Table 4.

Treatment of Manifestations in Individuals with DDON Syndrome

Manifestation/
Concern		TreatmentConsiderations/Other
Poor visual acuity /
Blindness 		Corrective lenses / standard treatmentCommunity vision services through early intervention or school district
Hearing impairment /
Deafness 		Treatment of SNHL, w/focus on auditory neuropathy, depends on degree of hearing impairment. 1
  • Start hearing habituation (auditory & speech training, sign language) as soon as possible.
  • Community hearing services through early intervention or school district
Cochlear implant
  • CT of bony landmarks & MRI of vestibular & facial nerves as part of pre-cochlear implant assessment 2, 3
  • Cochlear implants may provide sound awareness & even speech recognition in presence of cochlear abnormalities. 4
  • Outcome is expected to be variable in auditory neuropathy.
Communication Depends on degree of hearing & vision impairmentRefer to community deaf-blind services & state Deafblind Project as soon as possible after birth. 8
Dystonia Physical medicine & rehab / PT & OT
  • To improve gross motor skills & mobility & prevent contractures
  • To improve fine motor skills
  • Provide adaptive devices to improve activities of daily living.
DD/ID See Developmental Delay / Intellectual Disability Management Issues.
Behavioral
concerns 		Standard medications for obsessive-compulsive disorderBehavioral therapy combined w/stress reduction is sometimes helpful.
Behavior therapy, stress reduction, & standard medications for pervasive developmental disorderBehaviors may mimic autism but are different; 9 may be exacerbated by sensory processing issues.
Establish an appropriate method of communication & provide adequate stimulation for exploration in a safe environment for ADHD.May be more helpful than medication
Family needs Ensure appropriate social work involvement to connect families w/local resources, respite, & support.Ongoing assessment for need of home nursing
Coordinate care to manage multiple subspecialty appointments, equipment, medications, & suppliesConsider involvement in adaptive sports or Special Olympics.

ADHD = attention-deficit/hyperactivity disorder; DD/ID = developmental delay / intellectual disability; OT = occupational therapy; PT = physical therapy; SNHL = sensorineural hearing loss

1.

See Hereditary Hearing Loss and Deafness Overview for details about treatment options.

From: Deafness-Dystonia-Optic Neuronopathy Syndrome

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