral X-linked cochlear anomaly and experienced cerebrospinal fluid (CSF) gushers. Straight electrodes were used in Patients 1 and 3, with perimodiolar electrodes used in the others. Patients 2 and 3, who were about the same age, had CAP scores of 6 or more at 18 months after activation of the implant despite the use of different electrode arrays. There were no statistically significant differences in MAIS and CAP scores between each patient with X-linked deafness and the control group. CONCLUSION: The audiologic performances of patient with X-linked deafness after CI are comparable to those of patients with normal inner ear structure after CI.


Assuntos
Cóclea/cirurgia , Implante Coclear , Doenças Genéticas Ligadas ao Cromossomo X/cirurgia , Perda Auditiva Neurossensorial/cirurgia , Percepção da Fala/fisiologia , Adolescente , Criança , Pré-Escolar , Cóclea/anormalidades , Cóclea/fisiopatologia , Doenças Genéticas Ligadas ao Cromossomo X/fisiopatologia , Perda Auditiva Neurossensorial/fisiopatologia , Humanos , Lactente , Masculino , Pessoas com Deficiência Auditiva
14.
Int J Pediatr Otorhinolaryngol ; 77(4): 530-3, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23352796

RESUMO

OBJECTIVE: The aim of this study was to characterize the various bony anomalies of the inner ear in patients with unilateral sensorineural hearing loss using high-resolution temporal bone computed tomography and establish the relationship between hearing and the diameter of the bony cochlear nerve canal. METHODS: We performed a retrospective review of 51 patients (mean age 11 years, range 0-20 years, 27 boys, 24 girls) who were diagnosed with unilateral sensorineural hearing loss with an audiogram. Coronal and axial diameter of the inner ear structures, including the internal auditory canal, bony cochlear nerve canal, and each turn of the cochlea and semicircular canals, were measured with high-resolution temporal bone computed tomography. The mean values (± 2 standard deviations) were calculated and compared between sensorineural hearing loss and normal ears, and between narrow bony cochlear nerve canal and normal bony cochlear nerve canal ears. Bony cochlear nerve canal atresia/stenosis was defined as a value less than 1.4mm in axial images. RESULTS: The diameter of the bony cochlear nerve canal was significantly smaller in sensorineural hearing loss ears than in normal ears (p<.05). Associated inner ear anomalies, such as IAC stenosis (24%), cochlear hypoplasia (7-17%), and narrow semicircular canal bony island (8%) were only observed in the narrow bony cochlear nerve canal group. This group also showed statistically significant, severe to profound hearing loss compared to the normal bony cochlear nerve canal group (p<.05, R(2)=12.8%). CONCLUSIONS: Most (57%) of the unilateral sensorineural hearing loss ears had bony cochlear nerve canal stenosis/atresia and this group showed associated inner ear anomalies. When the diameter of the bony cochlear nerve canal was less than 1.4mm, pure tone audio averages were more than 70 dB HL in most ears.


Assuntos
Cóclea/anormalidades , Nervo Coclear/anormalidades , Orelha Interna/anormalidades , Perda Auditiva Neurossensorial/diagnóstico , Osso Temporal/anormalidades , Adolescente , Criança , Pré-Escolar , Cóclea/diagnóstico por imagem , Nervo Coclear/diagnóstico por imagem , Orelha Interna/diagnóstico por imagem , Feminino , Perda Auditiva Neurossensorial/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Masculino , Radiografia , Estudos Retrospectivos , Osso Temporal/diagnóstico por imagem , Adulto Jovem
15.
Laryngoscope ; 122(5): 1109-14, 2012 May.
Artigo em Inglês | MEDLINE | ID: mdl-22374919

RESUMO

OBJECTIVES/HYPOTHESIS: To assess the normative size of the cochlear nerve (CN) and facial nerve (FN) in normal-hearing ears and to determine whether nerve size varies with age. STUDY DESIGN: Cross-sectional. METHODS: We included 169 ears with normal hearing between 2005 and 2010. The height, width, and cross-sectional area (CSA) of the CN and FN in the middle of the internal auditory canal were measured on oblique sagittal images of 3.0-T magnetic resonance imaging. Results were compared by age. Young subjects were divided into three age groups, 0-5, 6-10, and 11-15 years. Subjects over age 40 years were divided into groups at 10-year intervals. RESULTS: Mean age was 32.6 years (range, 0.75-79 years). We found that the CN had significantly greater vertical (1.10 ± 0.21 mm vs. 0.95 ± 0.21 mm) and horizontal (1.11 ± 0.20 mm vs. 1.03 ± 0.22 mm) diameters than the FN. The CSA of the CN was larger than that of the FN (0.98 ± 0.33 mm(2) vs. 0.79 ± 0.31 mm(2) ). Except for the CN vertical diameter, there were no significant differences between right and left ears. Sex did not affect the nerve size. Although the CN was not affected by age, the FN vertical diameter and CSA of children <5 years were significantly smaller than those of children aged >5 years. The size of the two nerves did not differ among groups over age 40 years. CONCLUSIONS: The CN is not affected by age in normal-hearing ears. The FN vertical diameter and CSA of children <5 years are smaller than those of older children.


Assuntos
Envelhecimento/fisiologia , Nervo Coclear/anatomia & histologia , Nervo Facial/anatomia & histologia , Audição/fisiologia , Imageamento por Ressonância Magnética/métodos , Nomogramas , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Nervo Coclear/fisiologia , Estudos Transversais , Nervo Facial/fisiologia , Feminino , Seguimentos , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Valores de Referência , República da Coreia , Estudos Retrospectivos , Adulto Jovem
16.
Acta Otolaryngol ; 131(9): 908-13, 2011 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-21534716

RESUMO

CONCLUSION: We suggest that cochlear implantation (CI) should be a good therapeutic modality for hearing restoration in patients with common cavity malformed ears. OBJECTIVE: To analyze hearing improvement from CI performed in common cavity malformed cochleae. METHODS: A total of 11 patients (5 male and 6 female, mean age 4.5 ± 2.8 years) and 12 ears were enrolled in this study. During the insertion of electrodes, we used C-arm fluoroscopy to avoid intrameatal placement. We evaluated hearing improvement every 6 months and the mean follow-up period was 80.5 ± 24.1 months (53-125 months). RESULTS: During the operation, there were only four cases with fully inserted electrodes. Cerebrospinal fluid gushed out in two cases during the cochleostomy and postoperative meningitis occurred in two patients. One patient had to undergo reimplantation 4 years later due to device failure and recurrent meningitis. During the 48 months follow-up hearing evaluation, the ability of hearing increased along with the age. The final average MAIS, CAP, SIR, and open set one- and two-syllable word scores were 90.3 ± 18.1%, 4.9 ± 1.6, 3.1 ± 0.9, 24.1 ± 25.9%, and 48.6 ± 38.7%, respectively.


Assuntos
Limiar Auditivo , Cóclea/anormalidades , Implante Coclear , Surdez/reabilitação , Testes de Discriminação da Fala , Teste do Limiar de Recepção da Fala , Otorreia de Líquido Cefalorraquidiano/etiologia , Criança , Pré-Escolar , Eletrodos Implantados , Feminino , Humanos , Lactente , Complicações Intraoperatórias/etiologia , Masculino , Meningites Bacterianas/etiologia , Complicações Pós-Operatórias/etiologia , Falha de Prótese , Reoperação , Estudos Retrospectivos
17.
J Korean Med Sci ; 25(9): 1343-51, 2010 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-20808679

RESUMO

The acculturation effect of immigrant women on birth outcomes varies by race. We examined birth outcomes of three groups of births for the period 1995-2004, USA births to the USA-born Korean mothers, USA births to the non-USA-born Korean mothers, and births in Korea. In singleton USA births to both Korean parents, average birth weight was 3,294 g for the USA-born Korean mothers and 3,323 g for the non-USA-born Korean mothers. However, this difference was not significant, once controlled for other maternal sociodemographic, obstetric and medical factors. Low birth weight and prematurity prevalence were not different by maternal nativity between these two singleton groups. Average birth weight of all births including multiplets in Korea was 3,270 g, compared to 3,297 g for all USA-born infants including multiplets and births either to both or one Korean parents. This difference might have reflected a significantly lower educational attainment of mothers in Korea compared to Korean mothers in the USA. Low birth weight rate was consistently lower in infants born in Korea compared to the USA-born, but this difference became less, 4.2% and 4.6% respectively by 2004. These observations suggest that in the USA acculturation effect of Korean immigrants on birth outcomes is negligible.


Assuntos
Peso ao Nascer , Aculturação , Adolescente , Adulto , Asiático , Feminino , Humanos , Recém-Nascido de Baixo Peso , Recém-Nascido , Recém-Nascido Prematuro , Mães , República da Coreia , Estados Unidos , Adulto Jovem
18.
Otolaryngol Head Neck Surg ; 143(1): 101-8, 2010 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-20620627

RESUMO

OBJECTIVE: To investigate postoperative outcomes after cochlear implantation in young children with cochlear nerve deficiency and compare the results with those of cochlear implantation in patients with normal cochlear nerve. STUDY DESIGN: Historical cohort study. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Six children under the age of 36 months with cochlear nerve deficiency, as determined by magnetic resonance imaging (MRI), received cochlear implantation between July 2006 and March 2008. Fifteen children under the age of 36 months with normal cochlear nerve in the implanted ear underwent cochlear implantation during the same period and were selected as the control group. Medical records, imaging studies, and speech evaluations were reviewed. RESULTS: Our study group scored lower in the Infant-Toddler Meaningful Auditory Integration Scale compared to the control group. Our study group produced categories of auditory performance scores not less than 4 except for one patient. Speech intelligibility rating scores in the control group gradually increased after cochlear implantation, but for the study group, the score remained zero 12 months after the implantation. In open-set one-syllable tests, five patients with cochlear nerve deficiency showed no response at the latest follow-up, whereas patient 6 began to show delayed improvement after 18 months. The results of the open-set two-syllable test were similar to those of the open-set one-syllable test. CONCLUSION: Young children with nonvisualized cochlear nerve on MRI showed worse outcomes compared to the children with normal cochlear nerve. Cochlear nerve deficiency on MRI is a marker of very poor outcome with cochlear implantations.


Assuntos
Implante Coclear , Implantes Cocleares , Perda Auditiva/patologia , Perda Auditiva/terapia , Imageamento por Ressonância Magnética , Doenças do Nervo Vestibulococlear/diagnóstico , Percepção Auditiva , Pré-Escolar , Estudos de Coortes , Feminino , Perda Auditiva/etiologia , Humanos , Lactente , Desenvolvimento da Linguagem , Masculino , Valor Preditivo dos Testes , Estudos Retrospectivos , Resultado do Tratamento , Doenças do Nervo Vestibulococlear/complicações , Doenças do Nervo Vestibulococlear/psicologia
19.
Otolaryngol Head Neck Surg ; 141(2): 237-42, 2009 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-19643258

RESUMO

OBJECTIVE: To evaluate changes in vertigo and hearing from patients with Ménière's disease managed by endolymphatic mastoid shunt (EMS). STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: Data from 16 patients were analyzed using 1995 AAO-HNS criteria. RESULTS: Among 16 patients, six had class A vertigo control two years after treatment, five had class B, one class C, three class D, and one class F. At four years after surgery, seven patients had class A, four class B, three class C, and two class F. The mean functional level before surgery was 4.8 and improved to 2.9 and 2.6 at two years and four years after surgery, respectively. The bone conduction pure-tone averages (four frequencies) were 43.3 before surgery and improved to 33.5 dB HL and 35.5 dB HL at three and six months after surgery, respectively. At two years of follow-up, the hearing level was 38.6 dB HL and was not different from the preoperative hearing level. Two years after surgery, the hearing level gradually decreased and was 42.0 dB HL at five years of follow-up. CONCLUSION: EMS appears to be beneficial in the short term for the symptomatic patients.


Assuntos
Audiometria de Tons Puros/métodos , Anastomose Endolinfática/métodos , Audição , Processo Mastoide/cirurgia , Doença de Meniere/cirurgia , Adolescente , Adulto , Idoso , Feminino , Seguimentos , Hospitais Universitários , Humanos , Masculino , Processo Mastoide/fisiopatologia , Doença de Meniere/fisiopatologia , Pessoa de Meia-Idade , Qualidade de Vida , Estudos Retrospectivos , Percepção da Fala , Fatores de Tempo , Resultado do Tratamento , Vertigem/cirurgia
20.
Acta Otolaryngol ; 129(6): 588-91, 2009 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-18720074

RESUMO

CONCLUSION: In this study, we concluded that electrode design and location did not have a high level of influence on the prevalence of facial nerve stimulation (FNS) in normal cochleae. OBJECTIVE: To analysis the prevalence of FNS after cochlear implantation with Nucleus 24-channel devices according to types of electrodes arrays. PATIENTS AND METHODS: We retrospectively analyzed medical and mapping records of 394 patients who received cochlear implants (CIs) manufactured by Cochlear Corporation from April 1999 to March 2007. RESULTS: In all, 23 of 394 (5.8%) patients had FNS (CI24M 4 of 39 [10.3%], CI24RCS 9 of 192 [4.7%], CI24RST 9 of 21 [42.9%], and CI24RECA 1 of 87 [1.1%]). In addition, 4 of 324 (1.2%) patients with normal cochleae complained of FNS (CI24M 1 of 33 [3.0%], CI24RCS 2 of 173 [1.2%], and CI24RECA 1 of 71 [1.4%]). There was no difference between straight and perimodiolar electrode arrays in patients with normal cochleae. In addition, when comparing two types of Contour electrodes, Contour Advance (soft-tip) electrodes offered significantly lower incidence of FNS than Contour electrode arrays. We could manage these patients with methods such as decrease of C-level, selective channel turning off, and changes of mapping strategies.


Assuntos
Implantes Cocleares/efeitos adversos , Eletrodos/efeitos adversos , Eletrodos/classificação , Análise de Falha de Equipamento , Doenças do Nervo Facial/epidemiologia , Doenças do Nervo Facial/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Implante Coclear/efeitos adversos , Desenho de Equipamento , Nervo Facial/fisiopatologia , Doenças do Nervo Facial/fisiopatologia , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Prevalência , Estudos Retrospectivos , Adulto Jovem
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