EARLY DETECTION and INTERVENTION IN CHILDREN DEAFNESS

EARLY DETECTION and INTERVENTION IN CHILDREN deafness
By:
Tutut SRIWILUDJENG T.
Dr. Wahidin Sudiro Husodo Mojokerto

INTRODUCTIONHealth Sense of hearing is an important requirement for improving the quality of human resources because most of the information is absorbed mulalui hearing process is good for the child affect hearing function in speech and language development, socialization and cognitive developments
 Hearing loss is different from other defects where the babies / children who have a hearing loss is often not detected or is not immediately known whether the parents themselves, this disorder also known as "the Invisible disability." The initial symptoms is difficult to know because deafness is not visible. Usually new parents aware of hearing loss in children when there is no response to loud noise or not / too late to talk. Therefore, information from parents is very helpful to know the child's response to the sound environment of the home, the ability of vocalizations and how to pronounce words.Introduction (detection) the existence of early deafness and then treated (intervention) that early would increase the child's ability to speak and spoke. Since the first years of life (0-3 years), is the development of language and speech are the most critical. Research shows that at times the quality of auditory stimulation affects the changes of anatomical, physiological and behavior caused by the development of the auditory system.The delay in diagnosis will also mean there is a delay to start the intervention and will bring serious impact on subsequent developments in the future which will add to burden the family, society and State.Therefore, early detection and intervention is very important to note and should be done immediately.One of the efforts to find cases of infants with hearing loss is to do a hearing in early infancy, mainly contained suspicion of hearing loss and also in groups of infants with high risk.Incidence of hearing loss cases moderate to severe average was detected in 20-24 months. In the case of a mild hearing loss was found the average age of 48 months. Even in cases of unilateral hearing loss which can only be identified at school age.Many researchers argue that early intervention can give better results in speech and language abilities. Handling cases of hearing loss is best done earlier under the age of 6 months.The incidence of hearing loss ranged from 1 to 3 incidents per 1000 live births. That number could increase 10 to 50-fold when it's done a survey on high-risk group.
UNDERSTANDING THE INTERRUPTION OF HEARING and deafnessAccording to the WHO definition of hearing loss and deafness is distinguished beasarkan following provisions.HEARING DISORDERS (hearing impaired): reduced ability to hear well in part or in whole, in one or both ears, either mild or more severe with the average hearing threshold over 26 dB at frequencies 500, 1000, 2000 or 4000 Hz.Deafness (deaf): loss of hearing ability in one or both sides of the ear, is a very severe hearing loss with hearing threshold average of more than 81 dB at frequencies 500, 1000, 2000 or 4000 Hz.WHO also divides the degree of hearing loss into 4 groups as in the following table:Table 1: The distribution of degree of hearing loss





According to WHO hearing loss and deafness problems need to be addressed more seriously considering the amount of losses incurred. Negative impacts such as:1. Interference or obstacle the development of speech, language and cognition in children, especially when it occurs at birth or in infants.2. Difficulty following the lessons in school so that results in lower academic achievement.3. Difficulty obtaining employment or interfere in the work assignment.4. Isolated from social life5. Adverse effects both socially and economically within the environmental community and the State.Classification method that more hearing loss is distinguished from the onset, the genetics, disease progression.In classifying hearing loss need to be considered:1. Type of interruption: type koduktif, sensorineural, mixed.2. Time progress: settled, temporarily, become heavy.3. The degree of hearing loss: rigan, medium, heavy, very heavy.4. The onset of hearing loss: congenital, prelingual period, or postlingual, elderly (presbiakusis)5. Factor: ototoksis, due to noise (GPAB).Auditory function in neonates with a child or adult. The physiology of hearing process is divided into 3 sequence of events, namely the transfer of physical energy in the form of sound keorgan auditory stimulus, conversion or transduction is the conversion of physical energy stimulation to the organ recipient and the delivery of nerve impulses to the auditory cortex.
DEAF congenitalCongenital deafness is deafness which occurs in an infant due to the factors that affect pregnancy or at birth.Deafness can be partially deaf (hearing impaired) or total deafness (deaf). Sebagia Deaf is a state of auditory function is reduced but still can be used to communicate with or without the aid of hearing devices, while the total deafness is a condition that such a disruption of auditory function that can not communicate even if menapat pavement noise (amplification).Deaf kengenital us for a genetic hereditary (adafaktor descent) and non-genetic.Types of deafness that occurs is usually a nerve deafness (sensorineural) degrees of heavy to very heavy on both ears (bilateral)Given congenital deafness have a large enough impact so early on should have no socialization / information to the public and health workers who work in the field for early detection.1. Genesis ScoreThe number of infants suffering from congenital deafness is 1 child in every 1000 live births, while infant being treated in intensive Rungan (Natonal Intensive Care Unit) number is 1 in 50 babies. Then at the age of 2 children under five are growing again in every 1,000 children. Crude birth rate (CBR) in East Java, 22.0 to 24.9 per 1000 population. If the population of East Java 37 million, then the birth rate of approximately 750,000, meaning there are approximately 750 babies are born deaf every year.Prevalence of congenital deafness in Indonesia in the estimate of 0.1%. This will increase each year by 4710 people saw the birth rate of 2.2% in the population of 214.1 million people. It will continue to grow given the risk factors that lead to congenital deafness during pregnancy and childbirth remain high.In developed countries congenital deafness rates ranged from 0.1 to 0.3% of live births .. while in Indonesia based on a survey conducted Dep. Kes in 7 Provinces in the year 1994 to 1996 that is equal to 0.1%.Congenital deafness in Indonesia is estimated as many as 214,100 people when a population of 214,100,000 million (Profile Kesetahan 2005). This amount will increase each year by the existence of population increase due to the high birth rate of 0.22%. This is of course an impact on the provision of educational facilities and employment in the future.WHO estimates that every year there are 38,000 deaf children born in Southeast Asia.WHO meeting in Colombo in 2000 mentapkan congenital deafness as one of the causes of deafness that should lowered prevalence. This of course requires cooperation with other disciplines and society in addition to health tenga.2. CauseCongenital deafness can occur in:Pregnancy (PARENTAL)First trimester pregnancy is an important period for bacterial or viral infection would have caused the deafness. Frequent infections affect hearing include TORCHS infection (Toxoplasmosis, Rubella, Cytomegalovirus, Herpes and Syphilis), in addition to measles and parotitis (mumps).Several types of drugs and teratogenic ototoksik like salicylate, quinine, gentamicin, streptomycin, etc. have pontensi cause disruption tyerjadinya organ formation process and the hair cells in the cochlea (the cochlea). Anatomical structure of ear disorders can also cause the occurrence of deafness among other cochlear aplasia (the cochlea is not formed) and ear canal atresia.At birth (perinatal)The cause of deafness at birth include: premature birth, low birth weight (<1500 g), actions with a tool in the process of birth (vacuum extraction, forceps), hyperbilirubinemia (baby yellow), asphyxia (birth did not burst into tears), and hypoxia brain (the value of Apgar <5 at 5 minutes).According to the Academy of American Joint Committee on Infant Hearing Statement (1994) in infants aged 0-28 days when it was found the following factors should be suspected as a possible cause of hearing loss.1. Family history of deaf since birth2. Prenatal infection; TORSCH3. Abnormalities in head and neck anatomy4. Syndrome associated with congenital deafness5. Low birth weight (LBW <1500 g)6. Bacterial meningitis7. Hiperbillirubinemia (baby yellow) that requires exchange transfusion8. Asfisia weight9. Ototoksik drug delivery10. Using the tool of respiratory / mechanical ventilation for more than 5 days (ICU)
Genetic Hearing LossMost cases of genetic hearing loss is Nonsindromik. Have been identified over 110 loci of chromosome and 65 genes (accessible at http://webhost.ua.ac.be/hhh).More than 300 kinds of syndromes associated with hearing loss, among others, which often Pendred's syndrome, Usher's and Teacher Collins, who is autosomal recessive, Waardenburg's (AD or AR), Mondini malformation, vestibularaqueduct enlargement. Case studies have as many as 20.8% of 810 children with SNHL (Sensory Neural Hearing Loss). Symptoms usually appear after birth and on average about 5.8 years of age referred.The best way to detect cases of prelingual hearing loss that did not appear as sub clinical lahiratau by performing molecular genetic testing of blood samples in infants. Type of inspection that is now available is the test GJB2 deafness and kondria A1555G mutation.The discovery of cases in infants with suspected hearing loss have a genetic cause has been made possible in the present through DNA sequencing techniques. Diperkirkan such an inspection would be a "trend" or stadart future. This progress is an important step in contributing to the handling of cases of deafness, from birth to the better. Infants with congenital deafness who "Late-Onset Hearing Loss Prelingual" depat immediately be identified from birth, either by genetic or environmental causes.
FUNCTION OF HEARING AND SPEAKING SKILLSIn accordance with the child's age, development uditorik as follows: Age 0-4 months: auditory response capabilities are still limited and reflexes. Can be expressed babies keget heard a loud noise while sleeping or awake. Auropalpebral a reflex response or reflex Moro. Age 4-7 months: the response turned his head toward a sound that lies horizontally in the field, although not yet consistent. At the age of 7 months of the neck muscles are strong enough so that the head can be rotated quickly towards the sound source. Age 7-9 months: to identify the precise origin of the sound source and the baby can turn his head firmly and quickly. Age 9-13 months: babies already have a huge desire to seek the source of the sound from all directions quickly. Age 2 years: pemerika should be more careful because children will not respond after several times received the same stimulus. This is because children are able pemperkirakn sound source.Speech development close relation to the stage of development hearing on abayi, so the presence of hearing loss need to be suspected when:Age 12 months: not to rant (babbling) or imitate soundsAge 18 months: not to mention a word that has meaningAge 24 months: vocabulary of less than 10 wordsAge 30 months: can not string 2 wordsTo analyze the early diagnosis of hearing loss is necessary. An easy way to check if there is no means of hearing is to give the sounds at a distance of 1 m behind the child:1. Sound PSS - PSS to describe high-frequency sound2. Beep uh - uh to describe low-frequency3. Sound swipe with a spoon on the edge of the cup (frequency 4 KHz)4. Knocking sound base cup with a spoon (frequency 900 KHz)5. Sound squeezed paper (frequency 6000 KHz)6. Bell sound (frequency 2000 KHz)HEARING EXAMINATIONEarly detection pendengarn disorders can be done by examination of Subjective and Objective. Subjective examination among others, by using acoustic stimuli (noises) that have a certain intensity and value of the response, namely with auropalpebral reflexes. Objective examination conducted by means of electrophysiologic tests of brainstem Auditory Evoked Reponses (ABR) and Otoacoustic Emission (OAE) and tympanometry examination.The important thing to consider before doing this examination is the ear canal should be clean and no abnormalities in the middle ear.Examination important to do is BOA (Behavioral Observation Audiometry), namely by looking at the child's behavior toward a given sound stimulus. Factors that affect the inspection include age, mental condition, will do the test, fear, neurologic conditions associated with motor development and perception.The use of BOA and VRA (Visual Reinforcement Audiometry) in infants and children have limited hearing to determine the valid threshold.












Figure 1. Hearing test with the BOA and the response given by infants and neonates.Quoted from: Nothem 1994
Screening for hearing loss in neonates is a process to test a simple and brief, to identify populations with a high probability of occurrence of hearing loss. There is a problem serious enough impact and there is a high prevalence in the community. There is strong evidence that screening a greater benefit than if the case of late caught early and immediate intervention than if the cases are found too late and only realized later. Early treatment should show greater benefits and to change the prognosis of the disease. screening methods have been evaluated, it is stated precisely and can be used in the intended target. Quite sensitive and specific method, the procedure can be applied at 100% of the target population, easily tolerated and free from risk, it can be widely affordable, low cost, easy target, rapid examination of large populations. The availability of diagnostic facilities and the facilities are adequate for the implementation of intervention programs with the aim of correcting the condition is detected during screening.For detection and early intervention programs should be applicable to all infants (target population) not only at high risk.Research has been done if screening is applied only in high-risk groups will only detect 50% of the number of infants with hearing loss. No doubt that early detection and intervention is important for all infants (New Bomb Universal Hearing Screening). Hearing Screening Recommendation dilakuakn since the first months of birth, then in follow-up and the diagnosis is established until the age of 3 months and subsequently included in the intervention program within 6 months of age.With today's technological advances allow for the detection of hearing loss since birth with the result that can be trusted. Currently, OAE (Otoacoustic Emission) and AABR (Automated Auditory brainstem Evoked Response) is the gold standard examination technique (gold standard) with the principle of checks fast, easy, not invasive with a sensitivity approaching 100%. Constraints is that these facilities are not owned by all provincial hospitals.Brainstem Auditory Evoked Response (ABR) recorded Araf auditory responses in areas along the auditory path to acoustic stimuli.ABR examination is an electrophysiologic measurement that assesses the functions and areas along the auditory nerve to the brain stem auditory path. Waves generated on the ABR recording there are 7 waves, as follows:Wave I: eighth nerveWave II: cochlear nucleus (CN)Wave III: superior olivary complex (SOC)Wave IV: The nucleus of the lateral lemnicus (LL)Wave V: inferior colliculus (IC)Wave VI: medial geniculate (MG)Wave VII: auditory cortex (AC)
ABR examination tool based on the clinical use were classified into 2 types namely:a) Automated brainstem Auditory Evoked Response (AABR)The use of AABR is usually done by placing surface electrodes on the upper forehead, mastoid and behind the neck. Stimulus clicks (usually set at 35dBHL) sent into the ear bayimelalui ear phone. Most systems compare AABR waveforms with the mold that formed the sound of a baby ABR normative data. Based on this comparison, it is determined whether the baby responds pass or fail. Is the system commercially available, can be used as an effective screening tool for infants younger than 6 months. AABR give the results of pass / fail without the need for interpretation, but it is not providing enough information on specific frequency.b) Manual brainstem Auditory Evoked ResponseManual ABR is generally known as the Automated Auditory Evoked brainstem response (ABR) course, unlike that caused AABR response to a click which is set at 35 dBHL, ABR stimulus intensity was varied to determine the low level needed to evoke a clear response. The result other than to determine the severity of hearing loss also can determine whether hearing loss is sensorineural or conductive. When using a click to generate ABR, auditory sensitivity is limited information about the limits 1000-4000 Hz. If necessary, an ABR can use the extra low frequency tone (750 Hz) to determine the configuration of hearing loss.By assessing the shape of waves and the time it takes from when the stimulus given to the resulting wave, can provide the clinical significance of the situation in the path of the auditory nerve or the surrounding area that affect the auditory nerve.





Figure 2. Image of ABR waves
Normal ABR recordings must exist a wave I, III and V with a clear peak in both ears. Wave IV is generally one with wave V.Limitations to detect hearing loss at birth is partly due to the occurrence or manifestation of the new hearing loss occurs at age greater, so the "early-onset hearing loss" is not Visible. In this regard the Joint Committee on Hearing has identified 10 risk as an indicator for monitoring the status of pendengaranya, although in a previous examination otherwise normal.For that JCIH determine risk factors for high risk groups with age limit of 29 days served until 2 years.Idikator need for monitoring the risk for progressive-delayed Sensorineural Hearing Loss in infants aged 29 days to 2 years are:1. Attention parents of suspicion of hearing loss, impaired speech, language and developmental delay2. Family history of childhood deafness3. Stigmata or syndrome is known to have a relationship with sensorineural deaf or hard of hearing conduction4. Postnatal infection such as meningitis5. Eg intra-uterine infection and syphilis TORCH6. Indicators such as neonatal hyperbilirubinemia. PPH (presisten pulmonary hypertension), mechanical ventilation disorders.7. Syndrome associated with progressive deafness such as Neurofibromatosis, Osteopetrosis, Usher's Syndrome.8. Neurodegenerative disorders such as Hunter syndrome or neuropathy disorders sensorimotorik or Friedrich's ataxia and Charcot-Marie Tooth syndrome.9. Head trauma.10. Recurrent otitis media or presisten for at least 3 months.
HEARING DISORDERS PREVENTION STRATEGY and PGP RENSTRANAS Sound Hearing deafness for the purpose of 2030 (MOH)WHO - SEARO (South Asia Regional Office) in 2005 has formed an organization: SOUND HEARING 2030 to coordinate the activities of prevention of hearing loss in the WHO - SEARO. Sound Hearing 2030 (SH 2030) has a mission to reduce preventable hearing loss in 2030 through the development of a sustainable health care system.Additionally Sound Hearing 2030 (SH 2030) also aims to reduce hearing loss can be prevented by 50% in 2015 and 90% in 2030. By carrying out activities SH 2030 Indonesia is expected that each resident has the right to have a degree of ear health and hearing 2030.untuk optimal in realizing the above, the sense of hearing health program management efforts directed at combating of hearing loss and deafness. In order to achieve the same perception and increase the ability of managers and implementers (cross-program and cross-sector) required continuous training activities.Until now WHO has not had a specific strategy that is global in the prevention of hearing loss. But at the Bangkok meeting on 4-5 October 2005 WHO - SEARO has formed an organization: SOUND HEARING 2030 to coordinate prevention activities in the area of ​​auditory ganggun WHO - SEARO.At the inaugural meeting SOUND HEARING 2030 (SH 2030) on October 4 to 5 in Bangkok has set the vision, mission and objectives SH 2030. Dr Bulantrisna Djelantik (Indonesia) at the meeting were set as chairman.Vision Sound Hearing 2030Improve health and welfare of Southeast Asia thanks to a better hearing.Mission Sound Hearing 2030Eliminating preventable hearing loss in 2030 through the development of a sustainable health care system.The purpose of Sound Hearing 2030Reduce hearing loss can be prevented by 50% in 2015 and 90% in 2030.
2030 SH conduct expected for each of Indonesia's population has the right to have a degree of ear health and hearing the optima in 2030.To realize the above, the sense of hearing health program management efforts directed at prevention of hearing loss and deafness.Difficulties and obstacles in developing countries is still limited health infrastructure ear and hearing in prevention, early detection, treatment and habilitation / rehabilitation.In 2002 the Central Government, has developed the Guidelines for National Strategy Plan for Hearing Loss and Deafness (Deafness Renstranas PGP). The guidelines contain six Deafness Prevention strategies need to be done in achieving reductions in deafness. Strategy 4 (four), which exists in PGP Renstranas Deafness is to strengthen management and infrastructure services and strategy 5 (five) to increase the quality and quantity of human resources (HR) in the context of prevention of hearing loss and deafness.Results meeting WHO SEARO Intercountry Consultation Meeting (Colombo 2002) in Sri Lanka recommends that priority issues that must ditanggualangi (including Indonesia) are: Deafness due to middle ear infection (otitis media suppurative Khronikal Omsk), congenital Deaf / Congenital, due to exposure to noise Deaf (Noise Induced Hearing Loss / NIHL) and Deafness of old age (presbycusis) and ganggauan another hearing in order to realize the objectives of Sound Hearing 2030. Better Hearing for All (each of Indonesia's population has the right to have a degree of ear health and hearing the optimum in 2030).PDH program aimed at implementing prevention of hearing loss and deafness through the steps of:1. Determining the magnitude of the problem (magnitude) and the main cause of hearing loss and deafness in certain populations.2. Prevent interference Ototoksik drugs freely.3. Lower prevalence of disorders due to exposure to noise pendegaran on high-risk groups.4. Develop basic ear health (basic ear care) as part of Basic Health Services Program in Public Health Centres.5. Improving early detection and treatment of hearing loss.6. Develop appropriate technology (Appropriate Technology) for examination and treatment of hearing loss.7. Entered into technical cooperation with the Government to Develop National Programme in the field of ear and hearing health.8. Having a system of management and administration for a global program.
Lai statement from WHO (Geneva, 2000) also states that 50% of hearing loss can be prevented (preventable Deafness) through the activities of Primary Health Centre (HEALTH). At the meeting the WHO also recommends that each state reduce preventable Deafness by 50% in 2010 (Better Hearng 2010).For this purpose the Ministry of Health of Indonesia has developed the National Strategy Plan for Hearing Loss and Deafness to achieve the goals Sound Hearing 2030, which will guide the activities of prevention of hearing loss and deafness in both the Central and Regional levelVarious health effort ear / hearing loss prevention (UKT / PGP) has been initiated since 1989 with the establishment of Hearing and Speech Center in the centers of Indonesia as the first step to national health program in Indonesia's ear.Since 2002 in Surabaya also has started to follow the program by opening the Center for Hearing and Communication (Hearing and Communication Center) is housed in poly Audiology Dr. Soetomo. It is expected to participate to support the efforts in implementing the detection and early intervention programs and to achieve the goals Sound Hearing 2030.
Visit patients in Dr profound SNHL. SOETOMO











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