What does ada scid stand for3/18/2024 ![]() ERT initiated at 7 days pp significantly improved the hearing of ADA–/– mice as well as the number of thymocytes and T lymphocytes, although not all normalized.Ĭonclusions: ADA deficiency is associated with hearing deficits and damage to cochlear hair cells. Progressive immune abnormalities were detected in ADA–/– mice from 4 days pp, initially affecting the thymus followed by peripheral lymphocytes and T cells in the spleen. ![]() The hearing defects were not reversed when hypoxia in ADA–/– mice was corrected. SEM demonstrated extensive damage to the cochlear hair cells of ADA–/– mice, which were splayed, short or missing, correlating with the hearing deficits. Results: Markedly abnormal hearing thresholds responses were found in ADA–/– mice at low and medium tone frequencies. The effects of polyethylene glycol conjugated ADA (PEG-ADA) ERT or 40% oxygen initiated at 7 days pp on the hearing and immune abnormalities were assessed. The cochlea was visualized by scanning electron microscopy (SEM). Methods: Auditory brainstem evoked responses, organ weights, thymocytes numbers, and subpopulations, lymphocytes in peripheral blood as well as T lymphocytes in spleen were analyzed in ADA–/– and ADA-proficient littermate post-partum (pp). We hypothesized that ADA-deficient (–/–) mice also exhibit hearing abnormalities and that ERT from an early age will improve the hearing and immune defects in these mice. Additionally, ADA-deficient patients suffer from hearing impairment.
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