RESUMEN
Hearing loss and its functional consequences were evaluated retrospectively in children with congenital hypothyroidism. From a cohort of 101 children followed longitudinally to evaluate newborn screening, 75 with previous hearing tests were studied. Fifteen (20%) were found to have hearing problems. Of these, nine had unilateral or sensorineural loss mostly at high frequencies, five had a conductive loss, and one had both problems. Hearing impaired children differed from children with normal hearing in age of treatment onset (22 vs 14 days) but not disease severity or duration. A comparison of language and auditory processing skills at ages 3, 5, and 7 years revealed that early speech was delayed in hearing impaired children, whereas deficits persisted in later receptive language and auditory discrimination skills. Comparing hearing impaired children and children with normal hearing with matched control subjects at grade 3 showed that hearing impaired children were poorer readers because of less adequate phonologic processing skills.
Asunto(s)
Hipotiroidismo Congénito , Trastornos de la Audición/congénito , Niño , Preescolar , Estudios de Cohortes , Femenino , Estudios de Seguimiento , Trastornos de la Audición/diagnóstico , Trastornos de la Audición/prevención & control , Pérdida Auditiva Conductiva/congénito , Pérdida Auditiva Conductiva/diagnóstico , Pérdida Auditiva Conductiva/prevención & control , Pérdida Auditiva de Alta Frecuencia/congénito , Pérdida Auditiva de Alta Frecuencia/diagnóstico , Pérdida Auditiva de Alta Frecuencia/prevención & control , Pérdida Auditiva Sensorineural/congénito , Pérdida Auditiva Sensorineural/diagnóstico , Pérdida Auditiva Sensorineural/prevención & control , Humanos , Hipotiroidismo/complicaciones , Hipotiroidismo/prevención & control , Lactante , Recién Nacido , Trastornos del Desarrollo del Lenguaje/diagnóstico , Trastornos del Desarrollo del Lenguaje/etiología , Trastornos del Desarrollo del Lenguaje/prevención & control , Estudios Longitudinales , Masculino , Tamizaje Neonatal , Ontario/epidemiología , Lectura , Estudios RetrospectivosRESUMEN
Behavioral investigations of Belgian Waterslager canaries (BWCs) have demonstrated a congenital hearing impairment that primarily affects high frequencies. Research into the surface anatomy of the basilar papilla of these birds has pointed to the hair cells as the site of the lesion. Given that the basilar papilla and the vestibular organs both develop from the otocyst, we were interested in ascertaining whether the vestibular sensory epithelia also displayed abnormal hair cells. The inner ear of adult BWCs was examined by scanning electron microscopy. As expected, hair cells in the basilar papilla of BWCs were abnormal. As for the vestibular parenchyma, abnormal hair cells were detected in only one structure: the sacculus. Morphological abnormalities of the cochlea and sacculus are pathognomonic signs of Scheibe's dysplasia, the most common inner ear defect associated with congenital hearing loss. Our results suggest that BWCs are afflicted by this genetic disorder.
Asunto(s)
Membrana Basilar/patología , Enfermedades de las Aves/congénito , Canarios , Células Ciliadas Auditivas/patología , Pérdida Auditiva de Alta Frecuencia/veterinaria , Análisis de Varianza , Animales , Membrana Basilar/citología , Membrana Basilar/ultraestructura , Conducta Animal/fisiología , Enfermedades de las Aves/patología , Cruzamiento , Cóclea/patología , Cóclea/ultraestructura , Células Epiteliales , Células Ciliadas Auditivas/citología , Células Ciliadas Auditivas/ultraestructura , Pérdida Auditiva de Alta Frecuencia/congénito , Pérdida Auditiva de Alta Frecuencia/patología , Microscopía Electrónica de Rastreo , Sáculo y Utrículo/citología , Sáculo y Utrículo/patologíaRESUMEN
Successful cadaveric renal transplantation was accomplished in a patient with Epstein syndrome, a triad of macrothrombocytopenia, partial high-frequency hearing loss, and nephritis, which often progresses to complete renal failure. The success of the transplant demonstrates that the macrothrombocytopenia which occurs in this syndrome is not a contraindication to aggressive management of end-stage renal disease.