Hearing Loss

Timothy C. Hain, MD
Last update: 10/2012

What is Hearing Loss?

Hearing loss is very common, especially in the older population. This page provides a general overview for the diagnosis and evaluation of hearing loss.

Anatomy of the Ear

Master ear drawing

The ear is divided into the outer, middle, and inner (Figure 1). The outer ear includes the auricle, the external auditory canal, and the lateral surface of the tympanic membrane (TM). The middle ear includes the medial surface of the TM, the ossicular chain, the eustachian tube, and the tympanic segment of the facial nerve. The inner ear includes the auditory-vestibular nerve, the cochlea and the vestibular system (semicircular canals). The auditory nerve, also called the cochlear nerve, transmits sound to the brain.

Types of Hearing Loss

There are three “pure” types of hearing loss encountered commonly in clinical practice: sensorineuralconductive, and central. A fourth type, denoted  “mixed,” is a combination of sensorineural and conductive hearing loss.

Disorders of the middle ear are generally responsible for conductive losses; disorders of the cochlea or cochlear nerve are generally responsible for sensorineural hearing loss; and disorders of the brainstem or brain are generally responsible for central hearing loss.

Sensorineural Hearing Loss

Sensorineural hearing loss is the most common type of hearing loss, occurring in 23% of population older than 65 years of age. The term “sensorineural” is used to indicate that there is either a cochlear or an eighth nerve lesion. The diagnosis of a sensorineural pattern hearing loss is made through audiometry, which shows a significant hearing loss without the “air-bone gap” that is characteristic of conductive hearing disturbances. Common causes include advanced age, where the hearing pattern is often called presbyacusis (see following section); Meniere’s disease; ototoxic medications (such as high-dose aspirin or certain strong diuretics); immune disorders; and noise exposure (see following section).  Head trauma can cause both temporary and permanent hearing loss. Trauma is more likely to affect hearing in patients who suffer a temporal bone fracture or are older in age (Bergemalm, 2003).

Infections, such as meningitis, are a common cause of hearing loss in children where it occurs in approximately 20% of those with streptococcus pneumoniae meningitis (Heckenberg et al 2011). Postmeningitic hearing loss can be due to lesions of the cochlea, brainstem and higher auditory pathways, but usually is related to suppurative labyrinthitis (cochlear damage).

Tumors are a very rare cause of sensorineural hearing loss. Examples are acoustic neuroma and glomus tumor.

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Noise Induced Hearing Loss

Noise induced hearing loss is a permanent hearing impairment resulting from prolonged exposure to high levels of noise. One in 10 Americans has a hearing loss that affects his or her ability to understand normal speech. Excessive noise exposure is the most common cause of hearing loss. The National Institute of Health reports that about 15 percent of Americans aged 20 to 69 have high frequency hearing loss related to occupational or leisure activities. Because of occupational risk of noise induced hearing loss, there are government standards regulating allowable noise exposure. People working before the mid 1960s may have been exposed to higher levels of noise where there were no laws mandating use of devices to protect hearing.

Congenital Hearing Loss

There are also many inherited causes of hearing loss. Much research has focused on the detection of genetic causes of hearing loss, and many of them can now be diagnosed at an early age (Blanchard et al 2012, Dror & Avraham 2009, Kochhar et al 2007, Kokotas et al 2007, Robin et al 2005, Xing et al 2007). Treatments are mainly aimed at preventing further damage and making do with what is left. If the loss is minor, then avoidance of noise and ototoxic medications may be an appropriate treatment. If the loss is significant, then a trial of a hearing aid is often worthwhile. Occasionally, persons with acquired deafness can be treated surgically with a surgically implanted device, a cochlear implant.

Nongenetic Causes of Neonatal Hearing Loss

Children who are born prematurely or contract certain infections in utero are at a higher risk of hearing loss (Hille et al 2007, Roizen 2003). Risk factors that correlate with hearing loss in premature infants include hypoxia, mechanical ventilation, hyperbilirubinemia, very low birth weight, and the use of ototoxic medications. These conditions are relatively common in premature infants, and the majority do not go on to develop hearing deficits.

Tumors

While very unusual, acoustic neuromas or metastatic cancer (particularly breast) can be a source of hearing loss. Typically there is a combination of a hearing problem or either dizziness or imbalance. Audiometry (see Figure 2) generally shows an asymmetrical sensorineural hearing loss.

Acoustic Neuroma Audiogram
Audio Key

Sudden Hearing Loss

Sudden hearing loss (SHL) is defined as greater than 30 dB hearing reduction, over at least three contiguous frequencies, occurring over 72 hours or less. It occurs most frequently in the 30- to 60-year age group and affects males and females equally. Refer to our Sudden Hearing Loss article for more information.

Presbyacusis

Presbyacusis is defined as hearing loss associated with degenerative changes of aging. There are four types:

  • sensory presbyacusis
  • neural presbyacusis
  • striatal presbyacusis
  • cochlear conductive presbyacusis

Sensory presbyacusis is caused by loss of sensory elements in the basal (high-frequency) end of the cochlea with preservation of neurons. These patients have symmetrical, high-frequency sensorineural hearing loss (as shown in Figure 3). Pathology shows loss of hair cells.

Neural presbyacusis is caused by loss of cochlear neurons. These patients have poorer discrimination than patients with sensory presbyacusis.

Striatal presbyacusis is caused by loss of the stria vascularis with aging. Patients have a flat or slightly sloping hearing loss with good speech discrimination. Mixed presbyacusis is also possible.

Cochlear conductive presbyacusis is caused by thickening of the basilar membrane caused by deposition of basophilic substance. This diagnosis is made on postmortem (Harris 1998).

Because presbycusis occurs in some individuals as they age but not in others, genetic or environmental factors must also play a role in its development (Liu & Yan 2007). Medical conditions that are common in the elderly, such as diabetes and stroke, may also increase the risk of hearing loss with aging (Bainbridge et al 2010, Helzner et al 2005, Maia & Campos 2005, Schreiber et al 2010).

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Conductive Hearing Loss

In conductive hearing loss, the second most common form of hearing loss, sound is not transmitted into the inner ear. Diagnosis is ordinarily made via observation of an “air-bone gap” on audiometry, meaning that hearing is superior when sound is transmitted in such a way that it bypasses the middle ear ossicular chain.

tympanosclerosis audiogram
Audio Key

Characteristically, hearing aids work well for conductive hearing loss. Causes include a buildup of ear wax, a foreign body in the ear canal, otosclerosis, tympanosclerosis (as shown in Figure 4), external or middle ear infections (otitis externa or media), allergy with serous otitis media, trauma to the ossicular chain such as temporal bone fracture, tumors of the middle ear, erosion of the ossicular chain (cholesteatoma) and perforation of the tympanic membrane.

Central Deafness

Central deafness is extremely rare compared to conductive and sensorineural. A discussion of central deafness is found here.

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Acknowledgments

Images marked as copyright Northwestern University were developed with the support of a National Institutes of Health (NIH) grant to Northwestern University Dept. of Otolaryngology, and are used with permission.

  • Figure 1, Master ear drawing — (c) Northwestern University
  • Figure 2, Acoustic neuroma audiogram — (c) Northwestern University
  • Figure 3, Presbyacusis audiogram — (c) Timothy C. Hain, MD
  • Figure 4, Tympanosclerosis audiogram — (c) Northwestern University

References

  • Bainbridge KE, Cheng YJ, Cowie CC. 2010. Potential mediators of diabetes-related hearing impairment in the U.S. population: National Health and Nutrition Examination Survey 1999-2004. Diabetes care 33: 811-6
  • Blanchard M, Thierry B, Marlin S, Denoyelle F. 2012. [Genetic aspects of congenital sensorineural hearing loss]. Archives de pediatrie : organe officiel de la Societe francaise de pediatrie 19: 886-9
  • Dror AA, Avraham KB. 2009. Hearing loss: mechanisms revealed by genetics and cell biology. Annual review of genetics 43: 411-37
  • Harris J. 1998. Hearing Loss. Audio-Digest Otolaryngology 31
  • Heckenberg SG, Brouwer MC, van der Ende A, Hensen EF, van de Beek D. 2011. Hearing loss in adults surviving pneumococcal meningitis is associated with otitis and pneumococcal serotype. Clinical microbiology and infection : the official publication of the European Society of Clinical Microbiology and Infectious Diseases
  • Helzner EP, Cauley JA, Pratt SR, Wisniewski SR, Zmuda JM, et al. 2005. Race and sex differences in age-related hearing loss: the Health, Aging and Body Composition Study. Journal of the American Geriatrics Society 53: 2119-27
  • Hille ET, van Straaten HI, Verkerk PH. 2007. Prevalence and independent risk factors for hearing loss in NICU infants. Acta Paediatr 96: 1155-8
  • Kochhar A, Hildebrand MS, Smith RJ. 2007. Clinical aspects of hereditary hearing loss. Genetics in medicine : official journal of the American College of Medical Genetics 9: 393-408
  • Kokotas H, Petersen MB, Willems PJ. 2007. Mitochondrial deafness. Clinical genetics 71: 379-91
  • Liu XZ, Yan D. 2007. Ageing and hearing loss. The Journal of pathology 211: 188-97
  • Maia CA, Campos CA. 2005. Diabetes mellitus as etiological factor of hearing loss. Brazilian journal of otorhinolaryngology 71: 208-14
  • Robin NH, Prucka SK, Woolley AL, Smith RJ. 2005. The use of genetic testing in the evaluation of hearing impairment in a child. Current opinion in pediatrics 17: 709-12
  • Roizen NJ. 2003. Nongenetic causes of hearing loss. Mental retardation and developmental disabilities research reviews 9: 120-7
  • Schreiber BE, Agrup C, Haskard DO, Luxon LM. 2010. Sudden sensorineural hearing loss. Lancet 375: 1203-11
  • Xing G, Chen Z, Cao X. 2007. Mitochondrial rRNA and tRNA and hearing function. Cell research 17: 227-39

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