RESUMEN
OBJECTIVE: Selection of diagnostic tests for children with sensorineural hearing loss (SNHL) is influenced by clinical suspicion. Testing results reported in the literature are similarly biased. We evaluate the usefulness of a comprehensive diagnostic battery for each child. STUDY DESIGN: Retrospective review. SETTING: Tertiary care university hospital. PATIENTS: A total of 270 children referred for severe to profound SNHL between January 2002 and June 2009. INTERVENTIONS: Results of the following were reviewed: magnetic resonance imaging, computed tomography, renal ultrasound, electrocardiography, fluorescent treponemal antibody absorption test, connexin 26 sequencing, genetic consultation, and ophthalmologic consultation. MAIN OUTCOME MEASURE: Diagnostic yield of each test was determined. RESULTS: Each diagnostic test or consultation was completed by at least 95% of patients for whom it was ordered. Magnetic resonance imaging revealed abnormalities explaining SNHL in 24% of patients. Computed tomography showed inner ear anomalies in 18% of patients. Biallelic connexin 26 mutations were found in 15%. Renal ultrasound found anomalies in 4% of patients. Electrocardiography found 1% of patients with prolonged QT intervals. Fluorescent treponemal antibody absorption test result was positive in 0.5%. Genetic consultation found a genetic cause for hearing loss in 25%. Ophthalmologic consultation found abnormalities associated with hearing loss in 8%. CONCLUSION: Diagnostic radiologic imaging is the highest yielding test for evaluating children with SNHL. Connexin 26 sequencing identifies a nearly nonoverlapping subset of children compared with imaging. Specialty consultations, particularly from a clinical geneticist, can improve diagnostic yield. Other tests, although of lower diagnostic yield for SNHL, can identify important diseases that significantly affect patient health.
Asunto(s)
Pérdida Auditiva Sensorineural/diagnóstico , Pruebas Auditivas , Encéfalo/patología , Niño , Conexina 26 , Conexinas/genética , Conducto Auditivo Externo , Electrocardiografía , Femenino , Prueba de Absorción de Anticuerpos Fluorescentes de Treponema , Pérdida Auditiva Sensorineural/genética , Pérdida Auditiva Sensorineural/patología , Humanos , Riñón/diagnóstico por imagen , Imagen por Resonancia Magnética , Masculino , Estudios Retrospectivos , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Hueso Temporal/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Ultrasonido , Ultrasonografía , Trastornos de la Visión/complicaciones , Pruebas de VisiónRESUMEN
OBJECTIVES/HYPOTHESIS: To examine the characteristics of pediatric cochlear implant channel malfunction preceding device failure. STUDY DESIGN: : Retrospective review. METHODS: All pediatric patients who underwent cochlear implantation at a tertiary academic medical center were reviewed regarding device type, reason for replacement, time to replacement, and timing and pattern of channel faults in failed versus nonfailed devices. RESULTS: Between 1993 and 2008, 264 pediatric cochlear implantations were performed. With an average 894-day follow-up, the replacement rate was 9.5% (25/264). Reasons for replacement were device failure (6.4%), medical/surgical failure (2.3%), and obsolescence (0.8%). Replacement rates were comparable among Advanced Bionics (13.3%), Cochlear Corporation (6.3%), and MED-EL (10.3%) devices. Fifty-two cochlear implants developed at least one channel fault, and 13 eventually progressed to failure requiring replacement. MED-EL devices comprised 12 of these 13 failures. At the 12-month follow-up interval, one, three, and five channel faults predicted 40%, 75%, and 100% probabilities of eventual electrode failure, respectively. Channels destined to fail demonstrated small, yet statistically significant, impedance elevations 12 months before failure and large elevations 3 months before failure. CONCLUSIONS: Replacement of cochlear implants in pediatric patients is common and is due to device malfunction about one half of the time. Earlier initial channel fault, earlier subsequent channel faults, adjacent channel faults, and a greater total number of channel faults were associated with the need for replacement surgery. Elevations in a channel's impedance should raise the concern for an impending failure. These predictors can help the cochlear implant team when considering surgery to replace the device.
Asunto(s)
Implantación Coclear , Implantes Cocleares , Falla de Equipo , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Reoperación , Adulto JovenRESUMEN
This case study describes the neurocognitive presentation of a child with identified genetic abnormalities of trisomy 6 and monosomy 21 who was evaluated as part of a standard medical protocol for cochlear implantation following diagnosis of profound sensorineural hearing loss. This child received neurocognitive testing prior to cochlear implantation and approximately 12 months post-activation of his cochlear implant. While he has not fully developed oral language, his presentation suggested improvement in overall skills since the activation of the cochlear implant; however, less than would be expected for a typically developing child.
Asunto(s)
Cromosomas Humanos Par 21 , Cromosomas Humanos Par 6 , Trastornos del Conocimiento/fisiopatología , Monosomía/fisiopatología , Trisomía/fisiopatología , Desarrollo Infantil/fisiología , Preescolar , Implantes Cocleares , Cognición/fisiología , Humanos , Masculino , Pruebas Neuropsicológicas , Índice de Severidad de la EnfermedadRESUMEN
OBJECTIVES: A considerable amount of literature has documented the impact of hearing impairment on spoken language skills in deaf children referred for cochlear implantation. Critical areas of neurocognitive development in the acquisition of visual (manual) language also appear to be impacted, although the evidence is less robust. The present study focused on the development of visual and fine motor skills in a sample of preschool-age children diagnosed with sensorineural hearing loss with no known neurologic conditions (n=36). STUDY DESIGN: Analysis of data collected as part of a standardized screening process for cochlear implantation at an academic medical center. METHOD: Children underwent a standardized neuropsychological assessment battery. Children were classified into three groups based on the etiology of their deafness (Connexin=15, Structural Malformation=11, and Unknown=10). RESULTS/CONCLUSIONS: Correlational analyses replicated previous research on the reduction in visual reception and fine motor skills as deaf children age. Children with genetic (Connexin) etiology exhibited a significant reduction in fine motor skills with age, whereas those with an etiology of Structural Abnormality exhibited a significant reduction in visual reception skills with age. Results of planned comparisons conducted as part of a multivariate analysis of variance (Skill x Group) indicated that the Connexin group was significantly better than the Unknown group with regard to fine motor skills. Implications for these findings and future studies are discussed.