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This shows a cross-section of the cochlea with the vestibular membrane, the membrane that separates endolymph and perilymph, bulging from excess endolymph. The term endolymphatic hydrops refers to a state of excessive fluid within the endolymphatic space.
Learn how Metropolitan NeuroEar Group gives back to the community through numerous philanthropic efforts. There are many sides to tinnitus suffering, but one of the more serious conditions that can physically cause aches, pains, and frustration is called Meniere’s Disease.
An attack typically starts with ear pressure as if the ears were full of water and being pushed on.
Injury tends to remove the “control” aspect of your ears inner workings causing fluid to no longer stay constant but rather to change with the body’s own fluids and blood pressures.
Many have attacks in close proximity to one another causing great frustration and making anything being done at the time increasingly difficult. Overall you want to maintain a low sodium diet, no caffeine, and keep sugary foods to a minimum. For some Meniere’s Disease can be permanent and managing it is the best you can hope for, but the condition is well known and easy to manage if you have the right information and put forth the effort to keep your diet under control. Our award-winning blog brings you insights on health, nutrition and wellness from experts you can trust. What is Meniere’s syndrome?Meniere’s syndrome (also known as endolymphatic hydrops) is the result of a fluid imbalance in the inner ear. In this post I discuss about interesting diseases, most people will consider these as weird diseases. Marco Fraccaro, discovered this syndrome in 1952 and he characterized this still born baby with severe micromelia and the cartilage change and described forms of chondrodys plasia, in human which are very lethal before or after birth. Apert sybdrome is a rare disorder (I also consider this as one of the interesting diseases) described by craniosynostosis, craniofacial, severe symmetrical syndactyly (cutaneous and bony fusion) of the hands and feet.
The major cause of this syndrome is the fetal Akinesia (decrease fetal movements) because of fetal abnormalities (neurogenic. Jeune syndrome or asphyxiating thoracic dystrophy, is an inherited form of dwarfism characterized by short limbs, small chests and kidney problems. Cornelia De Lange Syndrome (CdLS) is a genetic disorder that can lead to severe developmental disorder affecting physical and intellectual development of a child.
Cri-du-chat syndrome otherwise known as chromosome 5p deletion syndrome, 5p minus syndrome or Lejeune’s syndrome, a rare genetic abnormalities. Crouzon syndrome or Bronchial-arch syndrome, lower ears on the fetus than of the adults, resulting to loss of hearing and in most cases, Menieres’ disease happened. Fabry disease also known as Angiokeratoma Corporis Diffusum, Alpha-galactosidase A difficiency, a rare X-link recessive lysosomal storage disease, which can cause a wide range of systemic symptoms lysosomal storage disease a group of 40 rare inherited metabolic disorders that result from defects diagnosed through blood tests to measure the level of Alpha-Galactosidase activity but misleading to the female carriers due to random nature of X-inactivation. Treacher-Collins syndrome or Treacher-collins Franceschetti syndrome or TCS, is a rare genetic abnormality describing TCS by craniofacial deformity, downward slanting eyes, micrognathia (small lower jaw), deaf, undeveloped skull-bone(Zygoma), drooping part of lateral lower eyelids, missing ear or deformed ears. Klipper-Feil syndrome is a rare disease describing congenital fusion of any 2 of the 7 cervical vertebrae, and occurs in heterogenous group of patients unified only by the presence of a congenital defect in the formation or segmentation of the cervical spine.
Menkes Kinky Hair syndrome, an inherited X-linked recessive abnormality related to deficient copper levels of the cells. The Neurofibromatosis (NF) this weird disease is a inherites genetics abnormality that affects the nerve tissue grows tumors (neurofibromas), may be harmless but causes damages by other tissues and compressive nerves. Noonan syndrome patient’s have unusual faces and multiple malformation that includes congenital heart disease. WHS have characteristic dysnorphic facial features, prominent glabella, hypertelorism, beaked nose, frontal bossing and described as “Greek Warrior Helmet” faces.
Waardenburg syndrome have higher risks for neural tube disorder, cleft lip and palate are present in some cases, limba abnormality and Hirschsprung disease is present, hearing impairment, dystopia Canthorun and pigmentary disorder of the hair, skin and eyes. Trisonomy 18 or commonly called Edwards syndrome, is another weird disease characterized by severe pschomotor and growth retardation, microcephaly, microphhtalmia, deformed ears, micrognathia or retrognathia, microsostomia, clenched fingers and other congenital deformity.

Ellis Van Creveld or Chondroectodermal dysplaxia is one of the interesting diseases I have researched about. Kearns-Sayre syndrome describes the onset of ophthalmoparesis and pigmentary retinophaty before age 20.
Klinefelter Syndrome is another weird disease in which the male patients described as having enlargement of breasts, sparse facial and body hair, small testes and inability to produce sperm, have an extra sex chromosome (genotype XXY), instead f the usual male sex complement (genotype XX) and this is the common chromosonal malfunction related to male hypogonadism and infertility. Endolymphatic hydrops can be caused by a number of diseases that can affect the rest of the body.
Sanjay Prasad MD FACS is a board certified physician and surgeon with over twenty years of sub-specialty experience in Otology, Neurotology, advanced head and neck oncologic surgery, and cranial base surgery.
Typically, those with this type of tinnitus get attacks, much like panic attacks but more physical and geared in the ears. It’s incredibly taxing to the energy and comfort levels often leading to prolonged naps from exhaustion. Normally, this fluid stays at a consistent rate and contains concentrations of sodium, potassium, chloride, and electrolytes which allow the inner ear sensory cells to function the way they are supposed to. This means stress, for example, can cause intense pain in the ears as can exercising and times when the heart is pumping harder than usual.
Things like taking in high salt or sugary diets, this increase can dis-balance the inner ear fluids. You must change your diet to help balance and maintain a good balance of the inner ear fluids. If possible, eliminate alcohol and tobacco completely- these also agitate the condition and can make it much worse. Many canned foods contain a lot of sugar so it’s not just about avoiding candy and soft drinks!
This method examines new born infant for genetic conditions that are treatable, these genetic conditions includes, hematologic and metabolic illnesses as well as endrocrinology.
Infant with Achondogenesis type ll, have larger heads, prominent and large forehead, flat-facial plane, flat nasal bridge, small nose with severe adverted nostrils, micrognathia, extremely short neck, short and flared thorax, protuberant abdomen and short upper extremities.
Cornelia de Lange’s syndrome features are low birth weight under 5lbs, developmental delay, missing portion of limbs, microcephaly, thick eyebrows meeting at synophrys (midline), long eyelashes, short upturned-nose, thin down-turned lips, long philtrum, excessive body hair, small hands and feet, small widely spaced teeth, hearing impairments, Vision disorder (ptosis, nystagmus, high myopia, hypertropia), seizures, partial joining of the second and third toes. Most common in female and described and signs of symptom are low-muscle tone (hypotonia), small-heads(microcephaly), growth retardation, round face with full cheeks, hypertelorism, epicanthal folds, down-slanting palpebral fissures, flat nasal bridge, down-turned mouth, micrognathia, low-set ears, short fingers, single palmer creases and heart defects.
Menieres disease is the disorder of the inner ear that can effect hearing and balance to a varying degree, characterized by episodes of vertigo(dizziness, nausea and vomiting), tinnitus(a ringing sound or perception of sound within the human ear in the absence of corresponding external sound) and progressive loss of hearing affecting one ear only in most cases.
Enzyme replacement therapy is not a cure, but can allow normal metabolism and prevent the progression of the disease as potential reverse symptoms. Related abnormality are scoliosis (side to side curvature of the spine), spina bifida, kidneys and ribs malfunction, cleft lip and palate,respiratory problem and heart disorder connected to the head and face, skeleton, sex organs, muscle, brain, spinal cord, arms and legs, fingers. Signs and symptoms are floppy baby(hypotonia), pudgy-rosy cheeks, irritable, brittle-kinky hair, seizures, difficult to feed, bone spurs and low body temperature. In most cases it affects all neural crest cells, schwann cells, melaocycles endoneural fibro blasts. Noonan syndromes are oftentimes identified as Turner Syndrome because of the most common symptoms and sign seen in Noonan syndrome, like short statures, mental-retardation, neurologic skeletal problem, genitourinary, lymphatic eye and skin findings may present to various degrees and bleeding diathesis is present in most cases of Noonan syndrome. It is characterized of mental retardation, seizures, facial appearance and midline closure deffect. The three main features are port-wine stain (nevus flammeus), deformities of lymphatic and venous, and hypertrophy soft tissue affecting limb. Ellis Van Creveld Syndrome is characterized by disproportionate dwarfism, postaxial polydactyly, ectodermal dysplaxia, small chest and high frequency of congenital heart problems and characterize autosomal recessive syndrome, which has increase occurrence with people of Old Order Amish descent. The bilateral sensorineural hearing loss is almost universal for those surviving the 4th year of life and hearing impairment cannot be corrected by hearing aids.
Adolescent male with Klinefelter syndrome has female-type distribution of pubic hair and testicular dysgenesis).

These include metabolic disorders such as diabetes, low or over-active thyroid functioning, and high cholesterol or triglyceride levels.
He is chief surgeon and founder of the private practice, Metropolitan NeuroEar Group, located in the metropolitan Washington D.C. Some symptoms of a severe attack include vertigo (dizziness, spinning feelings), the body balance feels strange and imbalanced, feeling ill (feelings of vomiting or nausea), and even exhaustion from experiencing these symptoms for extended periods intensely.
When something injures the inner workings of the ear such as concussion, falling off a bike, car accident, or anything that can shake up the inner workings of your ears.
This misbalance changes the balance of trace minerals in the fluid enough to cause vertigo, hearing loss, hot and cold spells, and other discomfort. Maintaining a regular meal plan that is consistent (including snacks) helps the body to maintain balance. The fetal akinesia can cause polyhydramnios, pulmonary hypoplasia, micrognathia, ocular hypertelorism and short umbilical cord.
Mental disorder is progressive and in most cases death occurs in the first year of baby’s life.
Most tumors causes bumps under the skin, colored spots, skeletal problems, pressure on spinal nerve roots.
Usual cause of death in WHS, is heart failure, aspiration of pneumonia, infections and seizure. The symptoms diagnosed on birth defect by the presence of a combination of these symptoms: One or more noticeable port-wine stains (commonly called birth marks) with sharp borders, varicose veins, increase in the volume of an organ or tissue due to the enlargement of its cells (hypertrophy) component of bony and soft tissues, that can result to local gigantism or shrinking, and the undeveloped lymph system.
For infants narrow chest and not proportioned dwarfism and postaxial polydactyly are visible signs of this syndrome. There are a number of autoimmune and infectious disorders that can also cause these symptoms. The process comprises pricking a 2 day old new born baby’s heel; thus the blood specimen is used for the examination. There are some cases, port-wine stains (capillary port wine type) are not present, and this are very rare cases and may be classified as atypical Klippel–Trenaunay syndrome. What is the purpose of a steroid injection for Meniere’s syndrome?Steroids are a powerful anti-inflammatory medication when placed in the ear in small doses. KTS can either affect blood vessels, lymph vessels, or both, but this syndrome condition commonly presents with a mixture of the two. When used in Meniere’s syndrome, a steroid injection reduces the frequency and severity of vertigo attacks. Those with affected venous or lymphatic condition can experience increased pain and complications. This Klippel-Trenaunay-Weber syndrome’s birth defect affects men and women equally, and is not limited to any racial group. There is also about a one percent chance that the hole will remain in the eardrum, which could require surgery to correct. What is the procedure for receiving a steroid injection?The procedure takes approximately 30 minutes.
First, patients lie down flat on their back on the examining table with head turned so that the affected ear is facing up.
The needle contains a small amount of a steroid (dexamethasone is commonly used) sometimes mixed with a very small amount of another ‘numbing’ drug (lidocaine is commonly used). What happens after the procedure?After the procedure, patients will lie down flat on their back on the examining table with head turned so that the treated ear is facing up.

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