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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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ENG in a dizzy patient with binaural hearing loss following surgery for Meniere's syndrome
From Ear, Nose & Throat Journal, 1/1/04 by Kenneth H. Brookler

A 20-year-old woman came to the office with a 6-year history of Meniere's syndrome. Her condition had come on suddenly; her symptoms had been preceded by the onset of tinnitus in both ears, which was followed by violent rotary vertigo with nausea and vomiting. During each recurrence, these spells would last from 20 minutes to 2 hours. She had undergone placement of a right endolymphatic mastoid shunt, but she noticed no change in her dizziness postoperatively.

At her visit to the author's office, the patient reported no significant dizziness. She was taking cortisone, a diuretic, and a nausea suppressant, which were controlling this symptom. She still experienced some hearing loss in both ears, more so on the right. Beginning 6 years earlier, her hearing had progressively deteriorated until she underwent the mastoid shunt surgery. Thereafter, the quality of her hearing fluctuated and had remained fluctuant until 3 months prior to her office visit. It had been stable since.

The patient's primary concern was the preservation of her hearing. Her tinnitus in the left ear had resolved, but it was still present in the right ear. It had been constant for the preceding 3 months. She reported aural fullness in both ears (more so on the right) upon the onset of dizziness.

On clinical examination, the patient experienced difficulty performing the sharpened tandem Romberg's test. Her family history was negative for hearing loss and dizziness. Electronystagmography in the absence of medications revealed no spontaneous, positional, or neck-torsion nystagmus. The alternate binaural bithermal test elicited a hypoactive (and sometimes absent) response to the cool stimulus on the right and no response to the warm stimulus in either ear (a reduced vestibular response of 66 to 100% right and a directional preponderance of 66 to 100% right). Ice-water calorics in the right ear elicited a normal response, which represented a form of vestibular recruitment and suggested that the source of the dizziness was the labyrinth. The simultaneous binaural bithermal test elicited a type 1 response--that is, no nystagmus was produced with either the warm or cool simultaneous stimulus.

Audiometry identified a bilateral sensorineural hearing loss. The loss was moderately fiat in the left ear. The right ear exhibited a moderate to severe loss of low-tone hearing and a moderate loss of middle-and high-tone hearing. Further testing revealed that the patient had a 50-dB speech reception threshold (SRT) and an 84% speech discrimination score (SDS) in the right ear, and a 30-dB SRT and a 92% SDS in the left ear.

The vestibular findings in this case are consistent with a bilateral peripheral vestibular disorder. However, these findings might also be explained by the fact that stimulation of the efferents from the right ear suppressed or inhibited the auditory and vestibular hair cell function in the apparently unaffected left inner ear.

From Neurotologic Associates, P.C., New York City.

COPYRIGHT 2004 Medquest Communications, LLC
COPYRIGHT 2004 Gale Group

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