الاثنين، 30 يوليو 2012

Syndromes (syndromes) associated with the lack of hearing

Syndromes (syndromes) associated with the lack of hearing
Syndromes Associated with Hearing Loss
Message to obtain a Doctor of Medicine at the University of DamascusBy Dr. Spring WarUnder the supervision of Prof. Dr. Mohammed Samer Postal



WEDMAN SPRANGER Syndrome(Vertebral dysplasia - congenital epiphyseal)

    
Sensorineural hearing deficiency 30% Sensoneural Hearing Loss.

    
Palace at the ends, and warp in the thigh.

    
Lack of tonic muscle.

    
Palace of the trunk with the duck walk.

    
Walking was delayed until the thirtieth week.

    
Palace eyes severe Severe Myopia retinal detachment may occur.

    
Cleft palate split 40% Cleft palate.


AMALRIC Syndrome

    
Habibi ill epithelium pigmentary macular Granular macular epitheliopathy.

    
Lack of sensory neural hearing.

    
Syndrome may be hereditary or acquired after infection and congenital German measles.


BARANY Syndrome

    
Headache Agafoa Back Headache.

    
AC was designed in the approval (peppered with periods where the normal hearing).

    
Dizziness and tinnitus Vertigo, Tinnitus.

    
This syndrome can be corrected Balreroh raised Induced Nystagmus.
BONNIER Syndrome

    
This syndrome is due to hit the nucleus of Deiters and contacts.

    
Visual disturbances Ocular Disturbances.

    
Matching paralysis Paralysis of Accommodation, nystagmus Nystagmus, diplopia Diplopia.

    
Designed Deafness, nausea Nausea, thirst Thirst, the top of Anorexia.

    
Some of the symptoms resulting from infection centers Almpehmah Vagal Centers, and the cranial nerves (8-9-10-11) and lateral vestibular nucleus.

    
Can induce disease Munir MENIERE disease.

 
BRANCHI0 - OTO - RENAL syndrome
(Nephrotic syndrome ear Alglsomah)


Is a genetic disorder characterized by injury of my body and the middle ear and internal phenomenon associated with abnormalities in the tissues in front of the ear Preaucular Tissues in the grooves Alglsomah Branchial Clefts. With varying degrees of poor kidney dystrophic includes many of the following symptoms:

    
Lack of conductive or mixed hearing MIXED or CONDUCTIVE hearing Loss.

    
Flap in the cup CUP SHAPED PINNAE swerved to the front with two side slits in front of the ear.

    
Fissures or fistulas bilaterally.

    
Renal dysplasia RENAL DYSPLASIA.


COGAN syndrome

    
This syndrome includes interstitial keratitis is syphilis Non Syphilitic interstitial keratitis.

    
Audio-vestibular symptoms Vestibuloauditory symptoms.

    
Interstitial keratitis leading to a decrease in the ability fast visual.

    
Symptoms include severe dizziness conjugated with paroxysmal tinnitus Tinnitus, sudden nystagmus Spontaneous Nystagmus, ataxia Ataxia, and the lack of sensory neural hearing Advanced Progressive Sensoneural Hearing Loss, permeates the cases of remission Remission and intensifying Exacerbation.

    
This may be a syndrome that relates to inflammation around the nodal artery Periarteritis nodosa.

    
Histopathology shows atrophy of the spiral ganglion with edema in the membrane of the cochlea and semi-circular canals Semicircular Canals accompanied by inflammation of the spiral ligament Spiral ligament.


DIDMOAD Syndrome

     
This syndrome is transmitted as an autosomal recessive hereditary includes: Albuminuria Tfhh DI, Diabetes DM, optical atrophy OA, designed and D.

    
Diabetes is usually the kind of youth on juvenile insulin Albelh insipidus may occur at any time for a sensitive Vosopressine refers to atrophy in the cells of the hypothalamus pituitary Hypothalamus or the way the upper visual Supraoptico Hypophiseal tract.

    
Lack of hearing in this case sensory nervous advanced and affects the high frequencies first Higher Tones.

    
Injuries range from urinary bladder of no rain until Artakaúah renal and ureteral Hydronephrosis, Hydroureter.


18Q Syndrome

     
This syndrome includes delays in the development of the spiritual dynamic: Psychomotor retardation.

     
Lack of vitamin Hypotonia - short stature short stature - small in the head Microcephaly - minus half the face of dystrophic Hypoplastic midface - lush Moukah Epicanthus.

    
Injuries in kind Ophtalmologic abnormalities - a split in the dome of the palate Cleft Palate.

    
Injury congenital heart Congenital heart disease - defects in the genital Abnormalities of the genitalia - fingers pointed Tapered Fingers.

    
Lack of hearing conductive Conductive hearing Loss.


FOVILLE Syndrome

    
Paralysis of the face Facial paralysis.

    
Paralysis of vision compatible Conjugate gaze.

    
Pyramidal hemiplegia on the opposite side Contralateral pyramidal hemiplegia.

    
Tinnitus Tinnitus, vertigo and designed Deafness and vertigo.

    
Injury to the nuclei under Infranuclear involvement.

    
A lack of taste in the anterior two-thirds of the tongue Loss of taste.

    
Lack of salivary secretions Salivary and lacrimal Lacrimal nerve injury with average Nervus intermedius.
HANHART Syndrome


This syndrome is a poor conformation types of facial Facial dysmorphia, which leads to:

    
The appearance of the bird Bird like profile because of the small lower jaw Micrognathia and includes:

    
Dental background positioning Opishodontia.

    
Deformation parties Peromelia.

    
Lack of growth of Small growth.

    
Conductive hearing deficiency due to poor conformation Algsama.

    
Malformations of the tongue in the palate with small Small jaw.

    
Shortage in the limbs sometimes.

    
And the ratio of normal intelligence Normal intelligence.


Large Vestibular Aqueduct syndrome
(Vestibular canal syndrome WAN)


Vestibular canal wide Ki_oh associated with isolated sensorineural hearing deficiency is more common in childhood, but in this syndrome is the coarse Rugose portion of the internal bag lymph Endolymphatic sac is also dilated. Interventions on the endolymphatic sac to correct the hearing loss is usually not successful.


SCHAFER Syndrome

    
Mental retardation and hereditary Hereditary mental Retardation.

    
Lack of sensory neural hearing Sensoneural Hearing loss.

    
Hematuria Hematuria.

    
Epilepsy Epilepsy.

    
Lack of Proline oxidase.
TROTTER Syndrome

    
Lower jaws of neuralgia Neuralgia.

    
Lack of hearing conductive Conductive hearing loss.

    
Channel blockade in Eustache Eustashian tube Blockage.

    
In front of the ear edema Preauricular edema due to the invasion of plant in the pocket of Morgagni.

    
Compatible paralysis Ipsilateral Akinesia in the soft palate.

    
Trismus Trismus.


NANSE Syndrome

 
Inherited syndrome affects males only and includes:

    
Middle ear infection and invisible Medial and Inner Ear Involvement.

    
Hardening of the passengers.

    
Vestibular injury Vestibular Abnormalities.

    
Deafness and designed with you.


FORNEY Syndrome

    
Autosomal dominant Autosomal dominant.

    
Disease moles Lentigines.

    
Mitral insufficiency Mitral insufficiency.

    
Structural abnormalities Skeletal malformations.

    
Lack of hearing conductive Conductive hearing loss.
LENTIGINES Syndrome

    
Autosomal dominant Autosomal dominant.

    
Brown patches on the skin begin to age two Brown Spots.

    
Increase in the capacity dimensions of the eye Ocular hypertelorism.

    
Pulmonary stenosis Pulmonary stenosis.

    
Abnormalities in the genital Abnormalities of genitalia.

     
Growth retardation Retarded growth.

    
Lack of sensory neural hearing Sensoneural hearing loss


PIEBALDNESS Syndrome
(Trout skin syndrome)

    
Hereditary sex-linked Sex linked recessive or my body is Autosomal.

    
Blue iris Blue irides.

    
Retinal pigmentation minutes Fine retinal pigmentation.

    
Lack of pigmentation of the hair and scalp Scalp Hair and face.

    
Hypopigmented areas on the limbs and trunk.

    
Lack of sensory neural hearing.
HAND-HEARING Syndrome

     
And autosomal dominant

    
Turning Tqfie Boys Congenital flexion contracture in the fingers and thumb of the foot Toe.

     
Lack of sensory neural hearing.


MARFAN Syndrome

     
(Spider fingers Arachnodactyly, lens meridian Ectopia lentis, was designed (Deafness.

    
Autosomal dominant.

     
People with long slender fingers and a long spider.

    
Issued erythema Pigeon breast.

    
Scoliosis Scoliosis.

    
Hammer toes are Hammertoes.

    
Lack of hearing mixed.


PIERRE ROBIN Syndrome

     
Autosomal dominant with variable permeability.

    
Occurs in about 1/30000 - 1 / 50,000 births.

    
May be due to intrauterine infection.

    
The emergence of the tongue Glossoptosis.

    
Small lower jaw Micrognathia.

    
Palate Cleft palate.

    
Lack of hearing mixed.

    
Mental retardation.

    
Deformity in the pinna.

    
Hypoplasia in the lower jaw Hypoplastic mandible.

    
Narrowing under Bagpipe Subglottic Stenosis.

    
Inhalation is the most common cause of death Aspiration.


ROAF Syndrome

     
Is hereditary.

    
Separation of the retina Retinal Detachment.

    
Cataract Cataract.

    
Palace eyes Myopia.

    
Hragafh varus Coxa Vara.

    
Humping scoliotic Kyphoscoliosis.

    
Lack of sensory neural hearing Advanced.


VANDER HOEVE Syndrome
Osteogenesis imperfecta
(Lack of bone formation)

     
Autosomal dominant with variable permeability.

    
Bones are fragile, with the loss of Fragile Bones ligaments Ligaments.

    
Solid blue or clear Clear Sclera, triangular face, the lack of dentin.

    
Solid blue and the lack of hearing Bashahdan in 60% of cases and most after the age of twenty.

    
Conductive hearing deficiency can be attributed to demonstrate Passenger Stapes Fixation result of hardening of the bone to break or Otosclerosis in the auditory ossicles.

    
The main disturbance is the effectiveness of the abnormal cells of bone Albanian.

    
There could be an increase in mucous plug several Alscrid Mucopolysaccharide in the solid.

    
You may see the fragility of the vessels Capillary fragility.

    
When overlap to such a patient should be aware not to break the vicious tympanic Tympanic ring or long arm of incus Incus and must be recognized that the rule of Passenger Stapes Footplate can float.


ALPORT Syndrome

     
Transmitted as an autosomal dominant Autosomal dominant.

    
Sensorineural hearing deficiency with progressive nephritis Progressive Nephritis.

    
Show hematuria Hematuria and Proteinuria protein after the second decade of life.

    
Affected by chronic kidney inflammation Kabb Chronic Glomerulonephritis with Archahaa lymph cells and foam Foam Cells.

    
There was a spherical Spherophalera Cataract.

    
Lack of vestibular function Hypo-function of the Vestibular organ.

    
Dying men with this disease Bthblon blood Uremia at age thirty and less influenced by women.

    
Hearing loss begins at the age of ten years, and although it is considered not linked to sex since it affects both men and some women, there is tissue degeneration in Corti's organ with a Corty blood filamentous Stria Vascularis.


ALSTROM Syndrome

     
And autosomal recessive.

    
Degeneration in the retina Retinal Degeneration leads to a decrease in visual ability Visual Loss.

    
Diabetes Diabetes with Obesity Obesity.

    
Lack of progressive sensorineural hearing.


DUANE Syndrome

     
Autosomal dominant and in some cases related to sex.

    
Inability to retraction eyes.

    
Narrowing of the palpebral fissure.

    
Torsion neck (torticollis) Torticollis.

    
Lack of conductive hearing

    
Cervical rib Cervical rib.


FLYN-AIRD Syndrome

     
An autosomal dominant trait.

    
Palace eyes with progressive cataract Progressive Myopia Cataract.

    
And inflammation of the retina pigmentosa Retinitis Pigmentosa.

    
Lack of progressive sensorineural hearing.

    
Ataxia Ataxia.

    
Popup pain in the joints Shooting Pains.


HALLGREN Syndrome

    
And autosomal recessive.

    
Inflammation of the retina pigmentosa Retinitis Pigmentosa.

    
Ataxia Advanced Progressive Ataxia.

    
Mental retardation in 25% of cases.

    
Lack of sensory neural hearing.


HERMANN Syndrome

     
Hereditary compelling.

    
Deficiency with sensorineural hearing Alaratjah muscle during childhood or adolescence.

    
Diabetes.

    
Progressive dementia Progressive Dementia.

    
Inflammation of the pelvis and the College of Pyelonephrits with inflammation of the kebab and the College of Glomerulonephritis.


NORRIE Syndrome

     
A genetic recessive.

    
Boys blindness Congenital Blindness due to retinal tumor liar Pseudotumor Retini.

    
Lack of progressive sensorineural hearing in one third of cases.


PENDRED Syndrome

    
And autosomal recessive.

    
Lack of hearing two-sided secondary to atrophy of Corti's organ with audiogram in AU shaped audiogram: AU.

    
Be Soye thyroid patients, but they develop a good spread at maturity, and the infection is bad metabolic iodization tyrosine Iodination of Tyrosine.

    
Perchlorate is a positive test.

    
The ratio of normal intelligence.

    
Cretinism and congenital Congenital Cretinism, and is the pyramid of petrous bone well developed.

    
This syndrome is 10% of cases of hereditary deafness.


REFSUM Syndrome

    
And autosomal recessive.

    
Inflammation of the retina pigmentosa Retinitis Pigmentosa.

    
Multiple neurological injuries Polyneuropathy.

    
Ataxia Ataxia.

    
Lack of sensory neural hearing Sensoneural hearing loss.

    
Visual injury usually starts in the second decade Second Decade.

    
Fish skin is often Ichthyosis.


TAYLOR Syndrome

    
And autosomal recessive.

    
Small one ear Microtia or absence Anotia.

    
Unilateral hypoplasia of facial bones.

    
Lack of conductive hearing.


PATAU Syndrome

    
(Trisomy 13-15) group D

    
Ear low positioning.

    
Atresia Atresia of external auditory canals.

    
With cleft lip palate.

    
Coloboma Coloboma of the eyelids.

    
Small lower jaw Micrognathia.

    
Tracheoesophageal fistula Tracheosophageal Fistula.

    
Tumors of bloody ethnic Hemangiomas.

    
Congenital cardiac injury.

    
Mental retardation.

    
Lack of hearing mixed.

    
Death usually occurs in early childhood Early Childhood.


WEIL Syndrome

    
Inflammation of the kidney Nephritis.

    
Lack of hearing.

    
Transmitted as an autosomal dominant hereditary Autosomal Dominant

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