Deafness and

A (Hex A) catalyzing the biodegradation of gangliosides. Accumulation of gangliosides in lysosomes primarily affects brain neurons. In the early onset form of the disease, children become blind, deaf, and unable to swallow. Children die usually before the age of three. A late onset form ofthe disease occurs in young adults and is usually nonfatal. 

Mutation of the human Cavl.l gene is associated with hypokalemic periodic paralysis. Deletion of the Cav1.3 gene leads to viable pups that are deaf and have cardiac arrhythmia at rest. Mutation of the human Cavl. 4 gene is associated with X-linked congenital stationary night blindness. 

Complex V catalyzes the phosphorylation of ADP to ATP. Deficiencies of complex V are rare and clinical presentation is usually nonprogressive muscle weakness. One subject included growth retardation, sensorineural deafness, and involvement of basal ganglia, which are occasional features of various other respiratory chain abnormalities. 

Anti catarrhal. The mucus-reducing activity of elder may be due to its high potassium content, but in any case this plant helps to clear up bronchial mucus, hay fever and sinus congestion. Both flowers and berries help to dry up a runny nose. When the flowers are taken before hay fever season they help to lessen its severity. A gargle made of elder blossoms steeped in vinegar can be used to treat sore throats and tonsillitis. Elder has even successfully treated catarrhal deafness and children s croup. A tea made of elder flowers can ease hoarseness and make the voice clear. Parts used flowers, berries. 

Since that night I have known that it is not so hard to kill a prisoner, not even one of your own blood. Not even a king. It is not hard when you have all the keys at your belt, and at your command the watch will close their eyes, and the woman that scrubs the stone floors afterwards is deaf and mute from birth. 

Sigesbeckia glabrescens Mak., or hi chum (Korean Fig. 21), is used in Korea to treat liver and kidney diseases, asthma, allergic disorders, costiveness, deafness, and blindness. In China, the plant is prescribed for rheumatic pain, numbness, weak bones, and to wash boils. The anti-inflammatory property of Sigesbeckia glabrescens Mak. is... 

About 1 in 1,000 children are born deaf, and another 1 in 1,000 develop deafness by adolescence. Although some deafness can arise during development due to external sources, much deafness in children is due to mutations in specific genes. As many forms of deafness are nonsyn-dromic — there is no other associated abnormality — identification of the responsible genes can lead to characterization of proteins that are essential for the auditory system (and often the vestibular system, as well). 

Usher syndrome is a deafness-blindness syndrome that is caused by mutations in a number of genes [29]. Usher syndrome is divided into three classes depending on the severity of the phenotype Usher I produces profound deafness and vestibular dysfunction, as well as adolescent blindness in Usher II, deafness is less profound. User III is less common in these patients, hearing and vision loss develop progressively, and vestibular dysfunction is variable. 

National Institute on Deafness and Other Communication Disorders (NIDCD) fact sheets and guidelines at httpvywww.nidcd.nih.gov/health/... 

Eibl-Eibesfeldt, I. (1973). The expressive behavior of the deaf-and-blind-bom. In M. von Cranach, 1. Vine (Eds.), Social Communication and Movement (pp. 163-194). New York Academic Press. 

Most cases of mercury poisoning led to handicap, chronic disease, or death. The most frequent symptoms include numbness of limbs, lips and tongue, speech abnormalities, limb function disorders, visual acuity disorders, deafness, and muscular atrophy. Insomnia, hyperactivity, and coma have also been reported. Methylmercury penetrates the blood-brain barrier and causes central nervous system injuries. Mercury also has a teratogenic effect, leading to congenital abnormalities or congenital Minamata disease. 

Brain The usual signs and symptoms include ataxia, dementia, deafness and sometimes seizures. Not surprisingly, there is failure of mental development in children. 

Ofofox/c/fy. Tinnitus, reversible and irreversible hearing impairment, deafness, and vertigo with a sense of fullness in the ears have been reported. Deafness is usually reversible and of short duration (1 to 24 hours) however, irreversible hearing impairment has occurred. Usually, ototoxicity is associated with rapid injection, with severe renal impairment, with doses several times the usual dose, and with concurrent use with other ototoxic drugs. 

Nephrotoxicity/Ototoxicity Because of reported cases of deafness and potential nephrotoxic effects, closely observe patients. Refer to the Warning Box in the Aminoglycosides, Parenteral monograph concerning aminoglycoside toxicity. Pregnancy. 

Chiidren with Krabbe disease exhibit irritabiiity, fever, seizures, iimb stiffness, deiayed mentai or motor deveiopment, vomiting, feeding difficuities, hypertonia, spasticity, deafness, and biindness. 

MERRF (myoclonic epilepsy with ragged red fibers) Is characterized by weakness on exertion, ataxia, and associated deafness and Is due to mutation of the mitochondrial tRNA gene. 

A 5-year-old boy complains of being tired all the time. His mother insists that he eats well but that he sleeps 11-14 hours per day and can t keep up with his friends on the ball fields. Physical examination reveals that he is at the 30th percentile for height and weight, with mild bilateral deafness and ataxic gait. After interviewing his mother, it becomes apparent that several other members of this family were deaf by their mid-twenties and a muscle problem was diagnosed in an uncle. 

Heynen, J. M., Blockmans, D., Verwilghen, L. R., et al, Congenital macrothrombocytopenia, leukocyte inclusions, deafness and proteinuria Functional and electronmicroscopic observations on platelets and megakaryocytes. Br. J. Haematol. 70, 441—448 (1998). 

Hypoprofhrombinemia, fhrombocytopenic purpura, hemoglobinuria, asfhma, agranulocyf osis, hypoglycemia, deafness, and opfic afrophy occur rarely... 

Electrical Stimulation Devices. Bioelectrodes that transmit electrical signals into the body are generally known as electrical stimulation devices, examples of which include cardiac pacemakers, transcutaneous electronic nerve stimulators (TENs) for pain suppression, and neural prostheses such as auditory stimulation systems for the deaf and phrenic nerve stimulators for artificial respiratory control. In these, and other similar devices, electrodes transmit current to appropriate areas of the body for direct control of, or indirect influence over, target cells. 

Capreomycin is nephrotoxic and ototoxic. Tinnitus, deafness, and vestibular disturbances occur. The injection causes significant local pain, and sterile abscesses may occur. 

Elder is known for its diaphoretic and anticatarrhal properties. Traditionally, it is used for influenza, colds, chronic nasal catarrh with deafness, and sinusitis. Elder is also believed to act as a diuretic, laxative, and local anti-inflammatory agent. 

Golden seal is stated to be a stimulant to involuntary muscle, and possesses stomachic, oxytocic, antihemorrhagic, and laxative properties. Traditionally, it has been used for digestive disorders, gastritis, peptic ulceration, colitis, anorexia, upper respiratory catarrh, menorrhagia, postpartum hemorrhage, dysmenorrhea, topically for eczema, pruritus, otorrhea, catarrhal deafness and tinnitus, conjuctivitis, and specifically for atonic dyspepsia with hepatic symptoms. 

Capreomycin is nephrotoxic and ototoxic. Tinnitus, deafness, and vestibular disturbances occur. 

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