Longer brainstem auditory evoked response latencies of individuals with fragile X syndrome related to sedation

Miezejeski, Charles; Heaney, G.; Belser, Richard C.; Brown, W. Ted; Jenkins, Edmund C.; Sersen, Eugene A.

doi:10.1002/(sici)1096-8628(19970418)74:2<167::aid-ajmg10>3.0.co;2-g

Cited by 8 publications

(13 citation statements)

References 26 publications

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“…It is thus possible that the detected ABR alterations may have resulted indirectly from impaired middle ear function. Indeed, more recent studies from FXS individuals without middle ear infection did not find any differences in the absolute peak latencies and IPLs of ABRs . However, one should be careful in attempting to interpret this result as implying that ABRs and FMRP expression are independent.…”

Section: Fmrp Regulates the Development Of Synaptic Transmission In Tmentioning

confidence: 89%

“…Because more than half of FXS children have otitis media, individuals without this peripheral pathology present only a specific population of FXS who are probably less sensitive to FMRP loss in both the periphery and the central nervous system. Other work compared children with FXS, with or without receiving sedation, during ABR testing and argued that differences seen in ABR testing across studies may be caused by the administration of sedatives . However, this study may have over‐sampled from high‐functional patients who may have been more tolerant of the ABR test.…”

Section: Fmrp Regulates the Development Of Synaptic Transmission In Tmentioning

confidence: 99%

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Mechanisms underlying auditory processing deficits in Fragile X syndrome

et al. 2020

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Autism spectrum disorders (ASD) are strongly associated with auditory hypersensitivity or hyperacusis (difficulty tolerating sounds). Fragile X syndrome (FXS), the most common monogenetic cause of ASD, has emerged as a powerful gateway for exploring underlying mechanisms of hyperacusis and auditory dysfunction in ASD. This review discusses examples of disruption of the auditory pathways in FXS at molecular, synaptic, and circuit levels in animal models as well as in FXS individuals. These examples highlight the involvement of multiple mechanisms, from aberrant synaptic development and ion channel deregulation of auditory brainstem circuits, to impaired neuronal plasticity and network hyperexcitability in the auditory cortex. Though a relatively new area of research, recent discoveries have increased interest in auditory dysfunction and mechanisms underlying hyperacusis in this disorder. This rapidly growing body of data has yielded novel research directions addressing critical questions regarding the timing and possible outcomes of human therapies for auditory dysfunction in ASD.

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Section: Fmrp Regulates the Development Of Synaptic Transmission In Tmentioning

confidence: 89%

Section: Fmrp Regulates the Development Of Synaptic Transmission In Tmentioning

confidence: 99%

Mechanisms underlying auditory processing deficits in Fragile X syndrome

et al. 2020

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“…Abnormalities consisting of prolongation of waves and increased interpeak latency intervals have been reported in a number of mental retardation syndromes [7] patients with head trauma [8], and hydrocephalus [9]. However, there have been some documented effects of sedation on the interpeak latency intervals of the ABR in children with mental retardation [10], in other neurologic disorders such as central alveolar hypoventilation [11,12] as well as in patients with respiratory insufficiency following encephalitis [13]. Due to the documented effects of sedation on estimates of interpeak latency intervals in some clinical populations, it has been suggested that abnormal interpeak latency intervals reported in patient populations such as fragile × syndrome may be related to the use of sedation as opposed to inherent features of the disorder [10].…”

Section: Introductionmentioning

confidence: 99%

“…However, there have been some documented effects of sedation on the interpeak latency intervals of the ABR in children with mental retardation [10], in other neurologic disorders such as central alveolar hypoventilation [11,12] as well as in patients with respiratory insufficiency following encephalitis [13]. Due to the documented effects of sedation on estimates of interpeak latency intervals in some clinical populations, it has been suggested that abnormal interpeak latency intervals reported in patient populations such as fragile × syndrome may be related to the use of sedation as opposed to inherent features of the disorder [10]. Miezejeski et al [10] demonstrated that irrespective of diagnosis, patients with mental retardation who were sedated for testing had longer III-V interpeak latency intervals than non-sedated patients with mental retardation.…”

Section: Introductionmentioning

confidence: 99%

“…Due to the documented effects of sedation on estimates of interpeak latency intervals in some clinical populations, it has been suggested that abnormal interpeak latency intervals reported in patient populations such as fragile × syndrome may be related to the use of sedation as opposed to inherent features of the disorder [10]. Miezejeski et al [10] demonstrated that irrespective of diagnosis, patients with mental retardation who were sedated for testing had longer III-V interpeak latency intervals than non-sedated patients with mental retardation.…”

Section: Introductionmentioning

confidence: 99%

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Effects of Sedation on Auditory Brainstem Response in Rett Syndrome

Pillion

Bibat

Naidu

2010

Pediatric Neurology

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Prolongation of the I-V interpeak latency intervals have been reported in Rett Syndrome and other neurodevelopmental disorders. It has been suggested that the use of sedation may account for differences in the interpeak latency intervals when comparisons are made across diagnostic groups if sedated control groups are not used for the basis of comparison. This study examined the effects of sedation on Auditory Brainstem Response interpeak latency intervals (i.e., I-III, III-V and I-V) in two groups of individuals: 1) a group with Rett Syndrome who were positive for mutations in the MeCP2 gene, and 2) a group negative for mutations in the MeCP2 gene but who were severe to profoundly delayed with other causes of mental retardation. A third group of sedated and unsedated female participants taken from our normative ABR data base was also included in order to further assess the effects of sedation. An analysis of variance indicated, 1) longer I-V interpeak latency intervals in the sedated participants with Rett Syndrome; 2) longer III-V interpeak latency intervals in the mutation positive participants as compared to non-Rett Syndrome mutation negative participants and 3) t-tests revealed no significant effects of sedation on the I-III, III-V or I-V interpeak latency intervals among the normative group participants. Our findings suggest a possible biological basis for the discrepancy in the literature on auditory brain stem responses in Rett Syndrome, and warrant cautious interpretation of auditory brain stem responses findings in sedated subjects with Rett Syndrome as well as in those with mental retardation and seizures.

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Intense and specialized dendritic localization of the fragile X mental retardation protein in binaural brainstem neurons: A comparative study in the alligator, chicken, gerbil, and human

Wang

Sakano

Beebe

et al. 2014

J of Comparative Neurology

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Neuronal dendrites are structurally and functionally dynamic in response to changes in afferent activity. The fragile X mental retardation protein (FMRP) is an mRNA binding protein that regulates activity-dependent protein synthesis and morphological dynamics of dendrites. Loss and abnormal expression of FMRP occur in fragile X syndrome (FXS) and some forms of autism spectrum disorders. To provide further understanding of how FMRP signaling regulates dendritic dynamics, we have examined dendritic expression and localization of FMRP in the reptilian and avian nucleus laminaris (NL) and its mammalian analogue, the medial superior olive (MSO), in rodents and humans. NL/MSO neurons are specialized for temporal processing of low frequency sounds for binaural hearing, which is impaired in FXS. Protein BLAST analyses first demonstrate that the FMRP amino acid sequences in the alligator and chicken are highly similar to human FMRP with identical mRNA-binding and phosphorylation sites, suggesting that FMRP functions similarly across vertebrates. Immunocytochemistry further reveals that NL/MSO neurons have very high levels of dendritic FMRP in low frequency hearing vertebrates including alligator, chicken, gerbil, and human. Remarkably, dendritic FMRP in NL/MSO neurons often accumulates at branch points and enlarged distal tips, loci known to be critical for branch-specific dendritic arbor dynamics. These observations support an important role for FMRP in regulating dendritic properties of binaural neurons that are essential for low frequency sound localization and auditory scene segregation, and support the relevance of studying this regulation in nonhuman vertebrates that use low frequencies in order to further understand human auditory processing disorders.

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Longer brainstem auditory evoked response latencies of individuals with fragile X syndrome related to sedation

Cited by 8 publications

References 26 publications

Mechanisms underlying auditory processing deficits in Fragile X syndrome

Mechanisms underlying auditory processing deficits in Fragile X syndrome

Effects of Sedation on Auditory Brainstem Response in Rett Syndrome

Intense and specialized dendritic localization of the fragile X mental retardation protein in binaural brainstem neurons: A comparative study in the alligator, chicken, gerbil, and human

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