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Hearing loss

A hearing impairment is a decrease in one's ability to hear (i.e. perceive auditory information). While some cases of hearing loss are reversible with medical treatment, many lead to a permanent disability (often called deafness). more...

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If the hearing loss occurs at a young age, it may interfere with the acquisition of spoken language and social development. Hearing aids and cochlear implants may alleviate some of the problems caused by hearing impairment, but are often insufficient. People who have hearing impairments, especially those who develop a hearing problem later in life, often require support and technical adaptations as part of the rehabilitation process.


There are four major causes of hearing loss: genetic, disease processes affecting the ear, medication and physical trauma.


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 recognised 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 precipiate hearing loss.
  • Presbyacusis is deafness due to loss of perception to high tones, mainly in the elderly. It is considered a degenerative process, and it is poorly understood why some elderly people develop presbyacusis while others do not.
  • Mumps (Epidemic parotitis) may result in profound sensorineural hearing loss (90 dB or more), unilateral (one ear) or bilateral (both ears).
  • 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.
  • Chlamydia may cause hearing loss in newborns to whom the disease has been passed at birth.
  • Fetal alcohol syndrome is reported to cause hearing loss in up to 64% of infants born to alcoholic mothers, from the ototoxic effect on the developing fetus plus malnutrition during pregnancy from the excess alcohol intake.
  • Premature birth results in sensorineural hearing loss approximately 5% of the time.
  • Syphilis is commonly transmitted from pregnant women to their fetuses, and about a third of the infected children will eventually become deaf.
  • Otosclerosis is a hardening of the stapes (or stirrup) in the middle ear and causes conductive hearing loss.


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Primary care approach to hearing loss: the hidden disability
From Ear, Nose & Throat Journal, 1/1/05 by Seth M. Cohen


We report the results of a survey designed to investigate audiologic referral patterns of primary care physicians and, more specifically, their referral of patients for hearing aids and cochlear implants. Three hundred internal medicine and family medicine physicians were identified from a referral basin of a tertiary care center and chosen randomly to be faxed questionnaires concerning their views about patients with hearing loss, hearing loss screening and referral practices, and availability of local resources. Of the 260 physicians who received a questionnaire, 85 (32.7%) responded. Of their communities (60% of which had populations of fewer than 50,000). 82.4% had an otolaryngologist and 40% had access to an academic center. Although 97.6% of the responding physicians indicated that hearing loss affected patients' quality off life, only 60% assessed patients for hearing loss. "Lack of time" and "more pressing issues" were the most common reasons given for not evaluating patients for hearing loss. Although 76 physicians (89.4%) said they were aware of cochlear implants, only 22 (25.9%) had referred patients for implant evaluation. Lack of referral most commonly resulted from uncertainties about "where to refer" and "which patients were potential candidates." The results of this survey suggest that a large percentage of primary care physicians do not routinely test for hearing impairment in adults.


As the population ages, more people have chronic health problems. Approximately 12.4% of the population is 65 years or older, and the segment older than 85 years has increased the most. (1) Hearing loss is the third most common chronic condition in older adults, affecting between 25 and 40% of adults over the age of 65. (2-4) Decreased hearing has many implications for older adults. Hearing loss has been associated with physical and psychosocial dysfunction, depression, and decreased well-being. (5-7) However, auditory rehabilitation, with hearing aids and cochlear implants, can ameliorate the adverse effects of hearing impairment and increase patients' physical, social, and psychological function. (8-10)

Primary care physicians (PCPs) have a unique opportunity to identify patients with hearing loss and direct them to appropriate treatment. They treat many chronic conditions, direct each patient's health maintenance, and are usually the first physicians to learn of a new problem. Because hearing impairment may be underdiagnosed, recent work has emphasized the importance of screening and has described practical approaches for evaluating hearing loss. (11-12) By screening for hearing impairment, PePs can uncover unrecognized cases and facilitate intervention. However, evaluating multiple chronic health problems and staying current with the ongoing improvements in hearing aids and the rapidly increasing field of cochlear implantation can be a difficult task. This study provides insights about the current state of hearing-loss screening among PCPs.

Materials and methods

After obtaining Institutional Review Board approval, a questionnaire was sent to PePs practicing in a tertiary care referral basin. Physicians were identified by community visits, lists from local hospitals, continuing medical education programs, telephone books, and local medical societies. This list of physicians is updated at least yearly and includes 1,581 physicians practicing internal medicine and family medicine in 43 Kentucky and Tennessee counties.

A random sample of 300 physicians was selected, and each physician was faxed a questionnaire. If no response was received in 1 month, a second questionnaire was faxed; and if still no response was received, a third questionnaire was sent. Forty physicians who did not have a working fax machine were excluded, leaving 260 physicians.

Questions concerned the physician's practice setting, medical specialty, attitudes regarding hearing loss, method of evaluating patients for hearing loss, and referral of patients for hearing aids and cochlear implants. When a respondent did not answer a question, the response was noted as "no response." Questions were of the yes/no variety or allowed the respondent to choose one of the provided answers or write in a response. Data are presented in numeric form and as a percentage. SigmaStat 2.03 (SPSS, Inc.; Chicago) software was used to perform chi-square or Fisher Exact Test analysis for categorical data.


Of the 260 questionnaires sent, 85 responses were received, producing a response rate of 32.7%. More than half of all respondents were from towns of fewer than 50,000 people, and one-fourth practiced in towns of more than 500,000 people. Of the respondents' communities, 82.4% had an otolaryngologist, 67.1% had an audiologist, 40.0% had an academic center, and 12.9% had none of the above.

Among the respondents, 34 (40.0%) reported that they do not routinely evaluate their patients for hearing loss. Among internal medicine physicians, 56.1% screen for hearing loss, compared with 70.6% of family medicine physicians (p = 0.3, chi-square test). Lack of time and the presence of more important issues were the most common reasons given for not evaluating hearing loss (table 1). Of the respondents, 17.6% reported that they assess hearing loss only when a patient recognizes a hearing problem; 11.8% examine possible hearing loss at yearly physicals; and 7.8% start investigating potential hearing impairment at age 40, 5.9% at age 50, and 5.9% at age 65. Responses indicated that various methods are used by PCPs when investigating patients' hearing (table 2). A patient's reporting a change in hearing was the most common reason given for referral to an audiologist or an otolaryngologist (table 3).

Of PCPs practicing in towns with an academic center, 50% assessed patients for hearing loss, compared with 66.7% of PCPs practicing in towns without an academic center (p = 0.1, chi-square test). Similarly, with respect to evaluating hearing loss, no statistically significant associations were seen between respondents' having an audiologist or an otolaryngologist available in the community (62.2%) and their having neither an audiologist nor an otolaryngologist available (45.5%; p = 0.3, Fisher Exact Test), or between PCPs practicing in towns of more than 500,000 people (74.5%) and those practicing in towns of fewer than 500,000 people (73.5%; p = 0.9, chi-square test).

All but two respondents thought that hearing loss affected their patients' quality of life. Among respondents, 70 (82.4%) thought patients with hearing aids were more likely to be socially active, 44 (51.8%) thought patients were satisfied with their hearing aids, and 8 (9.4%) thought patients were marginally satisfied. Although two-thirds of the physicians responded that they send patients back to the audiologist if the patients are unhappy with their aids, 12.9% do not.

Seventy-six respondents (89.4%) stated that they know about cochlear implants, and 22 (25.9%) refer deaf patients for evaluation. Eight of those who refer elderly adults stated that they have no age limit at which they stop referring. Of the 9 physicians who were unaware of cochlear implants, 5 had an audiologist, an otolaryngologist, and an academic center in their community. One of the 9 had none of the above. Of the 73 respondents who do not refer patients for cochlear implant evaluation, not knowing which patients are appropriate candidates or where to refer them were the most common reasons preventing referral (table 4). Among physicians with academic centers in their communities, 35.3% referred deaf patients to an otolaryngologist, compared with 19.6% of those without academic centers in their communities (p = 0.2, chi-square test). Of respondents with local audiologists and otolaryngologists, 27.8% referred deaf patients for evaluation, compared with 15.4% of those without an audiologist or otolaryngologist (p = 0.7, Fisher Exact Test).


As the population ages, the prevalence of hearing loss and its adverse effects on quality of life and communication will escalate. Because hearing loss poses a significant disease burden and effective screening methods and treatment options are available, routine screening should be performed. (11) Because PCPs are already involved in health promotion, they have the potential to identify elderly patients suffering from hearing impairment. This study was undertaken to uncover PCPs' attitudes regarding hearing loss and to identify the obstacles they face when referring impaired adults for treatment. Despite the resulting dysfunction hearing loss presents, various obstacles prevented a large portion of PCPs in our study from regularly evaluating their elderly patients for hearing loss.

Although the American Academy of Family Physicians recommends screening for hearing loss at yearly physicals, (13) 40.0% of respondents reported that they do not screen for hearing loss. Similarly, Logan et al found that 80% of physicians surveyed did not routinely screen for hearing loss in elderly patients. (14) Bess et al found that when patients do complain, only half are referred for management. (15) Thus, many potential patients with hearing impairment do not receive the benefits of auditory rehabilitation. Respondents who had local audiologists, otolaryngologists, and academic centers were no more likely to evaluate their patients for hearing loss than those without these resources. Despite having the means for auditory rehabilitation within their communities, respondents were not taking full advantage of these resources by identifying patients with impaired hearing and referring them for interventions.

Various screening methods have been developed to assist PCPs in addressing hearing loss. Lichtenstein et al validated the use of a screening questionnaire and a portable audioscope--an otoscope with a built-in audiometer. (16) Not only can the ear be examined with this device, but an estimate of hearing thresholds also can quickly be obtained. The American Academy of Otolaryngology-Head and Neck Surgery also has developed a screening questionnaire. (17) The U.S. Preventive Services Task Force and other professional organizations, geriatric experts, and available literature recommend screening elderly patients with a combination of questionnaires and audiometry. (11,18) Subsequently, patients who require further evaluation of potential hearing loss can be identified and directed to treatment.

Despite all these resources, only 40% of our respondents use some type of health-directed questionnaire and audiometric assessment (table 2). Responses showed that certain barriers prevented PCPs from inquiring about hearing loss, although knowledge of the adverse impact of hearing impairment on patients' lives was evident among respondents and most acknowledged that hearing loss affects their patients' quality of life. Respondents expressed that the practical aspects of screening patients deterred them from assessing patients' hearing. Of the respondents who do not evaluate patients for hearing loss, almost 40.0% stated that time constraints and other health issues prevented them from screening patients (table 1). Additionally, despite the availability of effective screening tools, one-fourth of respondents were not sure which method to use. To increase the frequency of primary care screening, programs explaining how to use the audioscope and discussing how to quickly identify patients requiring referral with questionnaires are still needed.

Because many patients either do not discuss their hearing loss or do not accept it, active screening is essential. A study of 2,304 hearing-impaired adults aged 50 and older found that although patients not wearing hearing aids are more likely to experience depression, anxiety, paranoia, and emotional problems compared with hearing aid users, many patients do not use hearing aids. (19) Denial about needing hearing aids, believing aids would not help, and the cost and stigma of wearing hearing aids prevented patients from inquiring about them. (19) Wilson et al also found that 55% of men admitted to some hearing loss, but only 12% sought help. (20) Hence, some patients suffer unnecessarily with hearing impairment until they discuss the problem. Otolaryngologists must educate PCPs about the necessity of searching for hearing loss, counseling patients about its adverse consequences, and directing them to care.

Furthermore, teaching PCPs about treatable causes of hearing impairment can bring further benefit to patients. For example, routine physical examinations can uncover cerumen impaction. Sudden hearing loss, unilateral hearing loss, tympanic membrane perforations, and cholesteatoma may be recognized and referred to otolaryngologists for treatment. (11,12) Additionally, patients with hearing aids need to be reassessed. Poorly fitting hearing aids, dead batteries, and poor patient dexterity are correctable causes of poor outcomes with hearing aids. (12) However, among our respondents, 12.9% make no recommendations to patients who report problems with their hearing aids and do not refer them back to audiologists or otolaryngologists. Discussing the limitations and benefits of hearing aids and asking about their effectiveness may help to reduce the social stigma of wearing an aid, minimize potential frustration, and identify patients with suboptimal improvement for reevaluation.

The need for increased education and outreach is most evident with respect to cochlear implantation. Almost 90% of our respondents were aware of cochlear implants, but only one-fourth referred their deaf patients for evaluation. More than half of respondents who were not aware of cochlear implants practiced in towns that had an academic center, as well as a local audiologist and otolaryngologist. PCPs need to know that options exist for deaf patients and others who do not benefit from conventional hearing aids. Otherwise, a segment of the hearing-impaired community may continue to suffer. Otolaryngologists need to actively teach PCPs about cochlear implants. Most respondents said they did not refer patients for cochlear implants because they did not know which patients were candidates or where to send patients (table 4). Surprisingly, fears about surgical risks and expenses were not common barriers to referral.

Through community lectures and educational programs, both patients and PCPs could be exposed to advances in hearing aids and cochlear implants, identification of likely candidates, and the resources available. However, 17.6% of our physicians did not have access to an otolaryngologist, and 32.9% did not have an audiologist in their community. Continued outreach from otolaryngologists and audiologists is essential to maximize the treatment of hearing loss. Furthermore, residents in primary care specialties could rotate through otolaryngology to increase their comfort level in evaluating hearing impairment. Using a team approach, otolaryngolgists, audiologists, and PCPs must take the initiative in managing this widespread source of physical, social, and psychological dysfunction.

A few points regarding study design are relevant. First, certain questions might have been misinterpreted, and the answer choices provided might have influenced the responses given. Respondents might have been unwilling to admit that they did not evaluate hearing loss or that they were not aware of cochlear implants, underestimating these results. Because only one-fourth of respondents practiced in towns with more than 500,000 people, this study may not adequately represent PCPs in larger cities. Similarly, the results might have been different if more than 40.0% of respondents had academic centers in their communities.

Finally, our response rate (32.7%) reduces our ability to draw firm generalizations about PCPs' screening for hearing loss. Because data from nonresponders could not be collected, a sensitivity analysis comparing responders and nonresponders is not possible. Therefore, the extent to which responders were similar to nonresponders is not known. Attempts were made to examine a representative group within the primary care community. A random sample of PCPs was selected, and questionnaires were sent three times to maximize the response rate. Despite its limitations, this study provides insights about how PCPs address hearing loss among the elderly and the barriers that exist in referring patients for intervention.


The potential exists to improve the means of evaluating adults, especially elderly patients, for hearing loss. As the primary patient advocates, PCPs must play an essential role in identifying patients with hearing loss and referring them for intervention. However, otolaryngologists and audiologists need to advocate for hearing-impaired patients and educate PCPs about the continually improving technology designed for auditory rehabilitation. Furthermore, screening techniques, basic disease entities, and therapeutic options should be part of the curriculum for residents in family medicine. Exciting advances in the field of hearing aids and cochlear implants bring increased advantages for patients. Future efforts should focus on developing screening programs, determining their effectiveness, and studying patient benefit from continually improving hearing aids and cochlear implants.


(1.) Hertzel L, Smith A. The 65 years and over population: 2000. Washington, D.C.: U.S. Census Bureau, 2001.

(2.) Cruickshanks KJ, Wiley TL, Tweed TS, et al. Prevalence of hearing loss in older adults in Beaver Dam, Wisconsin. The Epidemiology of Hearing Loss Study. Am J Epidemiol 1998;148:879-86.

(3.) U.S. Department of Commerce. Statistical Abstract of the United States. 117th ed. Washington, D.C.: U.S. Census Bureau, 1997.

(4.) Reuben DB, Walsh K, Moore AA, et al. Hearing loss in community-dwelling older persons: National prevalence data and identification using simple questions. J Am Geriatr Soc 1998;46:1008-11.

(5.) Bess FH, Lichtenstein MJ, Logan SA, et al. Hearing impairment as a determinant of function in the elderly. J Am Geriatr Soc 1989;37: 123-8.

(6.) Thomas AJ. Acquired deafness and mental health. Br J Med Psychol 1981;54:219-29.

(7.) Seherer MJ, Frisina DR. Characteristics associated with marginal hearing loss and subjective well-being among a sample of older adults. J Rehabil Res Dev 1998;35:420-6.

(8.) Mulrow CD, Aguilar C, Endicott JE, et al. Quality-of-life changes and hearing impairment. A randomized trial. Ann Intern Med 1990;113: 188-94.

(9.) Labadie RF, Carrasco VN, Gilmer CH, Pillsbury HC III. Cochlear implant performance in senior citizens. Otolaryngol Head Neck Surg 2000;123:419-24.

(10.) Maillet CJ, Tyler RS, Jordan HN. Change in the quality of life of adult cochlear implant patients. Ann Otol Rhinol Laryngol Suppl 1995;165:31-48.

(11.) Yueh B, Shapiro N, MacLean CH, Shekelle PG. Screening and management of adult hearing loss in primary care: Scientific review. JAMA 2003;289:1976-85.

(12.) Bogardus ST, Jr., Yueh B, Shekelle PG. Screening and management of adult hearing loss in primary care: Clinical applications. JAMA 2003;289:1986-90.

(13.) American Academy of Family Physicians. Summary of Policy Recommendations for Periodic Health Examinations. Available at: Accessed October 18, 2003.

(14.) Logan SA, Ahlstrom JB, Bess FH. Identification and referral of hearing impaired elderly by primary care physicians. Presented at: American Speech-Language-Hearing Association Convention: November 21-23, 1985; Washington. D.C.

(15). Bess FH, Logan SA, Lichtenstein M J, et al. Early identification and referral of hearing impaired elderly. In: Robinette MS, Buach CD, eds. Proceedings of a Symposium in Audiology. Rochester, Minn.: Mayo Clinic/Mayo Foundation, 1987;1-27.

(16). Lichtenstein MJ, Bess FH, Logan SA. Validation of screening tools for identifying hearing-impaired elderly in primary care. JAMA 1988;259:2875-8.

(17). Koike KJ, Hurst MK, Wetmore SJ. Correlation between the American Academy of Otolaryngology Head and Neck Surgery five-minute hearing test and standard audiologic data. Otolaryngol Head Neck Surg 1994;111:625-32.

(18.) Beers MH, Fink A, Beck JC. Screening recommendations for the elderly. Am J Public Health 1991;81:1131-40.

(19.) National Council on the Aging. The Consequences of Untreated Hearing Loss in Older Persons. Washington D.C., 1999.

(20.) Wilson PS, Fleming DM, Donaldson I. Prevalence of hearing loss among people aged 65 years and over: Screening and hearing aid provision. Br J Gen Pract 1993;43:406-9.

From the Department of Otolaryngology, Vanderbilt University Medical Center, Nashville.

Reprint requests: Robert F. Labadie, MD, PhD, Vanderbilt University Medical Center, Department of Otolaryngology, S-2100 Medical Center North. Nashville, TN 37232-2559. Phone: (615) 343-6972: fax: (615) 343-7604; e-mail:

Originally presented at the Southern Section Meeting of the Triological Society; January 9-11, 2003; Naples, Fla.

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