Variable inner ear malformations mondini malformation

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variable inner ear malformations (Mondini malformation with or without enlarged vestibular aqueduct). Abn. Vestibular function Recessive- 3 rd most common Jervell and Lange Nielsen Prolonged QT syndrome- history of SIDS or sudden death
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Laboratory testing Dx in first 2 weeks- culture of saliva or urine for CMV In older children with hx of renal disease ; urinalysis for hematuria and proteinuria suggestive of Alport Syndrome
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Other Testing Electrocardiography is indicated if there is a family history of prolonged QT interval or sudden death, or if medical history is significant for syncope, arrhythmia, or history of ALTE. An ECG should also be considered in all infants with severe-profound sensorineural hearing loss of unknown etiology.
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Referral to Pediatric Otolarygology Performs a comprehensive evaluation of the head and neck and can help in the identification of craniofacial malformations associated with hearing loss. Should work closely with the audiology team (including Early Intervention specialist) to determine intervention planning for hearing loss. Performs surgical intervention if necessary (e.g., tympanostomy tubes, reconstruction, cochlear implant, bone-anchored hearing aids).
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Pediatric Ophthalmology Every child with confirmed permanent HL should undergo annual evaluation by an ophthalmologist to determine visual acuity and evaluate for concomitant vision disorders (e.g., Usher Syndrome, cataracts ).
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Summarizes the Medical Work-up Physical exam Skin dysplasia, or atresia, craniofacial abnormalities, skeletal abnormalities Dysmorphism MEE Family History of HL onset <30 years History of care in NICU for > 5 days Urine Culture for CMV- most common infectious cause CT of inner ear to r/o anatomic abnormalities Genetic studies to r/o Connexin 26 and 30/other EKG for severe/profound to r/o long QT Syndrome Consults with otolaryngology, genetics, and ophthalmology
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New Protocol in WBN Well-babies screened and not passed by AABR may be rescreened and passed by OAE ; The WBN rate of AN < 1%. Although there is a possibility of missing AN, there is an unacceptable high “loss-to-follow-up” rate for infants who do not pass the birth screen and fail to return for rescreening. The revised recommendation is that WBN screen and second in-hospital screen may use either OAE or AABR. However, the recommendation to rescreen using only AABR for the infant who does not pass initial AABR continues to be the Committee’s preferred protocol
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Frequently Asked Questions by parents
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EHDI A reconsideration for 1-3-6 m screening completed by 1 m, audiologic diagnosis by 3 m, enrollment in Early Intervention by 6 m be updated to the recommendation of 1-2-3 m Earliest age of identification is encouraged to provide audiologic testing in natural sleep and to facilitate early access to language
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Surveillance in the Medical Home All infants should have regular surveillance by their PCP consistent with the pediatric periodicity schedule of: auditory skills developmental milestones parent concerns regarding hearing or language middle ear effusion
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  • Fall '19
  • Otology, Sensorineural Hearing Loss, Tinnitus, Hearing impairment, Long QT syndrome

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