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INTRODUCTION TO PAEDIATRIC AUDIOLOGY

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Prenatal (Infections, Medications, Alcohol consumption, Overseas, ... 4. Electrocardiogram (in severe and profound SNHL: Jervell and Lange-Nielsen syndrome) ... – PowerPoint PPT presentation

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Title: INTRODUCTION TO PAEDIATRIC AUDIOLOGY


1
  • INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Dr. Juan Mora
  • (Consultant Audiological Physician)
  • and Paediatric Audiology Team
  • (Yorkhill Hospital, Glasgow)
  • October 2007

2
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • HISTORY
  • PARENTAL/PROFESSIONAL CONCERNS
  • PREGNANCY/PERINATAL HISTORY
  • PAST MEDICAL HISTORY
  • CHILD DEVELOPMENT
  • FAMILY HISTORY OF HEARING LOSS

3
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • History
  • Prenatal (Infections, Medications, Alcohol
    consumption, Overseas, Diabetes, Epilepsy)
  • Perinatal (Gestation length, Delivery, Birth
    weight, Perinatal health, Age at discharge)
  • Postnatal (General health, Vaccinations,
    Meningitis, Drug history, Head injuries)
  • Developmental history
  • Speech and language, Education, Motor, Cognitive
    and Behaviour

4
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Family History
  • of Hearing Loss
  • of Speech and Language development/delay
  • of Learning difficulties
  • of Renal, Heart, Thyroid, Ocular, hair iris
    pigmentation changes which could be associated
    with hearing loss
  • of Consanguinity
  • Social History

5
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Examination
  • Ear, Oral and Nasal cavities, Head, Eyes, Neck,
    Vestibular
  • Trunk, Abdomen
  • Childs attitude
  • Observe the family (!)

6
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • AETIOLOGICAL INVESTIGATIONS
  • Websites
  • http//www.baap.org.uk
  • http//hearing.screening.nhs.uk

7
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Why investigate the aetiology?
  • Parents frequently feel anxious if the cause of
    the hearing loss if unknown
  • To diagnose and treat any co-existing conditions
    (e.g. cardiac conduction defects)
  • Genetic counselling (for the parents and
    eventually later the child)

8
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Why investigate the aetiology?
  • May help prevent deterioration of the hearing
    levels (antiviral treatment for congenital CMV
    infection, susceptibility to aminoglycosides
    ototoxicity in A1555G mitochondrial mutations)
  • Allows better advice/management of the hearing
    loss in some cases (e.g. progressive hearing loss
    after head injuries if wide vestibular aqueduct)

9
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Who organises the investigations?
  • Medical (Otolaryngologist, Paediatrician,
    Community Doctor in Audiology, Audiological
    Physician, Geneticist)
  • Parents must have comprehensive and unbiased
    information about the medical investigations into
    the aetiology of the hearing loss. Parents make
    an informed decision about the investigations,
    and their views should be respected

10
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Who to investigate?
  • These guidelines apply to bilateral permanent
    hearing loss gt40 dB HL (moderate, severe and
    profound losses)
  • These guidelines are not intended for mild
    hearing loss or unilateral hearing loss

11
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • How and When to investigate?
  • Aetiological investigation of hearing loss is not
    a static process but an ongoing one
  • Investigate as early as possible (yield may be
    age dependent e.g. congenital cytomegalovirus
    infection investigating early may be safer for
    the patient)
  • If the aetiology has not been established
    clearly, it should be reviewed periodically on
    the emergence of new evidence
  • Aetiology can be multiple

12
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Which investigations?
  • History
  • 2. Physical Examination (includes
    developmental assessment)
  • 3. Family audiograms (1st degree relatives)

13
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Which investigations?
  • 4. Electrocardiogram (in severe and profound
    SNHL Jervell and Lange-Nielsen syndrome)
  • (a type of long QT syndrome))
  • 5. Imaging
  • -MRI of Inner ears/Internal auditory meatus
  • -CT of Petrous temporal bones
  • (When early if possible progression/post-meningi
    tis)
  • 6. Renal Ultrasound
  • (If history of renal problems, multi-system
    abnormalities)
  • (If suspected branchio-oto-renal syndrome)

14
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Which investigations?
  • 7. Genetics
  • -Connexin 26 and 30 mutations commonest cause of
    non- syndromal autosomal recessive hearing loss
    in caucasians
  • -A1555G mitochondrial mutation
  • -Kariotype chromosomal abnormalities
  • -Others Pendrin gene, BOR, Jervell and
    Lange-Nielsen,..
  • 8.Infection screen
  • -Cytomegalovirus
  • -Other Rubella, Toxoplasma, Syphilis

15
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Which investigations?
  • 9. Ophthalmology
  • (Not infrequently ophthalmic conditions coexist
    with sensorineural hearing loss. E.g. Usher
    syndrome, Congenital cytomegalovirus, Rubella,
    CHARGE)
  • 10. Others
  • -Urine examination Urine microscopy if family
    history of haematuria
  • -Urea, electrolytes and serum creatinine to
    investigate renal function abnormalities
    (Alports syndrome)
  • -Other investigations as required

16
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Investigations recommended NHSP England
  • CORE If bilateral hearing loss gt70 dbHL (may be
    required in other cases)
  • ADDITIONAL If there is a specific clinical
    indication

17
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • CORE
  • General History Pregnancy, Perinatal, Postnatal,
    Developmental.
  • Family History (Hearing Loss and risk factors
    associated with hearing loss).
  • Physical examination and developmental assessment.

18
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • CORE
  • Family audiograms 1st degree relatives
  • Radiology of the Head and Neck (CT of petrous
    temporal bone or MRI of inner ears and internal
    auditory meati)

19
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • CORE
  • ECG
  • Genetics (Connexins 26 and 30, Mitochondrial
    A1555G mutations, Chromosomal abnormalities,
    others)
  • Infection Screen (CMV and Rubella)
  • Ophthalmology

20
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • ADDITIONAL
  • Renal Ultrasound
  • Infection Screen (Toxoplasma, Syphilis)
  • Biochemistry (UAEs and serum creatinine) (TSH
    already available)
  • Urine examination (metabolic screen and urine
    microscopy)

21
INTRODUCTION TO PAEDIATRIC AUDIOLOGY
  • Paediatric Audiology involves multidisciplinary
    work
  • Doctors and Audiologists
  • and
  • Teachers for the Hearing Impaired, Educational
    Audiologists, Speech and Language Therapists,
    Teachers in Hearing Impaired Units,
    Psychologists, Social Workers, other Medical
    colleagues and others.
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