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.