sensorineural adj : of or relating to the neural process of sensation

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.

Sensorineural hearing loss can be mild, moderate, or severe, including total deafness.

The great majority of human sensorineural hearing loss is caused by abnormalities in the hair cells of the organ of Corti in the cochlea. There are also very unusual sensorineural hearing impairments that involve the VIIIth cranial nerve (the Vestibulocochlear nerve) or the auditory portions of the brain. In the rarest of these sorts of hearing loss, only the auditory centers of the brain are affected. In this situation, central hearing loss, sounds may be heard at normal thresholds, but the quality of the sound perceived is so poor that speech can not be understood.

Most sensory hearing loss is due to poor hair cell function. The hair cells may be abnormal at birth, or damaged during the lifetime of an individual. There are both external causes of damage, like noise trauma and infection, and intrinsic abnormalities, like deafness genes.

Sensory hearing loss (also called sensorineural hearing loss) may also result from abnormalities of the VIII cranial nerve.

Sensory hearing loss that results from abnormalities of the central auditory system in the brain is called Central Hearing Impairment. Since the auditory pathways cross back and forth on both sides of the brain, deafness from a central cause is unusual.

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 is normally present on either side of the tympanic membrane. Externally, within the external auditory meatus or ear canal and internally within the middle ear. Within the middle ear the simple epithelium gradually transitions into ciliated pseudostratified epithelium lining the Eustachian tube now known as the pharyngotympanic tube becoming continuous with the respiratory epithelium in the pharynx. The squamous epithelium hyperplasia within the middle ear 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)
  • Antimetabolites (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 (>90 dB) causes hearing loss which begins at 4000Hz (high frequency). The normal hearing range is from 20 Hz to 20,000 Hz.

• Presbyacusis - age-related hearing loss that occurs in the high frequency range (4000Hz to 8000Hz).

• Sudden hearing loss
  • Idiopathic (ISSHL: idiopathic sudden sensoneurinal hearing loss), H91.2, German Wikipedia: "Hörsturz"
  • 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.

Long term exposure to environmental noise

Populations of people living near airports or freeways are exposed to levels of noise typically in the 65 to 75 dbA range. If lifestyles include significant outdoor or open window conditions, these exposures over time can degrade hearing. The U.S. EPA and various states have set noise standards to protect people from these adverse health risks. The EPA has identified the level of 70 db(A) for 24 hour exposure as the level necessary to protect the public from hearing loss (EPA, 1974).

• Noise-Induced Hearing Loss (NIHL) typically is centered at 4000 Hz.
• The louder the noise is, the shorter the safe amount of exposure is. Normally, the safe amount of exposure is reduced by a factor 2 for every additional 3 dB. For example, the safe daily exposure amount at 85 dB is 8 hours, while the safe exposure at 91 dB(A) is only 2 hours (National Institute for Occupational Safety and Health, 1998). Sometimes, a factor 2 per 5 dB is used.
• Personal electronic audio devices, such as iPods (iPods often reaching 115 decibels or higher), can produce powerful enough sound to cause significant Noise-Induced Hearing Loss, given that lesser intensities of even 70 dB can also cause hearing loss.

Genetic

Hearing loss can be inherited. Both dominant and recessive genes exist which can cause mild to profound impairment. If a family has a dominant gene for deafness it will persist across generations because it will manifest itself in the offspring even if it is inherited from only one parent. If a family had genetic hearing impairment caused by a recessive gene it will not always be apparent as it will have to be passed onto offspring from both parents Dominant and recessive hearing impairment can be syndromic or nonsyndromic. Recent gene mapping has identified dozens of nonsyndromic dominant (DFNA#) and recessive (DFNB#) forms of deafness.

• The most common type of congenital hearing impairment in developed countries is DFNB1, also known as Connexin 26 deafness or GJB2-related deafness.
• The most common dominant syndromic forms of hearing impairment include Stickler syndrome and Waardenburg syndrome.
• The most common recessive syndromic forms of hearing impairment are Pendred syndrome, Large vestibular aqueduct syndrome and Usher syndrome.

Disease or illness

• Measles may result in auditory nerve damage
• Meningitis may damage the auditory nerve or the cochlea
• Autoimmune disease has only recently been recognized as a potential cause for cochlear damage. Although probably rare, it is possible for autoimmune processes to target the cochlea specifically, without symptoms affecting other organs. Wegener's granulomatosis is one of the autoimmune conditions that may precipitate hearing loss.
• Mumps (Epidemic parotitis) may result in profound sensorineural hearing loss (90 dB or more), unilateral (one ear) or bilateral (both ears).
• Presbyacusis is deafness due to loss of perception to high tones, mainly in the elderly. It is considered by some to be a degenerative process, although there has never been a proven link to aging. (See impact of environmental noise exposure above.)
• Adenoids that do not disappear by adolescence may continue to grow and may obstruct the Eustachian tube, causing conductive hearing impairment and nasal infections that can spread to the middle ear.
• AIDS and ARC patients frequently experience auditory system anomalies.
• HIV (and subsequent opportunistic infections) may directly affect the cochlea and central auditory system.

Table 1. A table comparing sensorineural to conductive hearing loss

Treatment

At present, sensorineural hearing loss is treated with hearing aids, which amplify sounds at pre-set frequencies to overcome a sensorineural hearing loss in that range; or cochlear implants, which stimulate the cochlear nerve directly.

External References

• Sensorineural Hearing Loss, Dr Peter Grant

References