11/9/2023 0 Comments Brain stem model![]() Clinically, auditory phenotypes present as reduced auditory attention, impaired habituation to auditory stimuli, reduced prepulse inhibition of acoustic startle, and overall hypersensitivity to auditory conditions (reviewed in Sinclair et al., 2017 Rais et al., 2018 Razak et al., 2021) that have likely both cortical and subcortical origins. One of the most common symptoms described in people with FXS and autism spectrum disorder (ASD) is auditory hypersensitivity ( Ethridge et al., 2017 Stefanelli et al., 2020). As a result, drug therapies have struggled to rescue the human disorder ( Dahlhaus, 2018). Despite the common use of these models to study the FXS, phenotypes are not always shared between species and background strains, particularly for sensory processing. FXS is a tractable genetic model for ASD with several commercially available models, including the rat and mouse ( The Dutch-Belgian Fragile X Consorthium et al., 1994 Till et al., 2015 Tian et al., 2017). These findings further clarify auditory brain stem processing in FXS by adding more information across genetic background strains allowing for a better understanding of shared phenotypes.įragile X syndrome (FXS) is the most common monogenic form of autism spectrum disorder (ASD) and shares many attributes of ASDs, including auditory hypersensitivity and other sensory disruptions ( Abbeduto and Hagerman, 1997 Chen and Toth, 2001 Hagerman and Hagerman, 2002 Arnett et al., 2014). Finally, males with FXS had an increased latency of the binaural interaction component (BIC) at 0 interaural timing difference compared with that in wild-type males. In contrast, female mice with homozygous FXS had a decreased amplitude of wave IV of the monaural ABR, while there was no difference in males for amplitudes and no change in latency of ABR waveforms across sexes and genotypes. There was no significant difference in hearing range between the sexes or genotypes, however there was a trend towards high frequency hearing loss in male FXS mice. Consistent with previous study, we showed no difference in morphological parameters across genotypes or sexes. We measured morphological features of pinna and head and used ABR to measure the hearing range, and monaural and binaural auditory responses in hemizygous males, homozygous females, and heterozygous females compared with those in wild-type mice. This study is the first to characterize the auditory brain stem response (ABR), a minimally invasive physiological readout of early auditory processing that is also used in humans, in a commonly used mouse background strain model of FXS, C57BL/6J. However, linking phenotypes across genetic background strains of mouse models has been a challenge and could underly some of the issues with translatability of drug studies to the human condition. ![]() ![]() ![]() Sensory hypersensitivity, especially in the auditory system, is a common symptom in Fragile X syndrome (FXS), the most common monogenic form of intellectual disability. Department of Integrative Biology, Oklahoma State University, Stillwater, OK, United States. ![]()
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