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Abstract
Cases of central deafness are rare but they can be most informative about the function and dysfunction of the central auditory nervous system. Previous information on the anatomy, physiology, and terminology related to central deafness is reviewed and a patient with central deafness is profiled. The patient suffered bilateral cerebrovascular accidents (CVAs) compromising Heschl's gyrus and some adjacent neural tissue on both sides of the brain. At 18 months post CVAs, this patient could not understand speech presented solely through the auditory modality. Environmental sounds were perceived, but rarely recognized. Pure-tone testing revealed a severe-to-profound hearing loss bilaterally, but otoacoustic emissions, acoustic reflexes, and the auditory brainstem response were essentially within normal ranges for both ears. Middle late and late auditory potentials were compromised, yielding complex modifications of the waveforms. These findings and the compromised vascular anatomy in this case are detailed in this article.
| Acronyms | ||
| ABR | = | Auditory brainstem response |
| AAC | = | Augmentative and alternative communication |
| CANS | = | Central auditory nervous system |
| CT | = | Computer tomography |
| CVA | = | Cerebrovascular accident |
| DPOAEs | = | Distortion product otoacoustic emissions |
| HL | = | Hearing level |
| LAER | = | Late auditory evoked response |
| MCA | = | Middle cerebral artery |
| MLR | = | Middle latency response |
| MMN | = | Mismatch negativity |
| MRI | = | Magnetic resonance imaging |
| nHL | = | Normal hearing level |
| OAEs | = | Otoacoustic emissions |
| PAM | = | Post auricular muscle artifact |
| SL | = | Sensation level |
| SPL | = | Sound pressure level |
| Acronyms | ||
| ABR | = | Auditory brainstem response |
| AAC | = | Augmentative and alternative communication |
| CANS | = | Central auditory nervous system |
| CT | = | Computer tomography |
| CVA | = | Cerebrovascular accident |
| DPOAEs | = | Distortion product otoacoustic emissions |
| HL | = | Hearing level |
| LAER | = | Late auditory evoked response |
| MCA | = | Middle cerebral artery |
| MLR | = | Middle latency response |
| MMN | = | Mismatch negativity |
| MRI | = | Magnetic resonance imaging |
| nHL | = | Normal hearing level |
| OAEs | = | Otoacoustic emissions |
| PAM | = | Post auricular muscle artifact |
| SL | = | Sensation level |
| SPL | = | Sound pressure level |
Sumario
Los casos de sordera central son raros pero pueden ser muy informativos sobre la función y disfunción del sistema nervioso central auditivo. Se hizo una revisión preliminar de información sobre anatomía, fisiología y terminología relacionada con la sordera central y se delineó el perfil de un paciente con sordera central. El paciente sufrió accidentes cerebro- vasculares bilaterales (CVA) comprometiendo el giro de Heschl y algún tejido neural adyacente en ambos lados del cerebro. Después de 18 meses de los CVA, este paciente no entendía el lenguaje que se presentaba únicamente a través de la modalidad auditiva. Los sonidos ambientales eran percibidos pero raramente reconocidos. La prueba con tonos puros reveló una pérdida auditiva bilateral severa a profunda, pero las emisiones otoacústicas, los reflejos acústicos y las respuestas auditivas del tallo cerebral, se encontraron esencialmente dentro de límites normales en ambos oídos. Los potenciales de latencia media y tardía estaban comprometidos llevando a modificaciones complejas de las ondas. Estos hallazgos y la anatomía vascular comprometida de este caso se detallan en este artículo.