scholarly journals Congenital Unilateral Hypoplasia of Depressor Anguli Oris

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Seckin O. Ulualp ◽  
Ronald Deskin

Objectives. Asymmetric facial appearance may originate from abnormalities of facial musculature or facial innervation. We describe clinical features of congenital hypoplasia of depressor anguli oris muscle in a child.Material and Methods. Chart of a 10-month-old female referred to a tertiary care pediatric hospital for assessment of facial paralysis was reviewed. Data included relevant history and physical examination, diagnostic work up, and management.Results. The child presented with asymmetric movement of lower lip since birth. Asymmetry of lower lip was more pronounced when she smiled and cried. Rest of the face movement was symmetric. On examination, the face appeared symmetric at rest. The child had inward deviation of right lower lip when she smiled. Facial nerve function, as determined by frowning/forehead, wrinkling, eye closure, nasolabial fold depth, and tearing, was symmetric. Magnetic resonance imaging of the temporal bones and internal auditory canals were within normal limits. Echocardiogram did not show cardiac abnormality. Auditory brainstem response showed no abnormality.Conclusions. Congenital hypoplasia of depressor anguli oris is a rare anomaly that causes asymmetric crying face. Pediatricians and otolaryngologists need to be cognizant of cardiac, head and neck, and central nervous system anomalies associated with congenital unilateral hypoplasia of depressor anguli oris.

2011 ◽  
Vol 114 (2) ◽  
pp. 414-425 ◽  
Author(s):  
Tetsuji Sekiya ◽  
Masahiro Matsumoto ◽  
Ken Kojima ◽  
Kazuya Ono ◽  
Yayoi S. Kikkawa ◽  
...  

Object Hearing levels following microsurgical treatment gradually deteriorate in a number of patients treated for vestibular schwannoma (VS), especially in the subacute postoperative stage. The cause of this late-onset deterioration of hearing is not completely understood. The aim of this study was to investigate the possibility that reactive gliosis is a contributory factor. Methods Mechanical damage to nerve tissue is a feature of complex surgical procedures. To explore this aspect of VS treatment, the authors compressed rat auditory nerves with 2 different degrees of injury while monitoring the compound action potentials of the auditory nerve and the auditory brainstem responses. In this experimental model, the axons of the auditory nerve were quantitatively and highly selectively damaged in the cerebellopontine angle without permanent compromise of the blood supply to the cochlea. The temporal bones were processed for immunohistochemical analysis at 1 week and at 8 weeks after compression. Results Reactive gliosis was induced not only in the auditory nerve but also in the cochlear nucleus following mechanical trauma in which the general shape of the auditory brainstem response was maintained. There was a substantial outgrowth of astrocytic processes from the transitional zone into the peripheral portion of the auditory nerve, leading to an invasion of dense gliotic tissue in the auditory nerve. The elongated astrocytic processes ran in parallel with the residual auditory neurons and entered much further into the cochlea. Confocal images disclosed fragments of neurons scattered in the gliotic tissue. In the cochlear nucleus, hypertrophic astrocytic processes were abundant around the soma of the neurons. The transverse diameter of the auditory nerve at and proximal to the compression site was considerably reduced, indicating atrophy, especially in rats in which the auditory nerve was profoundly compressed. Conclusions The authors found for the first time that mechanical stress to the auditory nerve causes substantial reactive gliosis in both the peripheral and central auditory pathways within 1–8 weeks. Progressive reactive gliosis following surgical stress may cause dysfunction in the auditory pathways and may be a primary cause of progressive hearing loss following microsurgical treatment for VS.


2019 ◽  
Author(s):  
Naomi Bramhall ◽  
Garnett McMillan ◽  
Frederick Gallun ◽  
Dawn Konrad-Martin

Tinnitus is one of the predicted perceptual consequences of cochlear synaptopathy, a type of age-, noise-, or drug-induced auditory damage that has been demonstrated in animal models to cause homeostatic changes in central auditory gain. Although synaptopathy has been observed in human temporal bones, assessment of this condition in living humans is limited to indirect non-invasive measures such as the auditory brainstem response (ABR). In animal models, synaptopathy is associated with a reduction in ABR wave I amplitude at suprathreshold stimulus levels. Several human studies have explored the relationship between wave I amplitude and tinnitus, with conflicting results. This study investigates the hypothesis that reduced peripheral auditory input due to synaptic/neuronal loss is associated with tinnitus. ABR wave I amplitude data from 193 individuals (43 with tinnitus (22%), 150 without tinnitus (78%)), who participated in up to three out of four different studies, were included in a logistic regression analysis to estimate the relationship between wave I amplitude and tinnitus at a variety of stimulus levels and frequencies. Statistical adjustment for sex and distortion product otoacoustic emissions was included in the analysis. The results suggest that smaller ABR wave I amplitudes are associated with an increased probability of reporting tinnitus.


2020 ◽  
Vol 18 (2) ◽  
pp. 57-61
Author(s):  
B.L. Shrestha ◽  
S. Karmacharya ◽  
A. Dhakal ◽  
A.K. KC ◽  
K.S. Shrestha ◽  
...  

Background Hearing loss among neonates is one of the important health issue in pediatric population which may remain unnoticed until the child reaches a certain age. The importance of universal early screening, diagnosis and intervention in reducing the negative impact of congenital hearing loss has been described all over the world. Objective To observe the outcome of hearing screening by Automated Auditory Brainstem Response (AABR) in newborns delivered in Dhulikhel Hospital and neonates admitted in an intensive care unit (NICU) of Dhulikhel Hospital. Method A prospective study was done in neonates who were born at Dhulikhel Hospital, Kathmandu University Hospital from February 15th, 2017 to October 30th, 2019. AABR was used for their hearing assessment within 24 hours of birth and again at about 6 weeks of age in those neonates who failed the initial test. All the neonates admitted in NICU were studied regarding the risk factors based on Joint committee on Infant Hearing. Those who failed the test for the second time were referred for detailed audiological diagnostic work up. Result The screening rate was 92.6% of the total deliveries. A total of 5517 neonates comprising of 2800 males and 2717 females were screened from total deliveries of 5956 neonates in the study period. Among them, NICU (sick) babies were 422 (7.7%) and well babies were 5095 (92.3%). Out of them, 1675 failed the test in the first screening and 374 failed in the second screening. So, the total number of referred babies in second screening was 6.7% (374) out of 5517 screened. Amongst them, well babies were 6.59% (336), out of 5095 screened and sick babies were 9% (38) out of 422 screened. Low birth weight and prematurity were found to be the commonest risk factor present among them, followed by the use of ototoxic medications, hyperbilirubinemia and prolonged use of mechanical ventilation. Conclusion Automated Auditory Brainstem Response (AABR) is a very useful tool for hearing screening which should preferably be done in all the neonates where possible. It should be done within one month of life and those with confirmed hearing loss should receive early appropriate intervention for better hearing in future.


1992 ◽  
Vol 107 (6_part_1) ◽  
pp. 763-768 ◽  
Author(s):  
J. Scott Greene ◽  
Neil A. Giddings ◽  
John T. Jacobson

A significant number of patients undergo Irradiation to the temporal bone for malignancies. Conflicting reports exist regarding the effects of irradiation on hearing thresholds. Although radiation-induced otitis media and osteoradionecrosis of the ossicles with resultant conductive hearing loss are well-documented, there is disagreement regarding the effect of irradiation on sensorineural hearing. Previous animal models, relying only on behavioral tests and reflex thresholds, have failed to reveal consistent threshold shifts after irradiation. However, with the advent of auditory brainstem response (ABR) testing, a reliable objective measurement of hearing in animals is available. Hearing thresholds were determined bilaterally by ABR testing in 21 albino guinea pigs. The left temporal bones of sixteen animals were then Irradiated with a total dose ranging from 5750 to 7000 cGy over 7 weeks. The right ears of these animals, plus both ears of five nonlrradlated guinea pigs, served as controls. Follow-up threshold ABRs were obtained immediately post-irradlation (RT), and at 6 and 12 months post-RT. Average thresholds in all groups increased over time: 60 dB in the control group; 53 dB in the control ears of the Irradiated animals; and 46 dB in the Irradiated ears. There were no statistically significant increases in ABR thresholds for Irradiated ears vs. control ears. At the 6-month followup, hearing was actually better in the Irradiated ears than the control ears and this difference between ears was significantly greater than the difference at baseline ( p < 0.026). Overall, there was no evidence that irradiation produces changes in ABR thresholds.


2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Guangwei Zhou ◽  
Briana Dornan ◽  
Wheaton Hinchion

Objectives. To review our experience of conducting auditory brainstem response (ABR) test on children in the operating room and discuss the benefits versus limitations of this practice.Methods. Retrospective review study conducted in a pediatric tertiary care facility. A total of 267 patients identified with usable data, including ABR results, medical and surgical notes, and follow-up evaluation.Results. Hearing status successfully determined in all patients based on the ABR results form the operating room. The degrees and the types of hearing loss also documented in most of the cases. In addition, multiple factors that may affect the outcomes of ABR in the operating room identified.Conclusions. Hearing loss in children with complicated medical issues can be accurately evaluated via ABR testing in the operating room. Efforts should be made to eliminate adverse factors to ABR recording, and caution should be taken when interpreting ABR results from the operating room.


2018 ◽  
Vol 128 (1) ◽  
pp. 62-65 ◽  
Author(s):  
Natalie A. Krane ◽  
Jeffrey D. Markey ◽  
Myriam Loyo

Introduction: Congenital unilateral lower lip palsy is an infrequently encountered condition that manifests as lower lip asymmetry during smiling, laughing, and crying. Treatment options are not well characterized. Methods: The authors present the case of a 51-year-old woman who was referred for surgical intervention for facial paralysis. Physical examination demonstrated a symmetric face at rest that became asymmetric when smiling. The asymmetry, evident by inappropriate inferior displacement of the lower lip, was secondary to unilateral contraction and presence of the depressor labii inferioris. The depressor anguli oris was symmetric bilaterally. Her presentation was consistent with congenital unilateral lower lip palsy. Results: Lidocaine was injected into the depressor labii inferioris on the side of the face that demonstrated unilateral presence and contraction. This resulted in symmetry of the smile and lower lip without untoward effect. Onabotulinum toxin A was thereafter injected into the depressor labii inferioris. In-office treatment with botulinum toxin injection resulted in a 4-month improvement in smile symmetry. Conclusion: Chemodenervation is a safe and minimally invasive method to improve smile symmetry and lower lip position in cases of congenital unilateral lower lip palsy.


2021 ◽  
pp. 019459982110441
Author(s):  
Evette A. Ronner ◽  
Cheryl K. Glovsky ◽  
Barbara S. Herrmann ◽  
Melissa A. Woythaler ◽  
Mark S. Pasternack ◽  
...  

Objective To assess the effectiveness and outcomes of a targeted cytomegalovirus (CMV) testing protocol. Study Design Retrospective chart review. Setting Tertiary care institution. Methods Targeted screening for CMV in children who did not pass the newborn hearing screening (NHS) was introduced in July 2015 for the level 2 and 3 nurseries at our hospital. In January 2016, this policy was extended to include all nurseries. Retrospective chart review was performed for all newborns who did not pass their NHS between 2013 and 2020. CMV testing rates and related outcomes were compared before and after rollout. Results A total of 891 charts were reviewed for infants who did not pass their NHS: 530 (60%) had CMV testing, of which 8 (1.5%) tested positive. Three cases were detected prior to and 5 occurred after initiation of targeted screening. Six CMV+ infants demonstrated hearing loss on confirmatory auditory brainstem response, all of whom began treatment with oral valganciclovir. Hearing remained stable in 3 patients, progressed in 2, and improved in 1. The rate of CMV testing in children who did not pass their NHS increased from 14% to 88% after full implementation of targeted screening ( P < .001). The average age at initial infectious disease consultation was significantly younger for infants born after targeted screening ( P < .001). Conclusion Targeted screening is a feasible and effective method to identify CMV+ infants early in life. Implementation of a targeted screening program for CMV in children who do not pass the NHS resulted in significantly higher rates of CMV testing and earlier referral to infectious disease.


1996 ◽  
Vol 5 (1) ◽  
pp. 90-96 ◽  
Author(s):  
Frank E. Musiek ◽  
Cynthia A. McCormick ◽  
Raymond M. Hurley

We performed a retrospective study of 26 patients with acoustic tumors and 26 patients with otologically diagnosed cochlear pathology to determine the sensitivity (hit rate), specificity (false-alarm rate), and efficiency of six auditory brainstem response indices. In addition, a utility value was determined for each of these six indices. The I–V interwave interval, the interaural latency difference, and the absolute latency of wave V provided the highest hit rates, the best A’ values and good utility. The V/I amplitude ratio index provided high specificity but low sensitivity scores. In regard to sensitivity and specificity, using the combination of two indices provided little overall improvement over the best one-index measures.


2019 ◽  
Vol 28 (1) ◽  
pp. 114-124
Author(s):  
Linda W. Norrix ◽  
Julie Thein ◽  
David Velenovsky

Purpose Low residual noise (RN) levels are critically important when obtaining electrophysiological recordings of threshold auditory brainstem responses. In this study, we examine the effectiveness and efficiency of Kalman-weighted averaging (KWA) implemented on the Vivosonic Integrity System and artifact rejection (AR) implemented on the Intelligent Hearing Systems SmartEP system for obtaining low RN levels. Method Sixteen adults participated. Electrophysiological measures were obtained using simultaneous recordings by the Vivosonic and Intelligent Hearing Systems for subjects in 2 relaxed conditions and 4 active motor conditions. Three averaging times were used for the relaxed states (1, 1.5, and 3 min) and for the active states (1.5, 3, and 6 min). Repeated-measures analyses of variance were used to examine RN levels as a function of noise reduction strategy (i.e., KWA, AR) and averaging time. Results Lower RN levels were obtained using KWA than AR in both the relaxed and active motor states. Thus, KWA was more effective than was AR under the conditions examined in this study. Using KWA, approximately 3 min of averaging was needed in the relaxed condition to obtain an average RN level of 0.025 μV. In contrast, in the active motor conditions, approximately 6 min of averaging was required using KWA. Mean RN levels of 0.025 μV were not attained using AR. Conclusions When patients are not physiologically quiet, low RN levels are more likely to be obtained and more efficiently obtained using KWA than AR. However, even when using KWA, in active motor states, 6 min of averaging or more may be required to obtain threshold responses. Averaging time needed and whether a low RN level can be attained will depend on the level of motor activity exhibited by the patient.


2020 ◽  
Vol 63 (11) ◽  
pp. 3877-3892
Author(s):  
Ashley Parker ◽  
Candace Slack ◽  
Erika Skoe

Purpose Miniaturization of digital technologies has created new opportunities for remote health care and neuroscientific fieldwork. The current study assesses comparisons between in-home auditory brainstem response (ABR) recordings and recordings obtained in a traditional lab setting. Method Click-evoked and speech-evoked ABRs were recorded in 12 normal-hearing, young adult participants over three test sessions in (a) a shielded sound booth within a research lab, (b) a simulated home environment, and (c) the research lab once more. The same single-family house was used for all home testing. Results Analyses of ABR latencies, a common clinical metric, showed high repeatability between the home and lab environments across both the click-evoked and speech-evoked ABRs. Like ABR latencies, response consistency and signal-to-noise ratio (SNR) were robust both in the lab and in the home and did not show significant differences between locations, although variability between the home and lab was higher than latencies, with two participants influencing this lower repeatability between locations. Response consistency and SNR also patterned together, with a trend for higher SNRs to pair with more consistent responses in both the home and lab environments. Conclusions Our findings demonstrate the feasibility of obtaining high-quality ABR recordings within a simulated home environment that closely approximate those recorded in a more traditional recording environment. This line of work may open doors to greater accessibility to underserved clinical and research populations.


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