Dr.

Supreet Singh Nayyar, AFMC

2012

Auditory Neuropathy
(for more topics & presentations, visit www.nayyarENT.com ) Pathophysiology
    Injury to the synaptic junction between inner hair cells of the cochlea and dendrites of spiral ganglion neurons Direct damage to the dendrites of the spiral ganglion neurons Direct injury to the spiral ganglion neurons Direct axonal damage to the auditory nerve that causes a cascade of damage to the lower auditory nuclei

Epidemiology  Rare  1-3 / 10000 births
  2-15% of deaf children M:F = 1:1
(FIBHOCVAMS)

Aetiology & Risk factors           Family history Infections – TORCHES Birth weight low (<1500 gm) Hyperbilirubenemia Ototoxic medications Craniofacial anomalies Ventilator / NICU (> 5 days) Apgar score low Meningitis Stigmata / syndromes

In addition    Viral diseases Seizure disorders High fever

Associated disorders  Friedrich ataxia  Stevens-Johnson syndrome  Ehlers-Danlos syndrome  Charcot-Marie-Tooth syndrome

History
   Risk factors associated with perinatal hearing loss Mild-to-severely abnormal hearing thresholds Severe speech and language delay out of proportion with the presumed hearing loss

Imaging Studies  To rule out inner ear malformations

Other Tests
 PTA 1

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Dr. Supreet Singh Nayyar, AFMC     Speech audiometry Acoustic reflex ↓ OAE Normal ABR reduced

2012

Criteria for the diagnosis of AN/AD are as follows:  Absent or severely abnormal ABR test results at maximal stimulus (100 dBnHL)  Normal outer hair cell function as determined by OAEs  Absent or elevated stapedial reflex thresholds

Medical Care
  Parental counselling explaining, disease, prognosis Children with AN/AD were once thought not to benefit from hearing aid amplification; however, recent studies demonstrate that 50% of children can benefit from placement of an amplification device. When children with AN/AD were tested with hearing aids, their speech discrimination scores improved and were more consistent with the degree of hearing loss expected via their pure tone audiometry scores. The use of hearing aids prior to cochlear implantation is currently recommended. Once the child is aged approximately 6 months, behavior audiometry thresholds should be obtained. Because the presentation and thresholds of AN/AD are so varied, the determination of more accurate levels of hearing loss helps to dictate the future intervention necessary for each child. Communicative devices, which are options for any child with mild-to-severe hearing loss, also pertain to children with AN/AD. The use of conventional hearing aids and frequency modulation (FM) systems can help a child develop necessary speech and language skills If a child does not progress with hearing aid devices and shows limited speech discrimination abilities, cochlear implantation is the next viable option.

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Surgical Care
  Cochlear implants  In 2001, the use of cochlear implantation was expanded to include children with AN  long-term results have been promising, with demonstrations that children with AN/AD and implants had equivalent hearing abilities to other children of similar age with implants Most recently, if cochlear implantation fails, the option of brainstem implantation has been reported in one child

Consultations
          Multidisciplinary approach Otologist Speech pathologist Genetic counsellor Audiologist Pediatric neurologist Teacher Parents Social worker Paediatrician (for more topics & presentations, visit www.nayyarENT.com )

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Dr. Supreet Singh Nayyar, AFMC

2012

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