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Sensorineural hearing loss

Sensorineural hearing loss is a type of hearing loss in which the root cause lies in the vestibulocochlear nerve (Cranial nerve VIII), the inner ear, or central processing centers of the brain.
The Weber test, in which a tuning fork is touched to the head, localizes to the normal ear in people with this condition. The Rinne test, which tests air conduction vs. bone conduction is positive (normal), though both bone and air conduction are reduced equally. more...

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Medicines

Sudden sensorineural hearing loss is an otologic emergency, and must be treated with a high dose of steroids.

Differential diagnosis

Sensorineural hearing loss may be congenital or acquired.

Congenital

  • lack of development (aplasia) of the cochlea
  • Chromosomal syndromes (rare)
  • Congenital cholesteatoma - squamous epithelium from the ear canal invades the middle ear, which is normally covered by respiratory epithelium. The squamous epithelium behaves like an invasive tumour and destroys middle ear structures if not removed
  • Delayed familial progressive

Acquired

  • Inflammatory
    • Suppurative labyrinthitis
    • Meningitis
    • Mumps
    • Measles
    • Viral
    • Syphilis
  • Ototoxic drugs
    • Aminoglycosides (most common cause; e.g., tobramycin)
    • Loop diuretics (e.g., Furosemide)
    • Anti-Metabolites (e.g., Methotrexate)
    • Salicylates (e.g., Aspirin)
  • Physical trauma - either due to a fracture of the temporal bone affecting the cochlea and middle ear, or a shearing injury affecting cranial nerve VIII.
  • Noise-induced - prolonged exposure to loud noises (>90dB) causes hearing loss which begins at 4000Hz (high frequency). The normal hearing range is from 125 Hz to 8,000 Hz.
  • Presbyacusis - age-related hearing loss that occurs in the high frequency range (4000Hz to 8000Hz).
  • Sudden hearing loss
    • Idiopathic
    • Vascular ischemia of the inner ear or CN 8
    • Perilymph fistula, usually due to a rupture of the round or oval windows and the leakage of perilymph. The patient will most likely also experience vertigo or imbalance. A history of an event that increased intracranial pressure or caused trauma is usually present).
  • Autoimmune - a prompt injection of steroids into ear is necessary.
  • Cerebellopontine angle tumour (junction of the pons and cerebellum) (the cerebellopontine angle is the exit site of both CN7 and CN8. Patients with these tumours often have signs and symptoms corresponding to compression of both nerves)
    • Acoustic neuroma (Vestibular schwannoma) - this is a schwannoma (benign neoplasm of Schwann cells)
    • Meningioma - benign tumour of the pia and arachnoid maters
  • Meniere's disease - causes sensorineural hearing loss in the low frequency range (125 Hz to 1000 Hz). Meniere's disesase is characterized by sudden attacks of vertigo lasting minutes to hours preceded by tinnitus, aural fullness, and fluctuating hearing loss.


Table 1. A table comparing sensorineural to conductive hearing loss

Read more at Wikipedia.org


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Hereditary hearing loss - Shorts
From Townsend Letter for Doctors and Patients, 10/1/02 by Jule Klotter

About 1 in 1000 infants are born with severe to profound sensorineural hearing loss, according to an article in Audiology HealthCare News (Autumn 2001). About half of these children inherit the condition from their parents, who may or may not be deaf themselves. In most of these cases (about 70%), hearing loss is not accompanied by a recognizable set of symptoms (nonsyndromic). In addition, hearing loss is autosomal (not on a sex chromosome) recessive in about 75% of the infants with inherited deafness.

Although several genes affect hearing, researchers have identified GJB2 (gap-junction beta 2) as accounting for "up to 50% of hereditary pre-lingual severe-to-profound nonsyndromic hearing loss," according to a study by W. McGuirt and R. Smith [Connexin as a Cause of Hereditary Hearing Loss. Amer J of Audiology (Dec. 1999), 8: 93-100]. This gene determines the function of the protein Connexin 26 (Cx26), which maintains high potassium concentrations in the cochlea's scala media. About 2.5% of the population in Midwestern United States carry a mutated form of this gene.

Genetic testing for Cx26 is available in the United States. The test may help clarify the etiology of pre-lingual severe to profound sensorineural hearing loss for some people. Most of the testing is done, however, on people with no family history of hearing loss, according to this article. W. McGuirt and R. Smith recommend that appropriate explanations and counseling accompany the genetic test so that participants understand what the results mean. If one parent has severe congenital sensorineural hearing loss caused by a Cx26 mutation and the other does not carry a form of the mutated gene, it is very unlikely that any of their children will be born with impaired hearing. All of their children, however, will carry the mutated gene. On the other hand, if two hearing parents each carry a mutated Cx26 gene, each of their children has a 25% chance of being born with severe to profound hearing loss. In addition, the couple's normal-hearing children have a 66% chance of carrying the mutated Cx26 gene.

Cennoxin 26 as a Cause of Hereditary Hearing Loss. Audiology HealthCare New., 2001 Autumn.

COPYRIGHT 2002 The Townsend Letter Group
COPYRIGHT 2003 Gale Group

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