Idiopathic Hearing Loss

by Sharon Hopkins


Idiopathic hearing loss or sudden hearing loss is a medical condition that continues to elude definitive diagnosis and treatment till date. Since majority of the cases of sudden hearing loss evade definitive diagnosis despite extensive evaluation, they are termed as idiopathic.

Some of the probable causes of idiopathic hearing loss can be classified under the following heads -

Viral and Infectious

  • Meningococcal meningitis
  • Herpes virus (simplex, zoster, varicella, cytomegalovirus)
  • Mumps
  • Human immunodeficiency virus
  • Cryptococcal meningitis
  • Toxoplasmosis
  • Syphilis
  • Rubeola
  • Rubella
  • Human spumaretrovirus
  • Lassa fever
  • Mycoplasma

Autoimmune

  • Autoimmune inner ear disease
  • Ulcerative colitis
  • Cogan's syndrome
  • Wegener's granulomatosis
  • Relapsing polychondritis
  • Polyarteritis nodosa

Labyrinthine membrane rupture/traumatic

  • Inner ear concussion
  • Otologic surgery (stapedectomy)
  • Perilymph fistula
  • Inner ear decompression sickness
  • Temporal bone fracture
  • Surgical complication of nonotologic surgery.

Vascular

  • Vascular disease/alteration of microcirculation
  • Red blood cell deformability
  • Sickle cell disease
  • Vascular disease associated with mitochondriopathy
  • Vertebrobasilar insufficiency
  • Cardiopulmonary bypass

Neurological

  • Multiple sclerosis
  • Focal pontine ischemia
  • Migraine

Neoplastic

  • Acoustic neuroma
  • Leukemia
  • Myeloma
  • Meningeal carcinomatosis
  • Contralateral deafness after acoustic neuroma surgery
  • Metastasis to internal auditory canal

Although majority of the cases of idiopathic sudden hearing loss are cases with no known cause, those with a background can be treated to eradicate the underlying condition. For e.g. Surgery for acoustic neuroma, ototoxicity can be treated with cessation of ototoxic drugs, sickle-cell crisis with oxygen and so on. Treatment for idiopathic sudden hearing loss continues to be a matter of debate. Steroids have been known to show significant improvement in hearing recovery in patients with moderate or severe hearing loss and are a favored method of treatment for autoimmune and idiopathic forms of hearing loss.


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