RESUMEN
OBJECTIVE: To determine the usefulness of both amplitude and threshold data from tone-burst cervical vestibular evoked myogenic potential (cVEMP) testing for the evaluation of superior canal dehiscence syndrome (SCDS). STUDY DESIGN: Case series with chart review. SUBJECTS AND METHODS: Sixty-seven patients underwent cVEMP testing. We correlated mean tone burst cVEMP amplitude and threshold data with temporal bone CT findings. Patients were excluded for Ménière's disease, middle ear disease, or otologic surgery. RESULTS: Superior canal dehiscence patients had higher mean cVEMP amplitudes (SCDS 173.8 microV vs non-SCDS 69.7 microV, P=0.031) and lower mean thresholds (SCDS 72.8 dB nHL vs non-SCDS 80.9 dB nHL) at 500 Hz. CONCLUSION: Patients with SCDS have larger amplitudes and lower thresholds on cVEMP testing at 500 Hz. This study supports the utility of tone burst cVEMPs for the evaluation of SCDS and is one of few large single-center studies to establish normative data.
Asunto(s)
Potenciales Evocados Auditivos/fisiología , Canales Semicirculares/fisiopatología , Enfermedades Vestibulares/fisiopatología , Pruebas de Función Vestibular/métodos , Estimulación Acústica/métodos , Adulto , Área Bajo la Curva , Conducción Ósea/fisiología , Electromiografía/métodos , Femenino , Humanos , Masculino , Contracción Muscular/fisiología , Músculos del Cuello/fisiopatología , Curva ROC , Reflejo Vestibuloocular/fisiología , Estudios Retrospectivos , Canales Semicirculares/patología , Estadísticas no Paramétricas , Síndrome , Enfermedades Vestibulares/patologíaRESUMEN
PURPOSE: To comment on a recently published article titled "Initial Audiologic Assessment of Infants Referred From Well Baby, Special Care, and Neonatal Intensive Care Unit Nurseries" by R. K. Karzon and J. E. C. Lieu (2006). METHOD: Comments are directed specifically at the sequence of testing described in the article. CONCLUSIONS: This letter challenges the efficiency of beginning infant audiologic evaluations with the auditory brainstem response evoked by high-intensity, unmasked click stimuli. Reasons as well as alternatives favored by the author and other researchers are presented and summarized.
Asunto(s)
Audiología/métodos , Diagnóstico Precoz , Trastornos de la Audición/diagnóstico , Trastornos de la Audición/epidemiología , Tamizaje Neonatal , Potenciales Evocados Auditivos del Tronco Encefálico/fisiología , Humanos , Recién NacidoRESUMEN
This study provides the first description of isolated cerebellar dysplasia associated with Waardenburg syndrome (WS) and includes a review of cochlear implant outcomes in 42 WS patients. A 1-year-old male infant presented with speech delay, iris heterochromia, profound hearing loss, and an asymmetric, underdeveloped right occipital skull on CT imaging. Brain MRI demonstrated a hypoplastic right cerebellum, no hydrocephalus, normal auditory nerves and brainstem. He underwent successful bilateral sequential cochlear implantation. Cochlear implants remain a reasonable habilitative option for WS patients with congenital deafness, including those with cerebellar abnormalities.