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Aromaticl-amino acid decarboxylase (AADC) deficiency is characterized by an almost complete absence of sympathetic autoregulation, because of very low levels of circulating catecholamines. Here, we report the successful management of four consecutive anesthesia procedures in a young child presenting with AADC deficiency. Our experience suggests that, with appropriate anticipation of the potential autonomic disturbances, anesthesia, at least for minor surgical and diagnostic procedures, can be conducted safely in patients with AADC deficiency. [ABSTRACT FROM AUTHOR]

A significant number of deaf patients that have received cochlear implants now achieve higher word recognition scores then those with conventional auditory prostheses. This situation makes the choice of which type of auditory rehabilitation to propose a complex matter in patients with remaining auditory function. Our paper aims at providing some arguments to these new questions by presenting the clinical experience and practice of the Centre romand d'implants cochléaires. We also address related legal issues. Clinical tools, such as testing the comprehension of lists of logatoms have proved very useful for the evaluation of these particular patients. The evaluation of cochlear implant candidates remains a highly individualized process, necessitating a case by case approach by an experienced multidisciplinary cochlear implant team.

Objective: To test the feasibility of using the deaf white cat model of early-onset deafness. We studied the neuronal effects of prosthetic intervention with a clinical, "off-the-shelf" multichannel cochlear implant., Methods: We placed cochlear implants in 5 deaf white kittens at age 12 and 24 weeks. The devices were activated and stimulated in the laboratory using a clinical speech processor programmed with a high-resolution continuous interleaved sampling (CIS) strategy for 8 to 24 weeks. Stimulus parameters were guided by electrically evoked brainstem responses and intracochlear-evoked potentials. Kittens were assessed with respect to their tolerance and general behavior in response to speech, music, and environmental sounds., Results: Surgical complications were minimal, and kittens tolerated the experimental procedures well. Subjects were able to detect and respond to a specific sound played from a computer speaker. Electrophysiologic responses were reliably attainable and showed consistency with observed behavioral responses to sound. This experimental paradigm, using clinical devices, can be used in a practical research setting in cats., Conclusions: Deafness and other variations in neural activity result in many distinct changes to the central auditory pathways. Animal models will facilitate assessment of the reversibility of deafness-associated changes at the level of the neuron and its connections. Our observations of the feasibility of using clinical devices in animal models will enable us to simulate clinical conditions in addressing questions about the effects of "replacement" activity on the structure and function within the central auditory pathways in deafness.