Cochlear Diseases
Retinal Vein Occlusion
Vascular Endothelial Growth Factor A
Intravitreal Injections
Macular Edema
Ophthalmology
Retinal Vein
Cytokines
3D MRI of the membranous labyrinth. An age related comparison of MR findings in patients with labyrinthine fibrosis and in persons without inner ear symptoms. (1/37)
PURPOSE: We compared MRI of the membranous labyrinth in patients with chronic non-neoplastic inner ear disease and MR signs of labyrinthine fibrosis and controls depending on their age, in order to establish whether there were any MR differences regarding patient age groups, control age groups and between the patients and controls themselves. MATERIALS AND METHODS: Clinical ENT examinations as well as a T2* weighted 3D CISS (Constructive Interference in Steady State) sequence with a slice thickness of 0.7 mm were performed. Our collective was subdivided as follows: 0-19 years (10 controls, 3 patients with chronic non-neoplastic inner ear disease), 20-49 years (55 controls, 8 patients), 50 years and older (40 controls, 22 patients). Detectability of labyrinthine structures (e.g. cochlea, vestibule, semicircular canals) and filling defects were evaluated. RESULTS: In the 3 age-groups of the control collective no significant differences were observed in the membranous labyrinth. However differences concerning labyrinthine detectability emerged between controls and patients in both the 20-49 years and 50 years and older age groups. In the patient collective the 3 age groups showed no significant discrepancy in the mean number of lesions. CONCLUSION: Filling defects of the membranous labyrinth on 3D CISS MR images are pathological even in older persons. We would therefore recommend high resolution T2* weighted MRI in the case of suspected labyrinthine fibrosis. (+info)Ototoxicity: an argument for evaluation of the cochlea in safety testing in animals. (2/37)
The cochlea is one of the more common targets for toxic effects, yet current toxicologic screening in animals does not routinely evaluate the cochlea as a potential target organ. Although histopathologic sections are routinely taken from the eye and the optic nerve and tract and most studies include at least 1 section through the nasal cavity and olfactory mucosa, the cochlea is not histopathologically examined in routine toxicity studies. Unfortunately, routine clinical examinations frequently miss ototoxicity because rodents and other species can lose most of their high-frequency hearing and still respond to most ambient noises. Ototoxicity as a deficiency in toxicologic screening can be remedied by using well-established histopathologic and behavioral methods or electrophysiologic methods, such as brain stem auditory evoked responses (BAERs). Once the equipment is in place, BAERs can be obtained quickly and easily for ototoxicity screening (approximately 15 minutes for paired testing of 2 rats and 30 minutes each for dogs). BAERs also can be used in virtually all mammalian species. Three or 4 probe frequencies (eg, 4, 8, 16, and 32 kHz), representing different areas of the cochlea, can be tested in a few minutes with subcutaneous electrodes under short-acting chemorestraint. Given the availability of several approaches to screening for ototoxicity and the importance of the auditory function in human health, safety tests of chemicals and drugs should include an effective screening test for ototoxicity. (+info)Spiral ligament pathology: a major aspect of age-related cochlear degeneration in C57BL/6 mice. (3/37)
Data from systematic, light microscopic examination of cochlear histopathology in an age-graded series of C57BL/6 mice (1.5-15 months) were compared with threshold elevations (measured by auditory brain stem response) to elucidate the functionally important structural changes underlying age-related hearing loss in this inbred strain. In addition to quantifying the degree and extent of hair cell and neuronal loss, all structures of the cochlear duct were qualitatively evaluated and any degenerative changes were quantified. Hair cell and neuronal loss patterns suggested two degenerative processes. In the basal half of the cochlea, inner and outer hair cell loss proceeded from base to apex with increasing age, and loss of cochlear neurons was consistent with degeneration occurring secondary to inner hair cell loss. In the apical half of the cochlea with advancing age, there was selective loss of outer hair cells which increased from the middle to the extreme apex. A similar gradient of ganglion cell loss was noted, characterized by widespread somatic aggregation and demyelination. In addition to these changes in hair cells and their innervation, there was widespread degeneration of fibrocytes in the spiral ligament, especially among the type IV cell class. The cell loss in the ligament preceded the loss of hair cells and/or neurons in both space and time suggesting that fibrocyte pathology may be a primary cause of the hearing loss and ultimate sensory cell degeneration in this mouse strain. (+info)Delayed inner ear maturation and neuronal loss in postnatal Igf-1-deficient mice. (4/37)
Insulin-like growth factor-1 (IGF-1) has been shown to play a key role during embryonic and postnatal development of the CNS, but its effect on a sensory organ has not been studied in vivo. Therefore, we examined cochlear growth, differentiation, and maturation in Igf-1 gene knock-out mice at postnatal days 5 (P5), P8, and P20 by using stereological methods and immunohistochemistry. Mutant mice showed reduction in size of the cochlea and cochlear ganglion. An immature tectorial membrane and a significant decrease in the number and size of auditory neurons were also evident at P20. IGF-1-deficient cochlear neurons showed increased caspase-3-mediated apoptosis, along with aberrant expression of the early neural markers nestin and Islet 1/2. Cochlear ganglion and fibers innervating the sensory cells of the organ of Corti presented decreased levels of neurofilament and myelin P(0) in P20 mouse mutants. In addition, an abnormal synaptophysin expression in the somata of cochlear ganglion neurons and sensory hair cells suggested the persistence of an immature pattern of synapses distribution in the organ of Corti of these animals. These results demonstrate that lack of IGF-1 in mice severely affects postnatal survival, differentiation, and maturation of the cochlear ganglion cells and causes abnormal innervation of the sensory cells in the organ of Corti. (+info)Dynamics of noise-induced cellular injury and repair in the mouse cochlea. (5/37)
To assess the dynamics of noise-induced tissue injury and repair, groups of CBA/CaJ mice were exposed to an octave-band noise for 2 hours at levels of 94, 100, 106, 112, or 116 dB SPL and evaluated at survival times of 0, 12, 24 hours or 1, 2, or 8 weeks. Functional change, assessed via auditory brainstem response (ABR), ranged from a reversible threshold shift (at 94 dB) to a profound permanent loss (at 116 dB). Light microscopic histopathology was assessed in serial thick plastic sections and involved quantitative evaluation of most major cell types within the cochlear duct, including hair cells (and their stereocilia), supporting cells, ganglion cells, spiral ligament fibrocytes, spiral limbus fibrocytes, and the stria vascularis. Morphometry allowed patterns of damage to be systematically assessed as functions of (1) cochlear location, (2) exposure level, and (3) postexposure survival. Insights into mechanisms of acute and chronic noise-induced cellular damage are discussed. (+info)Acrylonitrile produces transient cochlear function loss and potentiates permanent noise-induced hearing loss. (6/37)
There is growing evidence that agents that produce oxidative stress in the cochlea have significant ototoxic potential by themselves and can potentiate noise-induced hearing loss as well. Acrylonitrile (ACN) metabolism entails conjugation with glutathione, resulting in rapid and pronounced depletion of this important antioxidant in many organs including brain, liver, and kidney. ACN metabolism also results in cyanide (CN) formation through a secondary oxidative pathway. The results of two physiological experiments are reported here. First, the acute effects of ACN (50 mg/kg sc) on auditory sensitivity are assessed using a within subject study. In the second study, persistent effects of ACN alone (50 mg/kg, sc and 2 x 50 mg/kg, sc) and ACN in combination with noise exposure (8 h, 108 dB octave-band noise) are evaluated using threshold sensitivity as the dependent measure. Auditory threshold shift and absolute thresholds were determined using the compound action potential (CAP) amplitude. Acute ACN administration produces a loss in auditory threshold sensitivity that reached a maximum 10-20 min following sc injection. Auditory thresholds returned to control levels 75-100 min following exposure. In the study of permanent auditory threshold shifts, ACN plus noise increased auditory threshold impairment relative to rats receiving noise only when thresholds were assessed 3 weeks following exposure. ACN by itself did not produce permanent threshold impairment 3 weeks following administration. Assays were undertaken in separate groups of rats to track the elevation in blood CN and the depletion of total glutathione in cochlea, brain, and liver following ACN treatment. Systemic blood CN levels were not significantly elevated until 60-120 min following injection, and cochlear glutathione levels showed significant depletion as little as 15 min after injection and remained depressed for about 4 h. The results confirm the prediction that ACN is acutely ototoxic and can enhance noise-induced hearing loss. (+info)Myo15 function is distinct from Myo6, Myo7a and pirouette genes in development of cochlear stereocilia. (7/37)
The unconventional myosin genes Myo15, Myo6 and Myo7a are essential for hearing in both humans and mice. Despite the expression of each gene in multiple organs, mutations result in identifiable phenotypes only in auditory or ocular sensory organs. The pirouette (pi) mouse also exhibits deafness and an inner ear pathology resembling that of Myo15 mutant mice and thus may be functionally related to Myo15. In order to investigate possible interactions between Myo15 and Myo6, Myo7a, and the gene affected in pirouette, we crossed Myo15(sh2/sh2) mice to the three other mutant mouse strains. Hearing in doubly heterozygous mice was similar to age-matched singly heterozygous animals, indicating that partial deficiency for both Myo15 and one of these other deafness genes does not reduce hearing. Viable double mutants were obtained from each cross, indicating that potential overlapping functions between these genes in other organs are not essential for viability. All critical cell types of the cochlear sensory epithelium were present in double mutant mice and cochlear stereocilia exhibited a superimposition of single mutant phenotypes. These data suggest that the function of Myo15 is distinct from that of Myo6, Myo7a or pi in development and/or maintenance of stereocilia. (+info)Abnormal auditory brainstem responses and cochlear pathology in rats induced by an exaggerated styrene exposure regimen. (8/37)
Groups of 12 male 42-day-old rats were exposed to 0 or 800 ppm styrene vapors for 14 hr/day, 5 days/week for 3 weeks. Tone-pip auditory brainstem responses (ABRs) at 4, 8, 16, and 30 kHz were obtained after the last exposure. ABRs were minimally affected at 4 kHz and moderately to severely affected at 8, 16, and 30 kHz as indicated by waveforms which had a decreased amplitude and increased latency as compared to the controls. Missing outer hair cell(s) were evident in the basal and lower middle turns of the organ of Corti. Outer hair cell loss was least in the first row and greatest in the second and third rows. Occasional inner hair cells were also missing in regions of severe outer hair cell loss. The distribution of hair cell loss within the cochlea was consistent with the pattern of ABR alterations. These data document mid-frequency auditory dysfunction in styrene-exposed young adult rats with significant damage to the organ of Corti following an exaggerated styrene exposure regimen. (+info)Cochlear diseases refer to conditions that affect the structure or function of the cochlea, which is a part of the inner ear responsible for hearing. These diseases can cause various types and degrees of hearing loss, ranging from mild to profound. Some common cochlear diseases include:
1. Cochlear otosclerosis: A condition where there is abnormal bone growth in the cochlea, which can lead to conductive or sensorineural hearing loss.
2. Cochlear Meniere's disease: A disorder that affects the inner ear and causes vertigo, tinnitus, and fluctuating hearing loss.
3. Cochlear damage due to exposure to loud noises: Prolonged or sudden exposure to loud noises can cause permanent cochlear damage and hearing loss.
4. Presbycusis: Age-related hearing loss that affects the cochlea and other structures of the auditory system.
5. Cochlear nerve tumors: Rare benign or malignant growths on the cochlear nerve can cause hearing loss, tinnitus, and balance problems.
6. Infections: Bacterial or viral infections such as meningitis, labyrinthitis, or otitis media can damage the cochlea and lead to hearing loss.
7. Ototoxicity: Certain medications can be toxic to the cochlea and cause hearing loss, tinnitus, or balance problems.
8. Genetic factors: Inherited genetic mutations can cause various types of cochlear diseases, such as connexin 26 deficiency, Waardenburg syndrome, or Usher syndrome.
It is important to note that early diagnosis and treatment of cochlear diseases can help prevent or minimize hearing loss and other complications.
Retinal vein occlusion (RVO) is a medical condition that occurs when one of the retinal veins, which drains blood from the retina, becomes blocked by a blood clot or atherosclerotic plaque. This blockage can cause hemorrhages, fluid accumulation, and damage to the retinal tissue, leading to vision loss.
There are two types of RVO: branch retinal vein occlusion (BRVO) and central retinal vein occlusion (CRVO). BRVO affects a smaller branch retinal vein, while CRVO affects the main retinal vein. CRVO is generally associated with more severe vision loss than BRVO.
Risk factors for RVO include hypertension, diabetes, high cholesterol levels, smoking, and glaucoma. Age is also a significant risk factor, as RVO becomes more common with increasing age. Treatment options for RVO may include controlling underlying medical conditions, laser therapy, intravitreal injections of anti-VEGF agents or steroids, and surgery in some cases.
Vascular Endothelial Growth Factor A (VEGFA) is a specific isoform of the vascular endothelial growth factor (VEGF) family. It is a well-characterized signaling protein that plays a crucial role in angiogenesis, the process of new blood vessel formation from pre-existing vessels. VEGFA stimulates the proliferation and migration of endothelial cells, which line the interior surface of blood vessels, thereby contributing to the growth and development of new vasculature. This protein is essential for physiological processes such as embryonic development and wound healing, but it has also been implicated in various pathological conditions, including cancer, age-related macular degeneration, and diabetic retinopathy. The regulation of VEGFA expression and activity is critical to maintaining proper vascular function and homeostasis.
An intravitreal injection is a medical procedure in which medication is delivered directly into the vitreous cavity of the eye, which is the clear, gel-like substance that fills the space between the lens and the retina. This type of injection is typically used to treat various eye conditions such as age-related macular degeneration, diabetic retinopathy, retinal vein occlusion, and uveitis. The medication administered in intravitreal injections can help to reduce inflammation, inhibit the growth of new blood vessels, or prevent the formation of abnormal blood vessels in the eye.
Intravitreal injections are usually performed in an outpatient setting, and the procedure typically takes only a few minutes. Before the injection, the eye is numbed with anesthetic drops to minimize discomfort. The medication is then injected into the vitreous cavity using a small needle. After the injection, patients may experience some mild discomfort or a scratchy sensation in the eye, but this usually resolves within a few hours.
While intravitreal injections are generally safe, there are some potential risks and complications associated with the procedure, including infection, bleeding, retinal detachment, and increased intraocular pressure. Patients who undergo intravitreal injections should be closely monitored by their eye care provider to ensure that any complications are promptly identified and treated.
Macular edema is a medical condition characterized by the accumulation of fluid in the macula, a small area in the center of the retina responsible for sharp, detailed vision. This buildup of fluid causes the macula to thicken and swell, which can distort central vision and lead to vision loss if not treated promptly. Macular edema is often a complication of other eye conditions such as diabetic retinopathy, age-related macular degeneration, retinal vein occlusion, or uveitis. It's important to note that while macular edema can affect anyone, it is more common in people with certain medical conditions like diabetes.
Ophthalmology is a branch of medicine that deals with the diagnosis, treatment, and prevention of diseases and disorders of the eye and visual system. It is a surgical specialty, and ophthalmologists are medical doctors who complete additional years of training to become experts in eye care. They are qualified to perform eye exams, diagnose and treat eye diseases, prescribe glasses and contact lenses, and perform eye surgery. Some subspecialties within ophthalmology include cornea and external disease, glaucoma, neuro-ophthalmology, pediatric ophthalmology, retina and vitreous, and oculoplastics.
A Retinal Vein is a vessel that carries oxygen-depleted blood away from the retina, a light-sensitive layer at the back of the eye. The retinal veins originate from a network of smaller vessels called venules and ultimately merge to form the central retinal vein, which exits the eye through the optic nerve.
Retinal veins are crucial for maintaining the health and function of the retina, as they facilitate the removal of waste products and help regulate the ocular environment. However, they can also be susceptible to various pathological conditions such as retinal vein occlusions, which can lead to vision loss or damage to the eye.
Cytokines are a broad and diverse category of small signaling proteins that are secreted by various cells, including immune cells, in response to different stimuli. They play crucial roles in regulating the immune response, inflammation, hematopoiesis, and cellular communication.
Cytokines mediate their effects by binding to specific receptors on the surface of target cells, which triggers intracellular signaling pathways that ultimately result in changes in gene expression, cell behavior, and function. Some key functions of cytokines include:
1. Regulating the activation, differentiation, and proliferation of immune cells such as T cells, B cells, natural killer (NK) cells, and macrophages.
2. Coordinating the inflammatory response by recruiting immune cells to sites of infection or tissue damage and modulating their effector functions.
3. Regulating hematopoiesis, the process of blood cell formation in the bone marrow, by controlling the proliferation, differentiation, and survival of hematopoietic stem and progenitor cells.
4. Modulating the development and function of the nervous system, including neuroinflammation, neuroprotection, and neuroregeneration.
Cytokines can be classified into several categories based on their structure, function, or cellular origin. Some common types of cytokines include interleukins (ILs), interferons (IFNs), tumor necrosis factors (TNFs), chemokines, colony-stimulating factors (CSFs), and transforming growth factors (TGFs). Dysregulation of cytokine production and signaling has been implicated in various pathological conditions, such as autoimmune diseases, chronic inflammation, cancer, and neurodegenerative disorders.
Refsum disease
Cilium
Causes of hearing loss
Hôtel-Dieu de Québec
MYH9
Cochlear hydrops
Cochlear implant
Meningitis
Otosclerosis
CI
List of MeSH codes (C09)
Neurotology
Microelectrode array
Hydrops
International Federation of Hard of Hearing People
Cogan syndrome
Combined Defence Services Examination
Norrie disease
The Traveling Awareness Bears
Green to Gold (album)
Second Oommen Chandy ministry
Vestibular membrane
Inner ear regeneration
Remote control animal
Neuroprosthetics
Bioelectricity
Oommen Chandy
Ménière's disease
Synaptopathy
Government ENT Hospital, Visakhapatnam
Cochlear Implantation in Charcot-Marie-Tooth Disease: Case Report and Review of the Literature
Group B Streptococcus Meningitis in a Child with Cochlear Implant - Volume 15, Number 10-October 2009 - Emerging Infectious...
Mayo Clinic Radio: Memory test scoring for Alzheimer's disease / adult cochlear implants / optimism and health / AI for...
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Refsum disease - Wikipedia
Cochlear Implant | MedlinePlus
Signal Intensity Ratio of Cochlear Basal Turn Increased in Affected Ear in Meniere Disease - MSD Manual Professional Edition
Tau phosphorylation and cochlear apoptosis cause hearing loss in 3×Tg-AD Mouse Model of Alzheimer's Disease<...
Indications for Cochlear Implants: Overview, Preoperative Considerations, Etiologies of Severe to Profound Hearing Loss
van Rooyen A[au] - Search Results - PubMed
Yuan F. Liu, MD | Loma Linda University
Indian Journal of Ophthalmology
David L Horn, MD
Refsum Disease: Practice Essentials, Background, Pathophysiology
ENT Surgery - Parkside Hospital
Otolaryngologists and ENT Specialists in Boston | Tufts Medical Center
Alport's Syndrome | Tufts Medical Center
Correlation between Vestibular Evoked Myogenic Potentials and Disease Progression in Ménière's Disease | ORL | Karger Publishers
ATSDR - Validation of Test Methods for Assessing Neurodevelopment in Children - Background
Kidshealth - Diseases & Conditions | Akron Children's Hospital
Immunization Schedule (for Parents) - Nemours KidsHealth
Essential Oils: My Ears Were Itching & Burning Terribly
Essential Oils: The Disappearance Of Ear Skin Cancer
Pneumococcal Vaccine - Infectious Diseases - Merck Manuals Professional Edition
Pneumococcal Vaccine - Infectious Diseases - Merck Manuals Professional Edition
Frontiers | Molecular Behavior of HMGB1 in the Cochlea Following Noise Exposure and in vitro
Dr Stephen Rodrigues, ENT Surgeon | healthdirect
Quality of Life and Cost-Effectiveness of Cochlear Implants: A Narrative Review | Audiology and Neurotology | Karger Publishers
Who Needs the Pneumococcal Vaccine? - Total Health Care
Implantation20
- Currently, the role of cochlear implantation in the setting of CMT and other progressive peripheral neurodegenerative disorders is not well established. (hindawi.com)
- Left-sided cochlear implantation was performed using a conventional length lateral wall electrode. (hindawi.com)
- We report the third case of cochlear implantation in a patient with CMT. (hindawi.com)
- In patients with CMT, cochlear implantation may reconstitute synchronous neural activity by way of supraphysiological electrical stimulation. (hindawi.com)
- Our results corroborate two earlier reports that cochlear implantation is a viable option for rehabilitation of SNHL in this unique subset of patients. (hindawi.com)
- We present a patient with CMT-associated deafness successfully treated with cochlear implantation. (hindawi.com)
- Cochlear implantation is the standard treatment for children and adults affected by severe and severe-to-profound sensorineural hearing loss. (cdc.gov)
- Although individual responses to cochlear implants are highly variable and depend on a number of physical and psychosocial factors, the trend toward improved performance with increasingly sophisticated electrodes and programming strategies has dramatically expanded indications for cochlear implantation. (medscape.com)
- Because preoperative expectations affect the patient's postoperative satisfaction and use of the implant, all patients and families require attention and counseling from an implant team before they embark on the life-changing journey of cochlear implantation. (medscape.com)
- The HINT measures word-recognition abilities to evaluate the patient's candidacy for cochlear implantation, in conjunction with conventional pure-tone and speech audiometry. (medscape.com)
- Visual-motor integration skills of prelingually deaf children: implications for pediatric cochlear implantation. (seattlechildrens.org)
- To review evidence regarding the health-related quality of life (HRQoL) and cost-effectiveness of unilateral and bilateral cochlear implantation (CI) among children and adults with severe-to-profound hearing loss. (karger.com)
- Since its introduction into clinical practice, hearing care clinicians have increasingly utilized cochlear implantation (CI) to restore auditory stimulation in selected patients with advanced sensorineural hearing loss. (karger.com)
- Objective: Cochlear implantation is the emerging treatment of choice for severe and profound sensorineural hearing loss, yet there are conflicting data on outcomes in adults. (scirp.org)
- Complications were retrospectively collected after each cochlear implantation. (scirp.org)
- The aim of this study was to evaluate the hearing outcome of cochlear implantation in. (libsyn.com)
- The results clearly demonstrate significant benefit of cochlear implantation in prelinguistically deafened children for speech perception ability when using either the SPEAK or ACE speech coding strategies. (cun.es)
- Surgical Labyrinthectomy and Cochlear Implantation in Menière's Disease. (menierestreatmentreport.com)
- OBJECTIVE: The aim of this study was to analyze the results of labyrinthectomy and cochlear implantation (CI) on hearing, vertigo, and tinnitus and evaluate the adequacy of labyrinthectomy and CI for the treatment of end stage Menière's Disease (MD). (menierestreatmentreport.com)
- After the shock of finding out our baby was completely deaf, we were thrilled to learn that Chicago had one of the top otolaryngology surgeons in the country who specializes in cochlear implantation," Max's mom, Kristen said. (luriechildrens.org)
Implants13
- thus, the role of cochlear implants in auditory rehabilitation of CMT-associated SNHL is poorly defined [ 1 , 2 ]. (hindawi.com)
- Also on the program, Dr. Matthew Carlson , a Mayo Clinic otorhinolaryngologist, and Dr. Aniket Saoji , a Mayo Clinic audiologist, discuss cochlear implants for adults. (mayoclinic.org)
- [ 6 ] However, many candidates for cochlear implants do not have access to this procedure, due to failure to recognize appropriate candidates or because of inadequate healthcare resources. (medscape.com)
- Cochlear implants can be fitted for those patients with permanent deafness / hearing loss. (parkside-hospital.co.uk)
- General paediatric and adult otolaryngology Sinus surgery Diseases of the ear Cochlear and other hearing implants Sees. (parkside-hospital.co.uk)
- Treatment of infected cochlear implants with novel agents such as tea tree oil could significantly improve salvage outcome. (oil-testimonials.com)
- Certain medical conditions (eg, chronic disorders, immunocompromising conditions, cerebrospinal fluid leaks, cochlear implants) increase the risk of pneumococcal disease. (merckmanuals.com)
- The fellowship allows you to work with faculty who specialize in all areas of pediatric otolaryngology including large volumes of cochlear implants, chronic ear disease, and open airway surgery. (wustl.edu)
- Cochlear implants have been used for many years to replace lost hearing resulting from inner ear damage. (news-medical.net)
- The most successful neuroprosthetic devices developed to date are cochlear implants for patients with hearing impairment, and prosthetic devices for amputees. (presenternet.com)
- We saw Dr. Nancy Young and she explained that the sooner Max gets cochlear implants , the better his chances at developing normal speech. (luriechildrens.org)
- Cochlear implants enable children to hear even high-pitched consonants, such as "s," when traditional hearing aids cannot because of the degree of loss, said Dr. Young, who has performed more than 1,700 cochlear procedures since 1991. (luriechildrens.org)
- Many children don't receive cochlear implants until they are older, and by that time their speech is impacted," she said. (luriechildrens.org)
Meniere's56
- Cochlear implant outcomes in patients with Meniere's disease: a large case series. (nih.gov)
- His research interests are broad, ranging from cochlear implant outcomes to Meniere's disease and vestibular migraine. (llu.edu)
- He is currently collaborating with other institutions in developing new treatments for Meniere's disease. (llu.edu)
- Predictive value of vestibular evoked myogenic potentials in the diagnosis of Meniere's disease and vestibular migraine. (llu.edu)
- Meniere's disease. (karger.com)
- for patients with Meniere's disease (MD), tinnitus, and cochlear hypoperfusion. (oil-testimonials.com)
- Meniere's Disease is a chronic condition that affects the inner ear and causes a variety of symptoms such as vertigo, tinnitus, hearing loss, and aural fullness. (hearingresearch.org)
- What is Meniere's Disease? (hearingresearch.org)
- Meniere's Disease is a condition that affects the inner ear and causes a variety of symptoms. (hearingresearch.org)
- Meniere's Disease is thought to be caused by an abnormal buildup of fluid in the inner ear , which can disrupt the balance and hearing mechanisms of the ear. (hearingresearch.org)
- Meniere's Disease is not a common condition, but it can affect anyone. (hearingresearch.org)
- Meniere's Disease is slightly more common in women than in men, and it is more commonly diagnosed in adults between the ages of 40 and 60. (hearingresearch.org)
- Although Meniere's Disease can affect people of all races and ethnicities, some studies have suggested that it may be more common in people of European descent. (hearingresearch.org)
- Meniere's Disease can have a significant impact on a person's quality of life. (hearingresearch.org)
- Fortunately, there are treatments available that can help manage the symptoms of Meniere's Disease. (hearingresearch.org)
- If you are experiencing symptoms of Meniere's Disease, it is important to speak with your doctor or an ear, nose, and throat specialist. (hearingresearch.org)
- With the right treatment, many people with Meniere's Disease are able to manage their symptoms and lead full, active lives. (hearingresearch.org)
- The symptoms of Meniere's Disease can be debilitating and can significantly impact a person's quality of life. (hearingresearch.org)
- Vertigo is a hallmark symptom of Meniere's Disease. (hearingresearch.org)
- Some people with Meniere's Disease experience vertigo attacks that are so severe they are unable to stand or walk during an attack. (hearingresearch.org)
- Tinnitus can be a source of significant distress for people with Meniere's Disease, as it can interfere with sleep and concentration. (hearingresearch.org)
- Hearing loss is another common symptom of Meniere's Disease. (hearingresearch.org)
- Some people with Meniere's Disease experience sudden hearing loss during an episode of vertigo. (hearingresearch.org)
- Some people with Meniere's Disease describe the sensation as feeling like their ears are "plugged. (hearingresearch.org)
- While the exact cause of Meniere's Disease is unknown, it is believed to be related to an abnormal buildup of fluid in the inner ear. (hearingresearch.org)
- Treatment for Meniere's Disease may include medication, dietary changes, or surgery. (hearingresearch.org)
- If you are experiencing symptoms of Meniere's Disease, it is important to see a healthcare provider for an accurate diagnosis and appropriate treatment. (hearingresearch.org)
- Sensorineural hearing loss in the inner ear or hearing nerve may be due to the effects of ageing, exposure to loud noise, Meniere's disease or some medications and infections. (healthhub.sg)
- Meniere's disease is an abnormality of the inner ear causing a host of symptoms, including vertigo or severe dizziness, tinnitus or a roaring sound in the ears, fluctuating hearing loss, and the sensation of pressure or pain in the affected ear. (news-medical.net)
- A team of scientists from the University of Granada (UGR) and GENYO (Pfizer-University of Granada-Andalusian Government Centre for Genomics and Oncological Research-led by Pablo Roman-Naranjo and Jose Antonio López-Escámez, researcher in charge of the Biohealth Research Institute in Granada (ibs.GRANADA)-has identified new genes associated with familial Meniere's disease. (news-medical.net)
- Sound Pharmaceuticals is pleased to announce that it began enrolling a clinical trial to test SPI-1005 in the treatment of Meniere's Disease (MD). MD or idiopathic endolymphatic hydrops is an inner ear disease that involves episodic vertigo, sensorineural hearing loss, and tinnitus. (news-medical.net)
- Otonomy, Inc., a clinical stage biopharmaceutical company developing innovative therapeutics for diseases and disorders of the inner and middle ear, today announced enrollment of the first patient in its pivotal Phase 2b study of OTO-104 for the reduction of vertigo in patients with unilateral Meniere's disease. (news-medical.net)
- Researchers at University of Colorado School of Medicine may have figured out what causes Meniere's disease and how to attack it. (news-medical.net)
- According to Carol Foster, MD, from the department of otolaryngology and Robert Breeze, MD, a neurosurgeon, there is a strong association between Meniere's disease and conditions involving temporary low blood flow in the brain such as migraine headaches. (news-medical.net)
- Meniere's disease is a disorder of the inner ear that affects balance and hearing. (medifocus.com)
- In the early stages, Meniere's disease is usually unilateral (occurring on one side). (medifocus.com)
- Meniere's disease is also called idiopathic endolymphatic hydrops . (medifocus.com)
- The cause of Meniere's disease is unknown. (medifocus.com)
- Genetic predisposition - Approximately 5%-20% of individuals diagnosed with Meniere's disease report that a family member has or had Meniere's disease or similar symptoms. (medifocus.com)
- In addition, chromosomal studies appear to be making progress in identifying certain genetic markers in individuals diagnosed with Meniere's disease. (medifocus.com)
- Allergies - Individuals with Meniere's disease report higher rates of allergies than people not affected with Meniere's, and some studies have shown higher rates of serum immune markers in individuals with Meniere's disease than those without. (medifocus.com)
- In addition, studies have shown that individuals with allergies and Meniere's disease report an improvement in the severity and duration of their Meniere's attacks when treated with immunotherapy and/or dietary adjustments. (medifocus.com)
- Migraine headaches - There appears to be an association between Meniere's disease and migraine headaches. (medifocus.com)
- and that 45% of individuals with Meniere's disease always experienced at least one symptom of migraine headaches (e.g., aura, or an aversion to light) during Meniere's attacks. (medifocus.com)
- Otosclerosis - Some individuals with otosclerosis (a rare condition of formation of spongy bone around the stapes) have symptoms of Meniere's disease. (medifocus.com)
- It is also not clear whether endolymphatic hydrops is the cause or the result of Meniere's disease, since there are known cases of individuals with endolymphatic hydrops who otherwise have no symptoms of Meniere's disease. (medifocus.com)
- While Meniere's disease is the second most common cause of vertigo in the U.S., it is listed in the National Organization of Rare Diseases (NORD) database as a rare disease. (medifocus.com)
- Information regarding the precise incidence and prevalence of Meniere's disease is scarce, but some estimates range from 15 to 150 cases per 100,000 people. (medifocus.com)
- Meniere's disease most often affects adults between the ages of 40 and 60. (medifocus.com)
- Approximately 40,000 new cases of Meniere's disease are diagnosed annually in the U.S. (medifocus.com)
- Approximately 3% of patients diagnosed with Meniere's disease are children. (medifocus.com)
- Although most cases of Meniere's disease are usually unilateral (occurring on one side only), up to 10% of individuals have bilateral Meniere's disease affecting both ears at the time of diagnosis. (medifocus.com)
- There is no consistent pattern for the progression of Meniere's disease. (medifocus.com)
- The diagnosis of Meniere's disease can be challenging because there are many other conditions that can present with similar clinical symptoms. (medifocus.com)
- Ironically, this aspect of their toxicity has therapeutic use in patients with intractable Meniere's disease. (medscape.com)
- Patients usually experience remission of Meniere's disease by day 12. (medscape.com)
Sensorineural hear6
- Charcot-Marie-Tooth (CMT) disease is a peripheral hereditary neuropathy associated with motor and sensory impairment and can result in profound sensorineural hearing loss (SNHL). (hindawi.com)
- In 80% of patients diagnosed with Refsum disease, sensorineural hearing loss has been reported. (wikipedia.org)
- For the purposes of this workshop, immune mediated ear disease was defined to include both primary autoimmune sensorineural hearing loss (ASNHL) originating in the inner ear and secondary immune mediated ear disease/hearing loss (IMED) from systemic immune/autoimmune disease originating outside the inner ear. (nih.gov)
- The presence of hearing loss in the elderly is described by the term "presbycusis" it typically presents as sensorineural hearing loss characterized by loss in the high frequencies[ 10 ] ( Figure 1 ) and sometimes may be associated to the presence of cochlear dead regions[ 11 ]. (tinnitusjournal.com)
- Sensorineural hearing loss is secondary to cochlear abnormalities and/or an abnormality of the auditory nerve or central auditory pathways. (medscape.com)
- Sensorineural hearing loss (SNHL) from cochlear implant surgery and samples (2 mL) were obtained on the is a type of hearing loss in which the serology findings. (who.int)
Registered cochlear implant1
- About 736,900 registered cochlear implant devices had, as of December 2019, been placed in patients worldwide. (medscape.com)
Auditory2
- Clinically typical dementia Alzheimer's disease (AD) is associated with abnormal auditory processing. (ntnu.edu.tw)
- Homozygous SLITRK6 c.1240C>T (p.Gln414Ter) nonsense mutations are associated with high myopia, cochlear dysfunction attributed to outer hair cell disease, and progressive auditory neuropathy. (harvard.edu)
Pediatric3
- Pediatric cochlear implant recipients were found to be at higher risk for developing bacterial meningitis than children in the general US population ( 3 ). (cdc.gov)
- Dr. Horn is the medical Co-Director of the Pediatric Cochlear Implant program at Seattle Childrens Hospital and sees patients with hearing loss, ear disorders, balance issues, as well as other general pediatric otolaryngological problems. (seattlechildrens.org)
- Dr. Young is the founder of the Lurie Children's Cochlear Implant Program , one of the largest pediatric implant programs in the world. (luriechildrens.org)
Congenital1
- The significant disease burden from congenital CMV infection (cCMV) led the US National Institute of Medicine to rank CMV vaccine development as the highest priority. (cdc.gov)
Cerebrospinal2
- Administration (FDA) approved an expanded usage for PCV15 aged 6-18 years with an immunocompromising condition,¶ to include persons aged 6 weeks-17 years, based on studies that cerebrospinal fluid leak, or cochlear implant (a subset of risk compared antibody responses to PCV15 with those to PCV13 conditions). (cdc.gov)
- A minimum interval of 8 weeks between PCV15 and PPSV23 can be considered for adults with an immunocompromising condition, cochlear implant, or cerebrospinal fluid (CSF) leak. (merckmanuals.com)
Risk of pneumococcal disease1
- Who is most at risk of pneumococcal disease? (hse.ie)
Basal turn2
- TUESDAY, Jan. 2, 2024 (HealthDay News) -- The affected ear in Meniere disease has significantly increased the signal intensity ratio (SIR) of the cochlear basal turn, indicating potential damage of the blood-labyrinth barrier, according to a study published online Dec. 6 in the European Archives of Oto-Rhino-Laryngology . (msdmanuals.com)
- Weidong Zhang, M.D., from the Zhengzhou University People's Hospital and Henan Provincial People's Hospital in China, and colleagues enrolled 30 patients with unilateral definite Meniere disease and 24 healthy controls to compare the SIR of the cochlear basal turn. (msdmanuals.com)
20221
- Starting in 2022, persons with certain underlying diseases will be offered vaccination against invasive pneumococcal infections within a national vaccination programme. (folkhalsomyndigheten.se)
Deafness3
- On July 22-23, 2008, the National Institute on Deafness and Other Communication Disorders (NIDCD) convened a workshop at the Hyatt Regency Bethesda in Bethesda, MD, titled Immune Mediated Ear Disease/Hearing Loss . (nih.gov)
- : http://www.who.int/pbd/deafness/estimates/en/ (accessed 11 April 2016). (who.int)
- Cochlear implanted children with prelinguistic sensorineural bilateral deafness of profound degree, using either the ACE or SPEAK coding strategy, were evaluated and compared. (cun.es)
Adult3
- Adult Refsum disease may be divided into the adult Refsum disease 1 and adult Refsum disease 2 subtypes. (wikipedia.org)
- Adult Refsum disease should not be confused with infantile Refsum disease, a peroxisome biogenesis disorder resulting from deficiencies in the catabolism of very long chain fatty acids and branched chain fatty acids (such as phytanic acid) and plasmalogen biosynthesis. (wikipedia.org)
- Based on the above, it was proposed that adult Refsum disease could be divided into types 1 and 2, depending on which gene is defective. (medscape.com)
Bilateral2
- Preoperative high-resolution computed tomography of the temporal bones showed bilateral inner ear malformations of both the cochlear and vestibular labyrinth, conditions consistent with bilateral Mondini deformity ( 5 ). (cdc.gov)
- The differences of bilateral SIR were compared between Meniere disease and healthy controls, and the correlation was examined between the SIR of the affected ear and the grades of cochlear and vestibular hydrops in Meniere disease. (msdmanuals.com)
Inner ear2
- The sound is then transmitted to the fluid and delicate hair cells of the inner ear (cochlear). (healthhub.sg)
- During cochlear implant surgery, electrodes are threaded into the inner ear while the body of the device is secured beneath the scalp behind the ear. (luriechildrens.org)
Nerve3
- SNHL in CMT is hypothesized to result from disruption of synchronous activity of the cochlear nerve. (hindawi.com)
- Most meningitis cases were associated with an implant with a positioner, a silastic wedge inserted next to the implanted electrode in the cochlea to position the electrode closer to the cochlear nerve endings and thus facilitate electrical signal transmission. (cdc.gov)
- Although considered a common childhood disease, the varicella zoster virus can reactivate later in life and cause a painful sensory-nerve rash called shingles. (texas.gov)
Unilateral1
- Unilateral CI across all age groups leads to reported sustained benefits in the recipients' overall and disease-specific QoL. (karger.com)
Diabetes3
- Certain components of the disease (such as obesity, diabetes, and renal problems) when noticed earlier offer a disease management benefit to the patients. (ijo.in)
- Individuals at higher risk of developing pneumococcal bacteremia include those with underlying health conditions, such as diabetes or chronic liver disease. (total-health-care.com)
- However, the fact is that the number of heart disease cases among the population below 30 years of age are increasing dramatically worldwide, majorly due to lifestyle choices which include but not limited to alcohol abuse, excessive smoking, obesity, diabetes, and others. (metrohospitals.com)
Tinnitus1
- Cochlear damage may also manifest as tinnitus. (medscape.com)
Centers6
- Centers for Disease Control and Prevention. (cdc.gov)
- [ 1 ] According to the US Centers for Disease Control and Prevention (CDC), of babies screened in the United States in 2019, 1.7 per 1000 had hearing impairment. (medscape.com)
- For more information, see Pneumococcal Advisory Committee on Immunization Practices Vaccine Recommendations and Centers for Disease Control and Prevention (CDC): Pneumococcal Vaccination . (merckmanuals.com)
- The Centers for Disease Control and Prevention (CDC) provides recommendations regarding pneumococcal vaccination for different age groups and high-risk individuals. (total-health-care.com)
- ACIP is chartered as a federal advisory committee to provide expert external advice and guidance to the Director of the Centers for Disease Control and Prevention (CDC) on use of vaccines and related agents for the control of vaccine-preventable diseases in the civilian population of the United States. (cdc.gov)
- The Centers for Disease Control and Prevention (CDC) cannot attest to the accuracy of a non-federal website. (cdc.gov)
Surgically3
- The cochlear implant is a surgically placed device that converts sound to an electrical signal. (medscape.com)
- A cochlear implant is an electronic device that is surgically implanted. (healthhub.sg)
- When he was just over six months old, Max's ears were both surgically implanted with a cochlear implant, an electronic device that allowed him to hear, learn to understand speech and speak clearly. (luriechildrens.org)
Vertigo1
- Vertigo is caused by both peripheral and central vestibular diseases. (physio-pedia.com)
Infections3
- If the rheumatic heart disease is diagnosed in the early stages with minimum damage to the heart valve, Antibiotics are used to treat strep infections and prevent rheumatic fever thereof. (metrohospitals.com)
- Before a vaccine was available, Hib disease was the most common cause of meningitis in children under the age of five, and often led to pneumonia, skin infections, hearing loss, permanent brain injury or death. (texas.gov)
- The vaccinations that are offered to all children protect against eleven diseases: rotavirus infection, diphtheria, tetanus, whooping cough, polio, infections caused by Haemophilus influenzae type b, measles, mumps, rubella, serious diseases caused by pneumococcus and human papillomavirus (HPV). (folkhalsomyndigheten.se)
Hearing loss11
- Early cochlear dysfunction, thereby indicating a subclinical hearing loss, was identified in stages 3, 4 and 5 by DPOAE testing. (smu.ac.za)
- Participants in the later stages of CKD presented with early cochlear dysfunction, presenting with subclinical hearing loss. (smu.ac.za)
- Wang, SE & Wu, CH 2021, ' Tau phosphorylation and cochlear apoptosis cause hearing loss in 3×Tg-AD Mouse Model of Alzheimer's Disease ', Chinese Journal of Physiology , vol. 64, no. 2, pp. 61-71. (ntnu.edu.tw)
- For patients with hearing loss that is not mitigated by hearing aids, a cochlear implant may provide an opportunity for hearing. (medscape.com)
- When hearing loss is more serious or when hearing is entirely impaired, a hearing or cochlear implant is a more effective option. (healthhub.sg)
- It is never "too late" to get a cochlear implant, nor is age-related hearing loss just something to put up with, said Dr Heywood. (healthhub.sg)
- Investigations of hearing loss associated with the presence of systemic autoimmune disease, e.g. (nih.gov)
- Many studies have focused on the relationship between hearing loss and Alzheimer's Disease (AD). (tinnitusjournal.com)
- These preliminary findings clearly delineate the importance of further research aimed at investigating hearing impairment in AD, to a) allow early detection of people with predisposition to AD, b) improve the quality of life in AD patients with hearing loss and c) possibly prevent the progression of the disease treating the hearing impairment. (tinnitusjournal.com)
- Examples included presbycusis, noise-induced hearing loss, Ménière disease, and retrocochlear lesions such as vestibular schwannoma. (medscape.com)
- Idiopathic (unexplained) hearing loss 119 SNHL children having cochlear Student t-test was used to determine may be the result of an infectious disease implant surgery, ranging in age from 3 significant differences in means and or an injury [1]. (who.int)
Patients17
- Cochlear function in patients with chronic kidney disease. (smu.ac.za)
- To evaluate cochlear functioning in patients (18-45 years old) with varying stages of chronic kidney disease (CKD). (smu.ac.za)
- Patients in stages 1 and 2 presented with normal puretone thresholds and DPOAEs, suggesting that cochlear functioning in these patients was normal. (smu.ac.za)
- It was concluded that audiological monitoring be included in the management of patients with CKD and that DPOAEs be introduced as part of the test battery to monitor cochlear function in patients with varying degrees of CKD. (smu.ac.za)
- Dive into the research topics of 'Cochlear function in patients with chronic kidney disease. (smu.ac.za)
- As increased permeability of the blood-labyrinth barrier precedes the formation of endolymphatic hydrops, it is suggested that disruption of the blood-labyrinth barrier may contribute to the development of Meniere disease in patients," the authors write. (msdmanuals.com)
- However, the awareness of the disease is comparatively low and most often noticed only after severe vision loss in patients, which is usually in the first decade of the patient's age. (ijo.in)
- The review in nutshell would provide the basic awareness of the disease that will have an impact in disease management and counseling benefits to the patients and their families. (ijo.in)
- We help patients understand what is known and what is not known about their disease and treatments to help them make the best treatment decisions. (tuftsmedicalcenter.org)
- Therefore, polymyalgia rheumatica is a concomitant disease frequently seen in patients with giant cell arteritis. (proprofs.com)
- MS patients will experience the condition in one of four disease courses (types of MS): Primary progressive MS, secondary progressive MS, relapsing-remitting MS, or clinically isolated syndrome. (hearingresearch.org)
- Speech Recognition During Follow-Up of Patients with Ménière's Disease: What Are We Missing? (cun.es)
- Distortion product otoacoustic emissions (DPOAEs) were absent in all ears tested and the cochlear microphonic (CM) was increased in amplitude and duration in young patients and absent in the two oldest subjects. (harvard.edu)
- Global Kinetics' Personal KinetiGraph (PKG), a wearable device for Parkinson's disease patients, provided more information about symptoms than a routine clinic visit, a recent study found. (massdevice.com)
- Medtronic (NYSE:MDT) said today that it won expanded approval for its deep-brain stimulation therapy for patients with Parkinson's disease, which 1st won approval from the safety watchdog in 2002. (massdevice.com)
- Patients with Refsum disease are unable to degrade phytanic acid because of a deficient activity of phytanoyl-CoA hydroxylase (PhyH), a peroxisomal enzyme catalyzing the first step of phytanic acid alpha-oxidation. (medscape.com)
- Blood levels of phytanic acid are increased in patients with Refsum disease. (medscape.com)
Dysfunction1
- 0.05) with DPOAEs being more sensitive in identifying early cochlear dysfunction. (smu.ac.za)
Otolaryngology-Head and Neck Surgery1
- The Clinical Practice Guideline: Ménière's Disease in Otolaryngology-Head and Neck Surgery has been published today. (news-medical.net)
Symptoms4
- What are the symptoms of pneumococcal disease? (hse.ie)
- Chest pain -Discomfort or severe pain in the chest or heaviness radiating to your left arm and back are the symptoms of the heart disease. (metrohospitals.com)
- Symptoms of the disease include fever, general feeling of illness and a rash of blister-like bumps. (texas.gov)
- Reuters) - The FDA last week said it approved a brain implant from St. Jude Medical (NYSE:STJ) that helps reduce symptoms of Parkinson's disease and essential tremor, a neurological disorder that causes rhythmic shaking. (massdevice.com)
Alzheimer's2
- On this Mayo Clinic Radio podcast, Dr. Michelle Mielke , a Mayo Clinic epidemiologist and an author on the study, explains how memory testing is used to diagnose Alzheimer's disease. (mayoclinic.org)
- Alzheimer's Disease (AD) is an acquired condition characterized by progressive cognitive and behavioural decline and is the second most common form of dementia in the general population after mild cognitive impairment[ 1 ]. (tinnitusjournal.com)
Hemoglobinopathies1
- cochlear hemoglobinopathies. (cdc.gov)
Infection5
- Pneumococcal disease is a serious infection caused by the bacterium Streptococcus pneumoniae, also known as pneumococcus. (total-health-care.com)
- Pneumococcal disease is a bacterial infection caused by streptococcus pneumoniae of which there are more than 90 serotypes. (hse.ie)
- Hepatitis B is a potentially serious infection of the liver that can cause chronic liver disease and can lead to liver cancer. (texas.gov)
- The younger a person is when becoming infected, the greater the risk of chronic infection, chronic liver disease and liver cancer. (texas.gov)
- The true global burden of cCMV disease is likely underestimated because most infected infants (85-90 %) have asymptomatic infection and are not identified. (cdc.gov)
Evaluate1
- We sought to evaluate the outcomes of cochlear implant performance stratified by age. (scirp.org)
Meningitis2
Spiral Ganglion2
- Our histochemical staining evidence showed the cochlear spiral ganglion neurons (SGN), but not the cochlear hair cells, were lost significantly in the cochlea of 3×Tg-AD mice from 9 months of age and thereafter. (ntnu.edu.tw)
- This electrical signal is transmitted via electrodes to the spiral ganglion cells in the cochlear modiolus. (medscape.com)
Invasive pneumococcal3
- PCV13 is effective against acute otitis media, pneumonia, and vaccine (PPSV23 [Merck Sharp & Dohme LLC]) have been invasive pneumococcal disease (IPD) in children ( 5 - 7 ). (cdc.gov)
- The decrease in this age group can largely be attributed to a 98% decline in Invasive pneumococcal disease (IPD) due to serotypes covered by PCV7 between 2008 and 2016. (hse.ie)
- Background: Chronic conditions increase the risk of invasive pneumococcal diseases (IPD). (bvsalud.org)
Acoustic1
- However, the precise therapeutic intervention must be based on a deeper understanding of its dynamic molecular distribution and function in cochlear pathogenesis after acoustic trauma. (frontiersin.org)
Ears1
- The SIR was higher in the unaffected ear in Meniere disease than that of both ears in healthy controls. (msdmanuals.com)
Refsum Disease19
- Refsum disease is an autosomal recessive neurological disease that results in the over-accumulation of phytanic acid in cells and tissues. (wikipedia.org)
- Refsum disease typically is adolescent onset and is diagnosed by above average levels of phytanic acid. (wikipedia.org)
- Individuals with Refsum disease present with neurologic damage, cerebellar degeneration, and peripheral neuropathy. (wikipedia.org)
- citation needed] Refsum disease is a peroxisomal disorder caused by the impaired alpha-oxidation of branched chain fatty acids resulting in buildup of phytanic acid and its derivatives in the plasma and tissues. (wikipedia.org)
- In general, Refsum disease is caused by PHYH mutations. (wikipedia.org)
- Refsum disease is inherited in an autosomal recessive pattern, meaning that it requires both copies of the mutation to inherit the disease. (wikipedia.org)
- Refsum disease 2 stems from mutations in the peroxin 7 (PEX7) gene. (wikipedia.org)
- Since phytanic acid is not endogenously produced in the human body, individuals with Refsum disease are commonly placed on a phytanic acid-restricted diet and avoid the consumption of fats from ruminant animals and certain fish, such as tuna, cod, and haddock. (wikipedia.org)
- Refsum disease (RD) is a neurocutaneous syndrome that is characterized biochemically by the accumulation of phytanic acid in plasma and tissues. (medscape.com)
- In Refsum disease (RD), the phytanic acid level in the blood is increased. (medscape.com)
- Refsum disease can be classified as a peroxisome biogenesis disorder. (medscape.com)
- [ 1 ] Infantile Refsum disease is a peroxisome biogenesis disorder. (medscape.com)
- Refsum disease is a recessive disorder characterized by defective peroxisomal alpha-oxidation of phytanic acid. (medscape.com)
- [ 7 ] A Refsum disease gene, phytanoyl-CoA hydroxylase ( PHYH ), has been localized to band 10p13 between the markers D10S226 and D10S223. (medscape.com)
- [ 8 ] Refsum disease is genetically heterogeneous, with up to 55% of cases not being linked to the PAHX gene locus at D10S547 to D10S223. (medscape.com)
- Recently, a mouse model for Refsum disease ( Phyh knockout mouse by targeted disruption of the PHYH gene). (medscape.com)
- An infantile form of Refsum disease also exists and is an autosomal recessive disorder of peroxisomal biogenesis, leading to many biochemical abnormalities, including elevated plasma concentration of phytanic acid, pristanic acid, very long chain fatty acids, and C27 bile acids. (medscape.com)
- Refsum disease is rare, with just 60 cases published worldwide. (medscape.com)
- Infantile Refsum disease makes its appearance in early infancy. (medscape.com)
Infectious Diseases1
- Community-acquired pneumonia (CAP) is one of the most common infectious diseases and an important cause of mortality and morbidity worldwide. (medscape.com)
Vaccines3
- Vaccines are directed against many of the serotypes that cause disease. (merckmanuals.com)
- CDC's National Center for Immunization and Respiratory Diseases (NCIRD) maintains the most current immunization schedules on the Vaccines and Immunizations pages of CDC's website ( http://www.cdc.gov/vaccines/schedules ), including the schedules published in this supplement. (cdc.gov)
- Most vaccines that are part of the Swedish child vaccination programme are given as combination vaccines, i.e. vaccines against several diseases are given in the same shot in order to minimize the number of injections. (folkhalsomyndigheten.se)
Pneumococcal diseases1
- The pneumococcal vaccine plays a critical role in protecting individuals, particularly those who are more susceptible to pneumococcal diseases. (total-health-care.com)
Severity1
- The Treatment depends upon the severity of the damage caused to the heart valve by the disease. (metrohospitals.com)
Electrodes1
- The electrodes were introduced without resistance, and a full insertion was achieved using the MED-EL™ Synchrony Flex® 28 cochlear implant. (hindawi.com)
Disorders3
- Charcot-Marie-Tooth (CMT) disease is a clinically and genetically heterogeneous group of disorders affecting the peripheral nervous system. (hindawi.com)
- Our Glomerular Disease clinic is a multi-disciplinary clinic designed to provide comprehensive diagnosis and treatment of kidney glomerular disorders. (tuftsmedicalcenter.org)
- Otonomy, Inc., a biopharmaceutical company developing and commercializing innovative therapeutics for diseases and disorders of the inner and middle ear, today announced it has secured $49 million in an oversubscribed Series D round of financing. (news-medical.net)
Exposure2
- Here, we have presented the spatiotemporal dynamics of the expression of HMGB1, exhibiting distribution variability in specific cochlear regions and cells following noise exposure. (frontiersin.org)
- The disease is spread through exposure to infected blood and body fluids. (texas.gov)
Surgery3
- Upon initial activation 3 weeks after surgery, the patient reported excellent access to sound in the cochlear implant-only condition. (hindawi.com)
- Rheumatic Heart Disease can lead to various other complications such as a ruptured heart valve, which would require required immediate surgery or replacement of a heart valve. (metrohospitals.com)
- Cochlear implant surgery is very safe and takes about two hours, Dr Heywood says. (healthhub.sg)
Asplenia1
- Pneumococcal disease can lead to significant morbidity and mortality, particularly amongst the very young, the very old, those with impaired immunity and those with anatomic or functional asplenia. (hse.ie)