Thursday 20 February 2020

SYNDROME THAT AFFECT HEARING


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USHER SYNDROME (VESTIBULOCERBELLAR ATAXIA)
It is a rare genetic condition caused by a mutation in any one of at least 11 genes resulting in a combination of visual impairment and hearing loss. Usher syndrome is characterized by partial or total loss of hearing loss and vision.
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Causes:
·         Autosomal ressive Type 1 – CDH23, MY07A, PCDH15, USH1C, USH1G
Type II – USH2A, GPR98
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Characteristics:
·         Type I – Retinitis Pigmentosa
·         Vestibular dysfunction
·         Profound hearing loss
·         Type II – Retinitis pigmentosa
·         Sloping hearing loss (Variable degree)
·         Type III – Retinitis Pigmentosa
·         Vestibular dysfunction
·         Initially normal hearing or mild loss
·         Then, hearing loss progresses
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Hearing Loss
·         Sensory neural (Congenital in Type I & II)
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SYNDROME THAT AFFECT HEARING


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DOWN SYNDROME
Down syndrome also known as Trisomy 21, Having extra 21 chromosome. It is genetic disorder. It is most commonly recognized disorder found in children.
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Behaviour of Down Syndrome
·         Depression
·         Social withdrawal
·         Diminished interest copying skills
·         Loss of cognitive and social skills
·         Trouble in communication
·         Short attention span
·         Slow learning
·         Delayed speech and language
·         Impulsive
·         Disruptive
·         Obsessive compulsive behaviour
·         Chronic sleep difficulty
·         Dementia
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Characteristics:
·         Decreased Muscle Tone
·         Excess skin in the nope of neck
·         Upward slanting eyes
·         Wide, short hands with short fingers
·         Single crease in the palm of the hand
·         White sports on the coloured. Part of the eyes
·         Small mouth, small ears
·         Separate joints between the bones of the skull
·         Intellectual developmental disorder
·         Open mouth with protruding tongue
·         Outer/middle/inner ear hypoplasis have been reported
·         Frequent upper respiratory infections
·         Recurrent otitis media
·         Hearing loss
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Causes:
·         Improper separation of chromosome pairs. Causing an extra copy of chromosome.
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Hearing Loss
·         Conductive, Mixed hearing loss
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SYNDROME THAT AFFECT HEARING


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KLIPPEL – FEIL SYNDROME
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Klippel syndrome is a congenital disorder characterized by abnormal fusion of two or more bones of spinal column (vertebrare) within the neck.
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Causes:
·         Autosomal dominant GDf3, GDF6
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Characteristics:
·         Fused cervical vertebrae
·         Low occipital hairline
·         Short neck
·         Decreased head mobility
·         Ossicle abnormalities
·         Hearing loss
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Hearing Loss
·         Sensory Neural, Conductive Hearing Loss (Congenital)
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SYNDROME THAT AFFECT HEARING

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SYNDROME THAT AFFECT HEARING
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ALPERT SYNDROME
Alpert syndrome is a congenital disorder characterized by malformation of the Hands, feets and skull & face. Babies with Alpert syndrome are both with a distorted shape of head and face. Children with Alpert syndrome may here intellectual disability.
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Causes:
·      Alpert syndrome is a genetic condition caused by mutation on a gene. That affect gene is autosomal dominant: FGFR2.
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Characteristics:
·        Craniofacial anomalies affecting the ears, stapes fixation, fused fingers and toes and spina bifida.
·        Associated with conductive hearing loss.
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Hearing Loss:
·        Conductive Hearing Loss (Congenital)
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Friday 14 February 2020

Hearing aid: WHAT ARE THE SYMPTOMS OF HEARING LOSS?

Hearing aid: WHAT ARE THE SYMPTOMS OF HEARING LOSS?: WHAT ARE THE SYMPTOMS OF HEARING LOSS? ·          Difficulty understanding words, specially in background noise or crowdie area. ·   ...

WHAT ARE THE SYMPTOMS OF HEARING LOSS?


WHAT ARE THE SYMPTOMS OF HEARING LOSS?
·         Difficulty understanding words, specially in background noise or crowdie area.
·         Ask people to repeat themselves.
·         Finding it hard to keep with a conversation.
·         Difficulty hearing other and misunderstanding what they say.
·         Ringing in ear (Tinnitus).
·         Listening to music, radio or watching TV with higher volume.
·         Difficulty hearing people on phone.
·         Headache.
·         You think that other people are mumbling or out speaking clearly.
·         Finding speech unclear.
·         Hearing is worse when sound comes from one side.
·         Fail to hear someone talking from behind.
·         Dread going to noisy parties and restaurants?
·         Turn off the volume on the TV or car radio?
·         Have trouble hearing in groups.
Symptoms of Hearing Loss in Children
·         Has difficulty understanding what people are saying.
·         Limited or poor or no speech.
·         Fail to respond his/her name.
·         Speaks differently than other children of his age.
·         Fail to respond conversation or answer appropriately.
·         Difficulty in learning.
·         Show sign of behavioural problems or social difficulties.
·         Do not seek out or detect where sound is coming from.
·         Respond in appropriately to questions.
·         Say what, several time.
·         Has speech or language delay or problem in articulation.
·         Do not follow simple commands or understand simple directions.
·         Do not react to voices.
·         Still babbles what not moving to more understandable speech.
What are the common causes of hearing loss?
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·         Excessive exposure of loud noise
·         Stroke
·         Ototoxic drugs like Asprin
·         Heridity
·         Infection of ear canal or middle ear
·         Hypertension
·         Perforation of Scarring of ear drum
·         Smoking
·         Excessive wax is the most common cause of the hearing loss
·         Hole in ear drum or traumatic brain injury to the middle ear also cause hearing loss
·         Ageing (Age related loss is called presvyacusis)
·         Diabetes is known for sensori-neural hearing loss
What are the prevention of hearing loss?
·         Protect your hearing during loud event like gigs or sports event and night clubs.
·         Families with a history of hearing loss should be monitoring their babies hearing.
·         Noise exposure is also an issue for babies and children. Avoid loud noises, noise is probably loud enough to damage your hearing if you can’t hear what people nearby are saying.
·         Health hearing, life style begin even begins before conception and pregnant mother should avoid alcohol and other drugs.
·         Avoid medication that may be harmful to your hearing. Be aware of drugs that causes hearing loss like Asprin etc.
·         Don’t listen music at 60% of the maximum volume. If you are using ear phones, limit them to an hour.
·         Smoking is a risk factor for hearing loss because tobacco, cigarettes contain nicotine that damage the hear cells in the ear.
·         If you are working/exposed to loud noise through your working hours, take a precautions or seek to your department manager to provide hearing precautions such as ear plugs or ear muffs.
·         Children are also exposed to loud noise like noisy toys or a noisy environment.
·         Get a hearing test as soon as possible if you worried you might be losing your hearing.

Wednesday 12 February 2020

Hearing aid: EARLY HEARING DETECTION AND INTERVENTION

Hearing aid: EARLY HEARING DETECTION AND INTERVENTION: EARLY HEARING DETECTION AND INTERVENTION SCREENING Screening programme are used to identify those individuals who have a particul...

EARLY HEARING DETECTION AND INTERVENTION


EARLY HEARING DETECTION AND INTERVENTION
SCREENING
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Screening programme are used to identify those individuals who have a particular disorder or group of disorder. When a person is affected by hearing impairment or hearing disorders. These are addressed by audiological screening programme. The term identification audiometery is used to refer screening or hearing impairment.
New Born Hearing Screening
Early hearing detection and intervention refer to the systematic identification of babies with hearing loss. A variety of approaches and techniques for identifying hearing loss in infants have been used over the years. In the broadest term these includes public awareness campaigns, high risk registers, screening programmes employing behavioural or psychological tests, often combined with the use of risk indicators.
Public Awareness Campaigns
The public awareness approach uses such methods as mass media campaigns, brouchers and checklist to educate the public about the danger and sign of hearing loss in children. Public awareness campaigns are valuable adjacent to hearing loss identification in general they are not effective themselves because there is great uncertainty who about actually receives and responds to these efforts and concern that many primary care health provider might tends to discount parental suspicions.
High risk registers
Another approach that has been used in early identification of hearing impaired babies involves determining whether the child meets any of several criteria associated with hearing loss. Infants identified with high risk register are then referred for evaluation. Risk indicators for congenital progressive and delayed onset hearing loss based on the JCIH (2007) position statement.
Screening Approaches
1. Behavioural screening
Behavioural screening involves presenting a stimulus to the child and watching for change in behaviour appropriate for her stage of development with new borns, this involves intense stimuli (typically 60dB SPL or more) evoke response such as startle response, an auropalpebral reflex or arosal from sleep.
Behavioural observations approaches are no longer use in neonatal screening because of several severe limitation that involves subjective judgement that are highly susceptible, false positive and false negative results and the use of high level stimuli presented from loud speakers, so that mild and moderate cannot be identified.
Psychological Screening
Psychological measures have become standard testing in neonatal hearing screening, the principle techniques are the Brain stem response (ABR), ASSR (Auditory Steady State Response), OAE (Otoacoustic Emission). These methods require more costly instrumentation than behavioural approaches.
The ABR is well established as a neonatal screening test and has impressive rates of sensitivity, specificity and reliability.
The EOAE shares excellent sensitivity with the ABR for detecting hearing loss infants.
The ABR can be used to screen for auditory neuropathy spectrum disorders where as the pre neural nature of OAE’s makes them inappropriate for this purpose.
Universal new born screening become more simpler, quicker and more efficient automated ABR. L
Principle of Effective Public Help Screening Programs
·         The condition to be detected by screening should be an important health problem. Congenital hearing loss is the most frequent, birth detect, affecting about three new borns per thousand.
·         Permanent hearing loss affects approx 1% of young children in industrialised nation and the incidence is probably higher.
·         There should be an accepted treatment for identified cases.
·         Facilities for diagnoses and treatment should be available.
·         There should be suitable screening test. Psychological screening test for hearing are sensitive, specific and relatively inexpensive.
·         Hearing screening test can be completed in less than 15 minutes per child, are painless and have no negative side effects.
·         The neonatal history of the condition should be understood because permanent hearing loss occur relatively frequent and consequences are so obvious.
·         Hearing screening and diagnosis are relatively inexpensive and there is now good evidence that the cost of identification and treatment is small.
·         Case finding should be continuing process is not a “once and for all” project.
(ASHA) Infant Guidelines
·         The screening protocol involves determining whether the child can be hear pure tone of 1k, 2k, and 4k Hz. Presented at a screening level of 20 dB HL.
·         Each ear is tested separately by air conduction.
·         Children who fail the rescreening should be referred for Audiological evaluation.
·         Screening are recommended when special circumstances or risk factor exist.
·         Hearing screening should took place are when a child initiates special educations, repeats  a grade, was absent for the last regularly scheduled screening.
The Risk factor indicating the needs foe Hearing Screening includes:-
·         Concern about the child’s hearing , speech and language or learning.
·         History of delayed or late onset of hearing loss in family .
·         Sign of syndrome that include hearing loss.
·         Exposure to ototoxic drugs.
·         Recurrent otitis media with effusion.
·         Sign of syndrome include hearing loss.
·         Exposure of potentially harmful noise level.
·         The children be referred for medical examination if any following condition are identified.
·         Drainage from the ear (otorrhea).
·         Ear canal anomalies of the ear that were not previously identified.
·         Ear structural anamalies.
·         Ear drum abnormalities or perforation.
National Goals for EHDI Programs
·         All new born will be screened for hearing loss before one month of age.
·         All infants who screen positive will have a diagnostic audiologic evaluation before 3 months of age.
·         All infants identified with hearing loss will receive appropriate early intervention services before 6 months of age.
·         All infants and children with late onset or progressive hearing loss will be identified at the earliest possible time.
·         All infants with hearing loss will have medical home as defined by American academy of paediatrics.
·         Every state will have an EHDI tracking and surveillance system that minimize loss to follow up.
·         Every state will have a system that monitor and evaluate the progress towards the EHDI goal and objectives.
Why early identification of hearing loss is important?
·         Early intervention of hearing loss can affect a child’s ability to develop speech, language and social skills.
·         Early identification help young children with hearing loss to learn language, skills and other communication skills.
·         Early intervention improves outcome for children development of cognitive, emotional and academic defects.
·         Early identification is important for better expressive vocabulary for better receptive and expressive language.
·         More improvement in speech/articulation skill who is having hearing loss.