Hipoacusia en Adulto

The n e w e ng l a n d j o u r na l of m e dic i n e
Review Article
Allan H. Ropper, M.D., Editor
Hearing Loss in Adults

Lisa L. Cunningham, Ph.D., and Debara L. Tucci, M.D., M.B.A.
H
earing loss in adults is encountered in all medical settings From the Section on Sensory Cell Biology,
and frequently influences medical encounters. This disorder constitutes a National Institute on Deafness and Other
Communication Disorders, Bethesda, MD
substantial burden on the adult population in the United States, yet (L.L.C.); and the Division of Head and
screening for hearing loss is not routine,1 and treatments are often inaccessible Neck Surgery and Communication Sci-
because of the high cost or perceived ineffectiveness. ences, Duke University Medical Center,
Durham, NC (D.L.T.). Address reprint re-
quests to Dr. Tucci at the Division of
Head and Neck Surgery and Communi-
Bur den of He a r ing L oss cation Sciences, Box 3805, Duke Univer-
sity Medical Center, Durham, NC 27710,
The Global Burden of Disease Study2 measured years lived with disability and or at debara.tucci@duke.edu.
found that hearing loss is the fourth leading cause of disability globally. In the
N Engl J Med 2017;377:2465-73.
United States, the prevalence of hearing loss doubles with every 10-year increase DOI: 10.1056/NEJMra1616601
in age. Approximately half of persons in their seventh decade (60 to 69 years of Copyright © 2017 Massachusetts Medical Society.
age)3 and 80% who are 85 years of age or older4 have hearing loss that is severe
enough to affect daily communication. Because of the aging population in this and
other developed countries, hearing loss is likely to become an increasingly preva-
lent disability.
The primary effect of adult hearing loss is impaired communication, which can
adversely affect relationships with family and friends and create difficulties in the
workplace. Untreated hearing loss in adults also has indirect health, psychosocial,
and economic effects and leads to social isolation and a reduced quality of life.5-7
As compared with age-matched adults with unimpaired hearing, older persons
with hearing loss have higher rates of hospitalization,8 death,9,10 and falls and
frailty,11,12 as well as higher rates of dementia13-15 and depression,16,17 even when
known risks for these disorders are taken into account.13 On a societal level, be-
cause of their hearing loss, persons with hearing loss achieve significantly lower
levels of education than do those with normal hearing; they also have higher
levels of unemployment or underemployment and lower levels of income than
those with normal hearing.18,19 Annual health care costs for middle-age U.S. adults
with hearing loss are significantly higher than the costs of care for those without
hearing loss.19
T y pe s of He a r ing L oss
Peripheral hearing loss is typically categorized as conductive (caused by impair-
ment of the outer or middle ear) or sensorineural (caused by dysfunction in the
cochlea or spiral ganglion). Hearing loss that has both conductive and sensorineu-
ral components is categorized as mixed. Conductive hearing loss results from
obstruction or disease of the outer or middle ear that prevents transmission of
sound energy to the inner ear. The causes of conductive hearing loss range from
cerumen impaction and otitis media to fixation of one or more of the middle-ear
n engl j med 377;25 nejm.org December 21, 2017 2465

The New England Journal of Medicine
Downloaded from nejm.org at DURHAM UNIVERSITY on December 20, 2017. For personal use only. No other uses without permission.
Copyright © 2017 Massachusetts Medical Society. All rights reserved.
bones, mainly fixation of the stapes due to oto- Figure 1 (facing page). Diseases Affecting the Auditory
sclerosis. Medical or surgical treatment of most System.
types of conductive hearing loss often results in Shown are the auditory system and diseases affecting
full restoration of hearing. each component of the system, including the ossicular
chain of the middle ear, the cochlea, the organ of Corti
and hair cells, and the auditory nerve.
A sse ssmen t of He a r ing
Audiologic testing is performed to assess hear-
ing thresholds across the range of frequencies mammalian cochlea are not regenerated after
that are important for human communication. they are lost, the resulting hearing loss is per-
Auditory thresholds are typically measured for manent. In many cases, the death of hair cells
air- and bone-conducted pure-tone stimuli in results in degeneration of spiral ganglion neu-
order to differentiate conductive from sensori- rons, which complicates the treatment of hear-
neural hearing loss and to characterize the pat- ing loss with cochlear implants because these
tern of hearing loss at various frequencies. The devices directly stimulate the spiral ganglion
term “threshold shift” refers to a change in hear- neurons.
ing thresholds between sequential audiologic Sensory hearing loss is the result of damage
tests; it may reflect improvement or worsening to the organ of Corti (the sensory organ within
of hearing. Testing the perception of speech the cochlea that houses the hair cells) or the
signals of low redundancy (monosyllabic words) stria vascularis, a portion of the inner ear that
presented at a comfortable listening level in the provides metabolic support for the organ of
absence of background noise is another method Corti and generates the electrochemical (endo-
of assessing hearing in adults. cochlear) potential that is the driving force for
The broad term “sensorineural hearing loss” transduction of sound by sensory hair cells.20 In
has been used by clinicians because, until re- contrast, neural hearing loss is the result of loss
cently, diagnostic tests could not determine or dysfunction of spiral ganglion neurons or of
whether a lesion was in the sensory or the neural more proximal auditory structures. Auditory
portion of the peripheral auditory system. This neuropathy is characterized by normal (or near-
distinction is now made by measuring otoacous- normal) sensory hair-cell function coupled with
tic emissions, performed by simultaneously pre- abnormal neural responses and, usually, poorer
senting tones of different frequencies and sound word recognition than is typical in sensory hear-
pressures to the external canal and detecting ing loss18,21; this type of hearing loss is caused
sound emissions from the cochlea itself; the re- by damage to the synapses that connect hair
sults reflect the functioning of outer hair cells of cells to spiral ganglion neurons or to asynchro-
the cochlea. Auditory brain-stem responses also nous neural firing within the auditory nerve. For
test the neural component of audition by record- this reason, patients with auditory neuropathy
ing synchronous neural activity of the auditory may benefit less from hearing aids than those
nerve and auditory brain-stem nuclei. with sensory hearing loss.
S t ruc t ur e s of the E a r In volv ed C ause s of He a r ing L oss

in He a r ing L oss
Hearing loss can be caused by damage to any
The peripheral auditory system consists of the portion of the peripheral and central auditory
outer ear, the middle ear, and the inner ear systems. The main causes of sensorineural hear-
(cochlea); the inner ear contains the mechano- ing loss are degenerative processes associated
sensory hair cells that convert sound energy into with aging, genetic mutations, noise exposure,
neural signals (Fig. 1). Cochlear hair cells are exposure to therapeutic drugs that have ototoxic
innervated by neurons of the spiral ganglion, side effects, and chronic conditions.
which project centrally to the auditory nuclei of
the brain stem through the auditory nerve. Sen- Age-Related Degenerative Processes
sory hair cells are susceptible to damage from a The leading cause of adult-onset hearing loss is
variety of stresses, and since hair cells in the the effects of aging on the auditory system.
2466 n engl j med 377;25 nejm.org December 21, 2017

Organs affected by disorders causing conductive hearing loss

BRAIN
Organs affected by disorders causing sensorineural hearing loss Auditory Region
Dysfunction or damage
Outer Ear
Outer Ear
Damage or abnormality
BRAIN
Middle Ear Inner Ear
Semicircular
Ossicles canals
Pinna
Damage or fixation
Tympanic Auditory Nerve

membrane Asynchronous firing
Damage or loss
BONE
Cochlea
Auditory Canal Middle Ear Damage from noise, aging,
Cerumen impaction Otitis media genetic abnormalities, or
ototoxic drugs
SCALA VESTIBULI Stria vascularis

Damage from aging,
vascular abnormalities,
Synapses or ototoxic drugs
Damage from noise or
from possible loss SCALA MEDIA Tectorial
due to hair-cell death membrane
Inner
hair cell Stereocilia
Outer
Spiral ganglion hair cell
Degeneration from primary or Organ of Corti
secondary causes
Hair cell
Damage from loud noises,
ototoxic drugs, aging, or
genetic abnormalities
SCALA TYMPANI

Hearing loss in older persons is caused not only by among people who report that their hearing is
the degenerative effects of aging on the cochlea “excellent” or “good,” nearly 20% have audio-
but also by the accumulated effects of exposure metric evidence of noise-induced hearing loss.32
to noise and ototoxic drugs. Age-related hearing Workplaces such as factories and certain jobs in
loss (presbycusis) is usually bilateral and sym- the military are associated with exposure to high
metric and is most pronounced at higher fre- noise levels; however, even people who do not
quencies (≥2000 Hz).19 A prominent feature of work in these environments have a risk of noise
this type of hearing loss is reduction in the abil- exposure in daily life that they often underesti-
ity to understand speech,22,23 even if the sounds mate. Loud sounds are pervasive in modern life,
are loud enough. Sensory presbycusis refers to and noise exposure can occur in a variety of
death or damage of the cochlear sensory hair seemingly innocuous settings, such as concerts,
cells with aging, and metabolic presbycusis re- movie theaters, and fitness classes with loud
fers to decreased functioning of the stria vascu- music, and through engagement in a range of
laris through age-related mechanisms that have activities, such as listening to music at home,
not been fully determined.24,25 participating in power sports (e.g., those involv-
ing motorcycles, all-terrain vehicles, speed boats,
Genetic Mutations or snowmobiles), shooting, and using power
More than 100 genes have known mutations tools.
that result in hearing loss that is not associated Noise damages the sensory hair cells of the
with disorders of other organs or with dysmor- inner ear through the direct mechanical stress
phic features (nonsyndromic hearing loss). Mu- of intense sound pressure and by activation of
tations in approximately 30 of these genes are stress-induced molecular pathways, including
associated with adult-onset or progressive hearing generation of reactive oxygen species and cal-
loss that is inherited as an autosomal dominant cium overload.33 Noise-induced hearing loss can
trait.26 In addition, more than 500 syndromes be temporary or permanent, depending on the
that include hearing loss have been described. intensity and duration of exposure. The term
Hereditary hearing loss is relatively common “temporary threshold shift” is used to describe
among newborns, affecting approximately 1 in objective changes in hearing acuity that can be
1000 live births,27 but it is difficult to estimate measured on audiometry immediately after an epi-
the heritability of adult-onset hearing loss, since sode of exposure to loud sounds (e.g., attendance
genetic and environmental factors are not easily at a concert) and that revert to preexposure levels
separable. Estimates of the heritability of adult- after a few days to 2 weeks. Temporary thresh-
onset hearing loss range from 25 to 55%.28 The old shifts are characterized subjectively by de-
majority of monogenetic causes of hearing loss creased hearing sensitivity, a feeling of fullness
involve mutations in genes that are required for in the ears, tinnitus (ringing), and a perception
normal functioning of the cochlea, and several that sounds are muffled. Prolonged or repeated
of these genes specifically affect the functioning exposure to noise can cause the death of sen-
of sensory hair cells.29 In addition, the genetics of sory hair cells and permanent hearing loss, re-
susceptibility to age-related and noise-induced ferred to as a “permanent threshold shift.” Death
hearing loss are beginning to be understood.30 of hair cells can be followed by a slower loss of
The identification of genetic modifiers that en- spiral ganglion neurons over a period of months
hance or reduce susceptibility to acquired hear- or years.34
ing loss will be important for the development In the past, temporary threshold shifts were
of therapies to preserve hearing. not thought to be associated with permanent
damage to the auditory system; however, recent
Noise Exposure data indicate that noise exposures that result in
Approximately 104 million people in the United temporary threshold shifts may cause permanent
States are exposed to levels of noise that can damage to the cochlea. In animal models, noise
cause hearing loss,31 and 1 in 4 adults in the exposures that result in temporary threshold
United States has measurable hearing loss shifts lead to permanent loss of hair-cell ribbon
caused by exposure to harmful noise.32 Even synapses,34,35 specialized synaptic structures that

release neurotransmitters from hair cells in re- of data from the National Health and Nutrition
sponse to sound. Although our understanding of Examination Survey for the years 1999 through
this type of synaptic loss is incomplete, it has 2004, a study of patients with diabetes who were
implications for hearing function and for recom- 20 to 69 years of age showed that low-density
mendations regarding safe noise levels.36 It is not and high-density lipoprotein levels, as well as
known whether synaptic loss is necessarily per- status with respect to coronary disease, periph-
manent, since in some animal models, there is eral neuropathy, and general health, were cor-
partial recovery of the functioning of these related with hearing impairment, whereas glyce-
structures.37,38 A reliable, noninvasive clinical mic control, number of years since diagnosis,
measure of cochlear synaptic damage has not and type of hypoglycemic medication were not.50
been developed, although this is an area of active These putative associations with chronic systemic
research.39-41 diseases suggest that some contributors to hear-
ing loss may be modifiable.
Exposure to Therapeutic Drugs Autoimmune forms of ear disease are charac-
Various chemicals and drugs adversely affect the terized by progressive, fluctuating hearing loss
auditory system; the main ones in clinical use with a variable time course. Although typically
are aminoglycoside antibiotics and cisplatin, both bilateral, symptoms may be more prominent in
of which are toxic to sensory hair cells. Hearing one ear, and one possible cause of sudden, uni-
loss develops in approximately 20% of patients lateral sensorineural hearing loss is an autoim-
receiving aminoglycosides,42,43 and the prevalence mune disorder. Ear disease may be associated
is as high as 56% among patients with cystic with rheumatoid arthritis, systemic lupus erythe-
fibrosis,44,45 a population exposed to repeated matosus, Cogan’s syndrome, sarcoidosis, or other
courses of aminoglycoside therapy. Among adults autoimmune disorders.51 Glucocorticoids are the
who have received cisplatin, clinically significant main treatment and are often used for long pe-
hearing loss develops in approximately 60% of riods. The complications of this prolonged treat-
patients with testicular cancer46 and 65% of pa- ment led to a trial in which prednisone and
tients with head and neck cancer.47 Susceptibility methotrexate were compared with prednisone
to cisplatin-induced hearing loss depends on the and placebo; the trial failed to show a benefit
cumulative dose of the drug, the age of the pa- from the addition of methotrexate.52
tient (children are more susceptible than adults),
and status with respect to concurrent cranial Sudden Hearing Loss
irradiation.48 Patients who have severe hearing The prevalence of sudden, idiopathic hearing loss
loss caused by ototoxic drugs are likely to be is 5 to 20 cases per 100,000 population, with
identified and referred for follow-up auditory approximately 4000 new cases per year in the
testing, but many more patients with mild-to- United States. Sudden sensorineural hearing loss,
moderate drug-induced hearing loss are not iden- defined as the onset of hearing loss over a period
tified and hence do not receive treatment for of 72 hours or less in one or both ears, is con-
their hearing loss.46 sidered an otologic emergency because of evi-
dence, albeit uncertain, that there is a benefit of
Smoking, Adiposity, and Chronic Diseases early treatment with glucocorticoids. The audio-
Strong associations between hearing loss and metric criterion for diagnosis is a decrease in
cigarette smoking, adiposity, diabetes mellitus, hearing of at least 30 dB, affecting at least three
and other risk factors for cardiovascular disease sound frequencies. The cause of sudden hearing
are supported by epidemiologic studies, but cau- loss is not known but is presumed to be viral,
sality remains uncertain. For example, in the vascular, or autoimmune. The diagnostic work-
Beaver Dam Eye Study, involving persons between up involves testing to exclude identifiable causes
the ages of 43 and 84 years, smoking, central of sudden hearing loss, such as acoustic neuroma
adiposity, and poorly controlled diabetes melli- and rare tumors of the internal auditory canal.
tus were associated with hearing loss in later A clinical practice guideline from the Ameri-
life, suggesting that vascular changes may con- can Academy of Otolaryngology–Head and Neck
tribute to age-related hearing loss.49 On the basis Surgery53 recommends that physicians evaluate

patients presenting with a sudden onset of hear- identify hearing loss, and they can play a role in
ing loss in order to distinguish between sensori- referring patients for hearing evaluation and
neural and conductive impairment, initially with management.56
a physical examination and tuning-fork tests,
followed by audiometry when possible. Although T r e atmen t of Sensor ineur a l
high doses of oral glucocorticoids (typical dose, He a r ing L oss
1 mg per kilogram of body weight) and of intra-
tympanic glucocorticoids have been used in the Despite increasing knowledge of the biology of
treatment of sudden, idiopathic hearing loss, the inner ear, hearing restoration is not cur-
spontaneous recovery in many patients and the rently available for most cases of hearing loss.57
lack of data from placebo-controlled trials make A review of novel therapies included 22 active
these treatment recommendations tentative. Never- clinical drug trials registered on the National
theless, most otolaryngologists recommend some Institutes of Health ClinicalTrials.gov website.58
form of glucocorticoid treatment for sudden Most of these proposed treatments address
hearing loss.53 cell-death pathways and mitigate the effects of
oxidative stressors on inner-ear hair cells. Com-
mercially sponsored trials of antibiotic and
Pr e v en t ion of He a r ing L oss
chemotherapeutic medications for the treatment
An epidemiologic study54 has shown that the or prevention of ototoxicity are planned or are
age-adjusted prevalence of hearing loss is declin- under way (e.g., ClinicalTrials.gov number,
ing in the United States. Possible explanations NCT02819856). One study uses a viral vector to
for this decrease include a reduction in exposure deliver gene therapy to the inner ear.59,60 Pre-
to occupational noise as a result of fewer manu- clinical studies in animal models suggest that
facturing jobs and more widespread use of hear- viral-vector gene therapy may be valuable in
ing protection, cessation of smoking, and better treating monogenetic hereditary hearing loss.
management of cardiovascular risk factors. Al- Restoration or partial restoration of hearing and
though the prevalence of hearing loss increases balance functions have been achieved with the
with age, it is not inevitable. Adults should be use of gene therapy in mouse models of human
made aware of the potentially damaging and deafness, including models of some forms of the
slowly cumulative effects of exposure to loud Usher syndrome.61-66
sounds such as the common sources of exposure
noted above. Hearing protection includes using Hearing Aids and Other Devices
ear protectors (earmuffs or earplugs), avoiding A hearing aid is defined by the Food and Drug
or limiting time spent in loud venues, and using Administration (FDA) as a “wearable sound-
personal music systems at moderate volumes, amplifying device that is intended to compen-
and wearing noise-canceling headphones or ear- sate for impaired hearing.” The goal of treat-
phones. ment with well-fitted hearing aids is to improve
Despite the high prevalence of hearing loss the audibility of even soft speech or music and
among older adults, routine auditory screening other sounds while ensuring that sounds do not
is not currently recommended for asymptomatic become uncomfortably loud. Hearing aids can be
persons. A review of data on primary care screen- sophisticated instruments with a variety of cus-
ing for hearing loss, performed for the U.S. tomizable features that contribute to their high
Preventive Services Task Force,1 concluded that costs; whether performance improves with higher-
additional research is needed to understand the cost devices is uncertain.67 A variety of noncus-
potential effects of such screening on health out- tomized devices, termed “hearing assistive tech-
comes but acknowledged that common screening nologies,” are also available; they include
tests, such as validated hearing-loss question- amplified telephones, visual technologies such
naires and the whisper, finger-rub, or watch-tick as captioning, video conferencing, and visual or
test, or screening audiometry55 can identify vibrotactile alerts.68 Hearing aids are regulated
patients at risk for hearing loss. Primary care by the FDA, and state laws may restrict access to
physicians are often the first to suspect and them. The devices are sold through audiologists

and hearing-aid dispensers. The costs of services these assessments have shown that cochlear im-
related to hearing-aid fitting are often bundled plants compare favorably with other treatments.
with the cost of the device, so the specific costs
of the services and the technology are not trans- Incr e a sing Ac ce ss t o He a r ing
parent.68 A ids
The frequency of use of hearing aids by adults
with hearing loss is low.68 In a survey published Hearing loss is increasingly being viewed as a
in 2012, only 14.2% of adults with hearing loss public health problem.68 In October 2015, the
reported wearing hearing aids.69 Although the President’s Council of Advisors on Science and
cost of the devices, typically $1,400 to $2,200, is Technology recommended that the FDA create a
probably a factor, other deterrents to the adop- new regulatory class for hearing aids that can be
tion of hearing aids include stigma, perceived sold over the counter for persons with mild or
ineffectiveness, ongoing costs (for batteries and moderate hearing loss.78 This recommendation
maintenance), lack of comfort, and cosmetic ap- was endorsed by the National Academies of Sci-
pearance.68 The United States is one of the few ences, Engineering, and Medicine in their report
developed countries that does not offer govern- titled “Hearing Health Care for Adults: Priorities
ment assistance for the purchase of hearing de- for Improving Access and Affordability,” released
vices.68 However, even in countries where assis- in June 2016.68 They recommended that the FDA
tance is provided, hearing-aid use is not universal create a category of over-the-counter, wearable
among candidates; for example, the rate of use hearing devices that would be regulated to meet
is less than 15% in Finland and is 50% in Den- specific safety and quality standards and label-
mark.68 Efforts to increase use will therefore ing specifications; the new FDA classification
have to address multiple impediments. would preempt current state laws and regula-
tions in order not to limit access to affordable
Cochlear Implants hearing aids. Legislation has recently been signed
Persons with severe or total sensorineural hear- into law that requires the FDA to create and
ing loss do not typically benefit from hearing regulate a category of over-the-counter hearing
aids, since inner-ear hair cells are not able to aids for adults who have mild to moderate hear-
stimulate the auditory nerve in response to sound. ing loss.79,80 Opening the market to these devices
In such cases, cochlear implants, which are sur- should increase the options available to patients,
gically implanted devices that bypass the cochlear decrease costs, and increase access.81 Bulk pur-
hair cells to electrically stimulate the auditory chasing by government agencies provides another
nerve, permit partial restoration of hearing and opportunity to decrease costs. The Department
have been shown to improve speech perception of Veterans Affairs, for example, purchased ap-
and vocational, social, and psychological func- proximately 20% of hearing aids on the U.S.
tioning,70-72 as well as the quality of life for market in 2013,82 at an average cost of $369 per
adults, including older adults.73 Details of the hearing aid as compared with $1,400 to $2,200
function and use of cochlear implants can be on the open market.68
found at https://www.nidcd.nih.gov/health/cochlear
-implants. C onclusions
The cost-effectiveness of cochlear implants
has been established in developed countries71 Hearing loss is a major source of disability in
and in some developing countries.71,74-77 These adults, associated with serious communication
analyses have evaluated the lifetime costs of and psychosocial problems and high health care
cochlear implants, including the costs of the costs, with economic implications at the societal
device and of surgery and rehabilitation, versus and individual levels. Technologies exist to ame-
the benefits, as determined on the basis of liorate hearing loss, but cost, health policies, and
health-preference measures. Measures of cost- regulations limit access to these therapies. Efforts
effectiveness have also been used to compare are under way to improve access to auditory
cochlear implants with other treatments, such health care for adults. Recent advances in our
as educational programs for deaf persons, and understanding of the underlying causes of hear-

ing loss have led to efforts to develop drugs and and consulting fees from Otonomy and serving as chair of a data
and safety monitoring board for Otonomy. No other potential
therapies that can prevent or reverse hearing loss. conflict of interest relevant to this article was reported.
Dr. Tucci reports receiving consulting fees and travel support Disclosure forms provided by the authors are available with
from Roche Pharmaceuticals, consulting fees from Otic Pharma, the full text of this article at NEJM.org.
References
1. Chou R, Dana T, Bougatsos C, Flem- 13. Lin FR, Metter EJ, O’Brien RJ, Resnick and neck surgery. 18th ed. Shelton, CT:
ing C, Beil T. Screening for hearing loss in SM, Zonderman AB, Ferrucci L. Hearing People’s Medical Publishing House, 2017:
adults ages 50 years and older:a review of loss and incident dementia. Arch Neurol 329-45.
the evidence for the U.S. Preventive Ser- 2011;68:214-20. 27. Morton NE. Genetic epidemiology of
vices Task Force. Rockville, MD:Agency 14. Lin FR, Yaffe K, Xia J, et al. Hearing hearing impairment. Ann N Y Acad Sci
for Health Policy and Research, 2011. loss and cognitive decline in older adults. 1991;630:16-31.
2. GBD 2015 Disease and Injury Inci- JAMA Intern Med 2013;173:293-9. 28. Christensen K, Frederiksen H, Hoff-
dence and Prevalence Collaborators. 15. Gallacher J, Ilubaera V, Ben-Shlomo Y, man HJ. Genetic and environmental in-
Global, regional, and national incidence, et al. Auditory threshold, phonologic de- fluences on self-reported reduced hearing
prevalence, and years lived with disability mand, and incident dementia. Neurology in the old and oldest old. J Am Geriatr Soc
for 310 diseases and injuries, 1990-2015: 2012;79:1583-90. 2001;49:1512-7.
a systematic analysis for the Global Bur- 16. Li CM, Zhang X, Hoffman HJ, Cotch 29. Richardson GP, de Monvel JB, Petit C.
den of Disease Study 2015. Lancet 2016; MF, Themann CL, Wilson MR. Hearing How the genetics of deafness illuminates
388:1545-602. impairment associated with depression in auditory physiology. Annu Rev Physiol
3. Agrawal Y, Platz EA, Niparko JK. Prev- US adults, National Health and Nutrition 2011;73:311-34.
alence of hearing loss and differences by Examination Survey 2005-2010. JAMA 30. Friedman RA, Van Laer L, Huentel-
demographic characteristics among US Otolaryngol Head Neck Surg 2014;140: man MJ, et al. GRM7 variants confer sus-
adults: data from the National Health and 293-302. ceptibility to age-related hearing impair-
Nutrition Examination Survey, 1999-2004. 17. Mener DJ, Betz J, Genther DJ, Chen D, ment. Hum Mol Genet 2009;18:785-96.
Arch Intern Med 2008;168:1522-30. Lin FR. Hearing loss and depression in 31. Hammer MS, Swinburn TK, Neitzel
4. Lin FR, Thorpe R, Gordon-Salant S, older adults. J Am Geriatr Soc 2013;61: RL. Environmental noise pollution in the
Ferrucci L. Hearing loss prevalence and 1627-9. United States: developing an effective
risk factors among older adults in the 18. Starr A, Rance G. Auditory neuropa- public health response. Environ Health
United States. J Gerontol A Biol Sci Med thy. Handb Clin Neurol 2015;129:495-508. Perspect 2014;122:115-9.
Sci 2011;66:582-90. 19. Allen PD, Eddins DA. Presbycusis 32. Carroll YI, Eichwald J, Scinicariello F,
5. Bainbridge KE, Wallhagen MI. Hear- phenotypes form a heterogeneous con- et al. Vital signs: noise-induced hearing
ing loss in an aging American population: tinuum when ordered by degree and con- loss among adults — United States 2011–
extent, impact, and management. Annu figuration of hearing loss. Hear Res 2010; 2012. MMWR Morb Mortal Wkly Rep
Rev Public Health 2014;35:139-52. 264:10-20. 2017;66:139-44.
6. Kamil RJ, Lin FR. The effects of hear- 20. Wangemann P. Supporting sensory 33. Sha SH, Schacht J. Emerging thera-
ing impairment in older adults on com- transduction: cochlear fluid homeostasis peutic interventions against noise-induced
munication partners: a systematic review. and the endocochlear potential. J Physiol hearing loss. Expert Opin Investig Drugs
J Am Acad Audiol 2015;26:155-82. 2006;576:11-21. 2017;26:85-96.
7. Mick P, Kawachi I, Lin FR. The asso- 21. Hood LJ. Auditory neuropathy/dys- 34. Kujawa SG, Liberman MC. Accelera-
ciation between hearing loss and social synchrony disorder: diagnosis and man- tion of age-related hearing loss by early
isolation in older adults. Otolaryngol agement. Otolaryngol Clin North Am noise exposure: evidence of a misspent
Head Neck Surg 2014;150:378-84. 2015;48:1027-40. youth. J Neurosci 2006;26:2115-23.
8. Genther DJ, Frick KD, Chen D, Betz J, 22. van Rooij JC, Plomp R. Auditive and 35. Kujawa SG, Liberman MC. Adding in-
Lin FR. Association of hearing loss with cognitive factors in speech perception by sult to injury: cochlear nerve degeneration
hospitalization and burden of disease in elderly listeners. III. Additional data and after “temporary” noise-induced hearing
older adults. JAMA 2013;309:2322-4. final discussion. J Acoust Soc Am 1992; loss. J Neurosci 2009;29:14077-85.
9. Contrera KJ, Betz J, Genther DJ, Lin 91:1028-33. 36. Liberman MC, Kujawa SG. Cochlear
FR. Association of hearing impairment 23. Dubno JR, Lee FS, Matthews LJ, Ahl- synaptopathy in acquired sensorineural
and mortality in the National Health and strom JB, Horwitz AR, Mills JH. Longitu- hearing loss: manifestations and mecha-
Nutrition Examination Survey. JAMA Oto- dinal changes in speech recognition in nisms. Hear Res 2017;349:138-47.
laryngol Head Neck Surg 2015;141:944-6. older persons. J Acoust Soc Am 2008;123: 37. Liu L, Wang H, Shi L, et al. Silent
10. Fisher D, Li CM, Chiu MS, et al. Im- 462-75. damage of noise on cochlear afferent in-
pairments in hearing and vision impact 24. Dubno JR, Eckert MA, Lee FS, Mat- nervation in guinea pigs and the impact
on mortality in older people: the AGES- thews LJ, Schmiedt RA. Classifying human on temporal processing. PLoS One 2012;
Reykjavik Study. Age Ageing 2014;43:69- audiometric phenotypes of age-related 7(11):e49550.
76. hearing loss from animal models. J Assoc 38. Shi LJ, Chang Y, Li XW, Aiken SJ, Liu
11. Lin FR, Ferrucci L. Hearing loss and Res Otolaryngol 2013;14:687-701. LJ, Wang J. Coding deficits in noise-
falls among older adults in the United 25. Schuknecht HF, Gacek MR. Cochlear induced hidden hearing loss may stem
States. Arch Intern Med 2012;172:369-71. pathology in presbycusis. Ann Otol Rhi- from incomplete repair of ribbon synaps-
12. Viljanen A, Kaprio J, Pyykkö I, et al. nol Laryngol 1993;102:1-16. es in the cochlea. Front Neurosci 2016;10:
Hearing as a predictor of falls and postural 26. Griffith AJ, Friedman TB. Hereditary 231.
balance in older female twins. J Gerontol A hearing loss. In:Wackym PA, Snow JB Jr, 39. Liberman MC, Epstein MJ, Cleveland
Biol Sci Med Sci 2009;64:312-7. eds. Ballenger’s otorhinolaryngology head SS, Wang H, Maison SF. Toward a differ-

ential diagnosis of hidden hearing loss in 54. Hoffman HJ, Dobie RA, Losonczy KG, 70. Wyatt JR, Niparko JK, Rothman ML,
humans. PLoS One 2016;11(9):e0162726. Themann CL, Flamme GA. Declining deLissovoy G. Cost effectiveness of the
40. Mehraei G, Hickox AE, Bharadwaj prevalence of hearing loss in US adults multichannel cochlear implant. Am J Otol
HM, et al. Auditory brainstem response aged 20 to 69 years. JAMA Otolaryngol 1995;16:52-62.
latency in noise as a marker of cochlear Head Neck Surg 2017;143:274-85. 71. Saunders JE, Francis HW, Skarzynski
synaptopathy. J Neurosci 2016;36:3755-64. 55. Pacala JT, Yueh B. Hearing deficits in PH. Measuring success: cost-effectiveness
41. Hickox AE, Larsen E, Heinz MG, Shi- the older patient: “I didn’t notice any- and expanding access to cochlear implan-
nobu L, Whitton JP. Translational issues thing.” JAMA 2012;307:1185-94. tation. Otol Neurotol 2016; 37(2):
e135-
in cochlear synaptopathy. Hear Res 2017; 56. Bogardus ST Jr, Yueh B, Shekelle PG. 40.
349:164-71. Screening and management of adult hear- 72. Wilson BS, Dorman MF. Cochlear im-
42. Forge A, Schacht J. Aminoglycoside ing loss in primary care: clinical applica- plants: a remarkable past and a brilliant
antibiotics. Audiol Neurootol 2000;5:3-22. tions. JAMA 2003;289:1986-90. future. Hear Res 2008;242:3-21.
43. Duggal P, Sarkar M. Audiologic moni- 57. Rubel EW, Furrer SA, Stone JS. A brief 73. Tang L, Thompson CB, Clark JH, Ceh
toring of multi-drug resistant tuberculo- history of hair cell regeneration research KM, Yeagle JD, Francis HW. Rehabilita-
sis patients on aminoglycoside treatment and speculations on the future. Hear Res tion and psychosocial determinants of co-
with long term follow-up. BMC Ear Nose 2013;297:42-51. chlear implant outcomes in older adults.
Throat Disord 2007;7:5. 58. Crowson MG, Hertzano R, Tucci DL. Ear Hear 2017;38:663-71.
44. Garinis AC, Cross CP, Srikanth P, et al. Emerging therapies for sensorineural hear- 74. McKinnon BJ. Cost effectiveness of
The cumulative effects of intravenous anti- ing loss. Otol Neurotol 2017;38:792-803. cochlear implants. Curr Opin Otolaryngol
biotic treatments on hearing in patients 59. Fukui H, Raphael Y. Gene therapy for Head Neck Surg 2014;22:344-8.
with cystic fibrosis. J Cyst Fibros 2017;16: the inner ear. Hear Res 2013;297:99-105. 75. Emmett SD, Francis HW. The socio-
401-9. 60. Staecker H, Rodgers B. Developments economic impact of hearing loss in U.S.
45. Al-Malky G, Dawson SJ, Sirimanna T, in delivery of medications for inner ear adults. Otol Neurotol 2015;36:545-50.
Bagkeris E, Suri R. High-frequency audi- disease. Expert Opin Drug Deliv 2013;10: 76. Saunders JE, Barrs DM, Gong W, Wil-
ometry reveals high prevalence of amino- 639-50. son BS, Mojica K, Tucci DL. Cost effective-
glycoside ototoxicity in children with cys- 61. Askew C, Rochat C, Pan B, et al. Tmc ness of childhood cochlear implantation
tic fibrosis. J Cyst Fibros 2015;14:248-54. gene therapy restores auditory function in and deaf education in Nicaragua: a dis-
46. Frisina RD, Wheeler HE, Fossa SD, deaf mice. Sci Transl Med 2015;7:295ra108. ability adjusted life year model. Otol Neu-
et al. Comprehensive audiometric analy- 62. Akil O, Seal RP, Burke K, et al. Resto- rotol 2015;36:1349-56.
sis of hearing impairment and tinnitus ration of hearing in the VGLUT3 knock- 77. Emmett SD, Tucci DL, Bento RF, et al.
after cisplatin-based chemotherapy in sur- out mouse using virally mediated gene Moving beyond GDP: cost effectiveness of
vivors of adult-onset cancer. J Clin Oncol therapy. Neuron 2012;75:283-93. cochlear implantation and deaf education
2016;34:2712-20. 63. Pan B, Askew C, Galvin A, et al. Gene in Latin America. Otol Neurotol 2016;37:
47. Theunissen EA, Zuur CL, Bosma SC, therapy restores auditory and vestibular 1040-8.
et al. Long-term hearing loss after chemo- function in a mouse model of Usher syn- 78. PCAST recommends changes to pro-
radiation in patients with head and neck drome type 1c. Nat Biotechnol 2017;35: mote innovation in hearing technologies.
cancer. Laryngoscope 2014;124:2720-5. 264-72. Washington, DC:The White House, October
48. Lambert EM, Gunn GB, Gidley PW. 64. Isgrig K, Shteamer JW, Belyantseva IA, 26, 2015 (https://obamawhitehouse.archives
Effects of radiation on the temporal bone et al. Gene therapy restores balance and .gov/blog/2015/10/26/%E2%80%8Bpcast
in patients with head and neck cancer. auditory functions in a mouse model of -recommends-changes-promote-innovation
Head Neck 2016;38:1428-35. Usher syndrome. Mol Ther 2017;25:780-91. -hearing-technologies).
49. Cruickshanks KJ, Nondahl DM, Dal- 65. Lentz JJ, Jodelka FM, Hinrich AJ, et al. 79. Warren E, Grassley C. Over-the-counter
ton DS, et al. Smoking, central adiposity, Rescue of hearing and vestibular function hearing aids: the path forward. JAMA
and poor glycemic control increase risk by antisense oligonucleotides in a mouse Intern Med 2017;177:609-10.
of hearing impairment. J Am Geriatr Soc model of human deafness. Nat Med 2013; 80. President Trump signs OTC hearing
2015;63:918-24. 19:345-50. aid legislation into law. Hearing Review.
50. Bainbridge KE, Hoffman HJ, Cowie 66. Shibata SB, Ranum PT, Moteki H, et al. August 19, 2017 (http://www.hearingreview
CC. Risk factors for hearing impairment RNA interference prevents autosomal- .com/2017/08/president-trump-signs-otc
among U.S. adults with diabetes: National dominant hearing loss. Am J Hum Genet -hearing-aid-legislation-law/).
Health and Nutrition Examination Survey 2016;98:1101-13. 81. Strom KE. HR 2013 hearing aid dis-
1999-2004. Diabetes Care 2011;34:1540-5. 67. Cox RM, Johnson JA, Xu J. Impact of penser survey:dispensing in the age of in-
51. Mijovic T, Zeitouni A, Colmegna I. advanced hearing aid technology on speech ternet and big box retailers. Hearing Review.
Autoimmune sensorineural hearing loss: understanding for older listeners with mild April 8, 2014 (http://www.hearingreview
the otology-rheumatology interface. Rheu- to moderate, adult-onset, sensorineural .com/2014/04/hr-2013-hearing-aid-dispenser
matology (Oxford) 2013;52:780-9. hearing loss. Gerontology 2014;60:557-68. -survey-dispensing-age-internet-big-box
52. Harris JP, Weisman MH, Derebery JM, 68. Blazer DG, Domnitz S, Liverman CT, -retailers-comparison-present-past-key
et al. Treatment of corticosteroid-respon- eds. Hearing health care for adults:pri- -business-indicators-dispensing-offices).
sive autoimmune inner ear disease with orities for improving access and afford- 82. Strom KE. Hearing aid sales rise 5%
methotrexate: a randomized controlled ability. Washington, DC:National Acade- in 2013;industry closes in on 3M unit
trial. JAMA 2003;290:1875-83. mies Press, 2016. mark. Hearing Review. February 26, 2014
53. Stachler RJ, Chandrasekhar SS, Ar- 69. Chien W, Lin FR. Prevalence of hear- (http://www.hearingreview.com/2014/02/
cher SM, et al. Clinical practice guideline: ing aid use among older adults in the staff-standpoint-hearing-aid-sales-rise-5
sudden hearing loss. Otolaryngol Head United States. Arch Intern Med 2012;172: -2013-industry-closes-3m-unit-mark).
Neck Surg 2012;1463 Suppl:S1-S35. 292-3. Copyright © 2017 Massachusetts Medical Society.


Hipoacusia en Adulto

Transféré par

Informations du document

Copyright

Formats disponibles

Partager ce document

Partager ou intégrer le document

Options de partage

Avez-vous trouvé ce document utile ?

Ce contenu est-il inapproprié ?

Droits d'auteur :

Formats disponibles

Hipoacusia en Adulto

Transféré par

Droits d'auteur :

Formats disponibles

The n e w e ng l a n d j o u r na l of m e dic i n e

Allan H. Ropper, M.D., Editor

Hearing Loss in Adults

n engl j med 377;25 nejm.org December 21, 2017 2465

S t ruc t ur e s of the E a r In volv ed C ause s of He a r ing L oss

2466 n engl j med 377;25 nejm.org December 21, 2017

The New England Journal of Medicine

Organs affected by disorders causing conductive hearing loss

Middle Ear Inner Ear

Tympanic Auditory Nerve

SCALA VESTIBULI Stria vascularis

n engl j med 377;25 nejm.org December 21, 2017 2467

2468 n engl j med 377;25 nejm.org December 21, 2017

The New England Journal of Medicine

n engl j med 377;25 nejm.org December 21, 2017 2469

2470 n engl j med 377;25 nejm.org December 21, 2017

The New England Journal of Medicine

n engl j med 377;25 nejm.org December 21, 2017 2471

2472 n engl j med 377;25 nejm.org December 21, 2017

The New England Journal of Medicine

n engl j med 377;25 nejm.org December 21, 2017 2473

Vous aimerez peut-être aussi