Computed tomography demonstrates abnormalities of contralateral ear in subjects with unilateral sensorineural hearing loss.

PubMed

Marcus, Sonya; Whitlow, Christopher T; Koonce, James; Zapadka, Michael E; Chen, Michael Y; Williams, Daniel W; Lewis, Meagan; Evans, Adele K

2014-02-01

Prior studies have associated gross inner ear abnormalities with pediatric sensorineural hearing loss (SNHL) using computed tomography (CT). No studies to date have specifically investigated morphologic inner ear abnormalities involving the contralateral unaffected ear in patients with unilateral SNHL. The purpose of this study is to evaluate contralateral inner ear structures of subjects with unilateral SNHL but no grossly abnormal findings on CT. IRB-approved retrospective analysis of pediatric temporal bone CT scans. 97 temporal bone CT scans, previously interpreted as "normal" based upon previously accepted guidelines by board certified neuroradiologists, were assessed using 12 measurements of the semicircular canals, cochlea and vestibule. The control-group consisted of 72 "normal" temporal bone CTs with underlying SNHL in the subject excluded. The study-group consisted of 25 normal-hearing contralateral temporal bones in subjects with unilateral SNHL. Multivariate analysis of covariance (MANCOVA) was then conducted to evaluate for differences between the study and control group. Cochlea basal turn lumen width was significantly greater in magnitude and central lucency of the lateral semicircular canal bony island was significantly lower in density for audiometrically normal ears of subjects with unilateral SNHL compared to controls. Abnormalities of the inner ear were present in the contralateral audiometrically normal ears of subjects with unilateral SNHL. These data suggest that patients with unilateral SNHL may have a more pervasive disease process that results in abnormalities of both ears. The findings of a cochlea basal turn lumen width disparity >5% from "normal" and/or a lateral semicircular canal bony island central lucency disparity of >5% from "normal" may indicate inherent risk to the contralateral unaffected ear in pediatric patients with unilateral sensorineural hearing loss. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

The clinical analysis of bilateral successive sudden sensorineural hearing loss.

PubMed

Wang, Yaowen; Zhang, Litao; Zhang, Jianhua; Zhang, Xuqun; Zhang, Weimin; Chen, Xing; Tang, Shixiong

2016-11-01

The objective of this study is to investigate the clinical characteristics of successive bilateral sudden sensorineural hearing loss (SSNHL) with an interval of more than 1 year to aid the evaluation and management of successive bilateral SSNHL (BSSNHL). 14 successive BSSNHL patients and 118 unilateral SSNHL patients with severe and profound hearing loss were reviewed retrospectively. Information about successive BSSNHL was collected included demographics, the intervals between the attacks of bilateral ears, the past medical history, inducing factors, accompanying symptoms, pure-tone tests, blood tests, b-ultrasound examinations of vertebral artery and carotid artery, and medical interventions. And the comparison of improvement rate was made between successive BSSNHL and unilateral SSNHL. SPSS 15.0 was used to analyze the data. In successive BSSNHL, there were six males and eight females; the average aged was 49.86 ± 15.45 years (20-73 years). The interval of the two attacks was 11.43 ± 12.07 years (1-50 years) on average. The onset of treatment was 18.86 ± 12.71 days. Tinnitus was seen in 100 % of the patients, followed by vertigo in 42.85 %, and ear fullness in 21.43 %. 3 of 14 patients described obvious inducing factor: tiredness. Five patients (35.71 %) had hypertension histories, three (21.43 %) had diabetes histories, two (14.29 %) had surgery histories, one (7.14 %) was with depression history, one (7.14 %) was with coronary heart disease history. 30 % (3/10) patients were with atherosclerotic plaque in carotid artery. 4 (28.57 %) patients were with high blood sugar. 8 patients (57.14 %) were with high blood lipids. Thyroid function tests were positive in 27.27 % (3/11) patients. No abnormality was found in antinuclear antibodies titer. The percentage of profound and severe hearing loss were 71.43 and 78.57 % in the recently affected ear and contralateral ear separately. The PTAs of the recently affected ear were 77.14 ± 27.12 dB before treatment and 68.84 ± 22.32 dB after treatment, the improvement rate was 28.57 %. No improvement was found in the contralateral ears. In unilateral SSNHL, the average age was 42.47 ± 14.18 years. The onset of treatment was 18.08 ± 15.84 days. The improvement rate was 57.63 %, which was much higher than that of successive BSSNHL. There was no difference in age and time of onset of treatment between successive BSSNHL and unilateral SSNHL (p > 0.05). Successive BSSNHL is rare, the hearing impairment is severe, and the outcome of treatment is poorer. It may have a completely different profile compared with the simultaneous BSSNHL and unilateral SSNHL.

Metabolomic analysis of urine with Nuclear Magnetic Resonance spectroscopy in patients with idiopathic sudden sensorineural hearing loss: A preliminary study.

PubMed

Carta, Filippo; Lussu, Milena; Bandino, Fabrizio; Noto, Antonio; Peppi, Marcello; Chuchueva, Natalia; Atzori, Luigi; Fanos, Vassilios; Puxeddu, Roberto

2017-08-01

Idiopathic sudden sensorineural hearing loss is a frequent emergency, with unknown aetiology and usually treated with empiric therapy. Steroids represent the only validated treatment but prognosis is unpredictable and the possibility to select the patients who will not respond to steroids could avoid unnecessary treatments. Metabolomic profiling of the biofluids target the analysis of the final product of genic expression and enzymatic activity, defining the biochemical phenotype of a whole biologic system. We studied the metabolomics of the urine of a cohort of patients with idiopathic sudden sensorineural hearing loss, correlating the metabolic profiles with the clinical outcomes. Metabolomic profiling of urine samples was performed by 1 H Nuclear Magnetic Resonance spectroscopy in combination with multivariate statistical approaches. 26 patients were included in the study: 5 healthy controls, 13 patients who did not recover after treatment at 6 months while the remaining 8 patients recovered from the hearing loss. The orthogonal partial least square-discriminant analysis score plot showed a significant separation between the two groups, responders and non-responders after steroid therapy, R 2 Y of 0.83, Q 2 of 0.38 and p value <0.05. The resulting metabolic profiles were characterized by higher levels of urinary B-Alanine, 3-hydroxybutyrate and Trimethylamine N-oxide, and lower levels of Citrate and Creatinine in patients with worst outcome. Idiopathic sudden sensorineural hearing loss is a specific disease with unclear systemic changes, but our data suggest that there are different types of this disorder or patients predisposed to effective action of steroids allowing the recover after treatment. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

The evidence base for the application of contralateral bone anchored hearing aids in acquired unilateral sensorineural hearing loss in adults.

PubMed

Baguley, D M; Bird, J; Humphriss, R L; Prevost, A T

2006-02-01

. Acquired unilateral sensorineural hearing loss reduces the ability to localize sounds and to discriminate in background noise. . Four controlled trials attempt to determine the benefit of contralateral bone anchored hearing aids over contralateral routing of signal (CROS) hearing aids and over the unaided condition. All found no significant improvement in auditory localization with either aid. Speech discrimination in noise and subjective questionnaire measures of auditory abilities showed an advantage for bone anchored hearing aid (BAHA) > CROS > unaided conditions. . All four studies have material shortfalls: (i) the BAHA was always trialled after the CROS aid; (ii) CROS aids were only trialled for 4 weeks; (iii) none used any measure of hearing handicap when selecting subjects; (iv) two studies have a bias in terms of patient selection; (v) all studies were underpowered (vi) double reporting of patients occurred. . There is a paucity of evidence to support the efficacy of BAHA in the treatment of acquired unilateral sensorineural hearing loss. Clinicians should proceed with caution and perhaps await a larger randomized trial. . It is perhaps only appropriate to insert a BAHA peg at the time of vestibular schwanoma tumour excision in patients with good preoperative hearing, as their hearing handicap increases most.

Cost-Utility Analysis of Cochlear Implantation in Australian Adults.

PubMed

Foteff, Chris; Kennedy, Steven; Milton, Abul Hasnat; Deger, Melike; Payk, Florian; Sanderson, Georgina

2016-06-01

Sequential and simultaneous bilateral cochlear implants are emerging as appropriate treatment options for Australian adults with sensory deficits in both cochleae. Current funding of Australian public hospitals does not provide for simultaneous bilateral cochlear implantation (CI) as a separate surgical procedure. Previous cost-effectiveness studies of sequential and simultaneous bilateral CI assumed 100% of unilaterally treated patients' transition to a sequential bilateral CI. This assumption does not place cochlear implantation in the context of the generally treated population. When mutually exclusive treatment options exist, such as unilateral CI, sequential bilateral CI, and simultaneous bilateral CI, the mean costs of the treated populations are weighted in the calculation of incremental cost-utility ratios. The objective was to evaluate the cost-utility of bilateral hearing aids (HAs) compared with unilateral, sequential, and simultaneous bilateral CI in Australian adults with bilateral severe to profound sensorineural hearing loss. Cost-utility analysis of secondary sources input to a Markov model. Australian health care perspective, lifetime horizon with costs and outcomes discounted 5% annually. Bilateral HAs as treatment for bilateral severe to profound sensorineural hearing loss compared with unilateral, sequential, and simultaneous bilateral CI. Incremental costs per quality adjusted life year (AUD/QALY). When compared with bilateral hearing aids the incremental cost-utility ratio for the CI treatment population was AUD11,160/QALY. The incremental cost-utility ratio was weighted according to the number of patients treated unilaterally, sequentially, and simultaneously, as these were mutually exclusive treatment options. No peer-reviewed articles have reported the incremental analysis of cochlear implantation in a continuum of care for surgically treated populations with bilateral severe to profound sensorineural hearing loss. Unilateral, sequential, and simultaneous bilateral CI were cost-effective when compared with bilateral hearing aids. Technologies that reduce the total number of visits for a patient could introduce additional cost efficiencies into clinical practice.

Inner ear disorders.

PubMed

Smouha, Eric

2013-01-01

To present a framework for the diagnosis and treatment of inner ear disorders, with an emphasis on problems common to neuro-rehabilitation. Disorders of the inner ear can cause hearing loss, tinnitus, vertigo and imbalance. Hearing loss can be conductive, sensorineural, or mixed; conductive hearing loss arises from the ear canal or middle ear, while sensorineural hearing loss arises from the inner ear or auditory nerve. Vertigo is a hallucination of motion, and is the cardinal symptom of vestibular system disease. It should be differentiated from other causes of dizziness: gait imbalance, disequilibrium, lightheadedness (pre-syncope). Vertigo can be caused by problems in the inner ear or central nervous system. The diagnosis of inner ear disorders begins with a targeted physical examination. The initial work-up of hearing loss is made by audiometry, and vertigo by electronystagmography (ENG). Supplemental tests and MRI are obtained when clinically indicated. The clinical pattern and duration of vertigo are the most important clinical features in the diagnosis. Common inner ear causes of vertigo include: vestibular neuritis (sudden, unilateral vestibular loss), Meniere's disease (episodic vertigo), benign paroxysmal positional vertigo (BPPV), and bilateral vestibular loss. Common central nervous system causes of vertigo include: post concussion syndrome, cervical vertigo, vestibular migraine, cerebrovascular disease, and acoustic neuroma. A basic knowledge of vestibular physiology, coupled with a understanding of common vestibular syndromes, will lead to correct diagnosis and treatment in most cases.

Idiopathic sudden sensorineural hearing loss and acute low-tone sensorineural hearing loss: a comparison of the results of a nationwide epidemiological survey in Japan.

PubMed

Yoshida, Tadao; Sone, Michihiko; Kitoh, Ryosuke; Nishio, Shin-Ya; Ogawa, Kaoru; Kanzaki, Sho; Hato, Naohito; Fukuda, Satoshi; Hara, Akira; Ikezono, Tetsuo; Ishikawa, Kotaro; Iwasaki, Satoshi; Kaga, Kimitaka; Kakehata, Seiji; Matsubara, Atsushi; Matsunaga, Tatsuo; Murata, Takaaki; Naito, Yasushi; Nakagawa, Takashi; Nishizaki, Kazunori; Noguchi, Yoshihiro; Sano, Hajime; Sato, Hiroaki; Suzuki, Mikio; Shojaku, Hideo; Takahashi, Haruo; Takeda, Hidehiko; Tono, Testuya; Yamashita, Hiroshi; Yamasoba, Tatsuya; Usami, Shin-Ichi

2017-01-01

The aim of this study was to investigate the differences between idiopathic sudden sensorineural hearing loss (SSNHL), and acute low-tone sensorineural hearing loss (ALHL) using the results of a nationwide survey database in Japan and to analyze the variables associated with their clinical features and the severity of hearing impairment, treatment, and prognosis. Participants were patients registered between April 2014 and March 2016 in a multicenter epidemiological survey database involving 30 university hospitals and medical centers across Japan. Statistical analysis was performed to clarify the factors associated with their clinical characteristics and the severity of hearing impairment, treatment, and prognosis. Idiopathic SSNHL and ALHL differed significantly in terms of male-to-female ratio, age distribution, and time from onset to start of treatment. The treatment methods and hearing prognosis also differed markedly between the two diseases. A majority (92%) of idiopathic SSNHL patients were administered some type of corticosteroid, while half of the ALHL patients received corticosteroids and a diuretic agent. The results suggested that idiopathic SSNHL and ALHL belonged to different categories of inner ear disease.

Cochlear implants as a treatment option for unilateral hearing loss, severe tinnitus and hyperacusis.

PubMed

Ramos Macías, Angel; Falcón González, Juan Carlos; Manrique, Manuel; Morera, Constantino; García-Ibáñez, Luis; Cenjor, Carlos; Coudert-Koall, Chrystellel; Killian, Matthijs

2015-01-01

Tinnitus is an incapacitating condition commonly affecting cochlear implant (CI) candidates. The aim of this clinical study is to assess the long-term effects of CI treatment in patients with severe-to-profound, sensorineural, unilateral hearing loss (UHL) and incapacitating tinnitus. We performed a prospective Cochlear™ company-sponsored multicentre study in five Spanish centres. Sixteen patients with UHL and incapacitating tinnitus, which was indicated by a Tinnitus Handicap Inventory (THI) score >58%, received a Nucleus® CI in their deaf ear. The study design includes repeated within-subject measures on hearing, tinnitus, hyperacusis and quality of life up to 12 months after initial CI fitting. In addition to hearing loss and tinnitus, all patients suffered from hyperacusis. Most patients had a sudden hearing loss and received a CI within 2 years after their hearing loss. Preliminary 6-month, post-CI activation data of 13 subjects showed that the majority of patients perceived a subjective benefit from CI treatment, which was assessed using the THI, a Visual Analogue Scale of tinnitus loudness/annoyance and the Speech, Spatial and Qualities of Hearing Scale. Preliminary 12-month data of 7 subjects showed that most patients also perceived a degree of relief from their hyperacusis. One patient showed no improvements in any of the applied scales, which could be explained by partial insertion of the electrode due to obstruction of the cochlea by otosclerosis. In conclusion, CI can successfully be used in the treatment of UHL patients with accompanying severe tinnitus and hyperacusis. Implantation resulted in hearing benefits and a durable relief from tinnitus and hyperacusis in the majority of patients. These findings support the hypothesis that pathophysiological mechanisms after peripheral sensorineural hearing loss are at least partly reversible when hearing is restored with a CI. © 2015 S. Karger AG, Basel.

Cochlear pathology in chronic suppurative otitis media.

PubMed

Walby, A P; Barrera, A; Schuknecht, H F

1983-01-01

Chronic suppurative otitis media (COM) is reported to cause elevation of bone-conduction thresholds either by damage to cochlear sensorineural structures or by alteration in the mechanics of sound transmission in the ear. A retrospective study was made of the medical records of 87 patients with unilateral uncomplicated COM to document that abnormality in bone conduction does exist. In a separate study the cochlear pathology in 12 pairs of temporal bones with unilateral COM was studied by light microscopy. Infected ears showed higher than normal mean bone-conduction thresholds by amounts ranging from 1 dB at 500 Hz to 9.5 dB at 4,000 Hz. The temporal bones showed no greater loss of specialized sensorineural structures in infected ears than in normal control ears. Because there is no evidence that COM caused destruction of hair cells or cochlear neurons, alteration in the mechanics of sound transmission becomes a more plausible explanation for the hearing losses.

The place of hyperbaric oxygen therapy and ozone therapy in sudden hearing loss.

PubMed

Ergun Taşdöven, Gülin; Derin, Alper Tunga; Yaprak, Neslihan; Özçağlar, Hasan Ümit

It is difficult to evaluate the effect of drugs clinically used for idiopathic sudden sensorineural hearing loss, mainly because its underlying mechanism remains unknown. This study assessed the efficacy of hyperbaric oxygen therapy or ozone therapy in the treatment of idiopathic sudden sensorineural hearing loss, when either therapy was included with steroid treatment. A retrospective analysis examined 106 patients with idiopathic sudden sensorineural hearing loss seen between January 2010 and June 2012. Those with an identified etiology were excluded. The patients were divided into three treatment groups: oral steroid only (n=65), oral steroid+hyperbaric oxygen (n=26), and oral steroid+ozone (n=17). Treatment success was assessed using Siegel criteria and mean gains using pre- and post-treatment audiograms. The highest response rate to treatment was observed in the oral steroid+ozone therapy group (82.4%), followed by the oral steroid+hyperbaric oxygen (61.5%), and oral steroid groups (50.8%). There were no significant differences in the response to treatment between the oral steroid and oral steroid+hyperbaric oxygen groups (p<0.355). The oral steroid+ozone group showed a significantly higher response rate to treatment than the oral steroid group (p=0.019). There were no significant differences between the oral steroid+hyperbaric oxygen and oral steroid+ozone groups (p=0.146). The efficiency of steroid treatment in patients with severe hearing loss was low. It was statistically ascertained that adding hyperbaric oxygen or ozone therapy to the treatment contributed significantly to treatment success. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Autoimmune Associated Systemic Vasculitis as the Cause of Sudden onset Bilateral Sensorineural Hearing Loss Following Lassa Virus Exposure in a Cynomolgus Macaque Deafness Model

DTIC Science & Technology

2017-01-10

and IL-15 has been considered as a potential therapeutic target for autoimmune diseases such as rheumatoid arthritis and GPA (36, 41, 42...22(1):19-33. 41. Baslund B, et al. (2005) Targeting interleukin-15 in patients with rheumatoid arthritis : a proof-of-concept study. Arthritis Rheum...Berrettini S, et al. (1998) Progressive sensorineural hearing impairment in systemic vasculitides. Semin Arthritis Rheum 27(5):301-318. 27. Webb AA

Cochlear implantation in chronic demyelinating inflammatory polyneuropathy.

PubMed

Mowry, Sarah E; King, Sarah

2017-03-01

To describe a case of chronic inflammatory demyelinating polyneuropathy (CDIP) with bilateral sudden sensorineural hearing loss who subsequently benefited from unilateral cochlear implantation. case history review and review of the literature for the terms CDIP, hearing loss, cochleovestibular dysfunction, and cochlear implantation. A 49-year-old woman presented with bilateral rapidly progressive sensorineural hearing loss (SNHL) 1 month after an upper respiratory tract infection. Hearing loss was not responsive to high-dose steroids and there were no other laboratory abnormalities or physical findings. Within 1 month, she developed ascending motor palsy, requiring long-term ventilator support. This neurologic condition was diagnosed as CDIP and she was successfully treated with plasmapheresis and intravenous immunoglobulin. Her hearing never recovered. At the time of cochlear implant, she had no response at the limits of the audiometer and obtained 0% on AzBio testing. No ABR could be recorded preoperatively. She underwent uneventful cochlear implantation with a perimodilar electrode. One year after activation, she had a PTA of 20 dB and 40% on AzBio sentence testing. Her eABR demonstrated a neuropathy pattern. Only two other cases of CDIP associated with dysfunction of the eighth nerve have been described, and neither had documented profound hearing loss. Severe SNHL associated with CDIP is rare. Although this patient has good access to sound, speech discrimination is poor at 1-year post implantation. This outcome may be due to incomplete recovery of myelination of the eighth nerve. Other possibilities include loss of peripheral nerve fibers due to the initial viral upper respiratory infection, which may lead to less neural substrate to stimulate.

Critical evaluation of round window membrane sealing in the treatment of idiopathic sudden unilateral hearing loss.

PubMed

Hoch, Stephan; Vomhof, Thomas; Teymoortash, Afshin

2015-03-01

Rupture of the round window membrane with consecutive development of a perilymphatic fistula (PLF) is still a matter of controversial debate in the pathogenesis of idiopathic sudden sensorineural hearing loss (SSHL). Until now no consensus exists about whether these patients benefit from performing an exploratory tympanotomy with sealing of the round window. The aim of the present study was to analyze critically the effectiveness of sealing the round window membrane in patients with SSHL. The clinical data of 51 patients with SSHL and a mean hearing decline of at least 60 dB over 5 frequencies who were treated with tympanotomy and sealing of the round window membrane were retrospectively analyzed. The results have been compared to the current state of the literature. Intraoperatively a round window membrane rupture or fluid leak was observed in none of the patients. After performing tympanotomy the mean improvement of hearing level was 32.7 dB. Twenty of 51 examined patients (39.2%) showed a mean improvement of the hearing level of more than 30 dB and a complete remission could be detected in 12 patients (23.5%). Reviewing the literature revealed no standard guidelines for definition or treatment of SSHL as well as for evaluation of hearing loss and its recovery. The results of the present study and the literature should be discussed critically. It is unclear whether tympanotomy and sealing of the round window membrane may be a meaningful treatment for SSHL. Therefore this procedure should be discussed as a therapeutic option only in selected patients with sudden deafness or profound hearing loss in which PLF is strongly suspicious or conservative treatment failed.

Sudden Bilateral Sensorineural Hearing Loss Following Postpartum Hemorrhage: A Case Report

PubMed Central

Mirzaeian, Sara; Ayati, Sedigheh; Maleki, Asieh

2017-01-01

The prevalence of bilateral sudden sensorineural hearing loss (SSNHL) is less than 5% and the etiology of most cases is unknown. Due to many structural and functional similarities between the kidney and inner ear, many conditions, diseases, and drugs have both renal and cochlear effects and toxicities. There are several reports of SSNHL in patients with CRF, uraemic patient, hemodialysis treatment, and ARF. Here, we report a rare manifestation of SSNHL following severe postpartum hemorrhage that has simultaneous renal failure and cochlear impairment. The patient was a 22-year-old primigravida woman with term pregnancy who after delivery and episiotomy hematoma and postpartum hemorrhage subsequently suffered from kidney failure, oliguria, and SSNHL that occurred after 3 days of delivery. In conditions such as severe postpartum bleeding leading to acute renal involvement, the possibility of simultaneous involvement of cochlea due to hypoxia or received drugs should be considered. PMID:28761208

Cochlear implantation in adults: a systematic review and meta-analysis.

PubMed

Gaylor, James M; Raman, Gowri; Chung, Mei; Lee, Jounghee; Rao, Madhumathi; Lau, Joseph; Poe, Dennis S

2013-03-01

Sensorineural hearing loss is the third leading cause of years lived with disability worldwide. Cochlear implants may provide a viable alternative to hearing aids for this type of hearing loss. The Coverage and Analysis Group at the Centers for Medicare & Medicaid Services was interested in an evaluation of recently published literature on this topic. In addition, this meta-analysis is to our knowledge the first to evaluate quality-of-life (QOL) outcomes in adults with cochlear implants. To evaluate the communication-related outcomes and health-related QOL outcomes after unilateral or bilateral cochlear implantation in adults with sensorineural hearing loss. MEDLINE, Cochrane Central Register of Controlled Trials, Scopus, and previous reports from January 1, 2004, through May 31, 2012. Published studies of adult patients undergoing unilateral or bilateral procedures with multichannel cochlear implants and assessments using open-set sentence tests, multisyllable word tests, or QOL measures. Five researchers extracted information on population characteristics, outcomes of interest, and study design and assessed the studies for risk of bias. Discrepancies were resolved by consensus. A total of 42 studies met the inclusion criteria. Most unilateral implant studies showed a statistically significant improvement in mean speech scores as measured by open-set sentence or multisyllable word tests; meta-analysis revealed a significant improvement in QOL after unilateral implantation. Results from studies assessing bilateral implantation showed improvement in communication-related outcomes compared with unilateral implantation and additional improvements in sound localization compared with unilateral device use or implantation only. Based on a few studies, the QOL outcomes varied across tests after bilateral implantation. Unilateral cochlear implants provide improved hearing and significantly improve QOL, and improvements in sound localization are noted for bilateral implantation. Future studies of longer duration, higher-quality reporting, and large databases or registries of patients with long-term follow-up data are needed to yield stronger evidence.

Ototoxic effect of erythromycin therapy.

PubMed

Schweitzer, V G; Olson, N R

1984-04-01

Thirty-two cases of sudden, symmetrical, high-frequency, sensorineural hearing loss associated with intravenous (IV) and oral erythromycin therapy have been published since 1973. We treated a patient with reversible ototoxicity associated with IV erythromycin for the treatment of legionnaire's disease.

Evaluation of the occurrence of canine congenital sensorineural deafness in puppies of predisposed dog breeds using the brainstem auditory evoked response.

PubMed

Płonek, Marta; Giza, Elżbieta; Niedźwiedź, Artur; Kubiak, Krzysztof; Nicpoń, Józef; Wrzosek, Marcin

2016-12-01

Canine congenital sensorineural deafness (CCSD) affects predisposed breeds of dogs and is primarily caused by an atrophy of the stria vascularis of the organ of Corti. The analysis of the brainstem auditory evoked response (BAER) is a reliable method for the evaluation of hearing in animals as it allows an accurate detection of unilateral or bilateral deafness. The occurrence of unilateral and bilateral deafness using the BAER was determined in a representative group of dogs in Poland, including Bull Terriers (n = 117), Australian Cattle Dogs (n = 62), English Setters (n = 32) and the Dogo Argentino (n = 32). Overall deafness, deafness in each dog breed and an association between deafness and phenotype were studied. Among the 243 dogs tested, 156 (81%) had a normal BAER, 27 (11%) were unilaterally deaf, and 12 (5%) were bilaterally deaf. The amplitudes and latencies of waves I, II, III, V, the V/I wave amplitude ratio, and wave I-V, I-III and III-V inter-peak intervals were recorded for each dog. Unilaterally and bilaterally deaf dogs were present in all the dog breeds studied. There were 17 (14.5%) deaf Bull Terriers, three (4.8%) deaf Australian Cattle Dogs, seven (21.9%) deaf English Setters, and 12 (37.5%) deaf Dogos Argentinos. Preventive BAER screening should be routinely performed in these four breeds to prevent the spread of genes responsible for deafness.

Isolated cochlear neuritis from varicella reactivation mimicking a vestibular schwannoma.

PubMed

Goodale, Adam D; Golub, Justin S; Cornelius, Rebecca S; Samy, Ravi N

2016-09-01

We present a case of a patient with progressive unilateral sensorineural hearing loss and tinnitus with internal auditory canal enhancement on magnetic resonance imaging (MRI) secondary to isolated cochlear neuritis from varicella reactivation. MRI following antiviral treatment showed resolution of enhancement. Varicella reactivation is commonly seen in the form of Ramsay Hunt syndrome, which is known to produce abnormal MRI enhancement from facial and vestibulocochlear neuritis; however, its characteristic clinical signs aid the diagnosis. This case is unique in that the only manifestation of varicella infection was unilateral hearing loss. This case outlines the importance of maintaining a broad differential diagnosis in the evaluation of unilateral hearing loss as well as recognizing the limited specificity of MRI.

Idiopathic sudden sensorineural hearing loss and ménière syndrome: The role of cerebral venous drainage.

PubMed

Ciccone, M M; Scicchitano, P; Gesualdo, M; Cortese, F; Zito, A; Manca, F; Boninfante, B; Recchia, P; Leogrande, D; Viola, D; Damiani, M; Gambacorta, V; Piccolo, A; De Ceglie, V; Quaranta, N

2018-02-01

To evaluate the influence of cerebral venous drainage on the pathogenesis of idiopathic sudden sensorineural hearing loss (ISSHL) and Ménière syndrome (MD). Observational, prospective, cohort study. ENT and Cardiology Departments (University of Bari, Policlinico Hospital, Bari, Italy). We enrolled 59 consecutive patients (32 males, mean age 53.05 + 15.37 years): 40 ISSHL and 19 MD. All patients underwent physical examination, biochemical evaluation (glycemic and lipid profile, viral serology, C reactive protein, etc), audiometric (tonal, vocal, vestibular evoked myogenic potentials and auditory brainstem response test) and impedentiometric examination. The pure tone average (PTA) was calculated for the following frequencies: 250, 500, 1000, 2000, 3000, 4000, 8000. An echo-color Doppler evaluation of the venous cerebral veins, internal jugular (IJV) and vertebral veins (VV) at supine and 90° position was performed. No morphological alterations were found both in patients and controls. There were no signs of stenosis, blocked flow, membranes, etc. We found lower minimum, mean and maximum velocities in distal IJVs (P = .019; P = .013; P = .022; respectively) and left VVs (P = .027; P = .008; P = .001; respectively) in supine (0°) position in both MD and ISSHL patients as compared to controls. The same was for orthostatic position (90°). We found negative correlations between the velocities in extracranial veins and PTA values: therefore, the worst the audiometric performance of the subjects, the lower the velocities in the venous cerebral drainage. Idiopathic sudden sensorineural hearing loss and Ménière syndrome patients showed altered venous flow in IJVs and VVs as compared to controls, independently from posture. This different behavior of venous tone control can influence the ear performance and may have a role in the pathogenesis of both diseases. © 2017 John Wiley & Sons Ltd.

Multivariate analysis of prognostic factors for idiopathic sudden sensorineural hearing loss treated with adjuvant hyperbaric oxygen therapy.

PubMed

Xie, Shaobing; Qiang, Qingfen; Mei, Lingyun; He, Chufeng; Feng, Yong; Sun, Hong; Wu, Xuewen

2018-01-01

The objective of this study is to evaluate possible prognostic factors of idiopathic sudden sensorineural hearing loss (ISSNHL) treated with adjuvant hyperbaric oxygen therapy (HBOT) using univariate and multivariate analyses. From January 2008 to October 2016, records of 178 ISSNHL patients treated with auxiliary hyperbaric oxygen therapy were reviewed to assess hearing recovery and evaluate associated prognostic factors (gender, age, localization, initial hearing threshold, presence of tinnitus, vertigo, ear fullness, hypertension, diabetes, onset of HBOT, number of HBOT, and audiogram), by using univariate and multivariate analyses. The overall recovery rate was 37.1%, including complete recovery (19.7%) and partial recovery (17.4%). According to multivariate analysis, later onset of HBOT and higher initial hearing threshold were associated with a poor prognosis in ISSNHL patients treated with HBOT. HBOT is a safe and beneficial adjuvant therapy for ISSNHL patients. 20 sessions of HBOT is possibly enough to show its therapeutic effect. Earlier HBOT onset and lower initial hearing threshold is associated with favorable hearing recovery.

Prevalence, heritability and genetic correlations of congenital sensorineural deafness and pigmentation phenotypes in the Border Collie.

PubMed

De Risio, Luisa; Lewis, Tom; Freeman, Julia; de Stefani, Alberta; Matiasek, Lara; Blott, Sarah

2011-06-01

The objectives of this study were to estimate prevalence, heritability and genetic correlations of congenital sensorineural deafness (CSD) and pigmentation phenotypes in the Border Collie. Entire litters of Border Collies that presented to the Animal Health Trust (1994-2008) for assessment of hearing status by brain stem auditory evoked response (BAER) at 4-10 weeks of age were included. Heritability and genetic correlations were estimated using residual maximum likelihood (REML). Of 4143 puppies that met the inclusion criteria, 97.6% had normal hearing status, 2.0% were unilaterally deaf and 0.4% were bilaterally deaf. Heritability of deafness as a trichotomous trait (normal/unilaterally deaf/bilaterally deaf) was estimated at 0.42 using multivariate analysis. Genetic correlations of deafness with iris colour and merle coat colour were 0.58 and 0.26, respectively. These results indicate that there is a significant genetic effect on CSD in Border Collies and that some of the genes determining deafness also influence pigmentation phenotypes. Copyright © 2010 Elsevier Ltd. All rights reserved.

Sudden Sensorineural Hearing Loss: The Question of Perilymph Fistula.

ERIC Educational Resources Information Center

Backous, Douglas D.; Niparko, John K.

1997-01-01

Perilymph fistula (PLF) is an abnormal communication between the fluid-containing spaces of the inner ear and the air-containing spaces of the temporal bone that can cause hearing loss, tinnitus, aural fullness, vertigo, and postural instability. Diagnosis of PLF and management of those with presumed PLF are discussed. (Contains extensive…

Brainstem auditory evoked responses in an equine patient population: part I--adult horses.

PubMed

Aleman, M; Holliday, T A; Nieto, J E; Williams, D C

2014-01-01

Brainstem auditory evoked response has been an underused diagnostic modality in horses as evidenced by few reports on the subject. To describe BAER findings, common clinical signs, and causes of hearing loss in adult horses. Study group, 76 horses; control group, 8 horses. Retrospective. BAER records from the Clinical Neurophysiology Laboratory were reviewed from the years of 1982 to 2013. Peak latencies, amplitudes, and interpeak intervals were measured when visible. Horses were grouped under disease categories. Descriptive statistics and a posthoc Bonferroni test were performed. Fifty-seven of 76 horses had BAER deficits. There was no breed or sex predisposition, with the exception of American Paint horses diagnosed with congenital sensorineural deafness. Eighty-six percent (n = 49/57) of the horses were younger than 16 years of age. The most common causes of BAER abnormalities were temporohyoid osteoarthropathy (THO, n = 20/20; abnormalities/total), congenital sensorineural deafness in Paint horses (17/17), multifocal brain disease (13/16), and otitis media/interna (4/4). Auditory loss was bilateral and unilateral in 74% (n = 42/57) and 26% (n = 15/57) of the horses, respectively. The most common causes of bilateral auditory loss were sensorineural deafness, THO, and multifocal brain disease whereas THO and otitis were the most common causes of unilateral deficits. Auditory deficits should be investigated in horses with altered behavior, THO, multifocal brain disease, otitis, and in horses with certain coat and eye color patterns. BAER testing is an objective and noninvasive diagnostic modality to assess auditory function in horses. Copyright © 2014 by the American College of Veterinary Internal Medicine.

Early investigational drugs for hearing loss

PubMed Central

Mukherjea, Debashree; Ghosh, Sumana; Bhatta, Puspanjali; Sheth, Sandeep; Tupal, Srinivasan; Borse, Vikrant; Brozoski, Thomas; Sheehan, Kelly E; Rybak, Leonard P; Ramkumar, Vickram

2017-01-01

Introduction Sensorineural hearing loss (HL) is becoming a global phenomenon at an alarming rate. Nearly 600 million people have been estimated to have significant HL in at least one ear. There are several different causes of sensorineural HL included in this review of new investigational drugs for HL. They are noise-induced, drug-induced, sudden sensorineural HL, presbycusis and HL due to cytomegalovirus infections. Areas covered This review presents trends in research for new investigational drugs encompassing a variety of causes of HL. The studies presented here are the latest developments either in the research laboratories or in preclinical, Phase 0, Phase I or Phase II clinical trials for drugs targeting HL. Expert opinion While it is important that prophylactic measures are developed, it is extremely crucial that rescue strategies for unexpected or unavoidable cochlear insult be established. To achieve this goal for the development of drugs for HL, innovative strategies and extensive testing are required for progress from the bench to bedside. However, although a great deal of research needs to be done to achieve the ultimate goal of protecting the ear against acquired sensorineural HL, we are likely to see exciting breakthroughs in the near future. PMID:25243609

Hyperbaric Oxygen Therapy Registry

ClinicalTrials.gov

2018-04-30

Air or Gas Embolism; Carbon Monoxide Poisoning; Clostridial Myositis and Myonecrosis (Gas Gangrene); Crush Injury, Compartment Syndrome & Other Acute Traumatic Ischemias; Decompression Sickness; Peripheral Arterial Insufficiency and Central Retinal Artery Occlusion; Severe Anemia; Intracranial Abscess; Necrotizing Soft Tissue Infections; Osteomyelitis (Refractory); Delayed Radiation Injury (Soft Tissue and Bony Necrosis); Compromised Grafts and Flaps; Acute Thermal Burn Injury; Idiopathic Sudden Sensorineural Hearing Loss

Clinical and audiologic characteristics of patients with sensorineural tinnitus and its association with psychological aspects: an analytic retrospective study.

PubMed

Al-Swiahb, Jamil Nasser; Hwang, Eul Seung; Kong, Ji Sun; Kim, Woo Jin; Yeo, Sang Won; Park, Shi Nae

2016-12-01

This study was performed to analyze clinical and audiologic characteristics of sensorineural tinnitus and to investigate the associating factors reflecting psychological aspects of stress and depression of the patients. This is a retrospective analytical study conducted in a tinnitus clinic of a tertiary referral center of a university hospital. The medical records of 216 patients suffering from sensorineural tinnitus were thoroughly evaluated to determine correlations between clinical and audiological characteristics, including age, sex, predisposing or etiologic factors, hearing levels up to extended high frequencies, and tinnitus severity. Psychological aspects of stress and depression were also evaluated and analyzed to seek the associations with tinnitus severity. All data were stored in our database bank and were statistically analyzed. Our study subjects showed a slight male predominance. The highest percentage of tinnitus was found in patients of 60-80 years old. Only 32.5 % of tinnitus patients were subjectively aware of their hearing loss, whereas 73 % of subjects had hearing deficits in some frequencies in their audiogram. Hearing impairments were of the low-frequency sensorineural type in 18.2 % of patients and were limited to the high frequencies in 77.9 % of patients. Tinnitus was unilateral in 51 % of patients and had a tonal nature in 45 % of patients. In total, 45.8 % of patients with high-frequency sensorineural hearing loss had high-pitched tinnitus. There were significant correlations between tinnitus severity, loudness and annoyance. Correlations with THI (Tinnitus Handicap Inventory) and Beck depression index scores were also found. Sensorineural tinnitus was related with hearing loss in some frequencies nevertheless of patients' own awareness of hearing loss. Loudness and annoyance of tinnitus seems to be two important factors reflecting psychological problems of patients' stress and depression.

Speech perception in noise in unilateral hearing loss.

PubMed

Mondelli, Maria Fernanda Capoani Garcia; Dos Santos, Marina de Marchi; José, Maria Renata

2016-01-01

Unilateral hearing loss is characterized by a decrease of hearing in one ear only. In the presence of ambient noise, individuals with unilateral hearing loss are faced with greater difficulties understanding speech than normal listeners. To evaluate the speech perception of individuals with unilateral hearing loss in speech perception with and without competitive noise, before and after the hearing aid fitting process. The study included 30 adults of both genders diagnosed with moderate or severe sensorineural unilateral hearing loss using the Hearing In Noise Test - Hearing In Noise Test-Brazil, in the following scenarios: silence, frontal noise, noise to the right, and noise to the left, before and after the hearing aid fitting process. The study participants had a mean age of 41.9 years and most of them presented right unilateral hearing loss. In all cases evaluated with Hearing In Noise Test, a better performance in speech perception was observed with the use of hearing aids. Using the Hearing In Noise Test-Brazil test evaluation, individuals with unilateral hearing loss demonstrated better performance in speech perception when using hearing aids, both in silence and in situations with a competing noise, with use of hearing aids. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Impact of unilateral conductive hearing loss due to aural atresia on academic performance in children.

PubMed

Kesser, Bradley W; Krook, Kaelyn; Gray, Lincoln C

2013-09-01

This study evaluates the effect of unilateral conductive hearing loss secondary to aural atresia on elementary school children's academic performance. Case control survey and review of audiometric data. One hundred thirty-two surveys were mailed to families of children with aural atresia, and 48 surveys were sent to families of children with unilateral sensorineural hearing loss (SNHL) to identify rates of grade retention, use of any resource, and behavioral problems. Audiometric data of the cohort were tabulated. Of the 40 atresia patients, none repeated a grade, but 65% needed some resources: 12.5% currently use a hearing aid, 32.5% use(d) a frequency-modulated system in school, 47.5% had an Individualized Education Plan, and 45% utilized speech therapy. Compared to the unilateral SNHL group and a cohort of children with unilateral SNHL in an earlier study, children with unilateral atresia were less likely to repeat a grade. Children in both unilateral atresia and SNHL groups were more likely to utilize some resource in the academic setting compared to the unilateral SNHL children in the prior study. Unilateral conductive hearing loss due to aural atresia has an impact on academic performance in children, although not as profound when compared to children with unilateral SNHL. The majority of these children with unilateral atresia utilize resources in the school setting. Parents, educators, and health care professionals should be aware of the impact of unilateral conductive hearing loss and offer appropriate habilitative services. Copyright © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

Jervell and Lange-Nielsen syndrome in a father and daughter from a large highly inbred family: a 16-year follow-up of 59 living members.

PubMed

Sanyal, Shyamal Kumar; Kaul, Kanwar K; Hussein, Akhtar; Wilroy, Robert S; Agarwal, Kisan; Sohel, Saira

2013-08-01

To report the autosomal dominant inheritance of the Jervell and Lange-Nielsen syndrome in a highly inbred family, the initiation of Torsades de Pointes, and the natural history of the syndrome based on a 16-year follow-up of the kindred. A family tree was constructed that included 66 blood relatives from three successive generations. Electrocardiograms were obtained from 59 living members including the proband, four members from a nuclear family, and 54 from the extended family. Evoked response audiometry was recorded for the proband and the nuclear family. All 59 family members were followed up regularly for 16 years. A total of 24 living members were affected--QTc: 480-680 ms. The proband had long QTc, bilateral high-tone sensorineural deafness, recurrent syncope, and Torsades de Pointes. The asymptomatic father had long QTc and unilateral high-tone sensorineural deafness that involved specifically the left ear. One asymptomatic sibling of the proband had long QTc and normal hearing. The mother and another sibling were asymptomatic; QTc and hearing were normal in both. A total of 21 affected members from the extended family had only long QTc, and all were asymptomatic. There were three congenitally deaf first cousins who had recurrent syncope and adrenergic-triggered sudden death. In all, seven of 10 parents had consanguineous marriage to a first cousin. Each affected offspring had at least one affected parent. The severely symptomatic proband who received only β-blocker therapy and the 23 affected members without antiadrenergic therapy, all remained asymptomatic throughout the 16-year follow-up period. Jervell and Lange-Nielsen syndrome was inherited as autosomal dominant in this kindred. The majority of the affected members had a mild phenotype. The severity of auditory and cardiac phenotypes corresponded.

Sudden sensorineural hearing loss with positional vertigo: Initial findings of positional nystagmus and hearing outcomes.

PubMed

Kim, Chang-Hee; Shin, Jung Eun; Yang, Young Soo; Im, Donghyuk

2016-10-01

To investigate the initial findings of positional nystagmus in patients with sudden sensorineural hearing loss (SSNHL) and positional vertigo, and to compare hearing improvement among patients with different types of positional nystagmus. The characteristics of positional nystagmus upon initial examination were analysed, and the initial mean pure-tone audiometry (PTA) threshold was compared with that at three months after treatment. Forty-four SSNHL patients with concomitant positional vertigo were included. Positional nystagmus was classified into five subgroups; persistent geotropic direction-changing positional nystagmus (DCPN) in head-roll test (HRT) and negative Dix-Hallpike test (DHT), persistent apogeotropic DCPN in HRT and negative DHT, positive DHT and negative HRT, persistent geotropic DCPN in HRT and positive DHT, and persistent apogeotropic DCPN in HRT and positive DHT. PTA threshold improvement was significantly greater in SSNHL patients with negative DHT than with positive DHT (p = 0.027). When geotropic DCPN was elicited by HRT, the nystagmus was persistent, which suggests that alteration of specific gravity of the endolymph, rather than the lateral canal canalolithiasis, may be a cause of this characteristic positional nystagmus. Positive DTH may be a prognostic factor for worse hearing recovery among patients with SSNHL and positional vertigo.

[Acute sensorineural hearing loss at the Otorhinolaryngology Department of the General Hospital in Subotica 1991-1996].

PubMed

Rakić, N

1999-01-01

Sudden sensorineural hearing loss is an acute hearing loss of a perceptive type, of unknown etiology, in most cases one-sided, or, a every sudden sensorineural deafness that occurs in the period of three days or less. Modern scientific literature points to three possible etiological factors: viral cochleitis, vascular lesion of the labyrinth and unrecognized rupture of the membranous labyrinth. The therapy for this disease is extremely various. There are dozens of reported different (or similar) healing protocols, including vasodilators, Hydroxyethyl starch, low molecular dextran, diuretics, defibrinogenation, fibrmolytic therapy, steroids, prostacyclin, oxygen therapy, hyperbaric oxygen therapy, vitamins, etc. The aim of the study was to recapitulate in brief modern views of etiology and therapy of the sudden sensorineural hearing loss, to analyze cases of this illness within the period of 1991-1996 in our Otorhinolaryngology Department and to compare these data with the same or similar data of other authors. In this study, medical records of patients admitted in the period of 1991-1996 in the Otorhinolaryngology Department of the Health Center in Subotica with a diagnosis of sudden sensorineural hearing loss were used. There were 53 patients with this diagnosis. All patients were treated in the hospital with infusions of rheoactive drugs. During the diagnostic procedure, all patients underwent a complete clinical otorhinolaryngologic examination, detailed anamnesis was taken, acoustic impedance tests (tympanometry) and tonal liminal audiometry were performed. Routine blood test was made (including complete blood picture, sedimentation rate, blood sugar) and a complete urine examination. Sometimes we demanded x-ray of the temporal bones (Schnller and Stenwers). A short increment sensitivity index (SISI) test and Carhart test were also made, if necessary. Function of the vestibular system was tested in cases when the vestibular symptoms were more strongly expressed. This test was made after passing an acute attack of vertiginous symptoms. Control audiometry was made on the seventh and on the fourteenth day after admission. This therapy consisted of an infusion of physiologic solution (NaCl) 250 or 500 ml twice daily to which 300 mg xanthinol nicotinate ampulla, paracetam 1 g ampulla, dipyridamole 10 mg ampulla and C-vitamin 10% 500 mg ampulla were applied. The number of paracetam and xanthinol nicotinate ampullas was raised on the 14/day, and then it fell to the starting value. The therapy lasted 17 days. Patients were given multivitamine peroral therapy, tranquilizers and diet without coffee, smoking and allergenic food. The study included 53 patients, 27 (50.94%) female and 26 (49.06%) males (Graph 1). Their age (Graph 2) ranged between 16-71 years. The biggest group of patients was between 40-49 (39.62%) years of age. Distribution per year (Graph 3) shows that the incidence of the acute sensorineural hearing loss differs from the findings of other authors, especially in the period of 1991-1994, due to very few cases. Connection of this illness with seasons is shown in Graph 4. We had most cases during summer time (August, 16.98%). There is a mild rise in winter (December, 13.21%). The degree of the hearing loss in decibels is shown in the Graph 5. Most of the patients (75.47%) had mild or severely damaged hearing (40-85 dB). Period of the deafness before patients were admitted to the hospital, is shown in Graph 7. Most of them came in the first seven days after they experienced acute deafness (52.83%). Symptomatology of the persons with acute hearing loss is shown in Table 1. All of them had the feeling of deafness, and 50.94% also had tinnitus. Table 2 shows that only two patients had a pathologic finding (hypo function) on the vestibular caloric test. 57.14% of patients tested by this method showed a normal function of the vestibular apparatus. The recovery of he

Pilot study of cognition in children with unilateral hearing loss.

PubMed

Ead, Banan; Hale, Sandra; DeAlwis, Duneesha; Lieu, Judith E C

2013-11-01

The objective of this study was to obtain preliminary data on the cognitive function of children with unilateral hearing loss in order to identify, quantify, and interpret differences in cognitive and language functions between children with unilateral hearing loss and with normal hearing. Fourteen children ages 9-14 years old (7 with severe-to-profound sensorineural unilateral hearing loss and 7 sibling controls with normal hearing) were administered five tests that assessed cognitive functions of working memory, processing speed, attention, and phonological processing. Mean composite scores for phonological processing were significantly lower for the group with unilateral hearing loss than for controls on one composite and four subtests. The unilateral hearing loss group trended toward worse performance on one additional composite and on two additional phonological processing subtests. The unilateral hearing loss group also performed worse than the control group on the complex letter span task. Analysis examining performance on the two levels of task difficulty revealed a significant main effect of task difficulty and an interaction between task difficulty and group. Cognitive function and phonological processing test results suggest two related deficits associated with unilateral hearing loss: (1) reduced accuracy and efficiency associated with phonological processing, and (2) impaired executive control function when engaged in maintaining verbal information in the face of processing incoming, irrelevant verbal information. These results provide a possible explanation for the educational difficulties experienced by children with unilateral hearing loss. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Brain activity in patients with unilateral sensorineural hearing loss during auditory perception in noisy environments.

PubMed

Yamamoto, Katsura; Tabei, Kenichi; Katsuyama, Narumi; Taira, Masato; Kitamura, Ken

2017-01-01

Patients with unilateral sensorineural hearing loss (UHL) often complain of hearing difficulties in noisy environments. To clarify this, we compared brain activation in patients with UHL with that of healthy participants during speech perception in a noisy environment, using functional magnetic resonance imaging (fMRI). A pure tone of 1 kHz, or 14 monosyllabic speech sounds at 65‒70 dB accompanied by MRI scan noise at 75 dB, were presented to both ears for 1 second each and participants were instructed to press a button when they could hear the pure tone or speech sound. Based on the activation areas of healthy participants, the primary auditory cortex, the anterior auditory association areas, and the posterior auditory association areas were set as regions of interest (ROI). In each of these regions, we compared brain activity between healthy participants and patients with UHL. The results revealed that patients with right-side UHL showed different brain activity in the right posterior auditory area during perception of pure tones versus monosyllables. Clinically, left-side and right-side UHL are not presently differentiated and are similarly diagnosed and treated; however, the results of this study suggest that a lateralityspecific treatment should be chosen.

Sudden bilateral sensorineural hearing loss after intravenous cocaine injection: a case report and review of the literature.

PubMed

Stenner, Markus; Stürmer, Konrad; Beutner, Dirk; Klussmann, Jens Peter

2009-12-01

Little is known about the effects of intravenous abuse of cocaine, especially on the inner ear. We report on a 26-year-old man who presented to our outpatient department with a sudden severe hearing loss after intravenous injection of cocaine. The audiogram on admission showed symmetric air conduction levels up to 80 dB at 4 kHz. After treatment with intravenous sodium chloride, prednisolone, and pentoxifylline, the audiogram 2 days later showed a bilateral normacusis. A review of the literature on the topic is given and possible reasons for inner ear damages caused by cocaine are discussed.

Sensorineural deafness following routine transurethral resection of the prostate

PubMed Central

Bowsher, Benjamin

2015-01-01

A man in his 50s presented to a rural Australian emergency department with complete unilateral hearing loss following transurethral resection of the prostate. His hearing impairment progressed from ‘muffled hearing’ with tinnitus on emergence from anaesthesia, to total sensorineural deafness by day three. His surgery and anaesthesia were uncomplicated and he had remained normotensive throughout. He had no pre-existing auditory disease. He had received 240 mg of intravenous gentamicin intraoperatively for surgical prophylaxis. Renal function was normal. Brain imaging was negative for structural pathology, stroke and circulatory insufficiency. Ear nose and throat advised 7 days of oral corticosteroids, transtympanic dexamethasone and hyperbaric oxygen therapy. A working diagnosis of gentamicin-induced ototoxicity was applied. Intervention has proven unsuccessful and there is no possibility for rehabilitation. The patient is permanently disabled. PMID:26564118

Epidemiological and clinical aspects of ear nose and throat sensorineural emergencies in the Yaoundé reference hospital.

PubMed

Djomou, François; Nkouo, Yves Christian Andjock; Mindja, Eko David; Nchinda, Choffor; Meka, Luc; Mbamyah-Lyonga, Emilia; Ndjolo, Alexis

2016-01-01

Sensorineural emergencies (SNE) are rare clinical situations. Few patients consult early explaining subsequent difficulty in having accurate data and management. Three clinical conditions are considered SNE in otolaryngology; they include sudden sensorineural hearing loss (SSHL), Bell's palsy and acute vertigo. There is very little data available on sensorineural emergencies in our setting. The aim of this study was to provide preliminary data on the management of Ear Nose and Throat (ENT) sensorineural emergency cases in Yaoundé Reference Hospital. A descriptive retrospective study was carried out based on data collected over a period of 5 years, January 2010 to July 2014 at the Yaoundé Reference Hospital. Information was obtained from patients' files collected from the archives of the institution. Patients presenting with SSHL, Bell's palsy, acute vertigo who consulted during the study period were included in the study. A total of 22 patients were included in the study out of 6406 patients who consulted at the ENT Unit. The prevalence of SNE in ENT consultations was 0.003, distributed as follows; 13 patients (59.1%) of SNE had Bell's palsy, seven (31.8%) had vestibular neuritis and two (9.1%) had SSHL. The prevalence of SNE was low with idiopathic Bell's palsy being the most frequent. There was a general delay in arrival of patients hence delay in diagnosis. This delay could equally be a factor for treatment failure and poor prognosis. More effort should be made in terms of population sensitization about the necessity of getting early medical attention.

Sensorineural hearing loss among cerebellopontine-angle tumor patients examined with pure tone audiometry and brainstem-evoked response audiometry

NASA Astrophysics Data System (ADS)

Rinindra, A. M.; Zizlavsky, S.; Bashiruddin, J.; Aman, R. A.; Wulani, V.; Bardosono, S.

2017-08-01

Tumor in the cerebellopontine angle (CPA) accurs for approximately 5-10% of all intracranial tumors, where unilateral hearing loss and tinnitus are the most frequent symptoms. This study aimed to collect data on sensorineural hearing loss in CPA tumor patients in Dr. Cipto Mangunkusumo Hospital (CMH) using pure tone audiometry and brainstem-evoked response audiometry (BERA). It also aimed to obtaine data on CPA-tumor imaging through magnetic resonance imaging (MRI). This was a descriptive, analytic, and cross-sectional study. The subjects of this study were gathered using a total sampling method from secondary data between July 2012 and November 2016. From 104 patients, 30 matched the inclusion criteria. The CPA-tumor patients in the ENT CMH outpatient clinic were mostly female, middle-aged patients (41-60 years) whose clinical presentation was mostly tinnitus and severe, asymmetric sensorineural hearing loss in 10 subjects. From 30 subjects, 29 showed ipsilaterally impaired BERA results, and 17 subjects showed contralaterally impaired BERA results. There were 24 subjects who with large-sized tumors and 19 subjects who had intracanal tumors that had spread until they were extracanal in 19 subjects.

Isolated Sensorineural Hearing Loss as a Sequela after Lightning Strike

PubMed Central

Turan, Mahfuz; Kalkan, Ferhat; Bozan, Nazım; Özçalimli, İsa; Zeki Erdem, Mehmet; Yalınkılıç, Abdülaziz; Garca, Mehmet Fatih

2015-01-01

In most of the surviving patients after a lightning strike, audiovestibular abnormalities have been reported. The most frequently reported type of abnormalities is a tympanic membrane perforation with hearing loss and external ear canal burn. However a sensor neural hearing loss and mixed type hearing loss can also occur, but these occur rarely. A nineteen-year-old female patient had, after a lightning strike, serious burns on the left ear, behind the ear, and on the chest and neck. She also had in her left ear 108 dB hearing loss with irregular central perforation and in her right ear 52 dB sensorineural hearing loss. There was no hearing loss before the strike. A hearing aid was recommended for the right ear and good care and follow-up were recommended for the left ear. A lightning strike can cause serious audiological damage. Therefore, it is necessary to make a careful audiovestibular evaluation of the patients. Although there exist rarely healed cases from sensorineural hearing loss after lightning strike in literature, in our case hearing loss occurred bilaterally and then it healed unilaterally. This condition is quite rare in literature. PMID:26161278

Comparison of 2 and 4 Intratympanic Steroid Injections in the Treatment of Idiopathic Sudden Sensorineural Hearing Loss.

PubMed

Suzuki, Hideaki; Wakasugi, Tetsuro; Kitamura, Takuro; Koizumi, Hiroki; Do, Ba Hung; Ohbuchi, Toyoaki

2018-04-01

We studied the effect of intratympanic steroid administration with different total injection times on hearing outcomes in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). The subjects were 191 consecutive patients (192 ears) with ISSNHL (hearing level ≥40 dB, interval between onset and treatment ≤30 days). They received systemic prednisolone (100 mg followed by tapered doses) combined with intratympanic injection of dexamethasone (4 mg/ml). Intratympanic injection was performed 4 times (days 1, 2, 4, and 7) in 92 patients (92 ears) or 2 times (days 1 and 2) in 99 patients (100 ears). The hearing outcomes were evaluated at 1 week from the start of treatment and 1 to 2 months after the completion of treatment. There was no significant difference in hearing outcomes between the 4- and 2-injection groups at either time point. Multiple regression analysis also showed that the hearing level after treatment did not depend on the total number of intratympanic steroid injections. These results indicate that a protocol using only 2 intratympanic steroid injections exerts a sufficient effect on the hearing outcomes of ISSNHL. This simplified treatment protocol would be greatly beneficial to relieve the physical and mental stress of patients.

Sudden sensorineural hearing loss: is there a relationship between routine haematological parameters and audiogram shapes?

PubMed

Salvago, Pietro; Rizzo, Serena; Bianco, Antonino; Martines, Francesco

2017-03-01

To investigate the relationship between haematological routine parameters and audiogram shapes in patients affected by sudden sensorineural hearing loss (SSNHL). A retrospective study. All patients were divided into four groups according to the audiometric curve and mean values of haematological parameters (haemoglobin, white blood cell, neutrophils and lymphocytes relative count, platelet count, haematocrit, prothrombin time, activated partial thromboplastin time, fibrinogen and neutrophil-to-lymphocite ratio) of each group were statistically compared. The prognostic role of blood profile and coagulation test was also examined. A cohort of 183 SSNHL patients without comorbidities. With a 48.78% of complete hearing recovery, individuals affected by upsloping hearing loss presented a better prognosis instead of flat (18.36%), downsloping (19.23%) and anacusis (2.45%) groups (p = 0.0001). The multivariate analysis of complete blood count values revealed lower mean percentage of lymphocytes (p = 0.041) and higher platelet levels (p = 0.015) in case of downsloping hearing loss; with the exception of fibrinogen (p = 0.041), none of the main haematological parameters studied resulted associated with poorer prognosis. Our work suggested a lack of association between haematological parameters and a defined audiometric picture in SSNHL patients; furthermore, only fibrinogen seems to influence the prognosis of this disease.

The current status of audiologic rehabilitation for profound unilateral sensorineural hearing loss.

PubMed

Bishop, Charles E; Eby, Thomas L

2010-03-01

Audiologic rehabilitation of individuals with profound unilateral sensorineural hearing loss (USNHL) has traditionally been limited to the use of air-conduction contralateral routing of sound (CROS) hearing aids. Treatment for these individuals has expanded with new applications of the bone-anchored hearing aid (BAHA), transcranial hearing aid (t-CROS), and the cochlear implant. In this article, the authors review the literature that addresses these various treatment options. Contemporary review Historical information is available that describes the limited efficacy of air-conduction CROS hearing aids in lifting hearing handicap associated with USNHL. Current investigations on providing cross hearing are generally focused on use of the BAHA. Little is known at present whether new developments in hearing aid technology can improve on conventional air-conduction CROS or t-CROS approaches. Interestingly, the cochlear implant seems to be a viable option for individuals with USNHL and tinnitus who also have intact auditory nerve pathways. There is indication in the literature that BAHA provides greater relief of hearing handicap associated with USNHL than CROS hearing aids; however, both have been found to provide limited patient satisfaction and seemingly fall short of restoring true sound localization. Adequate trials have not been performed comparing BAHA with the best CROS hearing aid technology. Transcranial hearing aids and cochlear implants are experimental methods to treat USNHL and hold promise, although there remains a lack of studies available to fully support this.

Sudden sensorineural hearing loss: results of intratympanic steroids as salvage treatment.

PubMed

Dispenza, Francesco; De Stefano, Alessandro; Costantino, Claudio; Marchese, Donatella; Riggio, Francesco

2013-01-01

The aim of the present study was to verify the efficacy and the safety of intratympanic dexamethasone to treat sudden sensorineural hearing loss as salvage therapy. A prospective study was conducted on patients affected by idiopathic sudden hearing loss who were treated before with some systemic therapy, but without recovery of the hearing The patients able to undergo the study, but who refused salvage treatment were considered as control group. A solution of Dexamethasone 4 mg/ml was then injected through the posterior-inferior quadrant filling completely the middle ear. The follow-up in the following 6 months included an audiogram every month. The number of patients treated with salvage therapy was 36. The patients who refused treatment were further 10. The salvage treatment was done with a mean delay of 24.3 days from the onset of symptoms. Mean hearing threshold after the onset of sudden hearing loss at PTA was 66.5 dB. After the failed treatment the mean PTA was 59.6 dB. The mean PTA after the intratympanic steroid administration was 46.8 dB, with a mean improvement of 12.8 dB. No hearing change was noted in the 10 patients who refused salvage therapy. The patients that assumed systemic steroid as first therapy showed a better PTA threshold after the salvage intratympanic treatment (p<0.01). A significant difference (p<0.05) of hearing recovery was evidenced between non-smoker patients and those with smoking habit. Our data showed that a salvage treatment with intratympanic dexamethasone should be suggested to all patients who failed the first systemic treatment. The systemic steroid therapy done before the salvage treatment seems to exert a protective role for the inner ear, as shown by our series. On the contrary the smoke habit is a negative prognostic factor in the hearing recovery. Copyright © 2013 Elsevier Inc. All rights reserved.

Persistent Thalamic Sound Processing Despite Profound Cochlear Denervation.

PubMed

Chambers, Anna R; Salazar, Juan J; Polley, Daniel B

2016-01-01

Neurons at higher stages of sensory processing can partially compensate for a sudden drop in peripheral input through a homeostatic plasticity process that increases the gain on weak afferent inputs. Even after a profound unilateral auditory neuropathy where >95% of afferent synapses between auditory nerve fibers and inner hair cells have been eliminated with ouabain, central gain can restore cortical processing and perceptual detection of basic sounds delivered to the denervated ear. In this model of profound auditory neuropathy, auditory cortex (ACtx) processing and perception recover despite the absence of an auditory brainstem response (ABR) or brainstem acoustic reflexes, and only a partial recovery of sound processing at the level of the inferior colliculus (IC), an auditory midbrain nucleus. In this study, we induced a profound cochlear neuropathy with ouabain and asked whether central gain enabled a compensatory plasticity in the auditory thalamus comparable to the full recovery of function previously observed in the ACtx, the partial recovery observed in the IC, or something different entirely. Unilateral ouabain treatment in adult mice effectively eliminated the ABR, yet robust sound-evoked activity persisted in a minority of units recorded from the contralateral medial geniculate body (MGB) of awake mice. Sound driven MGB units could decode moderate and high-intensity sounds with accuracies comparable to sham-treated control mice, but low-intensity classification was near chance. Pure tone receptive fields and synchronization to broadband pulse trains also persisted, albeit with significantly reduced quality and precision, respectively. MGB decoding of temporally modulated pulse trains and speech tokens were both greatly impaired in ouabain-treated mice. Taken together, the absence of an ABR belied a persistent auditory processing at the level of the MGB that was likely enabled through increased central gain. Compensatory plasticity at the level of the auditory thalamus was less robust overall than previous observations in cortex or midbrain. Hierarchical differences in compensatory plasticity following sensorineural hearing loss may reflect differences in GABA circuit organization within the MGB, as compared to the ACtx or IC.

Systemic Polyarteritis Nodosa as the Cause of Sudden Onset Bilateral Sensorineural Hearing Loss Following Lassa Virus Infection

DTIC Science & Technology

2016-07-05

occlusion of the anterior inferior cerebellar artery (AICA) and downstream vessels leading to cochlear hypoxia (18-24). Relevant to this work, PAN has...macaques showed moderate lymphoplasmacytic to chronic-active perivascular inflammation of the inner ear adjacent to the cochlear nerve The...inflammation occasionally surrounded smaller branches of the cochlear nerve, resembling pathological changes seen in humans diagnosed with PAN (Fig. 3B-E) (21

Neuromagnetic Index of Hemispheric Asymmetry Prognosticating the Outcome of Sudden Hearing Loss

PubMed Central

Li, Lieber Po-Hung; Shiao, An-Suey; Chen, Kuang-Chao; Lee, Po-Lei; Niddam, David M.; Chang, Shyue-Yih; Hsieh, Jen-Chuen

2012-01-01

The longitudinal relationship between central plastic changes and clinical presentations of peripheral hearing impairment remains unknown. Previously, we reported a unique plastic pattern of “healthy-side dominance” in acute unilateral idiopathic sudden sensorineural hearing loss (ISSNHL). This study aimed to explore whether such hemispheric asymmetry bears any prognostic relevance to ISSNHL along the disease course. Using magnetoencephalography (MEG), inter-hemispheric differences in peak dipole amplitude and latency of N100m to monaural tones were evaluated in 21 controls and 21 ISSNHL patients at two stages: initial and fixed stage (1 month later). Dynamics/Prognostication of hemispheric asymmetry were assessed by the interplay between hearing level/hearing gain and ipsilateral/contralateral ratio (I/C) of N100m latency and amplitude. Healthy-side dominance of N100m amplitude was observed in ISSNHL initially. The pattern changed with disease process. There is a strong correlation between the hearing level at the fixed stage and initial I/Camplitude on affected-ear stimulation in ISSNHL. The optimal cut-off value with the best prognostication effect for the hearing improvement at the fixed stage was an initial I/Clatency on affected-ear stimulation of 1.34 (between subgroups of complete and partial recovery) and an initial I/Clatency on healthy-ear stimulation of 0.76 (between subgroups of partial and no recovery), respectively. This study suggested that a dynamic process of central auditory plasticity can be induced by peripheral lesions. The hemispheric asymmetry at the initial stage bears an excellent prognostic potential for the treatment outcomes and hearing level at the fixed stage in ISSNHL. Our study demonstrated that such brain signature of central auditory plasticity in terms of both N100m latency and amplitude at defined time can serve as a prognostication predictor for ISSNHL. Further studies are needed to explore the long-term temporal scenario of auditory hemispheric asymmetry and to get better psychoacoustic correlates of pathological hemispheric asymmetry in ISSNHL. PMID:22532839

Incidence of tympanic membrane perforation after intratympanic steroid treatment through myringotomy tubes.

PubMed

Rutt, Amy L; Hawkshaw, Mary J; Sataloff, Robert T

2011-04-01

Intratympanic (IT) steroids are often used to treat inner ear disorders such as sudden idiopathic sensorineural hearing loss, autoimmune inner ear disease, and Ménière disease. Administration of corticosteroids via IT injection, via application with a pledget to the round window, or via catheter has been used for this purpose. The frequency of adverse events related to the IT injection of steroids is low, with pain, short-lasting vertigo, otitis media, and tympanic perforations being the most common complications. However, the safety of IT steroid therapy has not yet been established in a randomized clinical trial. In this article, we discuss a group of 11 patients with sensorineural hearing loss who underwent myringotomy and tube placement for home-based dexamethasone instillation and subsequently developed the complication of tympanic membrane perforation. It appears that there is a significantly increased incidence of tympanic membrane perforations in this population.

Diffusion Tensor Imaging of Central Auditory Pathways in Patients with Sensorineural Hearing Loss: A Systematic Review.

PubMed

Tarabichi, Osama; Kozin, Elliott D; Kanumuri, Vivek V; Barber, Samuel; Ghosh, Satra; Sitek, Kevin R; Reinshagen, Katherine; Herrmann, Barbara; Remenschneider, Aaron K; Lee, Daniel J

2018-03-01

Objective The radiologic evaluation of patients with hearing loss includes computed tomography and magnetic resonance imaging (MRI) to highlight temporal bone and cochlear nerve anatomy. The central auditory pathways are often not studied for routine clinical evaluation. Diffusion tensor imaging (DTI) is an emerging MRI-based modality that can reveal microstructural changes in white matter. In this systematic review, we summarize the value of DTI in the detection of structural changes of the central auditory pathways in patients with sensorineural hearing loss. Data Sources PubMed, Embase, and Cochrane. Review Methods We used the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement checklist for study design. All studies that included at least 1 sensorineural hearing loss patient with DTI outcome data were included. Results After inclusion and exclusion criteria were met, 20 articles were analyzed. Patients with bilateral hearing loss comprised 60.8% of all subjects. Patients with unilateral or progressive hearing loss and tinnitus made up the remaining studies. The auditory cortex and inferior colliculus (IC) were the most commonly studied regions using DTI, and most cases were found to have changes in diffusion metrics, such as fractional anisotropy, compared to normal hearing controls. Detectable changes in other auditory regions were reported, but there was a higher degree of variability. Conclusion White matter changes based on DTI metrics can be seen in patients with sensorineural hearing loss, but studies are few in number with modest sample sizes. Further standardization of DTI using a prospective study design with larger sample sizes is needed.

Effect of Antioxidant Vitamins as Adjuvant Therapy for Sudden Sensorineural Hearing Loss: Systematic Review Study.

PubMed

Ibrahim, Iman; Zeitouni, Anthony; da Silva, Sabrina Daniela

2018-06-22

Sudden sensorineural hearing loss (SSNHL) is an otological emergency of unknown etiology. Recent reports showed that antioxidant drugs can benefit patients with SSNHL. This study attempted to evaluate the effect of adding antioxidant vitamins as an adjuvant therapy alongside with corticosteroids. To evaluate the effects of the 3 major antioxidant vitamins (A, C, and E) as an adjuvant therapy, administered with corticosteroids, for the treatment of SSNHL in adult patients (≥18 years). MEDLINE, EMBASE, PubMed, Web of Science and Cochrane electronic databases from January 1, 1995, through September 25, 2017. Published studies of adult patients who received antioxidant vitamins (A, C, E, or any combination of these vitamins) as an adjuvant therapy in addition to the regular treatment (corticosteroids) for SSNHL. Quality assessment was performed using the Cochrane Collaboration Tool for Assessing Risk of Bias. Each study had a control group (conventional treatment + placebo) and a trial group (antioxidant vitamin(s) + conventional treatment). From 446 manuscripts identified in the literature, 3 studies were included in the review with 279 patients. The most common vitamins used to treat SSNHL were the 3 major antioxidant vitamins A, C, and E, combined sometimes with other antioxidants such as selenium. The success of the treatment is increased in patients who received antioxidant vitamins in combination with conventional therapy. © 2018 S. Karger AG, Basel.

Prediction of hearing outcomes by multiple regression analysis in patients with idiopathic sudden sensorineural hearing loss.

PubMed

Suzuki, Hideaki; Tabata, Takahisa; Koizumi, Hiroki; Hohchi, Nobusuke; Takeuchi, Shoko; Kitamura, Takuro; Fujino, Yoshihisa; Ohbuchi, Toyoaki

2014-12-01

This study aimed to create a multiple regression model for predicting hearing outcomes of idiopathic sudden sensorineural hearing loss (ISSNHL). The participants were 205 consecutive patients (205 ears) with ISSNHL (hearing level ≥ 40 dB, interval between onset and treatment ≤ 30 days). They received systemic steroid administration combined with intratympanic steroid injection. Data were examined by simple and multiple regression analyses. Three hearing indices (percentage hearing improvement, hearing gain, and posttreatment hearing level [HLpost]) and 7 prognostic factors (age, days from onset to treatment, initial hearing level, initial hearing level at low frequencies, initial hearing level at high frequencies, presence of vertigo, and contralateral hearing level) were included in the multiple regression analysis as dependent and explanatory variables, respectively. In the simple regression analysis, the percentage hearing improvement, hearing gain, and HLpost showed significant correlation with 2, 5, and 6 of the 7 prognostic factors, respectively. The multiple correlation coefficients were 0.396, 0.503, and 0.714 for the percentage hearing improvement, hearing gain, and HLpost, respectively. Predicted values of HLpost calculated by the multiple regression equation were reliable with 70% probability with a 40-dB-width prediction interval. Prediction of HLpost by the multiple regression model may be useful to estimate the hearing prognosis of ISSNHL. © The Author(s) 2014.

Delayed restoration of maximum speech discrimination scores in patients with idiopathic sudden sensorineural hearing loss.

PubMed

Noguchi, Yoshihiro; Takahashi, Masatoki; Ito, Taku; Fujikawa, Taro; Kawashima, Yoshiyuki; Kitamura, Ken

2016-10-01

To assess possible delayed recovery of the maximum speech discrimination score (SDS) when the audiometric threshold ceases to change. We retrospectively examined 20 patients with idiopathic sudden sensorineural hearing loss (ISSNHL) (gender: 9 males and 11 females, age: 24-71 years). The findings of pure-tone average (PTA), maximum SDS, auditory brainstem responses (ABRs), and tinnitus handicap inventory (THI) were compared among the three periods of 1-3 months, 6-8 months, and 11-13 months after ISSNHL onset. No significant differences were noted in PTA, whereas an increase of greater than or equal to 10% in maximum SDS was recognized in 9 patients (45%) from the period of 1-3 months to the period of 11-13 months. Four of the 9 patients showed 20% or more recovery of maximum SDS. No significant differences were observed in the interpeak latency difference between waves I and V and the interaural latency difference of wave V in ABRs, whereas an improvement in the THI grade was recognized in 11 patients (55%) from the period of 1-3 months to the period of 11-13 months. The present study suggested the incidence of maximum SDS restoration over 1 year after ISSNHL onset. These findings may be because of the effects of auditory plasticity via the central auditory pathway. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Secondhand smoke and sensorineural hearing loss in adolescents.

PubMed

Lalwani, Anil K; Liu, Ying-Hua; Weitzman, Michael

2011-07-01

To investigate the hypothesis that second-hand smoke (SHS) exposure is associated with sensorineural hearing loss (SNHL) in adolescents. A complex, multistage, stratified geographic area design for collecting representative data from the noninstitutionalized US population. Cross-sectional data from National Health and Nutrition Examination Survey (2005-2006) were available for 1533 participants 12 to 19 years of age who underwent audiometric testing, had serum cotinine levels available, and were not actively smoking. SNHL was defined as an average pure-tone level greater than 15 dB for 0.5, 1, and 2 kHz (low frequency) and 3, 4, 6, and 8 kHz (high frequency). Secondhand smoke exposure, as assessed by serum cotinine levels, was associated with elevated pure-tone hearing thresholds at 2, 3, and 4 kHz, a higher rate of unilateral low-frequency SNHL (11.8% vs 7.5%; P < .04), and a 1.83-fold increased risk of unilateral low-frequency SNHL in multivariate analyses (95% confidence interval, 1.08-3.41). The prevalence of SNHL was directly related to level of SHS exposure as reflected by serum cotinine levels. In addition, nearly 82% of adolescents with SNHL did not recognize hearing difficulties. Secondhand smoke is associated with elevated pure-tone thresholds and an increased prevalence of low-frequency SNHL that is directly related to level of exposure, and most affected individuals are unaware of the hearing loss. Thus, adolescents exposed to SHS may need to be closely monitored for early hearing loss with periodic audiologic testing.

[Combined treatment supported by piracetam and/or acyclovir in idiopathic sudden sensorineural hearing loss: experience with 81 cases].

PubMed

Karakurt, Süleyman Emre; Ozkul, Mehmet Doğan; Cukurova, Ibrahim; Demirhan, Erhan; Yiğitbaşi, Orhan Gazi

2009-01-01

To investigate the efficiency of piracetam and acyclovir in treating sudden hearing loss. Eightyone patients (44 males, 37 females; mean age 40.4 year; range 18 to 72 years) who had treatment between January 2002 and December 2006 with diagnosis of idiopathic sudden hearing loss were evaluated retrospectively. These patients were divided into four groups according to the treatment they received. The patients who had combined treatment constituted the first group; those who had combined treatment and piracetam the second; those who had combined treatment and acyclovir the third; those who had combined treatment, acyclovir, and piracetam the fourth group. For the four treatment groups, in the pre-and post-treatment (10th day) evaluation of the treatment efficiency made by calculation of the hearing thresholds in 250-8000 Hz frequencies, no significant difference between the groups was determined (p>0.05). No additional benefit was obtained with acyclovir and piracetam in treatment.

Initial experience with the Sophono Alpha 1 osseointegrated implant.

PubMed

Escorihuela-García, Vicente; Llópez-Carratalá, Ignacio; Pitarch-Ribas, Ignacia; Latorre-Monteagudo, Emilia; Marco-Algarra, Jaime

2014-01-01

In the last several years, bone anchored hearing aids have proven to be useful in treating conductive and mixed unilateral or bilateral hearing loss, as well as for sensorineural unilateral hearing loss. The Sophono Alpha 1 model has the advantage of not requiring an abutment, with it being coupled by magnetism instead. We report the cases of 3 infants with congenital malformations of external and middle ear. Audiometry showed conductive hearing loss. All 3 patients were implanted with Alpha 1 model (Sophono). Patients evolved satisfactorily. After 30 days we applied the processor and the control audiometry showed a marked improvement of hearing thresholds, although without a complete closure of the gap. With minimal care, the skin over the implant remained in excellent condition, with a very satisfactory cosmetic outcome. Copyright © 2013 Elsevier España, S.L.U. y Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

Effect of edaravone on acute brainstem-cerebellar infarction with vertigo and sudden hearing loss.

PubMed

Inoue, Yuta; Yabe, Takao; Okada, Kazunari; Nakamura, Yuka

2014-06-01

We report 2 cases with acute brainstem and brainstem-cerebellar infarction showed improvement of their signs and symptoms after administration of edaravone. Case 1, a 74-year-old woman who experienced sudden vertigo, also had dysarthria and left hemiplegia. Magnetic resonance imaging (MRI) showed an abnormal region in the right ventrolateral medulla oblongata. The patient's vertigo and hemiplegia improved completely after treatment. Case 2, a 50-year-old man who experienced sudden vertigo and sensorineural hearing loss (SNHL), developed dysarthria after admission. MRI revealed acute infarction in the right cerebellar hemisphere. Magnetic resonance angiography revealed dissection of the basilar artery and occlusion of the right anterior inferior cerebellar artery. The patient's vertigo and hearing remarkably improved. We have described 2 patients whose early symptoms were vertigo and sudden SNHL, but who were later shown to have ischemic lesions of the central nervous system. Edaravone is neuroprotective drug with free radical-scavenging actions. Free radicals in the ear are responsible for ischemic damage. Edaravone, a free radical scavenger, may be useful in the treatment of vertigo and SNHL. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Ear diseases among secondary school students in Xi'an, China: the role of portable audio device use, insomnia and academic stress.

PubMed

Chen, Yang; Li, Xu; Xu, Zhan; Li, Zonghua; Zhang, Pengzhi; He, Ya; Wang, Fangyuan; Qiu, Jianhua

2011-06-08

Hearing impairment negatively impacts students' development of academic, language and social skills. Even minimal unilateral hearing loss can hinder educational performance. We investigated the prevalence of ear diseases among secondary school students in the city of Xi'an, China in order to provide a foundation for evidence-based hearing healthcare. A stratified random sampling survey was conducted in 29 secondary schools. Demographics and medical histories were collected, and otologic examinations were performed. Questionnaires were administered to assess insomnia, academic stress and use of portable audio devices. Logistic regression analysis was used to identify factors associated with hearing impairment, and the association of sensorineural hearing loss with insomnia, academic stress and the use of portable audio devices was analyzed with the chi-square test. The percentage of students with some form of ear disease was 3.32%. External ear disease, middle ear disease and sensorineural hearing loss occurred in 1.21%, 0.64% and 1.47% of the students, respectively. Boys had a relatively higher prevalence of ear disease than girls. According to our survey, the prevalence of sensorineural hearing loss increased significantly among the students with insomnia and extended use of portable audio devices, but not among those with elevated levels of academic stress. Hearing aids and surgical treatment were needed in 1.47% and 0.89% of the students, respectively. There is a high prevalence of ear disease among secondary school students, and this should be given more attention. Insomnia and the excessive use of portable audio devices may be related to adolescent sensorineural hearing loss. It is important to establish and comply with an evidence-based preventive strategy.

[Subclinical sensorineural hearing loss in female patients with rheumatoid arthritis].

PubMed

Treviño-González, José Luis; Villegas-González, Mario Jesús; Muñoz-Maldonado, Gerardo Enrique; Montero-Cantu, Carlos Alberto; Nava-Zavala, Arnulfo Hernán; Garza-Elizondo, Mario Alberto

2015-01-01

The rheumatoid arthritis is a clinical entity capable to cause hearing impairment that can be diagnosed promptly with high frequencies audiometry. To detect subclinical sensorineural hearing loss in patients with rheumatoid arthritis. Cross-sectional study on patients with rheumatoid arthritis performing high frequency audiometry 125Hz to 16,000Hz and tympanometry. The results were correlated with markers of disease activity and response to therapy. High frequency audiometry was performed in 117 female patients aged from 19 to 65 years. Sensorineural hearing loss was observed at a sensitivity of pure tones from 125 to 8,000 Hz in 43.59%, a tone threshold of 10,000 to 16,000Hz in 94.02% patients in the right ear and in 95.73% in the left ear. Hearing was normal in 8 (6.84%) patients. Hearing loss was observed in 109 (93.16%), and was asymmetric in 36 (30.77%), symmetric in 73 (62.37%), bilateral in 107 (91.45%), unilateral in 2 (1.71%), and no conduction and/or mixed hearing loss was encountered. Eight (6.83%) patients presented vertigo, 24 (20.51%) tinnitus. Tympanogram type A presented in 88.90% in the right ear and 91.46% in the left ear, with 5.98 to 10.25% type As. Stapedius reflex was present in 75.3 to 85.2%. Speech discrimination in the left ear was significantly different (p = 0.02)in the group older than 50 years. No association was found regarding markers of disease activity, but there was an association with the onset of rheumatoid arthritis disease. Patients with rheumatoid arthritis had a high prevalence of sensorineural hearing loss for high and very high frequencies. Copyright © 2015 Academia Mexicana de Cirugía A.C. Published by Masson Doyma México S.A. All rights reserved.

Altered Expression of Middle and Inner Ear Cytokines in Mouse Otitis Media

PubMed Central

MacArthur, Carol J.; Pillers, De-Ann M.; Pang, Jiaqing; Kempton, J. Beth; Trune, Dennis R.

2010-01-01

Objectives/Hypothesis The inner ear is at risk for sensorineural hearing loss in both acute and chronic otitis media (OM), but the underlying mechanisms underlying sensorineural hearing loss are unknown. Previous gene expression array studies showed cytokine genes might be upregulated in the cochleas of mice with acute and chronic otitis media. This implies that the inner ear could manifest a direct inflammatory response to OM that may cause sensorineural damage. Therefore, to better understand inner ear cytokine gene expression during OM, quantitative RT-PCR and immunohistochemistry were performed on mouse models to evaluate middle and inner ear inflammatory and remodeling cytokines. Study Design Basic science experiment. Methods An acute OM model was created in Balb/c mice by a transtympanic injection of S. pneumoniae in one ear; the other ear used as a control. C3H/HeJ mice were screened for unilateral chronic OM with the non-infected ear serving as control. Results Both acute and chronic OM caused both the middle ear and inner tissues in these two mouse models to over express numerous cytokine genes related to tissue remodeling (TNFα, FGF, BMP) and angiogenesis (VEGF), as well as inflammatory cell proliferation (IL-1α,β, IL-2, IL-6). Immunohistochemistry confirmed that both the middle ear and inner ear tissues expressed these cytokines. Conclusion Cochlear tissues are capable of expressing cytokine mRNA that contributes to the inflammation and remodeling that occur in association with middle ear disease. This provides a potential molecular basis for the transient and permanent sensorineural hearing loss often reported with acute and chronic OM. Level of Evidence N/A PMID:21271590

A software tool for pure‑tone audiometry. Classification of audiograms for inclusion of patients in clinical trials. English version.

PubMed

Rahne, T; Buthut, F; Plößl, S; Plontke, S K

2016-03-01

Selecting subjects for clinical trials on hearing loss therapies relies on the patient meeting the audiological inclusion criteria. In studies on the treatment of idiopathic sudden sensorineural hearing loss, the patient's acute audiogram is usually compared with a previous audiogram, the audiogram of the non-affected ear, or a normal audiogram according to an ISO standard. Generally, many more patients are screened than actually fulfill the particular inclusion criteria. The inclusion criteria often require a calculation of pure-tone averages, selection of the most affected frequencies, and calculation of hearing loss differences. A software tool was developed to simplify and accelerate this inclusion procedure for investigators to estimate the possible recruitment rate during the planning phase of a clinical trial and during the actual study. This tool is Microsoft Excel-based and easy to modify to meet the particular inclusion criteria of a specific clinical trial. The tool was retrospectively evaluated on 100 patients with acute hearing loss comparing the times for classifying automatically and manually. The study sample comprised 100 patients with idiopathic sudden sensorineural hearing loss. The age- and sex-related normative audiogram was calculated automatically by the tool and the hearing impairment was graded. The estimated recruitment rate of our sample was quickly calculated. Information about meeting the inclusion criteria was provided instantaneously. A significant reduction of 30 % in the time required for classifying (30 s per patient) was observed.

Sudden sensorineural hearing loss: subclinical viral and toxoplasmosis infections as aetiology and how they alter the clinical course.

PubMed

Kikidis, Dimitrios; Nikolopoulos, Thomas P; Kampessis, Georgios; Stamatiou, Georgios; Chrysovergis, Aristeidis

2011-01-01

To explore in a prospective study the evidence of certain viral and toxoplasmosis infections in sudden sensorineural hearing loss (SSHL). 84 consecutive patients with SSHL meeting certain criteria. All patients were assessed for specific IgM antibodies against cytomegalovirus, herpes simplex virus, toxoplasma and Epstein-Barr virus. All were treated with intravenous steroids and assigned to two groups: 76 IgM negative (NV group) and 8 IgM positive (no history of acute infection - V group). The mean hearing level at presentation was 86.5 dB HL (median, 100) in the V group and 60.7 dB HL (median, 61) in the NV group. The difference was statistically significant (p = 0.003). The mean hearing level following treatment was 81.8 dB HL (median, 88) in the V group and 48.7 dB HL (median, 39) in the NV group. The difference was statistically significant (p = 0.004). There was a considerable improvement in hearing after treatment only in the NV group (p < 0.000001). Recent subclinical viral or toxoplasmosis infections may be involved in the pathogenesis of SSHL (in approx. 10% of cases), suggesting that SSHL is not a single disease. When certain viruses or toxoplasmoses are involved, the hearing is much worse in comparison to patients with no such indication of infection. An alteration in treatment dosage or method of steroid administration may be needed in such cases. Copyright © 2011 S. Karger AG, Basel.

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

PubMed Central

Sekiya, Kenichi; Fukushima, Munehisa; Teismann, Henning; Lagemann, Lothar; Kakigi, Ryusuke; Pantev, Christo; Okamoto, Hidehiko

2016-01-01

Sudden sensorineural hearing loss (SSHL) is characterized by acute, idiopathic hearing loss. The estimated incidence rate is 5-30 cases per 100,000 people per year. The causes of SSHL and the mechanisms underlying SSHL currently remain unknown. Based on several hypotheses such as a circulatory disturbance to the cochlea, viral infection, and autoimmune disease, pharmaco-therapeutic approaches have been applied to treat SSHL patients; however, the efficacy of the standard treatment, corticosteroid therapy, is still under debate. Exposure to intense sounds has been shown to cause permanent damage to the auditory system; however, exposure to a moderate level enriched acoustic environment after noise trauma may reduce hearing impairments. Several neuroimaging studies recently suggested that the onset of SSHL induced maladaptive cortical reorganization in the human auditory cortex, and that the degree of cortical reorganization in the acute SSHL phase negatively correlated with the recovery rate from hearing loss. This article reports the development of a novel neuro-rehabilitation approach for SSHL, "constraint-induced sound therapy (CIST)". The aim of the CIST protocol is to prevent or reduce maladaptive cortical reorganization by using an enriched acoustic environment. The canal of the intact ear of SSHL patients is plugged in order to motivate them to actively use the affected ear and thereby prevent progress of maladaptive cortical reorganization. The affected ear is also exposed to music via a headphone for 6 hr per day during hospitalization. The CIST protocol appears to be a safe, easy, inexpensive, and effective treatment for SSHL. PMID:26863274

Utility of anti-HSP 70, TNF-alpha, ESR, antinuclear antibody, and antiphospholipid antibodies in the diagnosis and treatment of sudden sensorineural hearing loss.

PubMed

Süslü, Nilda; Yilmaz, Taner; Gürsel, Bülent

2009-02-01

To investigate the performance of various laboratory tests used for patients with sudden sensorineural hearing loss (SSNHL). Prospective clinical trial. Thirty patients who presented with SSNHL and 30 healthy people with no cochleovestibular disorders were selected as study and control groups. The laboratory panel includes the following tests: anti-HSP 70 antibody immunoassay, tumor necrosis factor-alpha (TNF-alpha), erythrocyte sedimentation rate (ESR), antinuclear antibody (ANA), and antiphospholipid antibodies. The study group was given corticosteroid therapy and separated into two groups: the corticosteroid responders and the corticosteroid nonresponders. In the follow-up, repeat audiograms were evaluated to determine the response to treatment. TNF-alpha was found at lower titers in the study group when compared with the control group in contrast to other studies. Also, anti-HSP 70 was not found in high titers in the study group. ANA and ESR were the two parameters that were significantly more positive in the study group compared with the control group. Because of the lack of association between a positive test and response to corticosteroid treatment, detection of the anti-HSP 70 antibody, TNF-alpha, ESR, and ANA, at present, do not offer clinically useful information in the treatment of SSNHL. Also, because of the lower titers of TNF-alpha documented in patients with SSNHL, we do not recommend the use of specific TNF-alpha inhibitors in SSNHL.

Changes of the directional brain networks related with brain plasticity in patients with long-term unilateral sensorineural hearing loss.

PubMed

Zhang, G-Y; Yang, M; Liu, B; Huang, Z-C; Li, J; Chen, J-Y; Chen, H; Zhang, P-P; Liu, L-J; Wang, J; Teng, G-J

2016-01-28

Previous studies often report that early auditory deprivation or congenital deafness contributes to cross-modal reorganization in the auditory-deprived cortex, and this cross-modal reorganization limits clinical benefit from cochlear prosthetics. However, there are inconsistencies among study results on cortical reorganization in those subjects with long-term unilateral sensorineural hearing loss (USNHL). It is also unclear whether there exists a similar cross-modal plasticity of the auditory cortex for acquired monaural deafness and early or congenital deafness. To address this issue, we constructed the directional brain functional networks based on entropy connectivity of resting-state functional MRI and researched changes of the networks. Thirty-four long-term USNHL individuals and seventeen normally hearing individuals participated in the test, and all USNHL patients had acquired deafness. We found that certain brain regions of the sensorimotor and visual networks presented enhanced synchronous output entropy connectivity with the left primary auditory cortex in the left long-term USNHL individuals as compared with normally hearing individuals. Especially, the left USNHL showed more significant changes of entropy connectivity than the right USNHL. No significant plastic changes were observed in the right USNHL. Our results indicate that the left primary auditory cortex (non-auditory-deprived cortex) in patients with left USNHL has been reorganized by visual and sensorimotor modalities through cross-modal plasticity. Furthermore, the cross-modal reorganization also alters the directional brain functional networks. The auditory deprivation from the left or right side generates different influences on the human brain. Copyright © 2015 IBRO. Published by Elsevier Ltd. All rights reserved.

The Relationship between Serum Lipids and Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-Analysis

PubMed Central

Chang, I Jen; Kang, Chung Jan; Yueh, Chen Yu; Fang, Ku Hao; Yeh, Re Ming; Tsai, Yao Te

2015-01-01

Background Sudden sensorineural hearing loss (SSNHL) is a relatively common condition that is usually of unknown etiology. A number of individual studies have investigated the association between various serum lipids and SSNHL; however, the findings have been inconsistent. In an attempt to obtain more definitive information on the relationship between serum lipids and SSNHL, we carried out a systematic review and meta-analysis. Methods Medline, the Cochrane Library, and EMBASE were searched using the following key words: lipid, cholesterol, triglyceride, fat, serum, blood, sudden hearing loss, hearing loss, hearing disorders. Randomized controlled trials, prospective cohort studies, and retrospective case-control studies involving patients with SSNHL and healthy controls that examined the relationship (reported as odds ratios [OR]) between lipid profiles and SSNHL were included. Primary outcomes were total cholesterol and low-density lipoprotein cholesterol (LDL-C) concentrations. Secondary outcomes were triglyceride, high-density lipoprotein cholesterol, and lipoprotein(a) concentrations. Results A total of 6 case-control studies were included in this systematic review/meta-analysis. The total number of participants ranged from 30 to 250 in the case group and from 43 to 271 in the control group. Meta-analysis revealed no significant difference in total cholesterol levels between the case and control groups (pooled OR = 1.79, 95% confidence interval [CI] = 0.98 to 3.26, P = 0.057). Likewise, meta-analysis revealed no significant difference in LDL-C concentrations between the case and control groups (pooled OR = 1.15, 95% CI = 0.64 to 2.07, P = 0.639). Since there were an insufficient number of studies reporting data for the secondary outcomes, meta-analysis was not possible. Conclusions Our results do not provide evidence for serum lipids being associated with SSNHL, nor do they definitively rule out such an association. Additional studies are needed to ascertain the relationship, or lack thereof, between serum lipids and SSNHL. PMID:25866869

Mondini dysplasia with recurrent bacterial meningitis caused by three different pathogens.

PubMed

Shikano, Hiroaki; Ohnishi, Hidenori; Fukutomi, Hisashi; Ito, Kimiko; Morimoto, Masahiro; Teramoto, Takahide; Aoki, Mitsuhiro; Nishihori, Takezumi; Akeda, Yukihiro; Oishi, Kazunori; Fukao, Toshiyuki

2015-12-01

Mondini dysplasia is rare, but has an important association with recurrent bacterial meningitis. We herein describe the case of a 3-year-old girl with unilateral sensorineural hearing loss who presented with three independent episodes of bacterial meningitis within 8 months. Temporal bone computed tomography indicated the characteristic features of Mondini dysplasia in the right inner ear. This was treated by surgical closure of the inner ear defect via oval window and additional vaccination was administered. Appropriate vaccination might prevent the recurrent bacterial meningitis associated with Mondini dysplasia. © 2015 Japan Pediatric Society.

Profound hearing loss associated with hydrocodone/acetaminophen abuse.

PubMed

Friedman, R A; House, J W; Luxford, W M; Gherini, S; Mills, D

2000-03-01

To describe profound hearing loss associated with hydrocodone overuse and the successful rehabilitation of these patients with cochlear implantation. Retrospective review. A tertiary otologic referral center. Twelve patients with rapidly progressive hearing loss and a concurrent history of hydrocodone overuse. Comprehensive medical histories, physical findings, audiometric tests, and, in those patients undergoing cochlear implantation, postimplantation performance data were reviewed. Clinical characteristics of hydrocodone-related hearing loss and open set word and sentence performance in those patients undergoing cochlear implantation. Hydrocodone overuse was associated with rapidly progressive sensorineural hearing loss in 12 patients. In four patients the initial presentation was unilateral, and two of the patients experienced vestibular symptoms. None of the 12 patients experienced improved thresholds after high-dose prednisone. Seven of the eight patients undergoing cochlear implantation have demonstrated early success with their devices. Hydrocodone is frequently prescribed in combination with acetaminophen for the relief of pain and has a side effects profile similar to other medications in its class. Although not described previously, overuse or abuse can be associated with a rapidly progressive sensorineural hearing loss. These patients can be successfully rehabilitated with cochlear implantation.

[Simultaneous triple semicircular canal plugging and cochlear implantation in advanced Meniere's disease].

PubMed

Zhang, D G; Xu, L; Han, Y C; Lyu, Y F; Luo, J F; Li, Y W; Wang, R J; Fan, Z M; Wang, H B

2017-01-07

Objective: To evaluate the feasibility of combining simultaneous triple semicircular canal plugging (TSCP) and cochlear implantation (CI) to treat vertigo and hearing loss in advanced Meniere's disease(MD) patients, so as to provide an alternative surgical procedure for treating this disorder. Methods: Data from seven patients, who were referred to our hospital and diagnosed with unilateral MD strictly meeting the criteria issued by Chinese Academy of Otolaryngology-Head and Neck Surgery Committee (2006), from Jan. 2015 to Jan. 2016, were retrospectively analyzed in this work. Seven patients, in whom the standardized conservative treatment had been given for at least one year and frequent vertigo still occurred, underwent simultaneous TSCP and CI under general anesthesia via mastoid approach. Postoperative follow-up time was more than six months. Vertigo control and auditory function were measured. Pure tone audiometry, speech perception scores, caloric test, head impulse test (HIT), and vestibular evoked myogenic potential (VEMP) were performed for evaluation of audiological and vestibular functions. Results: All patients had bilateral severe sensorineural hearing loss preoperatively. One side hearing loss was due to MD and another side was due to reasons including sudden sensorineural hearing loss, mumps and other unknown reason. The total control rate of vertigo in seven MD patients was 100.0% in the six-month follow-up, with complete control rate of 85.7% (6/7) and substantial control rate of 14.3% (1/7). Improved hearing threshold and speech perception scores were observed in all study participants. Postoperative average aided hearing threshold was 32.5 dBHL, the average monosyllabic word score was 42.6% and speech perception scores of sentences tested in quiet was 52.3%. Tinnitus improved in five cases, and no significant change in two patients. Post-operatively, all patients suffered from temporary vertigo and balance disorders. Vertigo was disappeared in all patients within 3-5 days, while, an average recovery time of balance disorders was 19.7 days. Six months after treatment, loss of semicircular canal function by caloric test was found in the operation side of all patients and no change in cVEMP or oVEMP test was noted. All patients had no facial paralysis, cerebrospinal fluid leakage, and other complications. Conclusions: A combined approach of TSCP and CI which could control vertigo effectively and improve hearing loss and tinnitus represents an effective and safe therapy for some advanced MD patients.

Sudden Unilateral Vision Loss Arising from Calcified Amorphous Tumor of the Left Ventricle

PubMed Central

Nazli, Yunus; Colak, Necmettin; Atar, Inci Asli; Alpay, Mehmet Fatih; Haltas, Hacer; Eryonucu, Beyhan; Cakir, Omer

2013-01-01

Calcified amorphous tumor of the heart is a very rare non-neoplastic intracavitary mass. The clinical presentation is similar to that of other cardiac masses. The precise cause and best approach to treatment remain unclear. We describe a case of cardiac calcified amorphous tumor presenting with refractory unilateral vision loss that was successfully treated by surgical excision. To our knowledge, this is only the 2nd reported case of retinal arterial embolism due to cardiac calcified amorphous tumor in the English-language literature. PMID:24082378

Lassa fever-induced sensorineural hearing loss: A neglected public health and social burden.

PubMed

Mateer, Elizabeth J; Huang, Cheng; Shehu, Nathan Y; Paessler, Slobodan

2018-02-01

Although an association between Lassa fever (LF) and sudden-onset sensorineural hearing loss (SNHL) was confirmed clinically in 1990, the prevalence of LF-induced SNHL in endemic countries is still underestimated. LF, a viral hemorrhagic fever disease caused by Lassa virus (LASV), is endemic in West Africa, causing an estimated 500,000 cases and 5,000 deaths per year. Sudden-onset SNHL, one complication of LF, occurs in approximately one-third of survivors and constitutes a neglected public health and social burden. In the endemic countries, where access to hearing aids is limited, SNHL results in a decline of the quality of life for those affected. In addition, hearing loss costs Nigeria approximately 43 million dollars per year. The epidemiology of LF-induced SNHL has not been characterized well. The complication of LF induced by SNHL is also an important consideration for vaccine development and treatments. However, research into the mechanism has been hindered by the lack of autopsy samples and relevant small animal models. Recently, the first animal model that mimics the symptoms of SNHL associated with LF was developed. Preliminary data from the new animal model as well as the clinical case studies support the mechanism of immune-mediated injury that causes SNHL in LF patients. This article summarizes clinical findings of hearing loss in LF patients highlighting the association between LASV infection and SNHL as well as the potential mechanism(s) for LF-induced SNHL. Further research is necessary to identify the mechanism and the epidemiology of LF-induced SNHL.

Hearing evaluation of intratympanic methylprednisolone perfusion for refractory sudden sensorineural hearing loss.

PubMed

She, Wandong; Dai, Yanhong; Du, Xiaoping; Yu, Chenjie; Chen, Feng; Wang, Junguo; Qin, Xiaoming

2010-02-01

To investigate the effectiveness and safety of intratympanic methylprednisolone perfusion (IMP) through a microcatheter in patients with sudden sensorineural hearing loss (SSNHL) who failed a conventional treatment. Prospective clinical study. This study was conducted in Nanjing Drum Tower Hospital, Nanjing University Medical School. Patients who had failed a minimum 10-day conventional treatment were included. Twenty-six patients in the study group (SG) received methylprednisolone perfusion through a microcatheter placed into the tympanum once a day for 10 days and the conventional treatment. Twenty-three patients who received a second conventional treatment (no steroid) served as the comparison group (CG). All patients were followed up for three months after the end of treatment. The effective rates for SG and CG were 50 percent (61.9% when only patients with an interval from onset to IMP < or = 60 days were included) and 21.7 percent, respectively (chi(2) = 4.194, P = 0.041). The pure-tone average improvement was 20.2 +/- 15.6 dB in SG, and 9.2 +/- 13.7 dB in CG (z = 2.51, P = 0.011). In SG, hearing improvement at low frequencies was better than that at high frequencies. The interval from onset to IMP affected the efficacy of IMP. IMP through a microcatheter is a promising treatment for refractory SSNHL. The data suggest that the treatment may be more effective when administered at the earlier stages of SSNHL when the conventional treatment has failed. Copyright 2010 American Academy of Otolaryngology-Head and Neck Surgery Foundation. Published by Mosby, Inc. All rights reserved.

[Cervical cord infarction associated with unilateral vertebral artery dissection due to golf swing].

PubMed

Tokumoto, Kazuki; Ueda, Nobuhiko

2014-01-01

A-68-year-old man experienced nuchal pain and bilateral shoulder weakness that occurred suddenly after he performed a golf swing. He was conscious. His cranial nerves were normal, but bilateral deltoid and biceps muscle strengths weakened. Magnetic resonance image (MRI) showed no brain stem infarctions or cervical epidural hematoma. We tentatively diagnosed him with concussion of the spinal cord because of mild recovery of his bilateral upper limb weakness after several hours; he was later discharged. The next day, he suddenly developed serious tetraplegia and was admitted to the emergency department. His breathing was controlled by a respirator as he had expectoration difficulty and respiratory muscle paralysis. A lesion in the cervical cord became apparent on MRI; the right vertebral artery was not detected on magnetic resonance angiography. Cervical MRI showed the intimal flap and a lack of flow void in the right vertebral artery. These findings revealed a right vertebral artery dissection. Cervical cord infarction due to unilateral vertebral artery dissection is rarer than posterior cerebral infarction due to the same pathogenesis; however, some such cases have been reported. We consider the present case to be caused by cervical cord infarction associated with unilateral vertebral artery dissection resulting from golf swing.

Comparative study between unilateral and bilateral cochlear implantation in children of 1 and 2 years of age.

PubMed

Escorihuela García, Vicente; Pitarch Ribas, María Ignacia; Llópez Carratalá, Ignacio; Latorre Monteagudo, Emilia; Morant Ventura, Antonio; Marco Algarra, Jaime

2016-01-01

The studies that have evaluated the effectiveness of bilateral cochlear implantation in children suggest an improvement in hearing about sound localization and speech discrimination. In this paper we show the differences in audio-linguistic achievements with early bilateral cochlear implantation versus unilateral, and differences between simultaneous and sequential bilateral implantation. We present 88 children with bilateral profound sensorineural hearing loss, treated with bilateral cochlear implantation in 32 cases and unilateral in 56 cases, during the first 12 months (27 children) of life and between 12 and 24 months (61 children). We conducted a statistical comparison of both groups in the audiometry, IT-Mais, Nottingham, LittlEars scales and verbal tests. No significant differences in hearing thresholds and questionnaires between unilateral and bilateral implantation were detected in either the first or second year. Verbal tests do show statistically significant differences: children with bilateral cochlear implant obtain 100% recognition of disyllabic and phrases within 2-3 years after implantation whilst children with one implant do not obtain those results at 5 years after surgery. No differences between simultaneous and sequential bilateral implantation were detected. We emphasize the importance of ensuring good early audiological screening, to carry out an early and bilateral cochlear implantation with the consequent development of audio-language skills similar to normal hearing children. Copyright © 2015 Elsevier España, S.L.U. y Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

[Aberrant topological properties of whole-brain functional network in chronic right-sided sensorineural hearing loss: a resting-state functional MRI study].

PubMed

Zhang, Lingling; Liu, Bin; Xu, Yangwen; Yang, Ming; Feng, Yuan; Huang, Yaqing; Huan, Zhichun; Hou, Zhaorui

2015-02-03

To investigate the topological properties of the functional brain network in unilateral sensorineural hearing loss patients. In this study, we acquired resting-state BOLD- fMRI data from 19 right-sided SNHL patients and 31 healthy controls with normal hearing and constructed their whole brain functional networks. Two-sample two-tailed t-tests were performed to investigate group differences in topological parameters between the USNHL patients and the controls. Partial correlation analysis was conducted to determine the relationships between the network metrics and USNHL-related variables. Both USNHL patients and controls exhibited small-word architecture in their brain functional networks within the range 0. 1 - 0. 2 of sparsity. Compared to the controls, USNHL patients showed significant increase in characteristic path length and normalized characteristic path length, but significant decrease in global efficiency. Clustering coefficient, local efficiency and normalized clustering coefficient demonstrated no significant difference. Furthermore, USNHL patients exhibited no significant association between the altered network metrics and the duration of USNHL or the severity of hearing loss. Our results indicated the altered topological properties of whole brain functional networks in USNHL patients, which may help us to understand pathophysiologic mechanism of USNHL patients.

Sudden Sensorineural Hearing Loss in the Department of Defense.

PubMed

Hughes, Charlotte K; Fischer, Jakob; Esquivel, Carlos R; Laury, Adrienne M

2018-04-01

Objective The American Academy of Otolaryngology-Head and Neck Surgery Foundation clinical practice guideline (CPG) proposes recommendations regarding sudden sensorineural hearing loss (SSNHL). SSNHL is managed by primary care, emergency medicine, and otolaryngology providers in the Department of Defense (DoD). However, their adherence to this CPG is unknown. We sought to determine provider compliance and identify areas for improvement. Study Design Case series with chart review. Setting DoD's electronic medical record. Subjects and Methods Patients with SSNHL (N = 204) were treated between March 1, 2012, and September 30, 2015. Time from onset of symptoms to evaluation by primary care, emergency department, audiology, and otolaryngology providers and treatments were analyzed. Results The average interval from onset of symptoms to evaluation by a primary care or emergency department provider was 4.86 days (95% CI, 3.46-6.26). Time from presentation to ear, nose, and throat and audiologic evaluation was 15.26 days (95% CI, 12.34-18.20) and 14.16 days (95% CI, 11.31-17.01), respectively. Diagnostic workup included magnetic resonance imaging (n = 150, 73.5%), computed tomography (n = 28, 13.7%), and laboratory testing (n = 50, 24.5%). Oral steroids were used in 137 (67.2%) patients, with 78.8% treated with the recommended dose. Intratympanic steroids were utilized in 65 (31.9%) patients, with variable dosing. Conclusion The DoD is uniquely positioned to evaluate adherence to CPGs on national and international levels given the robust and standardized electronic medical record. Areas of improvement include timely identification of SSNHL with rapid referral to ear, nose, and throat and audiology providers; minimizing unnecessary imaging, laboratory testing, and medications; and correct dosing of oral and intratympanic steroids.

Clinical Study to Evaluate the Association Between Sensorineural Hearing Loss and Diabetes Mellitus in Poorly Controlled Patients Whose HbA1c >8.

PubMed

Srinivas, C V; Shyamala, V; Shiva Kumar, B R

2016-06-01

The relationship between sensorineural hearing loss (SNHL) and Diabetes mellitus has been known since more than 150 years. The pathophysiology of diabetes related hearing loss is speculative. Hearing loss is usually, bilateral, gradual onset, affecting higher frequencies. This study aims at knowing the prevalence of SNHL in DM and its relation to age, sex, duration of DM and control of DM. A total of 50 type 2 diabetics of age group 30-65 years were involved in the study. FBS, PPBS, HbA1c of all the subjects were done and later subjected to PTA. The type and severity of hearing loss was noted. Occurrence of SNHL was later compared with age, sex, duration, and control of DM. Sensorineural hearing loss was found in 66 % of type II diabetic patients and 34 % were found normal. Out of 50 diabetes mellitus patients, 33 patients had SNHL. All cases of SNHL detected were of gradual in onset and no one had hearing loss of sudden onset. Normal hearing was found in 34 % of patients, whereas 54 % of patients had mild hearing loss and 12 % of patients had moderate hearing loss. Association of hearing loss of DM patients with sex of the patient is insignificant. However there is significant association between older age group, longer duration and uncontrolled DM with that of SNHL. In subjects with HbA1c more than 8 and duration of diabetes mellitus more than 10 years prevalence of SNHL is more than 85 %, which is statistically significant. Sensorineural hearing loss in diabetes mellitus is gradually progressive involving high frequency thresholds. Hearing threshold increases with increasing age duration of diabetes and also high level of HbA1c greater than 8 %.

The diagnostic value of earlier and later components of Vestibular Evoked Myogenic Potentials (VEMP) in multiple sclerosis.

PubMed

Eleftheriadou, A; Deftereos, S N; Zarikas, V; Panagopoulos, G; Sfetsos, S; Karageorgiou, C L; Ferekidou, E; Kandiloros, D; Korres, S

2009-01-01

To evaluate the ability of VEMP to disclose spatial dissemination of Multiple Sclerosis. Forty-six MS patients with auditory and/or vestibular symptoms were studied. Patients were divided in two groups. Group 1 included 24 patients with brainstem MRI findings, and Group 2 included 22 patients without MRI findings. VEMP and BAEP have been recorded and assessed. Abnormal p13n23 wave was observed in 50%, while unilateral absence or bilateral delay of the n34p44 in 43% of the patients. The overall diagnostic value considering abnormal cases suggested by both first and second VEMP waves was increased to 71%. Statistically significant differences revealed between patients and controls for p13 latency (p=0.018). The p13n23 was abnormal in 7 patients, although MRI scanning did not reveal brainstem lesions. In 9 out of 18 MS patients suffering from unilateral hearing loss, n34p44 was present in the unaffected ears and absent in the affected side, although p13n23 was normal. Abnormal VEMP imply the presence of lesions undetected by MRI neuroimaging, which verifies the diagnostic value of the method. Unilateral absence of n34p44 complex was related with sensorineural hearing loss, supporting the hypothesis that n34p44 is of cochlear origin.

Sensorineural hearing loss in patients with chronic suppurative otitis media: Is there a significant correlation?

PubMed

Amali, Amin; Hosseinzadeh, Nima; Samadi, Shahram; Nasiri, Shirin; Zebardast, Jayran

2017-02-01

Hearing loss as a sequel of chronic suppurative otitis media (CSOM) is often conductive, but recent studies have found an additional sensorineural component in these patients, thus demonstrating inner ear damage. The aim of the study was to determine the association between CSOM and sensorineural hearing loss (SNHL) and to assess the influence of patient's age, duration of disease, and presence of cholesteatoma and ossicular erosion on the degree of SNHL. In a retrospective study, the medical records of 119 patients who underwent surgery was reviewed. Seventy patients met the inclusion criteria of unilateral otorrhea, normal contralateral ear on otoscopy, and age between 10-65 years with no history of head trauma or ear surgery or familial hearing loss. Bone conduction (BC) thresholds for affected and contralateral ear were measured at frequencies of 500, 1000, 2000, and 4000 Hz. Data analysis was performed using SPSS 13 with independent-samples t-test, Pearson correlation test, and two-tailed analysis. A p ≤ 0.05 was considered statistically significant. Significant higher BC thresholds were found in the affected ear than in the normal ear for each frequency (p < 0.001), which increased with increasing frequency (7.00 dB at the 500 Hz and 9.71 dB at the 4000 Hz). There was a significant correlation between age and degree of SNHL (r = 0.422, p < 0.001) but no significant correlation was in duration of the disease (r = 0.119, p > 0.05). There was no relationship between presence of cholesteatoma and ossicular erosion with SNHL (p > 0.05). These findings demonstrate that CSOM is associated with some degree of SNHL and cochlear damage, and higher frequencies are more affected. Aging can act as a precipitating factor in this pathological process.

Vestibular Evoked Myogenic Potential Produced by Bone-Conducted Stimuli: A Study on its Basics and Clinical Applications in Patients with Conductive and Sensorineural Hearing Loss and a Group with Vestibular Schawannoma.

PubMed

Mahdi, Parvane; Amali, Amin; Pourbakht, Akram; Karimi Yazdi, Alireza; Bassam, Ali

2013-06-01

Vestibular evoked myogenic potential (VEMP) has recently been broadly studied in vestibular disorders. As it is evoked by loud sound stimulation, even mild conductive hearing loss may affect VEMP results. Bone-conducted (BC) stimulus is an alternative stimulation for evoking this response. This study aims to assess the characteristics of BC-VEMP in different groups of patients. We performed a cross sectional analysis on 20 healthy volunteers with normal pure-tone audiometry as a control group; and on a group of patients consisted of 20 participants with conductive hearing loss, five with bilateral sensorineural hearing loss and four with vestibular schawannoma. AC and BC-VEMP were performed in all participants. In control group the VEMP responses to both kinds of stimuli had an acceptable morphology and consisted of p13 and n23 waves. Latency value of these main components in each type of stimulus was not significantly different (P>0.05). However, the mean amplitude was larger in BC modality than AC stimulation (P=0.025). In the group with conductive hearing loss, the VEMP response was absent in fifteen (46.87%) of the 32 ears using the AC method, whereas all (100%) displayed positive elicitability of VEMP by BC method. Normal VEMP responses in both stimuli were evoked in all patients with sensorineural hearing loss. In patients with unilateral vestibular schwannomas (VS), 2 (50.00%) had neither AC-VEMP nor BC-VEMP. Auditory stimuli delivered by bone conduction can evoke VEMP response. These responses are of vestibular origin and can be used in vestibular evaluation of patients with conductive hearing loss.

Vestibular Evoked Myogenic Potential Produced by Bone-Conducted Stimuli: A Study on its Basics and Clinical Applications in Patients with Conductive and Sensorineural Hearing Loss and a Group with Vestibular Schawannoma

PubMed Central

Mahdi, Parvane; Amali, Amin; Pourbakht, Akram; Karimi Yazdi, Alireza; Bassam, Ali

2013-01-01

Introduction: Vestibular evoked myogenic potential (VEMP) has recently been broadly studied in vestibular disorders. As it is evoked by loud sound stimulation, even mild conductive hearing loss may affect VEMP results. Bone-conducted (BC) stimulus is an alternative stimulation for evoking this response. This study aims to assess the characteristics of BC-VEMP in different groups of patients. Materials and Methods: We performed a cross sectional analysis on 20 healthy volunteers with normal pure-tone audiometry as a control group; and on a group of patients consisted of 20 participants with conductive hearing loss, five with bilateral sensorineural hearing loss and four with vestibular schawannoma. AC and BC-VEMP were performed in all participants. Results: In control group the VEMP responses to both kinds of stimuli had an acceptable morphology and consisted of p13 and n23 waves. Latency value of these main components in each type of stimulus was not significantly different (P>0.05). However, the mean amplitude was larger in BC modality than AC stimulation (P=0.025). In the group with conductive hearing loss, the VEMP response was absent in fifteen (46.87%) of the 32 ears using the AC method, whereas all (100%) displayed positive elicitability of VEMP by BC method. Normal VEMP responses in both stimuli were evoked in all patients with sensorineural hearing loss. In patients with unilateral vestibular schwannomas (VS), 2 (50.00%) had neither AC-VEMP nor BC-VEMP. Conclusion: Auditory stimuli delivered by bone conduction can evoke VEMP response. These responses are of vestibular origin and can be used in vestibular evaluation of patients with conductive hearing loss. PMID:24303434

Investigation of Tinnitus Patients in Italy: Clinical and Audiological Characteristics

PubMed Central

Martines, Francesco; Bentivegna, Daniela; Di Piazza, Fabiola; Martines, Enrico; Sciacca, Vincenzo; Martinciglio, Gioacchino

2010-01-01

Objective. 312 tinnitus sufferers were studied in order to analyze: the clinical characteristics of tinnitus; the presence of tinnitus-age correlation and tinnitus-hearing loss correlation; the impact of tinnitus on subjects' life and where possible the etiological/predisposing factors of tinnitus. Results. There is a slight predominance of males. The highest percentage of tinnitus results in the decades 61–70. Of the tinnitus sufferers, 197 (63.14%) have a hearing deficit (light hearing loss in 37.18% of cases). The hearing impairment results of sensorineural type in 74.62% and limited to the high frequencies in 58.50%. The tinnitus is referred as unilateral in 59.93%, a pure tone in 66.99% and 10 dB above the hearing threshold in 37.7%. It is limited to high frequencies in 72.10% of the patients with sensorineural hearing loss (SNHL) while the 88.37% of the patients with high-frequency SNHL have a high-pitched tinnitus (χ 2 = 66.26;P < .005). Conclusion. Hearing status and age represent the principal tinnitus related factors; there is a statistically significant association between high-pitched tinnitus and high-frequency SNHL. There is no significant correlation between tinnitus severity and tinnitus loudness confirming the possibility that neural connection involved in evoking tinnitus-related negative reactions are governed by conditioned reflexes. PMID:20652075

Cost-Effectiveness of Pediatric Cochlear Implantation in Rural China.

PubMed

Qiu, Jianxin; Yu, Chongxian; Ariyaratne, Thathya V; Foteff, Chris; Ke, Zhangmin; Sun, Yi; Zhang, Li; Qin, Feifei; Sanderson, Georgina

2017-07-01

To evaluate the cost utility of cochlear implantation (CI) for severe to profound sensorineural hearing loss (SNHL) among children from rural settings in P.R. China (China). A cost-utility analysis (CUA) was undertaken using data generated from a single-center substudy of the Cochlear Pediatric Implanted Recipient Observational Study (Cochlear P-IROS). The data were projected over a 20-year time horizon using a decision tree model. The Chinese healthcare payer and patient perspectives were adopted. Unilateral CI of children with a severe-to-profound SNHL compared with their preimplantation state of no treatment or amplification with hearing aids ("no CI" status). Incremental costs per quality adjusted life year (QALY) gained. The mean total discounted cost of unilateral CI was CNY 252,506 (37,876 USD), compared with CNY 29,005 (4,351 USD) for the no CI status from the healthcare payer plus patient perspective. A total discounted benefit of 8.9 QALYs was estimated for CI recipients compared with 6.7 QALYs for the no CI status. From the healthcare payer plus patient perspective, incremental cost-effectiveness ratio (ICER) for unilateral CI compared with no CI was CNY 100,561 (15,084 USD) per QALY. The healthcare payer perspective yielded an ICER of CNY 40,929 (6,139 USD) per QALY. Both ICERs fell within one to three times China's gross domestic product per capita (GDP, 2011-2015), considered "cost-effective" by World Health Organization (WHO) standards. Treatment with unilateral CI is a cost-effective hearing solution for children with severe to profound SNHL in rural China. Increased access to mainstream education and greater opportunities for employment, are potential downstream benefits of CI that may yield further societal and economic benefits. CI may be considered favorably for broader inclusion in medical insurance schemes across China.

[Vestibulo-cochlear disturbances in the course of vaso-neural conflict of vestibule-cochlear nerve--diagnostic and therapeutic problems].

PubMed

Orendorz-Fraczkowska, Krystyna; Jaworska, Marzena; Gawron, Wojciech; Badowski, Roman; Nadolska, Beata

2007-01-01

Symptoms encompassing sensorineural hearing loss, tinnitus and vertigo occur in many diseases of various origin. The diagnostics in such cases is especially difficult and often requires interdisciplinary cooperation. Despite of that many cases remain unexplained. The two cases with above mentioned symptoms (52 year-old woman and 46 year-old man) with differentiated clinical course were presented. The woman for one year experienced left sided, extreme tinnitus with paroxysmal vertigo and dizziness. The man with sudden monolateral hearing loss and tinnitus that disappeared after corticosteroid therapy, complained about recurrence of fluctuating hearing loss and tinnitus accompanied by chronic instability. The diagnostics of hearing and balance organs was performed (pure tone audiometry, impedance audiometry, DPOAE, ABR, ENG) complemented with computed tomography and Nuclear Resonance. Female patient presented bilateral mild sensorineural hearing loss, more intensive on the left side, male patient right sided sensorineural hearing loss in the frequencies from 250 to 1500 Hz. The elongation of some peak values in ABR test (with bilaterally proper otoacoustic emissions) as well as partial canal paresis on the hearing loss side suggested primary diagnosis of neoplasmatic process. It was an indication to perform radiological diagnostics. The radiologic findings revealed the contact of vestibulo-cochlear nerve with the loop of cerebellar inferior anterior artery within the internal acoustic canal. The course of the disease and diagnostic tests do not provide characteristic data that let diagnose neuro-vascular conflict. One has to be aware of such possibility in patients with intensive vertigo and dizziness that do not improve after treatment when other causes do not justify the symptoms.

Bell's Palsy as a Possible Complication of Hepatitis B Vaccination in A Child

PubMed Central

Tan, Hüseyin; Orbak, Zerrin

2009-01-01

Bell's Palsy is the sudden onset of unilateral temporary paralysis of facial muscles resulting from seventh cranial nerve dysfunction. Presented here is a two-year old female patient with right peripheral facial palsy following hepatitis B vaccination. Readers’ attention is drawn to an uncommon cause of Bell's Palsy, as a rare complication of hepatitis B vaccination. PMID:19902808

Confined anterior cerebral artery infarction manifesting as isolated unilateral axial weakness.

PubMed

Honig, Asaf; Eliahou, Ruth; Auriel, Eitan

2017-02-15

We describe isolated unilateral axial weakness in three patients eventually diagnosed with anterior cerebral artery infarction (ACAI), a new clinical observation. Files of three ACAI patients (2 females, 1 male, ages 55-80) were retrospectively reviewed. All three presented to the ED with sudden unsteadiness. On initial neurological examination, all three patients manifested unilateral truncal deviation to the side contralateral to the weakness, even while seated. There was significant unilateral hypotonia due to substantial paravertebral weakness. None had pyramidal signs or increased limb tone. Speech, language, and cognitive performance were intact during admission examination. In all three patients, initial diffusion-weighted imaging (DWI) MRI showed small confined regions of restriction involving the posterolateral border of ACA territory; CT angiography was normal in one patient with a newly diagnosed atrial fibrillation but showed atherosclerotic vasculature with severe narrowing of the A3 segment of the ACA in two. Awareness of ACAI presenting as unilateral axial weakness is warranted. We suggest that optimal diagnostic management should include examination of axial tone. Ischemic involvement of distal ACA branches may herald a more extensive ACAI. Prompt diagnosis may enable thrombolysis or endovascular treatment, and blood pressure maintenance may allow adequate perfusion to damaged tissue. Copyright © 2016 Elsevier B.V. All rights reserved.

The clinical features and prognosis of mumps-associated hearing loss: a retrospective, multi-institutional investigation in Japan.

PubMed

Morita, Shinya; Fujiwara, Keishi; Fukuda, Atsushi; Fukuda, Satoshi; Nishio, Shin-Ya; Kitoh, Ryosuke; Hato, Naohito; Ikezono, Tetsuo; Ishikawa, Kotaro; Kaga, Kimitaka; Matsubara, Atsushi; Matsunaga, Tatsuo; Murata, Takaaki; Naito, Yasushi; Nishizaki, Kazunori; Ogawa, Kaoru; Sano, Hajime; Sato, Hiroaki; Sone, Michihiko; Suzuki, Mikio; Takahashi, Haruo; Tono, Tetsuya; Yamashita, Hiroshi; Yamasoba, Tatsuya; Usami, Shin-Ichi

2017-01-01

The majority of hearing loss due to mumps presents as unilateral profound sensorineural hearing loss, which is refractory to treatment. In rare cases of bilateral total deafness, cochlear implants were beneficial for speech perception. Vaccination against mumps is recommended to prevent mumps-associated hearing loss. The objective of this study is to investigate the clinical characteristics of hearing loss due to mumps and to evaluate hearing outcomes. The clinical parameters were analyzed under a retrospective multi-institutional study design in patients diagnosed with hearing loss due to mumps at the Otolaryngology departments of 19 hospitals between 1987 and 2016. Sixty-seven patients with hearing loss due to mumps were enrolled. The study population consisted of 35 males and 32 females, ranging in age from 1 to 54, with a median age of 9.5 years. Sixty-three patients presented with unilateral, and 4 with bilateral hearing loss. Profound hearing loss was observed in 65 ears. Only one ear with severe hearing loss showed complete recovery. Four patients with bilateral hearing loss received cochlear implant surgery. Most of the patients with hearing loss due to mumps had no history of vaccination.

A case-control analysis on the association between erectile dysfunction and sudden sensorineural hearing loss in Taiwan.

PubMed

Keller, Joseph J; Chen, Yi-Kuang; Lin, Herng-Ching

2012-05-01

Although the cause of sudden sensorineural hearing loss (SSNHL) is yet to be elucidated, many theories have been proposed regarding potentially contributory etiologies. One increasingly well-supported theory purports an underlying vascular pathomechanism. If this is the case, SSNHL may also associate with conditions comorbid with vascular diseases, such as erectile dysfunction (ED). However, no studies to date have investigated the association between ED and SSNHL. This study set out to estimate a putative association between ED and having been previously diagnosed with SSNHL using a population-based dataset with a case-control design. This study used administrative claim data from the Taiwan National Health Insurance program. We identified 4,504 patients with ED as the study group and randomly selected 22,520 patients as the comparison group. Conditional logistic regression was used to examine the association between ED and having previously received a diagnosis of SSNHL. The prevalence and risk of SSNHL between cases and controls were calculated. Of the sampled patients, 41 (0.15%) had been diagnosed with SSNHL before the index date; 22 (0.49% of the cases) were from the study group and 19 (0.08% of controls) were from the control group. Conditional logistic regression analysis revealed that after adjusting for the patient's monthly income, geographic location, hypertension, diabetes, hyperlipidemia, coronary heart disease, obesity, and alcohol abuse/alcohol dependence syndrome status, patients with ED were more likely than controls to have been diagnosed with SSNHL before the index date (odds ratio = 6.06, 95% confidence interval = 3.25-11.29). There was an association between ED and prior SSNHL. The results of this study add to the evidence supporting an underlying vascular pathomechanism regarding the development of SSNHL and highlight a need for clinicians dealing with SSNHL patients to be alert to the development of ED. © 2012 International Society for Sexual Medicine.

Vitamins A, C, and E and selenium in the treatment of idiopathic sudden sensorineural hearing loss.

PubMed

Kaya, Hakan; Koç, Arzu Karaman; Sayın, İbrahim; Güneş, Selçuk; Altıntaş, Ahmet; Yeğin, Yakup; Kayhan, Fatma Tülin

2015-05-01

This study evaluated the effectiveness of vitamins A, C, and E, with selenium, in the treatment of idiopathic sudden sensorineural hearing loss (ISSNHL). This was a prospective, controlled study performed at a tertiary teaching and research hospital. Over a 32-month period, patients were treated with either our standard ISSNHL treatment regimen plus vitamins A, C, and E and selenium (ACE+ group) or with only our standard ISSNHL treatment regimen (ACE- group). The demographics, additional symptoms, mean initial and final hearing levels, mean hearing gain, and recovery data were compared between the two groups. The ACE+ group, consisting of 70 (55.5 %) patients, received vitamin A (natural beta-carotene, 26,000 IU), vitamin C (ascorbic acid, 200 mg), vitamin E (d-alpha-tocopherol, 200 IU), and selenium (50 μg) twice daily for 30 days in addition to our ISSNHL treatment regimen: methylprednisolone at an initial dose of 1 mg/kg body weight per day, tapered over 14 days; Rheomacrodex(®) [(10 g of dextran and 0.9 g of NaCl)/100 ml] 500 ml daily for 5 days; Vastarel(®) 20-mg tablet (20 mg of trimetazidine dihydrochloride) three times daily for 30 days; and ten 60-min hyperbaric oxygen (HBO) sessions (2.5 absolute atmospheres of 100 % O2), once daily, starting the day of hospitalization. The ACE- group comprised 56 (44.4 %) patients, who received only our ISSNHL treatment regimen. The mean hearing gains were 36.2 ± 20.3 dB in the ACE+ group and 27.1 ± 20.6 dB in the ACE- group. The mean hearing gain rates were significantly higher in the ACE+ group than in the ACE- group (p = 0.014). Treatment with vitamins A, C, and E and selenium was effective in ISSNHL patients undergoing treatment with methylprednisolone, dextran, trimetazidine dihydrochloride, and HBO, and might be more effective when the initial hearing level is below 46 dB.

The efficacy and safety of systemic injection of Ginkgo biloba extract, EGb761, in idiopathic sudden sensorineural hearing loss: a randomized placebo-controlled clinical trial.

PubMed

Koo, Ja-Won; Chang, Mun Young; Yun, Sung-Cheol; Kim, Tae Su; Kong, Soo-Keun; Chung, Jong Woo; Goh, Eui-Kyung

2016-09-01

Steroids are currently the most frequently accepted agents for idiopathic sudden sensorineural hearing loss (ISSNHL). However, the therapeutic effect of steroids is not always satisfactory. In this pilot study, we evaluated whether systemic treatment with Ginkgo biloba extract (EGb761) has an additive therapeutic effect in patients receiving a systemic steroid due to ISSNHL. A multicenter, randomized, double-blind clinical trial was performed. Fifty-six patients with ISSNHL were allocated to either EGb761 or placebo. In both groups, methylprednisolone was administered for 14 days. EGb761 was infused intravenously for 5 days in the EGb761 group, while the same amount of normal saline was infused in the placebo group. For the efficacy evaluation, pure-tone audiometry, speech audiometry, tinnitus handicap inventory (THI) and short form-36 health (SF-36) survey outcomes were obtained before administration and on days 3, 5, 14 and 28 of administration. Twenty-four patients in each group completed the study protocol. There was no difference in hearing loss between the two groups before treatment. At day 28, air conduction threshold values in the placebo and EGb761 groups were 34.63 ± 28.90 and 23.84 ± 25.42 dB, respectively (p = 0.082). Speech discrimination scores in the placebo and EGb761 groups were 69.17 ± 40.89 and 87.48 ± 28.65 %, respectively (p = 0.050). THI and SF-36 scores in the placebo and EGb761 groups were similar. Although a combination of steroid and EGb761 for initial treatment did not show better pure tone threshold, compared with steroid alone, speech discrimination was significantly improved in combination therapy. Further studies will be needed to know if addition of EGb761 actually improves the outcome of ISSNHL treatment.

Transcanal endoscopic ear surgery for perilymphatic fistula after electric acoustic stimulation.

PubMed

Omichi, Ryotaro; Kariya, Shin; Maeda, Yukihide; Nishizaki, Kazunori

2018-06-01

Transcanal endoscopic ear surgery (TEES) will become a very useful therapeutic option. A perilymphatic fistula (PLF) is defined as sudden sensorineural hearing loss and/or vertigo caused by leakage of the perilymph through a fistula from the oval window and/or round window. We report a case of PLF after electric acoustic stimulation (EAS), a kind of cochlear implant, successfully treated by TEES. A 38-year-old man presented to our hospital with vertigo and hearing loss (HL). His vertigo was induced by Valsalva maneuvers. Eight months ago, he underwent EAS for his right ear for congenital sensorineural HL. Although he maintained his hearing level after EAS, his pure tone audiogram this time showed deterioration of hearing at low frequencies in his right ear. A diagnosis of right PLF was made. After confirming the non-effectiveness of oral prednisolone treatment, PLF repair surgery to patch the oval and round windows by TEES was performed. His vertigo did not recur after the surgery. To the best of our knowledge, this is the first report of PLF repair surgery by TEES without a microscope. The wide-field view of the middle ear by TEES was useful to prevent electrode damage in a PLF patient with a cochlear implant. Copyright © 2017 Elsevier B.V. All rights reserved.

Hearing Instruments for Unilateral Severe-to-Profound Sensorineural Hearing Loss in Adults: A Systematic Review and Meta-Analysis

PubMed Central

Smith, Sandra Nelson; Lucas, Laura

2016-01-01

Objectives: A systematic review of the literature and meta-analysis was conducted to assess the nature and quality of the evidence for the use of hearing instruments in adults with a unilateral severe to profound sensorineural hearing loss. Design: The PubMed, EMBASE, MEDLINE, Cochrane, CINAHL, and DARE databases were searched with no restrictions on language. The search included articles from the start of each database until February 11, 2015. Studies were included that (a) assessed the impact of any form of hearing instrument, including devices that reroute signals between the ears or restore aspects of hearing to a deaf ear, in adults with a sensorineural severe to profound loss in one ear and normal or near-normal hearing in the other ear; (b) compared different devices or compared a device with placebo or the unaided condition; (c) measured outcomes in terms of speech perception, spatial listening, or quality of life; (d) were prospective controlled or observational studies. Studies that met prospectively defined criteria were subjected to random effects meta-analyses. Results: Twenty-seven studies reported in 30 articles were included. The evidence was graded as low-to-moderate quality having been obtained primarily from observational before-after comparisons. The meta-analysis identified statistically significant benefits to speech perception in noise for devices that rerouted the speech signals of interest from the worse ear to the better ear using either air or bone conduction (mean benefit, 2.5 dB). However, these devices also degraded speech understanding significantly and to a similar extent (mean deficit, 3.1 dB) when noise was rerouted to the better ear. Data on the effects of cochlear implantation on speech perception could not be pooled as the prospectively defined criteria for meta-analysis were not met. Inconsistency in the assessment of outcomes relating to sound localization also precluded the synthesis of evidence across studies. Evidence for the relative efficacy of different devices was sparse but a statistically significant advantage was observed for rerouting speech signals using abutment-mounted bone conduction devices when compared with outcomes after preoperative trials of air conduction devices when speech and noise were colocated (mean benefit, 1.5 dB). Patients reported significant improvements in hearing-related quality of life with both rerouting devices and following cochlear implantation. Only two studies measured health-related quality of life and findings were inconclusive. Conclusions: Devices that reroute sounds from an ear with a severe to profound hearing loss to an ear with minimal hearing loss may improve speech perception in noise when signals of interest are located toward the impaired ear. However, the same device may also degrade speech perception as all signals are rerouted indiscriminately, including noise. Although the restoration of functional hearing in both ears through cochlear implantation could be expected to provide benefits to speech perception, the inability to synthesize evidence across existing studies means that such a conclusion cannot yet be made. For the same reason, it remains unclear whether cochlear implantation can improve the ability to localize sounds despite restoring bilateral input. Prospective controlled studies that measure outcomes consistently and control for selection and observation biases are required to improve the quality of the evidence for the provision of hearing instruments to patients with unilateral deafness and to support any future recommendations for the clinical management of these patients. PMID:27232073

Invasive rhino-orbito-cerebral mucormycosis in a diabetic patient – the need for prompt treatment

PubMed Central

Chow, Vanessa; Khan, Sheema; Balogun, Adeola; Mitchell, David; Mühlschlegel, Fritz A.

2014-01-01

Mucormycosis is a rare life threatening fungal infection predominately seen in immunocompromised or diabetic patients. The following case is of a known type II diabetic patient who presented with sepsis and sudden unilateral loss of vision secondary to infective rhino-orbito-cerebral mucormycosis. Treatment of the condition required extensive surgical intervention and medical management for a life saving outcome. PMID:25750854

Different clinical presentation and management of temporal bone fibrous dysplasia in children.

PubMed

Mierzwiński, Józef; Kosowska, Justyna; Tyra, Justyna; Haber, Karolina; Drela, Maria; Paczkowski, Dariusz; Burduk, Paweł

2018-01-15

Fibrous dysplasia is a slowly progressive benign fibro-osseous disorder that involves one or multiple bones with a unilateral distribution in most cases. It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. Temporal bone involvement is the least frequently reported type, especially in children. We reviewed available articles regarding fibrous dysplasia with temporal bone involvement in children and added four patients aged 7 to 17 years who were diagnosed and treated in our institution from 2006 to 2017. The patients' clinical picture comprised head deformity, external canal stenosis, headache, progressive conductive and/or sensorineural hearing loss, tinnitus, and sudden deafness. Two patients had experienced severe episodic vertigo with nausea and vomiting. Two were referred to us with external canal obstruction and secondary cholesteatoma formation with broad middle ear destruction. One was diagnosed with acute mastoiditis and intracranial complications. Optimal management of fibrous dysplasia is unclear and can be challenging, especially in children. In our two patients with disease expansion and involvement of important structures, surgical treatment was abandoned and a "wait-and-scan" policy was applied. The other two were qualified for surgical treatment. One patient underwent two surgeries: modified lateral petrosectomy (canal left open) with pathological tissue removal, cavity obliteration, and subsequent tympanoplasty. Another patient with extensive destruction of the left temporal bone underwent canal wall down mastoidectomy with perisinus abscess drainage and revision 12 months later. Tympanoplasty was unsatisfactory in both patients because of slow progression of the middle ear pathology. None of our patients underwent pharmacological treatment. In younger patients, observation and a "wait-and-scan" protocol is relevant until significant function, or cosmetic deficits are obvious. Surgery is not preferred and should be delayed until puberty because fibrous dysplasia has a tendency to stabilize after adolescence. In patients with severe symptoms medical treatment can be implemented, but safety of this treatment in children remain controversial.

Hearing loss and enlarged internal auditory canal in children.

PubMed

Santos, Saturnino; Domínguez, M Jesús; Cervera, Javier; Suárez, Alicia; Bueno, Antonio; Bartolomé, Margarita; López, Rafael

2014-01-01

Among the temporal bone abnormalities that can be found in the etiological study of paediatric sensorineural hearing loss (SNHL) by imaging techniques, those related to the internal auditory canal (IAC) are the least frequent. The most prevalent of these abnormalities that is associated with SNHL is stenotic IAC due to its association with cochlear nerve deficiencies. Less frequent and less concomitant with SNHL is the finding of an enlarged IAC (>8mm). Retrospective and descriptive review of clinical associations, imaging, audiological patterns and treatment of 9 children with hearing loss and enlarged IAC in the period 1999 to 2012. Two groups of patients are described. The first, without association with vestibulocochlear dysplasias, consisted of: 2 patients with SNHL without other temporal bone or systemic abnormalities, one with bilateral mixed HL from chromosome 18q deletion, one with a genetic X-linked DFN3 hearing loss, one with unilateral hearing loss in neurofibromatosis type 2 with bilateral acoustic neuroma, and one with unilateral hearing loss with cochlear nerve deficiency. The second group, with association with vestibulocochlear dysplasias, was comprised of: one patient with moderate bilateral mixed hearing loss in branchio-oto-renal syndrome, one with profound unilateral SNHL with recurrent meningitis, and another with profound bilateral SNHL with congenital hypothyroidism. The presence of an enlarged IAC in children can be found in different clinical and audiological settings with relevancies that can range from life-threatening situations, such as recurrent meningitis, to isolated hearing loss with no other associations. Copyright © 2013 Elsevier España, S.L. All rights reserved.

Hybrid cochlear implantation: quality of life, quality of hearing, and working performance compared to patients with conventional unilateral or bilateral cochlear implantation.

PubMed

Härkönen, Kati; Kivekäs, Ilkka; Kotti, Voitto; Sivonen, Ville; Vasama, Juha-Pekka

2017-10-01

The objective of the present study is to evaluate the effect of hybrid cochlear implantation (hCI) on quality of life (QoL), quality of hearing (QoH), and working performance in adult patients, and to compare the long-term results of patients with hCI to those of patients with conventional unilateral cochlear implantation (CI), bilateral CI, and single-sided deafness (SSD) with CI. Sound localization accuracy and speech-in-noise test were also compared between these groups. Eight patients with high-frequency sensorineural hearing loss of unknown etiology were selected in the study. Patients with hCI had better long-term speech perception in noise than uni- or bilateral CI patients, but the difference was not statistically significant. The sound localization accuracy was equal in the hCI, bilateral CI, and SSD patients. QoH was statistically significantly better in bilateral CI patients than in the others. In hCI patients, residual hearing was preserved in all patients after the surgery. During the 3.6-year follow-up, the mean hearing threshold at 125-500 Hz decreased on average by 15 dB HL in the implanted ear. QoL and working performance improved significantly in all CI patients. Hearing outcomes with hCI are comparable to the results of bilateral CI or CI with SSD, but hearing in noise and sound localization are statistically significantly better than with unilateral CI. Interestingly, the impact of CI on QoL, QoH, and working performance was similar in all groups.

Hearing Status in Pediatric Renal Transplant Recipients.

PubMed

Gulleroglu, Kaan; Baskin, Esra; Aydin, Erdinc; Ozluoglu, Levent; Moray, Gokhan; Haberal, Mehmet

2015-08-01

Renal transplant provides a long-term survival. Hearing impairment is a major factor in subjective health status. Status of hearing and the cause of hearing impairment in the pediatric renal transplant group have not been evaluated. Here, we studied to evaluate hearing status in pediatric renal transplant patients and to determine the factors that cause hearing impairment. Twenty-seven pediatric renal transplant recipients were investigated. All patients underwent audiologic assessment by means of pure-tone audiometry. The factors on hearing impairment were performed. Sensorineural hearing impairment was found in 17 patients. There was marked hearing impairment for the higher frequencies between 4000 and 8000 Hz. Sudden hearing loss developed in 2 patients, 1 of them had tinnitus. Decrease of speech understanding was found in 8 patients. The cyclosporine level was significantly high in patients with hearing impairment compared with group without hearing impairment. Cyclosporine levels also were found to be statistically significantly high when compared with the group with decrease of speech understanding and the group without decrease of speech understanding. Similar relations cannot be found between tacrolimus levels and hearing impairment and speech understanding. Sensorineural hearing impairment prevalence was high in pediatric renal transplant recipients when compared with the general population of children. Cyclosporine may be responsible for causing hearing impairment after renal transplant. We suggest that this effect is a dose-dependent toxicity.

An evidence based protocol for managing neonatal middle ear effusions in babies who fail newborn hearing screening.

PubMed

Weber, Brittany C; Whitlock, Scott M; He, Kaidi; Kimbrell, Blake S; Derkay, Craig S

2018-04-12

To evaluate the prevalence of middle ear disease in infants referred for failed newborn hearing screening (NBHS) and to review patient outcomes after intervention in order to propose an evidence-based protocol for management of newborns with otitis media with effusion (OME) who fail NBHS. 85 infants with suspected middle ear pathology were retrospectively reviewed after referral for failed NBHS. All subjects underwent a diagnostic microscopic exam with myringotomy with or without placement of a ventilation tube in the presence of a middle ear effusion and had intra-operative auditory brainstem response (ABR) testing or testing at a later date. At the initial office visit, a normal middle ear space bilaterally was documented in 5 babies (6%), 29/85 (34%) had an equivocal exam while 51/85 (60%) had at least a unilateral OME. Myringotomy with or without tube placement due to presence of an effusion was performed on 65/85 (76%) neonates. Normal hearing was established in 17/85 (20%) after intervention, avoiding the need for any further audiologic workup. Bilateral or unilateral sensorineural hearing loss (SNHL) or mixed hearing loss was noted in 54/85 (64%) and these children were referred for amplification. Initially observation with follow up outpatient visits was initiated in 27/85 (32%) however, only 3/27 (11%) resolved with watchful waiting and 24/27 (89%) ultimately required at least unilateral tube placement due to OME and 14/24 (59%) were found to have at least a unilateral mixed or SNHL. An effective initial management plan for children with suspected middle ear pathology and failed NBHS is diagnostic operative microscopy with placement of a ventilation tube in the presence of a MEE along with either intra-operative ABR or close follow-up ABR. This allows for the identification and treatment of babies with a conductive component due to OME, accurate diagnosing of an underlying SNHL component and for prompt aural rehabilitation. Copyright © 2018 Elsevier Inc. All rights reserved.

Detection of Unilateral Hearing Loss by Stationary Wavelet Entropy.

PubMed

Zhang, Yudong; Nayak, Deepak Ranjan; Yang, Ming; Yuan, Ti-Fei; Liu, Bin; Lu, Huimin; Wang, Shuihua

2017-01-01

Sensorineural hearing loss is correlated to massive neurological or psychiatric disease. T1-weighted volumetric images were acquired from fourteen subjects with right-sided hearing loss (RHL), fifteen subjects with left-sided hearing loss (LHL), and twenty healthy controls (HC). We treated a three-class classification problem: HC, LHL, and RHL. Stationary wavelet entropy was employed to extract global features from magnetic resonance images of each subject. Those stationary wavelet entropy features were used as input to a single-hidden layer feedforward neuralnetwork classifier. The 10 repetition results of 10-fold cross validation show that the accuracies of HC, LHL, and RHL are 96.94%, 97.14%, and 97.35%, respectively. Our developed system is promising and effective in detecting hearing loss. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

The round window diameter in congenital aural atresia and comparison with sensorineural hearing loss and control group.

PubMed

Saylisoy, Suzan; Incesulu, Armagan; Kaya, Ercan; Pinarbasli, Ozgur; Adapinar, Baki

2014-01-01

The aim of this study was to measure round window (RW) diameters in patients with congenital aural atresia (CAA) or sensorineural hearing loss (SNHL) and a normal control group and to analyze whether differences exist between these groups. Temporal bone computed tomographic scans of 12 patients with CAA (5 males, 7 females) aged 1 to 50 years (median age, 6 years), 12 patients with SNHL (8 males, 4 females) aged 2 to 32 years (median age, 5 years), and 11 patients (3 males, 7 females) aged 2 months to 53 years (median age, 8 years) randomly selected from a pool of patients with unilateral chronic otitis media or cholesteatoma were reviewed. We measured RW diameter on oblique reconstruction planes. To prevent possible individual differences, skull width was measured. There were no statistically significant differences between all groups for skull width. Both RW diameter and RW membrane width were found smaller in the CAA group than both SNHL group and control group with statistical significance, whereas there were no statistically significant differences between the SNHL group and the control group. We found that both the RW diameter and RW membrane width in CAA were smaller than those in the control group. If this finding is supported in future studies, the production of floating mass transducer with different sizes may be useful. We suggest that RW diameter should be measured in each patient before operation and thus a floating mass transducer with the appropriate caliber should be chosen.

Clinical variability of Waardenburg-Shah syndrome in patients with proximal 13q deletion syndrome including the endothelin-B receptor locus.

PubMed

Tüysüz, Beyhan; Collin, Anna; Arapoğlu, Müjde; Suyugül, Nezir

2009-10-01

Waardenburg-Shah syndrome (Waardenburg syndrome type IV-WS4) is an auditory-pigmentary disorder that combines clinical features of pigmentary abnormalities of the skin, hair and irides, sensorineural hearing loss, and Hirschsprung disease (HSCR). Mutations in the endothelin-B receptor (EDNRB) gene on 13q22 have been found to cause this syndrome. Mutations in both alleles cause the full phenotype, while heterozygous mutations cause isolated HSCR or HSCR with minor pigmentary anomalies and/or sensorineural deafness. We investigated the status of the EDNRB gene, by FISH analysis, in three patients with de novo proximal 13q deletions detected at cytogenetic analysis and examined the clinical variability of WS4 among these patients. Chromosome 13q was screened with locus specific FISH probes and breakpoints were determined at 13q22.1q31.3 in Patients 1 and 3, and at 13q21.1q31.3 in Patient 2. An EDNRB specific FISH probe was deleted in all three patients. All patients had common facial features seen in proximal 13q deletion syndrome and mild mental retardation. However, findings related to WS4 were variable; Patient 1 had hypopigmentation of the irides and HSCR, Patient 2 had prominent bicolored irides and mild bilateral hearing loss, and Patient 3 had only mild unilateral hearing loss. These data contribute new insights into the pathogenesis of WS4.

Transient facial nerve paralysis (Bell's palsy) following administration of hepatitis B recombinant vaccine: a case report.

PubMed

Paul, R; Stassen, L F A

2014-01-01

Bell's palsy is the sudden onset of unilateral transient paralysis of facial muscles resulting from dysfunction of the seventh cranial nerve. Presented here is a 26-year-old female patient with right lower motor neurone facial palsy following hepatitis B vaccination. Readers' attention is drawn to an uncommon cause of Bell's palsy, as a possible rare complication of hepatitis B vaccination, and steps taken to manage such a presentation.

Acupuncture therapy for sudden sensorineural hearing loss: a systematic review and meta-analysis of randomized controlled trials.

PubMed

Zhang, Xin-chang; Xu, Xiu-ping; Xu, Wen-tao; Hou, Wen-zhen; Cheng, Ying-ying; Li, Chang-xi; Ni, Guang-xia

2015-01-01

Acupuncture has commonly been used in China, either alone or in combination with Western medicine, to treat sudden sensorineural hearing loss (SSHL). The purpose of this systematic review is to assess the efficacy and safety of acupuncture therapy for patients with SSHL. We searched PubMed, the Cochrane Library, Embase, China National Knowledge Internet (CNKI), Database for Chinese Technical Periodicals (VIP), and Chinese Biomedical literature service system (SinoMed) to collect randomized controlled trials of acupuncture for SSHL published before July 2014. A meta-analysis was conducted according to the Cochrane systematic review method using RevMan 5.2 software. The evidence level for each outcome was assessed using the GRADE methodology. Twelve trials involving 863 patients were included. A meta-analysis showed that the effect of manual acupuncture combined with Western medicine comprehensive treatment (WMCT) was better than WMCT alone (RR 1.33, 95%CI 1.19-1.49) and the same as the effect of electroacupuncture combined with WMCT (RR 1.33, 95%CI 1.19-1.50). One study showed a better effect of electroacupuncture than of WMCT (RR 1.34, 95%CI 1.24-1.45). For mean changes in hearing over all frequencies, the meta-analysis showed a better effect with the combination of acupuncture and WMCT than with WMCT alone (MD 10.85, 95%CI 6.84-14.86). However, the evidence levels for these interventions were low or very low due to a high risk of bias and small sample sizes in the included studies. There was not sufficient evidence showing that acupuncture therapy alone was beneficial for treating SSHL. However, interventions combining acupuncture with WMCT had more efficacious results in the treatment of SSHL than WMCT alone. Electroacupuncture alone might be a viable alternative treatment besides WMCT for SSHL. However, given that there were fewer eligible RCTs and limitations in the included trials, such as methodological drawbacks and small sample sizes, large-scale RCTs are required to confirm the current findings regarding acupuncture therapy for SSHL.

Simultaneous versus Sequential Intratympanic Steroid Treatment for Severe-to-Profound Sudden Sensorineural Hearing Loss.

PubMed

Yoo, Myung Hoon; Lim, Won Sub; Park, Joo Hyun; Kwon, Joong Keun; Lee, Tae-Hoon; An, Yong-Hwi; Kim, Young-Jin; Kim, Jong Yang; Lim, Hyun Woo; Park, Hong Ju

2016-01-01

Severe-to-profound sudden sensorineural hearing loss (SSNHL) has a poor prognosis. We aimed to compare the efficacy of simultaneous and sequential oral and intratympanic steroids for this condition. Fifty patients with severe-to-profound SSNHL (>70 dB HL) were included from 7 centers. The simultaneous group (27 patients) received oral and intratympanic steroid injections for 2 weeks. The sequential group (23 patients) was treated with oral steroids for 2 weeks and intratympanic steroids for the subsequent 2 weeks. Pure-tone averages (PTA) and word discrimination scores (WDS) were compared before treatment and 2 weeks and 1 and 2 months after treatment. Treatment outcomes according to the modified American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria were also analyzed. The improvement in PTA and WDS at the 2-week follow-up was 23 ± 21 dB HL and 20 ± 39% in the simultaneous group and 31 ± 29 dB HL and 37 ± 42% in the sequential group; this was not statistically significant. Complete or partial recovery at the 2-week follow-up was observed in 26% of the simultaneous group and 30% of the sequential group; this was also not significant. The improvement in PTA and WDS at the 2-month follow-up was 40 ± 20 dB HL and 37 ± 35% in the simultaneous group and 41 ± 25 dB HL and 48 ± 41% in the sequential group; this was not statistically significant. Complete or partial recovery at the 2-month follow-up was observed in 33% of the simultaneous group and 35% of the sequential group; this was also not significant. Seven patients in the sequential group did not need intratympanic steroid injections for sufficient improvement after oral steroids alone. Simultaneous oral/intratympanic steroid treatment yielded a recovery similar to that produced by sequential treatment. Because the addition of intratympanic steroids can be decided upon based on the improvement after an oral steroid, the sequential regimen can be recommended to avoid unnecessary intratympanic injections. © 2017 S. Karger AG, Basel.

Cerebellar infarction in the territory of the medial branch of the superior cerebellar artery.

PubMed

Sohn, Sung-Il; Lee, Hyung; Lee, Seong-Ryong; Baloh, Robert W

2006-01-10

The authors studied 14 patients with an isolated cerebellar infarct in the territory of the medial branch of the superior cerebellar artery (MSCA). The most common clinical finding was severe gait ataxia with sudden falling (n = 9) or severe veering (n = 2). Cerebellar dysarthria was found in 8 patients. Eight patients had a mild unilateral limb ataxia. These findings emphasize that MSCA territory cerebellar infarction presented with the prominent gait ataxia and cerebellar dysarthria.

Inner Ear Drug Delivery for Auditory Applications

PubMed Central

Swan, Erin E. Leary; Mescher, Mark J.; Sewell, William F.; Tao, Sarah L.; Borenstein, Jeffrey T.

2008-01-01

Many inner ear disorders cannot be adequately treated by systemic drug delivery. A blood-cochlear barrier exists, similar physiologically to the blood-brain barrier, which limits the concentration and size of molecules able to leave the circulation and gain access to the cells of the inner ear. However, research in novel therapeutics and delivery systems has led to significant progress in the development of local methods of drug delivery to the inner ear. Intratympanic approaches, which deliver therapeutics to the middle ear, rely on permeation through tissue for access to the structures of the inner ear, whereas intracochlear methods are able to directly insert drugs into the inner ear. Innovative drug delivery systems to treat various inner ear ailments such as ototoxicity, sudden sensorineural hearing loss, autoimmune inner ear disease, and for preserving neurons and regenerating sensory cells are being explored. PMID:18848590

38 CFR 17.149 - Sensori-neural aids.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 1 2014-07-01 2014-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact lenses...

38 CFR 17.149 - Sensori-neural aids.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 1 2012-07-01 2012-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact lenses...

38 CFR 17.149 - Sensori-neural aids.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 1 2013-07-01 2013-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact lenses...

38 CFR 17.149 - Sensori-neural aids.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 1 2011-07-01 2011-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact lenses...

Assessment of auditory and psychosocial handicap associated with unilateral hearing loss among Indian patients.

PubMed

Augustine, Ann Mary; Chrysolyte, Shipra B; Thenmozhi, K; Rupa, V

2013-04-01

In order to assess psychosocial and auditory handicap in Indian patients with unilateral sensorineural hearing loss (USNHL), a prospective study was conducted on 50 adults with USNHL in the ENT Outpatient clinic of a tertiary care centre. The hearing handicap inventory for adults (HHIA) as well as speech in noise and sound localization tests were administered to patients with USNHL. An equal number of age-matched, normal controls also underwent the speech and sound localization tests. The results showed that HHIA scores ranged from 0 to 60 (mean 20.7). Most patients (84.8 %) had either mild to moderate or no handicap. Emotional subscale scores were higher than social subscale scores (p = 0.01). When the effect of sociodemographic factors on HHIA scores was analysed, educated individuals were found to have higher social subscale scores (p = 0.04). Age, sex, side and duration of hearing loss, occupation and income did not affect HHIA scores. Speech in noise and sound localization were significantly poorer in cases compared to controls (p < 0.001). About 75 % of patients refused a rehabilitative device. We conclude that USNHL in Indian adults does not usually produce severe handicap. When present, the handicap is more emotional than social. USNHL significantly affects sound localization and speech in noise. Yet, affected patients seldom seek a rehabilitative device.

Amplification in the rehabilitation of unilateral deafness: speech in noise and directional hearing effects with bone-anchored hearing and contralateral routing of signal amplification.

PubMed

Lin, Li-Mei; Bowditch, Stephen; Anderson, Michael J; May, Bradford; Cox, Kenneth M; Niparko, John K

2006-02-01

Vibromechanical stimulation with a semi-implantable bone conductor (Entific BAHA device) overcomes some of the head-shadow effects in unilateral deafness. What specific rehabilitative benefits are observed when the functional ear exhibits normal hearing versus moderate sensorineural hearing loss (SNHL)? The authors conducted a prospective trial of subjects with unilateral deafness in a tertiary care center. This study comprised adults with unilateral deafness (pure-tone average [PTA] > 90 dB; Sp.D. < 20%) and either normal monaural hearing (n = 18) or moderate SNHL (PTA = 25-50 dB: Sp.D. > 75%) in the contralateral functional ear (n = 5). Subjects were fit with contralateral routing of signal (CROS) devices for 1 month and tested before (mastoid) implantation, fitting, and testing with a bone-anchored hearing aid (BAHA). Outcome measures were: 1) subjective benefit; 2) source localization tests (Source Azimuth Identification in Noise Test [SAINT]); 3) speech discrimination in quiet and in noise assessed with Hearing In Noise Test (HINT) protocols. There was consistent satisfaction with BAHA amplification and poor acceptance of CROS amplification. General directional hearing decreased with CROS use and was unchanged by BAHA and directional microphone aids. Relative to baseline and CROS, BAHA produced significantly better speech recognition in noise. Twenty-two of 23 subjects followed up in this study continue to use their BAHA device over an average follow-up period of 30.24 months (range, 51-12 months). BAHA amplification on the side of a deaf ear yields greater benefit in subjects with monaural hearing than does CROS amplification. Advantages likely related to averting the interference of speech signals delivered to the better ear, as occurs with conventional CROS amplification, while alleviating the negative head-shadow effects of unilateral deafness. The advantages of head-shadow reduction in enhancing speech recognition with noise in the hearing ear outweigh disadvantages inherent in head-shadow reduction that can occur by introducing noise from the deaf side. The level of hearing impairment correlates with incremental benefit provided by the BAHA. Patients with a moderate SNHL in the functioning ear perceived greater increments in benefit, especially in background noise, and demonstrated greater improvements in speech understanding with BAHA amplification.

Safety and otoprotection of metformin in radiation-induced sensorineural hearing loss in the guinea pig.

PubMed

Mujica-Mota, Mario A; Salehi, Pezhman; Devic, Slobodan; Daniel, Sam J

2014-05-01

There is currently no treatment available to prevent radiation-induced sensorineural hearing loss. Metformin has antineoplastic effects and is able to regulate the mitochondrial production of reactive oxygen species after cellular stress, which is one of the mechanisms involved in apoptosis after radiation damage. The objective of this study was to determine the safety and radioprotective properties of metformin against radiation-induced cochlear damage both in vitro and in vivo. In vitro and prospective animal study. Animal Care Facilities of the Montreal Children's Hospital Research Institute. Cultured auditory hair cells (HEI-OC1) were exposed to different concentrations of metformin to determine its safety. Cells were incubated with different metformin concentrations and subjected to radiation. Cell viability after experiments was determined with the 3-(4,5-dimethylthiazol-2-yl)-5-(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)-2H-tetrazolium (MTS) assay. Sixteen guinea pigs were divided in 2 groups: drinking tap water (n = 8) and drinking water containing metformin (n = 8). The animals were unilaterally irradiated for 20 days (total dose 70 Gy), and the ears were divided in 4 groups: control (n = 8), irradiated (n = 8), metformin (n = 8), and experimental (n = 8). Auditory brainstem responses were assessed before and 1, 6, and 16 weeks after completion of radiotherapy. Metformin was not cytotoxic or radioprotective in cultured auditory hair cells. Experimental ears had less hearing loss than radiated ones; however, differences were not statistically significant (P > .05). Metformin is not ototoxic or radioprotective in vitro or in vivo. Ears solely subjected to metformin had better hearing thresholds than the rest of the groups.

Acute auditory agnosia as the presenting hearing disorder in MELAS.

PubMed

Miceli, Gabriele; Conti, Guido; Cianfoni, Alessandro; Di Giacopo, Raffaella; Zampetti, Patrizia; Servidei, Serenella

2008-12-01

MELAS is commonly associated with peripheral hearing loss. Auditory agnosia is a rare cortical auditory impairment, usually due to bilateral temporal damage. We document, for the first time, auditory agnosia as the presenting hearing disorder in MELAS. A young woman with MELAS (A3243G mtDNA mutation) suffered from acute cortical hearing damage following a single stroke-like episode, in the absence of previous hearing deficits. Audiometric testing showed marked central hearing impairment and very mild sensorineural hearing loss. MRI documented bilateral, acute lesions to superior temporal regions. Neuropsychological tests demonstrated auditory agnosia without aphasia. Our data and a review of published reports show that cortical auditory disorders are relatively frequent in MELAS, probably due to the strikingly high incidence of bilateral and symmetric damage following stroke-like episodes. Acute auditory agnosia can be the presenting hearing deficit in MELAS and, conversely, MELAS should be suspected in young adults with sudden hearing loss.

Contribution of audiovestibular tests to the topographic diagnosis of sudden deafness

PubMed Central

Oiticica, Jeanne; Bittar, Roseli Saraiva Moreira; Castro, Claudio Campi de; Grasel, Signe; Pereira, Larissa Vilela; Bastos, Sandra Lira; Ramos, Alice Carolina Mataruco; Beck, Roberto

2013-01-01

Summary Introduction: Sudden hearing loss (SHL) is an ENT emergency defined as sensorineural hearing loss (SNHL) ≥ 30 dB HL affecting at least 3 consecutive tonal frequencies, showing a sudden onset, and occurring within 3 days. In cases of SHL, a detailed investigation should be performed in order to determine the etiology and provide the best treatment. Otoacoustic emission (OAE) analysis, electronystagmography (ENG), bithermal caloric test (BCT), and vestibular evoked myogenic potential (VEMP) assessments may be used in addition to a number of auxiliary methods to determine the topographic diagnosis. Objective To evaluate the contribution of OAE analysis, BCT, VEMP assessment, and magnetic resonance imaging (MRI) to the topographic diagnosis of SHL. Method Cross-sectional and retrospective studies of 21 patients with SHL, as defined above, were performed. The patients underwent the following exams: audiometry, tympanometry, OAE analysis, BCT, VEMP assessment, and MRI. Sex, affected side, degree of hearing loss, and cochleovestibular test results were described and correlated with MRI findings. Student's t-test was used for analysis of qualitative variables (p < 0.05). Results The mean age of the 21 patients assessed was 52.5 ± 15.3 years; 13 (61.9%) were women and 8 (38.1%) were men. Most (55%) had severe hearing loss. MRI changes were found in 20% of the cases. When the audiovestibular test results were added to the MRI findings, the topographic SHL diagnosis rate increased from 20% to 45%. Conclusion Only combined analysis via several examinations provides a precise topographic diagnosis. Isolated data do not provide sufficient evidence to establish the extent of involvement and, hence, a possible etiology. PMID:25992027

Noonan Syndrome: An Underestimated Cause of Severe to Profound Sensorineural Hearing Impairment. Which Clues to Suspect the Diagnosis?

PubMed

Ziegler, Alban; Loundon, Natalie; Jonard, Laurence; Cavé, Hélène; Baujat, Geneviève; Gherbi, Souad; Couloigner, Vincent; Marlin, Sandrine

2017-09-01

To highlight Noonan syndrome as a clinically recognizable cause of severe to profound sensorineural hearing impairment. New clinical cases and review. Patients evaluated for etiological diagnosis by a medical geneticist in a reference center for hearing impairment. Five patients presenting with confirmed Noonan syndrome and profound sensorineural hearing impairment. Diagnostic and review of the literature. Five patients presented with profound sensorineural hearing impairment and molecularly confirmed Noonan syndrome. Sensorineural hearing impairment has been progressive for three patients. Cardiac echography identified pulmonary stenosis in two patients and was normal for the three other patients. Short stature was found in two patients. Mild intellectual disability was found in one patient. Inconspicuous clinical features as facial dysmorphism, cryptorchidism, or easy bruising were of peculiar interest to reach the diagnosis of Noonan syndrome. Profound sensorineural hearing impairment can be the main feature of Noonan syndrome. Associated features are highly variable; thus, detailed medical history and careful physical examination are mandatory to consider the diagnosis in case of a sensorineural hearing impairment.

[Successful treatment with anti-epileptic-drug of an 83-year-old man with musical hallucinosis].

PubMed

Futamura, Akinori; Katoh, Hirotaka; Kawamura, Mitsuru

2014-05-01

An 83-year-old man with 3 years symptomatic hearing loss suddenly experienced musical hallucinosis. He heard children's songs, folk songs, military songs, and the Japanese national anthem for seven months every day. He sometime had paroxysmal nausea, dull headaches and depressive mood. On examination he had no psychosis or neurological symptoms except sensorineural hearing loss in both ears. MRI brain imaging and electroencephalography showed no significant abnormalities, however ¹²³I-IMP brain SPECT showed decreased activity in the right temporal lobe and increased activity in the left temporal and parietal lobes. Late phase ¹²³I-iomazenil brain SPECT showed decreased accumulation in the right temporal lobe compared to the early phase. This indicates right temporal lobe epilepsy. He was diagnosed with epilepsy because of paroxysmal nausea and headache and the laterality of ¹²³I-IMP brain SPECT and ¹²³I-iomazenil brain SPECT. The musical hallucinosis was much reduced by carbamazepine 200mg per day. Nine months after beginning carbamazepine we detected decreased activity in the right temporal lobe and increased activity in left temporal and parietal lobes was improved. We do not believe he had epileptogenic musical hallucinosis because his musical hallusinosis was neither paroxysmal nor lateral. We diagnosed auditory Charles Bonnet syndrome with onset 3 years after sensorineural hearing loss due to reversible epileptic like discharge in temporal and parietal lobes. There is no established treatment for musical hallucinosis, but anti-epileptic drugs may be of some help.

Persistent pseudomyopia following a whiplash injury in a previously emmetropic woman.

PubMed

Hughes, Fintan E; Treacy, Maxwell P; Duignan, Emma S; Mullaney, Paul B

2017-12-01

Accommodative spasm, which manifests as ciliary muscle spasm, convergent strabismus or miosis, is a recognised consequence of head trauma. In whiplash cases, cervical spine hyperextension poses a risk of contra-coup injury and brainstem trauma, and is known to affect the visual system. However, to date, no cases of accommodative spasm due to whiplash injury have been reported. We present the case of a 34-year-old female who developed sudden onset blurred distance vision after a rear impact car crash, having previously been emmetropic. Her unaided distance visual acuity was 20/70 in the right eye and 20/20 in the left. Best-corrected visual acuity in the right eye was 20/20 with a correction that progressed from -1.75 to -3.50 DS over the 12 months following the accident.This patient's sudden unilateral myopia, with unilaterally increased amplitude of accommodation suggests pseudomyopia due to accommodative spasm. Magnetic resonance imaging showed no evidence of injury to her brain stem, frontal lobes or oculomotor nerve. The patient is now well adjusted with a -3.50DS corrective lens for the right eye. The accommodation reflex is susceptible to injury at the occipital lobe, frontal eye fields, Edinger-Westphal nuclei and oculomotor nerves. As such it should be examined in patients who present with visual disturbances following whiplash injury.It is important that such cases are identified at presentation, as early intervention can improve outcomes in accommodative spasm and reduce the long term psychological effects often associated with whiplash injuries.

Sequential Bilateral Cochlear Implantation in a Patient with Bilateral Meniere’s Disease

PubMed Central

Holden, Laura K.; Neely, J. Gail; Gotter, Brenda D.; Mispagel, Karen M.; Firszt, Jill B.

2012-01-01

This case study describes a 45 year old female with bilateral, profound sensorineural hearing loss due to Meniere’s disease. She received her first cochlear implant in the right ear in 2008 and the second cochlear implant in the left ear in 2010. The case study examines the enhancement to speech recognition, particularly in noise, provided by bilateral cochlear implants. Speech recognition tests were administered prior to obtaining the second implant and at a number of test intervals following activation of the second device. Speech recognition in quiet and noise as well as localization abilities were assessed in several conditions to determine bilateral benefit and performance differences between ears. The results of the speech recognition testing indicated a substantial improvement in the patient’s ability to understand speech in noise and her ability to localize sound when using bilateral cochlear implants compared to using a unilateral implant or an implant and a hearing aid. In addition, the patient reported considerable improvement in her ability to communicate in daily life when using bilateral implants versus a unilateral implant. This case suggests that cochlear implantation is a viable option for patients who have lost their hearing to Meniere’s disease even when a number of medical treatments and surgical interventions have been performed to control vertigo. In the case presented, bilateral cochlear implantation was necessary for this patient to communicate successfully at home and at work. PMID:22463939

A homozygous MYO7A mutation associated to Usher syndrome and unilateral auditory neuropathy spectrum disorder.

PubMed

Xia, Hong; Hu, Pengzhi; Yuan, Lamei; Xiong, Wei; Xu, Hongbo; Yi, Junhui; Yang, Zhijian; Deng, Xiong; Guo, Yi; Deng, Hao

2017-10-01

Usher syndrome (USH) is an autosomal recessive disorder characterized by sensorineural hearing loss, progressive visual loss and night blindness due to retinitis pigmentosa (RP), with or without vestibular dysfunction. The purpose of this study was to detect the causative gene in a consanguineous Chinese family with USH. A c.3696_3706del (p.R1232Sfs*72) variant in the myosin VIIa gene (MYO7A) was identified in the homozygous state by exome sequencing. The co‑segregation of the MYO7A c.3696_3706del variant with the phenotype of deafness and progressive visual loss in the USH family was confirmed by Sanger sequencing. The variant was absent in 200 healthy controls. Therefore, the c.3696_3706del variant may disrupt the interaction between myosin VIIa and other USH1 proteins, and impair melanosome transport in retinal pigment epithelial cells. Notably, bilateral auditory brainstem responses were absent in two patients of the USH family, while distortion product otoacoustic emissions were elicited in the right ears of the two patients, consistent with clinical diagnosis of unilateral auditory neuropathy spectrum disorder. These data suggested that the homozygous c.3696_3706del variant in the MYO7A gene may be the disease‑causing mutation for the disorder in this family. These findings broaden the phenotype spectrum of the MYO7A gene, and may facilitate understanding of the molecular pathogenesis of the disease, and genetic counseling for the family.

Prevalence of unilateral and bilateral deafness in border collies and association with phenotype.

PubMed

Platt, Simon; Freeman, Julia; di Stefani, Alberta; Wieczorek, Lara; Henley, William

2006-01-01

Congenital sensorineural deafness (CSD) occurs in Border Collies, but its prevalence and inheritance are unknown. This study estimated the prevalence of CSD in Border Collies and investigated its association with phenotypic attributes linked to the merle gene, including coat pigmentation and iris color. Deafness in Border Collies is associated with pigmentation patterns linked to the merle gene. A total of 2597 Border Collies from the United Kingdom. A retrospective study of Border Collies tested, during 1994-2002, by using brainstem auditory evoked responses. Associations between deafness and phenotypic attributes were assessed by using generalized logistic regression. The prevalence of CSD in puppies was estimated as 2.8%. The corresponding rates of unilateral and bilateral CSD were 2.3 and 0.5%, respectively. Adjustment for clustering of hearing status by litter reduced the overall prevalence estimate to 1.6%. There was no association between CSD and sex (P = .2). Deaf Border Collies had higher rates of merle coat pigmentation, blue iris pigment, and excess white on the head than normal hearing Border Collies (all P < .001). The odds of deafness were increased by a factor of 14 for Border Collies with deaf dams, relative to the odds for dogs with normal dams (P = .007), after adjustment for phenotypic attributes. Associations between CSD and pigmentation patterns linked to the merle gene were demonstrated for Border Collies. Evidence for an inherited component to CSD in Border Collies supports selective breeding from only tested and normal parents to reduce the prevalence of this disease.

The effect of symmetrical and asymmetrical hearing impairment on music quality perception.

PubMed

Cai, Yuexin; Zhao, Fei; Chen, Yuebo; Liang, Maojin; Chen, Ling; Yang, Haidi; Xiong, Hao; Zhang, Xueyuan; Zheng, Yiqing

2016-09-01

The purpose of this study was to investigate the effect of symmetrical, asymmetrical and unilateral hearing impairment on music quality perception. Six validated music pieces in the categories of classical music, folk music and pop music were used to assess music quality in terms of its 'pleasantness', 'naturalness', 'fullness', 'roughness' and 'sharpness'. 58 participants with sensorineural hearing loss [20 with unilateral hearing loss (UHL), 20 with bilateral symmetrical hearing loss (BSHL) and 18 with bilateral asymmetrical hearing loss (BAHL)] and 29 normal hearing (NH) subjects participated in the present study. Hearing impaired (HI) participants had greater difficulty in overall music quality perception than NH participants. Participants with BSHL rated music pleasantness and naturalness to be higher than participants with BAHL. Moreover, the hearing thresholds of the better ears from BSHL and BAHL participants as well as the hearing thresholds of the worse ears from BSHL participants were negatively correlated to the pleasantness and naturalness perception. HI participants rated the familiar music pieces higher than unfamiliar music pieces in the three music categories. Music quality perception in participants with hearing impairment appeared to be affected by symmetry of hearing loss, degree of hearing loss and music familiarity when they were assessed using the music quality rating test (MQRT). This indicates that binaural symmetrical hearing is important to achieve a high level of music quality perception in HI listeners. This emphasizes the importance of provision of bilateral hearing assistive devices for people with asymmetrical hearing impairment.

Congenital cytomegalovirus infection as a cause of sensorineural hearing loss in a highly immune population.

PubMed

Yamamoto, Aparecida Y; Mussi-Pinhata, Marisa Marcia; Isaac, Myriam de Lima; Amaral, Fabiana R; Carvalheiro, Cristina G; Aragon, Davi C; Manfredi, Alessandra K da Silva; Boppana, Suresh B; Britt, William J

2011-12-01

The burden of congenital cytomegalovirus (CMV)-associated sensorineural hearing loss (SNHL) in populations with CMV seroprevalence approaching 100% is unknown. The purpose of this study was to assess the rate, associated factors, and predictors of SNHL in CMV-infected infants identified by newborn screening in a highly seropositive maternal population. Newborns with positive saliva CMV-DNA that was confirmed by virus isolation in the first 2 weeks of life were enrolled in a prospective follow-up study to monitor hearing outcome. Of 12,195 infants screened, 121 (1%) were infected with CMV and 12 (10%) had symptomatic infection at birth. Hearing function could be assessed in 102/121 children who underwent at least one auditory brainstem evoked response testing at a median age of 12 months. SNHL was observed in 10/102 (9.8%; 95% confidence interval: 5.1-16.7) children. Median age at the latest hearing evaluation was 47 months (12-84 months). Profound loss (>90 dB) was found in 4/5 children with bilateral SNHL while all 5 children with unilateral loss had moderate to severe deficit. The presence of symptomatic infection at birth (odds ratio, 38.1; 95% confidence interval: 1.6-916.7) was independently associated with SNHL after adjusting for intrauterine growth restriction, gestational age, gravidity, and maternal age. Among 10 infants with SNHL, 6 (60%) were born to mothers with nonprimary CMV infection. Even in populations with near universal immunity to CMV, congenital CMV infection is a significant cause of SNHL demonstrating the importance of CMV as a major cause of SNHL in children worldwide. As in other populations, SNHL is more frequently observed in symptomatic CMV infection.

[Clinical features of a Chinese pedigree with Waardenburg syndrome type 2].

PubMed

Yang, Shu-zhi; Yuan, Hui-jun; Bai, Lin-na; Cao, Ju-yang; Xu, Ye; Shen, Wei-dong; Ji, Fei; Yang, Wei-yan

2005-10-12

To investigate detailed clinical features of a Chinese pedigree with Waardenburg syndrome type 2. Members of this pedigree were interviewed to identify personal or family medical histories of hearing loss, the use of aminoglycosides, and other clinical abnormalities by filling questionnaire. The audiological and other clinical evaluations of the proband and other members of this family were conducted, including pure-tone audiometry, immittance and auditory brain-stem response and ophthalmological, dermatologic, hair, temporal bone CT examinations. This family is categorized as Waardenburg syndrome type 2 according to its clinical features. It's an autosomal dominant disorder with incomplete penetrance. The clinical features varied greatly among family members and characterized by sensorineural hearing loss, heterochromia irides, freckle on the face and premature gray hair. Hearing loss can be unilateral or bilateral, congenital or late onset in this family. This Chinese family has some unique clinical features comparing with the international diagnostic criteria for Waardenburg syndrome. This study may provide some evidences to amend the diagnostic criteria for Waardenburg syndrome in Chinese population.

Alteration of frequency range for binaural beats in acute low-tone hearing loss.

PubMed

Karino, Shotaro; Yamasoba, Tatsuya; Ito, Ken; Kaga, Kimitaka

2005-01-01

The effect of acute low-tone sensorineural hearing loss (ALHL) on the interaural frequency difference (IFD) required for perception of binaural beats (BBs) was investigated in 12 patients with unilateral ALHL and 7 patients in whom ALHL had lessened. A continuous pure tone of 30 dB sensation level at 250 Hz was presented to the contralateral, normal-hearing ear. The presence of BBs was determined by a subjective yes-no procedure as the frequency of a loudness-balanced test tone was gradually adjusted around 250 Hz in the affected ear. The frequency range in which no BBs were perceived (FRNB) was significantly wider in the patients with ALHL than in the controls, and FRNBs became narrower in the recovered ALHL group. Specifically, detection of slow BBs with a small IFD was impaired in this limited (10 s) observation period. The significant correlation between the hearing level at 250 Hz and FRNBs suggests that FRNBs represent the degree of cochlear damage caused by ALHL.

Bilateral Ramsay Hunt syndrome in a diabetic patient

PubMed Central

Syal, Rajan; Tyagi, Isha; Goyal, Amit

2004-01-01

Background Herpes zoster oticus accounts for about 10% cases of facial palsy, which is usually unilateral and complete and full recovery occurs in only about 20% of untreated patients. Bilateral herpes zoster oticus can sometime occur in immunocompromised patients, though incidence is very rare. Case presentation Diabetic male, 57 year old presented to us with bilateral facial palsy due to herpes zoster oticus. Patient was having bilateral mild to moderate sensorineural hearing loss. Patient was treated with appropriate metabolic control, anti-inflammatory drugs and intravenous acyclovir. Due to uncontrolled diabetes, glucocorticoids were not used in this patient. Significant improvement in hearing status and facial nerve functions were seen in this patient. Conclusions Herpes zoster causes severe infections in diabetic patients and can be a cause of bilateral facial palsy and bilateral Ramsay Hunt syndrome. Herpes zoster in diabetic patients should be treated with appropriate metabolic control, NSAIDS and intravenous acyclovir, which we feel should be started at the earliest. Glucocorticoids should be avoided in diabetic patients. PMID:15575957

Bilateral Ramsay Hunt syndrome in a diabetic patient.

PubMed

Syal, Rajan; Tyagi, Isha; Goyal, Amit

2004-12-02

BACKGROUND: Herpes zoster oticus accounts for about 10% cases of facial palsy, which is usually unilateral and complete and full recovery occurs in only about 20% of untreated patients. Bilateral herpes zoster oticus can sometime occur in immunocompromised patients, though incidence is very rare. CASE PRESENTATION: Diabetic male, 57 year old presented to us with bilateral facial palsy due to herpes zoster oticus. Patient was having bilateral mild to moderate sensorineural hearing loss. Patient was treated with appropriate metabolic control, anti-inflammatory drugs and intravenous acyclovir. Due to uncontrolled diabetes, glucocorticoids were not used in this patient. Significant improvement in hearing status and facial nerve functions were seen in this patient. CONCLUSIONS: Herpes zoster causes severe infections in diabetic patients and can be a cause of bilateral facial palsy and bilateral Ramsay Hunt syndrome. Herpes zoster in diabetic patients should be treated with appropriate metabolic control, NSAIDS and intravenous acyclovir, which we feel should be started at the earliest. Glucocorticoids should be avoided in diabetic patients.

Sub-acute neuropathy in patients with African tick bite fever.

PubMed

Jensenius, Mogens; Fournier, Pierre-Edouard; Fladby, Tormod; Hellum, Kjell Block; Hagen, Tormod; Priø, Tine; Christiansen, Merete Skovdal; Vene, Sirkka; Raoult, Didier; Myrvang, Bjørn

2006-01-01

African tick bite fever (ATBF) caused by Rickettsia africae is an emerging health problem in travellers to sub-Saharan Africa. We here present 6 patients with evidence of long-lasting sub-acute neuropathy following ATBF contracted during safari trips to southern Africa. Three patients developed radiating pain, paresthaesia and/or motor weakness of extremities, 2 had hemi-facial pain and paresthaesia, and 1 developed unilateral sensorineural hearing loss. When evaluated 3-26 months after symptom onset, cerebrospinal fluid samples from 5 patients were negative for R. africae PCR and serology, but revealed elevated protein content in 3 and mild pleocytosis in 1 case. Despite extensive investigations, no plausible alternative causes of neuropathy could be identified. Treatment with doxycycline in 2 patients had no clinical effect. Given the current increase of international safari tourism to sub-Saharan Africa, more cases of sub-acute neuropathy following ATBF may well be encountered in Europe and elsewhere in the y to come.

Evaluation of cochlear involvement by distortion product otoacoustic emission in Behçet's disease.

PubMed

Dagli, Muharrem; Eryilmaz, Adil; Tanrikulu, Salih; Aydin, Acar; Gonul, Muzeyyen; Gul, Ulker; Gocer, Celil

2008-09-01

The aim of this study was to investigate cochlear involvement in patients with Behçet's disease. Twenty-six Behçet's disease patients (52 ears) and 24 sex and age-matched healthy control subjects (48 ears) were included in the study. Pure-tone audiometry at frequencies 250, 500, 1000, 2000, 4000, 6000 Hz, immittance measures including tympanometry and acoustic reflex testing and DPOAE (distortion product otoacoustic emission) testing were performed in the patients and controls. A sensorineural hearing loss was found in eight patients (30.7%) as it was bilateral in five and unilateral in three patients. Although no typical audiometric configuration was found, one patient had a flat type audiogram, and the others had a high frequency hearing loss. The DPOAE responses of the patients and controls were significantly different in all frequencies (p<0.05). These results indicate that cochlea is affected by damage of outer hair cells in Behçet's disease.

The dissociation of video head impulse test (vHIT) and bithermal caloric test results provide topological localization of vestibular system impairment in patients with "definite" Ménière's disease.

PubMed

McCaslin, Devin L; Rivas, Alejandro; Jacobson, Gary P; Bennett, Marc L

2015-03-01

We report 3 patients with Ménière's disease and describe how the combination of audiometry, video head impulse testing, and caloric results may prove helpful in the diagnosis of Ménière's disease. Three patients with "definite" Ménière's disease were evaluated in a tertiary care medical center. Each patient underwent videonystagmography, horizontal canal video head impulse testing, and audiometry. All 3 patients demonstrated moderate, flat, sensorineural hearing losses; significant caloric asymmetries; and bilaterally normal video head impulse testing. This pattern of findings suggests differential preservation of high-frequency function (video head impulse testing) with impairment of low-frequency function (unilaterally abnormal caloric test results) in these patients. Ipsilesional abnormal caloric testing in the presence of normal video head impulse testing is a pattern of findings observed in a cohort of patients who have "definite" Ménière's disease.

Clinical and logopaedic results of simultaneous and sequential bilateral implants in children with severe and/or profound bilateral sensorineural hearing loss: A literature review.

PubMed

López-Torrijo, Manuel; Mengual-Andrés, Santiago; Estellés-Ferrer, Remedios

2015-06-01

This article carries out a literature review of the advantages and limitations of the simultaneous bilateral cochlear implantation (SCI) compared to those of the sequential bilateral cochlear implantation (SBCI) and the unilateral cochlear implantation (UCI). The variables analysed in said comparison are: safety and surgical technique, SCI incidence, effectiveness, impact of the inter-implant interval, costs and financing, impact on brain plasticity, impact on speech and language development, main benefits, main disadvantages and concerns, and predictive factors of prognosis. Although the results are not conclusive, all variables analysed seem to point towards observable benefits of SCI in comparison with SBCI or UCI. This tendency should be studied in more depth in multicentre studies with higher methodological rigour, more comprehensive samples and periods and other determining variables (age at the time of implantation, duration and degree of the hearing loss, rehabilitation methodologies used, family involvement, etc.). Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Musical Experience, Sensorineural Auditory Processing, and Reading Subskills in Adults.

PubMed

Tichko, Parker; Skoe, Erika

2018-04-27

Developmental research suggests that sensorineural auditory processing, reading subskills (e.g., phonological awareness and rapid naming), and musical experience are related during early periods of reading development. Interestingly, recent work suggests that these relations may extend into adulthood, with indices of sensorineural auditory processing relating to global reading ability. However, it is largely unknown whether sensorineural auditory processing relates to specific reading subskills, such as phonological awareness and rapid naming, as well as musical experience in mature readers. To address this question, we recorded electrophysiological responses to a repeating click (auditory stimulus) in a sample of adult readers. We then investigated relations between electrophysiological responses to sound, reading subskills, and musical experience in this same set of adult readers. Analyses suggest that sensorineural auditory processing, reading subskills, and musical experience are related in adulthood, with faster neural conduction times and greater musical experience associated with stronger rapid-naming skills. These results are similar to the developmental findings that suggest reading subskills are related to sensorineural auditory processing and musical experience in children.

Bilateral versus unilateral hearing aids for bilateral hearing impairment in adults.

PubMed

Schilder, Anne Gm; Chong, Lee Yee; Ftouh, Saoussen; Burton, Martin J

2017-12-19

Acquired hearing loss is common and its incidence increases markedly with age. In most people, 'age-related' hearing loss is sensorineural (due to the loss of cochlear hair cells) and bilateral, affecting both ears to the same degree. Hearing loss categorised as mild, moderate or severe is primarily managed with hearing aids. People with bilateral hearing loss may be offered one aid, fitted to one specific ear, or two aids fitted to both ears. There is uncertainty about the relative benefits to people with hearing loss of these different strategies. To assess the effects of bilateral versus unilateral hearing aids in adults with a bilateral hearing impairment. The Cochrane ENT Information Specialist searched the ENT Trials Register; Cochrane Register of Studies Online; PubMed; Ovid Embase; CINAHL; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 8 June 2017. Randomised controlled trials (RCTs) comparing the fitting of two versus one ear-level acoustic hearing aids in adults (over 18 years) with a bilateral hearing impairment, both ears being eligible for hearing aids. We used the standard methodological procedures expected by Cochrane. Our primary outcomes were patient preference for bilateral or unilateral aids, hearing-specific health-related quality of life and adverse effects (pain or discomfort in the ear, initiation or exacerbation of middle or outer ear infection). Secondary outcomes included: usage of hearing aids (as measured by, for example, data logging or battery consumption), generic health-related quality of life, listening ability and audiometric benefit measured as binaural loudness summation. We used GRADE to assess the quality of the evidence for each outcome; this is indicated in italics. We included four cross-over RCTs with a total of 209 participants, ranging in age from 23 to 85 and with a preponderance of men. All the studies allowed the use of hearing aids for a total period of at least eight weeks before questions on preference were asked. All studies recruited patients with bilateral hearing loss but there was considerable variation in the types and degree of sensorineural hearing loss that the participants were experiencing.Three of the studies were published before the mid-1990s whereas the fourth study was published in 2011. Therefore, only the most recent study used hearing aids incorporating technology comparable to that currently readily available in high-income settings. Of the four studies, two were conducted in the UK in National Health Service (NHS - public sector) patients: one recruited patients from primary care with hearing loss detected by a screening programme whereas the other recruited patients who had been referred by their primary care practitioner to an otolaryngology department for hearing aids. The other two studies were conducted in the United States: one study recruited only military personnel or veterans with noise-induced hearing loss whereas about half of the participants in the other study were veterans.Only one primary outcome (patient preference) was reported in all studies. The percentage of patients who preferred bilateral hearing aids varied between studies: this was 54% (51 out of 94 participants), 39% (22 out of 56), 55% (16 out of 29) and 77% (23 out of 30), respectively. We have not combined the data from these four studies. The evidence for this outcome is of very low quality.The other outcomes of interest were not reported in the included studies. This review identified only four studies comparing the use of one hearing aid with two. The studies were small and included participants of widely varying ages. There was also considerable variation in the types and degree of sensorineural hearing loss that the participants were experiencing.For the most part, the types of hearing aid evaluated would now be regarded, in high-income settings, as 'old technology', with only one study looking at 'modern' digital aids. However, the relevance of this is uncertain, as this review did not evaluate the differences in outcomes between the different types of technology.We were unable to pool data from the four studies and the very low quality of the evidence leads us to conclude that we do not know if people with hearing loss have a preference for one aid or two. Similarly, we do not know if hearing-specific health-related quality of life, or any of our other outcomes, are better with bilateral or unilateral aids.

Uveitis in spondyloarthritis including psoriatic arthritis, ankylosing spondylitis, and inflammatory bowel disease.

PubMed

Rosenbaum, James T

2015-06-01

Uveitis is a common complication of spondyloarthritis. The "phenotype" of the uveitis characteristic of ankylosing spondylitis (sudden onset, anterior, unilateral, recurrent, more often male) may differ from the phenotype often seen with either psoriatic arthritis or inflammatory bowel disease (insidious onset, anterior and intermediate, bilateral, chronic, and/or more often female). The frequency of uveitis is also much greater in association with ankylosing spondylitis than with either inflammatory bowel disease or psoriasis. Uveitis may affect the choice of therapy and can rarely be a complication of therapy. Uveitis and arthritis also co-exist in several animal models.

Total serum bilirubin levels and sensorineural hearing loss in the US adolescents: NHANES 2007-2010.

PubMed

Zhou, Guoli; Fu, Wenjiang

2018-02-01

We aimed to investigate whether current levels of total serum bilirubin are associated with different subtypes of sensorineural hearing loss (SNHL) in adolescents. A set of cross-sectional data from the National Health and Nutrition Examination Survey (NHANES) (2007-2010) was used. A subset of 1404 adolescents was sampled for measurements of total serum bilirubin, tympanometry, and average pure tone threshold at low-frequencies (LPTA: 500, 1000, 2000 Hz) or high-frequencies (HPTA: 3000, 4000, 6000, and 8000 Hz). SNHL was defined as the hearing loss that had type A tympanograms with a peak admittance of 0.3 ml or greater. Associations between serum bilirubin (square-root transformed) and different subtypes of SNHL were evaluated using binary or multinomial logistic regression models with 4-year sampling weights. The bootstrap method was used for estimation of variance and 10-fold cross-validation for assessment of overfitting issue. Total serum bilirubin levels were found to be associated with any high-frequency (HPTA>15 dB in at least one ear, adjusted odds-ratio (OR a )(bootstrap 95% confidence interval) = 3.29(1.31-8.19), p = 0.011), but not with any low-frequency (LPTA>15 dB in at least one ear), SNHL in the US adolescents. Furthermore, high-frequency SNHL with HPTA>15 dB in both ears (bilateral) or HPTA≥25 dB in at least one ear, compared to that with HPTA>15 dB in one ear only (unilateral) or HPTA = 15-25 dB in at least one ear, had a stronger association with total serum bilirubin levels (OR a  = 5.37(1.27-22.65), p = 0.022 for bilateral; OR a  = 2.64(0.84-8.25), p = 0.094 for unilateral; OR a  = 5.00(0.95-26.58), p = 0.058 for HPTA≥25 dB in at least one ear; as well as OR a  = 3.06(1.15-8.25), p = 0.025 for HPTA = 15-25 dB in at least one ear). No severe overfitting problems were found. Our findings suggest that current levels of total serum bilirubin may be informative in predicting and/or targeting high-frequency SNHL in the US adolescents. Copyright © 2017 Elsevier B.V. All rights reserved.

Musical Experience, Sensorineural Auditory Processing, and Reading Subskills in Adults

PubMed Central

Tichko, Parker; Skoe, Erika

2018-01-01

Developmental research suggests that sensorineural auditory processing, reading subskills (e.g., phonological awareness and rapid naming), and musical experience are related during early periods of reading development. Interestingly, recent work suggests that these relations may extend into adulthood, with indices of sensorineural auditory processing relating to global reading ability. However, it is largely unknown whether sensorineural auditory processing relates to specific reading subskills, such as phonological awareness and rapid naming, as well as musical experience in mature readers. To address this question, we recorded electrophysiological responses to a repeating click (auditory stimulus) in a sample of adult readers. We then investigated relations between electrophysiological responses to sound, reading subskills, and musical experience in this same set of adult readers. Analyses suggest that sensorineural auditory processing, reading subskills, and musical experience are related in adulthood, with faster neural conduction times and greater musical experience associated with stronger rapid-naming skills. These results are similar to the developmental findings that suggest reading subskills are related to sensorineural auditory processing and musical experience in children. PMID:29702572

Unilateral uveitis masquerade syndrome caused by diffuse large B-cell lymphoma diagnosed using multiparametric flow cytometry of the aqueous humor.

PubMed

Monsalvo, Silvia; Serrano, Cristina; Prieto, Elena; Fernández-Sanz, Guillermo; Puente, Maria-Camino; Rodriguez-Pinilla, Maria; Garcia Raso, Aranzazu; Llamas, Pilar; Cordoba, Raul

2017-07-01

The uveitis masquerade syndromes (UMS) are a group of ocular diseases that may mimic chronic intraocular inflammation. Many malignant entities such as non-Hodgkin's lymphomas may masquerade as uveitis. We report a case of an HIV-positive patient with masquerade syndrome presenting unilateral uveitis. 45-year-old Caucasian man with a diagnosis of diffuse large B-cell lymphoma (DLBCL). The patient was diagnosed by a biopsy of an abdominal mass which showed fragments of gastric mucosa with diffuse growth of neoplastic cells. At diagnosis, the patient suffered from unilateral blurring of vision and a sudden decrease of left-eye visual acuity. A slit-lamp examination of the left eye revealed a diagnosis of anterior uveitis. The patient exhibited no signs of posterior uveitis. An anterior-chamber paracentesis was performed and analyzed by multiparameter flow cytometry (MFC), showing cells CD45, CD19, CD20, CD22, and CD38 positives, and moderate expression of CD10 with kappa light chain restriction, showing a monoclonal B-cell population. The patient received CHOP-R with intrathecal methotrexate followed by consolidation high dose methotrexate obtaining a complete response which is ongoing. Differential diagnosis between chronic uveitis and ocular lymphoma may be challenging. We advocate anterior-chamber paracentesis in cases of refractory uveitis in patients with hematologic malignancies. © 2016 International Clinical Cytometry Society. © 2016 International Clinical Cytometry Society.

Adjustment of the dynamic weight distribution as a sensitive parameter for diagnosis of postural alteration in a rodent model of vestibular deficit

PubMed Central

Tighilet, Brahim; Péricat, David; Frelat, Alais; Cazals, Yves; Rastoldo, Guillaume; Boyer, Florent; Dumas, Olivier

2017-01-01

Vestibular disorders, by inducing significant posturo-locomotor and cognitive disorders, can significantly impair the most basic tasks of everyday life. Their precise diagnosis is essential to implement appropriate therapeutic countermeasures. Monitoring their evolution is also very important to validate or, on the contrary, to adapt the undertaken therapeutic actions. To date, the diagnosis methods of posturo-locomotor impairments are restricted to examinations that most often lack sensitivity and precision. In the present work we studied the alterations of the dynamic weight distribution in a rodent model of sudden and complete unilateral vestibular loss. We used a system of force sensors connected to a data analysis system to quantify in real time and in an automated way the weight bearing of the animal on the ground. We show here that sudden, unilateral, complete and permanent loss of the vestibular inputs causes a severe alteration of the dynamic ground weight distribution of vestibulo lesioned rodents. Characteristics of alterations in the dynamic weight distribution vary over time and follow the sequence of appearance and disappearance of the various symptoms that compose the vestibular syndrome. This study reveals for the first time that dynamic weight bearing is a very sensitive parameter for evaluating posturo-locomotor function impairment. Associated with more classical vestibular examinations, this paradigm can considerably enrich the methods for assessing and monitoring vestibular disorders. Systematic application of this type of evaluation to the dizzy or unstable patient could improve the detection of vestibular deficits and allow predicting better their impact on posture and walk. Thus it could also allow a better follow-up of the therapeutic approaches for rehabilitating gait and balance. PMID:29112981

Adjustment of the dynamic weight distribution as a sensitive parameter for diagnosis of postural alteration in a rodent model of vestibular deficit.

PubMed

Tighilet, Brahim; Péricat, David; Frelat, Alais; Cazals, Yves; Rastoldo, Guillaume; Boyer, Florent; Dumas, Olivier; Chabbert, Christian

2017-01-01

Vestibular disorders, by inducing significant posturo-locomotor and cognitive disorders, can significantly impair the most basic tasks of everyday life. Their precise diagnosis is essential to implement appropriate therapeutic countermeasures. Monitoring their evolution is also very important to validate or, on the contrary, to adapt the undertaken therapeutic actions. To date, the diagnosis methods of posturo-locomotor impairments are restricted to examinations that most often lack sensitivity and precision. In the present work we studied the alterations of the dynamic weight distribution in a rodent model of sudden and complete unilateral vestibular loss. We used a system of force sensors connected to a data analysis system to quantify in real time and in an automated way the weight bearing of the animal on the ground. We show here that sudden, unilateral, complete and permanent loss of the vestibular inputs causes a severe alteration of the dynamic ground weight distribution of vestibulo lesioned rodents. Characteristics of alterations in the dynamic weight distribution vary over time and follow the sequence of appearance and disappearance of the various symptoms that compose the vestibular syndrome. This study reveals for the first time that dynamic weight bearing is a very sensitive parameter for evaluating posturo-locomotor function impairment. Associated with more classical vestibular examinations, this paradigm can considerably enrich the methods for assessing and monitoring vestibular disorders. Systematic application of this type of evaluation to the dizzy or unstable patient could improve the detection of vestibular deficits and allow predicting better their impact on posture and walk. Thus it could also allow a better follow-up of the therapeutic approaches for rehabilitating gait and balance.

Ischemia-reperfusion injury of the cochlea: pharmacological strategies for cochlear protection and implications of glutamate and reactive oxygen species.

PubMed

Tabuchi, Keiji; Nishimura, Bungo; Tanaka, Shuho; Hayashi, Kentaro; Hirose, Yuki; Hara, Akira

2010-06-01

A large amount of energy produced by active aerobic metabolism is necessary for the cochlea to maintain its function. This makes the cochlea vulnerable to blockade of cochlear blood flow and interruption of the oxygen supply. Although certain forms of human idiopathic sudden sensorineural hearing loss reportedly arise from ischemic injury, the pathological mechanism of cochlear ischemia-reperfusion injury has not been fully elucidated. Recent animal studies have shed light on the mechanisms of cochlear ischemia-reperfusion injury. It will help in the understanding of the pathology of cochlear ischemia-reperfusion injury to classify this injury into ischemic injury and reperfusion injury. Excitotoxicity, mainly observed during the ischemic period, aggravates the injury of primary auditory neurons. On the other hand, oxidative damage induced by hydroxyl radicals and nitric oxide enhances cochlear reperfusion injury. This article briefly summarizes the generation mechanisms of cochlear ischemia-reperfusion injury and potential therapeutic targets that could be developed for the effective management of this injury type.

Vertigo with sudden hearing loss: audio-vestibular characteristics.

PubMed

Pogson, Jacob M; Taylor, Rachael L; Young, Allison S; McGarvie, Leigh A; Flanagan, Sean; Halmagyi, G Michael; Welgampola, Miriam S

2016-10-01

Acute vertigo with sudden sensorineural hearing loss (SSNHL) is a rare clinical emergency. Here, we report the audio-vestibular test profiles of 27 subjects who presented with these symptoms. The vestibular test battery consisted of a three-dimensional video head impulse test (vHIT) of semicircular canal function and recording ocular and cervical vestibular-evoked myogenic potentials (oVEMP, cVEMP) to test otolith dysfunction. Unlike vestibular neuritis, where the horizontal and anterior canals with utricular function are more frequently impaired, 74 % of subjects with vertigo and SSNHL demonstrated impairment of the posterior canal gain (0.45 ± 0.20). Only 41 % showed impairment of the horizontal canal gains (0.78 ± 0.27) and 30 % of the anterior canal gains (0.79 ± 0.26), while 38 % of oVEMPs [asymmetry ratio (AR) = 41.0 ± 41.3 %] and 33 % of cVEMPs (AR = 47.3 ± 41.2 %) were significantly asymmetrical. Twenty-three subjects were diagnosed with labyrinthitis/labyrinthine infarction in the absence of evidence for an underlying pathology. Four subjects had a definitive diagnosis [Ramsay Hunt Syndrome, vestibular schwannoma, anterior inferior cerebellar artery (AICA) infarction, and traction injury]. Ischemia involving the common-cochlear or vestibulo-cochlear branches of the labyrinthine artery could be the simplest explanation for vertigo with SSNHL. Audio-vestibular tests did not provide easy separation between ischaemic and non-ischaemic causes of vertigo with SSNHL.

Wavelet Entropy and Directed Acyclic Graph Support Vector Machine for Detection of Patients with Unilateral Hearing Loss in MRI Scanning

PubMed Central

Wang, Shuihua; Yang, Ming; Du, Sidan; Yang, Jiquan; Liu, Bin; Gorriz, Juan M.; Ramírez, Javier; Yuan, Ti-Fei; Zhang, Yudong

2016-01-01

Highlights We develop computer-aided diagnosis system for unilateral hearing loss detection in structural magnetic resonance imaging.Wavelet entropy is introduced to extract image global features from brain images. Directed acyclic graph is employed to endow support vector machine an ability to handle multi-class problems.The developed computer-aided diagnosis system achieves an overall accuracy of 95.1% for this three-class problem of differentiating left-sided and right-sided hearing loss from healthy controls. Aim: Sensorineural hearing loss (SNHL) is correlated to many neurodegenerative disease. Now more and more computer vision based methods are using to detect it in an automatic way. Materials: We have in total 49 subjects, scanned by 3.0T MRI (Siemens Medical Solutions, Erlangen, Germany). The subjects contain 14 patients with right-sided hearing loss (RHL), 15 patients with left-sided hearing loss (LHL), and 20 healthy controls (HC). Method: We treat this as a three-class classification problem: RHL, LHL, and HC. Wavelet entropy (WE) was selected from the magnetic resonance images of each subjects, and then submitted to a directed acyclic graph support vector machine (DAG-SVM). Results: The 10 repetition results of 10-fold cross validation shows 3-level decomposition will yield an overall accuracy of 95.10% for this three-class classification problem, higher than feedforward neural network, decision tree, and naive Bayesian classifier. Conclusions: This computer-aided diagnosis system is promising. We hope this study can attract more computer vision method for detecting hearing loss. PMID:27807415

Wavelet Entropy and Directed Acyclic Graph Support Vector Machine for Detection of Patients with Unilateral Hearing Loss in MRI Scanning.

PubMed

Wang, Shuihua; Yang, Ming; Du, Sidan; Yang, Jiquan; Liu, Bin; Gorriz, Juan M; Ramírez, Javier; Yuan, Ti-Fei; Zhang, Yudong

2016-01-01

Highlights We develop computer-aided diagnosis system for unilateral hearing loss detection in structural magnetic resonance imaging.Wavelet entropy is introduced to extract image global features from brain images. Directed acyclic graph is employed to endow support vector machine an ability to handle multi-class problems.The developed computer-aided diagnosis system achieves an overall accuracy of 95.1% for this three-class problem of differentiating left-sided and right-sided hearing loss from healthy controls. Aim: Sensorineural hearing loss (SNHL) is correlated to many neurodegenerative disease. Now more and more computer vision based methods are using to detect it in an automatic way. Materials: We have in total 49 subjects, scanned by 3.0T MRI (Siemens Medical Solutions, Erlangen, Germany). The subjects contain 14 patients with right-sided hearing loss (RHL), 15 patients with left-sided hearing loss (LHL), and 20 healthy controls (HC). Method: We treat this as a three-class classification problem: RHL, LHL, and HC. Wavelet entropy (WE) was selected from the magnetic resonance images of each subjects, and then submitted to a directed acyclic graph support vector machine (DAG-SVM). Results: The 10 repetition results of 10-fold cross validation shows 3-level decomposition will yield an overall accuracy of 95.10% for this three-class classification problem, higher than feedforward neural network, decision tree, and naive Bayesian classifier. Conclusions: This computer-aided diagnosis system is promising. We hope this study can attract more computer vision method for detecting hearing loss.

The Use of Standardized Test Batteries in Assessing the Skill Development of Children with Mild-to-Moderate Sensorineural Hearing Loss.

ERIC Educational Resources Information Center

Plapinger, Donald S.; Sikora, Darryn M.

1995-01-01

This study of 12 children (ages 7-13) with mild to moderate bilateral sensorineural hearing loss found that psychoeducational diagnostic tests standardized on students with normal hearing may be used with confidence to assess both cognitive and academic levels of functioning in students with sensorineural hearing loss. (Author/JDD)

Sudden unexpected death owing to unilateral medial medullary infarction with early involvement of the respiratory center.

PubMed

Hata, Yukiko; Yoshida, Koji; Kinoshita, Koshi; Nishida, Naoki

2014-05-01

A 64-year-old woman was found dead in her home. At autopsy, although relatively fresh bruises were found on her body, no lethal injury was observed in an internal observation. Mild edematous swelling of the right half of the medulla oblongata was observed. There was acute medial medullary infarction (MMI), which mainly involved the nucleus hypoglossi, medial lemniscus, hypoglossal root, inferior olivary nucleus, and pyramidal tract. Subacute infarction of the lower part of the cerebellum was also found, and severe atherosclerosis of the right vertebral artery containing thrombi was found as the culprit lesion. Immunohistochemistry using amyloid precursor protein (APP) was positive in neuronal tissue in the nucleus ambiguus, despite not showing coagulative necrosis in the nucleus. Therefore, acute ischemic necrosis of the nucleus ambiguus, which is considered to be a component of the dorsal respiratory group, may be a significant finding for her expected death. Immunohistochemistry of APP may be useful for confirming the precise extent of acute ischemia in brain stem infarction, such as unilateral MMI. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Hearing loss in very preterm and very low birthweight infants at the age of 5 years in a nationwide cohort.

PubMed

Veen, S; Sassen, M L; Schreuder, A M; Ens-Dokkum, M H; Verloove-Vanhorick, S P; Brand, R; Grote, J J; Ruys, J H

1993-02-01

In a geographically defined population of very preterm and very low birthweight infants (gestational age < 32 weeks and/or birthweight < 1500 g) hearing was evaluated in 890 children by pure-tone audiometry at the age of 5 years. Hearing loss was conductive/unspecified in 123 (13.8%) and sensorineural in 13 (1.5%) children. The prevalence of sensorineural hearing loss was 15 times as high as in 5-7 year old children in the Dutch population at large. The sensorineural hearing loss prevalence in very low birthweight and extremely low birthweight infants was similar. On account of communication disorders 10 (1.1%) children were classified as disabled and 6 (0.7%) as handicapped, following the definitions of the International Classification of Impairments, Disabilities, and Handicaps of the World Health Organisation. Children with conductive hearing loss had a higher risk of impairments, disabilities and handicaps of language and speech development, than children with normal hearing, the difference being statistically significant. The same holds for children with sensorineural hearing loss; moreover they had a significantly higher risk of impairments, disabilities and handicaps of mental development. Overall comparison of children with and without sensorineural hearing loss proved that the children with sensorineural hearing loss had a significantly less favourable outcome, based on 15 perinatal factors simultaneously. The age at which sensorineural hearing loss in very preterm and/or very low birthweight infants is detected has to be improved.

Knowledge of Stroke Risk Factors and Warning Signs in Patients with Recurrent Stroke or Recurrent Transient Ischaemic Attack in Thailand.

PubMed

Saengsuwan, Jittima; Suangpho, Pathitta; Tiamkao, Somsak

2017-01-01

Stroke is a global burden. It is not known whether patients who are most at risk of stroke (recurrent stroke or recurrent transient ischaemic attack) have enough knowledge of stroke risk factors and warning signs. The aim of this study was to assess the knowledge of stroke risk factors and warning signs in this high-risk population. We performed a cross-sectional questionnaire-based study of patients with recurrent stroke or recurrent TIA admitted to Srinagarind Hospital and Khon Kaen Hospital, Thailand. A total of 140 patients were included in the study (age 65.6 ± 11.3 years [mean ± SD], 62 females). Using an open-ended questionnaire, nearly one-third of patients (31.4%) could not name any risk factors for stroke. The most commonly recognized risk factors were hypertension (35%), dyslipidemia (28.6%), and diabetes (22.9%). Regarding stroke warning signs, the most commonly recognized warning signs were sudden unilateral weakness (61.4%), sudden trouble with speaking (25.7%), and sudden trouble with walking, loss of balance, or dizziness (21.4%). Nineteen patients (13.6%) could not identify any warning signs. The results showed that knowledge of stroke obtained from open-ended questionnaires is still unsatisfactory. The healthcare provider should provide structured interventions to increase knowledge and awareness of stroke in these patients.

Recurrent Isolated Oculomotor Nerve Palsy after Radiation of a Mesencephalic Metastasis. Case Report and Mini Review

PubMed Central

Grabau, Olga; Leonhardi, Jochen; Reimers, Carl D.

2014-01-01

Introduction: Recurrent oculomotor nerve palsies are extremely rare clinical conditions. Case report: Here, we report on a unique case of a short-lasting recurrent unilateral incomplete external and complete internal oculomotor nerve palsy. The episodic palsies were probably caused by an ipsilateral mesencephalic metastasis of a breast carcinoma and occurred after successful brain radiation therapy. Discussion: While the pathogenic mechanism remains unclear, the recurrent sudden onset and disappearance of the palsies and their decreasing frequency after antiepileptic treatment suggest the occurrence of epilepsy-like brainstem seizures. A review of case reports of spontaneous reversible oculomotor nerve palsies is presented. PMID:25104947

Assessment of thermotactile and vibrotactile thresholds for detecting sensorineural components of the hand-arm vibration syndrome (HAVS).

PubMed

Ye, Ying; Griffin, Michael J

2018-01-01

Thermotactile thresholds and vibrotactile thresholds are measured to assist the diagnosis of the sensorineural component of the hand-arm vibration syndrome (HAVS). This study investigates whether thermotactile and vibrotactile thresholds distinguish between fingers with and without numbness and tingling. In 60 males reporting symptoms of the hand-arm vibration syndrome, thermotactile thresholds for detecting hot and cold temperatures and vibrotactile thresholds at 31.5 and 125 Hz were measured on the index and little fingers of both hands. In fingers reported to suffer numbness or tingling, hot thresholds increased, cold thresholds decreased, and vibrotactile thresholds at both 31.5 and 125 Hz increased. With sensorineural symptoms on all three phalanges (i.e. numbness or tingling scores of 6), both thermotactile thresholds and both vibrotactile thresholds had sensitivities greater than 80% and specificities around 90%, with areas under the receiver operating characteristic curves around 0.9. There were correlations between all four thresholds, but cold thresholds had greater sensitivity and greater specificity on fingers with numbness or tingling on only the distal phalanx (i.e. numbness or tingling scores of 1) suggesting cold thresholds provide better indications of early sensorineural disorder. Thermotactile thresholds and vibrotactile thresholds can provide useful indications of sensorineural function in patients reporting symptoms of the sensorineural component of HAVS.

Prevalence of deafness and association with coat variations in client-owned ferrets.

PubMed

Piazza, Stéphanie; Abitbol, Marie; Gnirs, Kirsten; Huynh, Minh; Cauzinille, Laurent

2014-05-01

To evaluate the prevalence of congenital sensorineural deafness (CSD) and its association with phenotypic markers in client-owned ferrets. Epidemiological study. 152 healthy European pet ferrets. Brainstem auditory evoked response tests were recorded in ferrets during general anesthesia. Phenotypic markers such as sex, coat color and pattern, coat length (Angora or not), and premature graying trait were assessed. Overall, 44 of the 152 (29%) ferrets were affected by CSD; 10 (7%) were unilaterally deaf, and 34 (22%) were bilaterally deaf. There was no association between CSD and sex or Angora trait, but a strong association between CSD and white patterned coat or premature graying was identified. All panda, American panda, and blaze ferrets were deaf. The ferrets in this study had a high prevalence of CSD that was strictly associated with coat color patterns, specifically white markings and premature graying. This seemed to be an emerging congenital defect in pet ferrets because white-marked coats are a popular new coat color. Breeders should have a greater awareness and understanding of this defect to reduce its prevalence for the overall benefit of the species.

[Sensorineural hearing loss due to neonatal hyperbilirubinemia].

PubMed

Clarós, P; Turcanu, D; Caballero, M; Costa, C; Clavería, M A; Clarós, A; Clarós, A

2003-01-01

In this article, the sensorineural hearing loss is presented as a possible sequelae of neonatal hyperbilirubinemia. In our program of early hipoacusia detection, 241 babies were examined from January 1996 until November 1999; 7 cases had a history of hyperbilirubinemia in the neonatal period and 2 of them were diagnosed of sensorineural hearing loss. We discuss how the bilirubin or any other associated factor might have been the cause and this could explain the selective affectation of some children.

Neurotrophins and electrical stimulation for protection and repair of spiral ganglion neurons following sensorineural hearing loss

PubMed Central

Shepherd, Robert K.; Coco, Anne; Epp, Stephanie B.

2008-01-01

Exogenous neurotrophins (NTs) have been shown to rescue spiral ganglion neurons (SGNs) from degeneration following a sensorineural hearing loss (SNHL). Furthermore, chronic electrical stimulation (ES) has been shown to retard SGN degeneration in some studies but not others. Since there is evidence of even greater SGN rescue when NT administration is combined with ES, we examined whether chronic ES can maintain SGN survival long after cessation of NT delivery. Young adult guinea pigs were profoundly deafened using ototoxic drugs; five days later they were unilaterally implanted with an electrode array and drug delivery system. Brain derived neurotrophic factor (BDNF) was continuously delivered to the scala tympani over a four week period while the animal simultaneously received ES via bipolar electrodes in the basal turn (i.e. turn 1) scala tympani. One cohort (n=5) received ES for six weeks (i.e. including a two week period after the cessation of BDNF delivery; ES6); a second cohort (n=5) received ES for 10 weeks (i.e. a six week period following cessation of BDNF delivery; ES10). The cochleae were harvested for histology and SGN density determined for each cochlear turn for comparison with normal hearing controls (n=4). The withdrawal of BDNF resulted in a rapid loss of SGNs in turns 2–4 of the deafened/BDNF-treated cochleae; this was significant as early as two weeks following removal of the NT when compared with normal controls (p<0.05). Importantly, there was not a significant reduction in SGNs in turn 1 (i.e. adjacent to the electrode array) two and six weeks after NT removal, as compared with normal controls. This result suggests that chronic ES can prevent the rapid loss of SGNs that occurs after the withdrawal of exogenous NTs. Implications for the clinical delivery of NTs are discussed. PMID:18243608

Electrocardiogram Screening in Children with Congenital Sensorineural Hearing Loss: Prevalence and Follow-up of Abnormalities.

PubMed

Farzal, Zainab; Walsh, Jonathan; Ahmad, Faisal I; Roberts, Jason; Ferns, Sunita J; Zdanski, Carlton J

2018-03-01

Objective The purpose is to determine the prevalence of electrocardiogram (ECG) abnormalities, including borderline and prolonged QT, among screened children with sensorineural hearing loss (SNHL) and to analyze their subsequent medical workup. Study Design Institutional Review Board-approved case series with chart review. Setting Tertiary academic center. Subjects and Methods Cases from 1996 to 2014 involving pediatric patients (N = 1994) with SNHL were analyzed. Abnormal ECGs were categorized as borderline/prolonged QT or other. A board-certified pediatric cardiologist retrospectively determined the clinical significance of ECG changes. For follow-up analysis, children with heart disease, known syndromes, or inaccessible records were excluded. Results Among 772 children who had ECGs, 215 (27.8%) had abnormal results: 35 (4.5%) with QT abnormalities and 180 (23.3%) with other abnormalities. For children with QT abnormalities meeting inclusion criteria (n = 30), follow-up measures included cardiology referral (46.6%), repeat ECG by ear, nose, and throat (ENT) specialist (20%), clearance by ENT specialist with clinical correlation and/or comparison with old ECGs (20%), and pediatrician follow-up (6.7%). Documentation of further workup by ENT or referral was absent for 6.7%. For children with other ECG changes meeting inclusion criteria (n = 136), abnormalities were documented for 57 (41.9%); normal QT without other abnormality was documented for 18 (13.2%). The most common follow-up referrals were to pediatricians (16.9%) and cardiologists (10.3%). Among patients with clinically significant non-QT abnormalities mandating further evaluation (n = 122), 38 (31.1%) had documented follow-up in medical records. Conclusion There is a high prevalence of ECG abnormalities among children with congenital SNHL. If findings are confirmed by future studies, screening should be considered for congenital unilateral or bilateral SNHL, regardless of severity. We describe a standardized protocol for ECG screening/follow-up.

Vestibular-evoked myogenic potential in the prediction of recovery from acute low-tone sensorineural hearing loss.

PubMed

Wang, Chi-Te; Fang, Kai-Min; Young, Yi-Ho; Cheng, Po-Wen

2010-04-01

Click and galvanic stimulations of vestibular-evoked myogenic potential (c-VEMP and g-VEMP) were applied to measure the interaural difference (IAD) of saccular responses in patients with acute low-tone sensorineural hearing loss (ALHL). This study intended to explore the relationship between saccular asymmetry and final hearing recovery. We hypothesize that greater extent of saccular dysfunction may be associated with lesser hearing recovery. Twenty-one patients with unilateral ALHL were prospectively enrolled to receive c-VEMP and g-VEMP tests in a random sequence. The IAD of the saccular responses for each patient was measured using three parameters-the raw and corrected amplitudes of c-VEMP, and corrected c-VEMP to g-VEMP amplitude ratio (C/G ratio). The IAD for each parameter was classified as depressed, normal, or augmented by calculating the difference between the affected and unaffected ears and dividing by its sum for both ears. After 3 consecutive months of oral medication and follow-up, 19 patients displayed a hearing recovery of >50%; only two had a recovery of <50%. The significant correlation between the IAD of corrected C/G ratios and hearing recovery demonstrated that subjects with depressed responses had a worse hearing outcome (percent recovery: 51% [45-80%], median [minimum-maximum]), compared with those with normal responses, who exhibited the best recovery (87% [56-100%]), whereas patients with augmented response showed an intermediate recovery (67% [54-100%]; p = 0.02, Kruskal-Wallis test). On the contrary, the raw and corrected amplitudes of c-VEMP did not reveal a significantly different hearing recovery among the three groups of saccular responses. The extent of saccular dysfunction in ALHL might be better explored by combining the results of c-VEMP and g-VEMP. Outcome analysis indicated that the corrected C/G ratio might be a promising prognostic factor for hearing recovery in ALHL.

Cost-effectiveness of pediatric bilateral cochlear implantation in Spain.

PubMed

Pérez-Martín, Jorge; Artaso, Miguel A; Díez, Francisco J

2017-12-01

To determine the incremental cost-effectiveness of bilateral versus unilateral cochlear implantation for 1-year-old children suffering from bilateral sensorineural severe to profound hearing loss from the perspective of the Spanish public health system. Cost-utility analysis. We conducted a general-population survey to estimate the quality-of-life increase contributed by the second implant. We built a Markov influence diagram and evaluated it for a life-long time horizon with a 3% discount rate in the base case. The incremental cost-effectiveness ratio of simultaneous bilateral implantation with respect to unilateral implantation for 1-year-old children with severe to profound deafness is €10,323 per quality-adjusted life year (QALY). For sequential bilateral implantation, it rises to €11,733/QALY. Both options are cost-effective for the Spanish health system, whose willingness to pay is estimated at around €30,000/QALY. The probabilistic sensitivity analysis shows that the probability of bilateral implantation being cost-effective reaches 100% for that cost-effectiveness threshold. Bilateral implantation is clearly cost-effective for the population considered. If possible, it should be done simultaneously (i.e., in one surgical operation), because it is as safe and effective as sequential implantation, and saves costs for the system and for users and their families. Sequential implantation is also cost-effective for children who have received the first implant recently, but it is difficult to determine when it ceases to be so because of the lack of detailed data. These results are specific for Spain, but the model can easily be adapted to other countries. 2C. Laryngoscope, 127:2866-2872, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

Unilateral Hearing Loss Is Associated With Impaired Balance in Children: A Pilot Study.

PubMed

Wolter, Nikolaus E; Cushing, Sharon L; Vilchez-Madrigal, Luis D; James, Adrian L; Campos, Jennifer; Papsin, Blake C; Gordon, Karen A

2016-12-01

To determine if children with unilateral sensorineural hearing loss (UHL) demonstrate impaired balance compared with their normal hearing (NH) peers. Prospective, case-control study. Balance was assessed in14 UHL and 14 NH children using the Bruininks-Oseretsky Test-2 (BOT-2) and time to fall (TTF) in an immersive, virtual-reality laboratory. Postural control was quantified by center of pressure (COP) using force plates. The effect of vision on balance was assessed by comparing scores and COP characteristics on BOT-2 tasks performed with eyes open and closed. Balance ability as measured by the BOT-2 score was significantly worse in children with UHL compared with NH children (p = 0.004). TTF was shorter in children with UHL compared with NH children in the most difficult tasks when visual and somatosensory inputs were limited (p < 0.01). Visual input improved postural control (reduced COP variability) in both groups in all tasks (p < 0.05) but postural control as measured by COP variability was more affected in children with UHL when visual input was removed while performing moderately difficult tasks (i.e., standing on one foot) (p = 0.02). In this pilot study, children with UHL show poorer balance skills than NH children. Significant differences in TTF between the two groups were only seen in the most difficult tasks and therefore may be missed on routine clinical assessment. Children with UHL appear to rely more on vision for maintaining postural control than their NH peers. These findings may point to deficits not only in the hearing but also the vestibular portion of the inner ear.

[Enlarged vestibular aqueduct syndrome. A review of 55 paediatric patients].

PubMed

Santos, Saturnino; Sgambatti, Luciano; Bueno, Antonio; Albi, Gustavo; Suárez, Alicia; Domínguez, Maria Jesús

2010-01-01

Enlarged vestibular aqueduct (EVA) is the commonest congenital anomaly found with imaging techniques in paediatric sensorineural hearing loss (SNHL). Our aim was to describe clinical and audiological findings in paediatric hearing loss associated to EVA. Retrospective review of 55 children with imaging-technique EVA findings from 2000 to 2009. Subjective and/or objective audiological tests were analysed and audiological findings related to clinical features were described. Thirty-seven patients (67.27%) showed bilateral EVA and 18 (32.72%) were unilateral. Hearing loss was bilateral in 46 (83.63%) patients and unilateral in 9 (16.36%). Mean age at diagnosis was 3.78 years. Fifty-three (96.36%) children showed SNHL (28 bilateral and profound), while 2 (3.63%) patients had mixed hearing loss. There were 3 cases of hearing loss progression, 2 fluctuations, 2 of them were asymmetric and 2 patients suffered from vestibular symptoms. Concomitant image findings were 6 cochlear hypoplasia, 2 enlarged internal auditory canals, 1 enlarged vestibule and 1 hypoplastic lateral semicircular canal. Six clinical syndromes were found (2 cases of Down's, and 1 each of Jacobsen, Pendred, Waardenburg and branchio-oto-renal). One child was positive for GJB2 mutation. Familial hearing loss was demonstrated on 12 (21.8%) cases. The clinical picture of hearing loss associated to EVA is characterised by great variability. It should be included in the differential diagnosis of unexplained mixed hearing loss. Familial and syndromic findings have to be taken into consideration in the diagnostic evaluations of such patients. Knowledge about the natural history of this illness is needed so as to give parents prognostic information. Copyright © 2010 Elsevier España, S.L. All rights reserved.

Safety of Monopolar Electrocautery in Patients With Cochlear Implants.

PubMed

Tien, Duc A; Woodson, Erika A; Anne, Samantha

2016-09-01

The outcomes of 2 patients with cochlear implants (CIs) who underwent adenotonsillectomy (AT) with inadvertent use of monopolar cautery are presented. The safety data regarding monopolar cautery use in CI recipients is also reviewed. This is a retrospective case series of 2 CI recipients that underwent AT with monopolar cautery and literature review of electrocautery safety in the setting of CI. Two patients with CIs underwent AT with use of monopolar cautery inadvertently by surgeons that do not routinely perform cochlear implants as part of his or her clinical practice. Patient 1 was a 9-year-old female who had AT for obstructive sleep apnea (OSA) after undergoing unilateral CI for profound congenital sensorineural hearing loss (SNHL) 8 years ago. Patient 2 was a 7-year-old female who underwent AT for OSA 4 months after undergoing unilateral CI for congenital SNHL. Both patients had no immediate signs of complications with their CI use postoperatively. Both patients demonstrated unchanged postoperative neural response telemetry and behavioral audiometric testing. Patient 1 continues to have no CI-related complications 3.5 years after the procedure. Patient 2 has been followed for at least 3 months by audiometric testing and 10 months by otolaryngologist with no CI-related complications. Although animal and cadaveric studies suggest that monopolar cautery may be safely used in patients with cochlear implants, there have been no in vivo human studies that have evaluated the risk to the patient or implant. This is a report of a small, unintended experience with 2 patients, both of whom exhibit no complications or changes to CI function thus far. © The Author(s) 2016.

Centre-level variation in outcomes and treatment for otitis media with effusion and hearing loss and the association of hearing loss with developmental outcomes at ages 5 and 7 years in children with non-syndromic unilateral cleft lip and palate: The Cleft Care UK study. Part 2.

PubMed

Hall, A; Wills, A K; Mahmoud, O; Sell, D; Waylen, A; Grewal, S; Sandy, J R; Ness, A R

2017-06-01

To explore centre-level variation in otitis media with effusion (OME), hearing loss and treatments in children in Cleft Care UK (CCUK) and to examine the association between OME, hearing loss and developmental outcomes at 5 and 7 years. Two hundred and sixty-eight 5-year-old British children with non-syndromic unilateral cleft lip and palate (UCLP) recruited to CCUK. Children had air and bone conduction audiometry at age 5. Information on grommet and hearing aid treatment was obtained from parental questionnaire and medical notes. Hearing loss at age 5 was defined as >20 dB in the better ear and history of OME and hearing loss was determined from past treatment. Children with sensorineural hearing loss were excluded. Associations were examined with speech, behaviour and self-confidence at age 5 and educational attainment at age 7. Centre variation was examined using hierarchical models and associations between hearing variables and developmental outcomes were examined using logistic regression. There was centre-level variation in early grommet placement (variance partition coefficient (VPC) 18%, P=.001) and fitting of hearing aids (VPC 8%, P=.03). A history of OME and hearing loss was associated with poor intelligibility of speech (adjusted odds ratio=2.87, 95% CI 1.42-5.77) and aspects of educational attainment. Hearing loss is an important determinant of poor speech and treatment variation across centres suggest management of OME and hearing loss could be improved. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

[The algorithm for the medical maintenance of the aircraft personnel suffering from chronic sensorineural impairment of hearing].

PubMed

Pankova, V B; Skryabina, L Yu; Barkhatova, O A

2016-01-01

The present study was designed to systematize the causes underlying the development of chronic sensorineural impairment of hearing in the aircraft personnel engaged in commercial aviation of the Russian Federation. A detailed clinical and audiological picture of chronic sensorineural loss of hearing in the aircraft personnel is presented with special reference to the criteria accepted in the civil aviationfor the evaluation of professional suitability and occupational selection in terms of hearing conditions. The study has demonstrated the paramount importance of the aviation medical expertise for the flight safety control in civil aviation. We analyzed the results of the audiological examination of the aircraft personnel suffering from chronic sensorineural impairment of hearing and proposed the algorithm for the rehabilitation of such subjects taking into consideration the stage of the chronic process.

Tinnitus pitch and minimum masking levels in different etiologies.

PubMed

Zagólski, Olaf; Stręk, Paweł

2014-07-01

We sought to determine whether the results of audiological tests and tinnitus characteristics, particularly tinnitus pitch and minimum masking level (MML), depend on tinnitus etiology, and what other etiology-specific tinnitus characteristics there are. The patients answered questions concerning tinnitus laterality, duration, character, aggravation, alleviation, previous treatment, and circumstances of onset. The results of tympanometry, pure-tone audiometry, distortion-product otoacoustic emissions, tinnitus likeness spectrum, MML, and uncomfortable loudness level were evaluated. Patients with several tinnitus etiological factors were excluded. The remaining participants were divided into groups according to medical history: acute acoustic trauma: 67 ears; chronic acoustic trauma: 82; prolonged use of oral estrogen and progesterone contraceptives: 46; Ménière's disease: 25; congenital hearing loss: 19; sensorineural sudden deafness: 40; dull head trauma: 19; viral labyrinthitis: 53; stroke: 6; presbycusis: 152. Data of 509 ears were analysed. Tinnitus pitch was highest in patients with acute acoustic trauma and lowest in patients receiving estrogen and progesterone. MML was lowest after acute acoustic trauma and in congenital hearing loss, and highest after a stroke and in the case of presbytinnitus. Tinnitus pitch and MML are etiology dependent.

[Some aspects of tinnitus].

PubMed

Reiss, M; Reiss, G

1999-09-16

Tinnitus is the sensation of sound, a sensation generated by the auditory system because of a pathology, without any external acoustic or electrical stimulation. Most often, it is associated with a sensorineural hearing loss. Tinnitus is still one of the most frequent symptoms encountered by the otorhinolaryngologist and other doctors. Diagnosis and therapy are demanding due to complex etiology and secondary symptoms. Tinnitus is a symptom and not a disease. Therefore a thorough diagnosis is necessary. First of all one has to evaluate whether there is a treatable underlying organic disease possibly responsible for symptoms like tinnitus. The evaluation of the patient includes the history, ENT-status, audiological and vestibular findings, investigative imaging and examinations by other specialists. The therapeutic aim is the compensation of tinnitus. There is no universal medical or surgical treatment. Acute tinnitus is treated like sudden deafness. For chronic forms, the analysis of the causes is particularly important for developing an individual consultation and therapy plan. Providing information to the patient is the first step for a sensible treatment of the symptoms. Supportive therapy includes a psychosomatic therapy and the use of medication or instrumentation.

Electromagnetic interference of bone-anchored hearing aids by cellular phones.

PubMed

Kompis, M; Negri, S; Häusler, R

2000-10-01

We report a case of electromagnetic interference between a bone-anchored hearing aid (BAHA) and a cellular phone. A 54-year-old women was successfully treated for severe mixed conductive and sensorineural hearing loss with a BAHA. Five years after implantation, the patient experienced a sudden feeling of dizziness, accompanied by a loud buzzing sound and by a sensation of head pressure while examining a digital mobile phone. During a subsequent experiment, the buzzing sound could be reproduced and was identified as electromagnetic interference between the BAHA and digital cellular phones. Seventeen adult BAHA users from our clinic participated in a subsequent survey. Of the 13 patients with some experience of digital cellular phones, 11 reported hearing annoying noises elicited by these devices. However, no other sensation, such as dizziness, was described. Owing to the increasing number of users of both hearing aids and cellular phones, the incidence of electromagnetic interference must be expected to increase as well. Although to date there is no evidence that such interference may be harmful or dangerous to users of conventional or bone-anchored hearing aids, unexpected interference can be a frightening experience.

Postural control assessment in students with normal hearing and sensorineural hearing loss.

PubMed

Melo, Renato de Souza; Lemos, Andrea; Macky, Carla Fabiana da Silva Toscano; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

2015-01-01

Children with sensorineural hearing loss can present with instabilities in postural control, possibly as a consequence of hypoactivity of their vestibular system due to internal ear injury. To assess postural control stability in students with normal hearing (i.e., listeners) and with sensorineural hearing loss, and to compare data between groups, considering gender and age. This cross-sectional study evaluated the postural control of 96 students, 48 listeners and 48 with sensorineural hearing loss, aged between 7 and 18 years, of both genders, through the Balance Error Scoring Systems scale. This tool assesses postural control in two sensory conditions: stable surface and unstable surface. For statistical data analysis between groups, the Wilcoxon test for paired samples was used. Students with hearing loss showed more instability in postural control than those with normal hearing, with significant differences between groups (stable surface, unstable surface) (p<0.001). Students with sensorineural hearing loss showed greater instability in the postural control compared to normal hearing students of the same gender and age. Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Flow fields and acoustics in a unilateral scarred vocal fold model.

PubMed

Murugappan, Shanmugam; Khosla, Sid; Casper, Keith; Oren, Liran; Gutmark, Ephraim

2009-01-01

From prior work in an excised canine larynx model, it has been shown that intraglottal vortices form between the vocal folds during the latter part of closing. It has also been shown that the vortices generate a negative pressure between the folds, producing a suction force that causes sudden, rapid closing of the folds. This rapid closing will produce increased loudness and increased higher harmonics. We used a unilateral scarred excised canine larynx model to determine whether the intraglottal vortices and resulting acoustics were changed, compared to those of normal larynges. Acoustic, flow field, and high-speed imaging measurements from 5 normal and 5 unilaterally scarred canine larynges are presented in this report. Scarring was produced by complete resection of the vocal fold mucosa and superficial layer of the lamina propria on the right vocal fold only. Two months later, these dogs were painlessly sacrificed, and testing was done on the excised larynges during phonation. High-speed video imaging was then used to measure vocal fold displacement during different phases. Particle image velocimetry and acoustic measurements were used to describe possible acoustic effects of the vortices. A higher phonation threshold was required to excite the motion of the vocal fold in scarred larynges. As the subglottal pressure increased, the strength of the vortices and the higher harmonics both consistently increased. However, it was seen that increasing the maximum displacement of the scarred fold did not consistently increase the higher harmonics. The improvements that result from increasing subglottal pressure may be due to a combination of increasing the strength of the intraglottal vortices and increasing the maximum displacement of the vocal fold; however, the data in this study suggest that the vortices play a much more important role. The current study indicates that higher subglottal pressures may excite higher harmonics and improve loudness for patients with unilateral vocal fold scarring. This finding implies that therapies that raise the subglottal pressure may be helpful in improving voice quality.

[The very severe sensorineural deafness patients caused by rubella virus infection: two cases report].

PubMed

Ma, Jing; Wan, Lang; Xu, Fen

2015-09-01

To explore the audiological features in children who were sever sensorineural hearing loss infected with rubella virus. There were two cases of rubella virus infection in children who were deaf, they conducted the distortion product otoacoustic emission, ABR and auditory steady-state evoked response (ASSR) examination, then analyzed the results comprehensively. Two patients' mothers were prompted to have infected rubella virus during the early three months pregnant period by history and laboratory tests. The two patients were not detected deafness gene mutation. Audiology results implied the two patients were very severe binaural sensorineural deafness, so they were recommended to equipped with hearing aids and cochlear implant surgery. Early pregnancy women infected with rubella virus can cause very severe offspring sensorineural deafness. The crowd whose mother were suspected to infect with rubella virus in early pregnancy, that should be tracked and detected hearing in order to achieve early detection, early intervention and early treatment.

Hearing status in patients with rheumatoid arthritis.

PubMed

Ahmadzadeh, A; Daraei, M; Jalessi, M; Peyvandi, A A; Amini, E; Ranjbar, L A; Daneshi, A

2017-10-01

Rheumatoid arthritis is thought to induce conductive hearing loss and/or sensorineural hearing loss. This study evaluated the function of the middle ear and cochlea, and the related factors. Pure tone audiometry, speech reception thresholds, speech discrimination scores, tympanometry, acoustic reflexes, and distortion product otoacoustic emissions were assessed in rheumatoid arthritis patients and healthy volunteers. Pure tone audiometry results revealed a higher bone conduction threshold in the rheumatoid arthritis group, but there was no significant difference when evaluated according to the sensorineural hearing loss definition. Distortion product otoacoustic emissions related prevalence of conductive or mixed hearing loss, tympanometry values, acoustic reflexes, and speech discrimination scores were not significantly different between the two groups. Sensorineural hearing loss was significantly more prevalent in patients who used azathioprine, cyclosporine and etanercept. Higher bone conduction thresholds in some frequencies were detected in rheumatoid arthritis patients that were not clinically significant. Sensorineural hearing loss is significantly more prevalent in refractory rheumatoid arthritis patients.

Change in and Long-Term Investigation of Neuro-Otologic Disorders in Disaster-Stricken Fukushima Prefecture: Retrospective Cohort Study before and after the Great East Japan Earthquake

PubMed Central

Kuriyama, Shinichi; Obara, Taku; Hashimoto, Ken; Tateda, Yutaka; Okumura, Yuri; Kobayashi, Toshimitsu; Katori, Yukio

2015-01-01

On March 11, 2011, Japan’s northeast Pacific coast was hit by a gigantic earthquake and subsequent tsunami. Soma City in Fukushima Prefecture is situated approximately 44 km north of Fukushima Daiichi Nuclear Power Plant. Soma General Hospital is the only hospital in Soma City that provides full-time otolaryngological medical care. We investigated the changes in new patients from one year before to three years after the disaster. We investigated 18,167 new patients treated at our department during the four years from April 1, 2010 to March 31, 2014. Of the new patients, we categorized the diagnoses into Meniere’s disease, acute low-tone sensorineural hearing loss, vertigo, sudden deafness, tinnitus, and facial palsy as neuro-otologic symptoms. We also investigated the changes in the numbers of patients whom we examined at that time concerning other otolaryngological disorders, including epistaxis, infectious diseases of the laryngopharynx, and allergic rhinitis. The total number of new patients did not change remarkably on a year-to-year basis. Conversely, cases of vertigo, Meniere’s disease, and acute low-tone sensorineural hearing loss increased in number immediately after the disaster, reaching a plateau in the second year and slightly decreasing in the third year. Specifically, 4.8% of patients suffering from these neuro-otologic diseases had complications from depression and other mental diseases. With regard to new patients in our department, there was no apparent increase in the number of patients suffering from diseases other than neuro-otologic diseases, including epistaxis, and allergic rhinitis. Patients suffering from vertigo and/or dizziness increased during the first few years after the disaster. These results are attributed to the continuing stress and tension of the inhabitants. This investigation of those living in the disaster area highlights the need for long-term support. PMID:25849607

Audio-vestibular signs and symptoms in Chiari malformation type i. Case series and literature review.

PubMed

Guerra Jiménez, Gloria; Mazón Gutiérrez, Ángel; Marco de Lucas, Enrique; Valle San Román, Natalia; Martín Laez, Rubén; Morales Angulo, Carmelo

2015-01-01

Chiari malformation is an alteration of the base of the skull with herniation through the foramen magnum of the brain stem and cerebellum. Although the most common presentation is occipital headache, the association of audio-vestibular symptoms is not rare. The aim of our study was to describe audio-vestibular signs and symptoms in Chiari malformation type i (CM-I). We performed a retrospective observational study of patients referred to our unit during the last 5 years. We also carried out a literature review of audio-vestibular signs and symptoms in this disease. There were 9 patients (2 males and 7 females), with an average age of 42.8 years. Five patients presented a Ménière-like syndrome; 2 cases, a recurrent vertigo with peripheral features; one patient showed a sudden hearing loss; and one case suffered a sensorineural hearing loss with early childhood onset. The most common audio-vestibular symptom indicated in the literature in patients with CM-I is unsteadiness (49%), followed by dizziness (18%), nystagmus (15%) and hearing loss (15%). Nystagmus is frequently horizontal (74%) or down-beating (18%). Other audio-vestibular signs and symptoms are tinnitus (11%), aural fullness (10%) and hyperacusis (1%). Occipital headache that increases with Valsalva manoeuvres and hand paresthesias are very suggestive symptoms. The appearance of audio-vestibular manifestations in CM-I makes it common to refer these patients to neurotologists. Unsteadiness, vertiginous syndromes and sensorineural hearing loss are frequent. Nystagmus, especially horizontal and down-beating, is not rare. It is important for neurotologists to familiarise themselves with CM-I symptoms to be able to consider it in differential diagnosis. Copyright © 2014 Elsevier España, S.L.U. y Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

Effects of sensorineural hearing loss on temporal coding of narrowband and broadband signals in the auditory periphery

PubMed Central

Henry, Kenneth S.; Heinz, Michael G.

2013-01-01

People with sensorineural hearing loss have substantial difficulty understanding speech under degraded listening conditions. Behavioral studies suggest that this difficulty may be caused by changes in auditory processing of the rapidly-varying temporal fine structure (TFS) of acoustic signals. In this paper, we review the presently known effects of sensorineural hearing loss on processing of TFS and slower envelope modulations in the peripheral auditory system of mammals. Cochlear damage has relatively subtle effects on phase locking by auditory-nerve fibers to the temporal structure of narrowband signals under quiet conditions. In background noise, however, sensorineural loss does substantially reduce phase locking to the TFS of pure-tone stimuli. For auditory processing of broadband stimuli, sensorineural hearing loss has been shown to severely alter the neural representation of temporal information along the tonotopic axis of the cochlea. Notably, auditory-nerve fibers innervating the high-frequency part of the cochlea grow increasingly responsive to low-frequency TFS information and less responsive to temporal information near their characteristic frequency (CF). Cochlear damage also increases the correlation of the response to TFS across fibers of varying CF, decreases the traveling-wave delay between TFS responses of fibers with different CFs, and can increase the range of temporal modulation frequencies encoded in the periphery for broadband sounds. Weaker neural coding of temporal structure in background noise and degraded coding of broadband signals along the tonotopic axis of the cochlea are expected to contribute considerably to speech perception problems in people with sensorineural hearing loss. PMID:23376018

Differences in individual susceptibility affect the development of trigeminal neuralgia☆

PubMed Central

Duransoy, Yusuf Kurtuluş; Mete, Mesut; Akçay, Emrah; Selçuki, Mehmet

2013-01-01

Trigeminal neuralgia is a syndrome due to dysfunctional hyperactivity of the trigeminal nerve, and is characterized by a sudden, usually unilateral, recurrent lancinating pain arising from one or more divisions of the nerve. The most accepted pathogenetic mechanism for trigeminal neuralgia is compression of the nerve at its dorsal root entry zone or in its distal course. In this paper, we report four cases with trigeminal neuralgia due to an unknown mechanism after an intracranial intervention. The onset of trigeminal neuralgia after surgical interventions that are unrelated to the trigeminal nerve suggests that in patients with greater individual susceptibility, nerve contact with the vascular structure due to postoperative pressure and changes in cerebrospinal fluid flow may cause the onset of pain. PMID:25206428

Hearing impairment in children with congenital cytomegalovirus (CMV) infection based on distortion product otoacoustic emissions (DPOAE) and brain evoked response audiometry stimulus click (BERA Click) examinations

NASA Astrophysics Data System (ADS)

Airlangga, T. J.; Mangunatmadja, I.; Prihartono, J.; Zizlavsky, S.

2017-08-01

Congenital cytomegalovirus (congenital CMV) infection is a leading factor of nongenetic sensorineural hearing loss in children. Hearing loss caused by CMV infection does not have a pathognomonic configuration hence further research is needed. The development of knowledge on hearing loss caused by congenital CMV infection is progressing in many countries. Due to a lack of research in the context of Indonesia, this study assesses hearing impairment in children with congenital CMV infection in Indonesia, more specifically in the Cipto Mangunkusumo Hospital. Our objective was to profile hearing impairment in children 0-5 years of age with congenital CMV infection using Distortion Product Otoacoustic Emissions (DPOAE) and Brain Evoked Response Audiometry Stimulus Click (BERA Click) examinations. This cross-sectional study was conducted in the Cipto Mangunkusum Hospital from November, 2015 to May 2016 with 27 children 0-5 years of age with congenital CMV infection. Of individual ears studied, 58.0% exhibited sensorineural hearing loss. There was a significant relationship between developmental delay and incidence of sensorineural hearing loss. Subjects with a developmental delay were 6.57 times more likely (CI 95%; 1.88-22.87) to experience sensorineural hearing loss. Congenital CMV infection has an important role in causing sensorineural hearing loss in children.

Head Position Comparison between Students with Normal Hearing and Students with Sensorineural Hearing Loss.

PubMed

Melo, Renato de Souza; Amorim da Silva, Polyanna Waleska; Souza, Robson Arruda; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

2013-10-01

Introduction Head sense position is coordinated by sensory activity of the vestibular system, located in the inner ear. Children with sensorineural hearing loss may show changes in the vestibular system as a result of injury to the inner ear, which can alter the sense of head position in this population. Aim Analyze the head alignment in students with normal hearing and students with sensorineural hearing loss and compare the data between groups. Methods This prospective cross-sectional study examined the head alignment of 96 students, 48 with normal hearing and 48 with sensorineural hearing loss, aged between 7 and 18 years. The analysis of head alignment occurred through postural assessment performed according to the criteria proposed by Kendall et al. For data analysis we used the chi-square test or Fisher exact test. Results The students with hearing loss had a higher occurrence of changes in the alignment of the head than normally hearing students (p < 0.001). Forward head posture was the type of postural change observed most, occurring in greater proportion in children with hearing loss (p < 0.001), followed by the side slope head posture (p < 0.001). Conclusion Children with sensorineural hearing loss showed more changes in the head posture compared with children with normal hearing.

Head Position Comparison between Students with Normal Hearing and Students with Sensorineural Hearing Loss

PubMed Central

Melo, Renato de Souza; Amorim da Silva, Polyanna Waleska; Souza, Robson Arruda; Raposo, Maria Cristina Falcão; Ferraz, Karla Mônica

2013-01-01

Introduction Head sense position is coordinated by sensory activity of the vestibular system, located in the inner ear. Children with sensorineural hearing loss may show changes in the vestibular system as a result of injury to the inner ear, which can alter the sense of head position in this population. Aim Analyze the head alignment in students with normal hearing and students with sensorineural hearing loss and compare the data between groups. Methods This prospective cross-sectional study examined the head alignment of 96 students, 48 with normal hearing and 48 with sensorineural hearing loss, aged between 7 and 18 years. The analysis of head alignment occurred through postural assessment performed according to the criteria proposed by Kendall et al. For data analysis we used the chi-square test or Fisher exact test. Results The students with hearing loss had a higher occurrence of changes in the alignment of the head than normally hearing students (p < 0.001). Forward head posture was the type of postural change observed most, occurring in greater proportion in children with hearing loss (p < 0.001), followed by the side slope head posture (p < 0.001). Conclusion Children with sensorineural hearing loss showed more changes in the head posture compared with children with normal hearing. PMID:25992037

Effect of a Bluetooth-implemented hearing aid on speech recognition performance: subjective and objective measurement.

PubMed

Kim, Min-Beom; Chung, Won-Ho; Choi, Jeesun; Hong, Sung Hwa; Cho, Yang-Sun; Park, Gyuseok; Lee, Sangmin

2014-06-01

The object was to evaluate speech perception improvement through Bluetooth-implemented hearing aids in hearing-impaired adults. Thirty subjects with bilateral symmetric moderate sensorineural hearing loss participated in this study. A Bluetooth-implemented hearing aid was fitted unilaterally in all study subjects. Objective speech recognition score and subjective satisfaction were measured with a Bluetooth-implemented hearing aid to replace the acoustic connection from either a cellular phone or a loudspeaker system. In each system, participants were assigned to 4 conditions: wireless speech signal transmission into hearing aid (wireless mode) in quiet or noisy environment and conventional speech signal transmission using external microphone of hearing aid (conventional mode) in quiet or noisy environment. Also, participants completed questionnaires to investigate subjective satisfaction. Both cellular phone and loudspeaker system situation, participants showed improvements in sentence and word recognition scores with wireless mode compared to conventional mode in both quiet and noise conditions (P < .001). Participants also reported subjective improvements, including better sound quality, less noise interference, and better accuracy naturalness, when using the wireless mode (P < .001). Bluetooth-implemented hearing aids helped to improve subjective and objective speech recognition performances in quiet and noisy environments during the use of electronic audio devices.

Delayed diagnosis of a patient with Usher syndrome 1C in a Louisiana Acadian family highlights the necessity of timely genetic testing for the diagnosis and management of congenital hearing loss.

PubMed

Umrigar, Ayesha; Musso, Amanda; Mercer, Danielle; Hurley, Annette; Glausier, Cassondra; Bakeer, Mona; Marble, Michael; Hicks, Chindo; Tsien, Fern

2017-01-01

Advances in sequencing technologies and increased understanding of the contribution of genetics to congenital sensorineural hearing loss have led to vastly improved outcomes for patients and their families. Next-generation sequencing and diagnostic panels have become increasingly reliable and less expensive for clinical use. Despite these developments, the diagnosis of genetic sensorineural hearing loss still presents challenges for healthcare providers. Inherited sensorineural hearing loss has high levels of genetic heterogeneity and variable expressivity. Additionally, syndromic hearing loss (hearing loss and additional clinical abnormalities) should be distinguished from non-syndromic (hearing loss is the only clinical symptom). Although the diagnosis of genetic sensorineural hearing loss can be challenging, the patient's family history and ethnicity may provide critical information, as certain genetic mutations are more common in specific ethnic populations. The early identification of the cause of deafness can benefit patients and their families by estimating recurrence risks for future family planning and offering the proper interventions to improve their quality of life. Collaboration between pediatricians, audiologists, otolaryngologists, geneticists, and other specialists are essential in the diagnosis and management of patients with hearing disorders. An early diagnosis is vital for proper management and care, as some clinical manifestations of syndromic sensorineural hearing loss are not apparent at birth and have a delayed age of onset. We present a case of Usher syndrome (congenital deafness and childhood-onset blindness) illustrating the challenges encountered in the diagnosis and management of children presenting with congenital genetic sensorineural hearing loss, along with helpful resources for clinicians and families.

Hearing Loss in Adults.

ERIC Educational Resources Information Center

House, John W.

1997-01-01

This article discusses hearing loss in adults. It begins with an explanation of the anatomy of the ear and then explains the three types of hearing loss: conductive hearing loss, sensorineural hearing loss, and mixed conductive-sensorineural hearing loss. Tinnitus, hearing aids, and cochlear implants are also addressed. (CR)

Bilateral electromyogram response latency following platform perturbation in unilateral transtibial prosthesis users: influence of weight distribution and limb position.

PubMed

Rusaw, David; Hagberg, Kerstin; Nolan, Lee; Ramstrand, Nerrolyn

2013-01-01

Appropriate muscular response following an external perturbation is essential in preventing falls. Transtibial prosthesis users lack a foot-ankle complex and associated sensorimotor structures on the side with the prosthesis. The effect of this lack on rapid responses of the lower limb to external surface perturbations is unknown. The aim of the present study was to compare electromyogram (EMG) response latencies of otherwise healthy, unilateral, transtibial prosthesis users (n = 23, mean +/- standard deviation [SD] age = 48 +/- 14 yr) and a matched control group (n = 23, mean +/- SD age = 48 +/- 13 yr) following sudden support-surface rotations in the pitch plane (toes-up and toes-down). Perturbations were elicited in various weight-bearing and limb-perturbed conditions. The results indicated that transtibial prosthesis users have delayed responses of multiple muscles of the lower limb following perturbation, both in the intact and residual limbs. Weight-bearing had no influence on the response latency in the residual limb, but did on the intact limb. Which limb received the perturbation was found to influence the muscular response, with the intact limb showing a significantly delayed response when the perturbation was received only on the side with a prosthesis. These delayed responses may represent an increased risk of falling for individuals who use transtibial prostheses.

Sensorineural deafness in congenital hypopituitarism with severe hypothyroidism.

PubMed

De Luca, F; Arrigo, T; Mangione, O A; Maiolino, M G; Muritano, M

1985-01-01

Marked sensorineural hearing loss was documented in an 18-year-old boy with untreated congenital anterior panhypopituitarism. The clinical manifestations of the hypothalamic thyroid failure were unusually severe in this patient. Seemingly this is the first case report of perceptive-cochlear deafness in a subject with non primary congenital hypothyroidism.

Acute hyperfibrinogenemia impairs cochlear blood flow and hearing function in guinea pigs in vivo.

PubMed

Ihler, Fritz; Strieth, Sebastian; Pieri, Nicos; Göhring, Peter; Canis, Martin

2012-03-01

Impairment of microcirculation is a possible cause of sudden sensorineural hearing loss (SSNHL). Fibrinogen is known as a risk factor for both microvascular dysfunction and SSNHL. Therefore, the aim of this study was to investigate the effect of elevated serum levels of fibrinogen on cochlear blood flow and hearing function in vivo. One group of guinea pigs received two consecutive injections of 100 mg fibrinogen while a control group received equimolar doses of albumin. Measurements of cochlear microcirculation by intravital microscopy and of hearing thresholds by auditory brainstem response (ABR) recordings were carried out before, after first and after second injection. Ten healthy guinea pigs were randomly assigned to a treatment group or a control group of five animals each. Serum fibrinogen levels were elevated after the first and second injections of fibrinogen compared to basal values and control group respectively. Increasing levels of fibrinogen were paralleled by decreasing cochlear blood flow as well as increasing hearing thresholds. Hearing threshold correlated negatively with cochlear blood flow. The effect of microcirculatory impairment on hearing function could be explained by a malfunction of the cochlear amplifier. Further investigation is needed to quantify cochlear potentials under elevated serum fibrinogen levels.

Using Standardized Psychometric Tests to Identify Learning Disabilities in Students with Sensorineural Hearing Impairments.

ERIC Educational Resources Information Center

Sikora, Darryn M.; Plapinger, Donald S.

1994-01-01

The use of standardized psychoeducational diagnostic instruments to identify learning disabilities was evaluated with 19 students (ages 7 to 13) with sensorineural hearing impairments. Students with hearing impairment were found to demonstrate learning disabilities with a frequency similar to that found in students with normal hearing, suggesting…

Sensorineural Hearing Impairment and Subclinical Atherosclerosis in Rheumatoid Arthritis Patients Without Traditional Cardiovascular Risk Factors

PubMed Central

MACIAS-REYES, Hector; DURAN-BARRAGAN, Sergio; CARDENAS-CONTRERAS, Cynthia R.; CHAVEZ-MARTIN, Cesar G.; GOMEZ-BAÑUELOS, Eduardo; NAVARRO-HERNANDEZ, Rosa E.; YANOWSKY-GONZALEZ, Carlos O.; GONZALEZ-LOPEZ, Laura; GAMEZ-NAVA, Jorge I.

2016-01-01

Objectives This study aims to evaluate the association of hearing impairment with carotid intima-media thickness and subclinical atherosclerosis in rheumatoid arthritis (RA) patients. Patients and methods A total of 41 RA patients (2 males, 39 females; mean age 46.5±10.2 years; range 20 to 63 years) with no known traditional cardiovascular risk factors were included. Routine clinical and laboratory assessments for RA patients were performed. Pure tone air (250-8000 Hz) and bone conduction (250-6000 Hz) thresholds were obtained, tympanograms and impedance audiometry were conducted. Sensorineural hearing impairment was defined if the average thresholds were ≥25 decibels. Carotid intima-media thickness was assessed and classified with a cut-off point of 0.6 mm. Results Thirteen patients (31.7%) had normal audition, while 28 (68.3%) had hearing impairment. Of these, 22 had bilateral sensorineural hearing impairment. Four patients had conductive hearing impairment (right in three patients and left in one patient). Patients with sensorineural hearing impairment had increased carotid intima-media thickness in the media segment of carotid common artery compared to patients with normal hearing (right ear p=0.007; left ear p=0.075). Thickening of the carotid intima-media thickness was associated with sensorineural hearing impairment in RA patients. Conclusion Rheumatoid arthritis patients should be evaluated by carotid intima-media thickness as a possible contributing factor of hearing impairment in patients without cardiovascular risk factors. PMID:29900940

The effects of repetitive vibration on sensorineural function: biomarkers of sensorineural injury in an animal model of metabolic syndrome

PubMed Central

Kiedrowski, Megan; Waugh, Stacey; Miller, Roger; Johnson, Claud; Krajnak, Kristine

2016-01-01

Exposure to hand-transmitted vibration in the work-place can result in the loss of sensation and pain in workers. These effects may be exacerbated by pre-existing conditions such as diabetes or the presence of primary Raynaud's phenomena. The goal of these studies was to use an established model of vibration-induced injury in Zucker rats. Lean Zucker rats have a normal metabolic profile, while obese Zucker rats display symptoms of metabolic disorder or Type II diabetes. This study examined the effects of vibration in obese and lean rats. Zucker rats were exposed to 4 h of vibration for 10 consecutive days at a frequency of 125 Hz and acceleration of 49 m/s2 for 10 consecutive days. Sensory function was checked using transcutaneous electrical stimulation on days 1, 5 and 9 of the exposure. Once the study was complete the ventral tail nerves, dorsal root ganglia and spinal cord were dissected, and levels of various transcripts involved in sensorineural dysfunction were measured. Sensorineural dysfunction was assessed using transcutaneous electrical stimulation. Obese Zucker rats displayed very few changes in sensorineural function. However they did display significant changes in transcript levels for factors involved in synapse formation, peripheral nerve remodeling, and inflammation. The changes in transcript levels suggested that obese Zucker rats had some level of sensory nerve injury prior to exposure, and that exposure to vibration activated pathways involved in injury and re-innervation. PMID:26433044

Sudden Death and Myocardial Lesions after Damage to Catecholamine Neurons of the Nucleus Tractus Solitarii in Rat

PubMed Central

Talman, William T.; Dragon, Deidre Nitschke; Jones, Susan Y.; Moore, Steven A.; Lin, Li-Hsien

2015-01-01

Lesions that remove neurons expressing neurokinin-1 (NK1) receptors from the nucleus tractus solitarii (NTS) without removing catecholaminergic neurons lead to loss of baroreflexes, labile arterial pressure, myocardial lesions and sudden death. Because destruction of NTS catecholaminergic neurons expressing tyrosine hydroxylase (TH) may also cause lability of arterial pressure and loss of baroreflexes, we sought to test the hypothesis that cardiac lesions associated with lability are not dependent on damage to neurons with NK1 receptors but would also occur when TH neurons in NTS are targeted. To rid the NTS of TH neurons we microinjected anti-dopamine β-hydroxylase conjugated to saporin (anti-DBH-SAP, 42ng/200nl) into the NTS. After injection of the toxin unilaterally, immunofluorescent staining confirmed that anti-DBH-SAP decreased the number of neurons and fibers that contain TH and DBH in the injected side of the NTS while sparing neuronal elements expressing NK1 receptors. Bilateral injections in 8 rats led to significant lability of arterial pressure. For example, on day 8 standard deviation of mean arterial pressure was 16.8 ± 2.5 mmHg when compared with a standard deviation of 7.83 ± 0.33 mmHg in 6 rats in which phosphate buffered saline (PBS) had been injected bilaterally. Two rats died suddenly at 5 and 8 days after anti-DBH-SAP injection. Seven treated animals demonstrated microscopic myocardial necrosis as reported in animals with lesions of NTS neurons expressing NK1 receptors. Thus, cardiac and cardiovascular effects of lesions directed toward catecholamine neurons of the NTS are similar to those following damage directed toward NK1 receptor containing neurons. PMID:22484855

Diplacusis, hearing threshold and otoacoustic emissions in an episode of sudden, unilateral cochlear hearing loss.

PubMed

Knight, Richard D

2004-01-01

Limited data are available on the relationship between diplacusis and otoacoustic emissions and sudden hearing threshold changes, and the detail of the mechanism underlying diplacusis is not well understood. Data are presented here from an intensively studied single episode of sudden, non-conductive, mild hearing loss with associated binaural diplacusis, probably due to a viral infection. Treatment with steroids was administered for 1 week. This paper examines the relationships between the hearing loss, diplacusis and otoacoustic emissions during recovery on a day-by-day basis. The hearing thresholds were elevated by up to 20 dB at 4kHz and upwards, and there was an interaural pitch difference up to 12% at 4 and 8 kHz. There was also a frequency-specific change in transient evoked otoacoustic emission (TEOAE) and distortion-product otoacoustic emission (DPOAE) level. DPOAE level was reduced by up to 20 dB. with the greatest change seen when a stimulus with a wide stimulus frequency ratio was used. Frequency shifts in the 2f2-fi DPOAE fine structure corresponded to changes in the diplacusis. Complete recovery to previous levels was observed for TEOAE, DPOAE and hearing threshold. The diplacusis recovered to within normal limits after 4 weeks. The frequency shift seen in the DPOAE fine structure did not quite resolve, suggesting a very slight permanent change. The time-courses of TEOAE. diplacusis and hearing threshold were significantly different: most notably, the hearing threshold was stable over a period when the diplacusis deteriorated. This suggests that the cochlear mechanisms involved in diplacusis, hearing threshold and OAE may not be identical.

Ranking Hearing Aid Input-Output Functions for Understanding Low-, Conversational-, and High-Level Speech in Multitalker Babble

ERIC Educational Resources Information Center

Chung, King; Killion, Mead C.; Christensen, Laurel A.

2007-01-01

Purpose: To determine the rankings of 6 input-output functions for understanding low-level, conversational, and high-level speech in multitalker babble without manipulating volume control for listeners with normal hearing, flat sensorineural hearing loss, and mildly sloping sensorineural hearing loss. Method: Peak clipping, compression limiting,…

Language Development and Impairment in Children with Mild to Moderate Sensorineural Hearing Loss

ERIC Educational Resources Information Center

Halliday, Lorna F.; Tuomainen, Outi; Rosen, Stuart

2017-01-01

Purpose: The goal of this study was to examine language development and factors related to language impairments in children with mild to moderate sensorineural hearing loss (MMHL). Method: Ninety children, aged 8-16 years (46 children with MMHL; 44 aged-matched controls), were administered a battery of standardized language assessments, including…

Profound Bilateral Sensorineural Hearing Loss in Nigerian Children: Any Shift in Etiology?

ERIC Educational Resources Information Center

Dunmade, A. D.; Segun-Busari, S.; Olajide, T. G.; Ologe, F. E.

2007-01-01

Deafness, profound hearing loss, is a global problem. However, the causes of, attitudes toward, and management options for deafness differ considerably from region to region. This study seeks to identify the present causes of profound sensorineural hearing loss in Nigeria, which in our environment is almost synonymous to a life sentence of silence…

Reports of sensorineural deafness after measles, mumps, and rubella immunisation.

PubMed Central

Stewart, B J; Prabhu, P U

1993-01-01

There have been nine reports of sensorineural hearing loss after measles, mumps, and rubella (MMR) immunisation. In three cases the deafness was unrelated to MMR immunisation. In six cases the cause was unknown and MMR remained a possible aetiology. Any risk associated with attenuated viruses must be weighed against the risks of the natural diseases. PMID:8024302

Strategies for Maximizing Speech-Recognition Performance: Adults with Mild to Moderately Severe Sensorineural Hearing Loss. EBP Briefs. Volume 11, Issue 4

ERIC Educational Resources Information Center

Donai, Jeremy J.; Schwartz, Jeremy C.

2016-01-01

Clinical Question: What high-frequency amplification strategy maximizes speechrecognition performance among adult hearing-impaired listeners with mild sloping to moderately severe sensorineural hearing loss? Method: Quick response review. Study Sources: EBSCO, PubMed, Google Scholar, as well as journals from the American Speech-Language-Hearing…

Neurodevelopmental Disorders in Children with Severe to Profound Sensorineural Hearing Loss: A Clinical Study

ERIC Educational Resources Information Center

Chilosi, Anna M.; Comparini, Alessandro; Scusa, Maria F.; Berrettini, Stefano; Forli, Francesca; Battini, Roberta; Cipriani, Paola; Cioni, Giovanni

2010-01-01

Aim: The effects of sensorineural hearing loss (SNHL) are often complicated by additional disabilities, but the epidemiology of associated disorders is not clearly defined. The aim of this study was to evaluate the frequency and type of additional neurodevelopmental disabilities in a sample of children with SNHL and to investigate the relation…

Variations in otological presentation of lightning strike victims: Clinical report of 3 patients.

PubMed

Kılıç, Erbil; Genç, Hakan; Aydın, Ümit; Aşık, Burak; Satar, Bülent

2017-03-01

Lightning strike can cause fatal or nonfatal injuries. Some nonfatal injuries are associated with otological symptoms and findings. Conductive hearing loss due to rupture of the tympanic membrane is the most common audiovestibular lesion of lightning strike. Various forms of sensorineural hearing loss and dizziness have also been reported. Presently described are 3 cases of lightning strike injury. First patient had mid-frequency hearing loss in right ear and high frequency sensorineural hearing loss in left ear. Second patient had high frequency sensorineural hearing loss in left ear, and the third had peripheral facial palsy with perilymphatic fistula on same side. This is the first documented case of mid-frequency hearing loss occurring after lightning strike.

Novel association of achalasia with hereditary sensory and motor neuropathy with sensorineural deafness.

PubMed

Asthana, A K; Lubel, J S; Kohn, G P

2016-08-01

Achalasia is a primary esophageal motility disorder. Unlike diffuse esophageal spasm, it has not previously been described in association with hereditary sensory and motor neuropathy (HSMN). An 18-year-old-male with HSMN with sensorineural deafness presented with a 2-day history of dysphagia to solids and liquids. Achalasia was diagnosed after extensive investigations, and his symptoms resolved with endoscopic and definitive surgical management. His monozygotic twin brother had also been diagnosed with HSMN and suffered from chronic dysphagia, which was also subsequently diagnosed with achalasia. This is the first case to illustrate an association between HSMN with sensorineural deafness and achalasia. © 2013 Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.

A multidisciplinary approach to paediatric hearing loss: programme at the centre for hearing intervention and language development, National University Hospital, Singapore.

PubMed

Lim, Lynne H Y

2008-12-01

The objective is to describe the multidisciplinary management programme at the National University Hospital (NUH) in Singapore for children with hearing impairment (HI). Over 99.95% of babies born at NUH have hearing tested with both otoacoustic emission and automated auditory brainstem response tests by 6 weeks of age. The referral rate to Otolaryngology is 0.5%. Acquired causes of congenital HI are decreasing. Thirty percent of patients at NUH with idiopathic congenital sensorineural HI have DFNB1/ GJB6 Connexin 26 HI. CT scan or MRI imaging has a higher diagnostic yield when there is unilateral, fluctuating or non-Connexin 26 related HI. Routine electrocardiogram and Opthalmology evaluations will exclude associations of fatal cardiac rhythm anomaly and retinopathy. Other investigations are directed by history and clinical examination. There is now a very wide range of increasingly sophisticated medication, neuro-otologic external, middle and inner ear surgery, hearing aids, middle ear implants and cochlear implants available to improve hearing. A multidisciplinary team from neonatology, paediatrics, otolaryngology, audiology, auditory verbal and speech therapy, ophthalmology, radiology, and psychology working closely with the child, family and schools is needed to develop a cost-effective and comprehensive management programme for paediatric HI.

Retrofacial approach to access the round window for cochlear implantation of malformed ears.

PubMed

Rizk, Habib; O'Connell, Brendan; Stevens, Shawn; Meyer, Ted

2015-03-01

To report the use of the retrofacial approach for cochlear implantation in three cases of malformed ears with inaccessible round windows through the standard facial recess. Two children with bilateral profound sensorineural hearing loss who were cochlear implant candidates. One patient had bilateral sequential cochlear implantations and the other a unilateral implant. Retrofacial approach to access the posterior mesotympanum and visualize the round window. Ability to complete the surgery with full insertion of the implant and no complications such as facial nerve injury. We implanted three ears in two patients with multiple external and middle ear malformations with an aberrant facial nerve or a posteriorly displaced round window niche. The standard facial recess approach did not allow visualization of the round window. We resorted to a retrofacial approach to access the posterior mesotympanum and proceeded with the surgery through an anterior and inferior cochleostomy or through the round window. In cases with an aberrant facial nerve or inaccessible round window through the facial recess, the retrofacial approach is a good alternative but requires a certain level of expertise and familiarity with temporal bone anatomy. The decision to use an unconventional approach should be considered before surgery, but the ultimate decision may require intraoperative assessment.

Vibrotactile sense and hand symptoms in blue collar workers in a manufacturing industry.

PubMed Central

Flodmark, B T; Lundborg, G

1997-01-01

OBJECTIVES: To study whether vibrotactile sense combined with questionnaires (subjective complaints) and a clinical examination (including scoring of the Stockholm workshop scale (sensorineural staging)) could serve as a screening procedure, in the health care service, for sensorineural symptoms. A group of blue collar workers exposed to vibration in a manufacturing industry (rock crushing plants) was used as the study group. Another group of workers not exposed to vibration but subjected to heavy manual work served as the control group. METHODS: Vibrotactile sense was determined. The index and the little fingers of both hands were investigated. A clinical examination was performed. Questionnaires were used for exposure data and for assessment of symptoms. RESULTS: Important findings were that impairment in vibrotactile sense correlated with impairment in grip force, cold sensitivity, and other sensorineural symptoms--such as numbness and tendency to drop items. Clinical findings such as Phalen's test and two point discrimination were related only in those workers with the poorest vibrotactile sense. There was a relation between vibrotactile sense and the Stockholm workshop scale (sensorineural staging) for the sensorineural symptoms. Muscle and joint problems were more often seen in workers with decreased vibrotactile sense. CONCLUSIONS: Tactilometry for assessment of vibrotactile sense is a useful tool in assessing and evaluating the severity of vibration induced neuromuscular symptoms and verifying the patients' clinical complaints. Heavy manual work without exposure to vibration may contribute to impairment of vibrotactile sense. The relation between impairment in vibrotactile sense and grip strength indicates that impaired sensory feedback may contribute to muscle weakness. PMID:9470896

In vivo imaging of saccular hydrops in humans reflects sensorineural hearing loss rather than Meniere's disease symptoms.

PubMed

Attyé, Arnaud; Eliezer, Michael; Medici, Maud; Tropres, Irène; Dumas, Georges; Krainik, Alexandre; Schmerber, Sébastien

2018-07-01

A case-controlled imaging study demonstrated that saccular hydrops was specific to Meniere's disease (MD), but only present in a subset of patients. Here, we compared patients with definite MD, vertigo and sensorineural hearing loss (SNHL) to elucidate the relationship between saccular hydrops and extent of SNHL. In this prospective study, we performed 3D-FLAIR sequences between 4.5 and 5.5 h after contrast media injection in patients with MD (n=20), SNHL (n=20), vertigo (n=20) and 30 healthy subjects. Two radiologists independently graded saccular hydrops. ROC analysis was performed to determine the hearing loss threshold to differentiate patients with saccular hydrops. Saccular hydrops was found in 11 of 20 MD patients, 10 of 20 SNHL patients and in none of the vertigo patients and healthy subjects. In SNHL patients, 45 dB was the threshold above which there was a significant association with saccular hydrops, with sensitivity of 100 % and specificity of 90 %. In MD patients, 40 dB was the threshold above which there was a significant association with saccular hydrops, with sensitivity of 100 % and specificity of 44 %. Our results indicate saccular hydrops as a feature of worse than moderate SNHL rather than MD itself. • MRI helps clinicians to assess patients with isolated low-tone sensorineural hearing loss. • Saccular hydrops correlates with sensorineural hearing loss at levels above 40 dB. • Vertigo patients without sensorineural hearing loss do not have saccular hydrops. • Saccular hydrops is described in patients without clinical diagnosis of Meniere's disease.

Horizontal plane localization in single-sided deaf adults fitted with a bone-anchored hearing aid (Baha).

PubMed

Grantham, D Wesley; Ashmead, Daniel H; Haynes, David S; Hornsby, Benjamin W Y; Labadie, Robert F; Ricketts, Todd A

2012-01-01

: One purpose of this investigation was to evaluate the effect of a unilateral bone-anchored hearing aid (Baha) on horizontal plane localization performance in single-sided deaf adults who had either a conductive or sensorineural hearing loss in their impaired ear. The use of a 33-loudspeaker array allowed for a finer response measure than has previously been used to investigate localization in this population. In addition, a detailed analysis of error patterns allowed an evaluation of the contribution of random error and bias error to the total rms error computed in the various conditions studied. A second purpose was to investigate the effect of stimulus duration and head-turning on localization performance. : Two groups of single-sided deaf adults were tested in a localization task in which they had to identify the direction of a spoken phrase on each trial. One group had a sensorineural hearing loss (SNHL group; N = 7), and the other group had a conductive hearing loss (CHL group; N = 5). In addition, a control group of four normal-hearing adults was tested. The spoken phrase was either 1250 msec in duration (a male saying "Where am I coming from now?") or 341 msec in duration (the same male saying "Where?"). For the longer-duration phrase, subjects were tested in conditions in which they either were or were not allowed to move their heads before the termination of the phrase. The source came from one of nine positions in the front horizontal plane (from -79° to +79°). The response range included 33 choices (from -90° to +90°, separated by 5.6°). Subjects were tested in all stimulus conditions, both with and without the Baha device. Overall rms error was computed for each condition. Contributions of random error and bias error to the overall error were also computed. : There was considerable intersubject variability in all conditions. However, for the CHL group, the average overall error was significantly smaller when the Baha was on than when it was off. Further analysis of error patterns indicated that this improvement was primarily based on reduced response bias when the device was on; that is, the average response azimuth was nearer to the source azimuth when the device was on than when it was off. The SNHL group, on the other hand, had significantly greater overall error when the Baha was on than when it was off. Collapsed across listening conditions and groups, localization performance was significantly better with the 1250 msec stimulus than with the 341 msec stimulus. However, for the longer-duration stimulus, there was no significant beneficial effect of head-turning. Error scores in all conditions for both groups were considerably larger than those in the normal-hearing control group. : On average, single-sided deaf adults with CHL showed improved localization ability when using the Baha, whereas single-sided deaf adults with SNHL showed a decrement in performance when using the device. These results may have implications for clinical counseling for patients with unilateral hearing impairment.

The effectiveness of the directional microphone in the Oticon Medical Ponto Pro in participants with unilateral sensorineural hearing loss.

PubMed

Oeding, Kristi; Valente, Michael

2013-09-01

Current bone anchored hearing solutions (BAHSs) have incorporated automatic adaptive multichannel directional microphones (DMs). Previous fixed single-channel hypercardioid DMs in BAHSs have provided benefit in a diffuse listening environment, but little data are available on the performance of adaptive multichannel DMs in BAHSs for persons with unilateral sensorineural hearing loss (USNHL). The primary goal was to determine if statistically significant differences existed in the mean Reception Threshold for Sentences (RTS in dB) in diffuse uncorrelated restaurant noise between unaided, an omnidirectional microphone (OM), split DM (SDM), and full DM (FDM) in the Oticon Medical Ponto Pro. A second goal was to assess subjective benefit using the Abbreviated Profile of Hearing Aid Benefit (APHAB) comparing the Ponto Pro to the participant's current BAHS, and the Ponto Pro and participant's own BAHS to unaided. The third goal was to compare RTS data of the Ponto Pro to data from an identical study examining Cochlear Americas' Divino. A randomized repeated measures, single blind design was used to measure an RTS for each participant for unaided, OM, SDM, and FDM. Fifteen BAHS users with USNHL were recruited from Washington University in St. Louis and the surrounding area. The Ponto Pro was fit by measuring in-situ bone conduction thresholds and was worn for 4 wk. An RTS was obtained utilizing Hearing in Noise Test (HINT) sentences in uncorrelated restaurant noise from an eight loudspeaker array, and subjective benefit was determined utilizing the APHAB. Analysis of variance (ANOVA) was used to analyze the results of the Ponto Pro HINT and APHAB data, and comparisons between the Ponto Pro and previous Divino data. No statistically significant differences existed in mean RTS between unaided, the Ponto Pro's OM, SDM, or FDM (p = 0.10). The Ponto Pro provided statistically significant benefit for the Background Noise (BN) (p < 0.01) and Reverberation (RV) (p < 0.05) subscales compared to the participant's own BAHS. The Ponto Pro (Ease of Communication [EC] [p < 0.01], BN [p < 0.001], and RV [p < 0.01] subscales) and participant's own BAHS (BN [p < 0.01] and RV [p < 0.01] subscales) overall provided statistically significant benefit compared to unaided. Clinically significant benefit of 5% was present for the Ponto Pro compared to the participant's own BAHS and 10% for the Ponto Pro and the participant's own BAHS compared to unaided. The Ponto Pro's OM (p = 0.05), SDM (p = 0.05), and FDM (p < 0.01) were statistically significantly better than the Divino's OM. No significant differences existed between the Ponto Pro's OM, SDM, and FDM compared to the Divino's DM. No statistically significant differences existed between unaided, OM, SDM, or FDM. Participants preferred the Ponto Pro compared to the participant's own BAHS and the Ponto Pro and participant's own BAHS compared to unaided. The RTS of the Ponto Pro's adaptive multichannel DM was similar to the Divino's fixed hypercardioid DM, but the Ponto Pro's OM was statistically significantly better than the Divino's OM. American Academy of Audiology.

An Evaluation of the BKB-SIN, HINT, QuickSIN, and WIN Materials on Listeners with Normal Hearing and Listeners with Hearing Loss

ERIC Educational Resources Information Center

Wilson, Richard H.; McArdle, Rachel A.; Smith, Sherri L.

2007-01-01

Purpose: The purpose of this study was to examine in listeners with normal hearing and listeners with sensorineural hearing loss the within- and between-group differences obtained with 4 commonly available speech-in-noise protocols. Method: Recognition performances by 24 listeners with normal hearing and 72 listeners with sensorineural hearing…

Active Duty - U.S. Army Noise Induced Hearing Injury Surveillance Calendar Years 2009-2013

DTIC Science & Technology

2014-06-01

rates for sensorineural hearing loss, significant threshold shift, tinnitus , and Noise-Induced Hearing Loss. The intention is to monitor the morbidity...surveillance. These code groups include sensorineural hearing loss (SNHL), significant threshold shift (STS), noise-induced hearing loss (NIHL) and tinnitus ... Tinnitus ) was analyzed using a regression model to determine the trend of incidence rates from 2007 to the current year. Statistical significance of a

P300 in individuals with sensorineural hearing loss.

PubMed

Reis, Ana Cláudia Mirandola Barbosa; Frizzo, Ana Claudia Figueiredo; Isaac, Myriam de Lima; Garcia, Cristiane Fregonesi Dutra; Funayama, Carolina Araújo Rodrigues; Iório, Maria Cecília Martinelli

2015-01-01

Behavioral and electrophysiological auditory evaluations contribute to the understanding of the auditory system and of the process of intervention. To study P300 in subjects with severe or profound sensorineural hearing loss. This was a descriptive cross-sectional prospective study. It included 29 individuals of both genders with severe or profound sensorineural hearing loss without other type of disorders, aged 11 to 42 years; all were assessed by behavioral audiological evaluation and auditory evoked potentials. A recording of the P3 wave was obtained in 17 individuals, with a mean latency of 326.97ms and mean amplitude of 3.76V. There were significant differences in latency in relation to age and in amplitude according to degree of hearing loss. There was a statistically significant association of the P300 results with the degrees of hearing loss (p=0.04), with the predominant auditory communication channels (p<0.0001), and with time of hearing loss. P300 can be recorded in individuals with severe and profound congenital sensorineural hearing loss; it may contribute to the understanding of cortical development and is a good predictor of the early intervention outcome. Copyright © 2014 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

A Cell Culture Model of Latent and Lytic Herpes Simplex Virus Type 1 Infection in Spiral Ganglion.

PubMed

Liu, Yuehong; Li, Shufeng

2015-01-01

Reactivation of latent herpes simplex virus type 1 (HSV-1) in spiral ganglion neurons (SGNs) is supposed to be one of the causes of idiopathic sudden sensorineural hearing loss. This study aims to establish a cell culture model of latent and lytic HSV-1 infection in spiral ganglia. In the presence of acyclovir, primary cultures of SGNs were latently infected with HSV-1 expressing green fluorescent protein. Four days later, these cells were treated with trichostatin A (TSA), a known chemical reactivator of HSV-1. TCID50 was used to measure the titers of virus in cultures on Vero cells. RNA from cultures was detected for the presence of transcripts of ICP27 and latency-associated transcript (LAT) using reverse transcription polymerase chain reaction. There is no detectable infectious HSV-1 in latently infected cultures, whereas they could be observed in both lytically infected and latently infected/TSA-treated cultures. LAT was the only detectable transcript during latent infection, whereas lytic ICP27 transcript was detected in lytically infected and latently infected/TSA-treated cultures. Cultured SGNs can be both latently and lytically infected with HSV-1. Furthermore, latently infected SGNs can be reactivated using TSA, yielding infectious virus.

Anterior ischemic optic neuropathy in a patient with Churg-Strauss syndrome.

PubMed

Lee, Ji Eun; Lee, Seung Uk; Kim, Soo Young; Jang, Tae Won; Lee, Sang Joon

2012-12-01

We describe a patient with Churg-Strauss syndrome who developed unilateral anterior ischemic optic neuropathy. A 54-year-old man with a history of bronchial asthma, allergic rhinitis, and sinusitis presented with sudden decreased visual acuity in his right eye that had begun 2 weeks previously. The visual acuity of his right eye was 20 / 50. Ophthalmoscopic examination revealed a diffusely swollen right optic disc and splinter hemorrhages at its margin. Goldmann perimetry showed central scotomas in the right eye and fluorescein angiography showed remarkable hyperfluorescence of the right optic nerve head. Marked peripheral eosinphilia, extravascular eosinophils in a bronchial biopsy specimen, and an increased sedimentation rate supported the diagnosis of Churg-Strauss syndrome. Therapy with methylprednisolone corrected the laboratory abnormalities, improved clinical features, and preserved vision, except for the right central visual field defect. Early recognition of this systemic disease by ophthalmologists may help in preventing severe ocular complications.

Anterior Ischemic Optic Neuropathy in a Patient with Churg-Strauss Syndrome

PubMed Central

Lee, Ji Eun; Lee, Seung Uk; Kim, Soo Young; Jang, Tae Won

2012-01-01

We describe a patient with Churg-Strauss syndrome who developed unilateral anterior ischemic optic neuropathy. A 54-year-old man with a history of bronchial asthma, allergic rhinitis, and sinusitis presented with sudden decreased visual acuity in his right eye that had begun 2 weeks previously. The visual acuity of his right eye was 20 / 50. Ophthalmoscopic examination revealed a diffusely swollen right optic disc and splinter hemorrhages at its margin. Goldmann perimetry showed central scotomas in the right eye and fluorescein angiography showed remarkable hyperfluorescence of the right optic nerve head. Marked peripheral eosinphilia, extravascular eosinophils in a bronchial biopsy specimen, and an increased sedimentation rate supported the diagnosis of Churg-Strauss syndrome. Therapy with methylprednisolone corrected the laboratory abnormalities, improved clinical features, and preserved vision, except for the right central visual field defect. Early recognition of this systemic disease by ophthalmologists may help in preventing severe ocular complications. PMID:23204805

Diagnosis and management of Bell's palsy.

PubMed

Santos, Renata de Faria; Brasileiro, Bernardo Ferreira

2011-01-01

Bell's palsy (BP) is an idiopathic peripheral facial nerve paralysis of sudden onset. Its most alarming symptom is unilateral facial weakness, which can result in the inability to close the eyelids, smile, or whistle. The pathogenesis of BP is controversial and is believed to be caused by inflammation of the facial nerve at the geniculate ganglion. Many viruses, especially herpes simplex and herpes zoster, have been suggested as initiators of this inflammatory process; however, this has not been proven. This report describes the case of a 14-year-old girl with right hemifacial palsy who sought treatment one month after the onset of palsy. The patient experienced a satisfactory recovery within 30 days of treatment and has maintained a stable physical outcome after 15 months of follow-up. Early treatment based on careful investigation of BP, with particular attention given to the differential diagnosis of BP, can improve the patient's function and esthetics.

A Challenge for Cochlear Implantation: Duplicated Internal Auditory Canal.

PubMed

Binnetoğlu, Adem; Bağlam, Tekin; Sarı, Murat; Gündoğdu, Yavuz; Batman, Çağlar

2016-08-01

Duplication of the internal auditory canal is an uncommon, congenital malformation that can be associated with sensorineural hearing loss owing to aplasia/hypoplasia of the vestibulocochlear nerve. Only 14 such cases have been reported to date. We report the case of a 13-month-old girl with bilateral, congenital, sensorineural hearing loss caused by narrow, duplicated internal auditory canals and discuss the challenges encountered in the diagnosis and treatment of this condition.

[Effect of chronic sensorineural hearing loss on several indicators of immune and endocrine systems of 7-11 year-old children].

PubMed

Beschasnyĭ, S P

2013-01-01

We investigated the effects of chronic bilateral sensorineural hearing loss of III-IV degree on the performance of interleukins, immunoglobulins serum and saliva, the functional activity of granulocyte-monocyte cell immunity, evaluated the activity of the hypothalamic-pituitary-adrenal system in children aged 7-11 years. It was found that due to stress activation of the sympathetic-adrenal system the function of granulocytes and monocytes is suppressed, with a predominance of production of anti-inflammatory interleukins. This leads to the dominance of T-helper type 2. Products granulocytes and T-helper type-2 anti-inflammatory interleukins IL-4, IL-5, IL-10, IL-13 leads to the activation of B-cells. Thus, in children 7-11 years of age with congenital bilateral sensorineural hearing loss is a decrease of non-specific humoral immunity dominated type of immune response to increased levels of IgG.

High-frequency toneburst-evoked ABR latency-intensity functions in sensorineural hearing-impaired humans.

PubMed

Fausti, S A; Olson, D J; Frey, R H; Henry, J A; Schaffer, H I; Phillips, D S

1995-01-01

The latency-intensity functions (LIFs) of ABRs elicited by high-frequency (8, 10, 12, and 14 kHz) toneburst stimuli were evaluated in 20 subjects with confirmed 'moderate' high-frequency sensorineural hearing loss. Wave V results from clicks and tonebursts revealed all intra- and intersession data to be reliable (p > 0.05). Linear regression curves were highly significant (p < or = 0.0001), indicating linear relationships for all stimuli analyzed. Comparisons between the linear regression curves from a previously reported normal-hearing subject group and this sensorineural hearing-impaired group showed no significant differences. This study demonstrated that tonebursts at 8, 10, and 12 kHz evoked ABRs which decreased in latency as a function of increasing intensity and that these LIFs were consistent and orderly (14 kHz was not determinable). These results will contribute information to facilitate the establishment of change criteria used to predict change in hearing during treatment with ototoxic medications.

Genetic Effects on Sensorineural Hearing Loss and Evidence-based Treatment for Sensorineural Hearing Loss.

PubMed

Yu, Yong-qiang; Yang, Huai-an; Xiao, Ming; Wang, Jing-wei; Huang, Dong-yan; Bhambhani, Yagesh; Sonnenberg, Lyn; Clark, Brenda; Jin, Yuan-zhe; Fu, Wei-neng; Zhang, Jie; Yu, Qian; Liang, Xue-ting; Zhang, Ming

2015-09-01

In this article, the mechanism of inheritance behind inherited hearing loss and genetic susceptibility in noise-induced hearing loss are reviewed. Conventional treatments for sensorineural hearing loss (SNHL), i.e. hearing aid and cochlear implant, are effective for some cases, but not without limitations. For example, they provide little benefit for patients of profound SNHL or neural hearing loss, especially when the hearing loss is in poor dynamic range and with low frequency resolution. We emphasize the most recent evidence-based treatment in this field, which includes gene therapy and allotransplantation of stem cells. Their promising results have shown that they might be options of treatment for profound SNHL and neural hearing loss. Although some treatments are still at the experimental stage, it is helpful to be aware of the novel therapies and endeavour to explore the feasibility of their clinical application.

The Bjornstad syndrome (sensorineural hearing loss and pili torti) disease gene maps to chromosome 2q34-36.

PubMed Central

Lubianca Neto, J F; Lu, L; Eavey, R D; Flores, M A; Caldera, R M; Sangwatanaroj, S; Schott, J J; McDonough, B; Santos, J I; Seidman, C E; Seidman, J G

1998-01-01

We report that the Bjornstad syndrome gene maps to chromosome 2q34-36. The clinical association of sensorineural hearing loss with pili torti (broken, twisted hairs) was described >30 years ago by Bjornstad; subsequently, several small families have been studied. We evaluated a large kindred with Bjornstad syndrome in which eight members inherited pili torti and prelingual sensorineural hearing loss as autosomal recessive traits. A genomewide search using polymorphic loci demonstrated linkage between the disease gene segregating in this kindred and D2S434 (maximum two-point LOD score = 4.98 at theta = 0). Haplotype analysis of recombination events located the disease gene in a 3-cM region between loci D2S1371 and D2S163. We speculate that intermediate filament and intermediate filament-associated proteins are good candidate genes for causing Bjornstad syndrome. PMID:9545407

[Genetic deafness].

PubMed

Marcolla, A; Bouchetemble, P; Lerosey, Y; Marie, J-P; Dehesdin, D

2006-06-01

The aim of this study was to review the different types of genetic deafness. We describe syndromic and isolated sensorineural deafness and transmission deafness. Genetic sensorineural syndromic deafness represents 30% of cases of genetic deafness. A frequent cause is Pendred syndrome, which associates congenital sensorineural deafness with goitre and malformations of the inner ear which can be identified on computed tomography scan. Isolated deafness which is responsible for 70% of cases of genetic deafness is then outlined. Among the different types of isolated deafness, 80% are autosomal recessive disorders. A frequent form of autosomal recessive deafness is due to mutations in the connexin 26 gene. Lastly, we detail transmission deafness dominated by aplasia. Major aplasia is characterized by a malformation of the external ear associated with malformations of the middle ear whereas, minor aplasia corresponds to a malformation of the middle ear, sometimes associated with minor external ear malformations. For each type of deafness we propose a systematic assessment.

Dominant ER Stress-Inducing WFS1 Mutations Underlie a Genetic Syndrome of Neonatal/Infancy-Onset Diabetes, Congenital Sensorineural Deafness, and Congenital Cataracts.

PubMed

De Franco, Elisa; Flanagan, Sarah E; Yagi, Takuya; Abreu, Damien; Mahadevan, Jana; Johnson, Matthew B; Jones, Garan; Acosta, Fernanda; Mulaudzi, Mphele; Lek, Ngee; Oh, Vera; Petz, Oliver; Caswell, Richard; Ellard, Sian; Urano, Fumihiko; Hattersley, Andrew T

2017-07-01

Neonatal diabetes is frequently part of a complex syndrome with extrapancreatic features: 18 genes causing syndromic neonatal diabetes have been identified to date. There are still patients with neonatal diabetes who have novel genetic syndromes. We performed exome sequencing in a patient and his unrelated, unaffected parents to identify the genetic etiology of a syndrome characterized by neonatal diabetes, sensorineural deafness, and congenital cataracts. Further testing was performed in 311 patients with diabetes diagnosed before 1 year of age in whom all known genetic causes had been excluded. We identified 5 patients, including the initial case, with three heterozygous missense mutations in WFS1 (4/5 confirmed de novo). They had diabetes diagnosed before 12 months (2 before 6 months) (5/5), sensorineural deafness diagnosed soon after birth (5/5), congenital cataracts (4/5), and hypotonia (4/5). In vitro studies showed that these WFS1 mutations are functionally different from the known recessive Wolfram syndrome-causing mutations, as they tend to aggregate and induce robust endoplasmic reticulum stress. Our results establish specific dominant WFS1 mutations as a cause of a novel syndrome including neonatal/infancy-onset diabetes, congenital cataracts, and sensorineural deafness. This syndrome has a discrete pathophysiology and differs genetically and clinically from recessive Wolfram syndrome. © 2017 by the American Diabetes Association.

Is there a best side for cochlear implants in post-lingual patients?

PubMed

Amaral, Maria Stella Arantes do; Damico, Thiago A; Gonçales, Alina S; Reis, Ana C M B; Isaac, Myriam de Lima; Massuda, Eduardo T; Hyppolito, Miguel Angelo

2017-07-29

Cochlear Implant is a sensory prosthesis capable of restoring hearing in patients with severe or profound bilateral sensorineural hearing loss. To evaluate if there is a better side to be implanted in post-lingual patients. Retrospective longitudinal study. Participants were 40 subjects, of both sex, mean age of 47 years, with post-lingual hearing loss, users of unilateral cochlear implant for more than 12 months and less than 24 months, with asymmetric auditor reserve between the ears (difference of 10dBNA, In at least one of the frequencies with a response, between the ears), divided into two groups. Group A was composed of individuals with cochlear implant in the ear with better auditory reserve and Group B with auditory reserve lower in relation to the contralateral side. There was no statistical difference for the tonal auditory threshold before and after cochlear implant. A better speech perception in pre-cochlear implant tests was present in B (20%), but the final results are similar in both groups. The cochlear implant in the ear with the worst auditory residue favors a bimodal hearing, which would allow the binaural summation, without compromising the improvement of the audiometric threshold and the speech perception. Copyright © 2017 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Clinical, electrophysiological, genetic, and imaging features of six Chinese Han patients with hereditary neuropathy with liability to pressure palsies (HNPP).

PubMed

Chen, Bin; Niu, Songtao; Wang, Xingao; Li, Wei; Chen, Na; Zhang, Zaiqiang

2018-02-01

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant peripheral neuropathy caused by mutations in the peripheral myelin protein 22 (PMP22) gene. This study summarizes the clinical, electrophysiological, genetic, and imaging features of six unrelated Chinese Han patients with HNPP. Age of onset was within the second decade in five patients, and 46 years of age in one patient. Weakness or numbness in a unilateral lower extremity was the most common symptom in 5 patients, and bilateral sensorineural hearing loss was also detected in one patient. Electrophysiological presentations suggested demyelinating sensory-motor polyneuropathy in the group. Magnetic resonance imaging (MRI) of the cervical and lumbar spine revealed varying degrees of degeneration in five patients, and mild kyphosis of cervical vertebral bodies in 2 teen-aged patients. In addition, cranial MRI of one patient showed scattered demyelination in the frontal lobes. Targeted next-generation-sequencing (NGS) revealed a PMP22 deletion in five patients and a heterozygous c.199G>A mutation in exon 4 of PMP22 in one patient. The I92V variant of lipopolysaccharide-induced tumor necrosis factor (LITAF) gene was found in one patient. There was no relationship between the Ile92Val variant of LITAF and age of onset in this group, albeit the sample size was very small. Copyright © 2017 Elsevier Ltd. All rights reserved.

Correlation Between White Matter Lesions and Intelligence Quotient in Patients With Congenital Cytomegalovirus Infection.

PubMed

Inaba, Yuji; Motobayashi, Mitsuo; Nishioka, Makoto; Kaneko, Tomoki; Yamauchi, Shoko; Kawasaki, Yoichiro; Shiba, Naoko; Nishio, Shin-ya; Moteki, Hideaki; Miyagawa, Maiko; Takumi, Yutaka; Usami, Shin-ichi; Koike, Kenichi

2016-02-01

It is well known that congenital cytomegalovirus infection exhibits white matter and other types of lesions in magnetic resonance imaging (MRI), but little is known on the clinical significance of white matter lesions because they are also present in asymptomatic congenital cytomegalovirus infection. We investigated for relationships among white matter lesions, intelligence quotient, and other neurodevelopmental features. Nine children (five boys and four girls; mean age: 87.4 months, range: 63-127 months) with sensorineural hearing loss (five bilateral and four unilateral) had been diagnosed as having congenital cytomegalovirus infection by positive polymerase chain reaction findings of dried umbilical cords. They were evaluated for the presence of autistic features, tested using Wechsler Intelligence Scale for Children-Fourth Edition for intelligence quotient, and underwent brain MRI to measure white matter lesion localization and volume. At the time of MRI examination (mean age: 69.4 months, range: 19-92 months), white matter lesions were detected in eight of nine patients. Five subjects were diagnosed as having autism spectrum disorders. We observed increased white matter lesion volume was associated with lower intelligence quotient scores (R(2) = 0.533, P = 0.026) but not with autism spectrum disorders. In individuals with congenital cytomegalovirus, an increased white matter lesion volume is associated with lower intelligence quotient scores but not with an increased likelihood of autistic behavior. Copyright © 2016 Elsevier Inc. All rights reserved.

Audiovestibular impairments associated with intracranial hypotension.

PubMed

Choi, Jae-Hwan; Cho, Kee-Yong; Cha, Seung-Yi; Seo, Jae-Deuk; Kim, Min-Ji; Choi, Yu Ri; Kim, Sung-Hee; Kim, Ji-Soo; Choi, Kwang-Dong

2015-10-15

To investigate the patterns and mechanisms of audiovestibular impairments associated with intracranial hypotension. We had consecutively recruited 16 patients with intracranial hypotension at the Neurology Center of Pusan National University Hospital for two years. Spontaneous, gaze-evoked, and positional nystagmus were recorded using 3D video-oculography in all patients, and the majority of them also had pure tone audiometry and bithermal caloric tests. Of the 16 patients, five (31.3%) reported neuro-otological symptoms along with the orthostatic headache while laboratory evaluation demonstrated audiovestibular impairments in ten (62.5%). Oculographic analyses documented spontaneous and/or positional nystagmus in six patients (37.5%) including weak spontaneous vertical nystagmus with positional modulation (n=4) and pure positional nystagmus (n=2). One patient presented with recurrent spontaneous vertigo and tinnitus mimicking Meniere's disease, and showed unidirectional horizontal and torsional nystagmus with normal head impulse tests during the attacks. Bithermal caloric tests were normal in all nine patients tested. Audiometry showed unilateral (n=6) or bilateral (n=1) sensorineural hearing loss in seven (53.8%) of the 13 patients tested. Intracranial hypotension frequently induces audiovestibular impairments. In addition to endolymphatic hydrops and irritation of the vestibulocochlear nerve, compression or traction of the brainstem or cerebellum due to loss of CSF buoyancy may be considered as a mechanism of frequent spontaneous or positional vertical nystagmus in patients with intracranial hypotension. Copyright © 2015 Elsevier B.V. All rights reserved.

External ear anomalies and hearing impairment in Noonan Syndrome.

PubMed

van Trier, Dorothée C; van Nierop, Josephine; Draaisma, Jos M Th; van der Burgt, Ineke; Kunst, Henricus; Croonen, Ellen A; Admiraal, Ronald J C

2015-06-01

This is the first cohort in which hearing impairment and external ear anomalies in Noonan Syndrome are described extensively. Retrospective analysis of the otorhinolaryngological and clinical genetic data from 97 Noonan Syndrome (NS) patients. Forty-four NS patients were seen by an otorhinolaryngologist for the analysis of hearing impairment. In our cohort 80 of the 97 patients were genetically tested. In 71 of these mutations were found: in 48 patients a mutation in PTPN11, in 10 patients in SOS1, in 5 patients in SHOC2, in 5 patients in RAF1, in 1 patient in MAP2K2, in 1 patient in KRAS and in 1 patient in A2ML1. External ear anomalies were reported in 75 NS patients (77%). In 69 patients the ears were low-set, 28 patients had posteriorly rotated ears, 14 patients showed protruding ears and 18 had thickened helices. Hearing impairment was detected in 34 NS patients. Nine patients had sensorineural hearing impairment, two a permanent conductive hearing impairment, two other patients had mixed hearing impairment and 20 patients had conductive hearing impairment in the past, caused by otitis media with effusion. Their temporary conductive hearing impairment resolved between the ages of 2 and 18 years. Sensorineural hearing impairment varied between mild high-frequency hearing impairment and profound (uni- and bilateral) hearing impairment and was progressive in three patients. Four NS patients received cochlear implants for their severe sensorineural hearing impairment. The cohort is small for genotype-phenotype correlations, but sensorineural hearing impairment, especially the bilateral severe hearing impairment, was only seen in patients with a PTPN11 mutation. NS is characterized by dysmorphic external ear anomalies and both sensorineural and conductive hearing impairment. Audiological examinations are recommended in all patients with Noonan Syndrome. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Presumed Arterial Gas Embolism After Breath-Hold Diving in Shallow Water.

PubMed

Harmsen, Stefani; Schramm, Dirk; Karenfort, Michael; Christaras, Andreas; Euler, Michael; Mayatepek, Ertan; Tibussek, Daniel

2015-09-01

Dive-related injuries are relatively common, but almost exclusively occur in recreational or scuba diving. We report 2 children with acute central nervous system complications after breath-hold diving. A 12-year-old boy presented with unilateral leg weakness and paresthesia after diving beneath the water surface for a distance of ∼25 m. After ascent, he suddenly felt extreme thoracic pain that resolved spontaneously. Neurologic examination revealed right leg weakness and sensory deficits with a sensory level at T5. Spinal MRI revealed a nonenhancing T2-hyperintense lesion in the central cord at the level of T1/T2 suggesting a spinal cord edema. A few weeks later, a 13-year-old girl was admitted with acute dizziness, personality changes, confusion, and headache. Thirty minutes before, she had practiced diving beneath the water surface for a distance of ∼25 m. After stepping out, she felt sudden severe thoracic pain and lost consciousness. Shortly later she reported headache and vertigo, and numbness of the complete left side of her body. Neurologic examination revealed reduced sensibility to all modalities, a positive Romberg test, and vertigo. Cerebral MRI revealed no pathologic findings. Both children experienced a strikingly similar clinical course. The chronology of events strongly suggests that both patients were suffering from arterial gas embolism. This condition has been reported for the first time to occur in children after breath-hold diving beneath the water surface without glossopharyngeal insufflation. Copyright © 2015 by the American Academy of Pediatrics.

Effect of day-case unilateral cochlear implantation in adults on general and disease-specific quality of life, postoperative complications and hearing results, tinnitus, vertigo and cost-effectiveness: protocol for a randomised controlled trial

PubMed Central

Derks, Laura S M; Wegner, Inge; Smit, Adriana L; Thomeer, Hans G X M; Topsakal, Vedat; Grolman, Wilko

2016-01-01

Introduction Cochlear implantation is an increasingly common procedure in the treatment of severe to profound sensorineural hearing loss (SNHL) in children and adults. It is often performed as a day-case procedure. The major drive towards day-case surgery has been from a logistical, economical and societal perspective, but we also speculate that the patient's quality of life (QoL) is at least equal to inpatient surgery if not increased as a result of rapid discharge and rehabilitation. Even though cochlear implantation seems well suited to a day-case approach and this even seems to be common practice in some countries, evidence is scarce and of low quality to guide us towards the preferred treatment option. Methods and analysis A single-centre, non-blinded, randomised, controlled trial was designed to (primarily) investigate the effect on general QoL of day-case cochlear implantation compared to inpatient cochlear implantation and (secondarily) the effect of both methods on (subjective) hearing improvement, disease-specific QoL, tinnitus, vertigo and cost-effectiveness. 30 adult patients with severe to profound bilateral postlingual SNHL who are eligible for unilateral cochlear implantation will be randomly assigned to either the day-case or inpatient treatment group. The outcome measures will be assessed using auditory evaluations, questionnaires (preoperatively, at 1-week, 3-week, 3-month and 1-year follow-up) and costs diaries (weekly during the first month postoperatively, after which once in a month until 1-year follow-up). Preoperative and postoperative outcomes will be compared. The difference in costs and benefit will be represented using the incremental cost utility/effectiveness ratio. The analyses will be carried out on an intention-to-treat basis. Ethics and dissemination This research protocol was approved by the Institutional Review Board of the UMC Utrecht (NL45590.041.13; V.5, November 2015). The trial results will be disseminated through peer-reviewed medical journals and presented at scientific conferences. Trial registration number NTR4464; Pre-results. PMID:27697874

The role of HRCT and three-dimensional VR CT findings in patients of congenital atresia combined with microtia.

PubMed

Gao, Ruzhen; Wang, Yun; Fan, Yue; Ai, Xing; Zhang, Xiaona; Xue, Huadan; Chen, Xiaowei; Jin, Zhengyu

2012-12-01

To determine the anatomic differences in patients of atresia by using high-resolution computed tomography (HRCT) and 3D volume rendered (VR) CT. High-resolution computed tomography (HRCT) was performed in 43 atresia patients including 34 unilateral atresia patients (n=34, 26 males, 8 females, mean age 13.82 years, range 8-19 years) and 9 bilateral atresia patients (6 males, 3 females, mean age 13.2 years, range 9-19 years). HRCT and 3D VR findings were compared with those in 43 normal ears of the unilateral atresia patients with normal PTA results (n=34, 26 males, 8 females, mean age 13.82 years, range 8-19 years) and 11 patients with sensorineural hearing loss but with no associated aplasia of the middle and inner ear (n=22, 7 males and 4 females, range 8-20.8 years, median age of 13.4 years) by using the independent one sample T test. On the HRCT images, the angle between the basic line and the tympanic segment of the facial nerve is more acute. And the area of the malleus-incus-joint or the malleus-incus-complex in the diseased ears is smaller than that in the control subjects (P<0.05). The tympanic segment is shorter and the area of the tympanic cavity is smaller in the atresia group, while the diameter of the oval window is also smaller in atresia group than that in the control group (P<0.05). The morphologic differences of the small ossicles and the entire length of the tympanic and mastoid segments can be depicted on a single 3D VR CT image. The facial nerve demonstrates abnormal lateral and anterior displacement in the CAA patients and the area of the Malleus-incus-joint and the tympanic cavity are significantly smaller, and the oval window is much narrower in the control group. HRCT and 3D VR CT provide valuable information about preoperative planning of patients with CAA. Measurements of all the angles and length serve as useful adjunct measurements in determining surgical candidacy. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

Digenic mutations involving both the BSND and GJB2 genes detected in Bartter syndrome type IV.

PubMed

Wang, Hong-Han; Feng, Yong; Li, Hai-Bo; Wu, Hong; Mei, Ling-Yun; Wang, Xing-Wei; Jiang, Lu; He, Chu-Feng

2017-01-01

Bartter syndrome type IV, characterized by salt-losing nephropathies and sensorineural deafness, is caused by mutations of BSND or simultaneous mutations of both CLCNKA and CLCNKB. GJB2 is the primary causative gene for non-syndromic sensorineural deafness and associated with several syndromic sensorineural deafness. Owing to the rarity of Bartter syndrome, only a few mutations have been reported in the abovementioned causative genes. To investigate the underlying mutations in a Chinese patient with Bartter syndrome type IV, genetic analysis of BSND, CLCNKA, CLCNKB and GJB2 were performed by polymerase chain reaction and direct sequencing. Finally, double homozygous mutations c.22C > T (p.Arg8Trp) and c.127G > A (Val43Ile) were detected in exon 1 of BSND. Intriguingly, compound heterozygous mutations c.235delC (p.Leu79CysfsX3) and c.109G > A (p.Val37Ile) were also revealed in exon 2 of GJB2 in the same patient. No pathogenic mutations were found in CLCNKA and CLCNKB. Our results indicated that the homozygous mutation c.22C > T was the key genetic reason for the proband, and a digenic effect of BSND and GJB2 might contributed to sensorineural deafness. To our knowledge, it was the first report showing that the GJB2 gene mutations were detected in Bartter syndrome. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Evaluation of very low birth weight (≤ 1,500 g) as a risk indicator for sensorineural hearing loss.

PubMed

Borkoski-Barreiro, Silvia A; Falcón-González, Juan C; Limiñana-Cañal, José M; Ramos-Macías, Angel

2013-01-01

Hearing plays an essential role in the acquisition, development and maintenance of the properties of the speech and language. Birth weight is an indicator of biological maturation of the newborn. Premature newborns with very low birth weight (VLBW<1,500 g) constitute a group with the highest risk of sensorineural hearing loss. Our objective was to ascertain the degree of hearing loss, sensorineural hearing loss and presence of the association to other risk factors for hearing loss in VLBW infants included in the Universal Hearing Loss Screening Programme at the University Mother-Child Hospital of Gran Canaria (Spain) in the 2007-2010 period. This was a retrospective study of 364 infants with VLBW, measured by transient evoked otoacoustic emissions and auditory brainstem response. There were 112 newborn (30.8%) referred for auditory brainstem response. A diagnosis of hearing loss was given to 22 newborns (2.2%), 14 had conductive hearing loss and 8, sensorineural hearing loss (SNHL), of which 2 had bilateral profound hearing loss. The VLBW newborn presented the association to another risk factor in more than a quarter of the sample studied. All those diagnosed with SNHL were premature. The percentage of VLBW newborns diagnosed with hearing loss is higher than expected in the general population. All those diagnosed with SNHL were premature and presented one or 2 hearing risk factors associated with VLBW. Copyright © 2013 Elsevier España, S.L. All rights reserved.

"Gun-shooting hearing loss": A pilot study.

PubMed

Sataloff, Joseph; Hawkshaw, Mary J; Sataloff, Robert T

2010-01-01

Gun-shooting deafness is the common terminology applied to sensorineural hearing loss caused by shooting firearms. Many characteristics of gun-shooting hearing loss have been proposed, but they have not been defined clearly or established conclusively. We studied 37 users of recreational firearms to obtain pilot data to help determine if it is true that right-handed gun shooters develop more hearing loss in the left ear and vice versa, whether everyone who frequently shoots guns develops sensorineural hearing loss, and whether significant hearing loss is typically prevented by wearing commercially available ear protectors while shooting.

[Norrie-Wardburg syndrome].

PubMed

Skevas, A; Kastanioudakis, I; Daniilidis, B; Exarchakos, G

1992-10-01

We describe a case of a 25-year old patient with typical Norrie-Warburg Syndrome. From the first year of his life he was found to be blind, with bilateral sensorineural loss of hearing. Audiological examination showed symmetrical moderate bilateral sensorineural hearing loss. His hearing loss was refractory to treatment for the last eight years. Because of timely diagnosis of hearing loss and timely fitting of a hearing aid, the patient could study at school and graduate from university education. Disease carriers who are clinically healthy can be identified only via chromosome analysis.

Co-segregation of Norrie disease and idiopathic pulmonary hypertension in a family with a microdeletion of the NDP region at Xp11.3-p11.4.

PubMed

Staropoli, John F; Xin, Winnie; Sims, Katherine B

2010-11-01

Norrie disease is a rare X-linked congenital retinal vasculopathy that may be accompanied by sensorineural deafness, mental retardation, and other neurological deficits. Here we present a family in which Norrie disease co-segregated with either early-onset idiopathic pulmonary hypertension or sudden death preceded by a period of progressive dyspnea. Neither Norrie disease, nor its atypical variants described to date, have been associated with this extended clinical phenotype. Molecular analysis of the Norrie disease gene (NDP) and adjacent loci was performed by multiplex ligation-dependent probe amplification and comparative genomic hybridisation. Affected males in this family showed an inherited hemizygous deletion restricted to NDP and two immediately telomeric genes, monoamine oxidase-B (MAO-B) and monoamine oxidase-A (MAO-A), which encode closely related enzymes that metabolize biogenic amines including serotonin, dopamine, and norepinephrine. Sequencing of the deletion junction showed an unusual pattern in which a region of microhomology flanked intervening genomic sequence. Because abnormalities of biogenic amines, particularly serotonin, have been implicated in the pathophysiology of pulmonary hypertension, we propose that presumed MAO deficiency in these patients may represent a novel risk factor for pulmonary hypertension, particularly forms with very early onset. Fine-mapping of other microdeletions at this locus may provide insights into additional mechanisms for nonrecurrent genomic rearrangements at this and other chromosomal loci.

[Intratympanic injection therapy for therapy refractory acute hearing loss: A safe option for secondary treatment].

PubMed

Mühlmeier, G; Maier, S; Maier, M; Maier, H

2015-10-01

High-dose corticosteroids are currently recommended for idiopathic sudden sensorineural hearing loss (ISSNHL) treatment. Intratympanic injections (ITI) are of growing importance, especially in cases of therapy resistance. The selection of patients for this procedure in SSNHL has not been adequately examined so far. A total of 77 patients with ISSNHL after ineffective systemic pretreatment underwent intratympanic administration of dexamethasone and hyaluronic acid. Improvement after treatment was determined by pure tone audiometry for both ears before and of the treated ear after ITI. In this study 34 female and 43 male patients with mean age of 57 years showed a pre-ITI hearing loss of 35 dB in the lower frequencies and 69 dB in the higher frequencies. The mean hearing gain was 10 dB and the response rate was 62%. Absolute hearing gain revealed significant improvements at 500 Hz, 1 kHz and 2 kHz. Under inclusion of contralateral thresholds there were hardly any differences up to 4 kHz. In a detailed analysis of responders moderate improvements could be observed even in higher frequencies. Overall, no relevant adverse events occurred. Treatment of ISSNHL resistant to systemic regimens by ITI of steroids provides an option that offers additional prospects of auditory improvement for affected patients. The presented results indicate that these modalities are also valid for patients with pancochlear ISSNHL.

Steroid intracochlear distribution differs by administration method: Systemic versus intratympanic injection.

PubMed

Lee, Jong Joo; Jang, Jeong Hun; Choo, Oak-Sung; Lim, Hye Jin; Choung, Yun-Hoon

2018-01-01

Steroids have been widely used to treat inner-ear diseases such as sudden sensorineural hearing loss, tinnitus, and Meniere's disease. They can be given via either systemic or intratympanic (IT) injection. The purpose of the present study was to explore differences in intracochlear steroid distribution by the administration method employed (systemic vs. IT injection). Animal study. Twenty-three Sprague-Dawley rats were given fluorescein isothiocyanate-labeled dexamethasone (FITC-DEX) three times (on successive days) via intraperitoneal (IP) or IT injection. Cochlear uptake of FITC-DEX was evaluated via immunohistochemistry and flow cytometry at 6 hours, and 3 and 7 days after the final injection. FITC-DEX uptake was evident in spiral ganglion cells (SGs), the organ of Corti (OC), and the lateral walls (LWs), the basal turns of which were stained relatively prominently in both groups. Animals receiving IP injections exhibited higher FITC-DEX uptakes by the SGs and OC, whereas IT injection triggered higher-level FITC-DEX accumulation by the OC and LWs. Flow cytometry revealed that intracochlear FITC-DEX uptake by IT-injected animals was higher and more prolonged than in animals subjected to IP injections. We thus describe differences in cochlear steroid distributions after systemic and IT injections. This finding could help our understanding of the pharmacokinetics of steroids in the cochlea. NA. Laryngoscope, 128:189-194, 2018. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

Factors predicting early diagnosis of pediatric laryngotracheobronchial foreign bodies.

PubMed

Parida, Pradipta; Nirmal, Nirmal Shanmugasundaram; Gopalakrishnan, Surianarayanan; Saxena, Sunil Kumar

2015-01-01

To study the clinical and radiological findings to make early diagnosis of foreign body (FB) aspiration in children. This prospective study was conducted on 45 children below 12 years of age with a clinical diagnosis of FB aspiration undergone rigid bronchoscopy from September 2008 to may 2010. Bronchoscopy was positive for FB in 37 children. The results of these 37 children (15 female and 22 male) were analyzed. 81% of children were in age group of 1-3 years. Penetration syndrome (sudden onset coughing, choking and gagging when the child is having something in the mouth) (89.2%) and decreased breath sounds (86.5%) were the most common clinical features. Cough, respiratory difficulty and fever were present in 83.8%, 78.4% and 27% respectively. Tachypnoea, tachycardia, chest retractions, decreased chest movements and wheeze were present in 83.3%, 83.3%, 83.3%, 51.4% and 43.2% respectively. Unilateral hyperinflation (64.9%), mediastinal shift (45.9%), and collapse (21.6%) were common radiological sign on chest radiograph and in 13.5% patients the chest X-rays were normal. Sites of FB lodgments were larynx, trachea, right main bronchus, left main bronchus and bilateral bronchi in 10.8%, 10.8%, 35.1%, 37.8 and 5.5% respectively. Food related FBs were present in 30 cases (peanut in 54.1%) and inorganic FBs were present in 7 cases. FBs were removed successfully by rigid bronchoscopy in all cases without any mortality. Penetration syndrome, localized decreased breath sounds, unilateral hyperinflation and/or mediastinal shift on radiology are predictors for early diagnosis of FB aspiration.

The relationship between neonatal hyperbilirubinemia and sensorineural hearing loss.

PubMed

Corujo-Santana, Cándido; Falcón-González, Juan Carlos; Borkoski-Barreiro, Silvia Andrea; Pérez-Plasencia, Daniel; Ramos-Macías, Ángel

2015-01-01

Severe jaundice that requires exchange transfusion has become a relatively rare situation today. About 60% of full term neonates and 80% of premature ones will suffer from jaundice within the first week of life. Hyperbilirubinemia at birth is a risk factor associated with hearing loss that is usually further linked to other factors that might have an effect on hearing synergistically. This study aimed to identify the relationship between hyperbilirubinemia at birth as a risk factor for sensorineural hearing loss in children born at Complejo Hospitalario Universitario Insular Materno-Infantil de Gran Canaria, in the 2007-2011 period. This was a retrospective study of 796 newborns that had hyperbilirubinemia at birth, using transient evoked otoacoustic emissions and evoked auditory brainstem response. Hundred eighty-five newborns (23.24%) were referred for evoked auditory brainstem response. Hearing loss was diagnosed for 35 (4.39%): 18 neonates (51.43%) with conductive hearing loss and 17 (48.57%) with sensorineural hearing loss, 3 of which were diagnosed as bilateral profound hearing loss. Half of the children had other risk factors associated, the most frequent being exposure to ototoxic medications. The percentage of children diagnosed with sensorineural hearing loss that suffered hyperbilirubinemia at birth is higher than for the general population. Of those diagnosed, none had levels of indirect bilirubin≥20mg/dl, only 47% had hyperbilirubinemia at birth as a risk factor and 53% had another auditory risk factor associated. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

Prevalence of otitis media and risk-factors for sensorineural hearing loss among infants attending Child Welfare Clinics in the Solomon Islands.

PubMed

Kaspar, Annette; Newton, Obiga; Kei, Joseph; Driscoll, Carlie; Swanepoel, De Wet; Goulios, Helen

2018-08-01

The present study aimed to assess the prevalence of otitis media and risk-factors for sensorineural hearing loss among infants in the Solomon Islands, in order to recommend an Infant Ear and Hearing Program that would be suitable to the Pacific Island context. Ear examinations and the JCIH Risk-Factor Questionnaire were administered to 288 infants attending Child Welfare Clinics in the Solomon Islands. Overall, 150 infants (52.084%) presented with bilateral normal ear examinations and no risk-factors for SNHL. There were 73 infants (25.34%) with ear pathology in at least one ear, 13 (4.5%) of whom required referral to the ENT Clinic for medical management. The most common pathology was otitis media with effusion (OME) (21.87%). Infants aged 7-12 months were significantly more likely to present with OME (p<0.001) and a history of otitis media (p=0.017) than infants aged 0-6 months. There were 71 infants (24.65%) with at least one risk-factor for sensorineural hearing loss. The most common risk-factors were ototoxicity (8.3%), non-elective caesarean delivery (6.59%), and possible in-utero syphilis infection (5.55%). The prevalence of otitis media and risk-factors for sensorineural hearing loss indicate the importance of initiating Infant Ear and Hearing Programs in the Solomon Islands. Program should facilitate early education on prevention of ear disease, as well as early diagnosis and management of children with hearing loss. Copyright © 2018. Published by Elsevier B.V.

Work disability after diagnosis of hand-arm vibration syndrome.

PubMed

Sauni, Riitta; Toivio, Pauliina; Pääkkönen, Rauno; Malmström, Jari; Uitti, Jukka

2015-11-01

Our aim was to study the course of vasospastic and sensorineural symptoms after the clinical diagnosis of hand-arm vibration syndrome (HAVS), and the association of current HAVS symptoms with occupational status, self-evaluation of health, quality of life, and work ability. We gathered all HAVS cases diagnosed at the Finnish Institute of Occupational Health in Helsinki and Tampere during 1990-2008. A questionnaire was sent to all these patients (n = 241). Altogether 149 of them (62 %) returned the questionnaire. Cumulative lifelong vibration exposure was evaluated on the basis of the data in the patient files. On average, 8.5 years after the diagnosis of HAVS, approximately one-third of the patients reported improvement in symptoms of vibration-induced white finger (VWF) and the sensorineural symptoms. Young age and shorter exposure time were associated with improvement in VWF symptoms (p = 0.033 and p < 0.001, respectively). Persistent or deteriorated symptoms of both VWF and sensorineural symptoms were associated with lowered work ability, quality of life (EQ-5D), and general health, also after adjusting for age, smoking, and diseases other than HAVS. The patients' own prediction of work ability in 2 years was more negative if the VWF symptoms or sensorineural symptoms had continued after diagnosis of HAVS (p = 0.065 and p = 0.001, respectively). Our results suggest that in about two-thirds of the patients, the HAVS symptoms may stabilize or deteriorate in the follow-up. Considering the effects on work ability, timely prevention measures should be taken more actively to help patients continue their working careers.

[Choroidal neovascularization followed in a patient with "Multiple Evanescent White Dot Syndrome" (MEWDS) -- a case report].

PubMed

Löw, U; Palmowski, A M; Weich, C-M; Ruprecht, K W

2004-12-01

Since the description of the "multiple evanescent white dot syndrome" (MEWDS) by Jampol et al, choroiditis has been in the focus of interest. But the classical type of MEWDS was an exceptional case in clinical routine. A 48-year-old female presented to our hospital with a sudden unilateral visual acuity decrease and an extension of the blind spot. Ophthalmoscopy and fluorescein angiography revealed typical multiple grey-white chorioretinal patches of the same stage with lesion areas of about 100 - 200 microm compatible with the diagnose of MEWDS. Although visual acuity increased continuously the patient developed a classical choroidal neovascularization within 4 weeks. She was treated with PDT and visual acuity as well as the ophthalmoscopic diagnosis remained stable. In spite of visual improvement in MEWDS, regular control is recommended. In addition we propose to consider the diagnosis of MEWDS if an enlargement of the blind spot and CNV without lesions of the retinal pigment epithelium are diagnosed.

Simulation of a slope adapting ankle prosthesis provided by semi-active damping.

PubMed

LaPrè, Andrew K; Sup, Frank

2011-01-01

Modern passive prosthetic foot/ankles cannot adapt to variations in ground slope. The lack of active adaptation significantly compromises an amputee's balance and stability on uneven terrains. To address this deficit, this paper proposes an ankle prosthesis that uses semi-active damping as a mechanism to provide active slope adaptation. The conceptual ankle prosthesis consists of a modulated damper in series with a spring foot that allows the foot to conform to the angle of the surface in the sagittal plane. In support of this approach, biomechanics data is presented showing unilateral transtibial amputees stepping on a wedge with their daily-use passive prosthesis. Based on this data, a simulation of the ankle prosthesis with semi-active damping is developed. The model shows the kinematic adaptation of the prosthesis to sudden changes in ground slope. The results show the potential of an ankle prosthesis with semi-active damping to actively adapt to the ground slope at each step.

Minimizing Confusion and Disorientation: Cognitive Support Work in Informal Dementia Caregiving

PubMed Central

Berry, Brandon

2015-01-01

Drawing from ethnographic fieldwork and in-depth interviews, I explain how informal dementia caregivers attempt to reduce the affected individual’s moments of confusion and disorientation through cognitive support work. I identify three stages through which such support takes shape and then gradually declines in usage. In a first stage, family members collaborate with affected individuals to first identify and then to avoid “triggers” that elicit sudden bouts of confusion. In a second stage, caregivers lose the effective collaboration of the affected individual and begin unilateral attempts to minimize confused states through pre-emptive conversational techniques, third-party interactional support, and social-environment shifts. In a third stage, caregivers learn that the affected individual has reached a level of impairment that does not respond well to efforts at reduction and begin abandoning strategies. I identify the motivations driving cognitive support work and discuss the role of lay health knowledge in dementia caregiving. I conclude by considering the utility of cognitive support as a concept within dementia caregiving. PMID:24984915

Knee Locking in Osteoarthritis due to Synovial Lipoma: A Case Report

PubMed Central

S. Amarjit, Kataria; Budhiraja, Shivali; Chandramouleeswari, K.; Anita, S.

2013-01-01

Intra–articular synovial lipomas are very rare and only few cases have been reported till now. We are reporting a rare case of a unilateral intra–articular lipoma of osteoarthritic knee joint in a 62 years old male. Patient had two episodes of sudden locking of knee joint, which resolved spontaneously. A plain X-ray showed changes which were suggestive of osteoarthritis. Clinically, patient was diagnosed as a case of loose bodies in left knee joint. An arthrotomy was performed. After a Histopathological Examination (HPE) of loose bodies, a diagnosis of an intra–articular synovial lipoma was made. Due to wide differentials and varied clinical behaviour of loose bodies, lipoma should be included in differential diagnosis of osteroarthritic patients who complain of episodic locking of knees. Intraarticular lipomas, on arthroscopic guided excision, get cured permanently, with no recurrence. The differentiation of an intra-articular lipoma from a relatively more common entity, Lipoma arborescens, has also been discussed. PMID:24086885

Common questions about Bell palsy.

PubMed

Albers, Janet R; Tamang, Stephen

2014-02-01

Bell palsy is an acute affliction of the facial nerve, resulting in sudden paralysis or weakness of the muscles on one side of the face. Testing patients with unilateral facial paralysis for diabetes mellitus or Lyme disease is not routinely recommended. Patients with Lyme disease typically present with additional manifestations, such as arthritis, rash, or facial swelling. Diabetes may be a comorbidity of Bell palsy, but testing is not needed in the absence of other indications, such as hypertension. In patients with atypical symptoms, magnetic resonance imaging with contrast enhancement can be used to rule out cranial mass effect and to add prognostic value. Steroids improve resolution of symptoms in patients with Bell palsy and remain the preferred treatment. Antiviral agents have a limited role, and may improve outcomes when combined with steroids in patients with severe symptoms. When facial paralysis is prolonged, surgery may be indicated to prevent ocular desiccation secondary to incomplete eyelid closure. Facial nerve decompression is rarely indicated or performed. Physical therapy modalities, including electrostimulation, exercise, and massage, are neither beneficial nor harmful.

[Built-in emergency brake in the balance system. Animal experiment research shows that a hierarchy of mechanisms compensate after acute peripheral vestibular decline].

PubMed

Magnusson, Anna K; Tham, Richard

A sudden unilateral loss of peripheral vestibular input results in the onset of acute dizziness and imbalance associated with spontaneous nystagmus, postural instability and nausea. Fortunately, these symptoms ameliorate rapidly, even without treatment, due to central nervous plastic changes which are collectively termed "vestibular compensation". This concept has become a widely accepted research model for studying lesion-induced plasticity. Recent research has dealt in particular with the plasticity of the medial vestibular nuclei that mediate the horizontal vestibulo-ocular reflex. Studies range from a cellular level in vitro to a functional level in vivo. Taken together, results from such studies have contributed greatly to what is known of vestibular compensation today. This article summarises evidence for several plasticity mechanisms that drive the recovery of spontaneous nystagmus, one of which is dependent on an endocrine stress-response. In the long run, such knowledge might influence the management and treatment of patients with balance disorders.

[Trigeminal motor paralysis and dislocation of the temporo-mandibular joints].

PubMed

Ohkawa, S; Yoshida, T; Ohsumi, Y; Tabuchi, M

1996-07-01

A 64-year-old woman with diabetes mellitus was admitted to our hospital with left hemiparesis of sudden onset. A brain MRI demonstrated a cerebral infarction in the ventral part of the right lower pons. When left hemiparesis worsened, she had dislocation of the temporo-mandibular joints repeatedly. Then, her lower jaw deviated to the right when she opened her mouth. Also, there was decreased contraction of the right masseter when she clenched her teeth. These findings suggest that there was trigeminal motor paralysis on the right side resulting from involvement of the intrapontine trigeminal motor nerve. She has no history of dislocation of the temporo-mandibular joints. An X-ray film showed that the temporo-mandibular joints were intact. Thus, it is possible that deviation of the lower jaw was the cause of this dislocation. We suspect that dislocation of the temporo-mandibular joints may occur as a complication of unilateral trigeminal motor paralysis. This has not been reported to our knowledge.

Exophthalmos associated to orbital zygomatic mucocele and complex maxillary malformation in a puppy

PubMed Central

Cirla, Alessandro; Rondena, Marco; Bertolini, Giovanna; Barsotti, Giovanni

2017-01-01

A case of exophthalmos due to zygomatic mucocele in a puppy with ipsilateral segmental maxillary atrophy is reported. A 7-month-old, mixed breed, male dog suffered the sudden-onset of unilateral painful exophthalmos and a gradual swelling of the right temporal region. A compressing, right retrobulbar mass was observed by ultrasound. Computed tomography revealed a large multiloculated cyst-like lesion of the right zygomatic gland projecting into the orbital space, thus displacing the eyeball. The ipsilateral molar part of the maxillary bone was underdeveloped, besides showing crowded, abnormal, multiple, unerupted maxillary molar teeth in the caudal maxillary region. Modified lateral orbitotomy and a selective caudal maxillary bone access were performed. The cyst-like lesion was removed and the zygomatic gland and the wall was collected for histology, which confirmed the mucocele. Clinical and imaging examinations six months after surgery showed neither recurrence of the mucocele nor ocular abnormalities. A possible common pathogenic mechanism involving these two conditions could be hypothesized. PMID:28884074

Asymmetric hearing loss in a random population of patients with mild to moderate sensorineural hearing loss.

PubMed

Segal, Nili; Shkolnik, Mark; Kochba, Anat; Segal, Avichai; Kraus, Mordechai

2007-01-01

We evaluated the correlation of asymmetric hearing loss, in a random population of patients with mild to moderate sensorineural hearing loss, to several clinical factors such as age, sex, handedness, and noise exposure. We randomly selected, from 8 hearing institutes in Israel, 429 patients with sensorineural hearing loss of at least 30 dB at one frequency and a speech reception threshold not exceeding 30 dB. Patients with middle ear disease or retrocochlear disorders were excluded. The results of audiometric examinations were compared binaurally and in relation to the selected factors. The left ear's hearing threshold level was significantly higher than that of the right ear at all frequencies except 1.0 kHz (p < .05). One hundred fifty patients (35%) had asymmetric hearing loss (more than 10 dB difference between ears). In most of the patients (85%) the binaural difference in hearing threshold level, at any frequency, was less than 20 dB. Age, handedness, and sex were not found to be correlated to asymmetric hearing loss. Noise exposure was found to be correlated to asymmetric hearing loss.

Impedance testing on cochlear implants after electroconvulsive therapy.

PubMed

McRackan, Theodore R; Rivas, Alejandro; Hedley-Williams, Andrea; Raj, Vidya; Dietrich, Mary S; Clark, Nathaniel K; Labadie, Robert F

2014-12-01

Cochlear implants (CI) are neural prostheses that restore hearing to individuals with profound sensorineural hearing loss. The surgically implanted component consists of an electrode array, which is threaded into the cochlea, and an electronic processor, which is buried under the skin behind the ear. The Food and Drug Administration and CI manufacturers contend that electroconvulsive therapy (ECT) is contraindicated in CI recipients owing to risk of damage to the implant and/or the patient. We hypothesized that ECT does no electrical damage to CIs. Ten functional CIs were implanted in 5 fresh cadaveric human heads. Each head then received a consecutive series of 12 unilateral ECT sessions applying maximum full pulse-width energy settings. Electroconvulsive therapy was delivered contralaterally to 5 CIs and ipsilaterally to 5 CIs. Electrical integrity testing (impedance testing) of the electrode array was performed before and after CI insertion, and after the first, third, fifth, seventh, ninth, and 12th ECT sessions. Electroconvulsive therapy was performed by a staff psychiatrist experienced with the technique. Explanted CIs were sent back to the manufacturer for further integrity testing. No electrical damage was identified during impedance testing. Overall, there were statistically significant decreases in impedances (consistent with no electrical damage) when comparing pre-ECT impedance values to those after 12 sessions. There was no statistically significant difference (P > 0.05) in impedance values comparing ipsilateral to contralateral ECT. Manufacturer testing revealed no other electrical damage to the CIs. Electroconvulsive therapy does not seem to cause any detectable electrical injury to CIs.

Hearing rehabilitation with single-stage bilateral vibroplasty in a child with Franceschetti syndrome.

PubMed

Sargsyan, Sona; Rahne, Torsten; Kösling, Sabrina; Eichler, Gerburg; Plontke, Stefan K

2014-05-01

Hearing is of utmost importance for normal speech and social development. Even children who have mild or unilateral permanent hearing loss may experience difficulties with understanding speech, as well as problems with educational and psycho-social development. The increasing advantages of middle-ear implant technologies are opening new perspectives for restoring hearing. Active middle-ear implants can be used in children and adolescents with hearing loss. In addition to the well-documented results for improving speech intelligibility and quality of hearing in sensorineural hearing loss active middle-ear implants are now successfully used in patients with conductive and mixed hearing loss. In this article we present a case of successful, single-stage vibroplasty, on the right side with the fixation of the FMT on the stapes and PORP CLiP vibroplasty on the left side in a 6-year-old girl with bilateral mixed hearing loss and multiple dyslalia associated with Franceschetti syndrome (mandibulofacial dysostosis). CT revealed bilateral middle-ear malformations as well as an atretic right and stenotic left external auditory canal. Due to craniofacial dysmorphia airway and (post)operative, management is significantly more difficult in patients with a Franceschetti syndrome which in this case favoured a single-stage bilateral procedure. No intra- or postoperative surgical complications were reported. The middle-ear implants were activated 4 weeks after surgery. In the audiological examination 6 months after surgery, the child showed 100% speech intelligibility with activated implants on each side.

Cochlear implantation for severe sensorineural hearing loss caused by lightning.

PubMed

Myung, Nam-Suk; Lee, Il-Woo; Goh, Eui-Kyung; Kong, Soo-Keun

2012-01-01

Lightning strike can produce an array of clinical symptoms and injuries. It may damage multiple organs and cause auditory injuries ranging from transient hearing loss and vertigo to complete disruption of the auditory system. Tympanic-membrane rupture is relatively common in patients with lightning injury. The exact pathogenetic mechanisms of auditory lesions in lightning survivors have not been fully elucidated. We report the case of a 45-year-old woman with bilateral profound sensorineural hearing loss caused by a lightning strike, who was successfully rehabilitated after a cochlear implantation. Copyright © 2012 Elsevier Inc. All rights reserved.

[Dichotic digit test. Case].

PubMed

Zenker Castro, Franz; Fernández Belda, Rafael; Barajas de Prat, José Juan

2008-12-01

In this study we present a case of a 71-year-old female patient with sensorineural hearing loss and fitted with bilateral hearing aids. The patient complained of scant benefit from the hearing aid fitting with difficulties in understanding speech with background noise. The otolaryngology examination was normal. Audiological tests revealed bilateral sensorineural hearing loss with threshold values of 51 and 50 dB HL in the right and left ear. The Dichotic Digit Test was administered in a divided attention mode and focalizing the attention to each ear. Results in this test are consistent with a Central Auditory Processing Disorder.

Narrow-band evoked oto-acoustic emission from ears with normal and pathologic conditions.

PubMed

Takeda, Taizo; Kakigi, Akinobu; Takebayashi, Shinji; Ohono, Satoshi; Nishioka, Rie; Nakatani, Hiroaki

2010-01-01

Evoked oto-acoustic emission (EOAE), in particular the slow component, is fragile with the inner ear lesions and is apt to disappear in impaired ears. This presence is thought to mean that inner ear is not badly damaged, and that the presence of EOAEs in early stage sudden deafness carries a good prognosis. Narrow-band EOAE analysis would open a potentially promising way to manage sensorineural deafness. The aim of present study was to evaluate the characteristics of EOAEs from pathologic ears by a narrow-band EOAE analysis, which allowed us to investigate amplitude, frequency content and latency of EOAEs simultaneously and also to easily detect weak echoes in cases with inner ear lesions. EOAEs were analyzed by investigating narrow-band frequency contents of EOAEs, filtered by a 100-Hz step of pass bandwidth in frequency regions from 1.0 to 2.0 kHz, and by 500 Hz of pass bandwidth in the frequency ranges of 0.5-1.0 and 2.0-5.0 kHz. EOAE testing was performed in 40 normal ears and 111 ears with pathologic disorders, including sudden deafness, Ménière's disease and surgically proven acoustic neurinomas. Spontaneous oto-acoustic emission was investigated in some cases. In acoustic neurinoma, especially computed tomography scan and magnetic resonance imaging tests were performed to assess the tumor size. (1) Narrow-band EOAE analysis revealed that EOAEs from normal ears were composed of two main echo trains and several sub-echoes. The main echo trains were divided into a fast component with a short latency of <10 ms and a slow component with a long latency of >10 ms. (2) EOAEs could often be detected from ears with moderate to severe hearing loss >45 dB HL in early stage sudden deafness. The prognosis of sudden deafness was good in cases where both a fast component and slow component were detected in the acute stage within 2 weeks after the deafness onset, and was pessimistic, when either or both of them failed to recover. (3) In Ménière's disease, EOAE was found in 6 (40%) of 15 cases with hearing loss >50 dB, and detected in 54 (90%) of 60 cases with slight to moderate deafness <50 dB HL. Echo duration tended to become shorter, and the slow component decreased in amplitude even in ears with slight deafness <30 dB. The detection threshold of the slow component was also elevated. In ears with more advanced deafness, the slow component disappeared and only the fast component with short latency persisted. Ultimately, the fast component also faded out if the hearing was severely impaired. (4) EOAEs were detectable in 20 (95.2%) of 21 ears with surgically proven acoustic neurinoma, 16 of which had both the slow and fast components. The echo pattern of acoustic neurinoma was basically similar to that of normal ears, but the detection threshold was elevated to a varying degree, although there were some cases with much better detection threshold as compared with severe deafness.

A Sound Therapy-Based Intervention to Expand the Auditory Dynamic Range for Loudness among Persons with Sensorineural Hearing Losses: Case Evidence Showcasing Treatment Efficacy

PubMed Central

Formby, Craig; Sherlock, LaGuinn P.; Hawley, Monica L.; Gold, Susan L.

2017-01-01

Case evidence is presented that highlights the clinical relevance and significance of a novel sound therapy-based treatment. This intervention has been shown to be efficacious in a randomized controlled trial for promoting expansion of the dynamic range for loudness and increased sound tolerance among persons with sensorineural hearing losses. Prior to treatment, these individuals were unable to use aided sound effectively because of their limited dynamic ranges. These promising treatment effects are shown in this article to be functionally significant, giving rise to improved speech understanding and enhanced hearing aid benefit and satisfaction, and, in turn, to enhanced quality of life posttreatment. These posttreatment sound therapy effects also are shown to be sustained, in whole or part, with aided environmental sound and to be dependent on specialized counseling to maximize treatment benefit. Importantly, the treatment appears to be efficacious for hearing-impaired persons with primary hyperacusis (i.e., abnormally reduced loudness discomfort levels [LDLs]) and for persons with loudness recruitment (i.e., LDLs within the typical range), which suggests the intervention should generalize across most individuals with reduced dynamic ranges owing to sensorineural hearing loss. An exception presented in this article is for a person describing the perceptual experience of pronounced loudness adaptation, which apparently rendered the sound therapy inaudible and ineffectual for this individual. Ultimately, these case examples showcase the enormous potential of a surprisingly simple sound therapy intervention, which has utility for virtually all audiologists to master and empower the adaptive plasticity of the auditory system to achieve remarkable treatment benefits for large numbers of individuals with sensorineural hearing losses. PMID:28286368

Perceived morbidity and community burden after a Chikungunya outbreak: the TELECHIK survey, a population-based cohort study

PubMed Central

2011-01-01

Background Persistent disabilities are key manifestations of Chikungunya virus (CHIKV) infection, especially incapacitating polyarthralgia and fatigue. So far, little is known about their impact on health status. The present study aimed at describing the burden of CHIKV prolonged or late-onset symptoms on the self-perceived health of La Réunion islanders. Methods At 18 months after an outbreak of Chikungunya virus, we implemented the TELECHIK survey; a retrospective cohort study conducted on a random sample of the representative SEROCHIK population-based survey. A total of 1,094 subjects sampled for CHIKV-specific IgG antibodies in the setting of La Réunion island in the Indian Ocean, between August 2006 and October 2006, were interviewed about current symptoms divided into musculoskeletal/rheumatic, fatigue, cerebral, sensorineural, digestive and dermatological categories. Results At the time of interview, 43% of seropositive (CHIK+) subjects reported musculoskeletal pain (vs 17% of seronegative (CHIK-) subjects, P < 0.001), 54% fatigue (vs 46%, P = 0.04), 75% cerebral disorders (vs 57%, P < 0.001), 49% sensorineural impairments (vs 37%, P = 0.001), 18% digestive complaints (vs 15%, P = 0.21), and 36% skin involvement (vs 34%, P = 0.20) on average 2 years after infection (range: 15-34 months). After controlling for confounders such as age, gender, body mass index or major comorbidities in different Poisson regression models, 33% of joint pains were attributable to CHIKV, 10% of cerebral disorders and 7.5% of sensorineural impairments, while Chikungunya did not enhance fatigue states, digestive and skin disorders. Conclusions On average, 2 years after infection 43% to 75% of infected people reported prolonged or late-onset symptoms highly attributable to CHIKV. These manifestations carry a significant burden in the community in the fields of rheumatology, neurology and sensorineural health. PMID:21235760

A novel mutation of LHX3 is associated with combined pituitary hormone deficiency including ACTH deficiency, sensorineural hearing loss, and short neck-a case report and review of the literature.

PubMed

Bonfig, Walter; Krude, Heiko; Schmidt, Heinrich

2011-08-01

The LHX3 LIM-homeodomain transcription factor gene is required for normal pituitary and motoneuron development. LHX3 mutations are associated with growth hormone, prolactin, gonadotropin, and TSH deficiency; abnormal pituitary morphology; and may be accompanied with limited neck rotation and sensorineural hearing loss. We report on a boy, who presented with hypoglycemia in the newborn period. He is the second child of healthy unrelated parents. Short neck, growth hormone deficiency, and central hypothyroidism were diagnosed at a general pediatric hospital. Growth hormone and levothyroxine treatment were started, and blood sugar normalized with this treatment. On cerebral MRI, the anterior pituitary gland was hypoplastic. Sensorineural hearing loss was diagnosed by auditory testing. During follow-up, six repeatedly low morning cortisol levels (<1 μg/dl) and low ACTH levels (<10 pg/ml) were documented, so ACTH deficiency had developed over time and therefore hydrocortisone replacement was started at 1.5 years of age. Mutation analysis of the LHX3 gene revealed a homozygous stop mutation in exon 2: c.229C>T (CGA > TGA), Arg77stop (R77X). A complete loss of function is assumed with this homozygous stop mutation. We report a novel LHX3 mutation, which is associated with combined pituitary hormone deficiency including ACTH deficiency, short neck, and sensorineural hearing loss. All patients with LHX3 defects should undergo longitudinal screening for ACTH deficiency, since corticotrope function may decline over time. All patients should have auditory testing to allow for regular speech development.

Initial clinical presentation of young children with N-methyl-d-aspartate receptor encephalitis.

PubMed

Favier, Marion; Joubert, Bastien; Picard, Géraldine; Rogemond, Véronique; Thomas, Laure; Rheims, Sylvain; Bailhache, Marion; Villega, Frédéric; Pédespan, Jean-Michel; Berzero, Giulia; Psimaras, Dimitri; Antoine, Jean-Christophe; Desestret, Virginie; Honnorat, Jérôme

2018-05-01

Autoimmune encephalitis with anti-N-methyl-d-aspartate receptor autoantibodies (NMDA-R-Abs) is a recently described disease affecting adult and pediatric patients. Symptoms of the disease are now perfectly described in the adult population but the clinical presentation is less known in young children. The aim of the present study was to describe the clinical presentation and the specificities of symptoms presented by young children with NMDA-R-Abs encephalitis to improve diagnosis of this disease, and to compare these to a series of previously published female adult patients. Fifty cases of children younger than twelve years of age diagnosed with NMDA-R-Abs encephalitis between January 1, 2007 and December 31, 2016 (27 females and 23 males) were retrospectively studied. The first neurological symptoms observed in young children with NMDA-R-Abs encephalitis were characterized by seizure (72%), especially focal seizure (42%), within a median of 15 days before other encephalitis symptoms; other patients mostly had behavioral disorders (26%). The seizures were frequently difficult to diagnose because of the transient unilateral dystonic or tonic posturing presentation or sudden unilateral pain in the absence of clonic movements. A post-ictal motor deficit was also frequently observed. This clinical presentation is different from that observed in adult females with NMDA-R-Abs encephalitis who initially present mainly psychiatric disorders (67%) or cognitive impairment (19%), and less frequently seizures (14%). The diagnosis of NMDA-R-Abs encephalitis should be systematically considered in young children of both sexes who present neurological symptoms suggesting recent seizures (focal or generalized) without obvious other etiology. Copyright © 2017 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

Imported Case of Lassa Fever in Sweden With Encephalopathy and Sensorineural Hearing Deficit.

PubMed

Grahn, Anna; Bråve, Andreas; Lagging, Martin; Dotevall, Leif; Ekqvist, David; Hammarström, Helena; Karlberg, Helen; Lagerqvist, Nina; Sansone, Martina; Tegnell, Anders; Ulleryd, Peter; Studahl, Marie

2016-10-01

We describe an imported case of Lassa fever with both encephalopathy and bilateral sensorineural hearing deficit. Absence of fever during hospitalization, initially nonspecific symptoms, and onset of hearing deficit in a late stage of disease probably contributed to delayed diagnosis (14 days after admittance to hospital). The pathogenesis of neurological manifestations of Lassa fever is poorly understood and no specific treatment was given. A total of 118 personnel had close contact with the patient, but no secondary cases occurred. This case highlights the importance of considering Lassa fever as a differential diagnosis in patients with recent travel to endemic areas.

Cogan's syndrome mimicking acute Lyme arthritis.

PubMed

Schwegmann, J P; Enzenauer, R J

1995-05-01

A pediatric case of Cogan's syndrome mimicking acute Lyme arthritis is described. A 12-year-old black boy was admitted to the pediatric service for presumed right knee septic arthritis. Symptoms included acute pain and swelling with decreased range-of-motion. Although the patient's right knee symptoms and positive Lyme serology were consistent with a diagnosis of Lyme arthritis, the presence of sensorineural hearing loss and interstitial keratitis with inflammatory arthritis suggested a diagnosis of Cogan's syndrome. Subsequent Western blot analysis was negative for Borrelia burgdorferi antigens. The patient had dramatic clinical improvement of musculoskeletal and ophthalmologic complaints shortly after receiving high-dose corticosteroids, although residual sensorineural hearing loss persisted.

Hearing gain with a BAHA test-band in patients with single-sided deafness.

PubMed

Kim, Do-Youn; Kim, Tae Su; Shim, Byoung Soo; Jin, In Suk; Ahn, Joong Ho; Chung, Jong Woo; Yoon, Tae Hyun; Park, Hong Ju

2014-01-01

It is assumed that preoperative use of a bone-anchored hearing aid (BAHA) test-band will give a patient lower gain compared to real post-operative gain because of the reduction of energy through the scalp when using a test-band. Hearing gains using a BAHA test-band were analyzed in patients with unilateral hearing loss. Nineteen patients with unilateral sensorineural hearing loss were enrolled. A test-band, which was connected to BAHA Intenso with full-on gain, was put on the mastoid. Conventional air-conduction (AC) pure-tone averages (PTAs) and sound-field PTAs and speech reception thresholds (SRTs) were obtained in conditions A (the better ear naked), B (the better ear plugged), and C (the better ear plugged with a test-band on the poorer mastoid). Air-conduction PTAs of the poorer and better ears were 91 ± 19 and 18 ± 8 dB HL. Sound-field PTAs in condition B were higher than those in condition A (54 vs. 26 dB HL), which means that earplugs can block the sound grossly up to 54 dB HL through the better ears. The aided PTAs (24 ± 6 dB HL) in condition C were similar to those of the better ears in condition A (26±9 dB HL), though condition C showed higher thresholds at 500 Hz and lower thresholds at 1 and 2kHz when compared to condition A. The hearing thresholds using a test-band were similar to the published results of BAHA users with the volume to most comfortable level (MCL). Our findings showed that a BAHA test-band on the poorer ear could transmit sound to the cochlea as much as the better ears can hear. The increased functional gain at 1 and 2kHz reflects the technical characteristics of BAHA processor. The reduction of energy through the scalp when using a test-band seems to be offset by the difference of output by setting the volume to full-on gain and using a high-powered speech processor. Preoperative hearing gains using a test-band with full-on gain seems to be similar to the post-operative gains of BAHA users with the volume to MCL. © 2013.

Unique presentation of LHON/MELAS overlap syndrome caused by m.13046T>C in MTND5.

PubMed

Kolarova, Hana; Liskova, Petra; Tesarova, Marketa; Kucerova Vidrova, Vendula; Forgac, Martin; Zamecnik, Josef; Hansikova, Hana; Honzik, Tomas

2016-12-01

Leber hereditary optic neuropathy (LHON) and mitochondrial encephalopathy, myopathy, lactic acidosis and stroke-like episodes (MELAS) syndromes are mitochondrially inherited disorders characterized by acute visual failure and variable multiorgan system presentation, respectively. A 12-year-old girl with otherwise unremarkable medical history presented with abrupt, painless loss of vision. Over the next few months, she developed moderate sensorineural hearing loss, vertigo, migraines, anhedonia and thyroiditis. Ocular examination confirmed bilateral optic nerve atrophy. Metabolic workup documented elevated cerebrospinal fluid lactate. Initial genetic analyses excluded the three most common LHON mutations. Subsequently, Sanger sequencing of the entire mitochondrial DNA (mtDNA) genome was performed. Whole mtDNA sequencing revealed a pathogenic heteroplasmic mutation m.13046T>C in MTND5 encoding the ND5 subunit of complex I. This particular variant has previously been described in a single case report of MELAS/Leigh syndrome (subacute necrotizing encephalopathy). Based on the constellation of clinical symptoms in our patient, we diagnose the condition as LHON/MELAS overlap syndrome. We describe a unique presentation of LHON/MELAS overlap syndrome resulting from a m.13046T>C mutation in a 12-year-old girl. In patients with sudden vision loss in which three of the most prevalent LHON mitochondrial mutations have been ruled out, molecular genetic examination should be extended to other mtDNA-encoded subunits of MTND5 complex I. Furthermore, atypical clinical presentations must be considered, even in well-described phenotypes.

Experimental results on mechanisms of action of electrical neuromodulation in chronic urinary retention.

PubMed

Schultz-Lampel, D; Jiang, C; Lindström, S; Thüroff, J W

1998-01-01

Sacral foramen neuromodulation--initially applied for the treatment of urinary incontinence--has proved to be effective in patients with chronic urinary retention. Thus far, the underlying neurophysiological mechanisms have not been elucidated. In an experimental study on the neurophysiological basis of sacral neurostimulation, one objective was to investigate the mechanisms responsible for initiation of micturition in chronic urinary retention. In ten female cats anesthetized with alpha-chloralose the clinical situation of sacral foramen stimulation was experimentally reproduced by isolated S2 nerve stimulation after L6-S3 laminectomy. Stimulation responses were recorded from the bladder, peripheral nerves, and striated muscles of the foot and pelvic floor. The effect of sudden cessation of prolonged S2 stimulation, during which the bladder was completely inhibited, was evaluated in 70 stimulation sequences in 5 cats. Sacral nerve stimulation induced excitatory and inhibitory effects on the bladder, depending on the frequency and intensity of stimulation. With unilateral S2 stimulation, bladder excitation was best at frequencies of 2-5 Hz and at intensities ranging between 0.8 and 1.4 times the threshold for the M-response of the foot muscle. Inhibition was the dominating effect at frequencies of 7-10 Hz and at intensities exceeding 1.4 times the threshold. Prolonged S2 stimulation above the threshold produced complete bladder inhibition during stimulation but induced strong bladder contractions after sudden interruption of stimulation, with amplitudes being significantly higher than that of spontaneous contractions preceding the stimulation. These results confirm the hypothesis of a "rebound" phenomenon as the mechanism of action for induction of spontaneous voiding in patients with chronic urinary retention.

A case of antenatal Bartter syndrome with sensorineural deafness.

PubMed

Lee, Hyun Seung; Cheong, Hae Il; Ki, Chang-Seok

2010-10-01

Bartter syndrome type IV, also known as Bartter syndrome with sensorineural deafness (BSND), is caused by loss-of-function mutations in the BSND gene, which encodes barttin, an accessory subunit of chloride channels located in the kidney and inner ear. Patients with BS IV have a highly variable clinical phenotype. This report concerns a Korean male patient with antenatal Bartter syndrome due to a homozygous BSND p.G47R mutation, who presented with severe perinatal symptoms followed by a relatively benign course with preserved renal function after early infancy. In addition, the clinical features and the laboratory data of the patient were compared with those of previously reported patients with the same mutation.

Newly recognized recessive syndrome characterized by dysmorphic features, hypogonadotropic hypogonadism, severe microcephaly, and sensorineural hearing loss maps to 3p21.3.

PubMed

Jenkinson, Emma M; Kingston, Helen; Urquhart, Jill; Khan, Naz; Melville, Athalie; Swinton, Martin; Crow, Yanick J; Davis, Julian R E; Trump, Dorothy; Newman, William G

2011-12-01

We present a newly recognized, likely autosomal recessive, pleiotropic disorder seen in four individuals (three siblings and their nephew) from a consanguineous family of Pakistani origin. The condition is characterized by hypogonadotropic hypogonadism, severe microcephaly, sensorineural deafness, moderate learning disability, and distinctive facial dysmorphic features. Autozygosity mapping using SNP array genotyping defined a single, large autozygous region of 13.1 Mb on chromosome 3p21 common to the affected individuals. The critical region contains 227 genes and initial sequence analysis of a functional candidate gene has not identified causative mutations. Copyright © 2011 Wiley Periodicals, Inc.

[The experimental research of inner ear metabolism and electrical physiology of autoimmune sensorineural hearing loss].

PubMed

Tan, C; Cao, Y; Hu, P

1998-09-01

Inquire into the mechanism of inner ear pathological physiology in autoimmune sensorineural hearing loss (ASHL). With the auditory electric-physiological techniques and enzyme-histochemical method, the change of inner ear hearing function and enzyme activity were observed. These animals, which threshold of auditory nerve compound active potential (CAP) and cochlear microphonic potential(CM) heightening evidently, showed that the amplitude of endolymphatic potential(EP) (include-EP) bring down in various degrees, which was related to the change of the active of Na(+)-K(+)-ATPase and SDH in vascularis stria and endolymphatic sac. The abnormality of enzymes metabolism in inner ear tissues, which following autoimmune inflammation damage, is the pathological foundation of hearing dysfunction.

Risk factors of sensorineural hearing loss in preterm infants.

PubMed

Borradori, C; Fawer, C L; Buclin, T; Calame, A

1997-01-01

Among 547 preterm infants of < or = 34 weeks gestation born between 1987 and 1991, 8 children (1.46%) developed severe progressive and bilateral sensorineural hearing loss. Perinatal risk factors of infants with hearing loss were compared with those of two control groups matched for gestation and birth weight and for perinatal complications. Our observations demonstrated an association of hearing loss with a higher incidence of perinatal complications. Ototoxicity appeared closely related to a prolonged administration and higher total dose of ototoxic drugs, particularly aminoglycosides and furosemide. Finally, we strongly recommend to prospectively and regularly perform audiologic assessment in sick preterm children as hearing loss is of delayed onset and in most cases bilateral and severe.

Predictors of round window accessibility for adult cochlear implantation based on pre-operative CT scan: a prospective observational study.

PubMed

Park, Edward; Amoodi, Hosam; Kuthubutheen, Jafri; Chen, Joseph M; Nedzelski, Julian M; Lin, Vincent Y W

2015-05-28

Cochlear implantation has become a mainstream treatment option for patients with severe to profound sensorineural hearing loss. During cochlear implant, there are key surgical steps which are influenced by anatomical variations between each patient. The aim of this study is to determine if there are potential predictors of difficulties that may be encountered during the cortical mastoidectomy, facial recess approach and round window access in cochlear implant surgery based upon pre-operative temporal bone CT scan. Fifty seven patients undergoing unilateral cochlear implantation were analyzed. Difficulty with 1) cortical mastoidectomy, 2) facial recess approach, and 3) round window access were scored intra-operatively by the surgeon in a blinded fashion (1 = "easy", 2 = "moderate", 3 = "difficult"). Pre-operative temporal bone CT scans were analyzed for 1) degree of mastoid aeration; 2) location of the sigmoid sinus; 3) height of the tegmen; 4) the presence of air cells in the facial recess, and 5) degree of round window bony overhang. Poor mastoid aeration and lower tegmen position, but not the location of sigmoid sinus, are associated with greater difficulty with the cortical mastoidectomy. Presence of an air cell around the facial nerve was predictive of easier facial recess access. However, the degree of round window bony overhang was not predictive of difficulty associated with round window access. Certain parameters on the pre-operative temporal bone CT scan may be useful in predicting potential difficulties encountered during the key steps involved in cochlear implant surgery.

Vestibular system paresis due to emergency endovascular catheterization.

PubMed

Simoceli, Lucinda; Sguillar, Danilo Anunciatto; Santos, Henrique Mendes Paiva; Caputti, Camilla

2012-04-01

 The objective of this story of case is to describe an uncommon cause of associated peripheral Vestibulopathy to the unilateral auditory loss in aged patient after catheterization of urgency. Story of case: Patient of the masculine sort, 82 years, submitted to the correction of abdominal ragged aneurism of aorta, in the intra-operative suffered heart attack acute from the myocardium needing primary angioplasty. High after hospital it relates to complaint of accented hearing loss to the right and crippling vertigo, without focal neurological signals. To the otorhinolaryngological clinical examination it presented: Test of Weber lateralized for the left, spontaneous nystagmus for the left, marches rocking, has taken normal disbasia and ataxia, index-nose and diadochokinesia, Test of Romberg with oscillation without fall and Fukuda with lateral shunting line for the right. The audiometric examination evidenced deafness to the right and sensorineural loss to the left in sharps, areflexia initial to the right in caloric test e, the computerized tomography of the secular bones and brainstem, presence of metallic connecting rod crossing the right secular bone, from the vein internal jugular vein and bulb jugular vein, crossing the posterior, superior and vestibule semicircular canals, projecting itself in temporal lobe. The radiological diagnoses was traumatic injury for guide to endovascular metallic during catheterization of urgency and the behavior, considering that the patient had not compensated the balance, it was vestibular rehabilitation.  Complaints of giddiness in the aged patient must be closely evaluated of its pathological clinical description because the antecedents of illnesses and previous treatments, in general, direct the diagnostic hypotheses however they can bring unexpected alterations.

Sensorineural hearing loss--a common finding in early-onset type 2 diabetes mellitus.

PubMed

Lerman-Garber, Israel; Cuevas-Ramos, Daniel; Valdés, Samantha; Enríquez, Lorena; Lobato, Marlette; Osornio, Melannie; Escobedo, Ana Rosa; Pascual-Ramos, Virginia; Mehta, Roopa; Ramírez-Anguiano, Jacqueline; Gómez-Pérez, Francisco J

2012-01-01

To evaluate the prevalence and potential associations of hearing impairment in patients 30 to 50 years old with diabetes diagnosed before age 40 years-early-onset type 2 diabetes mellitus (T2DM). The study cohorts consisted of 46 consecutive patients with early-onset T2DM and 47 age-matched control subjects with rheumatoid arthritis. All study subjects completed clinical, serologic, and auditory assessments. The patients with T2DM had a mean age of 42 ± 6 years and a mean disease duration of 11 ± 6 years. Microalbuminuria was present in 26.1%, proliferative retinopathy in 26.1%, and symptomatic peripheral neuropathy in 23.9%. The prevalence of unilateral or bilateral hearing loss was significantly higher in the patients with T2DM than in the patients with rheumatoid arthritis (21.7% versus 6.4%, respectively; P = .01). Most cases of hearing loss were mild and involved high or acute tones. After multivariate analysis with adjustment for age, there was a significant association between hearing loss and hemoglobin A1c (odds ratio, 1.3; 95% confidence interval, 1.02 to 1.81; P = .035). In the patients with T2DM, the lengthening of the brainstem response was not significantly increased; however, the wave morphologic features were abnormal and the reproducibility was poor in both ears in 11 patients (24%). Patients with early-onset T2DM and poor glycemic control have an increased prevalence of subclinical hearing loss and impaired auditory brainstem responses. Hearing impairment may be an underrecognized complication of diabetes.

Refractory episodic vertigo: role of intratympanic gentamicin and vestibular evoked myogenic potentials.

PubMed

Celis-Aguilar, Erika; Hinojosa-González, Ramon; Vales-Hidalgo, Olivia; Coutinho-Toledo, Heloisa

Even today, the treatment of intractable vertigo remains a challenge. Vestibular ablation with intratympanic gentamicin stands as a good alternative in the management of refractory vertigo patients. To control intractable vertigo through complete saccular and horizontal canal vestibular ablation with intratympanic gentamicin treatment. Patients with refractory episodic vertigo were included. The inclusion criteria were: unilateral ear disease, moderate to profound sensorineural hearing loss, and failure to other treatments. Included patients underwent 0.5-0.8mL of gentamicin intratympanic application at a 30mg/mL concentration. Vestibular ablation was confirmed by the absence of response on cervical vestibular evoked myogenic potentials and no response on caloric tests. Audiometry, electronystagmography with iced water, and vestibular evoked myogenic potentials were performed in all patients. Ten patients were included; nine patients with Meniere's disease and one patient with (late onset) delayed hydrops. Nine patients showed an absent response on vestibular evoked myogenic potentials and no response on caloric tests. The only patient with low amplitude on cervical vestibular evoked myogenic potentials had vertigo recurrence. Vertigo control was achieved in 90% of the patients. One patient developed hearing loss >30dB. Cervical vestibular evoked myogenic potentials confirmed vestibular ablation in patients treated with intratympanic gentamicin. High-grade vertigo control was due to complete saccular and horizontal canal ablation (no response to iced water in electronystagmography and no response on cervical vestibular evoked myogenic potentials). Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

A rare case of renal infarction caused by infective endocarditis.

PubMed

Zakaria, Rasheed; Forsyth, Vhari; Rosenbaum, Tomas

2009-10-01

A 29-year-old man presented to the emergency department of a general hospital complaining of sudden onset left loin pain, radiating to the groin, which had started 48 h previously. He described no urological symptoms and had no medical history of note. Physical examination, electrocardiography, dipstick testing of urine, radiography of the chest and abdomen, blood tests (white blood cell count and serum urea, creatinine, sodium, potassium and C-reactive protein levels), CT of the renal tract, blood and urine cultures, renal angiography, thromboembolic blood panel, urine and blood tests for illicit drugs, transthoracic echocardiography, transesophageal echocardiography, renal ultrasonography. Infective endocarditis resulting in thromboembolic unilateral renal infarction. The patient was started on anticoagulation therapy with low-molecular-weight heparin and treated with intravenous gentamicin and benzylpenicillin for 4 weeks. He was seen in an outpatient clinic 4 weeks after discharge, at which time serum urea and creatinine levels and repeat ultrasonography of the renal tract confirmed normal renal function. He will be followed up regularly by cardiologists and urologists, at 6 weeks initially, and every 6 months to 1 year thereafter by his family physician.

When the bell tolls on Bell's palsy: finding occult malignancy in acute-onset facial paralysis.

PubMed

Quesnel, Alicia M; Lindsay, Robin W; Hadlock, Tessa A

2010-01-01

This study reports 4 cases of occult parotid malignancy presenting with sudden-onset facial paralysis to demonstrate that failure to regain tone 6 months after onset distinguishes these patients from Bell's palsy patients with delayed recovery and to propose a diagnostic algorithm for this subset of patients. A case series of 4 patients with occult parotid malignancies presenting with acute-onset unilateral facial paralysis is reported. Initial imaging on all 4 patients did not demonstrate a parotid mass. Diagnostic delays ranged from 7 to 36 months from time of onset of facial paralysis to time of diagnosis of parotid malignancy. Additional physical examination findings, especially failure to regain tone, as well as properly protocolled radiologic studies reviewed with dedicated head and neck radiologists, were helpful in arriving at the diagnosis. An algorithm to minimize diagnostic delays in this subset of acute facial paralysis patients is presented. Careful attention to facial tone, in addition to movement, is important in the diagnostic evaluation of acute-onset facial paralysis. Copyright 2010 Elsevier Inc. All rights reserved.

Prosthodontic Rehabilitation of Patients with Bell's Palsy: Our Experience.

PubMed

Rajapur, Anand; Mitra, Nirban; Prakash, V Jeevan; Rah, Sajad Ahmad; Thumar, Sagar

2015-01-01

Bell's palsy is an idiopathic unilateral lower motor neuron paresis or paralysis of the facial nerve of sudden onset. It involves loss of muscular control on the affected side of the face. This paper reports the prosthodontic management of patients with Bell's palsy and also describes a technique to stabilize the jaw movements in complete denture patients using interim dentures. A 65-year-old male edentulous patient and a 55-year-old female edentulous patient reported to the department of prosthodontics to get their missing teeth replaced. They both gave history of facial paralysis and were diagnosed for Bell's palsy. Interim training dentures with flat occlusal tables were fabricated first to correct and stabilize their mandibular movements. During initial 4 weeks, there was poor functioning of the interim dentures. Gradually by 8(th) week the patients started stabilizing the interim dentures and were functional. After observing the improvement when the patients had no pain and could stabilize and use the treatment dentures successfully, definitive complete dentures were fabricated. This case report presents a systematic approach to successively rehabilitate edentulous patients with Bell's palsy.

Optic neuritis in pediatric population: a review in current tendencies of diagnosis and management.

PubMed

Pérez-Cambrodí, Rafael José; Gómez-Hurtado Cubillana, Aránzazu; Merino-Suárez, María L; Piñero-Llorens, David P; Laria-Ochaita, Carlos

2014-01-01

Optic neuritis is an inflammation of the optic nerve and may be related to different systemic conditions. The clinical presentation of this pathology usually includes sudden loss of visual acuity (VA) which may be unilateral or bilateral, visual field restriction, pain with eye movements, dyschromatopsia, a relative afferent pupillary defect and optic disk swelling. Optic neuritis in children has specific clinical features and a better prognosis than in adulthood. Although usually appears an underlying viral disease, the main concern for practitioners is the relationship of optic neuritis with multiple sclerosis. In addition to the classical techniques as magnetic resonance imaging (MRI), current tendencies of diagnosis for eye practitioners include new imaging devices as optical coherence tomography (OCT), useful to show a thinning of the retinal fibers layer (RFL) after the inflammatory episode. Regarding the management of these patients, short-term intravenous steroid dosages seem to be the best option to treat acute attacks characterized by a very poor bilateral VA. Copyright © 2013 Spanish General Council of Optometry. Published by Elsevier Espana. All rights reserved.

Bilateral orbital emphysema and pneumocephalus as a result of accidental compressed air exposure.

PubMed

Yuksel, Murvet; Yuksel, K Zafer; Ozdemir, Gokhan; Ugur, Tuncay

2007-01-01

Orbital emphysema is a rare condition in the absence of trauma or sinus disease. A 22-year-old man suffering from left orbital trauma due to sudden exposure to compressed air tube was admitted with severe pain in the left eye, swelling, and mild periorbital ecchymosis. Physical examination revealed a large conjunctival laceration in the left orbit. Multislice computed tomographic scanning of the head and orbits showed extensive radiolucencies consistent with the air in both orbits, more prominent in the left. There was also subcutaneous air in the left periorbital soft tissue extending through fronto-temporal and zygomatic areas. Air was also demonstrated adjacent to the left optic canal and within the subarachnoid space intracranially. There was no evidence of any orbital, paranasal sinus, or cranial fracture. Visual acuity was minimally decreased bilaterally. The conjunctiva was sutured under local anesthesia. After 3 weeks of follow-up, the patient completely recovered without visual loss. Bilateral orbital emphysema with pneumocephalus can occur from a high-pressure compressed air injury after unilateral conjunctival trauma without any evidence of fracture.

Prosthodontic Rehabilitation of Patients with Bell’s Palsy: Our Experience

PubMed Central

Rajapur, Anand; Mitra, Nirban; Prakash, V Jeevan; Rah, Sajad Ahmad; Thumar, Sagar

2015-01-01

Bell’s palsy is an idiopathic unilateral lower motor neuron paresis or paralysis of the facial nerve of sudden onset. It involves loss of muscular control on the affected side of the face. This paper reports the prosthodontic management of patients with Bell’s palsy and also describes a technique to stabilize the jaw movements in complete denture patients using interim dentures. A 65-year-old male edentulous patient and a 55-year-old female edentulous patient reported to the department of prosthodontics to get their missing teeth replaced. They both gave history of facial paralysis and were diagnosed for Bell’s palsy. Interim training dentures with flat occlusal tables were fabricated first to correct and stabilize their mandibular movements. During initial 4 weeks, there was poor functioning of the interim dentures. Gradually by 8th week the patients started stabilizing the interim dentures and were functional. After observing the improvement when the patients had no pain and could stabilize and use the treatment dentures successfully, definitive complete dentures were fabricated. This case report presents a systematic approach to successively rehabilitate edentulous patients with Bell’s palsy. PMID:26668488

A new type of headache, headache associated with airplane travel: preliminary diagnostic criteria and possible mechanisms of aetiopathogenesis.

PubMed

Berilgen, M Said; Müngen, Bulent

2011-09-01

In recent years, there has been an increase in the reports indicating a form of headache that occurs during commercial aircraft travel. This headache, called airplane headache by some authors, is believed to be a new type of headache. The headache has very specific characteristics and all of the cases exhibited very stereotypical symptoms. The headache starts suddenly during the ascent and/or descent of the commercial aircraft. It has a mean duration of 20 minutes, which is usually unilateral and commonly localized to periorbital region. The headache is described to be severe, and has a stabbing or jabbing nature, and generally subsides in a short time. In some cases, an organic cause can be identified whereas in others no organic pathology could be found. We described the clinical features of 22 cases who suffered from a headache that occurred during airplane travel. We examined other cases with similar features reported in the literature and proposed preliminary diagnostic criteria for this new form of headache. We also discussed the possible patholophysiological mechanisms that may cause this headache.

[Chronic pain and syringomyelic slit of the posterior horns of the spinal cord].

PubMed

Rémillard, G M; Robitaille, Y; Bertrand, G

1985-01-01

Two male patients 46 and 44 y.o. respectively, were admitted for a syndrome of chronic pain characterized by: sudden onset, spontaneous or following spasmodic coughing, of an anterior hemithoracic pain slowly progressing to involve several unilateral cervicothoracic dermatomes, a continuous burning sensation made worse by light touch, limb movements and cold water, and partially relieved by warm water or deep palpation. On examination, patient 1 revealed no sensorimotor deficit after repeated observations during 8 years. At autopsy, a syrinx localized at the cord segments corresponding to the symptoms was found without documentation of specific causal factors. It involved the posterior horn of the cord selectively. In patient 2, pain was associated with slight hypesthesia to pinprick and heat from C2 to T5 on the left without motor deficit since 18 months. A high resolution C.A.T. scan showed an intramedullary cavity 0.3 cm from the midline in the projection of the posterior horn without anomalies at the cervicomedullary junction. These observations link chronic pain syndromes with predominantly posterior horn lesions, which so far have failed to respond to conventional therapeutic measures.

Cochlear implantation in patients with bilateral cochlear trauma.

PubMed

Serin, Gediz Murat; Derinsu, Ufuk; Sari, Murat; Gergin, Ozgül; Ciprut, Ayça; Akdaş, Ferda; Batman, Cağlar

2010-01-01

Temporal bone fracture, which involves the otic capsule, can lead to complete loss of auditory and vestibular functions, whereas the patients without fractures may experience profound sensorineural hearing loss due to cochlear concussion. Cochlear implant is indicated in profound sensorineural hearing loss due to cochlear trauma but who still have an intact auditory nerve. This is a retrospective review study. We report 5 cases of postlingually deafened patients caused by cochlear trauma, who underwent cochlear implantation. Preoperative and postoperative hearing performance will be presented. These patients are cochlear implanted after the cochlear trauma in our department between 2001 and 2006. All patients performed very well with their implants, obtained open-set speech understanding. They all became good telephone users after implantation. Their performance in speech understanding was comparable to standard postlingual adult patients implanted. Cochlear implantation is an effective aural rehabilitation in profound sensorineural hearing loss caused by temporal bone trauma. Preoperative temporal bone computed tomography, magnetic resonance imaging, and promontorium stimulation testing are necessary to make decision for the surgery and to determine the side to be implanted. Surgery could be challenging and complicated because of anatomical irregularity. Moreover, fibrosis and partial or total ossification within the cochlea must be expected. Copyright 2010. Published by Elsevier Inc.

Perspectives for the treatment of sensorineural hearing loss by cellular regeneration of the inner ear.

PubMed

Almeida-Branco, Mario S; Cabrera, Sonia; Lopez-Escamez, Jose A

2015-01-01

Sensorineural hearing loss is a caused by the loss of the cochlear hair cells with the consequent deafferentation of spiral ganglion neurons. Humans do not show endogenous cellular regeneration in the inner ear and there is no exogenous therapy that allows the replacement of the damaged hair cells. Currently, treatment is based on the use of hearing aids and cochlear implants that present different outcomes, some difficulties in auditory discrimination and a limited useful life. More advanced technology is hindered by the functional capacity of the remaining spiral ganglion neurons. The latest advances with stem cell therapy and cellular reprogramming have developed several possibilities to induce endogenous regeneration or stem cell transplantation to replace damaged inner ear hair cells and restore hearing function. With further knowledge of the cellular and molecular biology of the inner ear and its embryonic development, it will be possible to use induced stem cells as in vitro models of disease and as replacement cellular therapy. Investigation in this area is focused on generating cellular therapy with clinical use for the treatment of profound sensorineural hearing loss. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

Extended high frequency audiometry can diagnose sub-clinic involvement in a seemingly normal hearing systemic lupus erythematosus population.

PubMed

Lasso de la Vega, Mar; Villarreal, Ithzel María; López Moya, Julio; García-Berrocal, José Ramón

2017-02-01

Sensorineural hearing loss must be considered within the clinical picture of systemic lupus erythematosus. The results confirm the usefulness of extended high-frequency audiometry in the audiologic testing of these patients, enabling the possibility of modifying or applying a preventive treatment for a possible hearing loss. Hearing involvement is usually under-diagnosed with routine auditory examination. This study proposes the use of extended high-frequency audiometry to achieve a correct detection of a possible asymptomatic hypoacusis in early stages of the disease. The aim of this study is to analyze the hearing levels in extended high-frequencies in these patients and to correlate the hearing loss with the severity of the disease and the immunological parameters. A descriptive cross-sectional study was performed. Fifty-five patients with systemic lupus erythematosus were included in the study. The control group consisted of 71 patients paired by age and sex with the study population. Both a pure tone audiometry and an extended high-frequency audiometry (8-18 KHz) were performed. In total, 70% were diagnosed with sensorineural hearing loss with extended high-frequency audiometry, overcoming the results obtained with pure tone audiometry (30.9%). Statistically significant correlations were found within the patients regarding sensorineural hearing loss related with age, disease activity and cryoglobulinemia.

Inner ear changes in mucopolysaccharidosis type I/Hurler syndrome.

PubMed

Kariya, Shin; Schachern, Patricia A; Nishizaki, Kazunori; Paparella, Michael M; Cureoglu, Sebahattin

2012-10-01

Mucopolysaccharidosis type I/Hurler syndrome is an autosomal recessive disease caused by a deficiency of α-L-iduronidase activity. Recurrent middle ear infections and hearing loss are common complications in Hurler syndrome. Although sensorineural and conductive components occur, the mechanism of sensorineural hearing loss has not been determined. The purpose of this study is to evaluate the quantitative inner ear histopathology of the temporal bones of patients with Hurler syndrome. Eleven temporal bones from 6 patients with Hurler syndrome were examined. Age-matched healthy control samples consisted of 14 temporal bones from 7 cases. Temporal bones were serially sectioned in the horizontal plane and stained with hematoxylin and eosin. The number of spiral ganglion cells, loss of cochlear hair cells, area of stria vascularis, and cell density of spiral ligament were evaluated using light microscopy. There was no significant difference between Hurler syndrome and healthy controls in the number of spiral ganglion cells, area of stria vascularis, or cell density of spiral ligament. The number of cochlear hair cells in Hurler syndrome was significantly decreased compared with healthy controls. Auditory pathophysiology in the central nerve system in Hurler syndrome remains unknown; however, decreased cochlear hair cells may be one of the important factors for the sensorineural component of hearing loss.

Audiological follow-up of 24 patients affected by Williams syndrome.

PubMed

Barozzi, Stefania; Soi, Daniela; Spreafico, Emanuela; Borghi, Anna; Comiotto, Elisabetta; Gagliardi, Chiara; Selicorni, Angelo; Forti, Stella; Cesarani, Antonio; Brambilla, Daniele

2013-09-01

Williams syndrome is a neurodevelopmental disorder associated with cardiovascular problems, facial abnormalities and several behavioural and neurological disabilities. It is also characterized by some typical audiological features including abnormal sensitivity to sounds, cochlear impairment related to the outer hair cells of the basal turn of the cochlea, and sensorineural or mixed hearing loss, predominantly in the high frequency range. The aim of this report is to describe a follow-up study of auditory function in a cohort of children affected by this syndrome. 24 patients, aged 5-14 years, were tested by means of air/bone conduction pure-tone audiometry, immittance test and transient evoked otoacoustic emissions. They were evaluated again 5 years after the first assessment, and 10 of them underwent a second follow-up examination after a further 5 years. The audiometric results showed hearing loss, defined by a pure tone average >15 dB HL, in 12.5% of the participants. The incidence of hearing loss did not change over the 5-year period and increased to 30% in the patients who underwent the 10-year follow-up. Progressive sensorineural hearing loss was detected in 20% of the patients. A remarkable finding of our study regarded sensorineural hearing impairment in the high frequency range, which increased significantly from 25% to 50% of the participants over the 5-year period. The increase became even more significant in the group of patients who underwent the 10-year follow-up, by which time the majority of them (80%) had developed sensorineural hearing loss. Otoacoustic emissions were found to be absent in a high percentage of patients, thus confirming the cochlear fragility of individuals with Williams syndrome. Our study verified that most of the young Williams syndrome patients had normal hearing sensitivity within the low-middle frequency range, but showed a weakness regarding the high frequencies, the threshold of which worsened significantly over time in most patients. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

Radiation Therapy and Hearing Loss

DOE Office of Scientific and Technical Information (OSTI.GOV)

Bhandare, Niranjan; Jackson, Andrew; Eisbruch, Avraham

2010-03-01

A review of literature on the development of sensorineural hearing loss after high-dose radiation therapy for head-and-neck tumors and stereotactic radiosurgery or fractionated stereotactic radiotherapy for the treatment of vestibular schwannoma is presented. Because of the small volume of the cochlea a dose-volume analysis is not feasible. Instead, the current literature on the effect of the mean dose received by the cochlea and other treatment- and patient-related factors on outcome are evaluated. Based on the data, a specific threshold dose to cochlea for sensorineural hearing loss cannot be determined; therefore, dose-prescription limits are suggested. A standard for evaluating radiation therapy-associatedmore » ototoxicity as well as a detailed approach for scoring toxicity is presented.« less

Muscle activity of the core during bilateral, unilateral, seated and standing resistance exercise.

PubMed

Saeterbakken, Atle Hole; Fimland, Marius Steiro

2012-05-01

Little is known about the effect of performing common resistance exercises standing compared to seated and unilaterally compared to bilaterally on muscle activation of the core. Thus, the purpose of this study was to compare the electromyographic activity (EMG) of the superficial core muscles (i.e. rectus abdominis, external oblique and erector spinae) between seated, standing, bilateral and unilateral dumbbell shoulder presses. 15 healthy males performed five repetitions at 80% of one-repetition maximum of the exercises in randomized order. Results were analyzed with a two-way analysis of variance and a Bonferroni post hoc test. The position × exercise interaction was significantly different for rectus abdominis (P = 0.016), but not for external oblique (P = 0.100) and erector spinae (P = 0.151). The following EMG results were observed: For rectus abdominis: ~49% lower in seated bilateral versus unilateral (P < 0.001), similar in standing bilateral versus unilateral (P = 0.408), ~81% lower in bilateral seated versus standing (P < 0.001), ~59% lower in unilateral seated versus standing (P < 0.001); For external oblique: ~81% lower in seated bilateral versus unilateral (P < 0.001), ~68% lower in standing bilateral than unilateral (P < 0.001), ~58% lower in bilateral seated versus standing (P < 0.001), ~28% lower in unilateral seated versus standing (P = 0.002); For erector spinae: similar in seated bilateral versus unilateral (P = 0.737), ~18% lower in standing bilateral versus unilateral (P = 0.001), similar in seated versus standing bilateral (P = 0.480) and unilateral (P = 0.690). In conclusion, to enhance neuromuscular activation of the superficial core muscles, standing exercises should be used instead of seated exercises, and unilateral exercises should be used instead of bilateral exercises.

A Chinese patient with pusher syndrome and unilateral spatial neglect syndrome.

PubMed

Chen, Xiao-Wei; Lin, Cheng-He; Zheng, Hua; Lin, Zhen-Lan

2014-07-01

To observe clinical manifestations, behavioral characteristics, and effects of rehabilitation on a patient with pusher syndrome and unilateral spatial neglect caused by right thalamic hemorrhage. Assessment of pusher syndrome was made by the Scale for Contraversive pushing (SCP), and unilateral spatial neglect syndrome was diagnosed using line cancellation, letter and star cancellation, line bisection tests and copy and continuation of graphic sequence test. Behavioral therapy, occupational therapy, reading training and traditional Chinese medicine methods were adopted for treatment of pusher syndrome and unilateral spatial neglect. The patient showed typical pusher syndrome and unilateral spatial neglect symptoms. The pusher syndrome and unilateral spatial neglect symptoms were significantly improved following rehabilitation treatments. Pusher syndrome and unilateral spatial neglect syndrome occurred simultaneously after right thalamic hemorrhage. Early rehabilitation therapy can reduce the symptoms of pusher syndrome and unilateral spatial neglect syndrome and improve motor function.

A PEX6-defective peroxisomal biogenesis disorder with severe phenotype in an infant, versus mild phenotype resembling Usher syndrome in the affected parents.

PubMed

Raas-Rothschild, Annick; Wanders, Ronald J A; Mooijer, Petra A W; Gootjes, Jeannette; Waterham, Hans R; Gutman, Alisa; Suzuki, Yasuyuki; Shimozawa, Nobuyuki; Kondo, Naomi; Eshel, Gideon; Espeel, Marc; Roels, Frank; Korman, Stanley H

2002-04-01

Sensorineural deafness and retinitis pigmentosa (RP) are the hallmarks of Usher syndrome (USH) but are also prominent features in peroxisomal biogenesis defects (PBDs); both are autosomal recessively inherited. The firstborn son of unrelated parents, who both had sensorineural deafness and RP diagnosed as USH, presented with sensorineural deafness, RP, dysmorphism, developmental delay, hepatomegaly, and hypsarrhythmia and died at age 17 mo. The infant was shown to have a PBD, on the basis of elevated plasma levels of very-long- and branched-chain fatty acids (VLCFAs and BCFAs), deficiency of multiple peroxisomal functions in fibroblasts, and complete absence of peroxisomes in fibroblasts and liver. Surprisingly, both parents had elevated plasma levels of VLCFAs and BCFAs. Fibroblast studies confirmed that both parents had a PBD. The parents' milder phenotypes correlated with relatively mild peroxisomal biochemical dysfunction and with catalase immunofluorescence microscopy demonstrating mosaicism and temperature sensitivity in fibroblasts. The infant and both of his parents belonged to complementation group C. PEX6 gene sequencing revealed mutations on both alleles, in the infant and in his parents. This unique family is the first report of a PBD with which the parents are themselves affected individuals rather than asymptomatic carriers. Because of considerable overlap between USH and milder PBD phenotypes, individuals suspected to have USH should be screened for peroxisomal dysfunction.

Speech recognition in individuals with sensorineural hearing loss.

PubMed

de Andrade, Adriana Neves; Iorio, Maria Cecilia Martinelli; Gil, Daniela

2016-01-01

Hearing loss can negatively influence the communication performance of individuals, who should be evaluated with suitable material and in situations of listening close to those found in everyday life. To analyze and compare the performance of patients with mild-to-moderate sensorineural hearing loss in speech recognition tests carried out in silence and with noise, according to the variables ear (right and left) and type of stimulus presentation. The study included 19 right-handed individuals with mild-to-moderate symmetrical bilateral sensorineural hearing loss, submitted to the speech recognition test with words in different modalities and speech test with white noise and pictures. There was no significant difference between right and left ears in any of the tests. The mean number of correct responses in the speech recognition test with pictures, live voice, and recorded monosyllables was 97.1%, 85.9%, and 76.1%, respectively, whereas after the introduction of noise, the performance decreased to 72.6% accuracy. The best performances in the Speech Recognition Percentage Index were obtained using monosyllabic stimuli, represented by pictures presented in silence, with no significant differences between the right and left ears. After the introduction of competitive noise, there was a decrease in individuals' performance. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Altered Brain Functional Activity in Infants with Congenital Bilateral Severe Sensorineural Hearing Loss: A Resting-State Functional MRI Study under Sedation.

PubMed

Xia, Shuang; Song, TianBin; Che, Jing; Li, Qiang; Chai, Chao; Zheng, Meizhu; Shen, Wen

2017-01-01

Early hearing deprivation could affect the development of auditory, language, and vision ability. Insufficient or no stimulation of the auditory cortex during the sensitive periods of plasticity could affect the function of hearing, language, and vision development. Twenty-three infants with congenital severe sensorineural hearing loss (CSSHL) and 17 age and sex matched normal hearing subjects were recruited. The amplitude of low frequency fluctuations (ALFF) and regional homogeneity (ReHo) of the auditory, language, and vision related brain areas were compared between deaf infants and normal subjects. Compared with normal hearing subjects, decreased ALFF and ReHo were observed in auditory and language-related cortex. Increased ALFF and ReHo were observed in vision related cortex, which suggest that hearing and language function were impaired and vision function was enhanced due to the loss of hearing. ALFF of left Brodmann area 45 (BA45) was negatively correlated with deaf duration in infants with CSSHL. ALFF of right BA39 was positively correlated with deaf duration in infants with CSSHL. In conclusion, ALFF and ReHo can reflect the abnormal brain function in language, auditory, and visual information processing in infants with CSSHL. This demonstrates that the development of auditory, language, and vision processing function has been affected by congenital severe sensorineural hearing loss before 4 years of age.

Association between unilateral or bilateral mastectomy and breast cancer death in patients with unilateral ductal carcinoma.

PubMed

Agarwal, Shailesh; Pappas, Lisa; Agarwal, Jayant

2017-01-01

Utilization of bilateral mastectomy for unilateral breast cancer is increasing despite cost and surgical risks with conflicting reports of survival benefit. Current studies evaluating death after bilateral mastectomy have included patients treated both with breast conservation therapy and unilateral mastectomy. In this study, we directly compared breast cancer-specific death of patients who underwent bilateral or unilateral mastectomy for unilateral breast cancer using a matched cohort analysis. This was an observational study of women diagnosed with unilateral breast cancer from 1998 through 2002, using the Surveillance, Epidemiology, and End Results (SEER) database. A 4-to-1 matched cohort of patients was selected including 14,075 patients. Mortality of the groups was compared using Cox proportional hazards models for cause-specific death. A total of 41,510 patients diagnosed with unilateral breast cancer were included. Unilateral mastectomy was performed in 93% of patients, while bilateral mastectomy was performed in the remaining 7% of patients. When 4-to-1 matching was performed, 11,260 unilateral mastectomy and 2,815 bilateral mastectomy patients were included. Patients with bilateral mastectomy did not have a significantly lower hazard of breast cancer-specific death when compared with patients with unilateral mastectomy (hazard ratio: 0.92 vs 1.00, p =0.11). Bilateral mastectomy did not provide a clinically or statistically significant breast cancer-specific mortality benefit over unilateral mastectomy based on a matched cohort analysis of a nationwide population database. These findings should be interpreted in the context of patient preference and alternative benefits of bilateral mastectomy.

Association between unilateral or bilateral mastectomy and breast cancer death in patients with unilateral ductal carcinoma

PubMed Central

Agarwal, Shailesh; Pappas, Lisa; Agarwal, Jayant

2017-01-01

Background Utilization of bilateral mastectomy for unilateral breast cancer is increasing despite cost and surgical risks with conflicting reports of survival benefit. Current studies evaluating death after bilateral mastectomy have included patients treated both with breast conservation therapy and unilateral mastectomy. In this study, we directly compared breast cancer–specific death of patients who underwent bilateral or unilateral mastectomy for unilateral breast cancer using a matched cohort analysis. Methods This was an observational study of women diagnosed with unilateral breast cancer from 1998 through 2002, using the Surveillance, Epidemiology, and End Results (SEER) database. A 4-to-1 matched cohort of patients was selected including 14,075 patients. Mortality of the groups was compared using Cox proportional hazards models for cause-specific death. Results A total of 41,510 patients diagnosed with unilateral breast cancer were included. Unilateral mastectomy was performed in 93% of patients, while bilateral mastectomy was performed in the remaining 7% of patients. When 4-to-1 matching was performed, 11,260 unilateral mastectomy and 2,815 bilateral mastectomy patients were included. Patients with bilateral mastectomy did not have a significantly lower hazard of breast cancer–specific death when compared with patients with unilateral mastectomy (hazard ratio: 0.92 vs 1.00, p=0.11). Conclusion Bilateral mastectomy did not provide a clinically or statistically significant breast cancer–specific mortality benefit over unilateral mastectomy based on a matched cohort analysis of a nationwide population database. These findings should be interpreted in the context of patient preference and alternative benefits of bilateral mastectomy. PMID:29180900

Toxicology study for magnetic injection of prednisolone into the rat cochlea.

PubMed

Shimoji, M; Ramaswamy, B; Shukoor, M I; Benhal, P; Broda, A; Kulkarni, S; Malik, P; McCaffrey, B; Lafond, J-F; Nacev, A; Weinberg, I N; Shapiro, B; Depireux, D A

2018-06-19

This paper investigates the safety of a novel 'magnetic injection' method of delivering therapy to the cochlea, in a rodent model. In this method of administration, a magnetic field is employed to actively transport drug-eluting iron oxide-core nanoparticles into the cochlea, where they then release their drug payload (we delivered the steroid prednisolone). Our study design and selection of control groups was based on published regulatory guidance for safety studies that involve local drug delivery. We tested for both single and multiple delivery doses to the cochlea, and found that magnetic delivery did not harm hearing. There was no statistical difference in hearing between magnetically treated ears versus ears that received intra-tympanic steroid (a mimic of a standard-of-care for sudden sensorineural hearing loss), both 2 and 30 days after treatment. Since our treatment is local to the ear, the levels of steroid and iron circulating systemically after our treatment were low, below mass-spectrometry detection limits for the steroid and no different from normal for iron. No adverse findings were observed in ear tissue histopathology or in animal gross behavior. At 2 and 30 days after treatment, inflammatory changes in the ear were limited to the middle ear, were very mild in severity, and by day 90 there was ongoing and almost complete reversibility of these changes. There were no ear tissue scarring or hemorrhage trends associated with magnetic delivery. In summary, after conducting a pre-clinical safety study based on FDA guidance documents, no adverse safety issues were observed. Copyright © 2017. Published by Elsevier B.V.

Sensorineural deafness

MedlinePlus

... or dizzy (more common with Meniere disease and acoustic neuromas ) Ringing or buzzing sound in the ears ( ... a long time Meniere disease Tumor, such as acoustic neuroma Use of certain medicines Working around loud ...

Massively Parallel Sequencing of a Chinese Family with DFNA9 Identified a Novel Missense Mutation in the LCCL Domain of COCH

PubMed Central

Gu, Xiaodong; Su, Wenling; Tang, Mingliang; Guo, Luo; Zhao, Liping

2016-01-01

DFNA9 is a late-onset, progressive, autosomal dominantly inherited sensorineural hearing loss with vestibular dysfunction, which is caused by mutations in the COCH (coagulation factor C homology) gene. In this study, we investigated a Chinese family segregating autosomal dominant nonsyndromic sensorineural hearing loss. We identified a missense mutation c.T275A p.V92D in the LCCL domain of COCH cosegregating with the disease and absent in 100 normal hearing controls. This mutation leads to substitution of the hydrophobic valine to an acidic amino acid aspartic acid. Our data enriched the mutation spectrum of DFNA9 and implied the importance for mutation screening of COCH in age related hearing loss with vestibular dysfunctions. PMID:28116169

Congenital sensori-neural deafness associated with onycho-osteo dystrophy and mental retardation (D.O.O.R. syndrome).

PubMed

Cantwell, R J

1975-01-01

A characteristic syndrome is described in which congenital sensori-neural deafness is associated not only with onychodystrophy but also with congenital bony anomalies the most characteristic of which are tri-phalangeal thumbs, bi-phalangeal digits of hands and feet, and dystrophic terminal phalanges of some of the fingers and toes. In addition, there is mental retardation and the dermatoglyphics are characterized by the presence of 10 arches and elevation of the atd angles. The syndrome is inherited as an autosomal recessive. It is suggested that this entity be named the D.O.O.R. Syndrome because of the deafness, onychodystrophy, osteodystrophy and retardation. A similar syndrome without retardation as described by Goodman et al. (1969) appears to be inherited as an autosomal dominant.

Improvements in speech understanding with wireless binaural broadband digital hearing instruments in adults with sensorineural hearing loss.

PubMed

Kreisman, Brian M; Mazevski, Annette G; Schum, Donald J; Sockalingam, Ravichandran

2010-03-01

This investigation examined whether speech intelligibility in noise can be improved using a new, binaural broadband hearing instrument system. Participants were 36 adults with symmetrical, sensorineural hearing loss (18 experienced hearing instrument users and 18 without prior experience). Participants were fit binaurally in a planned comparison, randomized crossover design study with binaural broadband hearing instruments and advanced digital hearing instruments. Following an adjustment period with each device, participants underwent two speech-in-noise tests: the QuickSIN and the Hearing in Noise Test (HINT). Results suggested significantly better performance on the QuickSIN and the HINT measures with the binaural broadband hearing instruments, when compared with the advanced digital hearing instruments and unaided, across and within all noise conditions.

Waardenburg syndrome type 2: an orthodontic perspective.

PubMed

Şuhani, Raluca Diana; Şuhani, Mihai Flaviu; Muntean, Alexandrina; Mesaroş, Michaela Florica; Badea, Mîndra Eugenia

2015-01-01

Waardenburg syndrome is a rare form of neurocristopathy. It is a disorder in the development of neural crest cells, caused by an altered cellular migration during the embryonic phase. That alteration causes an association of different abnormalities such as pigmentary disturbances of the hair, iris, skin, stria vascularis of the cochlea, dystopia canthorum and sensorineural hearing loss. We report a case of a 14-year-old Romanian male, with a family history of Waardenburg syndrome (mother) and Usher syndrome (father - congenitally sensorineural hearing loss and retinal degeneration). The case particularities are: the correlation between malocclusion and Waardenburg syndrome due to hypoplastic alae nasi and also factors that produced hearing loss, which could be Waardenburg syndrome, Usher syndrome or the presence of the connexin 26 (W24X) gene mutation.

The sudden death and sudden birth of quantum discord.

PubMed

Xia, Wei; Hou, Jin-Xing; Wang, Xiao-Hui; Liu, Si-Yuan

2018-03-28

The interaction of quantum system and its environment brings out abundant quantum phenomenons. The sudden death of quantum resources, including entanglement, quantum discord and coherence, have been studied from the perspective of quantum breaking channels (QBC). QBC of quantum resources reveal the common features of quantum resources. The definition of QBC implies the relationship between quantum resources. However, sudden death of quantum resources can also appear under some other quantum channels. We consider the dynamics of Bell-diagonal states under a stochastic dephasing noise along the z-direction, and the sudden death and sudden birth of quantum discord are investigated. Next we explain this phenomenon from the geometric structure of quantum discord. According to the above results, the states with sudden death and sudden birth can be filtered in three-parameter space. Then we provide two necessary conditions to judge which kind of noise channels can make Bell-diagonal states sudden death and sudden birth. Moreover, the relation between quantum discord and coherence indicates that the sudden death and sudden birth of quantum discord implies the sudden death and sudden birth of coherence in an optimal basis.

Rapidly fluctuating anosmia: A clinical sign for unilateral smell impairment.

PubMed

Negoias, Simona; Friedrich, Hergen; Caversaccio, Marco D; Landis, Basile N

2016-02-01

Reports about fluctuating olfactory deficits are rare, as are reports of unilateral olfactory loss. We present a case of unilateral anosmia with contralateral normosmia, presenting as rapidly fluctuating anosmia. The olfactory fluctuation occurred in sync with the average nasal cycle duration. Examination after nasal decongestion, formal smell testing, and imaging revealed unilateral, left-sided anosmia of sinonasal cause, with right-sided normosmia. We hypothesize that the nasal cycle induced transient anosmia when blocking the normosmic side. Fluctuating olfactory deficits might hide a unilateral olfactory loss and require additional unilateral testing and thorough workup. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

Molecular Etiology of Hereditary Single-Side Deafness: Its Association With Pigmentary Disorders and Waardenburg Syndrome.

PubMed

Kim, Shin Hye; Kim, Ah Reum; Choi, Hyun Seok; Kim, Min Young; Chun, Eun Hi; Oh, Seung-Ha; Choi, Byung Yoon

2015-10-01

Unilateral sensorineural hearing loss (USNHL)/single-side deafness (SSD) is a frequently encountered disability in children. The etiology of a substantial portion of USNHL/SSD still remains unknown, and genetic causes have not been clearly elucidated. In this study, the authors evaluated the heritability of USNHL/SSD.The authors sequentially recruited 50 unrelated children with SSD. For an etiologic diagnosis, we performed a rigorous review on the phenotypes of family members of all children and conducted, if necessary, molecular genetic tests including targeted exome sequencing of 129 deafness genes.Among the 50 SSD children cohort, the authors identify 4 (8%) unrelated SSD probands from 4 families (SH136, SB173, SB177, and SB199) with another hearing impaired family members. Notably, all 4 probands in our cohort with a familial history of SSD also have pigmentary abnormalities such as brown freckles or premature gray hair within first degree relatives, which may indicate that genes whose products are involved with pigmentary disorder could be candidates for heritable SSD. Indeed, SH136 and SB199 turned out to segregate a mutation in MITF and PAX3, respectively, leading to a molecular diagnosis of Waardenburg syndrome (WS).We report, for the first time in the literature, a significant heritability of pediatric SSD. There is a strong association between the heritability of USNHL/SSD and the pigmentary abnormality, shedding a new light on the understanding of the molecular basis of heritable USNHL/SSD. In case of children with congenital SSD, it would be mandatory to rigorously screen pigmentary abnormalities. WS should also be included in the differential diagnosis of children with USNHL/SSD, especially in a familial form.

Hearing outcomes of the active bone conduction system Bonebridge® in conductive or mixed hearing loss.

PubMed

Carnevale, Claudio; Til-Pérez, Guillermo; Arancibia-Tagle, Diego J; Tomás-Barberán, Manuel D; Sarría-Echegaray, Pedro L

2018-05-18

The active transcutaneous bone conduction implant Bonebridge ® , is indicated for patients affected by bilateral conductive/mixed hearing loss or unilateral sensorineural hearing loss, showing hearing outcomes similar to other percutaneous bone conduction implants, but with a lower rate of complications. The aim of this study was to analyze the hearing outcomes in a series of 26 patients affected by conductive or mixed hearing loss and treated with Bonebridge ® . 26 of 30 patients implanted with Bonebridge ® between October 2012 and May 2017, were included in the study. We compared the air conduction thresholds at the frequencies 500, 1000, 2000, 3000, 4000Hz, the SRT50% and the percentage of correct answers at an intensity of 50dB with and without the implant. "Pure tone average" with the implant was 34.91dB showing an average gain of 33.46dB. Average SRT 50% with the implant was 34.33dB, whereas before the surgery no patient achieved 50% of correct answers at a sound intensity of 50dB. The percentage of correct answers at 50dB changed from 11% without the implant to 85% with it. We only observed one complication consisting of an extrusion of the implant in a patient with a history of 2 previous rhytidectomies. The hearing outcomes obtained in our study are similar to those published in the literature. Bonebridge ® represents an excellent alternative in the treatment of conductive or mixed hearing loss, and with a lower rate of complications. Copyright © 2018 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Publicado por Elsevier España, S.L.U. All rights reserved.

Hearing Loss in Children With Asymptomatic Congenital Cytomegalovirus Infection

PubMed Central

Chung, Winnie; Flores, Marily; Blum, Peggy; Caviness, A. Chantal; Bialek, Stephanie R.; Grosse, Scott D.; Miller, Jerry A.; Demmler-Harrison, Gail

2017-01-01

OBJECTIVES: To assess the prevalence, characteristics, and risk of sensorineural hearing loss (SNHL) in children with congenital cytomegalovirus infection identified through hospital-based newborn screening who were asymptomatic at birth compared with uninfected children. METHODS: We included 92 case-patients and 51 controls assessed by using auditory brainstem response and behavioral audiometry. We used Kaplan–Meier survival analysis to estimate the prevalence of SNHL, defined as ≥25 dB hearing level at any frequency and Cox proportional hazards regression analyses to compare SNHL risk between groups. RESULTS: At age 18 years, SNHL prevalence was 25% (95% confidence interval [CI]: 17%–36%) among case-patients and 8% (95% CI: 3%–22%) in controls (hazard ratio [HR]: 4.0; 95% CI: 1.2–14.5; P = .02). Among children without SNHL by age 5 years, the risk of delayed-onset SNHL was not significantly greater for case-patients than for controls (HR: 1.6; 95% CI: 0.4–6.1; P = .5). Among case-patients, the risk of delayed-onset SNHL was significantly greater among those with unilateral congenital/early-onset hearing loss than those without (HR: 6.9; 95% CI: 2.5–19.1; P < .01). The prevalence of severe to profound bilateral SNHL among case-patients was 2% (95% CI: 1%–9%). CONCLUSIONS: Delayed-onset and progression of SNHL among children with asymptomatic congenital cytomegalovirus infection continued to occur throughout adolescence. However, the risk of developing SNHL after age 5 years among case-patients was not different than in uninfected children. Overall, 2% of case-patients developed SNHL that was severe enough for them to be candidates for cochlear implantation. PMID:28209771

Monitoring the Hearing Handicap and the Recognition Threshold of Sentences of a Patient with Unilateral Auditory Neuropathy Spectrum Disorder with Use of a Hearing Aid.

PubMed

Lima, Aline Patrícia; Mantello, Erika Barioni; Anastasio, Adriana Ribeiro Tavares

2016-04-01

Introduction Treatment for auditory neuropathy spectrum disorder (ANSD) is not yet well established, including the use of hearing aids (HAs). Not all patients diagnosed with ASND have access to HAs, and in some cases HAs are even contraindicated. Objective To monitor the hearing handicap and the recognition threshold of sentences in silence and in noise in a patient with ASND using an HA. Resumed Report A 47-year-old woman reported moderate sensorineural hearing loss in the right ear and high-frequency loss of 4 kHz in the left ear, with bilateral otoacoustic emissions. Auditory brainstem response suggested changes in the functioning of the auditory pathway (up to the inferior colliculus) on the right. An HA was indicated on the right. The patient was tested within a 3-month period before the HA fitting with respect to recognition threshold of sentences in quiet and in noise and for handicap determination. After HA use, she showed a 2.1-dB improvement in the recognition threshold of sentences in silence, a 6.0-dB improvement for recognition threshold of sentences in noise, and a rapid improvement of the signal-to-noise ratio from +3.66 to -2.4 dB when compared with the same tests before the fitting of the HA. Conclusion There was a reduction of the auditory handicap, although speech perception continued to be severely limited. There was a significant improvement of the recognition threshold of sentences in silence and in noise and of the signal-to-noise ratio after 3 months of HA use.

Bilateral uveitis and Usher syndrome: a case report.

PubMed

Benson, Matthew D; MacDonald, Ian M

2015-03-15

Usher syndrome is a genetically heterogeneous condition and represents the most common cause of inherited combined vision and hearing loss. Deficits manifest as sensorineural hearing loss that typically develops at a young age and retinitis pigmentosa that can lead to peripheral vision loss and night blindness. As a result, this syndrome can have a significant impact on a patient's quality of life. Previous studies have described an association between Usher syndrome and Fuchs' heterochromic iridocyclitis, a form of non-granulomatous uveitis that generally presents in a unilateral manner. We present a rare finding of bilateral uveitis and, to the best of our knowledge, the first report of granulomatous uveitis as a feature in a patient with Usher syndrome. A 45-year-old Caucasian woman with a known history of retinitis pigmentosa presented to our clinic with suspected Usher syndrome, given her report of long-standing hearing loss. Aside from a mild loss in visual acuity, our patient was otherwise asymptomatic. Visual field testing, audiology and electroretinography findings supported the diagnosis of Usher syndrome. With slit lamp examination she was found to have bilateral keratic precipitates, with large, greasy-white, mutton-fat keratic precipitates on the endothelial surface of her left eye. A thorough work-up that included blood tests and imaging was negative for an alternative cause of her uveitis. We present a rare finding of bilateral uveitis and what we believe to be the first reported instance of mutton-fat keratic precipitates and granulomatous uveitis as a feature in a patient with Usher syndrome. By identifying atypical presentations of the disease, we hope to contribute to the range of ophthalmic conditions that may be seen in association with Usher syndrome.

Hearing loss in children with growth hormone deficiency.

PubMed

Muus, John S; Weir, Forest W; Kreicher, Kathryn L; Bowlby, Deborah A; Discolo, Christopher M; Meyer, Ted A

2017-09-01

Although insulin-like growth factor 1 (IGF-1) has been shown to be important for inner-ear development in animal models, little is known about the otologic and audiologic findings of children with growth hormone deficiency (GHD). The goal of this study is to evaluate the prevalence, type, and severity of hearing impairment in children with GHD. Audiologic, otologic, and demographic data were recorded for children with a diagnosis of GHD in the AudGen database. Data for each patient were selected based on the first encounter with available complete audiometric data or the first encounter with a type of hearing loss documented. The patients were then stratified by type and severity of hearing loss, and otologic issues were documented. A separate cohort comprised of children with GHD without hearing loss was compared as a control. 209 children with GHD met inclusion criteria. 173 (83%) of these patients had hearing loss. 79% of losses were bilateral and 21% were unilateral (309 total ears with hearing loss). 293 of the 309 ears with hearing loss had audiograms with ear-specific thresholds; 47 had conductive, 24 had sensorineural, 65 had mixed and 157 had undefined hearing loss with incomplete audiograms. Pure-tone averages (PTA) were higher among patients with mixed hearing loss compared to patients with all other loss types. Hearing loss is prevalent in children with GHD with a predisposition to be bilateral. These findings suggest the need for increased awareness and routine hearing screening for patients with GHD. Further studies may elucidate the etiology of the hearing impairment in children with GHD to better aid pediatricians, endocrinologists, otolaryngologists and audiologists when assessing and managing these children. Copyright © 2017 Elsevier B.V. All rights reserved.

Malformation of the eighth cranial nerve in children.

PubMed

de Paula-Vernetta, Carlos; Muñoz-Fernández, Noelia; Mas-Estellés, Fernando; Guzmán-Calvete, Abel; Cavallé-Garrido, Laura; Morera-Pérez, Constantino

2016-01-01

Prevalence of congenital sensorineural hearing loss (SNHL) is approximately 1.5-6 in every 1,000 newborns. Dysfunction of the auditory nerve (auditory neuropathy) may be involved in up to 1%-10% of cases; hearing losses because of vestibulocochlear nerve (VCN) aplasia are less frequent. The objectives of this study were to describe clinical manifestations, hearing thresholds and aetiology of children with SNHL and VCN aplasia. We present 34 children (mean age 20 months) with auditory nerve malformation and profound HL taken from a sample of 385 children implanted in a 10-year period. We studied demographic characteristics, hearing, genetics, risk factors and associated malformations (Casselman's and Sennaroglu's classifications). Data were processed using a bivariate descriptive statistical analysis (P<.05). Of all the cases, 58.8% were bilateral (IIa/IIa and I/I were the most common). Of the unilateral cases, IIb was the most frequent. Auditory screening showed a sensitivity of 77.4%. A relationship among bilateral cases and systemic pathology was observed. We found a statistically significant difference when comparing hearing loss impairment and patients with different types of aplasia as defined by Casselman's classification. Computed tomography (CT) scan yielded a sensitivity of 46.3% and a specificity of 85.7%. However, magnetic resonance imaging (MRI) was the most sensitive imaging test. Ten percent of the children in a cochlear implant study had aplasia or hypoplasia of the auditory nerve. The degree of auditory loss was directly related to the different types of aplasia (Casselman's classification) Although CT scan and MRI are complementary, the MRI is the test of choice for detecting auditory nerve malformation. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

Vestibular system paresis due to emergency endovascular catheterization

PubMed Central

Simoceli, Lucinda; Sguillar, Danilo Anunciatto; Santos, Henrique Mendes Paiva; Caputti, Camilla

2012-01-01

Summary Objective: The objective of this story of case is to describe an uncommon cause of associated peripheral Vestibulopathy to the unilateral auditory loss in aged patient after catheterization of urgency. Story of case: Patient of the masculine sort, 82 years, submitted to the correction of abdominal ragged aneurism of aorta, in the intra-operative suffered heart attack acute from the myocardium needing primary angioplasty. High after hospital it relates to complaint of accented hearing loss to the right and crippling vertigo, without focal neurological signals. To the otorhinolaryngological clinical examination it presented: Test of Weber lateralized for the left, spontaneous nystagmus for the left, marches rocking, has taken normal disbasia and ataxia, index-nose and diadochokinesia, Test of Romberg with oscillation without fall and Fukuda with lateral shunting line for the right. The audiometric examination evidenced deafness to the right and sensorineural loss to the left in sharps, areflexia initial to the right in caloric test e, the computerized tomography of the secular bones and brainstem, presence of metallic connecting rod crossing the right secular bone, from the vein internal jugular vein and bulb jugular vein, crossing the posterior, superior and vestibule semicircular canals, projecting itself in temporal lobe. The radiological diagnoses was traumatic injury for guide to endovascular metallic during catheterization of urgency and the behavior, considering that the patient had not compensated the balance, it was vestibular rehabilitation. Conclusion: Complaints of giddiness in the aged patient must be closely evaluated of its pathological clinical description because the antecedents of illnesses and previous treatments, in general, direct the diagnostic hypotheses however they can bring unexpected alterations. PMID:25991947

Autosomal dominant transmission of a Goldenhar-like syndrome: Description of a family and report of a sporadic case with a de novo 4p16;8q24.11 translocation

DOE Office of Scientific and Technical Information (OSTI.GOV)

Graganm H.N. Jr.; Hixon, H.; Bacino, C.A.

1994-09-01

We report vertical transmission of a Goldenhar-like syndrome, including a father and 5 offspring, with male-to-male transmission and variable features that include hearing loss, ear anomalies (microtia, ear tags/pits), branchial cysts, ocular/periocular dermoids, micrognathia and seizures. We also report an individual with an apparently balanced de novo reciprocal translocation with breakpoints at 4p16 and 8q24.11. This individual has unilateral microtia, an epibulbar dermoid cyst, facial asymmetry with a small chin, and seizures. In addition to these features resembling those seen in the family above, she has multiple exostoses, supraventricular tachycardia, hypoglycemia and mild developmental delays. Based on the overlap inmore » physical findings between this family and the individual with the de novo reciprocal translocation, linkage studies on the family were intiated. Preliminary results exclude linkage to HOX 7 at 4p16.1 but not to 8q. The brancho-oto-renal syndrome has previously been localized to 8q11-8q13, but linkage to this region appears unlikely. Although most cases of Goldenhar syndrome appear to be sporadic, there are a few reports of autosomal dominant inheritance (MIM No. 164210). One such family showed vertical transmission of dermoids, ear anomalies, hearing loss, micrognathia and vertebral anomalies, but no branchial cysts. Another family showed sensorineural deafness, preauricular pits, and branchial fistulae, and other families reveal ear anomalies, branchial fistulas, and hearing loss. These latter families appear to lack ocular/periocular dermoids, and appear to be affected by a different disorder (MIM No. 125100). Further clinical delineation of such families, combined with genetic linkage analysis, should help to sort out this heterogeneity.« less

Sclerosteosis involving the temporal bone: histopathologic aspects.

PubMed

Nager, G T; Hamersma, H

1986-01-01

Sclerosteosis is a rare, potentially lethal, autosomal recessive, progressive craniotubular sclerosing bone dysplasia with characteristic facial and skeletal features. The temporal bone changes include a marked increase in overall size, extensive sclerosis, narrowing of the external auditory canal, and severe constriction of the internal auditory meatus, fallopian canal, eustachian tube, and middle ear cleft. Attenuation of the bony canals of the 9th, 10th, and 11th cranial nerves, reduction in size of the internal carotid artery, and severe obliteration of the sigmoid sinus and jugular bulb also occur. Loss of hearing, generally bilateral, is a frequent symptom. It often manifests in early childhood and initially is expressed as sound conduction impairment. Later, a sensorineural hearing loss and loss of vestibular nerve function often develop. Impairment of facial nerve function is another feature occasionally present at birth. In the beginning, a unilateral intermittent facial weakness may occur which eventually progresses to a bilateral permanent facial paresis. The histologic examination of the temporal bones from a patient with sclerosteosis explains the mechanisms involved in the progressive impairment of sound conduction and loss of cochlear, vestibular, and facial nerve function. There is a decrease of the arterial blood supply to the brain and an obstruction of the venous drainage from it. The histopathology reveals the obstacles to decompression of the middle ear cleft, ossicular chain, internal auditory and facial canals, and the risks, and in many instances the contraindications, to such procedures. On the other hand, decompression of the sigmoid sinus and jugular bulb should be considered as an additional life-saving procedure in conjunction with the prophylactic craniotomy recommended in all adult patients.

Monitoring the Hearing Handicap and the Recognition Threshold of Sentences of a Patient with Unilateral Auditory Neuropathy Spectrum Disorder with Use of a Hearing Aid

PubMed Central

Lima, Aline Patrícia; Mantello, Erika Barioni; Anastasio, Adriana Ribeiro Tavares

2015-01-01

Introduction Treatment for auditory neuropathy spectrum disorder (ANSD) is not yet well established, including the use of hearing aids (HAs). Not all patients diagnosed with ASND have access to HAs, and in some cases HAs are even contraindicated. Objective To monitor the hearing handicap and the recognition threshold of sentences in silence and in noise in a patient with ASND using an HA. Resumed Report A 47-year-old woman reported moderate sensorineural hearing loss in the right ear and high-frequency loss of 4 kHz in the left ear, with bilateral otoacoustic emissions. Auditory brainstem response suggested changes in the functioning of the auditory pathway (up to the inferior colliculus) on the right. An HA was indicated on the right. The patient was tested within a 3-month period before the HA fitting with respect to recognition threshold of sentences in quiet and in noise and for handicap determination. After HA use, she showed a 2.1-dB improvement in the recognition threshold of sentences in silence, a 6.0-dB improvement for recognition threshold of sentences in noise, and a rapid improvement of the signal-to-noise ratio from +3.66 to −2.4 dB when compared with the same tests before the fitting of the HA. Conclusion There was a reduction of the auditory handicap, although speech perception continued to be severely limited. There was a significant improvement of the recognition threshold of sentences in silence and in noise and of the signal-to-noise ratio after 3 months of HA use. PMID:27096026

Auditory Speech Perception Development in Relation to Patient's Age with Cochlear Implant

PubMed Central

Ciscare, Grace Kelly Seixas; Mantello, Erika Barioni; Fortunato-Queiroz, Carla Aparecida Urzedo; Hyppolito, Miguel Angelo; Reis, Ana Cláudia Mirândola Barbosa dos

2017-01-01

Introduction  A cochlear implant in adolescent patients with pre-lingual deafness is still a debatable issue. Objective  The objective of this study is to analyze and compare the development of auditory speech perception in children with pre-lingual auditory impairment submitted to cochlear implant, in different age groups in the first year after implantation. Method  This is a retrospective study, documentary research, in which we analyzed 78 reports of children with severe bilateral sensorineural hearing loss, unilateral cochlear implant users of both sexes. They were divided into three groups: G1, 22 infants aged less than 42 months; G2, 28 infants aged between 43 to 83 months; and G3, 28 older than 84 months. We collected medical record data to characterize the patients, auditory thresholds with cochlear implants, assessment of speech perception, and auditory skills. Results  There was no statistical difference in the association of the results among groups G1, G2, and G3 with sex, caregiver education level, city of residence, and speech perception level. There was a moderate correlation between age and hearing aid use time, age and cochlear implants use time. There was a strong correlation between age and the age cochlear implants was performed, hearing aid use time and age CI was performed. Conclusion  There was no statistical difference in the speech perception in relation to the patient's age when cochlear implant was performed. There were statistically significant differences for the variables of auditory deprivation time between G3 - G1 and G2 - G1 and hearing aid use time between G3 - G2 and G3 - G1. PMID:28680487

Molecular Etiology of Hereditary Single-Side Deafness

PubMed Central

Kim, Shin Hye; Kim, Ah Reum; Choi, Hyun Seok; Kim, Min Young; Chun, Eun Hi; Oh, Seung-Ha; Choi, Byung Yoon

2015-01-01

Abstract Unilateral sensorineural hearing loss (USNHL)/single-side deafness (SSD) is a frequently encountered disability in children. The etiology of a substantial portion of USNHL/SSD still remains unknown, and genetic causes have not been clearly elucidated. In this study, the authors evaluated the heritability of USNHL/SSD. The authors sequentially recruited 50 unrelated children with SSD. For an etiologic diagnosis, we performed a rigorous review on the phenotypes of family members of all children and conducted, if necessary, molecular genetic tests including targeted exome sequencing of 129 deafness genes. Among the 50 SSD children cohort, the authors identify 4 (8%) unrelated SSD probands from 4 families (SH136, SB173, SB177, and SB199) with another hearing impaired family members. Notably, all 4 probands in our cohort with a familial history of SSD also have pigmentary abnormalities such as brown freckles or premature gray hair within first degree relatives, which may indicate that genes whose products are involved with pigmentary disorder could be candidates for heritable SSD. Indeed, SH136 and SB199 turned out to segregate a mutation in MITF and PAX3, respectively, leading to a molecular diagnosis of Waardenburg syndrome (WS). We report, for the first time in the literature, a significant heritability of pediatric SSD. There is a strong association between the heritability of USNHL/SSD and the pigmentary abnormality, shedding a new light on the understanding of the molecular basis of heritable USNHL/SSD. In case of children with congenital SSD, it would be mandatory to rigorously screen pigmentary abnormalities. WS should also be included in the differential diagnosis of children with USNHL/SSD, especially in a familial form. PMID:26512583

Evaluation of Long-Term Cochlear Implant Use in Subjects With Acquired Unilateral Profound Hearing Loss: Focus on Binaural Auditory Outcomes.

PubMed

Mertens, Griet; De Bodt, Marc; Van de Heyning, Paul

Cochlear implantation (CI) in subjects with unilateral profound sensorineural hearing loss was investigated. The authors of the present study demonstrated the binaural auditory outcomes in a 12- and 36-month prospective cohort outcome study. The present study aimed to do a long-term (LT) evaluation of the auditory outcomes in an analogous study group. LT evaluation was derived from 12 single-sided deaf (SSD) CI recipients and from 11 CI recipients with asymmetric hearing loss (AHL). A structured interview was conducted with each subjects. Speech perception in noise and sound localization were assessed in a CIOFF and in a CION condition. Four binaural effects were calculated: summation effect (S0N0), squelch effect (S0NCI), combined head shadow effect (SCIN0), and spatial release from masking (SRM). At the LT evaluation, the contribution of a CI or a bone conduction device on speech perception in noise was investigated in two challenging spatial configurations in the SSD group. All (23/23) subjects wore their CI 7 days a week at LT follow-up evaluation, which ranged from 3 to 10 years after implantation. In the SSD group, a significant combined head shadow effect of 3.17 dB and an SRM benefit of 4.33 dB were found. In the AHL group, on the other hand, the summation effect (2.00 dB), the squelch effect (2.67 dB), the combined head shadow effect (3.67 dB), and SRM benefit (2.00 dB) were significant at LT testing. In both the spatial challenging configurations, the speech in noise results was significantly worse in the condition with the bone conduction device compared with the unaided condition. No negative effect was found for the CION condition. A significant benefit in the CION condition was found for sound localization compared with the CIOFF condition in the SSD group and in the AHL group. All subjects wore their CI 7 days a week at LT follow-up evaluation. The presence of binaural effects has been demonstrated with speech in noise testing, sound localization, and subjective evaluation. In the AHL group, all investigated binaural effects were found to be significant. In the SSD group on the other hand, only SRM and the head shadow, the two most robust binaural effects, were significantly present. However, it took 12M before the SSD and the AHL subjects significantly benefit from the head shadow effect. These reported results could guide counseling of future CI candidates with SSD and AHL in general.

Are bilateral idiopathic clubfeet more severe than unilateral feet? A severity and treatment analysis.

PubMed

Agarwal, Anil; Agrawal, Nargesh; Barik, Sitanshu; Gupta, Neeraj

2018-01-01

Evidences suggest that different subgroups of idiopathic clubfoot exist with differences in severity and treatment outcomes. This study compares the severity and treatment outcomes of unilateral and bilateral clubfoot. We retrospectively studied 161 patients (bilateral 66, unilateral 95) with primary idiopathic clubfeet to evaluate the differences in severity and treatment. The parameters analyzed were precasting Pirani score, number of casts required, pretenotomy Pirani score, pretenotomy dorsiflexion, rate of tenotomy, and post-tenotomy dorsiflexion achieved. A Pirani score of at least 5 was classified as very severe and 4.5 or less was classified as less severe. There were 49=(74.24%) male and 17 (25.75%) female patients in the bilateral group and 76 (80%) male and 19 (20%) female patients in the unilateral group. Out of 95 unilateral patients, 34 were left sided (35.8%). Comparing severity, the mean precasting Pirani score in bilateral patients (5.4 ± 0.6) was statistically more than the unilateral patients (4.9 ± 0.7). The number of casts required was significantly more in bilateral feet compared to unilateral (bilateral 5.3 ± 1.7, unilateral 4.7 ± 1.7; p < 0.011). Achilles tenotomy was required in all feet. Post Ponseti treatment, the foot deformity correction achieved (pretenotomy Pirani score, pretenotomy, and post-tenotomy dorsiflexion) was statistically similar in both unilateral and bilateral feet. Idiopathic bilateral clubfoot was more severe than unilateral foot at initial presentation and required more number of corrective casts. Post Ponseti treatment, the deformity correction in bilateral foot was similar to unilateral foot.

Unilateral photophobia or phonophobia in migraine compared with trigeminal autonomic cephalalgias.

PubMed

Irimia, P; Cittadini, E; Paemeleire, K; Cohen, A S; Goadsby, P J

2008-06-01

Our objective was to compare the presence of self-reported unilateral photophobia or phonophobia, or both, during headache attacks comparing patients with trigeminal autonomic cephalalgias (TACs)--including cluster headache, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and paroxysmal hemicrania--or hemicrania continua, and other headache types. We conducted a prospective study in patients attending a referral out-patient clinic over 5 months and those admitted for an intramuscular indomethacin test. Two hundred and six patients were included. In episodic migraine patients, two of 54 (4%) reported unilateral photophobia or phonophobia, or both. In chronic migraine patients, six of 48 (13%) complained of unilateral photophobia or phonophobia, or both, whereas none of the 24 patients with medication-overuse headache reported these unilateral symptoms, although these patients all had clinical symptoms suggesting the diagnosis of migraine. Only three of 22 patients (14%) suffering from new daily persistent headache (NDPH) experienced unilateral photophobia or phonophobia. In chronic cluster headache 10 of 21 patients (48%) had unilateral photophobia or phonophobia, or both, and this symptom appeared in four of five patients (80%) with episodic cluster headache. Unilateral photophobia or phonophobia, or both, were reported by six of 11 patients (55%) with hemicrania continua, five of nine (56%) with SUNCT, and four of six (67%) with chronic paroxysmal hemicrania. Unilateral phonophobia or photophobia, or both, are more frequent in TACs and hemicrania continua than in migraine and NDPH. The presence of these unilateral symptoms may be clinically useful in the differential diagnosis of primary headaches.

Genetics Home Reference: Burn-McKeown syndrome

MedlinePlus

... ear (sensorineural hearing loss) and changes in the middle ear (conductive hearing loss). Other features that can occur in Burn-McKeown syndrome include mild short stature and congenital heart defects ...

[Functional hearing examinations in patients suffering from diabetes mellitus type 1 in regard to disease duration].

PubMed

Pudar, Goran; Vlaski, Ljiljana; Filipović, Danka; Tanackov, Ilija

2010-01-01

Problems of hearing disturbances in persons suffering from diabetes have been attracting great attention for many decades. In this study we examined the auditory function of 50 patients suffering from diabetes mellitus type 1 of different duration by analyzing results of pure-tone audiometry and brainstem auditory evoked potentials. The obtained results of measuring were compared to 30 healthy subjects from the corresponding age and gender group. The group of diabetic patients was divided according to the disease duration (I group 0-5 years; II group 6-10 years, III group over 10 years). A statistically significant increase of sensorineural hearing loss was found in the diabetics according to the duration of their disease (I group = 14.09%, II group = 21.39%, III group = 104.89%). The results of the brain stem auditory evoked potentials, the significance threshold being p = 0.05 between the controls and the diabetics at all levels of absolute latency of right and left sides, did not show significant differences in the mean values. In the case of interwave latencies, the diabetic patients were found to have a significant qualitative difference of intervals I-III and I-V on both ears in the sense of internal distribution of response. In cases of sensorineural hearing loss we found a significant connection with prolonged latencies of I wave on the right ear and of I and V waves on the left ear. In all probability, the cause of these results could be found in distinctive individuality of the organism reactions to the consequences of this disease (disturbance in the distal part of N. cochlearis). The results of research have shown the existence of a significant sensorineural hearing loss in the patients with diabetes mellitus type 1 in accordance to the disease duration. We also found qualitative changes of brainstem auditory evoked potentials in the diabetic patients in comparison to the controls as well as significant quantitative changes in regard to the presence of sensorineural hearing loss of the patients.

Unilateral scrotal angiomas: An expression of underlying varicocele.

PubMed

Tromp, Elise E; Kouwenhoven, Stijn T P; Quint, Koen D; Gmelig Meijling, Kevin A; Genders, Roel E

2016-01-01

The current case report describes a 35-year-old man who presented with unilateral scrotal angiomas. The presence of unilateral scrotal angiomas was associated with an underlying varicocele on the ipsilateral side due to increased venous pressure. In case of unilateral scrotal angiomas further examination for underlying pathology is necessary.

Bilateral tympanokeratomas (cholesteatomas) with bilateral otitis media, unilateral otitis interna and acoustic neuritis in a dog.

PubMed

Østevik, Liv; Rudlang, Kathrine; Holt Jahr, Tuva; Valheim, Mette; Njaa, Bradley Lyndon

2018-05-22

An aural cholesteatoma, more appropriately named tympanokeratoma, is an epidermoid cyst of the middle ear described in several species, including dogs, humans and Mongolian gerbils. The cyst lining consists of stratified, keratinizing squamous epithelium with central accumulation of a keratin debris. This case report describes vestibular ganglioneuritis and perineuritis in a dog with chronic otitis, bilateral tympanokeratomas and presumed extension of otic infection to the central nervous system. An 11-year-old intact male Dalmatian dog with chronic bilateral otitis externa and sudden development of symptoms of vestibular disease was examined. Due to the dog's old age the owner opted for euthanasia without any further examination or treatment and the dog was submitted for necropsy. Transection of the ears revealed grey soft material in the external ear canals and pearly white, dry material consistent with keratin in the tympanic bullae bilaterally. The brain and meninges were grossly unremarkable. Microscopical findings included bilateral otitis externa and media, unilateral otitis interna, ganglioneuritis and perineuritis of the spiral ganglion of the vestibulocochlear nerve and multifocal to coalescing, purulent meningitis. A keratinizing squamous epithelial layer continuous with the external acoustic meatus lined the middle ear compartments, consistent with bilateral tympanokeratomas. Focal bony erosion of the petrous portion of the temporal bone and squamous epithelium and Gram-positive bacterial cocci were evident in the left cochlea. The findings suggest that meningitis developed secondary to erosion of the temporal bone and ganglioneuritis and/or perineuritis of the vestibulocochlear nerve. Middle ear tympanokeratoma is an important and potentially life-threatening otic condition in the dog. Once a tympanokeratoma has developed expansion of the cyst can lead to erosion of bone and extension of otic infection to the inner ear, vestibulocochlear ganglion and nerve potentially leading to bacterial infection of the central nervous system.

Investigation of hypoxia-inducible factor-1α in hippocampal sclerosis: a postmortem study.

PubMed

Feast, Alexandra; Martinian, Lillian; Liu, Joan; Catarino, Claudia B; Thom, Maria; Sisodiya, Sanjay M

2012-08-01

Hypoxia-inducible factor-1α (HIF-1α) is involved in critical aspects of cell survival in response to hypoxia and regulates vascular endothelial growth factor (VEGF) expression. Previous experimental and human studies in epilepsy show up-regulation of VEGF following seizures, although expression of HIF-1α as its potential regulator has not been explored. We used a postmortem (PM) series from patients with epilepsy and hippocampal sclerosis (HS) to investigate patterns of expression of HIF-1α and VEGF and their potential contribution to neuroprotection. In 33 PMs (17 cases with unilateral HS, 3 with bilateral HS, 3 with No-HS, and 10 controls), we quantified neuronal immunolabeling for HIF-1α and VEGF in hippocampal subfields. HIF-1α- and VEGF-immunopositive hippocampal neurones were observed in HS, No-HS, and also in control cases; there was no significant difference in overall labeling between epilepsy cases and controls. In positive cases, HIF-1α and VEGF neuronal labeling localized primarily in CA1, CA4, and CA3 subfields in all groups; significantly more positive neurons were seen in the entorhinal cortex in epilepsy cases (p < 0.05). Labeling lateralized to the side of sclerosis in unilateral HS cases, with significant differences between hemispheres (p < 0.05). There was a trend for high HIF-1α labeling scores in patients with Dravet syndrome without HS and sudden unexpected death in epilepsy (SUDEP) cases, and lower scores with long seizure-free periods prior to death. Hippocampal HIF-1α and VEGF labeling showed a significant correlation. There was neuronal colocalization of HIF-1α and VEGF. Regional expression patterns are in keeping with seizure-related activation of HIF-1α and VEGF. The prominent expression in non-HS cases could support an overall neuroprotective effect. Correlation between HIF-1α and VEGF neuronal immunolabeling supports HIF-1α-mediated induction of VEGF in epilepsy. Wiley Periodicals, Inc. © 2012 International League Against Epilepsy.

Unilateral advancement of the maxillary minor segment by distraction osteogenesis in patients with repaired unilateral cleft lip and palate: report of two cases.

PubMed

Kuroe, Kazuto; Iino, Shoichiro; Shomura, Kenji; Okubo, Akiro; Sugihara, Kazumasa; Ito, Gakuji

2003-05-01

Collapse of the maxillary minor segment with lateral crossbite is a common feature in patients with repaired unilateral cleft lip/palate because of maxillary alveolar bony defect and palatal scar tissue. Distraction osteogenesis (DOG) is an effective technique of lengthening and augmentation for bone and gingiva. This case report describes the effects of unilateral advancement of the maxillary minor segment by DOG in two patients with the repaired unilateral cleft lip/palate.

The effect of the photobiomodulation in the treatment of Bell's palsy: clinical experience

NASA Astrophysics Data System (ADS)

Colombo, Fabio; Marques, Aparecida Maria C.; Carvalho, Carolina M.; Paraguassu, Gardenia M.; de Sousa, José A. C.; Magalhaes, Edival; Cangussu, Maria Cristina T.; de A. Reis, Silvia Regina; Pinheiro, Antonio Luiz B.

2012-03-01

The Bell's palsy (G51) consists of a unilateral face paralysis that sudden begins with unknown cause and can result in complete mimic loss or partial paralysis of the face. Damage to the VII cranial nerve can be found in the pathology, promoting mussel's inactivity. The light Photobiomodulation (LPBM) has presented ability of rush the tissue repair, favoring the regeneration of neural structures. The present study aimed to assess the effectiveness use of the 780nm laser and 850nm LED (light-emitting diode) in the treatment of the face paralysis. Were evaluated 14 patients that suffer of Bell's palsy whom were submitted to the light administration, on the Laser Clinic of the UFBA between 2005 and 2010. The treatment was performed by infrared Laser in 11 patients (78.57%), and by LED in 3 patients (21.42%). At the end of the 12 sections, 11 patients (78.57%) had presented themselves cure or with substantial improvement of the initial picture, however 3 patients (21.42%) dealt with infra-red Laser λ780nm had not evolution. The light presented as an effective method for the treatment of Bell's palsy, but the association with the physiotherapy and medications is important.

Stability in skipping gaits

NASA Astrophysics Data System (ADS)

Andrada, Emanuel; Müller, Roy; Blickhan, Reinhard

2016-11-01

As an alternative to walking and running, humans are able to skip. However, adult humans avoid it. This fact seems to be related to the higher energetic costs associated with skipping. Still, children, some birds, lemurs and lizards use skipping gaits during daily locomotion. We combined experimental data on humans with numerical simulations to test whether stability and robustness motivate this choice. Parameters for modelling were obtained from 10 male subjects. They locomoted using unilateral skipping along a 12 m runway. We used a bipedal spring loaded inverted pendulum to model and to describe the dynamics of skipping. The subjects displayed higher peak ground reaction forces and leg stiffness in the first landing leg (trailing leg) compared to the second landing leg (leading leg). In numerical simulations, we found that skipping is stable across an amazing speed range from skipping on the spot to fast running speeds. Higher leg stiffness in the trailing leg permits longer strides at same system energy. However, this strategy is at the same time less robust to sudden drop perturbations than skipping with a stiffer leading leg. A slightly higher stiffness in the leading leg is most robust, but might be costlier.

Management of optic neuritis

PubMed Central

Menon, Vimla; Saxena, Rohit; Misra, Ruby; Phuljhele, Swati

2011-01-01

Optic neuritis is an inflammatory condition of the optic nerve characterized by a sudden onset of unilateral visual loss, usually affecting young females. Demyelination associated with multiple sclerosis (MS) is the most common cause in regions where MS is prevalent; while in other places, there are a substantial proportion of cases where infective or autoimmune causes are seen. Optic Neuritis Treatment Trial (ONTT) was the first major study that provided information on the natural history, role of steroids in treatment and risk of development of MS. Subsequently, numerous clinical trials have evaluated different modalities of management of optic neuritis and MS. The Controlled High-Risk Subjects Avonex Multiple Sclerosis Prevention Study (CHAMPS); the Prevention of Relapses and Disability by Interferon β-1a Subcutaneously in Multiple Sclerosis (PRISMS) Trial; and, most recently, the Betaferon in Newly Emerging Multiple Sclerosis for Initial Treatment (BENEFIT) Study have provided large amount of information on the natural history of optic neuritis and management options available. However, due to the low prevalence of MS reported in Asian studies, high cost of therapy and indefinite time period of treatment, it may not be cost effective to start interferon therapy in most cases. PMID:21350281

Intermittent Haemoptysis due to an Aortobronchial Fistula in a Warmblood Mare.

PubMed

Versnaeyen, H; Saey, V; Vermeiren, D; Chiers, K; Ducatelle, R

2016-01-01

A 7-year-old warmblood mare showed sudden onset of mild intermittent haemoptysis. Clinical examination revealed no significant abnormalities. Haematological examination showed mild anaemia, hypoalbuminaemia and neutrophilia. Coagulation tests were normal. Endoscopic examination revealed unilateral pulmonary haemorrhage with blood clots in the bronchi and trachea. Treatment with antibiotics was started and the horse was given stable rest. Two weeks later, the horse was found dead with blood and frothy sputum leaking from the nostrils. Post-mortem examination revealed a large thoracic aortic aneurysm communicating with a pseudoaneurysm that had formed a fistula into a right bronchial branch. Microscopical examination of the aneurysm showed extensive medial fibrosis with prominent degeneration, fragmentation and mineralization of the elastic fibres and deposition of mucoid material in the tunica media. The pseudoaneurysm was lined by collagen bundles admixed with fibroblasts and a small amount of adipose tissue. Aortobronchial fistula is a rare condition in man that is usually associated with primary aortic pathology, most often aneurysms. To the authors' knowledge this is the first case of a fatal aortobronchial fistula in a horse or any other animal species. Copyright © 2016 Elsevier Ltd. All rights reserved.

Pain in trigeminal neuralgia: neurophysiology and measurement: a comprehensive review

PubMed Central

Kumar, S; Rastogi, S; Kumar, S; Mahendra, P; Bansal, M; Chandra, L

2013-01-01

Abstract Trigeminal neuralgia (TN) is defined as sudden, usually unilateral, severe, brief, stabbing recurrent episodes of pain within the distribution of one or more branches of the trigeminal nerve. It is the most frequent cranial neuralgia, the incidence being 1 per 1,000,00 persons per year. Pain attacks start abruptly and last several seconds but may persist 1 to 2 minutes. The attacks are initiated by non painful physical stimulation of specific areas (trigger points or zones) that are located ipsilateral to the pain. After each episode, there is usually a refractive period during which stimulation of the trigger zone will not induce the pain. According to the European Federation of Neurological Societies (EFNS) guidelines on neuropathic pain assessment and the American Academy of Neurology (AAN)-EFNS guidelines on TN management the neurophysiological recording of trigeminal reflexes represents the most useful and reliable test for the neurophysiological diagnosis of trigeminal pains. The present article discusses different techniques for investigation of the trigeminal system by which an accurate topographical diagnosis and profile of sensory fiber pathology can be determined. With the aid of neurophysiological recordings and quantitative sensory testing, it is possible to approach a mechanism-based classification of orofacial pain. PMID:24701256

Pain in trigeminal neuralgia: neurophysiology and measurement: a comprehensive review.

PubMed

Kumar, S; Rastogi, S; Kumar, S; Mahendra, P; Bansal, M; Chandra, L

2013-01-01

Trigeminal neuralgia (TN) is defined as sudden, usually unilateral, severe, brief, stabbing recurrent episodes of pain within the distribution of one or more branches of the trigeminal nerve. It is the most frequent cranial neuralgia, the incidence being 1 per 1,000,00 persons per year. Pain attacks start abruptly and last several seconds but may persist 1 to 2 minutes. The attacks are initiated by non painful physical stimulation of specific areas (trigger points or zones) that are located ipsilateral to the pain. After each episode, there is usually a refractive period during which stimulation of the trigger zone will not induce the pain. According to the European Federation of Neurological Societies (EFNS) guidelines on neuropathic pain assessment and the American Academy of Neurology (AAN)-EFNS guidelines on TN management the neurophysiological recording of trigeminal reflexes represents the most useful and reliable test for the neurophysiological diagnosis of trigeminal pains. The present article discusses different techniques for investigation of the trigeminal system by which an accurate topographical diagnosis and profile of sensory fiber pathology can be determined. With the aid of neurophysiological recordings and quantitative sensory testing, it is possible to approach a mechanism-based classification of orofacial pain.

Explicit and implicit motor learning in children with unilateral cerebral palsy.

PubMed

van der Kamp, John; Steenbergen, Bert; Masters, Rich S W

2017-07-30

The current study aimed to investigate the capacity for explicit and implicit learning in children with unilateral cerebral palsy. Children with left and right unilateral cerebral palsy and typically developing children shuffled disks toward a target. A prism-adaptation design was implemented, consisting of pre-exposure, prism exposure, and post-exposure phases. Half of the participants were instructed about the function of the prism glasses, while the other half were not. For each trial, the distance between the target and the shuffled disk was determined. Explicit learning was indicated by the rate of adaptation during the prism exposure phase, whereas implicit learning was indicated by the magnitude of the negative after-effect at the start of the post-exposure phase. Results No significant effects were revealed between typically developing participants and participants with unilateral cerebral palsy. Comparison of participants with left and right unilateral cerebral palsy demonstrated that participants with right unilateral cerebral palsy had a significantly lower rate of adaptation than participants with left unilateral cerebral palsy, but only when no instructions were provided. The magnitude of the negative after-effects did not differ significantly between participants with right and left unilateral cerebral palsy. The capacity for explicit motor learning is reduced among individuals with right unilateral cerebral palsy when accumulation of declarative knowledge is unguided (i.e., discovery learning). In contrast, the capacity for implicit learning appears to remain intact among individuals with left as well as right unilateral cerebral palsy. Implications for rehabilitation Implicit motor learning interventions are recommended for individuals with cerebral palsy, particularly for individuals with right unilateral cerebral palsy Explicit motor learning interventions for individual with cerebral palsy - if used - best consist of singular verbal instruction.

Acupuncture Treatment for Deafness: An Eyewitness Report

ERIC Educational Resources Information Center

Shimizu, Hiroshi

1974-01-01

It is reported that a professor of otolaryngology in Japan found no evidence that acupuncture improved sensorineural hearing impairment during observations of education and medical treatment of aurally handicapped children in mainland China. (MC)

The Verbotonal Method of Aural Rehabilitation: A Case Study

ERIC Educational Resources Information Center

Eisenberg, Diane; Santore, Frances

1976-01-01

A case study is presented of a 12-year-old child with a congenital profound bilateral sensori-neural hearing loss, who received rehabilitative audio-therapy according to the verbotonal method. (Author/LS)

Progressive sensorineural hearing loss, subjective tinnitus and vertigo caused by elevated blood lipids.

PubMed

Pulec, J L; Pulec, M B; Mendoza, I

1997-10-01

The otologist frequently sees patients with progressive sensorineural hearing loss, subjective aural tinnitus and vertigo with no apparent cause. Elevated blood lipids may be a cause of inner ear malfunction on a biochemical basis. To establish the true incidence of this condition, all new patients (4,251) seen during an eight-year period were evaluated; of these, 2,332 patients had complaints of inner ear disease. All had a complete neurotologic examination, appropriate audiometric and vestibular studies and imaging, and blood tests including lipid phenotype studies. Hyperlipoproteinemia was found in 120 patients (5.1%). Most patients were found to be overweight and had additional coexisting conditions such as diabetes mellitus. Treatment with vasodilators and a 500-calorie, high-protein, low-carbohydrate diet yielded improvement of symptoms in 83% of patients within five months of initiation of treatment.

Plasticity in the Developing Auditory Cortex: Evidence from Children with Sensorineural Hearing Loss and Auditory Neuropathy Spectrum Disorder

PubMed Central

Cardon, Garrett; Campbell, Julia; Sharma, Anu

2013-01-01

The developing auditory cortex is highly plastic. As such, the cortex is both primed to mature normally and at risk for re-organizing abnormally, depending upon numerous factors that determine central maturation. From a clinical perspective, at least two major components of development can be manipulated: 1) input to the cortex and 2) the timing of cortical input. Children with sensorineural hearing loss (SNHL) and auditory neuropathy spectrum disorder (ANSD) have provided a model of early deprivation of sensory input to the cortex, and demonstrated the resulting plasticity and development that can occur upon introduction of stimulation. In this article, we review several fundamental principles of cortical development and plasticity and discuss the clinical applications in children with SNHL and ANSD who receive intervention with hearing aids and/or cochlear implants. PMID:22668761

Speech-evoked auditory brainstem responses in children with hearing loss.

PubMed

Koravand, Amineh; Al Osman, Rida; Rivest, Véronique; Poulin, Catherine

2017-08-01

The main objective of the present study was to investigate subcortical auditory processing in children with sensorineural hearing loss. Auditory Brainstem Responses (ABRs) were recorded using click and speech/da/stimuli. Twenty-five children, aged 6-14 years old, participated in the study: 13 with normal hearing acuity and 12 with sensorineural hearing loss. No significant differences were observed for the click-evoked ABRs between normal hearing and hearing-impaired groups. For the speech-evoked ABRs, no significant differences were found for the latencies of the following responses between the two groups: onset (V and A), transition (C), one of the steady-state wave (F), and offset (O). However, the latency of the steady-state waves (D and E) was significantly longer for the hearing-impaired compared to the normal hearing group. Furthermore, the amplitude of the offset wave O and of the envelope frequency response (EFR) of the speech-evoked ABRs was significantly larger for the hearing-impaired compared to the normal hearing group. Results obtained from the speech-evoked ABRs suggest that children with a mild to moderately-severe sensorineural hearing loss have a specific pattern of subcortical auditory processing. Our results show differences for the speech-evoked ABRs in normal hearing children compared to hearing-impaired children. These results add to the body of the literature on how children with hearing loss process speech at the brainstem level. Copyright © 2017 Elsevier B.V. All rights reserved.

Diabetes mellitus and sensorineural hearing loss: is there an association? Baseline of the Brazilian Longitudinal Study of Adult Health (ELSA-Brasil).

PubMed

Samelli, Alessandra G; Santos, Itamar S; Moreira, Renata R; Rabelo, Camila M; Rolim, Laurie P; Bensenõr, Isabela J; Lotufo, Paulo A

2017-01-01

Although several studies have investigated the effects of diabetes on hearing loss, the relationship between these two conditions remains unclear. Some studies have suggested that diabetes may cause sensorineural hearing loss, whereas others have failed to find an association. The biggest challenge in investigating the association between diabetes and hearing loss is the presence of confounding variables and the complexity of the auditory system. Our study investigated the association between diabetes and sensorineural hearing loss. We evaluated the influence of time from diabetes diagnosis on this association after controlling for age, gender, and hypertension diagnosis and excluding those subjects with exposure to noise. This cross-sectional study evaluated 901 adult and elderly Brazilian Longitudinal Study of Adult Health (ELSA-Brasil) participants from São Paulo, Brazil who underwent audiometry testing as part of ELSA-Brasil's baseline assessment. Hearing thresholds and speech test results were significantly worse in the group with diabetes than in the group without diabetes. However, no significant differences were found between participants with and without diabetes after adjusting for age, gender, and the presence of hypertension. Hearing thresholds were not affected by occupational noise exposure in the groups with and without diabetes. In addition, no association between the duration of diabetes and hearing thresholds was observed after adjusting for age, gender, and hypertension. We found no association between the duration of diabetes and worse hearing thresholds after models were adjusted for age, gender, and the presence of hypertension.

[Etiological, clinical and neuroradiological investigation of deaf children with additional neuropsychiatric disabilities].

PubMed

Chilosi, A M; Scusa, M F; Comparini, A; Genovese, E; Forli, F; Berrettini, S; Cipriani, P

2012-04-01

Sensorineural hearing loss (SNHL) is complicated by additional disabilities in about 30% of cases, but the epidemiology of associated disorders, in terms of type, frequency and aetiology is still not clearly defined. Additional disabilities in a deaf child have important consequences in assessing and choosing a therapeutic treatment, in particular when considering cochlear implantation (CI) or hearing aids (HA). The aim of this paper was to evaluate frequency, type and severity of additional neurodevelopmental disabilities in children with profound bilateral sensorineural hearing loss and to investigate the relationship between disability and the etiology of deafness. Eighty children with profound bilateral sensorineural hearing loss (mean age 5.4 years) were investigated by means of a diagnostic protocol including clinical, neurodevelopmental, and audiological procedures together with genetic and neurometabolic tests and neuroradiological investigation by brain MRI. Fifty-five percent of the sample exhibited one or more disabilities in addition to deafness, with cognitive, behavioural-emotional and motor disorders being the most frequent. The risk of additional disabilities varied according to aetiology, with a higher incidence in hereditary syndromic deafness, in cases due to pre-perinatal pathology (in comparison to unknown and hereditary non syndromic forms) and in the presence of major brain abnormalities at MRI. Our results suggest that the aetiology of deafness may be a significant risk indicator for the presence of neuropsychiatric disorders. A multidimensional evaluation, including aetiological, neurodevelopmental and MRI investigation is needed for formulating prognosis and for planning therapeutic intervention, especially in those children candidated to cochlear implant.

Cochlear Homocysteine Metabolism at the Crossroad of Nutrition and Sensorineural Hearing Loss.

PubMed

Partearroyo, Teresa; Vallecillo, Néstor; Pajares, María A; Varela-Moreiras, Gregorio; Varela-Nieto, Isabel

2017-01-01

Hearing loss (HL) is one of the most common causes of disability, affecting 360 million people according to the World Health Organization (WHO). HL is most frequently of sensorineural origin, being caused by the irreversible loss of hair cells and/or spiral ganglion neurons. The etiology of sensorineural HL (SNHL) is multifactorial, with genetic and environmental factors such as noise, ototoxic substances and aging playing a role. The nutritional status is central in aging disability, but the interplay between nutrition and SNHL has only recently gained attention. Dietary supplementation could therefore constitute the first step for the prevention and potential repair of hearing damage before it reaches irreversibility. In this context, different epidemiological studies have shown correlations among the nutritional condition, increased total plasma homocysteine (tHcy) and SNHL. Several human genetic rare diseases are also associated with homocysteine (Hcy) metabolism and SNHL confirming this potential link. Accordingly, rodent experimental models have provided the molecular basis to understand the observed effects. Thus, increased tHcy levels and vitamin deficiencies, such as folic acid (FA), have been linked with SNHL, whereas long-term dietary supplementation with omega-3 fatty acids improved Hcy metabolism, cell survival and hearing acuity. Furthermore, pharmacological supplementations with the anti-oxidant fumaric acid that targets Hcy metabolism also improved SNHL. Overall these results strongly suggest that cochlear Hcy metabolism is a key player in the onset and progression of SNHL, opening the way for the design of prospective nutritional therapies.

Cochlear Homocysteine Metabolism at the Crossroad of Nutrition and Sensorineural Hearing Loss

PubMed Central

Partearroyo, Teresa; Vallecillo, Néstor; Pajares, María A.; Varela-Moreiras, Gregorio; Varela-Nieto, Isabel

2017-01-01

Hearing loss (HL) is one of the most common causes of disability, affecting 360 million people according to the World Health Organization (WHO). HL is most frequently of sensorineural origin, being caused by the irreversible loss of hair cells and/or spiral ganglion neurons. The etiology of sensorineural HL (SNHL) is multifactorial, with genetic and environmental factors such as noise, ototoxic substances and aging playing a role. The nutritional status is central in aging disability, but the interplay between nutrition and SNHL has only recently gained attention. Dietary supplementation could therefore constitute the first step for the prevention and potential repair of hearing damage before it reaches irreversibility. In this context, different epidemiological studies have shown correlations among the nutritional condition, increased total plasma homocysteine (tHcy) and SNHL. Several human genetic rare diseases are also associated with homocysteine (Hcy) metabolism and SNHL confirming this potential link. Accordingly, rodent experimental models have provided the molecular basis to understand the observed effects. Thus, increased tHcy levels and vitamin deficiencies, such as folic acid (FA), have been linked with SNHL, whereas long-term dietary supplementation with omega-3 fatty acids improved Hcy metabolism, cell survival and hearing acuity. Furthermore, pharmacological supplementations with the anti-oxidant fumaric acid that targets Hcy metabolism also improved SNHL. Overall these results strongly suggest that cochlear Hcy metabolism is a key player in the onset and progression of SNHL, opening the way for the design of prospective nutritional therapies. PMID:28487633

The role of alternative GJB2 transcription in screening for neonatal sensorineural deafness in Austria.

PubMed

Parzefall, Thomas; Lucas, Trevor; Koenighofer, Martin; Ramsebner, Reinhard; Frohne, Alexandra; Czeiger, Shelly; Baumgartner, Wolf-Dieter; Schoefer, Christian; Gstoettner, Wolfgang; Frei, Klemens

2017-04-01

Alterations within a novel putative Exon 1a within the gap junction beta 2 (GJB2) gene may play a role in the development of genetic hearing impairment in Austria. Mutations in the GJB2 gene are the most common cause of hereditary sensorineural deafness. Genome-wide screening for alternative transcriptional start sites in the human genome has revealed the presence of an additional GJB2 exon (E1a). This study tested the hypothesis of whether alternative GJB2 transcription involving E1a may play a role in the development of congenital sensorineural deafness in Austria. GJB2 E1a and flanking regions were sequenced in randomized normal hearing control subjects and three different patient groups with non-syndromic hearing impairment (NSHI), and bioinformatic analysis was performed. Statistical analysis of disease association was carried out using the Cochran-Armitage test for trend. A single change 2410 bp proximal to the translational start site (c.-2410T > C, rs7994748, NM_004004.5:c.-23 + 792T > C) was found to be significantly associated with the common c.35delG GJB2 mutation (p = .009). c.35delG in combination with c.-2410CC occurred at a 6.9-fold increased frequency compared to the control group. Additionally, one patient with idiopathic congenital hearing loss was found to be homozygous c.-2410CC.

The novel c.247_249delTTC (p.F83del) GJB2 mutation in a family with prelingual sensorineural deafness.

PubMed

Petersen, Michael B; Grigoriadou, Maria; Koutroumpe, Maria; Kokotas, Haris

2012-07-01

Non-syndromic hearing loss is one of the most common hereditary determined diseases in human, and the disease is a genetically heterogeneous disorder. Mutations in the GJB2 gene, encoding connexin 26 (Cx26), are a major cause of non-syndromic recessive hearing impairment in many countries and are largely dependent on ethnic groups. Due to the high frequency of the c.35delG GJB2 mutation in the Greek population, we have previously suggested that Greek patients with sensorineural, non-syndromic deafness should be tested for the c.35delG mutation and the coding region of the GJB2 gene should be sequenced in c.35delG heterozygotes. Here we present on the clinical and molecular genetic evaluation of a family suffering from prelingual, sensorineural, non-syndromic deafness. A novel c.247_249delTTC (p.F83del) GJB2 mutation was detected in compound heterozygosity with the c.35delG GJB2 mutation in the proband and was later confirmed in the father, while the mother was homozygous for the c.35delG GJB2 mutation. We conclude that compound heterozygosity of the novel c.247_249delTTC (p.F83del) and the c.35delG mutations in the GJB2 gene was the cause of deafness in the proband and his father. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

Sudden gains in cognitive therapy and interpersonal therapy for social anxiety disorder.

PubMed

Bohn, Christiane; Aderka, Idan M; Schreiber, Franziska; Stangier, Ulrich; Hofmann, Stefan G

2013-02-01

The present study examined the effects of sudden gains on treatment outcome in a randomized controlled trial including individual cognitive therapy (CT) and interpersonal therapy (IPT) for social anxiety disorder (SAD). Participants were 67 individuals with SAD who received 16 treatment sessions. Symptom severity at each session was assessed using the Social Phobia Weekly Summary Scale (Clark et al., 2003). Results indicate that 22.4% of participants experienced a sudden gain during treatment. Individuals with sudden gains had significantly lower social anxiety symptoms at post-treatment and follow-up compared to individuals without sudden gains. Sudden gains in CT and IPT had similar magnitudes, frequencies, and timings. However, sudden gains resulted in lower levels of post-treatment symptoms in CT compared to IPT. Cognitive changes did not precede sudden gains, but sudden gains resulted in cognitive changes. Sudden gains in CT and IPT for SAD are predictive of long-term outcome. In addition, the effect of sudden gains may be greater in CT compared to IPT.

Managing Hearing Loss | NIH MedlinePlus the Magazine

MedlinePlus

... certain medications, or long-term exposure to loud noises. Sensorineural hearing loss occurs when there is damage ... Many people may have a combination of both noise-induced hearing loss and hearing loss from aging. ...

The Relationship between Binaural Benefit and Difference in Unilateral Speech Recognition Performance for Bilateral Cochlear Implant Users

PubMed Central

Yoon, Yang-soo; Li, Yongxin; Kang, Hou-Yong; Fu, Qian-Jie

2011-01-01

Objective The full benefit of bilateral cochlear implants may depend on the unilateral performance with each device, the speech materials, processing ability of the user, and/or the listening environment. In this study, bilateral and unilateral speech performances were evaluated in terms of recognition of phonemes and sentences presented in quiet or in noise. Design Speech recognition was measured for unilateral left, unilateral right, and bilateral listening conditions; speech and noise were presented at 0° azimuth. The “binaural benefit” was defined as the difference between bilateral performance and unilateral performance with the better ear. Study Sample 9 adults with bilateral cochlear implants participated. Results On average, results showed a greater binaural benefit in noise than in quiet for all speech tests. More importantly, the binaural benefit was greater when unilateral performance was similar across ears. As the difference in unilateral performance between ears increased, the binaural advantage decreased; this functional relationship was observed across the different speech materials and noise levels even though there was substantial intra- and inter-subject variability. Conclusions The results indicate that subjects who show symmetry in speech recognition performance between implanted ears in general show a large binaural benefit. PMID:21696329

Father- and Mother-Adolescent Decision-Making in Mexican-Origin Families

PubMed Central

Perez-Brena, Norma; Updegraff, Kimberly A.; Umaña-Taylor, Adriana J.

2013-01-01

Understanding the prevalence and correlates of decisional autonomy within specific cultural contexts is necessary to fully understand how family processes are embedded within culture. The goals of this study were to describe mothers’ and fathers’ decision-making with adolescents (M = 12.51 years, SD = 0.58; 51% female), including parent-unilateral, joint, and youth-unilateral decision-making, and to examine the socio-cultural and family characteristics associated with these different types of decision-making in 246 Mexican-origin families. Mothers reported more joint and youth-unilateral decision-making and less parent-unilateral decision-making than did fathers. Fathers reported more youth-unilateral decision-making with sons than with daughters. Further, for mothers, more traditional gender role attitudes and higher levels of mother-adolescent conflict were associated with more parent-unilateral and less joint decision-making. In contrast, for fathers, lower levels of respect values were associated with more youth-unilateral decision-making with sons, and higher levels of parent-adolescent warmth was associated with more youth-unilateral decision-making with daughters. The importance of understanding the different correlates of mothers’ and fathers’ decision-making with sons versus daughters is discussed. PMID:21484288

Distraction of the temporomandibular joint condyle in patients with unilateral non-reducing disc displacement: Fact or fiction?

PubMed

Yıldız, Melih; Çağatay Dayan, Süleyman; Şakar, Olcay; Sülün, Tonguç

2017-07-24

This study investigated the distractive effect of a unilateral pivot splint on patients with unilateral disc displacement without reduction. The study group was comprised of 18 patients who had no history of treatment with removable prosthetic restorations of molars, premolars, or canine teeth, and no previous treatment for temporomandibular disorder. Joint spaces measurements made on magnetic resonance images indicated the affected side to be narrower than the healthy side. Unilateral distraction splints were made for all patients. An ultrasonic motion analyzer was used to measure the vertical shift occurring on the affected side as patients closed their mouths with maximal force with the splint in their mouths. Closing with maximal force on the unilateral distraction splint led to a noticeable downward movement of the affected condyle. The findings of this study indicate that the TMJ condyle of patients with unilateral disc displacement without reduction may be unilaterally distracted if the articular space is narrowed.

A virtual reality assessment and training system for unilateral neglect.

PubMed

Kim, Kwanguk; Kim, Jaehun; Ku, Jeonghun; Kim, Deog Young; Chang, Won Hyek; Shin, Dong Ik; Lee, Jang Han; Kim, In Young; Kim, Sun I

2004-12-01

Patients with unilateral neglect have problems reporting, responding, or orienting to novel or meaningful stimuli that is presented to the side opposite to that of a brain lesion. This creates a serous problem in regards to daily living activities. However, the established methods for assessing and training of unilateral neglect patients have several deficits. Recently, virtual reality (VR) technologies have been used as an assessment and treatment tool for rehabilitation. Hence, this study designed a VR system to assess and train unilateral neglect patients. In addition, the suitability and feasibility of our VR system for unilateral neglect patients was verified.

Investigating the Impact of Hearing Aid Use and Auditory Training on Cognition, Depressive Symptoms, and Social Interaction in Adults With Hearing Loss: Protocol for a Crossover Trial

PubMed Central

Meyer, Denny; Blamey, Peter J; Pipingas, Andrew; Bhar, Sunil

2018-01-01

Background Sensorineural hearing loss is the most common sensory deficit among older adults. Some of the psychosocial consequences of this condition include difficulty in understanding speech, depression, and social isolation. Studies have shown that older adults with hearing loss show some age-related cognitive decline. Hearing aids have been proven as successful interventions to alleviate sensorineural hearing loss. In addition to hearing aid use, the positive effects of auditory training—formal listening activities designed to optimize speech perception—are now being documented among adults with hearing loss who use hearing aids, especially new hearing aid users. Auditory training has also been shown to produce prolonged cognitive performance improvements. However, there is still little evidence to support the benefits of simultaneous hearing aid use and individualized face-to-face auditory training on cognitive performance in adults with hearing loss. Objective This study will investigate whether using hearing aids for the first time will improve the impact of individualized face-to-face auditory training on cognition, depression, and social interaction for adults with sensorineural hearing loss. The rationale for this study is based on the hypothesis that, in adults with sensorineural hearing loss, using hearing aids for the first time in combination with individualized face-to-face auditory training will be more effective for improving cognition, depressive symptoms, and social interaction rather than auditory training on its own. Methods This is a crossover trial targeting 40 men and women between 50 and 90 years of age with either mild or moderate symmetric sensorineural hearing loss. Consented, willing participants will be recruited from either an independent living accommodation or via a community database to undergo a 6-month intensive face-to-face auditory training program (active control). Participants will be assigned in random order to receive hearing aid (intervention) for either the first 3 or last 3 months of the 6-month auditory training program. Each participant will be tested at baseline, 3, and 6 months using a neuropsychological battery of computer-based cognitive assessments, together with a depression symptom instrument and a social interaction measure. The primary outcome will be cognitive performance with regard to spatial working memory. Secondary outcome measures include other cognition performance measures, depressive symptoms, social interaction, and hearing satisfaction. Results Data analysis is currently under way and the first results are expected to be submitted for publication in June 2018. Conclusions Results from the study will inform strategies for aural rehabilitation, hearing aid delivery, and future hearing loss intervention trials. Trial Registration ClinicalTrials.gov NCT03112850; https://clinicaltrials.gov/ct2/show/NCT03112850 (Archived by WebCite at http://www.webcitation.org/6xz12fD0B). PMID:29572201

Investigating the Impact of Hearing Aid Use and Auditory Training on Cognition, Depressive Symptoms, and Social Interaction in Adults With Hearing Loss: Protocol for a Crossover Trial.

PubMed

Nkyekyer, Joanna; Meyer, Denny; Blamey, Peter J; Pipingas, Andrew; Bhar, Sunil

2018-03-23

Sensorineural hearing loss is the most common sensory deficit among older adults. Some of the psychosocial consequences of this condition include difficulty in understanding speech, depression, and social isolation. Studies have shown that older adults with hearing loss show some age-related cognitive decline. Hearing aids have been proven as successful interventions to alleviate sensorineural hearing loss. In addition to hearing aid use, the positive effects of auditory training-formal listening activities designed to optimize speech perception-are now being documented among adults with hearing loss who use hearing aids, especially new hearing aid users. Auditory training has also been shown to produce prolonged cognitive performance improvements. However, there is still little evidence to support the benefits of simultaneous hearing aid use and individualized face-to-face auditory training on cognitive performance in adults with hearing loss. This study will investigate whether using hearing aids for the first time will improve the impact of individualized face-to-face auditory training on cognition, depression, and social interaction for adults with sensorineural hearing loss. The rationale for this study is based on the hypothesis that, in adults with sensorineural hearing loss, using hearing aids for the first time in combination with individualized face-to-face auditory training will be more effective for improving cognition, depressive symptoms, and social interaction rather than auditory training on its own. This is a crossover trial targeting 40 men and women between 50 and 90 years of age with either mild or moderate symmetric sensorineural hearing loss. Consented, willing participants will be recruited from either an independent living accommodation or via a community database to undergo a 6-month intensive face-to-face auditory training program (active control). Participants will be assigned in random order to receive hearing aid (intervention) for either the first 3 or last 3 months of the 6-month auditory training program. Each participant will be tested at baseline, 3, and 6 months using a neuropsychological battery of computer-based cognitive assessments, together with a depression symptom instrument and a social interaction measure. The primary outcome will be cognitive performance with regard to spatial working memory. Secondary outcome measures include other cognition performance measures, depressive symptoms, social interaction, and hearing satisfaction. Data analysis is currently under way and the first results are expected to be submitted for publication in June 2018. Results from the study will inform strategies for aural rehabilitation, hearing aid delivery, and future hearing loss intervention trials. ClinicalTrials.gov NCT03112850; https://clinicaltrials.gov/ct2/show/NCT03112850 (Archived by WebCite at http://www.webcitation.org/6xz12fD0B). ©Joanna Nkyekyer, Denny Meyer, Peter J Blamey, Andrew Pipingas, Sunil Bhar. Originally published in JMIR Research Protocols (http://www.researchprotocols.org), 23.03.2018.

Associations of Anisometropia with Unilateral Amblyopia, Interocular Acuity Difference and Stereoacuity in Preschoolers

PubMed Central

Ying, Gui-shuang; Huang, Jiayan; Maguire, Maureen; Quinn, Graham; Kulp, Marjean Taylor; Ciner, Elise; Cyert, Lynn; Orel-Bixler, Deborah

2012-01-01

Purpose To evaluate the relation of anisometropia with unilateral amblyopia, interocular acuity difference (IAD) and stereoacuity, among Head Start preschoolers, using both clinical notation and vector notation analyses. Design Multicenter, cross-sectional study. Participants 3- to 5-year-old participants in the Vision In Preschoolers (VIP) Study (N=4040). Methods Secondary analysis of VIP data from participants who had comprehensive eye examinations including monocular visual acuity (VA) testing, stereoacuity testing, and cycloplegic refraction. VA was retested with full cycloplegic correction when retest criteria were met. Unilateral amblyopia was defined as IAD ≥2 lines in logarithm of the Minimum Angle of Resolution (logMAR). Anisometropia was defined as ≥0.25 D (diopter) difference in spherical equivalent (SE) or in cylinder power, and also two approaches using power vector notation. The percentage with unilateral amblyopia, mean IAD, and mean stereoacuity were compared between anisometropic and isometropic children. Main Outcomes Measures The percentage with unilateral amblyopia, mean IAD, and mean stereoacuity. Results Compared with isometropic children, anisometropic children had a higher percentage of unilateral amblyopia (8% vs. 2%), larger mean IAD (0.07 vs. 0.05 logMAR) and worse mean stereoacuity (145 vs.117 arc sec) (all p<0.0001). Larger amounts of anisometropia were associated with higher percentages of unilateral amblyopia, larger IAD, and worse stereoacuity (trend p<0.001). Percentage of unilateral amblyopia was significantly increased with spherical equivalent (SE) anisometropia >0.5 D, cylindrical anisometropia >0.25 D, the vertical/horizontal meridian (J0) or oblique meridian (J45) >0.125 D, or vector dioptric distance (VDD) >0.35 D (all p<0.001). VDD had higher ability in detecting unilateral amblyopia than cylinder, SE, J0 and J45 (p<0.001). Conclusions The presence of and amount of anisometropia were associated with the presence of unilateral amblyopia, larger IAD and worse stereoacuity. The threshold level of anisometropia at which unilateral amblyopia becomes significant was lower than current guidelines. VDD is more accurate than spherical equivalent anisometropia or cylindrical anisometropia in identifying preschoolers with unilateral amblyopia. PMID:23174398

Are unilateral and bilateral patellar tendinopathy distinguished by differences in anthropometry, body composition, or muscle strength in elite female basketball players?

PubMed Central

Gaida, J; Cook, J; Bass, S; Austen, S; Kiss, Z

2004-01-01

Background: Overuse injury to the patellar tendon (patellar tendinopathy) is a major reason for interrupted training and competition for elite athletes. In both sexes, the prevalence of unilateral and bilateral tendinopathy has been shown to differ. It has been proposed that bilateral pathology may have a different aetiology from unilateral pathology. Investigation of risk factors that may be unique to unilateral and bilateral patellar tendinopathy in female athletes may reveal insights into the aetiology of this condition. Objectives: To examine whether anthropometry, body composition, or muscle strength distinguished elite female basketball players with unilateral or bilateral patellar tendinopathy. Methods: Body composition, anthropometry, and muscle strength were compared in elite female basketball players with unilateral (n = 8), bilateral (n = 7), or no (n = 24) patellar tendinopathy. Body composition was analysed using a dual energy x ray absorptiometer. Anthropometric measures were assessed using standard techniques. Knee extensor strength was measured at 180°/s using an isokinetic dynamometer. z scores were calculated for the unilateral and bilateral groups (using the no tendinopathy group as controls). z scores were tested against zero. Results: The tibia length to stature ratio was approximately 1.3 (1.3) SDs above zero in both the affected and non-affected legs in the unilateral group (p<0.05). The waist to hip ratio was 0.66 (0.78) SD above zero in the unilateral group (p<0.05). In the unilateral group, leg lean to total lean ratio was 0.42 (0.55) SD above zero (p<0.07), the trunk lean to total lean ratio was 0.63 (0.68) SD below zero (p<0.05), and leg fat relative to total fat was 0.47 (0.65) SD below zero (p<0.09). In the unilateral group, the leg with pathology was 0.78 (1.03) SD weaker during eccentric contractions (p<0.07). Conclusions: Unilateral patellar tendinopathy has identifiable risk factors whereas bilateral patellar tendinopathy may not. This suggests that the aetiology of these conditions may be different. However, interpretation must respect the limitation of small subject numbers. PMID:15388543

Unilateral versus bilateral neck exploration for primary hyperparathyroidism: a prospective randomized controlled trial.

PubMed

Bergenfelz, Anders; Lindblom, Pia; Tibblin, Sten; Westerdahl, Johan

2002-11-01

To compare unilateral and bilateral neck exploration for primary hyperparathyroidism in a prospective randomized controlled trial. Based on the assumption that unilateral neck exploration for a solitary parathyroid adenoma should reduce operating time and morbidity, a variety of minimally invasive procedures have challenged the idea that bilateral neck exploration is the gold standard for the surgical treatment of primary hyperparathyroidism. However, to date, no open prospective randomized trial has been published comparing unilateral and bilateral neck exploration. Ninety-one patients with the preoperative diagnosis of primary hyperparathyroidism were randomized to unilateral or bilateral neck exploration. Preoperative scintigraphy and intraoperative parathyroid hormone measurement guided the unilateral exploration. Gross morphology and frozen section determined the extent of parathyroid tissue resection in the bilateral group. The primary end-point was the use of postoperative medication for hypocalcemic symptoms. Eighty-eight patients (97%) were cured. Histology and cure rate did not differ between the two groups. Patients in the bilateral group consumed more oral calcium, had lower serum calcium values on postoperative days 1 to 4, and had a higher incidence of early severe symptomatic hypocalcemia compared with patients in the unilateral group. In addition, for patients undergoing surgery for a solitary parathyroid adenoma, unilateral exploration was associated with a shorter operative time. The cost for the two procedures did not differ. Patients undergoing a unilateral procedure had a lower incidence of biochemical and severe symptomatic hypocalcemia in the early postoperative period compared with patients undergoing bilateral exploration. Unilateral neck exploration with intraoperative parathyroid hormone assessment is a valid surgical strategy in patients with primary hyperparathyroidism with distinct advantages, especially for patients with solitary parathyroid adenoma.

Unilateral Versus Bilateral Neck Exploration for Primary Hyperparathyroidism

PubMed Central

Bergenfelz, Anders; Lindblom, Pia; Tibblin, Sten; Westerdahl, Johan

2002-01-01

Objective To compare unilateral and bilateral neck exploration for primary hyperparathyroidism in a prospective randomized controlled trial. Summary Background Data Based on the assumption that unilateral neck exploration for a solitary parathyroid adenoma should reduce operating time and morbidity, a variety of minimally invasive procedures have challenged the idea that bilateral neck exploration is the gold standard for the surgical treatment of primary hyperparathyroidism. However, to date, no open prospective randomized trial has been published comparing unilateral and bilateral neck exploration. Methods Ninety-one patients with the preoperative diagnosis of primary hyperparathyroidism were randomized to unilateral or bilateral neck exploration. Preoperative scintigraphy and intraoperative parathyroid hormone measurement guided the unilateral exploration. Gross morphology and frozen section determined the extent of parathyroid tissue resection in the bilateral group. The primary end-point was the use of postoperative medication for hypocalcemic symptoms. Results Eighty-eight patients (97%) were cured. Histology and cure rate did not differ between the two groups. Patients in the bilateral group consumed more oral calcium, had lower serum calcium values on postoperative days 1 to 4, and had a higher incidence of early severe symptomatic hypocalcemia compared with patients in the unilateral group. In addition, for patients undergoing surgery for a solitary parathyroid adenoma, unilateral exploration was associated with a shorter operative time. The cost for the two procedures did not differ. Conclusions Patients undergoing a unilateral procedure had a lower incidence of biochemical and severe symptomatic hypocalcemia in the early postoperative period compared with patients undergoing bilateral exploration. Unilateral neck exploration with intraoperative parathyroid hormone assessment is a valid surgical strategy in patients with primary hyperparathyroidism with distinct advantages, especially for patients with solitary parathyroid adenoma. PMID:12409657

Genetics Home Reference: sensorineural deafness and male infertility

MedlinePlus

... 502. Citation on PubMed Hildebrand MS, Avenarius MR, Smith RJH. CATSPER-Related Male Infertility. 2009 Dec 3 [ ... Bean LJH, Bird TD, Ledbetter N, Mefford HC, Smith RJH, Stephens K, editors. GeneReviews® [Internet]. Seattle (WA): ...

Tumor Necrosis Factor-induced Decrease of Cochlear Blood Flow Can Be Reversed by Etanercept or JTE-013.

PubMed

Sharaf, Kariem; Ihler, Friedrich; Bertlich, Mattis; Reichel, Christoph A; Berghaus, Alexander; Canis, Martin

2016-08-01

This study aimed to quantify the effects of tumor necrosis factor (TNF) inhibitor Etanercept and sphingosine-1-phosphate receptor 2 antagonist JTE-013 on cochlear blood flow in guinea pigs after TNF-induced decrease. Sudden sensorineural hearing loss is a common cause for disability and reduced quality of life. Good understanding of the pathophysiology and strong evidence-based therapy concepts are still missing. In various inner ear disorders, inflammation and impairment of cochlear blood flow (CBF) have been considered factors in the pathophysiology. A central mediator of inflammation and microcirculation in the cochlea is TNF. S1P acts downstream in one TNF pathway. Cochlea lateral wall vessels were exposed surgically and assessed by intravital microscopy in guinea pigs in vivo. Twenty-eight animals were randomly distributed into four groups of seven each. Exposed vessels were superfused by TNF (5.0 ng/ml) and afterward repeatedly either by Etanercept (1.0 μg/ml), JTE-013 (10 μmol/L), or vehicle (0.9 % NaCl solution or ethanol: phosphate-buffered saline buffer, respectively). After decreasing CBF with TNF (p <0.001, two-way RM ANOVA), both treatments reversed CBF, compared with vehicle (p <0.001, two-way RM ANOVA). The comparison of the vehicle groups showed no difference (p = 0.969, two-way RM ANOVA), while there was also no difference between the treatment groups (p = 0.850, two-way RM ANOVA). Both Etanercept and JTE-013 reverse the decreasing effect of TNF on cochlear blood flow and, therefore, TNF and the S1P-signalling pathway might be targets for treatment of microcirculation-related hearing loss.

Connexin 26 (GJB2) mutation in an Argentinean patient with keratitis-ichthyosis-deafness (KID) syndrome: a case report.

PubMed

Dalamón, Viviana Karina; Buonfiglio, Paula; Larralde, Margarita; Craig, Patricio; Lotersztein, Vanesa; Choate, Keith; Pallares, Norma; Diamante, Vicente; Elgoyhen, Ana Belén

2016-05-04

Keratitis-Ichthyosis-Deafness (KID) syndrome is a rare condition characterized by pre-lingual sensorineural deafness with skin hyperkeratinization. The primary cause of the disease is a loss-of-function mutation in the GJB2 gene. Mutations in Argentinean patients have not been described. We studied a 2 year-old boy with bilateral congenital sensorineural deafness with dry skin over the entire body, hypotrichosis of the scalp, thin and light-blond hair. Analysis of the GJB2 gene nucleotide sequence revealed the substitution of guanine-148 by adenine predicted to result in an Asp50Asn amino acid substitution. This is the first KID report in a patient from Argentina. This de novo mutation proved to be the cause of keratitis-ichthyosis-deafness syndrome (KID-syndrome) in the patient, and has implications in medical genetic practice.

Unilateral hippocampal inactivation or lesion selectively impairs remote contextual fear memory.

PubMed

Zhou, Heng; Zhou, Qixin; Xu, Lin

2016-10-01

Contextual fear memory depends on the hippocampus, but the role of unilateral hippocampus in this type of memory remains unclear. Herein, pharmacological inactivation or excitotoxic lesions were used to study the role of unilateral hippocampus in the stages of contextual fear memory. The pharmacological experiments revealed that compared with the control groups, unilateral hippocampal blockade did not impair 1-day recent memory following learning, whereas bilateral hippocampal blockade significantly impaired this memory. The lesion experiments showed that compared with the control groups, the formed contextual fear memory was retained for 7 days and that 30-day remote memory was markedly reduced in unilateral hippocampal lesion groups. These results indicate that an intact bilateral hippocampus is required for the formation of remote memory and that unilateral hippocampus is sufficient for recent contextual fear memory.

Importance of unilateral examination in olfactometry.

PubMed

Furukawa, M; Kamide, M; Miwa, T; Umeda, R

1988-01-01

Hyposmia, the decreased sense of smell, and anosmia, the loss of sense of smell, may be unilateral or bilateral. If the olfactory acuity examined by means of bilateral test is normal, olfactory disorders are not found; unilateral examination is therefore necessary for definite evaluation of olfactory acuity. As evidence, 7 cases out of 94 patients with chronic rhinosinusitis and 6 cases out of 12 patients who received the surgery of anterior cranial fossa showed definite different olfactory threshold between nasal cavities, and there were no patients who recognized the diminished sense of smell in spite of unilateral high olfactory threshold. Additionally, we have experienced that a patient with brain tumor was diagnosed by the help of unilateral olfactory test. We thus strongly recommend the unilateral olfactometry as a method for simple and reliable test in clinical measurement of the sense of smell.

[Characteristics of opening movement in patients with unilateral mastication].

PubMed

Jia, Ling; Wang, Yun; Wang, Mengya

2016-08-01

To analyze characteristics of mandibular movement in patients with unilateral mastication.
 Undergraduate students in oral medicine from Grade 2011 and 2012 in Wannan Medical College were enrolled for this study by cluster sampling method, which include 30 people with unilateral mastication and 30 people with bilateral mastication. The surface electromyogram (sEMG) of masseter muscle and anterovent of digastric muscle were recorded and the trajectory of mandibular incisor point was recorded simultaneously in the maximum opening and closing movement. The results were analyzed by SPSS 19.0 software.
 Average electrical peak of left anterior digastric muscle and right anterior digastric muscle in the unilateral chewing group was lower than that in the bilateral chewing group (P<0.05). The jaw tangent point trajectory was separate in the unilateral chewing group. There were significant differences at the opening type between the 2 groups. The vertical displacement and the sagittal displacement in the unilateral chewing group were significantly lower than those in the bilateral chewing group (P<0.01). There was significant positive correlation between the average peak potential of masseter muscle and displacement on the right side.
 Average electrical peak of left masseter muscle, left anterior digastric muscle, and right anterior digastric muscle decreases in the unilateral chewing group. Jaw tracking in most people deflects to the working side. Opening and closing jaw tracking is separate in 50% unilateral chewing individuals with the decreased opening degree. Unilateral chewing leads to changes in muscle performance accompanied by trajectory anomalies.

Unilateral retinitis pigmentosa sine pigmento.

PubMed Central

Pearlman, J T; Saxton, J; Hoffman, G

1976-01-01

A patient presented with unilateral findings of night blindness shown by impaired rod function and dark adaptation, constricted visual fields with good central acuity, a barely recordable electro-retinographic b-wave, and a unilaterally impaired electro-oculogram. There were none of the pigmentary changes usually associated with retinitis pigmentosa. The unaffected right eye was normal in all respects. Therefore the case is most probably one of unilateral retinitis pigmentosa sine pigmento. Images PMID:952804

Unilateral retinitis pigmentosa sine pigmento.

PubMed

Pearlman, J T; Saxton, J; Hoffman, G

1976-05-01

A patient presented with unilateral findings of night blindness shown by impaired rod function and dark adaptation, constricted visual fields with good central acuity, a barely recordable electro-retinographic b-wave, and a unilaterally impaired electro-oculogram. There were none of the pigmentary changes usually associated with retinitis pigmentosa. The unaffected right eye was normal in all respects. Therefore the case is most probably one of unilateral retinitis pigmentosa sine pigmento.

Sudden birth versus sudden death of entanglement for the extended Werner-like state in a dissipative environment

NASA Astrophysics Data System (ADS)

Shan, Chuan-Jia; Chen, Tao; Liu, Ji-Bing; Cheng, Wei-Wen; Liu, Tang-Kun; Huang, Yan-Xia; Li, Hong

2010-06-01

In this paper, we investigate the dynamical behaviour of entanglement in terms of concurrence in a bipartite system subjected to an external magnetic field under the action of dissipative environments in the extended Werner-like initial state. The interesting phenomenon of entanglement sudden death as well as sudden birth appears during the evolution process. We analyse in detail the effect of the purity of the initial entangled state of two qubits via Heisenberg XY interaction on the apparition time of entanglement sudden death and entanglement sudden birth. Furthermore, the conditions on the conversion of entanglement sudden death and entanglement sudden birth can be generalized when the initial entangled state is not pure. In particular, a critical purity of the initial mixed entangled state exists, above which entanglement sudden birth vanishes while entanglement sudden death appears. It is also noticed that stable entanglement, which is independent of different initial states of the qubits (pure or mixed state), occurs even in the presence of decoherence. These results arising from the combination of the extended Werner-like initial state and dissipative environments suggest an approach to control and enhance the entanglement even after purity induced sudden birth, death and revival.

Contact area affects frequency-dependent responses to vibration in the peripheral vascular and sensorineural systems.

PubMed

Krajnak, Kristine; Miller, G R; Waugh, Stacey

2018-01-01

Repetitive exposure to hand-transmitted vibration is associated with development of peripheral vascular and sensorineural dysfunctions. These disorders and symptoms associated with it are referred to as hand-arm vibration syndrome (HAVS). Although the symptoms of the disorder have been well characterized, the etiology and contribution of various exposure factors to development of the dysfunctions are not well understood. Previous studies performed using a rat-tail model of vibration demonstrated that vascular and peripheral nervous system adverse effects of vibration are frequency-dependent, with vibration frequencies at or near the resonant frequency producing the most severe injury. However, in these investigations, the amplitude of the exposed tissue was greater than amplitude typically noted in human fingers. To determine how contact with vibrating source and amplitude of the biodynamic response of the tissue affects the risk of injury occurring, this study compared the influence of frequency using different levels of restraint to assess how maintaining contact of the tail with vibrating source affects the transmission of vibration. Data demonstrated that for the most part, increasing the contact of the tail with the platform by restraining it with additional straps resulted in an enhancement in transmission of vibration signal and elevation in factors associated with vascular and peripheral nerve injury. In addition, there were also frequency-dependent effects, with exposure at 250 Hz generating greater effects than vibration at 62.5 Hz. These observations are consistent with studies in humans demonstrating that greater contact and exposure to frequencies near the resonant frequency pose the highest risk for generating peripheral vascular and sensorineural dysfunction.

Genetic studies in a patient with X-linked retinoschisis coexisting with developmental delay and sensorineural hearing loss.

PubMed

Sudha, Dhandayuthapani; Patric, Irene Rosita Pia; Ganapathy, Aparna; Agarwal, Smitha; Krishna, Shuba; Neriyanuri, Srividya; Sripriya, Sarangapani; Sen, Parveen; Chidambaram, Subbulakshmi; Arunachalam, Jayamuruga Pandian

2017-01-01

In this study, we present a juvenile retinoschisis patient with developmental delay, sensorineural hearing loss, and reduced axial tone. X-linked juvenile retinoschisis (XLRS) is a retinal dystrophy, most often not associated with systemic anomalies and also not showing any locus heterogeneity. Therefore it was of interest to understand the genetic basis of the condition in this patient. RS1 gene screening for XLRS was performed by Sanger sequencing. Whole genome SNP 6.0 array analysis was carried out to investigate gross chromosomal aberrations that could result in systemic phenotype. In addition, targeted next generation sequencing (NGS) was employed to determine any possible involvement of X-linked syndromic and non-syndromic mental retardation genes. This NGS panel consisted of 550 genes implicated in several other rare inherited diseases. RS1 gene screening revealed a pathogenic hemizygous splice site mutation (c.78+1G>T), inherited from the mother. SNP 6.0 array analysis did not indicate any significant chromosomal aberrations that could be disease-associated. Targeted resequencing did not identify any mutations in the X-linked mental retardation genes. However, variations in three other genes (NSD1, LARGE, and POLG) were detected, which were all inherited from the patient's unaffected father. Taken together, RS1 mutation was found to segregate with retinoschisis phenotype while none of the other identified variations were co-segregating with the systemic defects. Hereby, we infer that the multisystemic defects harbored by the patient are a rare coexistence of XLRS, developmental delay, sensorineural hearing loss, and reduced axial tone reported for the first time in the literature.

Ozone Prevents Cochlear Damage From Ischemia-Reperfusion Injury in Guinea Pigs.

PubMed

Onal, Merih; Elsurer, Cagdas; Selimoglu, Nebil; Yilmaz, Mustafa; Erdogan, Ender; Bengi Celik, Jale; Kal, Oznur; Onal, Ozkan

2017-08-01

The cochlea is an end organ, which is metabolically dependent on a nutrient and oxygen supply to maintain its normal physiological function. Cochlear ischemia and reperfusion (IR) injury is considered one of the most important causes of human idiopathic sudden sensorineural hearing loss. The aim of the present study was to study the efficacy of ozone therapy against cochlear damage caused by IR injury and to investigate the potential clinical use of this treatment for sudden deafness. Twenty-eight guinea pigs were randomized into four groups. The sham group (S) (n = 7) was administered physiological saline intraperitoneally (i.p.) for 7 days. The ozone group (O) (n = 7) was administered 1 mg/kg of ozone i.p. for 7 days. In the IR + O group (n = 7), 1 mg/kg of ozone was administered i.p. for 7 days before IR injury. On the eighth day, the IR + O group was subjected to cochlear ischemia for 15 min by occluding the bilateral vertebral artery and vein with a nontraumatic clamp and then reperfusion for 2 h. The IR group was subjected to cochlear IR injury. After the IR procedure, the guinea pigs were sacrificed on the same day. In a general histological evaluation, cochlear and spiral ganglionic tissues were examined with a light microscope, and apoptotic cells were counted by terminal deoxynucleotidyl transferase dUTP nick end labeling (TUNEL) staining. The apoptotic index (AI) was then calculated. Blood samples were sent for analyses of superoxide dismutase (SOD), glutathione peroxidase (GSH-Px), catalase, malondialdehyde (MDA), the total oxidant score (TOS), and total antioxidant capacity (TAC). Data were evaluated statistically using the Kruskal-Wallis test. The AI was highest in the IR group. The AI of the IR + O group was lower than that of the IR group. The biochemical antioxidant parameters SOD and GSH-Px and the TAC values were highest in the O group and lowest in the IR group. The MDA level and TOS were highest in the IR group and lowest in the O group. Controlled ozone administration stimulated endogenous antioxidant defense systems, thereby helping the body to combat IR injury. Although this study revealed a statistically significant decrease in cochlear IR damage following ozone therapy, further studies will be necessary to explain the protective mechanisms of ozone therapy in cochlear IR injury. © 2017 International Center for Artificial Organs and Transplantation and Wiley Periodicals, Inc.

Effects of bilateral and unilateral locus coeruleus lesions on beam-walking recovery after subsequent unilateral sensorimotor cortex suction-ablation in the rat.

PubMed

Goldstein, L B

1997-01-01

The recovery of beam-walking ability following a unilateral sensorimotor cortex lesion in the rat is hypothesized to be noradrenergically-mediated. We carried out two experiments to further test this hypothesis. In the first experiment, bilateral 6-hydroxydopamine locus coeruleus (LC) lesions or sham LC lesions were made 2 weeks prior to a right sensorimotor cortex suction-ablation lesion or sham cortex lesion. In the second experiment, unilateral left or right LC lesions or sham LC lesions were made 2 weeks prior to a right sensorimotor cortex lesion or sham cortex lesion. Beam-walking recovery was measured over the 12 days following cortex lesioning in each experiment. Bilateral, unilateral left, and unilateral right LC lesions resulted in impaired recovery. These data provide additional support for the hypothesis that beam-walking recovery after sensorimotor cortex injury is, at least in part, noradrenergically mediated.

Metastatic carcinoma of the breast: a case with the unusual presentation of unilateral periorbital edema.

PubMed

Kuo, Shu-Chen; Hsiao, Shih-Chuan; Chiou, Chien-Chun; Chen, Fen-Fen; Huang, Kuo-Chan

2008-01-01

Metastasis of mammary cancer involving the orbit is common, and it typically presents with enophthalmos and restrictions of ocular motility. We report a case of mammary cancer with the unusual presentation of unilateral periorbital edema only. The possible mechanisms of unilateral periorbital swelling are discussed. Metastasis of breast cancer involving unilateral eyelid edema was diagnosed in a 66-year-old woman. A biopsy was performed to confirm the etiology after vague neuroimaging findings. The diagnosis was based on the histopathologic features of carcinomatous cells in the excised specimen. Insidious, progressive unilateral upper and lower eyelid swelling of the right eye disappeared after one cycle of palliative chemotherapy. Neither restriction nor proptosis developed in the whole course. Possible metastasis should be considered as a possible etiology of unilateral eyelid edema, even without a palpable mass or limitation of ocular motility. A biopsy should be performed in cases of unexplained eyelid edema.

Sudden gains in exposure therapy for obsessive-compulsive disorder.

PubMed

Collins, Lindsey M; Coles, Meredith E

2017-06-01

Prior research in the treatment of depression and anxiety has demonstrated that a sudden reduction in symptoms between two consecutive sessions (sudden gain) is related to lower post-treatment symptom severity (e.g. Hofmann, Schulz, Meuret, Moscovitch, & Suvak, 2006; Tang & DeRubeis, 1999). However, only one study has examined sudden gains in the treatment of obsessive compulsive disorder (OCD). In that study, one-third of the patients with OCD experienced a sudden gain (Aderka et al., 2012). Further, patients who had a sudden gain had lower clinician-rated OCD symptom severity post-treatment (Aderka et al., 2012). In replication, the current study examined the frequency, characteristics, and clinical impact of sudden gains in 27 OCD patients during exposure and response prevention (ERP) therapy. Fifty two percent of patients experienced a sudden gain. The mean magnitude of a sudden gain represented, on average, 61.4% of total symptom reduction. Following treatment, individuals who had experienced a sudden gain were rated as less severe on the clinical global impression scale, but they did not experience a greater reduction in OCD symptoms (pre-to post-treatment) than those without a sudden gain. None of the pre-treatment characteristics tested were found to significantly predict whether a patient would have a sudden gain. Additional research examining predictors of, and patterns of, change in OCD symptoms is warranted. Copyright © 2017 Elsevier Ltd. All rights reserved.

A comparison of the brainstem auditory evoked response in healthy ears of unilaterally deaf dogs and bilaterally hearing dogs.

PubMed

Plonek, M; Nicpoń, J; Kubiak, K; Wrzosek, M

2017-03-01

Auditory plasticity in response to unilateral deafness has been reported in various animal species. Subcortical changes occurring in unilaterally deaf young dogs using the brainstem auditory evoked response have not been evaluated yet. The aim of this study was to assess the brainstem auditory evoked response findings in dogs with unilateral hearing loss, and compare them with recordings obtained from healthy dogs. Brainstem auditory evoked responses (amplitudes and latencies of waves I, II, III, V, the V/I wave amplitude ratio, wave I-V, I-III and III-V interpeak intervals) were studied retrospectively in forty-six privately owned dogs, which were either unilaterally deaf or had bilateral hearing. The data obtained from the hearing ears in unilaterally deaf dogs were compared to values obtained from their healthy littermates. Statistically significant differences in the amplitude of wave III and the V/I wave amplitude ratio at 75 dB nHL were found between the group of unilaterally deaf puppies and the control group. The recordings of dogs with single-sided deafness were compared, and the results showed no statistically significant differences in the latencies and amplitudes of the waves between left- (AL) and right-sided (AR) deafness. The recordings of the brainstem auditory evoked response in canines with unilateral inborn deafness in this study varied compared to recordings from healthy dogs. Future studies looking into electrophysiological assessment of hearing in conjunction with imaging modalities to determine subcortical auditory plasticity and auditory lateralization in unilaterally deaf dogs are warranted.

The Role of Unilateral Balloon Kyphoplasty for the Treatment of Patients with OVCFS: A Systematic Review and Meta-Analysis.

PubMed

Xiang, Guang-Heng; Tong, Min-Ji; Lou, Chao; Zhu, Si-Pin; Guo, Wei Jun; Ke, Chen Rong

2018-05-01

An increasing number of studies have been conducted to apply unilateral balloon kyphoplasty in the treatment of ostroporotic vertebral compression fractures (OVCFs). However, the efficacy and safety of unilateral kyphoplasty and whether a unilateral or a bilateral approach is superior is controversial. The purpose of this study was to evaluate the role of unilateral balloon kyphoplasty and use meta-analysis to compare the efficacy and safety of unilateral and bilateral kyphoplasty in patients with OVCFs. A systematic literature search was conducted from 1970 to April 2017 using Medline database and the Cochrane Central Register of Controlled Trials. Articles were limited to those published in English. Randomized controlled trials and nonrandomized comparative studies were also included. The following search terms were used: "osteoporotic vertebral compression fractures," or "OVCF," and "unilateral kyphoplasty," or "unipedicular approach," or "single balloon kyphoplasty," or "one balloon kyphoplasty." A comprehensive search of reference lists of retrieved articles and previous published reviews was also performed to ensure inclusion of all possible studies. All potential articles were independently reviewed by 2 investigators for inclusion into the final analysis. MINORS score was used for nonrandomized studies, and Detsky quality index was applied for prospective randomized controlled trials. Systematic review and meta-analysis was performed for the included studies. After unilateral balloon kyphoplasty the mean postoperative visual analog score (VAS) was from 1.74 to 4.77, mean postoperative kyphotic angle was from 5.9º to 11.22º, and complications involving cement leaks was from 6.8 to 21.9% or adjacent level fractures was from 0 to 5.6%). Unilateral kyphoplasty had significantly lower operative time, and less bone cement volume; however, the postoperative VAS, Oswestry Disability Index (ODI), vertebral height restoration rate, and cement leakage and adjacent vertebral fracture rate, were similar to bilateral kyphoplasty. Only 6 randomized controlled trials and 3 retrospective comparative studies were selected for analysis. Heterogeneity was detected among the studies when we pooled the outcomes. Based on the available evidence, the clinical and radiological results of unilateral balloon kyphoplasty were as good as those of bilateral balloon kyphoplasty for the treatment of OVCFs. And unilateral kyphoplasty had advantages in terms of operation time, radiation exposure, and cost. Unilateral balloon kyphoplasty, bilateral balloon kyphoplasty, osteoporotic vertebral compression fractures, complications of balloon kyphoplasty, meta-analysis.

Unilateral retinitis pigmentosa. A case report.

PubMed

Nazar, C; Feldman, M; González, R; Espinoza, R

2017-06-01

A 27-year-old woman with a history of nyctalopia and constriction of visual field of the right eye. The ophthalmological examination showed a visual field and electroretinogram that were compatible with unilateral retinitis pigmentosa (RP). After a one year follow-up, the unilateral condition remained. Unilateral retinitis pigmentosa is a rare condition, with a frequency between 0.2%-5% of the RP. It mainly affects women and older age groups than bilateral RP. For a definitive diagnosis, it is necessary to have a funduscopy and electroretinogram (ERG) altered unilaterally, and exclude infectious, inflammatory, and vascular causes. Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

Progressive Hearing Impairment in Children with Congenital Cytomegalovirus Infection.

ERIC Educational Resources Information Center

Dahle, Arthur J.; And Others

1979-01-01

Audiological assessment of 86 children (mean age 38 months at last evaluation time) with congenital cytomegalovirus infection revealed progressive hearing loss in four of 12 Ss with sensorineural hearing impairments. Case descriptions documented the progression of the hearing loss. (Author)

A Psychophysical Evaluation of Spectral Enhancement

ERIC Educational Resources Information Center

DiGiovanni, Jeffrey J.; Nelson, Peggy B.; Schlauch, Robert S.

2005-01-01

Listeners with sensorineural hearing loss have well-documented elevated hearing thresholds; reduced auditory dynamic ranges; and reduced spectral (or frequency) resolution that may reduce speech intelligibility, especially in the presence of competing sounds. Amplification and amplitude compression partially compensate for elevated thresholds and…

Visual outcome and impact on quality of life after surgeries differ in children operated for unilateral and bilateral cataract (Pune study 2011)

PubMed Central

Paryani, Mukesh; Khandekar, Rajiv B.; Dole, Kuldeep; Dharmadhikari, Sheetal; Rishikeshi, Nikhil

2012-01-01

Background: We compared vision and quality of life (VQL) of children aged 5-15 years and operated for unilateral and bilateral cataract between 2008 and 2010 in western India. Materials and Methods: In this cohort study, ophthalmologists assessed vision, anterior and posterior segment of eyes with cataract. Children completed a functional vision questionnaire (LVP-FVQ). Follow up at 6 months after surgery included the best corrected visual acuity (BCVA), FVQ and eye assessment. The improvement of BCVA and quality of life were compared in group of unilateral and bilateral cataract. Result: A total of 20 (70%) bilateral and 7 (39%) unilateral cataract were operated within 1 month of detection. All 48 eyes with bilateral cataract were congenital and 12 (67%) unilateral cataract were traumatic. Among bilateral group, 27 eyes [56.2% (95% confidence interval (CI) 44.4-72.2)] and in unilateral group 11 eyes [61.1% (95% CI 38.6-83.6)] had vision ≥ 20/60 at 6 months follow up. The visual gain was significantly higher in children who were operated between 1 month and 1 year of detection (adjusted Odds ratio (OR) = 15.6 P = 0.03). Positive impact on VQL in bilateral group was noted in 50%, 27%, and 13% children for subscale of distant vision, near vision, and field of vision, respectively. There was positive impact in these subscales among children with unilateral cataract. Thirty percent eyes with bilateral cataract and 22% of eyes with unilateral cataract improved their vision. Surgery within 1 month of cataract was significant predictor of improved vision (OR = 16.6 P = 0.02). Conclusion: Vision and VQL improved in children with unilateral and bilateral cataract. However, it was better 6 months following surgery in children with bilateral cataract than in children with unilateral cataract. PMID:23439722

The Clinical and Service Outcomes of Unilateral and Bilateral ECT Electrode Placements in Australian Aged Psychiatry Services.

PubMed

D'Cunha, Craig; Plakiotis, Christos; Macfarlane, Stephen; Moss, Francine; Reddy, Murali; Singh, Dhiren; Tofler, David; White, Erica; O'Connor, Daniel W

2016-03-01

The aim of the study was to determine whether depressed aged inpatients treated with brief pulse unilateral electroconvulsive therapy (ECT) differed from those treated with bilateral (bitemporal or bifrontal) ECT with respect to numbers of treatments, length of hospital admission, changes in scores on depression and cognitive scales, and serious adverse effects. An audit of routinely collected data regarding 221 acute ECT courses in 7 public aged psychiatry services in Victoria, Australia. Patients given unilateral, bifrontal, and bitemporal treatments were similar with respect to personal, clinical, and treatment characteristics. Most treatments were administered in line with local clinical guidelines and were rated as effective. Psychiatrists preferred unilateral ECT in the first instance with stimulus dosing based on patients' seizure thresholds. Approximately a quarter of unilateral courses were switched later to bitemporal placement, most probably because of insufficient progress. Bilateral treatments were associated with a larger number of treatments, less improvement in scores on mood and cognitive scales, and more refusals to continue treatment than unilateral-only ECT. Brief pulse unilateral ECT proved more effective than bitemporal and bifrontal ECT for most aged patients, especially when coupled with stimulus dosing based on seizure threshold.

Unilateral spinal anesthesia using low-flow injection through a 29-gauge Quincke needle.

PubMed

Meyer, J; Enk, D; Penner, M

1996-06-01

Restriction of sympathetic denervation during spinal anesthesia may minimize hemodynamic alterations. Theoretically, the use of nonisobaric anesthetics may allow unilateral anesthesia and thus restrict sympathetic denervation to one side of the body. The present prospective study investigates the incidence of unilateral spinal anesthesia using hyperbaric bupivacaine 0.5% (1.4 mL, 1.6 mL, 1.8 mL, or 2.0 mL) injected via a 29-gauge Quincke needle with a pump-controlled injection flow of 1 mL/min. In 96 consecutive patients undergoing unilateral surgery of the lower extremities, spinal anesthesia was performed in the lateral decubitus position, which was maintained for 20 min postinjection. Increases in foot temperature of at least 0.5 degrees C were defined as sympathetic blockade. The incidence of unilateral block was not significantly influenced by the amount of bupivacaine. For all 96 patients, the incidence of unilateral sympathetic and complete motor block was 69% and 77%, respectively. Frequency of unilateral sensory block (assessed by pinprick and temperature discrimination) was significantly lower (28%). Strict unilateral spinal anesthesia was achieved in 24 cases (25%). Twenty minutes after injection of the local anesthetic, mean arterial blood pressure decreased significantly in patients with bilateral sympathetic blockade from 87 +/- 8 to 83 +/- 8 mm Hg (P < 0.01) but not in patients with unilateral sympathetic blockade (from 87 +/- 11 to 85 +/- 10 mm Hg). In conclusion, low-flow injection (1 mL/min) of hyperbaric bupivacaine 0.5% via a 29-gauge Quincke needle prevented bilateral sympathetic blockade in more than 69% of the patients. The data further suggest that loss of temperature discrimination alone is not a reliable estimation of sympathetic block.

Effect of unilateral and bilateral use of laterally wedged insoles with arch supports on impact loading in medial knee osteoarthritis.

PubMed

Abd El Megeid Abdallah, Amira Abdallah

2016-04-01

Increased impact loading is implicated in knee osteoarthritis development and progression. This study examined the impact ground reaction force (GRF) peak, its loading rate, its relative timing to stance phase timing, and walking speed during unilateral and bilateral use of laterally wedged insoles with arch supports. Within-subject design. Thirty-three female patients with medial knee osteoarthritis were examined with (unilateral 6° and 11°, and bilateral 0°, 6°, and 11°) and without insole use. Repeated measures MANOVA revealed that the impact force increased significantly in bilateral 11° versus unilateral 6° and without-insole conditions. The loading rate decreased significantly in unilateral 11° versus bilateral 6° insoles. The relative timing increased significantly in each of bilateral 6°, bilateral 11°, and unilateral 11° versus bilateral 0° insoles and in each of bilateral 11° and unilateral 11° versus without-insole condition. There were significant positive correlations between the walking speed and each of the force and loading rate. The Chi-square test revealed insignificant association between the insole condition and the presence of impact forces. Unilateral 11° insoles are capable of reducing impact loading possibly through increasing foot pronation. Walking slowly is another possible strategy to reduce loading. Unilaterally applied 11° laterally wedged insoles are capable of reducing and delaying the initial impact ground reaction forces and reducing their loading rates during walking in patients with medial knee osteoarthritis, thus reducing osteoarthritis progression. Walking slowly could also be used as a strategy to reduce impact loading. © The International Society for Prosthetics and Orthotics 2015.

Unilateral versus bilateral neck exploration for primary hyperparathyroidism: five-year follow-up of a randomized controlled trial.

PubMed

Westerdahl, Johan; Bergenfelz, Anders

2007-12-01

To compare long-term patient outcome in a prospective randomized controlled trial between unilateral and bilateral neck exploration for primary hyperparathyroidism (pHPT). Minimal invasive and/or focused parathyroidectomy has challenged the traditional bilateral neck exploration for pHPT. Between 1997 and 2001, we conducted the first unselected randomized controlled trial of unilateral versus bilateral neck exploration for pHPT. The results showed that unilateral exploration is a surgical strategy with distinct advantages in the early postoperative period. However, concerns have been raised that limited parathyroid exploration could increase the risk for recurrent pHPT during long-term follow-up. Ninety-one patients with the diagnosis of pHPT were randomized to unilateral or bilateral neck exploration. Preoperative scintigraphy and intraoperative parathyroid hormone measurement guided the unilateral exploration. Gross morphology and frozen section determined the extent of parathyroid tissue resection in the bilateral group. Follow-up was performed after 6 weeks, 1 year, and 5 years postoperatively. Seventy-one patients were available for 5-year follow-up. There were no differences in serum ionized calcium and parathyroid hormone, respectively, between patients in the unilateral and bilateral group. Overall 6 patients have been found to have persistent (n = 3) or recurrent (n = 3) pHPT; 4 patients in the unilateral group (3 of these 4 patients were bilaterally explored) and 2 patients in the bilateral group. Three of 6 failures were unexpectedly found to have multiple endocrine neoplasia mutations. One patient with solitary adenoma in the bilateral group still required vitamin D substitution 5 years after surgery. Unilateral neck exploration with intraoperative parathyroid hormone assessment provides the same long-term results as bilateral neck exploration, and is thus a valid strategy for the surgical treatment of pHPT.

Sudden gains in group cognitive-behavioral therapy for panic disorder.

PubMed

Clerkin, Elise M; Teachman, Bethany A; Smith-Janik, Shannan B

2008-11-01

The current study investigates sudden gains (rapid symptom reduction) in group cognitive-behavioral therapy for panic disorder. Sudden gains occurring after session 2 of treatment predicted overall symptom reduction at treatment termination and some changes in cognitive biases. Meanwhile, sudden gains occurring immediately following session 1 were not associated with symptom reduction or cognitive change. Together, this research points to the importance of examining sudden gains across the entire span of treatment, as well as the potential role of sudden gains in recovery from panic disorder.

Sudden Gains in Group Cognitive-Behavioral Therapy for Panic Disorder

PubMed Central

Clerkin, Elise M.; Teachman, Bethany A.; Smith-Janik, Shannan B.

2008-01-01

The current study investigates sudden gains (rapid symptom reduction) in group cognitive-behavioral therapy for panic disorder. Sudden gains occurring after session 2 of treatment predicted overall symptom reduction at treatment termination and some changes in cognitive biases. Meanwhile, sudden gains occurring immediately following session 1 were not associated with symptom reduction or cognitive change. Together, this research points to the importance of examining sudden gains across the entire span of treatment, as well as the potential role of sudden gains in recovery from panic disorder. PMID:18804199

Eight indicators of unilateral pregnancy.

PubMed

Melchionne, Kevin

2010-12-01

Unintended pregnancy often leads to undesirable outcomes for both mothers and children. However, the definition of unintended pregnancy in the sociology of family formation has been restricted to the intentions of mothers. The intentions of fathers--and, with them, the possible role of disagreement about pregnancy intention--remain outside most conceptual frameworks and research programs. This article draws together a number of indicators of unilateral pregnancy in research on contemporary family formation in the United States. Studies of pregnancy intendedness and contraceptive use consistently provide evidence suggesting a significant role for unilateral pregnancy in family formation. Working on the assumption that unilateral pregnancy presents great potential for social dislocation, this article argues for the integration of the concept of unilateral pregnancy into the theoretical framework informing research on family formation.

Sudden Death in Young People--Heart Problems Often Blamed

MedlinePlus

Sudden death in young people: Heart problems often blamed Sudden death in young people is rare, but those at ... causes and treatments. By Mayo Clinic Staff Sudden death in people younger than 35, often due to ...

Geomagnetic sudden impulses and storm sudden commencements - A note on terminology

NASA Technical Reports Server (NTRS)

Joselyn, J. A.; Tsurutani, B. T.

1990-01-01

The definitions of and distinctions between storm sudden commencements (SSCs) and geomagnetic sudden impulses (SIs) are examined and present definitions of SIs and SSCs are modernized. Quantitative definitions of the two terms are recommended.

The roles of amensalistic and commensalistic interactions in large ecological network stability

PubMed Central

Mougi, Akihiko

2016-01-01

Ecological communities comprise diverse species and their interactions. Notably, ecological and evolutionary studies have revealed that reciprocal interactions such as predator–prey, competition, and mutualism, are key drivers of community dynamics. However, there is an argument that many species interactions are asymmetric, where one species unilaterally affects another species (amensalism or commensalism). This raises the unanswered question of what is the role of unilateral interactions in community dynamics. Here I use a theoretical approach to demonstrate that unilateral interactions greatly enhance community stability. The results suggested that amensalism and commensalism were more stabilizing than symmetrical interactions, such as competition and mutualism, but they were less stabilizing than an asymmetric antagonistic interaction. A mix of unilateral interactions increased stability. Furthermore, in communities with all interaction types, unilateral interactions tended to increase stability. This study suggests that unilateral interactions play a major role in maintaining communities, underlining the need to further investigate their roles in ecosystem dynamics. PMID:27406267

Look over there! Unilateral gaze increases geographical memory of the 50 United States.

PubMed

Propper, Ruth E; Brunyé, Tad T; Christman, Stephen D; Januszewskia, Ashley

2012-02-01

Based on their specialized processing abilities, the left and right hemispheres of the brain may not contribute equally to recall of general world knowledge. US college students recalled the verbal names and spatial locations of the 50 US states while sustaining leftward or rightward unilateral gaze, a procedure that selectively activates the contralateral hemisphere. Compared to a no-unilateral gaze control, right gaze/left hemisphere activation resulted in better recall, demonstrating left hemisphere superiority in recall of general world knowledge and offering equivocal support for the hemispheric encoding asymmetry model of memory. Unilateral gaze- regardless of direction- improved recall of spatial, but not verbal, information. Future research could investigate the conditions under which unilateral gaze increases recall. Sustained unilateral gaze can be used as a simple, inexpensive, means for testing theories of hemispheric specialization of cognitive functions. Results support an overall deficit in US geographical knowledge in undergraduate college students. Copyright © 2011 Elsevier Inc. All rights reserved.

Comparative study of unilateral versus bilateral inferior oblique recession/anteriorization in unilateral inferior oblique overaction.

PubMed

Mostafa, Attiat M; Kassem, Rehab R

2018-05-01

To compare the effect of, and the rate of subsequent development of iatrogenic antielevation syndrome after, unilateral versus bilateral inferior oblique graded recession-anteriorization to treat unilateral inferior oblique overaction. Thirty-four patients with unilateral inferior oblique overaction were included in a randomized prospective study. Patients were equally divided into 2 groups. Group UNI underwent unilateral, group BI bilateral, inferior oblique graded recession-anteriorization. A successful outcome was defined as orthotropia, or within 2 ∆ of a residual hypertropia, in the absence of signs of antielevation syndrome, residual inferior oblique overaction, V-pattern, dissociated vertical deviation, or ocular torticollis. A successful outcome was achieved in 11 (64.7%) and 13 (76.5%) patients in groups UNI and BI, respectively (p = 0.452). Antielevation syndrome was diagnosed as the cause of surgical failure in 6 (35.3%) and 2 (11.8%) patients, in groups UNI and BI, respectively (p = 0.106). The cause of surgical failure in the other 2 patients in group BI was due to persistence of ocular torticollis and hypertropia in a patient with superior oblique palsy and a residual V-pattern and hypertropia in the other patient. The differences between unilateral and bilateral inferior oblique graded recession-anteriorization are insignificant. Unilateral surgery has a higher tendency for the subsequent development of antielevation syndrome. Bilateral surgery may still become complicated by antielevation syndrome, although at a lower rate. In addition, bilateral surgery had a higher rate of undercorrection. Further studies on a larger sample are encouraged.

Survival in 76 cats with epilepsy of unknown cause: a retrospective study

PubMed Central

Szelecsenyi, A.; Giger, U.; Golini, L.; Mothersill, I.; Torgerson, P. R.; Steffen, F.

2017-01-01

Survival of cats with epilepsy of unknown cause (EUC) has not been reported. Seizure semiology and its relationship to treatment outcome and survival was studied in a population of 76 cats. A questionnaire for seizure semiology was developed based upon experimental data. Seizure semiology was characterized by owner interviews at least one year after discharge. Seizures were classified as: (1) primary generalized and (2) focal without and (3) with secondary generalization. Median age at seizure onset was four (range 0.3 to 18) years. One third of cats with EUC presented with primary generalized seizures and 78% of those with initially focal seizures progressed to secondary generalized seizures. Clinical signs of generalized seizures included sudden onset of loss of consciousness and tonic-clonic seizures, while cats with focal seizures had unilateral signs. Antiepileptic drug (AED) therapy was initiated in 62 cats. Complete remission rate was 42% and median survival time was 3.2 (range 1 to 11) years with or without AED, and 91% were still alive at the time of interview. Neither semiology nor seizure type predicted survival, response to treatment, and outcome in cats with EUC. A seizure-free status of >12 months was observed in 79% of cats without AED. PMID:29097567

Static and dynamic balance of children and adolescents with sensorineural hearing loss.

PubMed

Melo, Renato de Souza; Marinho, Sônia Elvira Dos Santos; Freire, Maryelly Evelly Araújo; Souza, Robson Arruda; Damasceno, Hélio Anderson Melo; Raposo, Maria Cristina Falcão

2017-01-01

To assess the static and dynamic balance performance of students with normal hearing and with sensorineural hearing loss. A cross-sectional study assessing 96 students, 48 with normal hearing and 48 with sensorineural hearing loss of both sexes, aged 7 and 18 years. To evaluate static balance, Romberg, Romberg-Barré and Fournier tests were used; and for the dynamic balance, we applied the Unterberger test. Hearing loss students showed more changes in static and dynamic balance as compared to normal hearing, in all tests used (p<0.001). The same difference was found when subjects were grouped by sex. For females, Romberg, Romberg-Barré, Fournier and Unterberger test p values were, respectively, p=0.004, p<0.001, p<0.001 and p=0.023; for males, the p values were p=0.009, p<0.001, p<0.001 and p=0.002, respectively. The same difference was observed when students were classified by age. For 7 to 10 years old students, the p values for Romberg, Romberg-Barré and Fournier tests were, respectively, p=0.007, p<0.001 and p=0.001; for those aged 11 and 14 years, the p values for Romberg, Romberg-Barré, Fournier and Unterberger tests were p=0.002, p<0.001, p<0.001 and p=0.015, respectively; and for those aged 15 and 18 years, the p values for Romberg-Barré, Fournier and Unterberger tests were, respectively, p=0.037, p<0.001 and p=0.037. Hearing-loss students showed more changes in static and dynamic balance comparing to normal hearing of same sex and age groups.

Comparison of characteristics observed in tinnitus patients with unilateral vs bilateral symptoms, with both normal hearing threshold and distortion-product otoacoustic emissions.

PubMed

Zagólski, Olaf; Stręk, Paweł

2017-02-01

Tinnitus characteristics in normal-hearing patients differ between the groups with unilateral and bilateral complaints. The study was to determine the differences between tinnitus characteristics observed in patients with unilateral vs bilateral symptoms and normal hearing threshold, as well as normal results of distortion-product otoacoustic emissions (DPOAEs). The patients answered questions concerning tinnitus duration, laterality, character, accompanying symptoms, and circumstances of onset. The results of tympanometry, auditory brainstem responses, tinnitus likeness spectrum, minimum masking level (MML), and uncomfortable loudness level were evaluated. Records of 380 tinnitus sufferers were examined. Patients with abnormal audiograms and/or DPOAEs were excluded. The remaining 66 participants were divided into groups with unilateral and bilateral tinnitus. Unilateral tinnitus in normal-hearing patients was diagnosed twice more frequently than bilateral. Tinnitus pitch was higher in the group with bilateral tinnitus (p < .001). MML was lower in unilateral tinnitus (p < .05). Mean age of patients was higher in the unilateral tinnitus group (p < .05). Mean tinnitus duration was longer (p < .05) and hypersensitivity to sound was more frequent (p < .05) in the bilateral tinnitus group. Repeated exposure to excessive noise was the most frequent cause in the bilateral tinnitus group.

A Comparison of Right Unilateral and Sequential Bilateral Repetitive Transcranial Magnetic Stimulation for Major Depression: A Naturalistic Clinical Australian Study.

PubMed

Galletly, Cherrie A; Carnell, Benjamin L; Clarke, Patrick; Gill, Shane

2017-03-01

A great deal of research has established the efficacy of repetitive transcranial magnetic stimulation (rTMS) in the treatment of depression. However, questions remain about the optimal method to deliver treatment. One area requiring consideration is the difference in efficacy between bilateral and unilateral treatment protocols. This study aimed to compare the effectiveness of sequential bilateral rTMS and right unilateral rTMS. A total of 135 patients participated in the study, receiving either bilateral rTMS (N = 57) or right unilateral rTMS (N = 78). Treatment response was assessed using the Hamilton depression rating scale. Sequential bilateral rTMS had a higher response rate than right unilateral (43.9% vs 30.8%), but this difference was not statistically significant. This was also the case for remission rates (33.3% vs 21.8%, respectively). Controlling for pretreatment severity of depression, the results did not indicate a significant difference between the protocols with regard to posttreatment Hamilton depression rating scale scores. The current study found no statistically significant differences in response and remission rates between sequential bilateral rTMS and right unilateral rTMS. Given the shorter treatment time and the greater safety and tolerability of right unilateral rTMS, this may be a better choice than bilateral treatment in clinical settings.

Acute antidepressant effects of right unilateral ultra-brief ECT: a double-blind randomised controlled trial.

PubMed

Mayur, Prashanth; Byth, Karen; Harris, Anthony

2013-07-01

Shortening the pulse width to 0.3 ms holds neurophysiological and clinical promise of making ECT safer by limiting cognitive side effects. However, the antidepressant effects of right ultra-brief unilateral ECT are under contention. In an acute ECT course, antidepressant equivalence of ultra-brief right unilateral ECT to the high-dose brief pulse right unilateral ECT was investigated. Severely depressed patients were randomised to 1 ms-brief pulse (n=18) or 0.3 ms ultra-brief pulse (n=17) right unilateral ECT, both at high-dose (6 times threshold stimulus dose) given thrice weekly. Depression severity was measured using the Montgomery Asberg Depression Rating Scale at baseline, after 8 treatments and after the acute course of ECT. Depression severity declined equally in both groups: F (1.27,41.97)=0.31, p=0.63. Median time in days to remission (95%CI) was in brief pulse ECT: 26 (18.6-33.4) and ultra-brief pulse ECT:28 (17.9-38.0). The small sample study in the study increases the likelihood of type 2 error. In severe depression, high-dose ultra-brief right unilateral ECT appears to show matching acute antidepressant response to an equally high-dose brief pulse right unilateral ECT. Copyright © 2012 Elsevier B.V. All rights reserved.

Unilateral vs bilateral hip bone mineral density measurement for the diagnosis of osteoporosis.

PubMed

Ikegami, Shota; Kamimura, Mikio; Uchiyama, Shigeharu; Mukaiyama, Keijiro; Kato, Hiroyuki

2014-01-01

It has not been established whether unilateral or bilateral hip dual-energy X-ray absorptiometry (DXA) is preferable for the diagnosis of osteoporosis. We investigated the discordance in DXA measurements in bilateral hips to determine whether unilateral DXA is valid for osteoporosis diagnosis. The subjects were 2964 Japanese patients without a previous diagnosis of primary osteoporosis. We measured bilateral femoral bone mineral density (BMD) and calculated indices, related to the unilateral results, for predicting contralateral hip osteoporosis. A likelihood ratio (LR) of a negative test (LR [-]) of less than 0.2 was considered to exclude the diagnosis. In the normal spinal BMD group, the sensitivity of unilateral DXA for women was 27-73% and LR (-) was 0.28-0.73; the sensitivity for men was 0-50% and LR (-) was 0.51-1.00; the diagnosis of contralateral osteoporosis was not excluded. Sensitivity increased and LR (-) increased with worsening spinal BMD status; however, LR (-) did not meet the cutoff for exclusion. We could exclude unilateral hip osteoporosis, in women only, by performing contralateral femoral DXA; this necessitated lowering the T-score cutoff from -2.5 to -2.0. Unilateral femoral DXA is not useful for excluding the diagnosis of contralateral hip osteoporosis. Copyright © 2014 The International Society for Clinical Densitometry. Published by Elsevier Inc. All rights reserved.

Unilateral versus bilateral thyroarytenoid Botulinum toxin injections in adductor spasmodic dysphonia: a prospective study

PubMed Central

Upile, Tahwinder; Elmiyeh, Behrad; Jerjes, Waseem; Prasad, Vyas; Kafas, Panagiotis; Abiola, Jesuloba; Youl, Bryan; Epstein, Ruth; Hopper, Colin; Sudhoff, Holger; Rubin, John

2009-01-01

Objectives In this preliminary prospective study, we compared unilateral and bilateral thyroarytenoid muscle injections of Botulinum toxin (Dysport) in 31 patients with adductor spasmodic dysphonia, who had undergone more than 5 consecutive Dysport injections (either unilateral or bilateral) and had completed 5 concomitant self-rated efficacy and complication scores questionnaires related to the previous injections. We also developed a Neurophysiological Scoring (NPS) system which has utility in the treatment administration. Method and materials Data were gathered prospectively on voice improvement (self-rated 6 point scale), length of response and duration of complications (breathiness, cough, dysphagia and total voice loss). Injections were performed under electromyography (EMG) guidance. NPS scale was used to describe the EMG response. Dose and unilateral/bilateral injections were determined by clinical judgment based on previous response. Time intervals between injections were patient driven. Results Low dose unilateral Dysport injection was associated with no significant difference in the patient's outcome in terms of duration of action, voice score (VS) and complication rate when compared to bilateral injections. Unilateral injections were not associated with any post treatment total voice loss unlike the bilateral injections. Conclusion Unilateral low dose Dysport injections are recommended in the treatment of adductor spasmodic dysphonia. PMID:19852852

Unilateral retinitis pigmentosa and cone-rod dystrophy

PubMed Central

Farrell, Donald F

2009-01-01

Purpose: The purpose of this paper is to report 14 new cases of unilateral retinitis pigmentosa and three new cases of cone-rod dystrophy and to compare the similarities and dissimilarities to those found in the bilateral forms of these disorders. Methods: A total of 272 cases of retinitis pigmentosa and 167 cases of cone-rod dystrophy were studied by corneal full field electroretinograms and electrooculograms. The student t-test was used to compare categories. Results: The percentage of familial and nonfamilial cases was the same for the bilateral and unilateral forms of the disease. In our series, unilateral retinitis pigmentosa makes up approximately 5% of the total population of retinitis pigmentosa, while unilateral cone-rod dystrophy makes up only about 2% of the total. In the familial forms of unilateral retinitis pigmentosa the most common inheritance pattern was autosomal dominant and all affected relatives had bilateral disease. Conclusion: Unilateral retinitis pigmentosa and cone-rod dystrophy appear to be directly related to the more common bilateral forms of these disorders. The genetic mechanisms which account for asymmetric disorders are not currently understood. It may be a different unidentified mutation at a single loci or it is possible that nonlinked mutations in multiple loci account for this unusual disorder. PMID:19668577

A review of patients who suddenly deteriorate in the presence of paramedics.

PubMed

Boyle, Malcolm J; Smith, Erin C; Archer, Frank

2008-07-26

The report of the Ministerial Review of Trauma and Emergency Services in Victoria, Australia, recommended that paramedics be permitted to divert to the closest hospital in incidences of life threatening situations prior to and during transport. An audit of patients that suddenly deteriorated in paramedic care was recommended by the Ministerial Review. The objective of the study was to identify the number and outcome of patients who suddenly deteriorated in the presence of paramedics. A retrospective cohort study of trauma patients who suddenly deteriorated in the presence of paramedics during 2002. As there was no standard definition, sudden deterioration was defined using a predetermined set of physiological criteria. Patient care record data of patients who suddenly deteriorated were compared with the State Trauma Registry to determine those who sustained hospital defined major trauma. Patient care records where hospital bypass was undertaken were identified and analysed. Ethics committee approval was obtained. There were 2,893 patients that suddenly deteriorated according to predefined criteria. 2,687 (5.1% of the total trauma patients for 2002) were suitable for further analysis. The majority of patients had a sudden decrease in BP (n = 2,463) with 4.3% having hospital defined major trauma. For patients with a sudden decrease in conscious state or a total GCS score of less than 13 (n = 77), 37.7% had hospital defined major trauma; and a sudden increase/decrease in pulse rate and sudden decrease in BP (n = 65), 26.2% had hospital defined major trauma. Only 28 documented incidents of hospital bypass were identified. This study suggests that the incidents of patients suddenly deteriorating in the presence of paramedics are low and the incidence of hospital bypass is not well documented.

Sudden gains in exposure-focused cognitive-behavioral group therapy for panic disorder.

PubMed

Nogueira-Arjona, Raquel; Santacana, Martí; Montoro, María; Rosado, Silvia; Guillamat, Roser; Vallès, Vicenç; Fullana, Miquel A

2017-11-01

In the context of psychological treatment, a sudden gain is a large and enduring improvement in symptom severity that occurs between two single therapy sessions. The influence of sudden gains on long-term outcomes and functional impairment in anxiety disorders is not well understood, and little is known with regard to panic disorder in particular. In addition, previous research on patients with anxiety disorders has produced inconsistent results regarding the relationship between sudden gains and cognitive change. We examined the incidence of sudden gains in a large sample (n = 116) of panic disorder patients undergoing exposure-focused cognitive-behavioral group therapy, and compared panic severity, functional impairment, and cognitive change in patients with and without sudden gains at posttreatment and 6-month follow-up. Participants who experienced sudden gains displayed lower levels of panic severity and functional impairment at posttreatment and 6-month follow-up than those who did not experience sudden gains. However, we observed no difference in cognitive changes between groups, either at posttreatment or at follow-up. Our results demonstrate that the beneficial effects of sudden gains on therapeutic outcomes not only extend to long-term and functional outcome measures but are also evident in less cognitive (i.e., exposure-focused) forms of psychological treatment. Sudden gains are common in panic disorder patients undergoing exposure-based cognitive-behavioral group therapy. Sudden gains during exposure-focused therapy are linked to greater improvement in panic disorder severity and functional impairment. The positive impact of sudden gains on panic disorder severity and functional impairment is maintained in the long term. Copyright © 2017 John Wiley & Sons, Ltd.

Syndrome in question*

PubMed Central

Peruzzo, Juliano; Nazar, Fernanda Luca; Tubone, Mariana Quirino; Escobar, Gabriela Fortes; Cestari, Tania Ferreira

2015-01-01

Waardenburg syndrome is an inherited disease characterized by sensorineural hearing loss, pigmentation changes and minor facial malformations. It has four clinical variants. We report the case of a girl who, like her mother, was affected by this syndrome. The diagnosis was made after detection and treatment of deafness. PMID:26375234

Genes and Syndromic Hearing Loss.

ERIC Educational Resources Information Center

Keats, Bronya J. B.

2002-01-01

This article provides a description of the human genome and patterns of inheritance and discusses genes that are associated with some of the syndromes for which hearing loss is a common finding, including: Waardenburg, Stickler, Jervell and Lange-Neilsen, Usher, Alport, mitochondrial encephalomyopathy, and sensorineural hearing loss. (Contains…

Prospective study of the effects of ventilation tubes on hearing after radiotherapy for carcinoma of nasopharynx

DOE Office of Scientific and Technical Information (OSTI.GOV)

Chowdhury, C.R.; Ho, J.H.; Wright, A.

1988-03-01

In patients with nasopharyngeal carcinoma, deafness sometimes occurs following radiotherapy. It is usually conductive, but may be sensorineural. Tinnitus is present frequently and usually is distressing. The role of ventilation tubes (grommets) in relieving these problems was assessed in a prospective randomized controlled trial of 115 patients. In the group with grommets, there was an improvement in hearing, with a reduction of the averaged air-bone gap (p less than .01). This was not found in the control group without ventilation tubes, who developed a larger conductive loss (p less than .01) and, in addition, a slight deterioration of the averagedmore » bone conduction threshold (p less than .01). Surprisingly, the group with grommets did not develop this sensorineural loss (p less than .01). In addition, tinnitus was improved significantly by the insertion of ventilation tubes (p less than .01). Neither necrosis nor stenosis of the external auditory meatus was found in either group.« less

Effects of sensorineural hearing loss on visually guided attention in a multitalker environment.

PubMed

Best, Virginia; Marrone, Nicole; Mason, Christine R; Kidd, Gerald; Shinn-Cunningham, Barbara G

2009-03-01

This study asked whether or not listeners with sensorineural hearing loss have an impaired ability to use top-down attention to enhance speech intelligibility in the presence of interfering talkers. Listeners were presented with a target string of spoken digits embedded in a mixture of five spatially separated speech streams. The benefit of providing simple visual cues indicating when and/or where the target would occur was measured in listeners with hearing loss, listeners with normal hearing, and a control group of listeners with normal hearing who were tested at a lower target-to-masker ratio to equate their baseline (no cue) performance with the hearing-loss group. All groups received robust benefits from the visual cues. The magnitude of the spatial-cue benefit, however, was significantly smaller in listeners with hearing loss. Results suggest that reduced utility of selective attention for resolving competition between simultaneous sounds contributes to the communication difficulties experienced by listeners with hearing loss in everyday listening situations.

Prospective study of the effects of ventilation tubes on hearing after radiotherapy for carcinoma of nasopharynx

DOE Office of Scientific and Technical Information (OSTI.GOV)

Chowdhury, C.R.; Ho, J.H.; Wright, A.

In patients with nasopharyngeal carcinoma, deafness sometimes occurs following radiotherapy. It is usually conductive, but may be sensorineural. Tinnitus is present frequently and usually is distressing. The role of ventilation tubes (grommets) in relieving these problems was assessed in a prospective randomized controlled trial of 115 patients. In the group with grommets, there was an improvement in hearing, with a reduction of the averaged air-bone gap (p less than .01). This was not found in the control group without ventilation tubes, who developed a larger conductive loss (p less than .01) and, in addition, a slight deterioration of the averagedmore » bone conduction threshold (p less than .01). Surprisingly, the group with grommets did not develop this sensorineural loss (p less than .01). In addition, tinnitus was improved significantly by the insertion of ventilation tubes (p less than .01). Neither necrosis nor stenosis of the external auditory meatus was found in either group.« less

A comparison of the effects of filtering and sensorineural hearing loss on patients of consonant confusions.

PubMed

Wang, M D; Reed, C M; Bilger, R C

1978-03-01

It has been found that listeners with sensorineural hearing loss who show similar patterns of consonant confusions also tend to have similar audiometric profiles. The present study determined whether normal listeners, presented with filtered speech, would produce consonant confusions similar to those previously reported for the hearing-impaired listener. Consonant confusion matrices were obtained from eight normal-hearing subjects for four sets of CV and VC nonsense syllables presented under six high-pass and six-low pass filtering conditions. Patterns of consonant confusion for each condition were described using phonological features in sequential information analysis. Severe low-pass filtering produced consonant confusions comparable to those of listeners with high-frequency hearing loss. Severe high-pass filtering gave a result comparable to that of patients with flat or rising audiograms. And, mild filtering resulted in confusion patterns comparable to those of listeners with essentially normal hearing. An explanation in terms of the spectrum, the level of speech, and the configuration of this individual listener's audiogram is given.

[Bilateral sensorineural hearing impairment due to Streptococcus suis meningitis 20 days after swine bite].

PubMed

Mori, Kousuke; Ishii, Nobuyuki; Mochizuki, Hitoshi; Taniguchi, Akitoshi; Shiomi, Kazutaka; Nakazato, Masamitsu

2013-01-01

Streptococcus suis (S. suis) is a zoonotic pathogen in pigs, which can be transmitted to humans by close contact. Meningitis is the most common clinical manifestations of S. suis infection and hearing impairment is a frequent complication. The risk of S. suis meningitis is higher in people who work in the swine industry. The patient was a 53-year-old woman working in the swine industry, who developed headache and fever 20 days after a swine bite. She was diagnosed as meningitis and S. suis was detected in the cerebrospinal fluid. We treated her with ceftriaxone, vancomycin, and dexamethasone, and signs of meningeal irritation diminished three days after admission. However, bilateral sensorineural hearing impairment occurred on the ninth day after admission. We added methylprednisolone (500 mg, 2 days) but moderate hearing impairment remained on the left. Antibiotic therapy should be considered for wounds of people involved in the swine industry for preventing S. suis infection.When S. suis meningitis occurs, symptoms of hearing impairment must be monitored carefully.

Cytomegalovirus induced sensorineural hearing loss with persistent cochlear inflammation in neonatal mice

PubMed Central

Schachtele, Scott J.; Mutnal, Manohar B.; Schleiss, Mark R.; Lokensgard, James R.

2011-01-01

Congenital cytomegalovirus (CMV) infection is the leading cause of sensorineural hearing loss (SNHL) in children. During murine (M)CMV-induced encephalitis, the immune response is important for both the control of viral dissemination and the clearance of virus from the brain. While the importance of CMV-induced SNHL has been described, the mechanisms surrounding its pathogenesis and the role of inflammatory responses remain unclear. This study presents a neonatal mouse model of profound SNHL in which MCMV preferentially infected both cochlear perilymphatic epithelial cells and spiral ganglion neurons. Interestingly, MCMV infection induced cochlear hair cell death by 21 days post-infection, despite a clear lack of direct infection of hair cells and the complete clearance of the virus from the cochlea by 14 dpi. Flow cytometric, immunohistochemical, and quantitative PCR analysis of MCMV-infected cochlea revealed a robust and chronic inflammatory response, including a prolonged increase in reactive oxygen species production by infiltrating macrophages. These data support a pivotal role for inflammation during MCMV-induced SNHL. PMID:21416394

Cytomegalovirus-induced sensorineural hearing loss with persistent cochlear inflammation in neonatal mice.

PubMed

Schachtele, Scott J; Mutnal, Manohar B; Schleiss, Mark R; Lokensgard, James R

2011-06-01

Congenital cytomegalovirus (CMV) infection is the leading cause of sensorineural hearing loss (SNHL) in children. During murine (M)CMV-induced encephalitis, the immune response is important for both the control of viral dissemination and the clearance of virus from the brain. While the importance of CMV-induced SNHL has been described, the mechanisms surrounding its pathogenesis and the role of inflammatory responses remain unclear. This study presents a neonatal mouse model of profound SNHL in which MCMV preferentially infected both cochlear perilymphatic epithelial cells and spiral ganglion neurons. Interestingly, MCMV infection induced cochlear hair cell death by 21 days post-infection, despite a clear lack of direct infection of hair cells and the complete clearance of the virus from the cochlea by 14 dpi. Flow cytometric, immunohistochemical, and quantitative PCR analysis of MCMV-infected cochlea revealed a robust and chronic inflammatory response, including a prolonged increase in reactive oxygen species production by infiltrating macrophages. These data support a pivotal role for inflammation during MCMV-induced SNHL.

Severe diarrhea-dehydration in infancy permanently alters auditory function.

PubMed

Todd, N Wendell

2012-02-01

Of the myriad etiologies of sensorineural hearing impairment, metabolic stress is rarely considered. I posit that severe dehydration in conjunction with hypoxia, at least during infancy, prompts permanent changes in the cochlea. In a population-based prospective study of otitis media, children without otitis were found to have at age 4-8 years, worse auditory thresholds if as an infant had been hospitalized for diarrhea-dehydration. What is more, stapedius reflex thresholds tended to be lower in children who had been hospitalized for diarrhea-dehydration: that is, less acoustic energy for arousal or to be frightening. The hypothesis that the transient metabolic stress of dehydration with hypoxia prompts permanent sensorineural hearing impairment with reduced uncomfortable loudness thresholds, is both (1) consistent in an evolutionary sense with a subsequent survival advantage, and (2) subject to verification both by descriptive studies of children undergoing ECMO (ExtraCorporeal Membrane Oxygenation) or care for congenital diaphragmatic hernia, and by animal studies. Copyright © 2011 Elsevier Ltd. All rights reserved.

Increasing knowledge of best practices for occupational therapists treating post-stroke unilateral spatial neglect: results of a knowledge-translation intervention study.

PubMed

Petzold, Anita; Korner-Bitensky, Nicol; Salbach, Nancy M; Ahmed, Sara; Menon, Anita; Ogourtsova, Tatiana

2012-02-01

The aim of this study was to investigate: (i) the feasibility of delivering a multi-modal knowledge translation intervention specific to the management of acute post-stroke unilateral spatial neglect; and (ii) the impact of the knowledge translation intervention on occupational therapists' knowledge of evidence-based unilateral spatial neglect problem identification, assessment and treatment, and self-efficacy related to evidence-based practice implementation. A 3-period (pre-post) repeated measures design. Acute care occupational therapists treating patients with post-stroke unilateral spatial neglect were recruited from two major Canadian cities. Participants completed two pre-intervention assessments, took part in a day-long interactive multi-modal knowledge translation intervention and a subsequent 8-week follow-up, and completed a post-intervention assessment. Knowledge of evidence-based problem identification, assessment and treatment of unilateral spatial neglect, and self-efficacy to perform evidence-based practice activities were measured using standard scales. The intervention was tested on 20 occupational therapists. Results indicate a significant improvement in knowledge of best practice unilateral spatial neglect management (p < 0.000) and evidence-based practice self-efficacy in carrying out evidence-based practice activities (p < 0.045) post-intervention. Use of a multi-modal knowledge translation intervention is feasible and can significantly improve occupational therapists' knowledge of unilateral spatial neglect best practices and self-efficacy. The findings should help advance best practices specific to the management of post-stroke unilateral spatial neglect as well as informing knowledge translation studies in other areas of practice.

RS3PE presenting in a unilateral pattern: case report and review of the literature.

PubMed

Keenan, Robert T; Hamalian, Gareen M; Pillinger, Michael H

2009-06-01

To review the clinical features and pathophysiologic implications of remitting seronegative symmetrical synovitis with pitting edema (RS(3)PE) presenting in a unilateral manner. We identified and characterized an index case of RS(3)PE presenting in a unilateral pattern. We subsequently performed a systematic literature search to identify other reports of patients with unilateral RS(3)PE. The index case was a 76-year-old male with a prior history of right hemiparesis owing to a cerebrovascular accident 25 years prior, who developed a classic picture of RS(3)PE involving hand (metacarpophalageal and wrist joint) arthritis and dorsal pitting edema, accompanied by an elevated erythrocyte sedimentation rate, but only in the nonhemiparetic hand. The condition responded rapidly to low-dose prednisone. Our literature search identified 5 other cases of unilateral RS(3)PE, including 2 presented only in the Italian or German literature. Of the 5 cases, 2 were in patients with preexisting neurologic disease, in which the neurologically affected side was spared. One additional case initially presented as unilateral disease but rapidly progressed to bilaterality. Two cases presented in a fully unilateral manner despite no reported neurologic abnormalities on the unaffected sides. While RS(3)PE is almost always a symmetric disease of the upper extremities, it may rarely present in a unilateral fashion. The apparent ability of neuropathic changes to protect against the expression of RS(3)PE in an extremity suggests a role for neural and possibly other local factors in the genesis/modulation of the onset or maintenance of RS(3)PE.

Is the side with the best masticatory performance selected for chewing?

PubMed

Rovira-Lastra, Bernat; Flores-Orozco, Elan Ignacio; Salsench, Juan; Peraire, Maria; Martinez-Gomis, Jordi

2014-12-01

This study assessed the degree of relationship between masticatory laterality and lateral asymmetry of masticatory performance using silicon pieces enclosed in a latex bag. Forty-two young adults with natural dentition participated in this cross-sectional, observational study. They performed four different masticatory assays, each consisting of five trials of chewing three pieces of silicon for 20 cycles. In one assay, they were asked to masticate unbagged silicon free-style, whilst in the three other assays they were asked to masticate bagged silicon free-style, unilaterally on the right-hand side and unilaterally on the left-hand side. The preferred chewing side was determined by calculating the asymmetry index for both the free-style assays. Masticatory performance was determined by sieving the silicon particles and the cycle duration was also recorded. Data were analysed using independent samples or paired t-test and linear regression. Masticatory function using the bagged silicon was similar to that using the unbagged silicon. A significant and positive relationship was observed between the preferred chewing side expressed as the asymmetry index and the side with better masticatory performance. Alternate unilateral chewers demonstrated better masticatory performance than unilateral chewers. However, when free-style and unilateral chewing were compared for each subject, unilateral chewing was found to be as efficient as - or even more efficient than - free-style chewing. There is a positive association between the preferred chewing side and the more efficient side. Alternate unilateral mastication per se does not promote better masticatory performance than consistently unilateral mastication. Copyright © 2014 Elsevier Ltd. All rights reserved.

Sudden Event Recognition: A Survey

PubMed Central

Suriani, Nor Surayahani; Hussain, Aini; Zulkifley, Mohd Asyraf

2013-01-01

Event recognition is one of the most active research areas in video surveillance fields. Advancement in event recognition systems mainly aims to provide convenience, safety and an efficient lifestyle for humanity. A precise, accurate and robust approach is necessary to enable event recognition systems to respond to sudden changes in various uncontrolled environments, such as the case of an emergency, physical threat and a fire or bomb alert. The performance of sudden event recognition systems depends heavily on the accuracy of low level processing, like detection, recognition, tracking and machine learning algorithms. This survey aims to detect and characterize a sudden event, which is a subset of an abnormal event in several video surveillance applications. This paper discusses the following in detail: (1) the importance of a sudden event over a general anomalous event; (2) frameworks used in sudden event recognition; (3) the requirements and comparative studies of a sudden event recognition system and (4) various decision-making approaches for sudden event recognition. The advantages and drawbacks of using 3D images from multiple cameras for real-time application are also discussed. The paper concludes with suggestions for future research directions in sudden event recognition. PMID:23921828

Reactive Balance Control in Response to Perturbation in Unilateral Stance: Interaction Effects of Direction, Displacement and Velocity on Compensatory Neuromuscular and Kinematic Responses

PubMed Central

Freyler, Kathrin; Gollhofer, Albert; Colin, Ralf; Brüderlin, Uli; Ritzmann, Ramona

2015-01-01

Unexpected sudden perturbations challenge postural equilibrium and require reactive compensation. This study aimed to assess interaction effects of the direction, displacement and velocity of perturbations on electromyographic (EMG) activity, centre of pressure (COP) displacement and joint kinematics to detect neuromuscular characteristics (phasic and segmental) and kinematic strategies of compensatory reactions in an unilateral balance paradigm. In 20 subjects, COP displacement and velocity, ankle, knee and hip joint excursions and EMG during short (SLR), medium (MLR) and long latency response (LLR) of four shank and five thigh muscles were analysed during random surface translations varying in direction (anterior-posterior (sagittal plane), medial-lateral (frontal plane)), displacement (2 vs. 3cm) and velocity (0.11 vs. 0.18m/s) of perturbation when balancing on one leg on a movable platform. Phases: SLR and MLR were scaled to increased velocity (P<0.05); LLR was scaled to increased displacement (P<0.05). Segments: phasic interrelationships were accompanied by segmental distinctions: distal muscles were used for fast compensation in SLR (P<0.05) and proximal muscles to stabilise in LLR (P<0.05). Kinematics: ankle joints compensated for both increasing displacement and velocity in all directions (P<0.05), whereas knee joint deflections were particularly sensitive to increasing displacement in the sagittal (P<0.05) and hip joint deflections to increasing velocity in the frontal plane (P<0.05). COP measures increased with increasing perturbation velocity and displacement (P<0.05). Interaction effects indicate that compensatory responses are based on complex processes, including different postural strategies characterised by phasic and segmental specifications, precisely adjusted to the type of balance disturbance. To regain balance after surface translation, muscles of the distal segment govern the quick regain of equilibrium; the muscles of the proximal limb serve as delayed stabilisers after a balance disturbance. Further, a kinematic distinction regarding the compensation for balance disturbance indicated different plane- and segment-specific sensitivities with respect to the determinants displacement and velocity. PMID:26678061

Reproducibility of Tactile Assessments for Children with Unilateral Cerebral Palsy

ERIC Educational Resources Information Center

Auld, Megan Louise; Ware, Robert S.; Boyd, Roslyn Nancy; Moseley, G. Lorimer; Johnston, Leanne Marie

2012-01-01

A systematic review identified tactile assessments used in children with cerebral palsy (CP), but their reproducibility is unknown. Sixteen children with unilateral CP and 31 typically developing children (TDC) were assessed 2-4 weeks apart. Test-retest percent agreements within one point for children with unilateral CP (and TDC) were…

Hemodynamic stability ensured by a low dose, low volume, unilateral hypobaric spinal block: modification of a technique.

PubMed

Elzinga, L; Marcus, M; Peek, D; Borg, P; Jansen, J; Koster, J; Enk, D

2009-01-01

We report the case of an 89-year-old female with a history of arterial hypertension, intermittent rapid atrial fibrillation and severe aortic valve stenosis, suffering from femoral neck fracture. Hyperbaric unilateral spinal anesthesia is a known technique to obtain stable hemodynamics combined with the possibility of continuous neurologic evaluation and preservation of cognitive functions. Because a hyperbaric unilateral technique can be very painful in case of traumatic hip fracture, a low dose, low volume, unilateral hypobaric spinal block may be an adequate alternative. In the present case report, a unilateral hypobaric spinal anesthesia was performed using 5 mg of bupivacaine in a 1.5 mL volume and a slow and steady, "air-buffered", directed injection technique, to allow an urgent hip arthroplasty. During surgery the patient was kept in the lateral recumbent position. Hemodynamics remained stable throughout the entire procedure without any need for vasoconstrictors. The impact of aortic valve stenosis combined with atrial fibrillation on anesthetic management and our considerations to opt for a unilateral hypobaric spinal anesthesia are discussed.

Sudden unexpected death in epilepsy genetics: Molecular diagnostics and prevention.

PubMed

Goldman, Alica M; Behr, Elijah R; Semsarian, Christopher; Bagnall, Richard D; Sisodiya, Sanjay; Cooper, Paul N

2016-01-01

Epidemiologic studies clearly document the public health burden of sudden unexpected death in epilepsy (SUDEP). Clinical and experimental studies have uncovered dynamic cardiorespiratory dysfunction, both interictally and at the time of sudden death due to epilepsy. Genetic analyses in humans and in model systems have facilitated our current molecular understanding of SUDEP. Many discoveries have been informed by progress in the field of sudden cardiac death and sudden infant death syndrome. It is becoming apparent that SUDEP genomic complexity parallels that of sudden cardiac death, and that there is a pauci1ty of analytically useful postmortem material. Because many challenges remain, future progress in SUDEP research, molecular diagnostics, and prevention rests in international, collaborative, and transdisciplinary dialogue in human and experimental translational research of sudden death. Wiley Periodicals, Inc. © 2016 International League Against Epilepsy.

Analysis of laterality and percentage of tumor involvement in 1386 prostatectomized specimens for selection of unilateral focal cryotherapy.

PubMed

Mouraviev, Vladimir; Mayes, Janice M; Madden, John F; Sun, Leon; Polascik, Thomas J

2007-04-01

In total, 1386 paraffin embedded radical prostatectomy specimens from patients with clinically localized prostate cancer (PCa) excised between 2002-06 were analyzed. Pathologic assessment paid particular attention to laterality and percentage of tumor involvement (PTI) along with pathologic Gleason Score (pGS). Completely unilateral cancers were identified in 254 (18.3%) patients, and in 39% cases of them the signs of clinically significant PCa were revealed. The majority of unilateral tumors (72%) were low volume with a PTI of < or =5. This study suggests that only a select group of men diagnosed with PCa have completely unilateral cancers that would be amenable to focal ablation therapy targeting 1 lobe. Further study is needed to develop predictive models for those patients likely to have small, unilateral cancers that may be amenable to focal therapy.

Proceedings of the Sudden Oak Death Fourth Science Symposium

Treesearch

Susan J. Frankel; John T. Kliejunas; Katharine M. Palmieri

2010-01-01

The Sudden Oak Death Fourth Science Symposium provided a forum for current research on sudden oak death, caused by the exotic, quarantine pathogen, Phytophthora ramorum. Ninety submissions describing papers or posters on the following sudden oak death/P. ramorum topics are included: biology, genetics, nursery and wildland...

Proceedings of the sudden oak death third science symposium

Treesearch

Susan J. Frankel; John T. Kliejunas; Katharine M. Palmieri

2008-01-01

The Sudden Oak Death Third Science Symposium provided a forum for current research on sudden oak death, caused by the exotic, quarantine pathogen, Phytophthora ramorum. One hundred and seventeen submissions describing papers and posters on the following sudden oak death/ P. ramorum topics are included: biology, genetics, nursery,...

Proceedings of the sudden oak death sixth science symposium

Treesearch

Susan J. Frankel; Katharine M. Harrell

2017-01-01

The Sudden Oak Death Sixth Science Symposium provided a forum for current research on sudden oak death, caused by the exotic quarantine pathogen Phytophthora ramorum. More than 50 submissions describing papers or posters on the following sudden oak death/P. ramorum topics are included: biology, genetics, nursery and wildland...

Sudden Death in Sleep of Laotian-Hmong Refugees in Thailand: A Case-Control Study.

ERIC Educational Resources Information Center

Munger, Ronald G.

1987-01-01

Surveillance for sudden deaths conducted among Laotian-Hmong refugees in Thailand revealed associations between sudden death in sleep and membership in the Green-Hmong subgroup, a family history of sudden death, and previous non-fatal sleep disturbances. Most victims are young men. (PS)

Cone-Beam Computed Tomography Analysis of the Nasopharyngeal Airway in Nonsyndromic Cleft Lip and Palate Subjects.

PubMed

Al-Fahdawi, Mahmood Abd; Farid, Mary Medhat; El-Fotouh, Mona Abou; El-Kassaby, Marwa Abdelwahab

2017-03-01

  To assess the nasopharyngeal airway volume, cross-sectional area, and depth in previously repaired nonsyndromic unilateral cleft lip and palate versus bilateral cleft lip and palate patients compared with noncleft controls using cone-beam computed tomography with the ultimate goal of finding whether cleft lip and palate patients are more liable to nasopharyngeal airway obstruction.   A retrospective analysis comparing bilateral cleft lip and palate, unilateral cleft lip and palate, and control subjects. Significance at P ≤ .05.   Cleft Care Center and the outpatient clinic that are both affiliated with our faculty.   Cone-beam computed tomography data were selected of 58 individuals aged 9 to 12 years: 14 with bilateral cleft lip and palate and 20 with unilateral cleft lip and palate as well as 24 age- and gender-matched noncleft controls.   Volume, depth, and cross-sectional area of nasopharyngeal airway were measured.   Patients with bilateral cleft lip and palate showed significantly larger nasopharyngeal airway volume than controls and patients with unilateral cleft lip and palate (P < .001). Patients with bilateral cleft lip and palate showed significantly larger cross-sectional area than those with unilateral cleft lip and palate (P < .001) and insignificant cross-sectional area compared with controls (P > .05). Patients with bilateral cleft lip and palate showed significantly larger depth than controls and those with unilateral cleft lip and palate (P < .001). Patients with unilateral cleft lip and palate showed insignificant nasopharyngeal airway volume, cross-sectional area, and depth compared with controls (P > .05).   Unilateral and bilateral cleft lip and palate patients did not show significantly less volume, cross-sectional area, or depth of nasopharyngeal airway than controls. From the results of this study we conclude that unilateral and bilateral cleft lip and palate patients at the studied age and stage of repaired clefts are not more prone to nasopharyngeal airway obstruction than controls.

Effects of unilateral and bilateral plyometric training on power and jumping ability in women.

PubMed

Makaruk, Hubert; Winchester, Jason B; Sadowski, Jerzy; Czaplicki, Adam; Sacewicz, Tomasz

2011-12-01

Makaruk, H, Winchester, JB, Sadowski, J, Czaplicki, A, and Sacewicz, T. Effects of unilateral and bilateral plyometric training on power and jumping ability in women. J Strength Cond Res 25(12): 3311-3318, 2011-The purpose of this study was to examine the effects of unilateral and bilateral plyometric exercise on peak power and jumping performance during different stages of a 12-week training and detraining in women. Forty-nine untrained but physically active female college students were randomly assigned to 1 of 3 groups: unilateral plyometric group (n = 16), bilateral plyometric group (BLE; n = 18), and a control group (n = 15). Peak power and jumping ability were assessed by means of the alternate leg tests (10-second Wingate test and 5 alternate leg bounds), bilateral leg test (countermovement jump [CMJ]) and unilateral leg test (unilateral CMJ). Performance indicators were measured pretraining, midtraining, posttraining, and detraining. Differences between dependent variables were assessed with a 3 × 4 (group × time) repeated analysis of variance with Tukey's post hoc test applied where appropriate. Effect size was calculated to determine the magnitude of significant differences between the researched parameters. Only the unilateral plyometric training produced significant (p < 0.05) improvement in all tests from pretraining to midtraining, but there was no significant (p < 0.05) increase in performance indicators from midtraining to posttraining. The BLE group significantly (p < 0.05) improved in all tests from pretraining to posttraining and did not significantly (p > 0.05) decrease power and jumping ability in all tests during detraining. These results suggest that unilateral plyometric exercises produce power and jumping performance during a shorter period when compared to bilateral plyometric exercises but achieved performance gains last longer after bilateral plyometric training. Practitioners should consider the inclusion of both unilateral and bilateral modes of plyometric exercise to elicit rapid improvements and guard against detraining.

Bitemporal Versus High-Dose Unilateral Twice-Weekly Electroconvulsive Therapy for Depression (EFFECT-Dep): A Pragmatic, Randomized, Non-Inferiority Trial.

PubMed

Semkovska, Maria; Landau, Sabine; Dunne, Ross; Kolshus, Erik; Kavanagh, Adam; Jelovac, Ana; Noone, Martha; Carton, Mary; Lambe, Sinead; McHugh, Caroline; McLoughlin, Declan M

2016-04-01

ECT is the most effective treatment for severe depression. Previous efficacy studies, using thrice-weekly brief-pulse ECT, reported that high-dose (6× seizure threshold) right unilateral ECT is similar to bitemporal ECT but may have fewer cognitive side effects. The authors aimed to assess the effectiveness and cognitive side effects of twice-weekly moderate-dose (1.5× seizure threshold) bitemporal ECT with high-dose unilateral ECT in real-world practice. This was a pragmatic, patient- and rater-blinded, noninferiority trial of patients with major depression (N=138; 63% female; age=56.7 years [SD=14.8]) in a national ECT service with a 6-month follow-up. Participants were independently randomly assigned to bitemporal or high-dose unilateral ECT. The primary outcome was change in the 24-item Hamilton Depression Rating Scale (HAM-D) score after the ECT course; the prespecified noninferiority margin was 4.0 points. Secondary outcomes included response and remission rates, relapse status after 6 months, and cognition. Of the eligible patients, 69 were assigned to bitemporal ECT and 69 to unilateral ECT. High-dose unilateral ECT was noninferior to bitemporal ECT regarding the 24-item HAM-D scores after the ECT course (mean difference=1.08 points in favor of unilateral ECT [95% CI=-1.67 to 3.84]). There were no significant differences for response and remission or 6-month relapse status. Recovery of orientation was quicker following unilateral ECT (median=19.1 minutes versus 26.4 minutes). Bitemporal ECT was associated with a lower percent recall of autobiographical information (odds ratio=0.66) that persisted for 6 months. Twice-weekly high-dose unilateral ECT is not inferior to bitemporal ECT for depression and may be preferable because of its better cognitive side-effect profile.

Prepubertal unilateral gynecomastia: report of 2 cases.

PubMed

Demirbilek, Hüseyin; Bacak, Gökhan; Baran, Rıza Taner; Avcı, Yahya; Baran, Ahmet; Keleş, Ayşenur; Özbek, Mehmet Nuri; Alanay, Yasemin; Hussain, Khalid

2014-12-01

Prepubertal unilateral gynecomastia is an extremely rare condition. At present, its etiology and management strategy are not well known. Two unrelated prepubertal boys of ages 8 and 9 who presented with complaints of unilateral enlargement of breast tissue are reported. Physical examination, biochemical, hormonal and oncologic work-up findings were normal. Both patients were treated with peripheral liposuction successfully. Histopathological and immunohistochemical examinations showed benign fibroglandular gynecomastia and intensive (3+) estrogen receptor expression in 100% of periductal epithelial cells. Although an extremely rare and generally benign condition, patients with prepubertal unilateral gynecomastia should have a full endocrine and oncologic work-up.

Prepubertal Unilateral Gynecomastia: Report of 2 Cases

PubMed Central

Demirbilek, Hüseyin; Bacak, Gökhan; Baran, Rıza Taner; Avcı, Yahya; Baran, Ahmet; Keleş, Ayşenur; Özbek, Mehmet Nuri; Alanay, Yasemin; Hussain, Khalid

2014-01-01

Prepubertal unilateral gynecomastia is an extremely rare condition. At present, its etiology and management strategy are not well known. Two unrelated prepubertal boys of ages 8 and 9 who presented with complaints of unilateral enlargement of breast tissue are reported. Physical examination, biochemical, hormonal and oncologic work-up findings were normal. Both patients were treated with peripheral liposuction successfully. Histopathological and immunohistochemical examinations showed benign fibroglandular gynecomastia and intensive (3+) estrogen receptor expression in 100% of periductal epithelial cells. Although an extremely rare and generally benign condition, patients with prepubertal unilateral gynecomastia should have a full endocrine and oncologic work-up. PMID:25541897

38 CFR 17.149 - Sensori-neural aids.

Code of Federal Regulations, 2010 CFR

2010-07-01

... attendance or by reason of being permanently housebound; (6) Those who have a visual or hearing impairment... normally occurring visual or hearing impairments; and (8) Those visually or hearing impaired so severely... frequency ranges which contribute to a loss of communication ability; however, hearing aids are to be...

Optimal dose of hyperbaric bupivacaine 0.5% for unilateral spinal anesthesia during diagnostic knee arthroscopy

PubMed Central

Atef, HM; El-Kasaby, AM; Omera, MA; Badr, MD

2010-01-01

Objective To determine the dose of hyperbaric bupivacaine 0.5% required for unilateral spinal anesthesia during diagnostic knee arthroscopy. Patients and methods This prospective, randomized, clinical study was performed in 80 patients who were assigned to four groups to receive different doses of intrathecal hyperbaric bupivacaine (5 mg, 7.5 mg, 10 mg and 12.5 mg in Groups 1, 2, 3, and 4 respectively). Onset of sensory and motor block, hemodynamic changes, regression of motor block, and incidence of complications were recorded. Results Unilateral sensory block was reported in 90% and 85% of patients in Group 1 and Group 2, respectively, but not in any patient in Group 3 and Group 4. Unilateral motor block (modified Bromage scale 0) was reported in 95% of patients in Group 1, 90% in Group 2, and only 5% in Group 3, while no patient in Group 4 showed unilateral motor block. The time required for regression of motor block (Bromage scale 0) was prolonged with higher doses. The incidence of nausea, vomiting, and urine retention was similar in the study groups. Conclusion Unilateral sensory and motor block can be achieved with doses of 5 mg and 7.5 mg hyperbaric bupivacaine 0.5% with a stable hemodynamic state. However, 7.5 mg of hyperbaric bupivacaine 0.5% was the dose required for adequate unilateral spinal anesthesia. PMID:22915874

Associations between orthopaedic disturbances and unilateral crossbite in children with asymmetry of the upper cervical spine.

PubMed

Korbmacher, Heike; Koch, L; Eggers-Stroeder, G; Kahl-Nieke, B

2007-02-01

The objective of the present study was to detect possible associations between unilateral crossbite and orthopaedic disturbances in children with asymmetry of the upper cervical spine. Fifty-five children aged 3-10 years (22 girls and 33 boys) with a unilateral crossbite and 55 gender- and age-matched children with a symmetric occlusion but no crossbite, who served as the control group, were selected from an orthopaedic cohort of 240 patients. In all children, asymmetry of the upper cervical region was confirmed by radiographs and palpation. The following orthopaedic aspects were investigated: oblique shoulder and pelvis, scoliosis, functional leg length difference, and laxity of ligaments of the foot. The differences between the groups were analysed by means of an unpaired t-test. An increased occurrence of orthopaedic parameters in the frontal plane was observed in children with a unilateral malocclusion. A unilateral crossbite was not necessarily combined with a pathological orthopaedic variable, but statistically, children with a unilateral malocclusion showed more often an oblique shoulder (P = 0.004), scoliosis (P = 0.04), an oblique pelvis (P = 0.007), and a functional leg length difference (P = 0.002) than children with symmetry. The results suggest that a unilateral crossbite in children with asymmetry of the upper cervical spine is associated with orthopaedic disturbances. There is no evidence of a causal link.

Gender comparisons between unilateral and bilateral landings.

PubMed

Weinhandl, Joshua T; Joshi, Mukta; O'Connor, Kristian M

2010-11-01

The increased number of women participating in sports has led to a higher knee injury rate in women compared with men. Among these injuries, those occurring to the ACL are commonly observed during landing maneuvers. The purpose of this study was to determine gender differences in landing strategies during unilateral and bilateral landings. Sixteen male and 17 female recreational athletes were recruited to perform unilateral and bilateral landings from a raised platform, scaled to match their individual jumping abilities. Three-dimensional kinematics and kinetics of the dominant leg were calculated during the landing phase and reported as initial ground contact angle, ranges of motion (ROM) and peak moments. Lower extremity energy absorption was also calculated for the duration of the landing phase. Results showed that gender differences were only observed in sagittal plane hip and knee ROM, potentially due to the use of a relative drop height versus the commonly used absolute drop height. Unilateral landings were characterized by significant differences in hip and knee kinematics that have been linked to increased injury risk and would best be classified as "stiff" landings. The ankle musculature was used more for impact absorption during unilateral landing, which required increased joint extension at touchdown and may increase injury risk during an unbalanced landing. In addition, there was only an 11% increase in total energy absorption during unilateral landings, suggesting that there was a substantial amount of passive energy transfer during unilateral landings.

Sudden Gains During Therapy of Social Phobia

PubMed Central

Hofmann, Stefan G.; Schulz, Stefan M.; Meuret, Alicia E.; Moscovitch, David A.; Suvak, Michael

2007-01-01

The present study investigated the phenomenon of sudden gains in 107 participants with social phobia (social anxiety disorder) who received either cognitive–behavioral group therapy or exposure group therapy without explicit cognitive interventions, which primarily used public speaking situations as exposure tasks. Twenty-two out of 967 session-to-session intervals met criteria for sudden gains, which most frequently occurred in Session 5. Individuals with sudden gains showed similar improvements in the 2 treatment groups. Although cognitive–behavioral therapy was associated with more cognitive changes than exposure therapy, cognitive changes did not precede sudden gains. In general, the results of this study question the clinical significance of sudden gains in social phobia treatment. PMID:16881776

Sudden unexpected death from natural diseases: Fifteen years' experience with 484 cases in Seychelles.

PubMed

Zhao, Peng; Wang, Ji-Gang; Gao, Peng; Li, Xia; Brewer, Rubell

2016-01-01

The aim of this study is to identify and subclassify sudden natural death (sudden death from natural diseases) cases in Seychelles. A total of 484 sudden natural death cases with autopsy at the Clinical Pathology Laboratory, Victoria Hospital, Seychelles between 1997 through 2012 were retrospectively reviewed. Among them, 363 cases (75%) were male and 121 (25%) were female. The most frequent sudden deaths were attributed to cardiovascular diseases (78.5%), and then followed by infectious diseases (9.9%), and gastrointestinal diseases (9.1%). This is the largest population-based study on sudden natural deaths in Seychelles. Copyright © 2015 Elsevier Ltd and Faculty of Forensic and Legal Medicine. All rights reserved.

Determinants of Autobiographical Memory in Patients with Unilateral Temporal Lobe Epilepsy or Excisions

ERIC Educational Resources Information Center

St-Laurent, Marie; Moscovitch, Morris; Levine, Brian; McAndrews, Mary Pat

2009-01-01

Patients with unilateral temporal lobe epilepsy from hippocampal origin and patients with unilateral surgical excision of an epileptic focus located in the medial temporal lobe were compared to healthy controls on a version of the Autobiographical Interview (AI) adapted to assess memory for event-specific and generic personal episodes. For both…

Children with Mild Bilateral and Unilateral Hearing Loss: Parents' Reflections on Experiences and Outcomes

ERIC Educational Resources Information Center

Fitzpatrick, Elizabeth; Grandpierre, Viviane; Durieux-Smith, Andrée; Gaboury, Isabelle; Coyle, Doug; Na, Eunjung; Sallam, Nusaiba

2016-01-01

Children with mild bilateral and unilateral hearing loss are now commonly identified early through newborn hearing screening initiatives. There remains considerable uncertainty about how to support parents and about which services to provide for children with mild bilateral and unilateral hearing loss. The goal of this study was to learn about…

Motor Learning of a Bimanual Task in Children with Unilateral Cerebral Palsy

ERIC Educational Resources Information Center

Hung, Ya-Ching; Gordon, Andrew M.

2013-01-01

Children with unilateral cerebral palsy (CP) have been shown to improve their motor performance with sufficient practice. However, little is known about how they learn goal-oriented tasks. In the current study, 21 children with unilateral CP (age 4-10 years old) and 21 age-matched typically developed children (TDC) practiced a simple bimanual…

Proceedings of the sudden oak death second science symposium: the state of our knowledge

Treesearch

Susan J. Frankel; Patrick J. Shea; Michael I. Haverty

2006-01-01

The Sudden Oak Death Second Science Symposium provided a forum for current research on sudden oak death, caused by the exotic, quarantine pathogen, Phytophthora ramorum. Ninety papers and forty-six posters on the following sudden oak death/P. ramorum topics are included: biology, genetics, nursery and wildland management,...

Sudden Gains in Cognitive Therapy of Depression and Depression Relapse/Recurrence

ERIC Educational Resources Information Center

Tang, Tony Z.; DeRubeis, Robert J.; Hollon, Steven D.; Amsterdam, Jay; Shelton, Richard

2007-01-01

Cognitive therapy (CT) may have significant advantages over antidepressants in preventing depression relapses. Many CT patients experience sudden gains: large symptom improvement in 1 between-session interval. Past studies have associated CT sudden gains with in-session cognitive changes but not with life events. This study examined sudden gains…

Effect of cage design, supplemental posterior instrumentation and approach on primary stability of a lumbar interbody fusion - A biomechanical in vitro study.

PubMed

Schmoelz, Werner; Sandriesser, Sabrina; Loebl, Oded; Bauer, Marlies; Krappinger, Dietmar

2017-10-01

There are various techniques and approaches for lumbar interbody fusion differing in access, cage type and type of supplemental posterior instrumentation. While a transforaminal access usually includes a hemifacetectomy, the facet joint can be preserved with a more lateral extraforaminal access. The supplemental posterior instrumentation required for both fusion techniques is still debated. The purpose of the present study was to compare primary stability of the two accesses for two different cage types with none, unilateral and bilateral supplemental posterior instrumentation. Six monosegmental lumbar functional spinal units (FSUs) were included in each of the two groups, and subjected to a flexibility test. As cages, a newly designed cage was compared to a standard cage in the following states: (a) native, (b) stand-alone cage, (c) bilateral internal fixator, (d) unilateral internal fixator, (e) unilateral facetectomy+bilateral internal fixator, (f) unilateral facetectomy+unilateral internal fixator and (g) unilateral facetectomy with stand-alone cage. For comparison the range of motion was normalized to the native state and the effects of the facetectomy, cage type, and supplemental instrumentation was compared. Within the subject comparison showed a significantly higher flexibility for the unilateral facetectomy in all motion directions (p<0.001). In between subject comparison showed a significant effect of cage type on flexibility in flexion/extension (p=0.002) and lateral bending (p=0.028) but not in axial rotation (p=0.322). The type of supplemental posterior fixation had a significant effect on the flexibility in all motion directions (stand-alone>unilateral fixator>bilateral fixator). Cage design and approach type are affecting the primary stability of lumbar interbody fusion procedures while the type of posterior instrumentation is the most influencing factor. Copyright © 2017 Elsevier Ltd. All rights reserved.

LogMAR and Stereoacuity in Keratoconus Corrected with Spectacles and Rigid Gas-permeable Contact Lenses.

PubMed

Nilagiri, Vinay Kumar; Metlapally, Sangeetha; Kalaiselvan, Parthasarathi; Schor, Clifton M; Bharadwaj, Shrikant R

2018-04-01

This study showed an improvement in three-dimensional depth perception of subjects with bilateral and unilateral keratoconus with rigid gas-permeable (RGP) contact lens wear, relative to spectacles. This novel information will aid clinicians to consider RGP contact lenses as a management modality in keratoconic patients complaining of depth-related difficulties with their spectacles. The aim of this study was to systematically compare changes in logMAR acuity and stereoacuity from best-corrected spherocylindrical spectacles to RGP contact lenses in bilateral and unilateral keratoconus vis-à-vis age-matched control subjects. Monocular and binocular logMAR acuity and random-dot stereoacuity were determined in subjects with bilateral (n = 30; 18 to 24 years) and unilateral (n = 10; 18 to 24 years) keratoconus and 20 control subjects using standard psychophysical protocols. Median (25th to 75th interquartile range) monocular (right eye) and binocular logMAR acuity and stereoacuity improved significantly from spectacles to RGP contact lenses in the bilateral keratoconus cohort (P < .001). Only monocular logMAR acuity of affected eye and stereoacuity improved from spectacles to RGP contact lenses in the unilateral keratoconus cohort (P < .001). There was no significant change in the binocular logMAR acuity from spectacles to RGP contact lenses in the unilateral keratoconus cohort. The magnitude of improvement in binocular logMAR acuity and stereoacuity was also greater for the bilateral compared with the unilateral keratoconus cohort. All outcome measures of cases with RGP contact lenses remained poorer than control subjects (P < .001). Binocular resolution and stereoacuity improve from spectacles to RGP contact lenses in bilateral keratoconus, whereas only stereoacuity improves from spectacles to RGP contact lenses in unilateral keratoconus. The magnitude of improvement in visual performance is greater for the binocular compared with the unilateral keratoconus cohort.

Application of strict criteria in adrenal venous sampling increases the proportion of missed patients with unilateral disease who benefit from surgery for primary aldosteronism.

PubMed

Kline, Gregory; Leung, Alexander; So, Benny; Chin, Alex; Harvey, Adrian; Pasieka, Janice L

2018-06-01

Adrenal vein sampling (AVS) is intended to confirm unilateral forms of primary aldosteronism, which are amenable to surgical cure. Excessively strict AVS criteria to define lateralization may result in many patients incorrectly categorized as bilateral primary aldosteronism and opportunity for surgical cure missed. Retrospective review of an AVS-primary aldosteronism database in which surgical cases are verified by standardized outcomes. Having used 'less strict' AVS criteria for lateralization, we examined the distribution of AVS lateralization indices in our confirmed unilateral primary aldosteronism cases both with and without cosyntropin stimulation. The proportion of proven unilateral cases that would have been missed with stricter AVS interpretation criteria was calculated. Particular focus was given to the proportion of missed cases according to use of international guidelines. False-positive lateralization with 'less strict' interpretation was also calculated. Of 80 surgical primary aldosteronism cases, 10-23% would have been missed with AVS lateralization indices of 3 : 1 to 5 : 1, with or without cosyntropin. If strict selectivity indices (for confirmation of catheterization) were combined with strict lateralization indices, up to 70% of unilateral primary aldosteronism cases could have been missed. Use of Endocrine Society AVS guidelines would have missed 21-43% of proven unilateral cases. 'Less strict' AVS interpretation yielded one case (1.2%) of false lateralization. Excessively strict AVS interpretation criteria will result in a high rate of missed unilateral primary aldosteronism with subsequent loss of opportunity for intervention. Use of more lenient lateralization criteria will improve the detection rate of unilateral primary aldosteronism with very low false-positive rate.

Sudden death after pediatric heart transplantation: analysis of data from the Pediatric Heart Transplant Study Group.

PubMed

Daly, Kevin P; Chakravarti, Sujata B; Tresler, Margaret; Naftel, David C; Blume, Elizabeth D; Dipchand, Anne I; Almond, Christopher S

2011-12-01

Sudden death is a well-recognized complication of heart transplantation. Little is known about the incidence and risk factors for sudden death after transplant in children. The purpose of this study was to determine the incidence of and risk factors for sudden death. This retrospective multicenter cohort study used the Pediatric Heart Transplant Study Group (PHTS) database, an event-driven registry of children aged <18 at listing undergoing heart transplantation between 1993 and 2007. Standard Kaplan-Meier and parametric analyses were used for survival analysis. Multivariate analysis in the hazard-function domain was used to identify risk factors for sudden death after transplant. Of 604 deaths in 2,491 children who underwent heart transplantation, 94 (16%) were classified as sudden. Freedom from sudden death was 97% at 5 years, and the hazard for sudden death remained constant over time at 0.01 deaths/year. Multivariate risk factors associated with sudden death included black race (hazard ratio [HR], 2.6; p < 0.0001), United Network of Organ Sharing (UNOS) status 2 at transplant (HR, 1.8; p = 0.008), older age (HR, 1.4/10 years of age; p = 0.03), and an increased number of rejection episodes in the first post-transplant year (HR, 1.6/episode; p = 0.03). Sudden death accounts for 1 in 6 deaths after heart transplant in children. Older recipient age, recurrent rejection within the first year, black race, and UNOS status 2 at listing were associated with sudden death. Patients with 1 or more of these risk factors may benefit from primary prevention efforts. Copyright © 2011 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.

Haloperidol and sudden cardiac death in dementia: autopsy findings in psychiatric inpatients.

PubMed

Ifteni, Petru; Grudnikoff, Eugene; Koppel, Jeremy; Kremen, Neil; Correll, Christoph U; Kane, John M; Manu, Peter

2015-12-01

Treatment with haloperidol has been shown, in studies using death certificates and prescription files, to be associated with an excess of sudden cardiac deaths, and regulatory warnings highlight this risk in patients with dementia. We used autopsy findings to determine whether the rate of sudden cardiac death is greater in cases of unexpected deaths of patients with dementia treated with haloperidol. From 1989 through 2013, 1219 patients with a primary diagnosis of dementia with behavioral disturbance were admitted to a psychiatric hospital, and 65 (5.3%) died suddenly. Sixty-five patients (5.3%) died unexpectedly. Complete post-mortem examinations after the sudden death were performed in 55 (84.6%) patients. Twenty-seven of the autopsied cases (49.1%) had been treated with haloperidol orally (2.2 mg ± 2.1 mg/day), the only antipsychotic used in this cohort. Univariable comparisons and multivariable regression analyses compared the groups of patients with or without sudden cardiac death. The leading causes of death were sudden cardiac death (32.7%), myocardial infarction (25.5% of patients), pneumonia (23.6%), and stroke (10.9%). Patients with sudden cardiac death and those with anatomically established cause of death were similar regarding the use of haloperidol (p = 0.5). Sudden cardiac death patients were more likely to suffer from Alzheimer's dementia (p = 0.027) and to have a past history of heart disease (p = 0.0094), and less likely to have been treated with a mood stabilizer (p = 0.024), but none of these variables were independent predictors of sudden cardiac death. Autopsy data suggest that oral haloperidol is not associated with increased risk of sudden cardiac death in psychiatric inpatients with dementia. Copyright © 2015 John Wiley & Sons, Ltd.

Structural imaging biomarkers of sudden unexpected death in epilepsy

PubMed Central

Wandschneider, Britta; Koepp, Matthias; Scott, Catherine; Micallef, Caroline; Balestrini, Simona; Sisodiya, Sanjay M.; Thom, Maria; Harper, Ronald M.; Sander, Josemir W.; Vos, Sjoerd B.; Duncan, John S.; Lhatoo, Samden

2015-01-01

Sudden unexpected death in epilepsy is a major cause of premature death in people with epilepsy. We aimed to assess whether structural changes potentially attributable to sudden death pathogenesis were present on magnetic resonance imaging in people who subsequently died of sudden unexpected death in epilepsy. In a retrospective, voxel-based analysis of T1 volume scans, we compared grey matter volumes in 12 cases of sudden unexpected death in epilepsy (two definite, 10 probable; eight males), acquired 2 years [median, interquartile range (IQR) 2.8] before death [median (IQR) age at scanning 33.5 (22) years], with 34 people at high risk [age 30.5 (12); 19 males], 19 at low risk [age 30 (7.5); 12 males] of sudden death, and 15 healthy controls [age 37 (16); seven males]. At-risk subjects were defined based on risk factors of sudden unexpected death in epilepsy identified in a recent combined risk factor analysis. We identified increased grey matter volume in the right anterior hippocampus/amygdala and parahippocampus in sudden death cases and people at high risk, when compared to those at low risk and controls. Compared to controls, posterior thalamic grey matter volume, an area mediating oxygen regulation, was reduced in cases of sudden unexpected death in epilepsy and subjects at high risk. The extent of reduction correlated with disease duration in all subjects with epilepsy. Increased amygdalo-hippocampal grey matter volume with right-sided changes is consistent with histo-pathological findings reported in sudden infant death syndrome. We speculate that the right-sided predominance reflects asymmetric central influences on autonomic outflow, contributing to cardiac arrhythmia. Pulvinar damage may impair hypoxia regulation. The imaging findings in sudden unexpected death in epilepsy and people at high risk may be useful as a biomarker for risk-stratification in future studies. PMID:26264515

[Primary hyperaldosteronism due to unilateral adrenal hyperplasia with surgical resolution].

PubMed

Rubio-Puchol, O; Garzón-Pastor, S; Salom-Vendrell, C; Hernández-Mijares, A

Unilateral adrenal hyperplasia is a rare cause of primary hyperaldosteronism (around a 3%) that has surgical treatment. A case of a patient with hypertension resistant to conventional therapy in treatment with 7 drugs who presented with primary hyperaldosteronism due to unilateral adrenal hyperplasia is presented. A left adrenalectomy was performed, and the patient had a good clinical response, with no need of any drug after 2 years of surgery. Unilateral adrenal hyperplasia is a different entity and it is not an asymmetric variant of the bilateral adrenal hyperplasia. In the study of patients with primary hyperaldosteronism and imaging tests with absence of adenoma is a diagnosis that must be considered before cataloguing patients with bilateral adrenal hyperplasia and start a medical treatment, because unilateral adrenal hyperplasia would have a surgical resolution. Copyright © 2016 SEH-LELHA. Publicado por Elsevier España, S.L.U. All rights reserved.

Unilateral retinitis pigmentosa occurring in an individual with a mutation in the CLRN1 gene.

PubMed

Sim, Peng Yong; Jeganathan, V Swetha E; Wright, Alan F; Cackett, Peter

2018-03-15

This case report depicts the clinical course of a female patient with unilateral retinitis pigmentosa, who first presented at the age of 12 years. Fundus photography at the time revealed unilateral pigmentary retinopathy, which was associated with extinguished electroretinogram (ERG) signal. At 35 years of age, fundus examination revealed deterioration of pre-existing unilateral pigmentary retinopathy with progressive visual field defect detected on Goldmann visual field testing. ERG findings remained unchanged and multifocal ERG showed unilateral decrease in amplitude in the affected eye. The patient was referred for genetic counselling. Next-generation sequencing identified a deleterious heterozygous c.118T>G (p.Cys40Gly) mutation in the CLRN1 gene. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Unilateral occipital nerve stimulation for bilateral occipital neuralgia: a case report and literature review

PubMed Central

Liu, Aijun; Jiao, Yongcheng; Ji, Huijun; Zhang, Zhiwen

2017-01-01

Objectives The aim of this study is to present a case of successful relief of bilateral occipital neuralgia (ON) using unilateral occipital nerve stimulation (ONS) and to discuss the possible underlying mechanisms. Materials and methods We present the case of a 59-year-old female patient with severe bilateral ON treated with unilateral ONS. We systematically reviewed previous studies of ONS for ON, discussing the possible mechanisms of ONS in the relief of ON. Results The patient reported complete pain relief after consistent unilateral ONS during the follow-up period. The underlying mechanisms may be linked to the relationship between pain and several brain regions, including the pons, midbrain, and periaqueductal gray. Conclusion ONS is an effective and safe option for treating ON. Future studies will be required to clarify the mechanisms by which unilateral occipital stimulation provided relief for bilateral neuralgia in this case. PMID:28176938

Unilateral occipital nerve stimulation for bilateral occipital neuralgia: a case report and literature review.

PubMed

Liu, Aijun; Jiao, Yongcheng; Ji, Huijun; Zhang, Zhiwen

2017-01-01

The aim of this study is to present a case of successful relief of bilateral occipital neuralgia (ON) using unilateral occipital nerve stimulation (ONS) and to discuss the possible underlying mechanisms. We present the case of a 59-year-old female patient with severe bilateral ON treated with unilateral ONS. We systematically reviewed previous studies of ONS for ON, discussing the possible mechanisms of ONS in the relief of ON. The patient reported complete pain relief after consistent unilateral ONS during the follow-up period. The underlying mechanisms may be linked to the relationship between pain and several brain regions, including the pons, midbrain, and periaqueductal gray. ONS is an effective and safe option for treating ON. Future studies will be required to clarify the mechanisms by which unilateral occipital stimulation provided relief for bilateral neuralgia in this case.

Does cochlear implantation and electrical stimulation affect residual hair cells and spiral ganglion neurons?

PubMed Central

Coco, Anne; Epp, Stephanie B.; Fallon, James B.; Xu, Jin; Millard, Rodney E.; Shepherd, Robert K.

2007-01-01

Increasing numbers of cochlear implant subjects have some level of residual hearing at the time of implantation. The present study examined whether (i) hair cells that have survived one pathological insult (aminoglycoside deafening), can survive and function following long-term cochlear implantation and electrical stimulation (ES); and (ii) chronic ES in these cochleae results in greater trophic support of spiral ganglion neurons (SGNs) compared with cochleae devoid of hair cells. Eight cats, with either partial (n=4) or severe (n=4) sensorineural hearing loss, were bilaterally implanted with scala tympani electrode arrays 2 months after deafening, and received unilateral ES using charge balanced biphasic current pulses for periods of up to 235 days. Frequency-specific compound action potentials and click-evoked auditory brainstem responses (ABRs) were recorded periodically to monitor the residual acoustic hearing. Electrically-evoked ABRs (EABRs) were recorded to confirm the stimulus levels were 3-6 dB above the EABR threshold. On completion of the ES program the cochleae were examined histologically. Partially deafened animals showed no significant increase in acoustic thresholds over the implantation period. Moreover, chronic ES of an electrode array located in the base of the cochlea did not adversely affect hair cells in the middle or apical turns. There was evidence of a small but statistically significant rescue of SGNs in the middle and apical turns of stimulated cochleae in animals with partial hearing. Chronic ES did not, however, prevent a reduction in SGN density for the severely deaf cohort, although SGNs adjacent to the stimulating electrodes did exhibit a significant increase in soma area (p<0.01). In sum, chronic ES in partial hearing animals does not adversely affect functioning residual hair cells apical to the electrode array. Moreover, while there is an increase in the soma area of SGNs close to the stimulating electrodes in severely deaf cochleae, this trophic effect does not result in increased SGN survival. PMID:17258411

Clinical, Radiologic, and Legal Significance of "Extensor Response" in Posttraumatic Coma.

PubMed

Firsching, Raimund; Woischneck, Dieter; Langejürgen, Alexander; Parreidt, Andreas; Bondar, Imre; Skalej, Martin; Röhl, Friedrich; Voellger, Benjamin

2015-11-01

The timely detection of neurologic deterioration can be critical for the survival of a neurosurgical patient following head injury. Because little reliable evidence is available on the prognostic value of the clinical sign "extensor response" in comatose posttraumatic patients, we investigated the correlation of this clinical sign with outcome and with early radiologic findings from magnetic resonance imaging (MRI). This retrospective analysis of prospectively obtained data included 157 patients who had remained in a coma for a minimum of 24 hours after traumatic brain injury. All patients received a 1.5-T MRI within 10 days (median: 2 days) of the injury. The correlations between clinical findings 12 and 24 hours after the injury-in particular, extensor response and pupillary function, MRI findings, and outcome after 1 year-were investigated. Statistical analysis included contingency tables, Fisher exact test, odds ratios (ORs) with confidence intervals (CIs), and weighted κ values. There were 48 patients with extensor response within the first 24 hours after the injury. Patients with extensor response (World Federation of Neurosurgical Societies coma grade III) statistically were significantly more likely to harbor MRI lesions in the brainstem when compared with patients in a coma who had no further deficiencies (coma grade I; p = 0.0004 by Fisher exact test, OR 10.8 with 95% CI, 2.7-42.5) and patients with unilateral loss of pupil function (coma grade II; p = 0.0187, OR 2.8 with 95% CI, 1.2-6.5). The correlation of brainstem lesions as found by MRI and outcome according to the Glasgow Outcome Scale after 1 year was also highly significant (p ≤ 0.016). The correlation of extensor response and loss of pupil function with an unfavorable outcome and with brainstem lesions revealed by MRI is highly significant. Their sudden onset may be associated with the sudden onset of brainstem dysfunction and should therefore be regarded as one of the most fundamental warning signs in the clinical monitoring of comatose patients. Georg Thieme Verlag KG Stuttgart · New York.

Strategies for Working with Children with Cochlear Implants

ERIC Educational Resources Information Center

Schraer-Joiner, Lyn; Prause-Weber, Manuela

2009-01-01

According to the National Institute on Deafness and Other Communication Disorders, 23,000 individuals in the United States, including 10,000 children, have a cochlear implant. This biomedical electronic device has been a breakthrough in the auditory rehabilitation of individuals diagnosed with severe or profound sensorineural hearing losses who…

Congenital Deafness with Cardiac Arrhythmias: The Jervell and Lange-Nielsen Syndrome.

ERIC Educational Resources Information Center

Wahl, Richard A.; Macdonald, Dick, II

1980-01-01

The Jervell and Lange-Nielsen syndrome, affecting 0.3 percent of congenitally deaf persons, consists of severe cardiac arrhythmias and sensorineural hearing loss. The authors recommend that every congenitally deaf child with suspicious symptoms receive an electrocardiogram and that professionals who work with deaf children not only inform…

Genetic and Pharmacological Intervention for Treatment/Prevention of Hearing Loss

ERIC Educational Resources Information Center

Cotanche, Douglas A.

2008-01-01

Twenty years ago it was first demonstrated that birds could regenerate their cochlear hair cells following noise damage or aminoglycoside treatment. An understanding of how this structural and functional regeneration occurred might lead to the development of therapies for treatment of sensorineural hearing loss in humans. Recent experiments have…

Future Approaches for Inner Ear Protection and Repair

ERIC Educational Resources Information Center

Shibata, Seiji B.; Raphael, Yehoash

2010-01-01

Health care professionals tending to patients with inner ear disease face inquiries about therapy options, including treatments that are being developed for future use but not yet available. The devastating outcome of sensorineural hearing loss, combined with the permanent nature of the symptoms, make these inquiries demanding and frequent. The…

Working Memory and Speech Comprehension in Older Adults with Hearing Impairment

ERIC Educational Resources Information Center

Nagaraj, Naveen K.

2017-01-01

Purpose: This study examined the relationship between working memory (WM) and speech comprehension in older adults with hearing impairment (HI). It was hypothesized that WM would explain significant variance in speech comprehension measured in multitalker babble (MTB). Method: Twenty-four older (59-73 years) adults with sensorineural HI…

Hearing loss in a glue sniffer.

PubMed

Williams, D M

1988-10-01

A case is presented of a 27-year-old glue sniffing woman with sensorineural hearing loss, optic atrophy and global brain damage. This form of addiction has not received much attention as a cause of otologic catastrophes, and should be borne in mind where similar cases come to the otolaryngologist.

Binaural Loudness Summation in the Hearing Impaired.

ERIC Educational Resources Information Center

Hawkins, David B.; And Others

1987-01-01

Binaural loudness summation was measured using three different paradigms with 10 normally hearing and 20 bilaterally symmetrical high-frequency sensorineural hearing loss subjects. Binaural summation increased with presentation level using the loudness matching procedure, with values in the 6-10 dB range. Summation decreased with level using the…

Grip Force Coordination during Bimanual Tasks in Unilateral Cerebral Palsy

ERIC Educational Resources Information Center

Islam, Mominul; Gordon, Andrew M.; Skold, Annika; Forssberg, Hans; Eliasson, Ann-Christin

2011-01-01

Aim: The aim of the study was to investigate coordination of fingertip forces during an asymmetrical bimanual task in children with unilateral cerebral palsy (CP). Method: Twelve participants (six males, six females; mean age 14y 4mo, SD 3.3y; range 9-20y;) with unilateral CP (eight right-sided, four left-sided) and 15 age-matched typically…

Hand Function in Relation to Brain Lesions and Corticomotor-Projection Pattern in Children with Unilateral Cerebral Palsy

ERIC Educational Resources Information Center

Holmstrom, Linda; Vollmer, Brigitte; Tedroff, Kristina; Islam, Mominul; Persson, Jonas Ke; Kits, Annika; Forssberg, Hans; Eliasson, Ann-Christin

2010-01-01

Aim: To investigate relationships between hand function, brain lesions, and corticomotor projections in children with unilateral cerebral palsy (CP). Method: The study included 17 children (nine males, eight females; mean age 11.4 [SD 2.4] range 7-16y), with unilateral CP at Gross Motor Function Classification System level I and Manual Ability…

Song Recognition by Young Children with Cochlear Implants: Comparison between Unilateral, Bilateral, and Bimodal Users

ERIC Educational Resources Information Center

Bartov, Tamar; Most, Tova

2014-01-01

Purpose: To examine song identification by preschoolers with normal hearing (NH) versus preschoolers with cochlear implants (CIs). Method: Participants included 45 children ages 3;8-7;3 (years;months): 12 with NH and 33 with CIs, including 10 with unilateral CI, 14 with bilateral CIs, and 9 bimodal users (CI-HA) with unilateral CI and…

The effect of severity of unilateral vestibular dysfunction on symptoms, disabilities and handicap in vertiginous patients.

PubMed

Bamiou, D E; Davies, R A; McKee, M; Luxon, L M

1999-02-01

This study compares the symptoms, disabilities and handicap, as assessed by means of a questionnaire, in two groups of patients with a unilateral peripheral vestibular disorder: those with a total canal paresis and those with a partial canal paresis, as judged by the duration parameter using the Fitzgerald Hallpike caloric test in the absence of optic fixation. The results of the study indicate that the severity of dizziness, the Dizziness Index (severity x frequency) and the overall level of disabilities related to visual vertigo are less severe in unilateral profound or total loss of vestibular function than in unilateral mild vestibular loss.

A stimulus-centered reading disorder for words and numbers: Is it neglect dyslexia?

PubMed

Arduino, Lisa S; Daini, Roberta; Caterina Silveri, Maria

2005-12-01

A single case, RCG, showing a unilateral reading disorder without unilateral spatial neglect was studied. The disorder was characterized by substitutions of the initial (left) letters of words, nonwords and Arabic numbers, independently of egocentered spatial coordinates. MRI showed a bilateral lesion with the involvement of the splenium. Although, within the framework of the visual word recognition model proposed by Caramazza and Hillis (1990), RCG disorder could be defined as a stimulus-centered neglect dyslexia, we discuss the hypothesis of a dissociation in neural correlates and mechanisms between the syndrome of unilateral spatial neglect and such a unilateral reading disorder.

Unilateral lesion of dorsal hippocampus in adult rats impairs contralateral long-term potentiation in vivo and spatial memory in the early postoperative phase.

PubMed

Li, Hongjie; Wu, Xiaoyan; Bai, Yanrui; Huang, Yan; He, Wenting; Dong, Zhifang

2012-05-01

It is well documented that bilateral hippocampal lesions or unilateral hippocampal lesion at birth causes impairment of contralateral LTP and long-term memory. However, effects of unilateral hippocampal lesion in adults on contralateral in vivo LTP and memory are not clear. We here examined the influence of unilateral electrolytic dorsal hippocampal lesion in adult rats on contralateral LTP in vivo and spatial memory during different postoperative phases. We found that acute unilateral hippocampal lesion had no effect on contralateral LTP. However, contralateral LTP was impaired at 1 week after lesion, and was restored to the control level at postoperative week 4. Similarly, spatial memory was also impaired at postoperative week 1, and was restored at postoperative week 4. In addition, the rats at postoperative week 1 showed stronger spatial exploratory behavior in a novel open-field environment. The sham operation had no effects on contralateral LTP, spatial memory and exploration at either postoperative week 1 or week 4. These results suggest that unilateral dorsal hippocampal lesion in adult rats causes transient contralateral LTP impairment and spatial memory deficit. Copyright © 2012 Elsevier B.V. All rights reserved.

Intracortical inhibition and facilitation with unilateral dominant, unilateral nondominant and bilateral movement tasks in left- and right-handed adults.

PubMed

McCombe Waller, Sandy; Forrester, Larry; Villagra, Federico; Whitall, Jill

2008-06-15

To investigate intracortical inhibition and facilitation in response to unilateral dominant, nondominant and bilateral biceps activation and short-term upper extremity training in right- and left-handed adults. Paired-pulse transcranial magnetic stimulation was used to measure intracortical excitability in motor dominant and nondominant cortices of 26 nondisabled adults. Neural facilitation and inhibition were measured in each hemisphere during unilateral dominant, nondominant and bilateral arm activation and after training in each condition. No differences were seen between right- and left-handed subjects. Intracortical facilitation and decreased inhibition were seen in each hemisphere with unilateral activation/training of contralateral muscles and bilateral muscle activation/training. Persistent intracortical inhibition was seen in each hemisphere with ipsilateral muscle activation/training. Inhibition was greater in the nondominant hemisphere during dominant hemisphere activation (dominant arm contraction). Strongly dominant individuals show no difference in intracortical responses given handedness. Intracortical activity with unilateral and bilateral arm activation and short-term training differs based on hemispheric dominance, with the motor dominant hemisphere exerting a larger inhibitory influence over the nondominant hemisphere. Bilateral activation and training have a disinhibitory effect in both dominant and nondominant hemispheres.

Forced in-plane vibration of a thick ring on a unilateral elastic foundation

NASA Astrophysics Data System (ADS)

Wang, Chunjian; Ayalew, Beshah; Rhyne, Timothy; Cron, Steve; Dailliez, Benoit

2016-10-01

Most existing studies of a deformable ring on elastic foundation rely on the assumption of a linear foundation. These assumptions are insufficient in cases where the foundation may have a unilateral stiffness that vanishes in compression or tension such as in non-pneumatic tires and bushing bearings. This paper analyzes the in-plane dynamics of such a thick ring on a unilateral elastic foundation, specifically, on a two-parameter unilateral elastic foundation, where the stiffness of the foundation is treated as linear in the circumferential direction but unilateral (i.e. collapsible or tensionless) in the radial direction. The thick ring is modeled as an orthotropic and extensible circular Timoshenko beam. An arbitrarily distributed time-varying in-plane force is considered as the excitation. The Equations of Motion are explicitly derived and a solution method is proposed that uses an implicit Newmark scheme for the time domain solution and an iterative compensation approach to determine the unilateral zone of the foundation at each time step. The dynamic axle force transmission is also analyzed. Illustrative forced vibration responses obtained from the proposed model and solution method are compared with those obtained from a finite element model.

Corneal thickness of eyes with unilateral age-related macular degeneration.

PubMed

Arikan, Sedat; Ersan, Ismail; Kara, Selcuk; Gencer, Baran; Korkmaz, Safak; Vural, Azer Sara

2015-01-01

To compare the central corneal thicknesses (CCT), peripheral corneal thicknesses, and corneal volumes (CV) of the 2 eyes of patients with unilateral age-related macular degeneration (AMD). Twenty patients who were diagnosed with unilateral AMD were included in this prospective study for the purpose of making comparison between the diseased and healthy eyes. Optical coherence tomography and fundus fluorescein angiography imaging were applied to all patients in order to confirm and reveal the presence of unilateral AMD. Then, the measurements of CCT, peripheral corneal thickness measured 4 mm distant from the center of the cornea (4 mm CT), and CV of each eye of these patients were obtained through the rotating Scheimpflug corneal topographer. Wilcoxon signed-rank test did not demonstrate a statistically significant difference between the 2 eyes of patients with unilateral AMD when we compared the CCT and CV of diseased and healthy eyes (p>0.05). However, 4 mm CT of the diseased eyes of these patients were statistically significantly thicker than the healthy eyes (p<0.05). The significant difference in terms of 4 mm CT between the diseased and healthy eyes of patients with unilateral AMD may demonstrate the possible effect of peripheral corneal thickness on the development of AMD.

Increased incidence of pseudarthrosis after unilateral instrumented transforaminal lumbar interbody fusion in patients with lumbar spondylosis: Clinical article.

PubMed

Gologorsky, Yakov; Skovrlj, Branko; Steinberger, Jeremy; Moore, Max; Arginteanu, Marc; Moore, Frank; Steinberger, Alfred

2014-10-01

Transforaminal lumbar interbody fusion (TLIF) with segmental pedicular instrumentation is a well established procedure used to treat lumbar spondylosis with or without spondylolisthesis. Available biomechanical and clinical studies that compared unilateral and bilateral constructs have produced conflicting data regarding patient outcomes and hardware complications. A prospective cohort study was undertaken by a group of neurosurgeons. They prospectively enrolled 80 patients into either bilateral or unilateral pedicle screw instrumentation groups (40 patients/group). Demographic data collected for each group included sex, age, body mass index, tobacco use, and Workers' Compensation/litigation status. Operative data included segments operated on, number of levels involved, estimated blood loss, length of hospital stay, and perioperative complications. Long-term outcomes (hardware malfunction, wound dehiscence, and pseudarthrosis) were recorded. For all patients, preoperative baseline and 6-month postoperative scores for Medical Outcomes 36-Item Short Form Health Survey (SF-36) outcomes were recorded. Patient follow-up times ranged from 37 to 63 months (mean 52 months). No patients were lost to follow-up. The patients who underwent unilateral pedicle screw instrumentation (unilateral cohort) were slightly younger than those who underwent bilateral pedicle screw instrumentation (bilateral cohort) (mean age 42 vs. 47 years, respectively; p = 0.02). No other significant differences were detected between cohorts with regard to demographic data, mean number of lumbar levels operated on, or distribution of the levels operated on. Estimated blood loss was higher for patients in the bilateral cohort, but length of stay was similar for patients in both cohorts. The incidence of pseudarthrosis was significantly higher among patients in the unilateral cohort (7 patients [17.5%]) than among those in the bilateral cohort (1 patient [2.5%]) (p = 0.02). Wound dehiscence occurred for 1 patient in the unilateral cohort. Reoperation was offered to 8 patients in the unilateral cohort and 1 patient in the bilateral cohort (p = 0.03). The physical component scores of the Medical Outcomes SF-36 outcomes improved significantly for all patients (p < 0.001). Transforaminal lumbar interbody fusion with either unilateral or bilateral segmental pedicular instrumentation is an effective treatment for lumbar spondylosis. Because patients with unilateral constructs were 7 times more likely to experience pseudarthrosis and require reoperation, TLIF with bilateral constructs might be the biomechanically superior technique.

Auditory Performance and Electrical Stimulation Measures in Cochlear Implant Recipients With Auditory Neuropathy Compared With Severe to Profound Sensorineural Hearing Loss.

PubMed

Attias, Joseph; Greenstein, Tally; Peled, Miriam; Ulanovski, David; Wohlgelernter, Jay; Raveh, Eyal

The aim of the study was to compare auditory and speech outcomes and electrical parameters on average 8 years after cochlear implantation between children with isolated auditory neuropathy (AN) and children with sensorineural hearing loss (SNHL). The study was conducted at a tertiary, university-affiliated pediatric medical center. The cohort included 16 patients with isolated AN with current age of 5 to 12.2 years who had been using a cochlear implant for at least 3.4 years and 16 control patients with SNHL matched for duration of deafness, age at implantation, type of implant, and unilateral/bilateral implant placement. All participants had had extensive auditory rehabilitation before and after implantation, including the use of conventional hearing aids. Most patients received Cochlear Nucleus devices, and the remainder either Med-El or Advanced Bionics devices. Unaided pure-tone audiograms were evaluated before and after implantation. Implantation outcomes were assessed by auditory and speech recognition tests in quiet and in noise. Data were also collected on the educational setting at 1 year after implantation and at school age. The electrical stimulation measures were evaluated only in the Cochlear Nucleus implant recipients in the two groups. Similar mapping and electrical measurement techniques were used in the two groups. Electrical thresholds, comfortable level, dynamic range, and objective neural response telemetry threshold were measured across the 22-electrode array in each patient. Main outcome measures were between-group differences in the following parameters: (1) Auditory and speech tests. (2) Residual hearing. (3) Electrical stimulation parameters. (4) Correlations of residual hearing at low frequencies with electrical thresholds at the basal, middle, and apical electrodes. The children with isolated AN performed equally well to the children with SNHL on auditory and speech recognition tests in both quiet and noise. More children in the AN group than the SNHL group were attending mainstream educational settings at school age, but the difference was not statistically significant. Significant between-group differences were noted in electrical measurements: the AN group was characterized by a lower current charge to reach subjective electrical thresholds, lower comfortable level and dynamic range, and lower telemetric neural response threshold. Based on pure-tone audiograms, the children with AN also had more residual hearing before and after implantation. Highly positive coefficients were found on correlation analysis between T levels across the basal and midcochlear electrodes and low-frequency acoustic thresholds. Prelingual children with isolated AN who fail to show expected oral and auditory progress after extensive rehabilitation with conventional hearing aids should be considered for cochlear implantation. Children with isolated AN had similar pattern as children with SNHL on auditory performance tests after cochlear implantation. The lower current charge required to evoke subjective and objective electrical thresholds in children with AN compared with children with SNHL may be attributed to the contribution to electrophonic hearing from the remaining neurons and hair cells. In addition, it is also possible that mechanical stimulation of the basilar membrane, as in acoustic stimulation, is added to the electrical stimulation of the cochlear implant.

Sudden transition and sudden change from open spin environments

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hu, Zheng-Da; School of Science, Jiangnan University, Wuxi 214122; Xu, Jing-Bo, E-mail: xujb@zju.edu.cn

2014-11-15

We investigate the necessary conditions for the existence of sudden transition or sudden change phenomenon for appropriate initial states under dephasing. As illustrative examples, we study the behaviors of quantum correlation dynamics of two noninteracting qubits in independent and common open spin environments, respectively. For the independent environments case, we find that the quantum correlation dynamics is closely related to the Loschmidt echo and the dynamics exhibits a sudden transition from classical to quantum correlation decay. It is also shown that the sudden change phenomenon may occur for the common environment case and stationary quantum discord is found at themore » high temperature region of the environment. Finally, we investigate the quantum criticality of the open spin environment by exploring the probability distribution of the Loschmidt echo and the scaling transformation behavior of quantum discord, respectively. - Highlights: • Sudden transition or sudden change from open spin baths are studied. • Quantum discord is related to the Loschmidt echo in independent open spin baths. • Steady quantum discord is found in a common open spin bath. • The probability distribution of the Loschmidt echo is analyzed. • The scaling transformation behavior of quantum discord is displayed.« less

Sudden Infant Death Syndrome: Facts for Caregivers.

ERIC Educational Resources Information Center

Texas Child Care, 2000

2000-01-01

Presents risk factors and prevention measures related to Sudden Infant Death Syndrome. Offers infant sleep recommendations and five discussion questions to test knowledge of Sudden Infant Death Syndrome. (DLH)

Longitudinal Development of Hand Function in Children with Unilateral Cerebral Palsy

ERIC Educational Resources Information Center

Holmefur, Marie; Krumlinde-Sundholm, Lena; Bergstrom, Jakob; Eliasson, Ann-Christin

2010-01-01

Aim: The aim of this study was to describe how the usefulness of the hemiplegic hand develops in children with unilateral cerebral palsy (CP) aged between 18 months and 8 years. Method: A prospective longitudinal study of 43 children (22 males, 21 females) with unilateral CP was conducted. Inclusion age was 18 months to 5 years 4 months (mean 2y…

Visual Field Function in School-Aged Children with Spastic Unilateral Cerebral Palsy Related to Different Patterns of Brain Damage

ERIC Educational Resources Information Center

Jacobson, Lena; Rydberg, Agneta; Eliasson, Ann-Christin; Kits, Annika; Flodmark, Olof

2010-01-01

Aim: To relate visual field function to brain morphology in children with unilateral cerebral palsy (CP). Method: Visual field function was assessed using the confrontation technique and Goldmann perimetry in 29 children (15 males, 14 females; age range 7-17y, median age 11y) with unilateral CP classified at Gross Motor Function Classification…

Gerstmann's syndrome and unilateral optic ataxia in the emergency department

PubMed Central

Barbosa, Breno José Alencar Pires; de Brito, Marcelo Houat; Rodrigues, Júlia Chartouni; Kubota, Gabriel Taricani; Parmera, Jacy Bezerra

2017-01-01

ABSTRACT. A 75-year-old right-handed woman presented to the emergency department with simultanagnosia and right unilateral optic ataxia. Moreover, the patient had agraphia, acalculia, digital agnosia and right-left disorientation, consistent with complete Gerstmann's syndrome. This case highlights the concurrence of Gerstmann's syndrome and unilateral optic ataxia in the acute phase of a left middle cerebral artery stroke. PMID:29354229

Effect of Observation of Simple Hand Movement on Brain Activations in Patients with Unilateral Cerebral Palsy: An fMRI Study

ERIC Educational Resources Information Center

Dinomais, Mickael; Lignon, Gregoire; Chinier, Eva; Richard, Isabelle; Minassian, Aram Ter; The Tich, Sylvie N'Guyen

2013-01-01

The aim of this functional magnetic resonance imaging (fMRI) study was to examine and compare brain activation in patients with unilateral cerebral palsy (CP) during observation of simple hand movement performed by the paretic and nonparetic hand. Nineteen patients with clinical unilateral CP (14 male, mean age 14 years, 7-21 years) participated…

Postero-Lateral Disc Prosthesis Combined With a Unilateral Facet Replacement Device Maintains Quantity and Quality of Motion at a Single Lumbar Level

PubMed Central

Nayak, Aniruddh N.; Doarn, Michael C.; Gaskins, Roger B.; James, Chris R.; Cabezas, Andres F.; Castellvi, Antonio E.

2014-01-01

Background Mechanically replacing one or more pain generating articulations in the functional spinal unit (FSU) may be a motion preservation alternative to arthrodesis at the affected level. Baseline biomechanical data elucidating the quantity and quality of motion in such arthroplasty constructs is non-existent. Purpose The purpose of the study was to quantify the motion-preserving effect of a posterior total disc replacement (PDR) combined with a unilateral facet replacement (FR) system at a single lumbar level (L4-L5). We hypothesized that reinforcement of the FSU with unilateral FR to replace the resected, native facet joint following PDR implantation would restore quality and quantity of motion and additionally not change biomechanics at the adjacent levels. Study Design In-vitro study using human cadaveric lumbar spines. Methods Six (n = 6) cadaveric lumbar spines (L1-S1) were evaluated using a pure-moment stability testing protocol (±7.5 Nm) in flexion-extension (F/E), lateral bending (LB) and axial rotation (AR). Each specimen was tested in: (1) intact; (2) unilateral FR; and (3) unilateral FR + PDR conditions. Index and adjacent level ROM (using hybrid protocol) were determined opto-electronically. Interpedicular travel (IPT) and instantaneous center of rotation (ICR) at the index level were radiographically determined for each condition. ROM, ICR, and IPT measurements were compared (repeated measures ANOVA) between the three conditions. Results Compared to the intact spine, no significant changes in F/E, LB or AR ROM were identified as a result of unilateral FR or unilateral FR + PDR. No significant changes in adjacent L3-L4 or L5-S1 ROM were identified in any loading mode. No significant differences in IPT were identified between the three test conditions in F/E, LB or AR at the L4-L5 level. The ICRs qualitatively were similar for the intact and unilateral FR conditions and appeared to follow placement (along the anterior-posterior (AP) direction) of the PDR in the disc space Conclusion Biomechanically, quantity and quality of motion are maintained with combined unilateral FR + PDR at a single lumbar spinal level. PMID:25694929

Sudden cardiac death in adults: causes, incidence and interventions.

PubMed

Walker, Wendy Marina

Many nurses will be familiar with the unexpected death of an adult patient following a sudden, life-threatening cardiac event. It is a situation that demands sensitive nursing care and skilled interventions to provide a foundation for recovery and promote healthy bereavement. This article examines the causes and incidence of sudden cardiac death in adults. Possible reactions of those who are suddenly bereaved are described and immediate care interventions aimed at dealing with the grief process are discussed. The article concludes by identifying ways in which the incidence of sudden cardiac death may be reduced.

[Effects on cervical spondylosis of vertebral artery type and the concentrations of plasma NPY and UII in the patients treated with the modified acupuncture at unilateral/bilateral Renying (ST 9)].

PubMed

Wang, Yanfu; Ma, Chaoyang; Li, Lingxiao; Zhang, Ting; Gui, Xinghua; Chen, Hao

2018-05-12

To observe the differences in the clinical therapeutic effects on cervical spondylosis of vertebral artery type (CSA) between the modified acupuncture and the routine acupuncture at unilateral/bilateral Renying (ST 9) as well as the impacts on the concentrations of plasma neuropeptide Y (NPY) and urotensinⅡ(UⅡ) in the patients. A total of 160 patients were divided into a modified bilateral acupuncture group, a modified unilateral acupuncture group, a routine bilateral acupuncture group and a routine unilateral acupuncture group, 40 cases in each one according to the random number table. In the modified bilateral acupuncture group, the modified acupuncture was applied bilaterally to Renying (ST 9). In the modified unilateral acupuncture group, the modified acupuncture was applied unilaterally to Renying (ST 9). In the routine bilateral acupuncture group, the routine acupuncture was applied bilaterally to Renying (ST 9). In the routine unilateral acupuncture group, the routine acupuncture was applied unilaterally to Renying (ST 9). The treatment was given once every day, continuously for 6 days as one course. Two courses of treatment were required at the interval of 1 day. In each group, before and after treatment, we observed the peak systolic blood flow velocity (Vs) of the vertebral artery (VA) and the basilar artery (BA), cervical vertigo symptoms and functional assessment scales (ESCV) and the concentration of plasma NPY and UⅡ. The clinical therapeutic effects were compared among the groups. After treatment, the clinical therapeutic effect in the modified bilateral acupuncture group was 90.0% (36/40), which was better than 80.0% (32/40) in the modified unilateral acupuncture group, 77.5% (35/40) in the routine bilateral acupuncture group and 65.0% (26/40) in the routine unilateral acupuncture group (all P <0.05). After treatment, Vs of VA and BA was improved remarkably in every group (all P <0.01), and the result in the modified bilateral acupuncture group was higher than those in the other groups (all P <0.01). After treatment, ESCV scores were all increased remarkably in every group (all P <0.01). ESCV score and improvement index in the modified bilateral acupuncture group were all higher than those in the other groups ( P <0.05, P <0.01). After treatment, the concentrations of plasma NPY and UⅡ were all reduced remarkably in every group (all P <0.01) and the differences were significant among the groups (all P <0.01). The modified bilateral acupuncture at Renying (ST 9) effectively regulates the blood supply of the vertebral basilar artery and improves the cerebral circulation. The effects are superior to those of the unilateral acupuncture at Renying (ST 9).

Asymmetric vestibular evoked myogenic potentials in unilateral Menière patients

PubMed Central

Wit, H. P.

2010-01-01

Vestibular evoked myogenic potentials (VEMPs) were measured in 22 unilateral Menière patients with monaural and binaural stimulation with 250 and 500 Hz tone bursts. For all measurement situations significantly lower VEMP amplitudes were on average measured at the affected side compared to the unaffected side. Unilateral Menière patients have, in contrast to normal subjects, asymmetric VEMPs, indicating a permanently affected vestibular (most likely otolith) system at the side of hearing loss. The diagnostic value of VEMP amplitude asymmetry measurement in individual patients is low, because of the large overlap of the VEMP amplitude asymmetry range for unilateral Menière patients with that for normal subjects. PMID:20665043

Unilateral RS3PE in a Patient of Seronegative Rheumatoid Arthritis.

PubMed

Varshney, Ankur Nandan; Kumar, Nilesh; Tiwari, Ashutosh; Anand, Ravi; Prasad, Sashi Ranjan; Anand, Arvind; Mishra, Abhinandan; Singh, N K

2013-01-01

Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare but well-reported clinical entity. It is classically described as symmetrical involvement of both upper extremities. Asymmetrical involvement had also been reported, but unilateral presentation is very rare. We hereby report a case of unilateral RS3PE in a patient of seronegative rheumatoid arthritis which was initially misdiagnosed as cellulitis and was given high dose antibiotics without any significant improvement. Later a rheumatologic consultation leads to a prompt diagnosis, and treatment with steroids leads to dramatic reversal of symptoms. This case demonstrates the rare presentation of this rare clinical entity and highlights the necessity of awareness regarding unilateral disease to clinicians.

Declining Risk of Sudden Death in Heart Failure.

PubMed

Shen, Li; Jhund, Pardeep S; Petrie, Mark C; Claggett, Brian L; Barlera, Simona; Cleland, John G F; Dargie, Henry J; Granger, Christopher B; Kjekshus, John; Køber, Lars; Latini, Roberto; Maggioni, Aldo P; Packer, Milton; Pitt, Bertram; Solomon, Scott D; Swedberg, Karl; Tavazzi, Luigi; Wikstrand, John; Zannad, Faiez; Zile, Michael R; McMurray, John J V

2017-07-06

The risk of sudden death has changed over time among patients with symptomatic heart failure and reduced ejection fraction with the sequential introduction of medications including angiotensin-converting-enzyme inhibitors, angiotensin-receptor blockers, beta-blockers, and mineralocorticoid-receptor antagonists. We sought to examine this trend in detail. We analyzed data from 40,195 patients who had heart failure with reduced ejection fraction and were enrolled in any of 12 clinical trials spanning the period from 1995 through 2014. Patients who had an implantable cardioverter-defibrillator at the time of trial enrollment were excluded. Weighted multivariable regression was used to examine trends in rates of sudden death over time. Adjusted hazard ratios for sudden death in each trial group were calculated with the use of Cox regression models. The cumulative incidence rates of sudden death were assessed at different time points after randomization and according to the length of time between the diagnosis of heart failure and randomization. Sudden death was reported in 3583 patients. Such patients were older and were more often male, with an ischemic cause of heart failure and worse cardiac function, than those in whom sudden death did not occur. There was a 44% decline in the rate of sudden death across the trials (P=0.03). The cumulative incidence of sudden death at 90 days after randomization was 2.4% in the earliest trial and 1.0% in the most recent trial. The rate of sudden death was not higher among patients with a recent diagnosis of heart failure than among those with a longer-standing diagnosis. Rates of sudden death declined substantially over time among ambulatory patients with heart failure with reduced ejection fraction who were enrolled in clinical trials, a finding that is consistent with a cumulative benefit of evidence-based medications on this cause of death. (Funded by the China Scholarship Council and the University of Glasgow.).

Back muscle response to sudden trunk loading can be modified by training among healthcare workers.

PubMed

Pedersen, Mogens Theisen; Essendrop, Morten; Skotte, Jørgen H; Jørgensen, Kurt; Schibye, Bente; Fallentin, Nils

2007-06-01

Experimental study of the effect of physical training on the reaction to sudden back loading. To investigate the effect and sustainability of "on the job training" on the reaction to sudden back loading among employees at a geriatric ward. Available data suggest that a delayed muscle reflex response to sudden trunk loading may increase the risk of low back injuries. We have previously shown that training may alter the response to sudden trunk loading in healthy subjects and decrease the time elapsed until stopping of the forward movement of the trunk (stopping time). Data on the possibilities of a training-induced improvement in the reflex response among workers exposed to sudden trunk loading on the job are, however, nonexistent, and there is no evidence of long-term benefits, i.e., the sustainability of a positive training effect. The study included 23 participants and 14 controls. All were healthy without prior history of low back pain (LBP). The training group participated in a total of 18 training sessions during a 9-week period. The training focused on reactions to a variety of sudden trunk loadings. Before and after the training intervention and at a 1-year follow-up, all subjects were tested for their reaction to expected and unexpected sudden trunk loading by applying a horizontal force of 58 N to the upper back of the subjects and measuring the electromyographic (EMG) response from the erector spinae muscles. In the training group, the stopping time and the distance moved after unexpected sudden trunk loading decreased significantly (13%-19%, P = 0.02). The improved stopping time was associated with marked changes in the time-wise distribution of the EMG signal after training. In addition, the follow-up study showed a high sustainability of the training effect. The results demonstrated a training-induced improvement of the response to sudden trunk loading that may be beneficial in workers, such as nurses, who are exposed to sudden trunk perturbations during patient handling.

Influence of nasoalveolar molding on skeletal development in patients with unilateral cleft lip and palate at 5 years of age.

PubMed

Akarsu-Guven, Bengisu; Arisan, Arda; Ozgur, Figen; Aksu, Muge

2018-04-01

The aim of this retrospective study was to assess the influence of presurgical nasoalveolar molding (NAM) on skeletal development in patients with operated unilateral cleft lip and palate at 5 years of age. Lateral cephalometric radiographs of 26 unilateral cleft lip and palate patients who had undergone presurgical NAM (NAM group) and 20 unilateral cleft lip and palate patients who did not have any presurgical NAM (non-NAM group) were analyzed. The radiographs were digitally traced using Quick Ceph Studio software (version 3.5.1.r (1151); Quick Ceph Systems, San Diego, Calif). Independent samples t tests were performed for statistical analysis. No significant differences were observed in sagittal and vertical skeletal measurements between the NAM and non-NAM groups. NAM resulted in no significant difference in skeletal development in unilateral cleft lip and palate patients compared with those without NAM in early childhood. Copyright © 2018. Published by Elsevier Inc.

Complexity vs. unity in unilateral spatial neglect.

PubMed

Rode, G; Fourtassi, M; Pagliari, C; Pisella, L; Rossetti, Y

Unilateral spatial neglect constitutes a heterogeneous syndrome characterized by two main entangled components: a contralesional bias of spatial attention orientation; and impaired building and/or exploration of mental representations of space. These two components are present in different subtypes of unilateral spatial neglect (visual, auditory, somatosensory, motor, allocentric, egocentric, personal, representational and productive manifestations). Detailed anatomical and clinical analyses of these conditions and their underlying disorders show the complexity of spatial cognitive deficits and the difficulty of proposing just one explanation. This complexity is in contrast, however, to the widely acknowledged effectiveness of rehabilitation of the various symptoms and subtypes of unilateral spatial neglect, exemplified in the case of prism adaptation. These common effects are reflections of the unity of the physiotherapeutic mechanisms behind the higher brain functions related to multisensory integration and spatial representations, whereas the paradoxical aspects of unilateral spatial neglect emphasize the need for a greater understanding of spatial cognitive disorders. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

Callosal disconnection syndrome in a left-handed patient due to infarction of the total length of the corpus callosum.

PubMed

Lausberg, H; Göttert, R; Münssinger, U; Boegner, F; Marx, P

1999-03-01

We report on a left-handed patient with an ischemic infarction affecting exclusively the total length of the corpus callosum. This lesion clinically correlated with an almost complete callosal disconnection syndrome as described in callosotomy subjects, including unilateral verbal anosmia, hemialexia, unilateral ideomotor apraxia, unilateral agraphia, unilateral tactile anomia, unilateral constructional apraxia, lack of somesthetic transfer and dissociative phenomena. Despite the patient's left-handedness, his pattern of deficits was similar to the disconnection syndrome found in right-handers. Our report focusses on motor dominance and praxis. We followed-up the improvement in left apraxia and investigated the ability to initiate and learn a new visuo-motor skill. The results permit two tentative assumptions: (1) that the improvement in left apraxia was due to a compensatory increase in ipsilateral proximal muscle control, and (2) that motor dominance, i.e. the competence to initiate and learn a new movement pattern, was hemispherically dissociable from manual dominance in the sense of praxis control.

Changes in regional blood flow induced by unilateral subthalamic nucleus stimulation in patients with Parkinson's disease.

PubMed

Tanei, Takafumi; Kajita, Yasukazu; Nihashi, Takashi; Kaneoke, Yoshiki; Takebayashi, Shigenori; Nakatsubo, Daisuke; Wakabayashi, Toshihiko

2009-11-01

Changes in regional cerebral blood flow (rCBF) induced by unilateral deep brain stimulation (DBS) of the subthalamic nucleus (STN) were investigated in 7 consecutive patients with Parkinson's disease, 4 men and 3 women (mean age 62.3 +/- 8.1 years), who underwent rCBF measurement by N-isopropyl-p-(iodine-123)-iodoamphetamine single photon emission computed tomography at rest before and after unilateral STN DBS preoperatively in the on-drug condition, and postoperatively in the on-drug and on-stimulation condition. Statistical parametric mapping was used to identify significant changes in rCBF from the preoperative to the postoperative conditions. rCBF was increased in the bilateral cingulate cortices and bilateral cerebellar hemispheres. rCBF was decreased in the bilateral medial frontal cortices and left superior temporal cortex. Unilateral STN DBS produced rCBF changes in the bilateral cingulate cortices, cerebellar hemispheres, and medial frontal cortices. These findings indicate that unilateral STN DBS affects rCBF in both hemispheres.

Avulsion of the puborectalis muscle is associated with asymmetry of the levator hiatus.

PubMed

Dietz, H P; Bhalla, R; Chantarasorn, V; Shek, K L

2011-06-01

To determine the degree of levator hiatal asymmetry in women with unilateral avulsion of the puborectalis muscle in order to estimate the likely effect of successful reconstruction. We reviewed datasets of women seen between May 2005 and January 2009 in a tertiary urogynecological unit. Archived ultrasound volume datasets were analyzed for hiatal dimensions and puborectalis muscle trauma in patients with unilateral avulsion. From 929 eligible datasets we identified 71 patients with isolated complete unilateral tears. Unilateral avulsion was associated with a significant increase in hiatal area (P ≤ 0.002). Relative to a hypothetical normal hiatus (intact hemi-area × 2), avulsion was associated with a larger hiatus on Valsalva maneuver (+2.1 cm(2) or 7%). Unilateral puborectalis muscle avulsion results in an asymmetric increase in the size of the levator hiatus. This increase is relatively minor, accounting for < 10% of hiatal area. Surgical repair of an avulsion may have only limited effects on hiatal dimensions. Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd.

How to quantify binaural hearing in patients with unilateral hearing using hearing implants.

PubMed

Snik, Ad; Agterberg, Martijn; Bosman, Arjan

2015-01-01

Application of bilateral hearing devices in bilateral hearing loss and unilateral application in unilateral hearing loss (second ear with normal hearing) does not a priori lead to binaural hearing. An overview is presented on several measures of binaural benefits that have been used in patients with unilateral or bilateral deafness using one or two cochlear implants, respectively, and in patients with unilateral or bilateral conductive/mixed hearing loss using one or two percutaneous bone conduction implants (BCDs), respectively. Overall, according to this overview, the most significant and sensitive measure is the benefit in directional hearing. Measures using speech (viz. binaural summation, binaural squelch or use of the head shadow effect) showed minor benefits, except for patients with bilateral conductive/mixed hearing loss using two BCDs. Although less feasible in daily practise, the binaural masking level difference test seems to be a promising option in the assessment of binaural function. © 2015 S. Karger AG, Basel.

Horizontal localization and speech intelligibility with bilateral and unilateral hearing aid amplification.

PubMed

Köbler, S; Rosenhall, U

2002-10-01

Speech intelligibility and horizontal localization of 19 subjects with mild-to-moderate hearing loss were studied in order to evaluate the advantages and disadvantages of bilateral and unilateral hearing aid (HA) fittings. Eight loudspeakers were arranged in a circular array covering the horizontal plane around the subjects. Speech signals of a sentence test were delivered by one, randomly chosen, loudspeaker. At the same time, the other seven loudspeakers emitted noise with the same long-term average spectrum as the speech signals. The subjects were asked to repeat the speech signal and to point out the corresponding loudspeaker. Speech intelligibility was significantly improved by HAs, bilateral amplification being superior to unilateral. Horizontal localization could not be improved by HA amplification. However, bilateral HAs preserved the subjects' horizontal localization, whereas unilateral amplification decreased their horizontal localization abilities. Front-back confusions were common in the horizontal localization test. The results indicate that bilateral HA amplification has advantages compared with unilateral amplification.

Advanced Electrocardiographic Predictors of Sudden Death in Familial Dysautonomia

NASA Technical Reports Server (NTRS)

Solaimanzadeh, I.; Schlegel, T. T.; Greco, E. C.; DePalma, J. L.; Starc, V.; Marthol, H.; Tutaj, M.; Buechner, S.; Axelrod, F. B.; Hilz, M. J.

2007-01-01

To identify accurate predictors for the risk of sudden death in patients with familial dysautonomia (FD). Ten-minute resting high-fidelity 12-lead ECGs were obtained from 14 FD patients and 14 age/gender-matched healthy subjects. Multiple conventional and advanced ECG parameters were studied for their ability to predict sudden death in FD over a subsequent 4.5-year period, including multiple indices of linear and non-linear heart rate variability (HRV); QT variability; waveform complexity; high frequency QRS; and derived Frank-lead parameters. Four of the 14 FD patients died suddenly during the follow-up period, usually with concomitant pulmonary disorder. The presence of low vagally-mediated HRV was the ECG finding most predictive of sudden death. Concomitant left ventricular hypertrophy and other ECG abnormalities such as increased QTc and JTc intervals, spatial QRS-T angles, T-wave complexity, and QT variability were also present in FD patients, suggesting that structural heart disease is fairly common in FD. Although excessive or unopposed cardiac vagal (relative to sympathetic) activity has been postulated as a contributor to sudden death in FD, the presence of low vagally-mediated HRV was paradoxically the best predictor of sudden death. However, we suggest that low vagally-mediated HRV be construed not as a direct cause of sudden death in FD, but rather as an effect of concurrent pathological processes, especially hypoxia due to pulmonary disorders and sleep apnea, that themselves increase the risk of sudden death in FD and simultaneously diminish HRV. We speculate that adenosine may play a role in sudden death in FD, possibly independently of vagal activity, and that adenosine inhibitors such as theophylline might therefore be useful as prophylaxis in this disorder.

The Metabolic Syndrome and Risk of Sudden Cardiac Death: The Atherosclerosis Risk in Communities Study.

PubMed

Hess, Paul L; Al-Khalidi, Hussein R; Friedman, Daniel J; Mulder, Hillary; Kucharska-Newton, Anna; Rosamond, Wayne R; Lopes, Renato D; Gersh, Bernard J; Mark, Daniel B; Curtis, Lesley H; Post, Wendy S; Prineas, Ronald J; Sotoodehnia, Nona; Al-Khatib, Sana M

2017-08-23

Prior studies have demonstrated a link between the metabolic syndrome and increased risk of cardiovascular mortality. Whether the metabolic syndrome is associated with sudden cardiac death is uncertain. We characterized the relationship between sudden cardiac death and metabolic syndrome status among participants of the ARIC (Atherosclerosis Risk in Communities) Study (1987-2012) free of prevalent coronary heart disease or heart failure. Among 13 168 participants, 357 (2.7%) sudden cardiac deaths occurred during a median follow-up of 23.6 years. Participants with the metabolic syndrome (n=4444) had a higher cumulative incidence of sudden cardiac death than those without it (n=8724) (4.1% versus 2.3%, P <0.001). After adjustment for participant demographics and clinical factors other than components of the metabolic syndrome, the metabolic syndrome was independently associated with sudden cardiac death (hazard ratio, 1.70, 95% confidence interval, 1.37-2.12, P <0.001). This relationship was not modified by sex (interaction P =0.10) or race (interaction P =0.62) and was mediated by the metabolic syndrome criteria components. The risk of sudden cardiac death varied according to the number of metabolic syndrome components (hazard ratio 1.31 per additional component of the metabolic syndrome, 95% confidence interval, 1.19-1.44, P <0.001). Of the 5 components, elevated blood pressure, impaired fasting glucose, and low high-density lipoprotein were independently associated with sudden cardiac death. We observed that the metabolic syndrome was associated with a significantly increased risk of sudden cardiac death irrespective of sex or race. The risk of sudden cardiac death was proportional to the number of metabolic syndrome components. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

Biomechanical differences between unilateral and bilateral landings from a jump: gender differences.

PubMed

Pappas, Evangelos; Hagins, Marshall; Sheikhzadeh, Ali; Nordin, Margareta; Rose, Donald

2007-07-01

To determine the effect of landing type (unilateral vs. bilateral) and gender on the biomechanics of drop landings in recreational athletes. This study used a repeated measures design to compare bilateral and unilateral landings in male and female athletes. A repeated measures multivariate analysis of variance (type of landing*gender) was performed on select variables. Biomechanics laboratory. Sixteen female and 16 male recreational athletes. Kinetic, kinematic, and electromyographic (EMG) data were collected on participants while performing bilateral and unilateral landings from a 40-cm platform. Compared to bilateral landings, subjects performed unilateral landings with increased knee valgus, decreased knee flexion at initial contact, decreased peak knee flexion, decreased relative hip adduction, and increased normalized EMG of the rectus femoris, medial hamstrings, lateral hamstrings, and medial gastrocnemius (P < 0.005). During both types of landing, females landed with increased knee valgus and normalized vertical ground reaction force (VGRF) compared to males (P < 0.009), however, the interaction of landing type*gender was not significant (P = 0.29). Compared to bilateral landings, male and female recreational athletes performed unilateral landings with significant differences in knee kinematic and EMG variables. Female athletes landed with increased knee valgus and VGRF compared to males during both types of landing.

Clinical and surgical management of unilateral prepubertal gynecomastia

PubMed Central

Ferraro, Giuseppe Andrea; De Francesco, Francesco; Romano, Tiziana; Grandone, Anna; D’Andrea, Francesco; Miraglia Del Giudice, Emanuele; Perrone, Laura; Nicoletti, Gianfranco

2014-01-01

INTRODUCTION Gynecomastia is the benign proliferation of the glandular tissue in the male breast. This condition is thought to be caused by the imbalance between estrogen action relative to androgen action at the breast tissue level. Bilateral gynecomastia is frequently found in the neonatal period, early in puberty, and with increasing age. Prepubertal unilateral gynecomastia in the absence of endocrine abnormalities is extremely rare, with only a few cases in literature. PRESENTATION OF CASE We present an otherwise healthy boy of 12 years old with unilateral breast masses. No abnormalities were found on ultrasonography and on all endocrine parameters. Treatment consisted in a new “modified” Webster technique. DISCUSSION The results confirmed validity of this technique in terms of esthetic and functional results, and patient satisfaction. Atypical presentations of gynecomastia are often not recognized. The main pathophysiology of gynecomastia is alteration in the balance between the stimulatory effect of estrogen and the inhibitory effects of androgens on the development of the breast. If there is no causal treatment, surgical resection is the therapy of first choice. CONCLUSION The exact mechanism of unilateral gynecomastia formation in our case is unclear. The evaluation of unilateral gynecomastia can therefore be complex. In conclusion, the surgical treatment of unilateral gynecomastia requires an individual approach, based on an appropriate diagnostic algorithm. PMID:25437663

Exposure of wide cerebellomedullary cisterns for vascular lesion surgeries in cerebellomedullary cisterns: opening of unilateral cerebellomedullary fissures combined with lateral foramen magnum approach.

PubMed

Matsushima, Toshio; Kawashima, Masatou; Inoue, Kohei; Matsushima, Ken; Miki, Koichi

2014-11-01

To clarify microsurgical anatomic features of the cerebellomedullary fissure (CMF), the natural cleavage plane between the cerebellum and the medulla, and its relationship to the cerebellomedullary cistern (CMC) and to describe a surgical technique that uses the unilateral trans-CMF approach for CMC surgeries. In the anatomic study, 2 formalin-fixed cadaver heads were used. In the clinical study, 3 patients with vertebral artery-posterior inferior cerebellar artery aneurysms and 3 patients with glossopharyngeal neuralgia were surgically treated through the unilateral trans-CMF approach combined with the transcondylar fossa approach, which is a lateral foramen magnum approach. The CMC was present at the lateral end of the CMF. The CMF was closed by arachnoidal adhesion, and the cerebellar hemisphere was superiorly attached to the cerebellar peduncle. After the unilateral CMF was completely opened, the cerebellar hemisphere was easily retracted rostrodorsally. Clinically, almost completely opening the unilateral CMF markedly enabled the retraction of the biventral lobule to obtain a wide surgical field safely for vascular CMC lesions. We present 2 representative cases. Combined unilateral trans-CMF/lateral foramen magnum approaches provide a wide and close surgical field in the CMC, allowing easy and safe CMC surgery. Copyright © 2014 Elsevier Inc. All rights reserved.

[EPIDEMIOLOGY OF SUDDEN CARDIAC DEATH: DATA FROM THE PARIS SUDDEN DEATH EXPERTISE CENTER REGISTRY].

PubMed

Jouven, Xavier; Bougouin, Wulfran; Karam, Nicole; Marijon, Eloi

2015-09-01

Sudden cardiac death is an unexpected cardiac arrest without obvious extra-cardiac cause. Epidemiology of sudden cardiac death has been poorly documented in France, mainly because of challenging requirement in order to capture all cases in a specific area. The Parisian registry (Sudden Death Expertise Center, European Georges Pompidou Hospital, Paris) was initiated in May 2011 and analyzed data of all sudden death in Paris and suburbs (6.6 millions inhabitants). Over 3 years, the annual incidence estimated to 50-70 per 100,000. Those occurred mainly in men (69%), with a mean age of 65 year, and at home (75%). The event was witnessed in 80% of cases, but bystander cardiopulmonary resuscitation was initiated in only half of cases. Initial cardiac rhythm was ventricular fibrillation in 25%. Survival to hospital discharge remains low (8%).

[Radiofrequency catheter ablation in children with Wolff-Parkinson-White syndrome and sudden cardiac death who had been resuscitated].

PubMed

Benito Bartolomé, F; Sánchez Fernández-Bernal, C

2001-04-01

Sudden death may be the first manifestation of the Wolff-Parkinson-White syndrome, especially in children and adolescents. The aim of this study was to evaluate the usefulness of radiofrequency catheter ablation in children with Wolff-Parkinson-White syndrome with aborted sudden death. We report four patients with Wolff-Parkinson-White syndrome who survived cardiac arrest. The patients were aged from 2.5 months to 16 years. The two first patients were lactating infants; in the first sudden death occurred during digoxin treatment for supraventricular tachycardia secondary to Wolff-Parkinson-White syndrome and in the second the syndrome was diagnosed after an episode of sudden death. In these patients a free wall accessory pathway (left posterior and left lateral, respectively) was successfully ablated using a transseptal approach. The third patient was diagnosed with asymptomatic Wolff-Parkinson-White syndrome; sudden death occurred during exercise. In the fourth patient, sudden death occurred after intravenous therapy with adenosine triphosphate and amiodarone for rapid atrial fibrillation. In both patients, one accessory pathway, located in right posteroseptal and right anterior free wall, respectively, was ablated. After a mean follow-up of 43.5 26.4 months, no recurrence of sudden death had occurred and electrocardiogram showed sinus rhythm without delta wave. The third patient presented severe sequelae of hypoxemic encephalopathy, which persisted during the follow-up. Radiofrequency catheter ablation is the treatment of choice in Wolff-Parkinson-White syndrome with episodes of aborted sudden death.