9. Disorders of the inner ear, congenital malformations, hereditary deafness

• Disorders of the inner ear
  • Benign paroxysmal positional vertigo
  • Trauma
  • Labyrinthitis
    • Complication of AOM
    • Hearing loss
    • Vertigo
  • Vestibular neuritis
    • Idiopathic
    • Possibly viral
    • No hearing loss
    • Vertigo
    • Positive head thrust test
  • Acoustic neuroma
  • Meniere syndrome

Congenital malformations

• Michel aplasia = complete labyrinthine aplasia
• Mondini aplasia
  • 1,5 turns of cochlea instead of 2,5
  • Sensorineural hearing loss
• Congenital CSF otorrhoea
  • Fistula of oval window
• Thalidomide embryopathy

Hereditary deafness

• Syndromic hereditary hearing loss
  • Down syndrome
• Non-syndromic hereditary hearing loss
  • Congenital form
    • Autosomal recessive
    • Only lack of hearing
    • Symmetrical
    • Present at birth
  • Later-onset form
    • Hearing loss occurs later
    • Autosomal dominant