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https://www.readbyqxmd.com/read/27909890/does-severity-of-cerebral-mri-lesions-in-congenital-cmv-infection-correlates-with-the-outcome-of-cochlear-implantation
#1
Stefan Lyutenski, Friedrich Götz, Alexandros Giourgas, Omid Majdani, Eva Bültmann, Heinrich Lanfermann, Thomas Lenarz, Anja M Giesemann
The objective is to investigate whether there is a correlation between the severity of typical brain lesions in congenital cytomegalovirus (cCMV) infection and cochlear implant (CI) outcome. The design of the study is a retrospective single-institutional chart review (2005-2015), performed in a tertiary academic referral center. 23 children with typical signs of cCMV infection on cerebral magnetic resonance imaging (MRI) and bilateral severe-to-profound sensorineural hearing loss were retrospectively evaluated...
December 1, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/27909888/off-the-ear-with-no-loss-in-speech-understanding-comparing-the-rondo-and-the-opus-2-cochlear-implant-audio-processors
#2
Stefan Dazert, Jan Peter Thomas, Andreas Büchner, Joachim Müller, John Martin Hempel, Hubert Löwenheim, Robert Mlynski
The RONDO is a single-unit cochlear implant audio processor, which omits the need for a behind-the-ear (BTE) audio processor. The primary aim was to compare speech perception results in quiet and in noise with the RONDO and the OPUS 2, a BTE audio processor. Secondary aims were to determine subjects' self-assessed levels of sound quality and gather subjective feedback on RONDO use. All speech perception tests were performed with the RONDO and the OPUS 2 behind-the-ear audio processor at 3 test intervals. Subjects were required to use the RONDO between test intervals...
December 1, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/27901041/bainbridge-ropers-syndrome-caused-by-loss-of-function-variants-in-asxl3-a-recognizable-condition
#3
Alma Kuechler, Johanna Christina Czeschik, Elisabeth Graf, Ute Grasshoff, Ulrike Hüffmeier, Tiffany Busa, Stefanie Beck-Woedl, Laurence Faivre, Jean-Baptiste Rivière, Ingrid Bader, Johannes Koch, André Reis, Ute Hehr, Olaf Rittinger, Wolfgang Sperl, Tobias B Haack, Thomas Wieland, Hartmut Engels, Holger Prokisch, Tim M Strom, Hermann-Josef Lüdecke, Dagmar Wieczorek
Truncating ASXL3 mutations were first identified in 2013 by Bainbridge et al. as a cause of syndromic intellectual disability in four children with similar phenotypes using whole-exome sequencing. The clinical features - postulated by Bainbridge et al. to be overlapping with Bohring-Opitz syndrome - were developmental delay, severe feeding difficulties, failure to thrive and neurological abnormalities. This condition was included in OMIM as 'Bainbridge-Ropers syndrome' (BRPS, #615485). To date, a total of nine individuals with BRPS have been published in the literature in four reports (Bainbridge et al...
November 30, 2016: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/27900365/genome-sequencing-in-a-case-of-niemann-pick-type-c
#4
Max Dougherty, John Lazar, Jason C Klein, Karina Diaz, Theodore Gobillot, Eli Grunblatt, Nicholas Hasle, Daniel Lawrence, Megan Maurano, Maria Nelson, Gregory Olson, Sanjay Srivatsan, Jay Shendure, C Dirk Keene, Thomas Bird, Marshall S Horwitz, Desiree A Marshall
Adult-onset Niemann-Pick disease type C (NPC) is an infrequent presentation of a rare neurovisceral lysosomal lipid storage disorder caused by autosomal recessive mutations in NPC1 (∼95%) or NPC2 (∼5%). Our patient was diagnosed at age 33 when he presented with a 10-yr history of difficulties in judgment, concentration, speech, and coordination. A history of transient neonatal jaundice and splenomegaly with bone marrow biopsy suggesting a lipid storage disorder pointed to NPC; biochemical ("variant" level cholesterol esterification) and ultrastructural studies in adulthood confirmed the diagnosis...
November 2016: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/27895549/the-repeatable-battery-for-the-assessment-of-neuropsychological-status-for-hearing-impaired-individuals-rbans-h-before-and-after-cochlear-implantation-a-protocol-for-a-prospective-longitudinal-cohort-study
#5
Annes J Claes, Griet Mertens, Annick Gilles, Anouk Hofkens-Van den Brandt, Erik Fransen, Vincent Van Rompaey, Paul Van de Heyning
Background: Currently, an independent relationship between hearing loss and cognitive decline in older adults is suggested by large prospective studies. In general, cochlear implants improve hearing and the quality of life in severely to profoundly hearing impaired older persons. However, little is known about the effects of cochlear implantation on the cognitive evolution in this population. Aim of the study: The primary goal of this prospective, longitudinal cohort study is to explore the cognitive profile of severely to profoundly postlingually hearing impaired subjects before and after cochlear implantation...
2016: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/27895351/frequency-of-sensorineural-hearing-loss-in-chronic-suppurative-otitis-media
#6
Syed Sajjad Ali Zaidi, Hamdan Ahmed Pasha, Anwar Suhail, Talha Ahmed Qureshi
Chronic suppurative otitis media (CSOM) is defined as chronic otorrhea (i.e., lasting > 6-12 weeks) through a perforated tympanic membrane. It is generally associated with some degree of conductive hearing loss. However, recurrent ear infections due to perforated eardrum result in absorption of toxins and macromolecules into the cochlea leading to sensorineural hearing loss (SNHL). We planned to determine the frequency of sensorineural hearing loss in chronic suppurative otitis media. A descriptive cross-sectional study was conducted at Aga Kgan University Hospital, Karachi, from October 2013 to March 2014...
October 2016: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/27894912/hearing-thresholds-at-high-frequency-in-patients-with-cystic-fibrosis-a-systematic-review
#7
Debora T M Caumo, Lúcia B Geyer, Adriana R Teixeira, Sérgio S M Barreto
INTRODUCTION: High-frequency audiometry may contribute to the early detection of hearing loss caused by ototoxic medications. Many ototoxic drugs are widely used in the treatment of patients with cystic fibrosis. Early detection of hearing loss should allow known harmful drugs to be identified before the damage affects speech frequencies. The damage caused by ototoxicity is irreversible, resulting in important social and psychological consequences. In children, hearing loss, even when restricted to high frequencies, can affect the development of language...
November 9, 2016: Brazilian Journal of Otorhinolaryngology
https://www.readbyqxmd.com/read/27890881/non-convulsive-status-epilepticus-with-takotsubo-cardiomyopathy-a-case-report
#8
Jyunichi Uemura, Yuko Wada, Yoshiki Yagita
A 61-year-old woman was admitted to our hospital for speech disturbance and mild consciousness impairment. Despite the absence of seizures or loss of consciousness, electroencephalographic abnormalities were observed, and the cerebral blood flow scintigraphy revealed an increase in cerebral blood flow in the left temporal lobe. These findings suggested a diagnosis of non-convulsive status epilepticus. On the third day, a twelve-lead electrocardiogram showed a negative T-wave in I, II, aVf, and V2-6. Moreover, a transthoracic echocardiogram revealed left ventricular apical akinesis...
November 25, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/27885979/gender-differences-in-audiological-findings-and-hearing-aid-benefit-in-255-individuals-with-auditory-neuropathy-spectrum-disorder-a-retrospective-study
#9
Vijaya Kumar Narne, Prashanth Prabhu, Hunsur S Chandan, Mahadeva Deepthi
BACKGROUND: There are many studies reported in the literature that have summarized audiological findings and possible rehabilitation in individuals with auditory neuropathy spectrum disorder (ANSD). However, there are very few studies that have attempted to delineate the gender differences in audiological characteristics and hearing aid benefit in individuals with ANSD. PURPOSE: The study aimed to explore the differences between males and females in terms of demographic details, audiogram, speech identification scores, otoacoustic emissions, acoustic reflexes, long latency responses, and hearing aid benefit...
November 2016: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/27885978/factors-affecting-daily-cochlear-implant-use-in-children-datalogging-evidence
#10
Vijayalakshmi Easwar, Joseph Sanfilippo, Blake Papsin, Karen Gordon
BACKGROUND: Children with profound hearing loss can gain access to sound through cochlear implants (CIs), but these devices must be worn consistently to promote auditory development. Although subjective parent reports have identified several factors limiting long-term CI use in children, it is also important to understand the day-to-day issues which may preclude consistent device use. In the present study, objective measures gathered through datalogging software were used to quantify the following in children: (1) number of hours of CI use per day, (2) practical concerns including repeated disconnections between the external transmission coil and the internal device (termed "coil-offs"), and (3) listening environments experienced during daily use...
November 2016: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/27885975/hearing-benefit-and-rated-satisfaction-in-children-with-unilateral-conductive-hearing-loss-using-a-transcutaneous-magnetic-coupled-bone-conduction-hearing-aid
#11
Melissa J Polonenko, Lora Carinci, Karen A Gordon, Blake C Papsin, Sharon L Cushing
BACKGROUND: Bilateral hearing is important for learning, development, and function in complex everyday environments. Children with conductive and mixed hearing loss (HL) have been treated for years with percutaneous coupling through an abutment, which achieves powerful output, but the implant site is susceptible to skin reactions and trauma. To overcome these complications, transcutaneous magnetic coupling systems were recently introduced. PURPOSE: The purpose of the study was to evaluate whether the new transcutaneous magnetic coupling is an effective coupling paradigm for bone-conduction hearing aids (BCHAs)...
November 2016: Journal of the American Academy of Audiology
https://www.readbyqxmd.com/read/27868434/communication-aid-requirements-of-intensive-care-unit-patients-with-transient-speech-loss
#12
Mohammad H Mobasheri, Dominic King, Simon Judge, Faizan Arshad, Marius Larsen, Zahra Safarfashandi, Hemal Shah, Anna Trepekli, Sanjay Trikha, Diamantis Xylas, Stephen J Brett, Ara Darzi
Alert and transiently nonvocal intensive care unit (ICU) patients are dependent on augmentative and alternative communication (AAC). Unfortunately, the literature demonstrates that existent AAC devices have not been widely adopted, and unaided methods are often the primary modalities used despite being insufficient, and frustrating. We present the results of a qualitative semi-structured interview study with 8 ex-ICU patients, 4 ICU patient relatives, and 6 ICU staff, exploring their AAC needs and requirements...
December 2016: Augmentative and Alternative Communication: AAC
https://www.readbyqxmd.com/read/27867345/language-impairment-resulting-from-a-de-novo-deletion-of-7q32-1q33
#13
María S Jiménez-Romero, Montserrat Barcos-Martínez, Isabel Espejo-Portero, Antonio Benítez-Burraco
We report on a girl who presents with hearing loss, behavioral disturbances (according to the Inventory for Client and Agency Planning) as well as motor and cognitive delay (according to Battelle Developmental Inventories) which have a significant impact on her speech and language abilities [according to the Peabody Picture Vocabulary Test (ed 3), and the Prueba de Lenguaje Oral de Navarra-Revisada (Navarra Oral Language Test, Revised)]. Five copy number variations (CNVs) were identified in the child: arr[hg18] 7q32...
October 2016: Molecular Syndromology
https://www.readbyqxmd.com/read/27863036/newborn-hearing-screening-in-prematurity-fate-of-screening-failures-and-auditory-maturation
#14
Hyung Chae Yang, Chung Man Sung, Dong Joo Shin, Yong Beom Cho, Chul Ho Jang, Hyong-Ho Cho
OBJECTIVES: The purpose of this study was to identify delayed auditory maturation and the fate of premature infants who failed the newborn hearing screening (NHS) in neonatal intensive care unit. MATERIALS AND METHODS: A total of 1,375 neonates underwent NHS using the transient evoked otoacoustic emission (TEOAE) in a tertiary hospital between 2007 and 2010 according to the Joint Committee on Infant Hearing Guidelines. In addition, a structured telephone survey was given to caregivers of infants who were lost to follow-up NHS...
November 12, 2016: Clinical Otolaryngology
https://www.readbyqxmd.com/read/27861465/universal-newborn-hearing-screening-methods-and-results-obstacles-and-benefits-newborn-hearing-screening
#15
Katarzyna E Wroblewska-Seniuk, Piotr Dabrowski, Witold Szyfter, Jan Mazela
The incidence of sensorineural hearing loss ranges from 1 to 3 per 1000 live births in term healthy neonates, and 2-4 per 100 in high-risk infants, a ten-fold increase. Early identification and intervention with hearing augmentation within 6 months yields optimal effect. If undetected and without treatment, significant hearing impairment may negatively impact speech development and lead to disorders in psychological and mental behaviors.Hearing screening programs in newborns enable detection of hearing impairment in the first days after birth...
November 18, 2016: Pediatric Research
https://www.readbyqxmd.com/read/27861389/characteristics-of-hearing-loss-in-patients-with-herpes-zoster-oticus
#16
Chang-Hee Kim, Hyerang Choi, Jung Eun Shin
Patients with herpes zoster oticus (HZO) may commonly show symptoms associated with 7th and 8th cranial nerve (CN VII and CN VIII) dysfunction. The aim of this study is to investigate the characteristics of hearing loss in patients with HZO and discuss possible mechanisms.Ninety-five HZO patients who showed at least one of the symptoms of CN VII and CN VIII dysfunction between January 2007 and October 2014 were included in this study. Hearing loss was defined when the mean thresholds of pure tone audiometry (PTA) in speech frequency (0...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27861193/a-comparison-of-surgical-treatments-for-superior-semicircular-canal-dehiscence-a-systematic-review
#17
Fuat Ziylan, Ahmet Kinaci, Andy J Beynon, Henricus P M Kunst
OBJECTIVE: We investigate the postoperative subjective and objective outcomes of different surgical treatments for superior semicircular canal dehiscence (SSCD): vestibular signs, auditory signs, vestibular evoked myogenic potential test, pure tone audiogram, speech audiogram, or video-nystagmography. DATA SOURCES: An electronic search performed in the PubMed, Cochrane Library, and EMBASE databases on 15th of September 2015. A systematic search was conducted. Articles were included if written in English, Dutch, German, or French language...
November 16, 2016: Otology & Neurotology
https://www.readbyqxmd.com/read/27858146/associations-between-the-probabilities-of-frequency-specific-hearing-loss-and-unaided-aphab-scores
#18
J Löhler, B Wollenberg, P Schlattmann, N Hoang, R Schönweiler
The Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire reports subjective hearing impairments in four typical conditions. We investigated the association between the frequency-specific probability of hearing loss and scores from the unaided APHAB (APHABu) to determine whether the APHABu could be useful in primary diagnoses of hearing loss, in addition to pure tone and speech audiometry. This retrospective study included database records from 6558 patients (average age 69.0 years). We employed a multivariate generalised linear mixed model to analyse the probabilities of hearing losses (severity range 20-75 dB, evaluated in 5-dB steps), measured at different frequencies (0...
November 17, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/27852135/long-term-results-of-hearing-preservation-cochlear-implant-surgery-in-patients-with-residual-low-frequency-hearing
#19
Hideaki Moteki, Shin-Ya Nishio, Maiko Miyagawa, Keita Tsukada, Satoshi Iwasaki, Shin-Ichi Usami
CONCLUSION: Differences were found between patients with stable hearing and those with progressive hearing loss in the lower frequencies with respect to the rate of progression in the contralateral ear. It is suggested that the electric acoustic stimulation (EAS) can provide improvement in hearing ability over the long-term if residual hearing might be lost to some extent. OBJECTIVE: To evaluate the long-term threshold changes in the low frequency hearing of the implanted ear as compared with the non-implanted ear, and the hearing abilities with EAS along with the extent of residual hearing...
November 17, 2016: Acta Oto-laryngologica
https://www.readbyqxmd.com/read/27849248/what-we-know-of-the-central-auditory-disorders-in-children-exposed-to-alcohol-during-pregnancy-systematic-review
#20
Humberto de Oliveira Simões, Sthella Zanchetta, Erikson Felipe Furtado
Purpose: To identify the effects of alcohol intake during pregnancy on the central auditory nervous system in relation to their possible diagnosis, Fetal Alcohol Syndrome, partial Fetal Alcohol Syndrome, Alcohol-Related Birth Defects and Alcohol-Related Neurodevelopmental Disorder, his extension and the hearing assessment method. Research strategy: Systematic and integrative review searched the databases PubMed, LILACS and SciELO, with terms in Portuguese and English "fetal alcohol syndrome", "alcohol-related disorders" associated with "hearing"...
September 2016: CoDAS
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