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Idiopathic Hearing Loss

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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