.HEARING LOSS Incidence Over all: 1-3 per 1000 live births In NICU: 2-4 per 100 NICU Graduates.

.infection .wardenberg syndrome . Genetic A) mutation in connexin 26 B) As part of syndromes . oto toxic medication. Non genetic . . hypoxia.usher syndrome 2.alport syndrome .pendred syndrome . metabolic disease. hyperbilirubinemia.Etiology 1.secondary to injury to developing auditory system . ischemia.

 Hyperbilirubinaemia at the level of exchange transfusion.  Low birth weight.  Craniofacial anomalies.  TORCH infections.  Bacterial Meningitis.  Ototoxic medications.  Mechanical ventilation for more than 5 days. .  Syndromes known to include conductive or sensorineural hearing loss.THE HIGH RISK REGISTER  Family history of hereditary childhood sensorineural hearing loss.

. OAE (Oto acoustic emissions).SCREENING TESTS 1. AABR (automated auditory brainstem response ) 2.

.BERA Potentials recorded form ear and vertex in response to brief auditory stimulation to assess the conduction through auditory pathway up to the midbrain.

Waveform compononents. lateral leminiscus inferior coliculi Auditory brainstem response evaluation (sensitivity 90% Specificity 70-90%) . waves generators wave I wave II wave III wave IV wave V ------ viii nerve cochlear nucleus superior olivary n.

but absence of any obligate wave is abnormal. can absent in some normal subjects.4.7.6.Normal BERA  classic baep consists of 5-8 vertex positive peaks  initial 5 are of clinical interest  obligate BAEP waves are 1.3.5.  2. .

III-V. Absolute latencies and interpeak intervals latencies IIII.prolonged as compared to normative data.prolonged as compared to normative data  Absent auditory brainstem response in involved ear. I-V.Abnormal BERA latency interaural difference wave (IT5) ² prolonged  I-V interpeak interval interaural differenceprolonged  Absolute latency of wave V.  Absolute .

Recording electrodes V E1 E2 .

inf. Colliculus. . Hyperbilirubine Cochlear nucleus. +/mia 8th N. inf.NEONATAL NEUROLOGICAL DISEASE AND PROBABLE REGION OF INVOLVEMENT Cochlea. cochlea. . Colliculus. infection ICH Cochlea. and cochlea Meningitis 8th N. HIE Congenital viral 8th N. cochlear nucleus.

OAE  sounds which arise in the ear canal when (paradoxical) the TM received vibration transmitted backwards through the middle ear from the cochlea.  first measurement reported in 1978 by David Kemp from the institute for laryngologist and otology .

The 4 types of otoacostic emission are as follows.sound emitted in response to a continuous tone. 3. usually clicks but can be tone bursts. TOAEs (transient otoacoustic emission) .sound emitted in response to 2 simultaneous tones of different frequencies. SOAEs (spontaneous otoacoustic emission) ² sound emitted without an acoustic stimulation. SFOAEs sustain-frequency otoacoustic emission). . 2. 1. 4. DPOAEs (distortion product otoacoustic emission).sound emitted in response to an acoustic stimuli of very short duration.

.Cont«« For infant hearing screening both DPOAEs and TOAEs used. similarly to immittamse audiometry. RECORDING -- insert a soft probe tip in the ear canal to obtain a hermetic seal.

FACTORS THAT CAN BE AFFECT OTOACOUSTIC EMISSIONS  poor probe tip placement or poor seal. Cerumen occluding the canal or blocking a probe part Vernix caseosa in neonates Debris and foreign body in outer ear canal Uncooperative patients .

5 hrs for ABR  less expensive sensitivity BERA OAE 97-100% 84% Specificity 86-96% 92% .OAE vs BERA  more easy to perform  rapid 2-10 min. vs 1.

. -The implementation of universal screening programmed has caused the definition of early identifation as early as 3 months with intervention by 6 months. Auditory structure Outer ear Middle ear Inner ear Auditory nerve Auditory Brainstem OAEs Yes Yes Yes No No BERA yes Yes Yes Yes Yes . Both tests are necessary to differentially diagnose an infant·s hearing impairment.Diagnostic OAE and ABR testing is recommended for any infant who does not pass the second screening session.NEWBORN HEARING SCREENING EFFICACY OF EARLY IDENTIFICATION AND INTERVENTION.

OAE) Repeat after 2 wks normal Diminished hearing Cont« .Flow chart for infant with hearing loss (failed screening for BERA.

re-hab.Mild. to profound deafness H . Test 3 months. exclude progressive hearing loss. sev. Aided audiometry and h. Aid speech therapy Rpt. Aid/ speech therapy h. Cochlear implants . mod. aid Inadequate help Adequate help ² cont. deafness Mod.