- [Preoperative diagnosis and evaluation of cochlear implantation in patients with cochlear nerve deficiency]. [Review]
- LCLin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2018; 32(10):794-798
- Cochlear nerve deficiency (CND) refers to a small or absent cochlear branch of the vestibulocochlear nerve (VCN) or cochlear nerve (CN). Congenitally deaf children with CND who received cochlear impl...
Cochlear nerve deficiency (CND) refers to a small or absent cochlear branch of the vestibulocochlear nerve (VCN) or cochlear nerve (CN). Congenitally deaf children with CND who received cochlear implants (CIs) generally exhibit poorer auditory performance than CI children without CND. It is important to confirm the integrity of the auditory pathway before surgery. High-resolution computed tomography (HRCT) and three-dimensional magnetic resonance imaging (3D MRI) are major techniques used to diagnosis CND. In this paper, we reviewed the methods of preoperative evaluation, cochlear nerve embryonic development, techniques for diagnosis and outcomes of cochlear implantation.
- Towards an objective test of chronic tinnitus: Properties of auditory cortical potentials evoked by silent gaps in tinnitus-like sounds. [Journal Article]
- HRHear Res 2018 Apr 17
- A common method designed to identify if an animal hears tinnitus assumes that tinnitus "fills-in" silent gaps in background sound. This phenomenon has not been reliably demonstrated in humans. One te...
A common method designed to identify if an animal hears tinnitus assumes that tinnitus "fills-in" silent gaps in background sound. This phenomenon has not been reliably demonstrated in humans. One test of the gap-filling hypothesis would be to determine if gap-evoked cortical potentials are absent or attenuated when measured within background sound matched to the tinnitus sensation. However the tinnitus sensation is usually of low intensity and of high frequency, and it is unknown if cortical responses can be measured with such "weak" stimulus properties. Therefore the aim of the present study was to test the plausibility of observing these responses in the EEG in humans without tinnitus. Twelve non-tinnitus participants heard narrowband noises centered at sound frequencies of 5 or 10 kHz at sensation levels of either 5, 15, or 30 dB. Silent gaps of 20 ms duration were randomly inserted into noise stimuli, and cortical potentials evoked by these gaps were measured by 64-channel EEG. Gap-evoked cortical responses were statistically identifiable in all conditions for all but one participant. Responses were not significantly different between noise frequencies or levels. Results suggest that cortical responses can be measured when evoked by gaps in sounds that mirror acoustic properties of tinnitus. This design can validate the animal model and be used as a tinnitus diagnosis test in humans.
- Hearing impairment in MELAS: new prospective in clinical use of microRNA, a systematic review. [Review]
- OJOrphanet J Rare Dis 2018 Feb 21; 13(1):35
- CONCLUSIONS: We suggest that due to their accuracy and sensitivity, miRs might help monitor the progression of SNHL in patients with MELAS.
- Slow Accumulations of Neural Activities in Multiple Cortical Regions Precede Self-Initiation of Movement: An Event-Related fMRI Study. [Journal Article]
- EeNeuro 2017 Sep-Oct; 4(5)
- The neural processes underlying self-initiated behavior (behavior that is initiated without an external stimulus trigger) are not well understood. This event-related fMRI study investigated the neura...
The neural processes underlying self-initiated behavior (behavior that is initiated without an external stimulus trigger) are not well understood. This event-related fMRI study investigated the neural origins of self-initiated behaviors in humans, by identifying brain regions that increased in neural activities several seconds prior to self-initiated movements. Subjects performed a hand grasping task under two conditions: a free-timing and cued timing condition. The supplementary motor area (SMA) began to activate several seconds prior to self-initiated movement (accounting for hemodynamic delay), representing a potential blood oxygenation level-dependent (BOLD) signal correlate of the readiness potential (RP) on electroencephalogram (EEG), referred to here as "readiness BOLD signals." Significant readiness BOLD signals were also observed in the right frontoparietal areas, precuneus, and insula, all of which are known to contribute to internally-generated behaviors, but with no prior evidence for such early and slow accumulation of neural activities. Moreover, visual and auditory cortices also exhibited clear readiness BOLD signals with similar early onsets, even absent external stimulation. Slow accumulation of neural activities throughout distributed cortical areas, including sensory, association, and motor cortices, underlies the generation of self-initiated behaviors. These findings warrant reconsideration of the prevailing view that the SMA or some other specific locus in frontoparietal cortex serves as the ultimate neural origin of self-initiated movement.
- Hallucinations in borderline personality disorder: Prevalence, characteristics and associations with comorbid symptoms and disorders. [Journal Article]
- SRSci Rep 2017 Oct 24; 7(1):13920
- To establish the point prevalence of hallucinations in borderline personality disorder (BPD), telephone interviews were conducted with 324 outpatients diagnosed with BPD. Then a subgroup (n = 98) was...
To establish the point prevalence of hallucinations in borderline personality disorder (BPD), telephone interviews were conducted with 324 outpatients diagnosed with BPD. Then a subgroup (n = 98) was interviewed in person to investigate the co-occurrence of these phenomena with other psychotic symptoms, comorbid psychiatric disorders, prior childhood adversities, and adult life stressors. For hallucinations in general a point prevalence of 43% was found, with rates for hallucinations in separate sensory modalities ranging from 8-21%. Auditory verbal hallucinations consisted mostly of verbal abuse and were generally experienced as distressing. A significant association was found between the severity of hallucinations on the one hand, and delusions and unusual thought content on the other; this association was absent for negative symptoms and disorganization. The presence of hallucinations also correlated with the number of comorbid psychiatric disorders, and with posttraumatic stress disorder (PTSD) specifically. Childhood emotional abuse and adult life stressors were also associated with hallucinations. The latter three associations suggest that patients with BPD might have an etiological mechanism in common with other patient/nonpatient groups who experience hallucinations. Based on these findings, we advise to treat PTSD and hallucinations when found to be present in patients with BPD.
- Identifying Otosclerosis with Aural Acoustical Tests of Absorbance, Group Delay, Acoustic Reflex Threshold, and Otoacoustic Emissions. [Journal Article]
- JAJ Am Acad Audiol 2017; 28(9):838-860
- CONCLUSIONS: Reflectance provides a middle-ear test that is sensitive to classifying ears as otosclerotic or normal, which may be useful in clinical applications.
- Biallelic mutations in the homeodomain of NKX6-2 underlie a severe hypomyelinating leukodystrophy. [Journal Article]
- BBrain 2017 Oct 01; 140(10):2550-2556
- Hypomyelinating leukodystrophies are genetically heterogeneous disorders with overlapping clinical and neuroimaging features reflecting variable abnormalities in myelin formation. We report on the id...
Hypomyelinating leukodystrophies are genetically heterogeneous disorders with overlapping clinical and neuroimaging features reflecting variable abnormalities in myelin formation. We report on the identification of biallelic inactivating mutations in NKX6-2, a gene encoding a transcription factor regulating multiple developmental processes with a main role in oligodendrocyte differentiation and regulation of myelin-specific gene expression, as the cause underlying a previously unrecognized severe variant of hypomyelinating leukodystrophy. Five affected subjects (three unrelated families) were documented to share biallelic inactivating mutations affecting the NKX6-2 homeobox domain. A trio-based whole exome sequencing analysis in the first family detected a homozygous frameshift change [c.606delinsTA; p.(Lys202Asnfs*?)]. In the second family, homozygosity mapping coupled to whole exome sequencing identified a homozygous nucleotide substitution (c.565G>T) introducing a premature stop codon (p.Glu189*). In the third family, whole exome sequencing established compound heterozygosity for a non-conservative missense change affecting a key residue participating in DNA binding (c.599G>A; p.Arg200Gln) and a nonsense substitution (c.589C>T; p.Gln197*), in both affected siblings. The clinical presentation was homogeneous, with four subjects having severe motor delays, nystagmus and absent head control, and one individual showing gross motor delay at the age of 6 months. All exhibited neuroimaging that was consistent with hypomyelination. These findings define a novel, severe form of leukodystrophy caused by impaired NKX6-2 function.
- The potential role of auditory evoked potentials to assess prognosis in comatose survivors from cardiac arrest. [Journal Article]
- RResuscitation 2017; 120:119-124
- CONCLUSIONS: In this series of patients after CA, at least one of the BAEP waves was absent bilaterally in half the survivors; however, their use for prediction of poor neurological outcome remains limited.
- A pediatric case with peripheral facial nerve palsy caused by a granulomatous lesion associated with cat scratch disease. [Journal Article]
- BDBrain Dev 2018; 40(2):159-162
- CONCLUSIONS: The suspected granulomatous lesion was considered to have resulted from the host's immune reaction to Bartonella infection and impaired the facial nerve. This is the first case report providing direct evidence of peripheral facial nerve palsy caused by a suspected granulomatous lesion associated with cat scratch disease and its treatment course.
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- Longitudinal Changes in Electrically Evoked Auditory Event-Related Potentials in Children With Auditory Brainstem Implants: Preliminary Results Recorded Over 3 Years. [Journal Article]
- EHEar Hear 2018 Mar/Apr; 39(2):318-325
- CONCLUSIONS: eERP responses in children with ABIs could change over a long period of time. Maturation of the central auditory system could not fully account for these observed changes. Children with ABIs need to be closely monitored for potential changes in auditory perception and unfavorable nonauditory sensations. Neuroimaging correlates are needed to better understand the emergence of nonauditory stimulation over time in these children.