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Hearing impairment

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Synonyms and keywords: Hearing impairment, deafness, decreased hearing; loss of hearing

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [[Mailto:charlesmichaelgibson@gmail.com|[1]]]; Associate Editor(s)-in-Chief:


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Overview

Hearing impairment may be a partial or complete decrease in the ability to perceive or comprehend sounds.[1] Caused by a good range of biological and environmental factors, loss of hearing can happen to any organism that perceives sound. It is highly prevalent impairment and chances increase as one’s age above 60.[2]Classification is based on laterality, severity, cause, anatomy of ear, symmetry, clinical characteristics, age of onset, and associated symptoms. Sound waves vary in amplitude and in frequency. Amplitude is that the sound wave’s peak pressure variation. Frequency is that the number of cycles per second of a sinusoidal component of a wave. Loss of the power to detect some frequencies, or to detect low-amplitude sounds, that an organism naturally detects, may be termed as a hearing disorder. Hearing sensitivity is indicated by the quietest sound that a person can detect, termed the hearing threshold. The normal hearing threshold is not the same for all frequencies of sounds. The Long term exposure to environmental noise, Genetics, Disease or illness, Medications[3], and Physical trauma are different biological mechanisms for hearing loss. 10% of the population in the United States is affected by hearing loss. The prevalence of clinically significant hearing loss doubles with each passing decade of life. Approximately two thirds of Americans aged 70 years or older have hearing loss. Any age people are susceptible for hearing impairment it depends on exposure to risk factors. Middle-aged adults can develop hearing loss due to genetic mutations, noise exposure, and ototoxic medications. Older adults also develop multifactorial age-related hearing deafness. Infections are the most common risk for hearing loss in young adults e-g labyrinthitis, meningitis, otitis media, otitis externa. There are different recommendations for screening for hearing loss, at what age, and the various methods for screening including whisper test, hand rub, audiometer, different questionnaire and using smart-phones. Different factors contribute to prognosis of hearing loss and psychological complications associated with deafness are a big concern for physicians. Many persons may not recognize changes in their hearing and others may be embarrassed or frustrated by their perceived deficits. As constant asking for repetition of information can be embarrassing. A focused otologic history with information on the chronicity and onset of hearing loss, along with otoscopy can help is early recognition and treatment. Physical exam along with history aid in the definitive diagnosis of hearing loss. Routine laboratory evaluation is insignificant unless a systemic illness is identified as a cause of hearing loss. Imaging studies could also be used to differentiate conductive hearing loss and, in some cases sensorineural hearing loss (asymmetric hearing loss) for diagnostic and treatment management, including surgical planning. Within 14 days of symptom onset in patients with sudden hearing loss, clinicians should obtain, or refer to a clinician who can obtain, audiometry as soon as possible to confirm the diagnosis of sudden sensorineural hearing loss. Another method for determining deafness is that the Hearing in Noise Test (HINT). Genetic testing could also be considered to be a strong tool for addressing hearing disorders in children. Medical therapy includes removal of impacted cerumen via irrigation, manually, cerumenolytics, and under microscopy. Sudden onset SNHL can be treated with a single dose of steroids. A surgical candidate is the patients with conductive hearing loss due to mechanical problems, such as perforation of the eardrum, ossicular disease, or a cholesteatoma in the middle ear. Hearing Aids and cochlear implantation technology and surgical techniques have advanced significantly. Hearing impairment can be prevented by preventing exposure to common risk factors, loud noise, ototoxic drugs, and trauma. There are no established measures for the secondary prevention of hearing loss. The use of hearing aids and cochlear implants is an expensive intervention for the patients experiencing hearing loss and it is one other major limiting factor for undergoing treatment if patient’s insurance does not cover the cost. Age-related hearing loss has been independently associated with worse quality of life, depression, social isolation, functional decline, increase falls, increased hospitalization and health care use, and accelerated cognitive decline, and increase risk of dementia. Hearing aids or cochlear implants may improve communication, social and emotional function, and cognitive function. Use of somatic cell within the prospect of regrowth in cochlea cells is under study. The tablet-based automated audiometer presents a new method for threshold hearing assessment. Similarly video conferencing, flashing lights to signal events and other telecommunications devices are under study.

References

  1. “Speech and Language Terms and Abbreviations”. Retrieved 2006-12-02.
  2. Nieman CL, Oh ES (2020). “Hearing Loss”. Ann Intern Med. 173 (11): ITC81–ITC96. doi:10.7326/AITC202012010. PMID 33253610 Check |pmid= value (help).
  3. Lanvers-Kaminsky C, Zehnhoff-Dinnesen AA, Parfitt R, Ciarimboli G (2017). “Drug-induced ototoxicity: Mechanisms, Pharmacogenetics, and protective strategies”. Clin Pharmacol Ther. 101 (4): 491–500. doi:10.1002/cpt.603. PMID 28002638.

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Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Overview

Noise-induced hearing loss is a centuries-old concern and is still prevalent worldwide. Harriet Martineau’s 1834 essay, Letters to the Deaf, was the earliest chronicle portraying the social challenges of deafness.

Historical perspective

Noise-induced hearing loss is a centuries-old concern and is still prevalent worldwide. [1]The ancient Egyptians and Greeks tried to elucidate hearing loss and wanted remedies, creating use not solely of empiric rational means that however conjointly magic and faith. Later, within the Middle Ages, examples from Christian ikon ar found demonstrating the miracle healing of hearing loss. Education of the deaf wasn’t thought of doable from the time of Aristotle to the sixteenth century, however from then on was organized on an oversized scale utilizing speech with gestures.[2]

Harriet Martineau was a 19th-century social scientist United Nations agency had a progressive style of hearing loss. Her 1834 essay, Letters to the Deaf, was the earliest chronicle portraying the social challenges of deafness. Martineau details advanced things that deaf individuals experienced within the nineteenth century like social isolation because of frustrations with communication, doc shortcomings, restricted music appreciation, and also the stigma of hearing amplification devices. Her descriptions of those experiences are faced by deaf individuals in current society. Advancements in technology and recognition of the negative social impact of deafness have improved the social expertise for the laborious of hearing; but, social challenges stay relevant. During this article, we tend to review Letters to the Deaf and note the ways in which within which this essay provides a twin perspective relating to the abundance we’ve advanced as a society and the way much we tend to still ought to overcome in addressing the social challenges of deafness.[3]

The hearing aid was unreal within the seventeenth century. The ear trumpet was invented in the seventeenth century and is considered the first device used to help the deaf. The hearing aid (electrical) was first invented in 1898 by Miller Reese Hutchison.

Up till the sixteenth century, it had been unremarkably accepted that people with hearing disorders additionally suffered from multiple different disabilities; this diode to them being heavily discriminated against. it had been not till a Spanish monk named Pedro offender educated a nobleman’s deaf sons a way to scan, write, speak and do the mathematics that this reality was disproven.

References

  1. Kerr MJ, Neitzel RL, Hong O, Sataloff RT (2017). “Historical review of efforts to reduce noise-induced hearing loss in the United States”. Am J Ind Med. 60 (6): 569–577. doi:10.1002/ajim.22627. PMID 28514024.
  2. Brosch S, Pirsig W (2003). “[Hearing loss in a cultural-historical context. Part 1]”. HNO. 51 (1): 25–9. doi:10.1007/s00106-002-0747-0. PMID 12557094.
  3. Naples J, Valdez TA (2020). “Letters to the Deaf: Present-Day Relevance of History’s Earliest Social Analysis of Deafness”. Otolaryngol Head Neck Surg. 162 (3): 319–321. doi:10.1177/0194599819900492. PMID 31959057.
Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Overview

Classification is based on laterality, severity, cause, anatomy of ear, symmetry, clinical characteristics, age of onset, and associated symptoms.

Classification

Hearing losses can be classified according to:[1]

  1. Laterality
  2. Symmetry
  3. Clinical characteristic (syndromic or not)
  4. Time of onset (congenital, perinatal, or postnatal)
  5. Hereditary (genetic or not)
  6. Time of manifestation (prelingual, perilingual, or post-lingual)
  7. Intensity (mild, moderate, severe, and profound)
CLASSIFICATION BASED OF SEVERITY OF DISEASE[2]
MILD MODERATE SEVERE DISABLING
HEARING LOSS 26–40 dB HL 41–60 dB HL 61–80 dB HL >80 dB HL
DESCRIPTION Patient hears fine Hear with some difficulty

People Mumble

Difficulty in hearing Patients rely on reading lips.

The term deaf is often used by persons

with profound hearing loss with >80 dB HL.

INTERVENTION Communication strategies Communication strategies

Amplification

Communication strategies

Hearing aids

Cochlear implant

Communication strategies

Hearing aids

Cochlear implant

Sign language

Lip reading


CLASSIFICATION BASED ON ANATOMY OF EAR[3]
CONDUCTIVE SENSORINEURAL MIXED
CHL occurs when sound is not conducted efficiently through the outer ear canal to the middle ear due to any cause. SNHL occurs when there’s damage to the internal ear or to the nerve pathways from the ear to the brain that is vestibulocochlear nerve or sensory relay center for auditory stimulus. So injury at the cochlea or proximal to the cochlea is termed as SNHL. Mixed HL is defined as CHL and SNHL.
CHL accounts for 90%–95% of all childhood HL. SNHL is more common in adults.
With pure conductive hearing loss, the quality of hearing and speech discrimination is good.
Often only mild and is never worse. It can be mild, moderate, or severe, including complete deafness.
Common causes in adults include: If SNHL takes hours or days to develop it can be due to:

If hearing loss develops in weeks:

If happens in years:

CLASSIFICATION BASED ON CAUSE[4][5]
CONGENITAL ACQUIRED
  • Neoplastic: Direct compression of the cochlear nerve by the tumor, occlusion or vascular compression of the internal auditory artery, intratumor bleeding, internal auditory channel occlusion, and toxic substances produced by the tumor that result in degeneration of the inner ear.


References

  1. Nieman CL, Oh ES (2020). “Hearing Loss”. Ann Intern Med. 173 (11): ITC81–ITC96. doi:10.7326/AITC202012010. PMID 33253610 Check |pmid= value (help).
  2. Nieman CL, Oh ES (2020). “Hearing Loss”. Ann Intern Med. 173 (11): ITC81–ITC96. doi:10.7326/AITC202012010. PMID 33253610 Check |pmid= value (help).
  3. Kral A (2017). “[Pathophysiology of hearing loss : Classification and treatment options]”. HNO. 65 (4): 290–297. doi:10.1007/s00106-016-0183-1. PMID 27299892.
  4. Kral A (2017). “[Pathophysiology of hearing loss : Classification and treatment options]”. HNO. 65 (4): 290–297. doi:10.1007/s00106-016-0183-1. PMID 27299892.
  5. Kenna MA (2015). “Acquired Hearing Loss in Children”. Otolaryngol Clin North Am. 48 (6): 933–53. doi:10.1016/j.otc.2015.07.011. PMID 26452421.
Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Overview

Sound waves vary in amplitude and frequency. Amplitude is that the sound wave’s peak pressure variation. Frequency is that the number of cycles per second of a sinusoidal component of a wave. Loss of the power to detect some frequencies, or to detect low-amplitude sounds, that an organism naturally detects, may be termed as a hearing disorder. Hearing sensitivity is indicated by the quietest sound that a person can detect, termed the hearing threshold. The normal hearing threshold is not the same for all frequencies of sounds. Long-term exposure to environmental noise, Genetics, Disease or illness, Medications[1], and Physical trauma are different biological mechanisms for hearing loss.

Pathophysiology

  • Sound waves vary in amplitude and frequency. Amplitude is that the sound wave’s peak pressure variation. Frequency is that the number of cycles per second of a sinusoidal component of a wave. Loss of the power to detect some frequencies, or to detect low-amplitude sounds, that an organism naturally detects, may be termed as a hearing disorder.[2]

Loudness, frequency, and discrimination deficiencies[3]

  • Hearing sensitivity is indicated by the quietest sound that a person can detect, termed the hearing threshold. This threshold is often accurately measured by a behavioral audiogram, in humans and a few animals. A record is formed of the quietest sound that consistently prompts a response from the listener. The test is administered for sounds of various frequencies. There also are some electrophysiological tests that will be performed without requiring a behavioral response of the individual.
  • The normal hearing threshold is not the same for all frequencies of sounds. If different frequencies of sound are played at an equivalent amplitude, some are going to be loud, quiet, or completely inaudible. Generally, if the amplitude is increased, a sound is more likely to be heard. Ordinarily, when animals use sound to speak, hearing therein sort of animal is most sensitive for the frequencies produced by calls, or, within the case of humans, speech. This tuning of hearing exists at many levels of the sensory system, all the way from the physical characteristics of the ear, to the nerves, and tracts that convey the auditory impulses to the portion of the brain that is sensitive to hearing sounds.[4]
  • A hearing disorder exists when a person isn’t sensitive to the sounds normally heard by its kind. The term hearing impairment is usually reserved for folks that have relative insensitivity to sound within the speech frequencies. The severity of hearing impairment is defined as; how much louder a sound must be made over the usual levels before the listener can perceive it. In profound deafness, even the loudest sounds which will be produced by the instrument wont to measure hearing like an audiometer, might not be detected.
  • There is a rare sort of hearing impairment that affects speech discrimination alone. There is another aspect to hearing that involves the quality of a sound instead of amplitude and frequency. This quality of sound is typically measured by tests of speech discrimination. These tests require that the sound isn’t only detected but understood.

Different biological mechanisms

Long term exposure to environmental noise[5][6]

  • Populations of individuals living near airports or freeways are exposed to levels of noise typically > 65 dB. If the lifestyle of a person includes significant outdoor activities, these exposures over time can degrade hearing. Various states have set noise standards to guard people against these adverse sound risks.
  • The EPA has identified the extent of 70 DB for twenty-four-hour exposure because of the level necessary to guard the general public against deafness (EPA, 1974).
  • Noise-Induced deafness (NIHL) typically is centered at 4000 Hz.
  • The louder the noise is, the shorter is the safe amount of exposure. Normally, the safe amount of exposure is reduced by an element 2 for each additional 3 dB. for instance, the safe daily exposure amount at 85 dB is 8 hours, while the safe exposure at 91 dB is merely 2 hours. Sometimes, an element 2 per 5 dB is employed.
  • Personal electronic audio devices, like iPods, can produce powerful enough sound to cause significant Noise-Induced deafness, iPods often reaching 115 decibels or higher, as long as lesser intensities of even 70 dB also can cause deafness.

Genetic[7][8]

  • Hearing loss is often inherited. Both dominant and recessive genes exist which may cause mild to profound impairment. If a family features a gene for deafness it’ll persist across generations because it’ll happen within the offspring albeit it’s inherited from just one parent. If a family had a genetic hearing disorder caused by a gene it’ll not always be apparent because it will need to be passed onto offspring from both parents Dominant and recessive hearing disorders are often syndromic or non-syndromic. Recent gene mapping has identified dozens of non-syndromic dominant and recessive sorts of deafness.
  • The most common sort of congenital hearing disorder in developed countries is non-syndromic recessive, also referred to as Connexin 26 deafness.

Disease or illness[9]

  • Autoimmune disease has only recently been recognized as a possible cause of cochlear damage. Although probably rare, autoimmune processes can focus on the cochlea specifically, without symptoms affecting other organs. Wegener’s granulomatosis is one of the autoimmune conditions which will precipitate deafness.
  • Presbycusis is deaf thanks to the loss of perception to high tones, mainly within the elderly people. It is caused by some to be a degenerative processes within the inner ear, although there is no proven link to aging.[10]
  • AIDS patients frequently experience sensory system anomalies.
  • Chlamydia may cause deafness in newborns to whom the disease has been passed at birth through the birth canal during normal vaginal delivery.
  • Syphilis is usually transmitted from pregnant women to their fetuses, and a few thirds of the infected children will eventually become deaf.

Medications[1]

See also Ototoxicity
  • Extremely heavy Vicodin and OxyContin abuse are understood to cause hearing disorders.

Physical trauma

  • There is often external damage either to the ear itself or to the brain centers that process the aural information conveyed by the ears.
  • People who sustain head injury are especially susceptible to deafness or tinnitus, either temporary or permanent.
  • Exposure to very bang (90 dB or more, like jet engines at close range) can cause progressive deafness. Exposure to one event of extreme bang (such as explosions) also can cause deafness. A typical source of acoustic trauma may be a too-loud music concert.

References

  1. 1.0 1.1 Lanvers-Kaminsky C, Zehnhoff-Dinnesen AA, Parfitt R, Ciarimboli G (2017). “Drug-induced ototoxicity: Mechanisms, Pharmacogenetics, and protective strategies”. Clin Pharmacol Ther. 101 (4): 491–500. doi:10.1002/cpt.603. PMID 28002638.
  2. Nieman CL, Oh ES (2020). “Hearing Loss”. Ann Intern Med. 173 (11): ITC81–ITC96. doi:10.7326/AITC202012010. PMID 33253610 Check |pmid= value (help).
  3. Beechey T, Buchholz JM, Keidser G (2020). “Hearing Impairment Increases Communication Effort During Conversations in Noise”. J Speech Lang Hear Res. 63 (1): 305–320. doi:10.1044/2019_JSLHR-19-00201. PMID 31846598.
  4. Moser T, Starr A (2016). “Auditory neuropathy–neural and synaptic mechanisms”. Nat Rev Neurol. 12 (3): 135–49. doi:10.1038/nrneurol.2016.10. PMID 26891769.
  5. Hammer MS, Swinburn TK, Neitzel RL (2014). “Environmental noise pollution in the United States: developing an effective public health response”. Environ Health Perspect. 122 (2): 115–9. doi:10.1289/ehp.1307272. PMC 3915267. PMID 24311120.
  6. Carroll YI, Eichwald J, Scinicariello F, Hoffman HJ, Deitchman S, Radke MS; et al. (2017). “Vital Signs: Noise-Induced Hearing Loss Among Adults – United States 2011-2012”. MMWR Morb Mortal Wkly Rep. 66 (5): 139–144. doi:10.15585/mmwr.mm6605e3. PMC 5657963. PMID 28182600.
  7. Meena R, Ayub M (2017). “Genetics Of Human Hereditary Hearing Impairment”. J Ayub Med Coll Abbottabad. 29 (4): 671–676. PMID 29331002.
  8. Ahmed S, Sheraz S, Malik SA, Ahmed NR, Malik SA, Farooq S; et al. (2018). “Frequency Of Congenital Hearing Loss In Neonates”. J Ayub Med Coll Abbottabad. 30 (2): 234–236. PMID 29938425.
  9. Paul A, Marlin S, Parodi M, Rouillon I, Guerlain J, Pingault V; et al. (2017). “Unilateral Sensorineural Hearing Loss: Medical Context and Etiology”. Audiol Neurootol. 22 (2): 83–88. doi:10.1159/000474928. PMID 28738350.
  10. Fischer N, Weber B, Riechelmann H (2016). “[Presbycusis – Age Related Hearing Loss]”. Laryngorhinootologie. 95 (7): 497–510. doi:10.1055/s-0042-106918. PMID 27392191.
Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Overview

Causes based on each organ system, mode of injury, anatomy of ear, and choronicity are listed below.

Causes

Conductive

Sensorineural

Mixed

Miscellaneous

Causes by Organ System

Cardiovascular Wegener’s granulomatosis
Chemical / poisoning Bromates, Lead poisoning, Lobeline, Mercury poisoning, Fetal methylmercury syndrome
Dermatologic Keratosis obturans
Drug Side Effect Amikacin, Amphotericin B, Aspirin, Bumetanide, Capreomycin, Carboplatin, Cisplatin, Deferasirox, Desferrioxamine, Etacrynic acid, Ethacrynic acid, Frusemide, Gentamicin, Isotretinoin, Kanamycin, Loop diuretics, Neomycin, Netilmicin, Nitrogen mustard, Quinine, Streptomycin, Sulfasalazine, Vancomycin
Ear Nose Throat Acoustic neuroma, Adenoid hypertrophy, Aural polyps, Branchiootic dysplasia, Cholesteatoma, Eustachian tube dysfunction, Glue ear, Hydrocodone bitartrate and acetaminophen, Keratosis obturans, Labrynthitis, Labyrinthitis ossificans, Meniere disease, Mondini Dysplasia, Nasopharyngeal cancer, Nonsuppurative otitis media, Oculovestibuloauditory syndrome, Otitis externa, Presbycusis, Suppurative otitis media, Tympanic membrane perforation, Hereditary sensorineural hearing loss, Otodental dysplasia, Otopalatodigital syndrome type 1, Otosclerosis, Vestibulocochlear dysfunction progressive familial, Middle ear effusion, Benign paroxysmal positional vertigo, Strachan syndrome, Malignant external ear canal tumor, Nasopharyngeal carcinoma, Barotitis Media, Tympanic membrane rupture, Cerumen impaction, Ear wax
Endocrine Hypothyroidism
Environmental No underlying causes
Gastroenterologic No underlying causes
Genetic 17- beta-hydroxysteroid dehydrogenase X deficiency, ABCD syndrome, Acrocephalosyndactylia, Alopecia mental retardation syndrome, Alpha-L-iduronidase deficiency (Hurler syndrome), Alport syndrome, Alström syndrome, Apert syndrome, Arias oculootoradial syndrome, ARTS syndrome, Autosomal recessive nonsyndromic deafness type 1, Barakat syndrome, Bart-Pumphrey syndrome, Bartter syndrome, Biotinidase deficiency, Bjornstad syndrome, Black locks, oculocutaneous albinism, and deafness of the sensorineural type, Blau syndrome, BOR syndrome, BOR-Duane hydrocephalus contiguous gene syndrome, Branchio-oculo-facial syndrome, BRESHECK syndrome, Brown-Vialetto-van Laere syndrome, Canavan disease, CATSPER-related male infertility, Cerebrocostomandibular syndrome, Chanarin-Dorfman syndrome, Charcot-Marie-Tooth disease, X-linked 1, Charcot-Marie-Tooth disease, X-linked recessive type 5, Charcot-Marie-Tooth-Hoffmann syndrome, CHARGE syndrome, Chromosome 1, deletion q21 q25, Chromosome 1, monosomy 1p22 p13, Chromosome 17 trisomy mosaicism, Chromosome 18, deletion 18q23, Chromosome 22q11.2 microduplication, Chromosome 3, trisomy 3q13 2 q25, Cockayne syndrome, Colobomatous microphthalmia-heart disease-hearing loss, Combined pituitary hormone deficiency 3 (LHX gene), Cornelia de Lange Syndrome, Craniofacial-deafness-hand syndrome, Craniometaphyseal dysplasia, Jackson type, Cushing proximal symphalangism, Davenport-Donlan syndrome, Deafness onychodystrophy syndrome, autosomal dominant, Dentinogenesis imperfecta-short stature-hearing loss-mental retardation, Diabetes-deafness syndrome maternally transmitted, DIDMOAD syndrome, Donnai-Barrow syndrome, Down syndrome, Duane-radial ray syndrome, Emanuel syndrome, Enlarged vestibular aqueduct syndrome, Epstein macrothrombocytopenia syndrome, Exostosis of external auditory canal, Fabry disease, Fabry’s Disease, Faisalabad histiocytosis, Familial Danish dementia, Fanconi anaemia, Fechtner syndrome, Fine-Lubinsky syndrome, Galactosamine-6-sulfatase deficiency, Galactose epimerase deficiency, Gangliosidosis GM1, type 1, Goldenhar syndrome, Goldscheider disease, Hajdu-Cheney Syndrome, Harboyan syndrome, Hereditary sensorineural hearing loss, Hereditary sensory and autonomic neuropathy type 1A, Hereditary sensory and autonomic neuropathy type 1B, Hereditary sensory and autonomic neuropathy type 1E, Hunter syndrome, Hunter-Mcdonald syndrome, Hystrix-like ichthyosis with deafness, I-cell disease, Iduronate-2-sulfatase deficiency, Infantile-onset spinocerebellar ataxia, Jacobsen syndrome, Jensen syndrome, Jervell and Lange-Nielsen syndrome, Johanson-Blizzard syndrome, Johnson-Mcmillin syndrome, Jones syndrome, Kallmann syndrome, Kearns-Sayre Syndrome, Keratitis-ichthyosis-deafness syndrome, autosomal recessive, Keutel syndrome, Kjer-type optic atrophy, Klippel Feil deformity conductive deafness absent vagina, Klippel-Feil syndrome, Kniest dysplasia, Lacrimoauriculodentodigital syndrome, LAMM syndrome, Lange Nielsen syndrome, Larsen syndrome, Lenz-Majewski hyperostosis syndrome, Levy-Hollister syndrome, Lobstein’s disease, Loken Senior syndrome, Mandibuloacral dysplasia, Marshall syndrome, May-White syndrome, MELAS, Melnick-Fraser syndrome, MERRF, Microcephaly, Growth Retardation, Cataract, Hearing Loss, and Unusual Appearance, MNGIE syndrome, Mohr-Claussen syndrome, Mohr-Tranebjaerg syndrome, Monosomy 1p36 syndrome, Morquio Disease, Muckle-Wells syndrome, Mucolipidosis II , Mucosulfatidosis, Muenke syndrome, Multiple lentigines syndrome, Multiple Sulfatase Deficiency, Multiple synostoses syndrome type 1, MURCS association, Myhre syndrome, Myositis ossificans progressiva, Nager acrofacial dysostosis, Nance perilymphatic gusher-deafness syndrome, Neurofibromatosis type 2, Neuropathy-ataxia-retinitis pigmentosa, Nievergelt-Pearlman syndrome syndrome, Nonsyndromic deafness, Norrie disease, Okihiro syndrome, Osteogenesis imperfecta, Osteopathia striata with cranial sclerosis, Osteopathia striata, cranial sclerosis, Osteopetrosis (malignant), Otodental dysplasia, Otopalatodigital syndrome type 1, Otosclerosis, Otospondylomegaepiphyseal dysplasia, Paget disease of bone, Palmoplantar keratoderma with deafness, Pendred syndrome, Pfeiffer syndrome, Phosphoribosylpyrophosphate synthetase deficiency, Phosphoribosylpyrophosphate synthetase superactivity, Polyneuropathy, Hearing Loss, Ataxia, Retinitis Pigmentosa and Cataract, Polyostotic osteolytic expansile dysplasia, Postaxial acrofacial dysostosis syndrome, Prolonged QT syndrome variant, Propionic acidemia, Recessive deafness-onychodystrophy-osteodystrophy-retardation syndrome, Refsum disease, Richards-Rundle syndrome, Robinow Syndrome, Robinson syndrome, Rosenberg-Chutorian Syndrome, Saethre-Chotzen syndrome, Sanfilippo disease, Sensory ataxic neuropathy, dysarthria, and ophthalmoparesis, SeSAME syndrome, Shprintzen-Goldberg syndrome, Smith-Magenis syndrome, Sotos syndrome, Soto’s syndrome, Spondylocarpotarsal synostosis syndrome, Spondyloepiphyseal dysplasia congenita, Spondyloperipheral dysplasia, Steinfeld syndrome, Stickler syndrome, Succinyl-CoA synthetase deficiency, Teunissen-Cremers syndrome, Thiamine-responsive megaloblastic anemia syndrome, Tietz hypopigmentation-deafness syndrome, Townes-Brocks syndrome, Treacher Collins type syndrome, Treacher Collins-Franceschetti syndrome, Upper limb defect eye and ear abnormalities, Usher syndrome, Van Buchem disease, Verloes Gillerot Fryns syndrome , Verloes-David Syndrome, Vestibulocochlear dysfunction progressive familial, Vohwinkel mutilating keratoderma, Vohwinkel syndrome, Wardenberg’s Syndrome , Wildervanck syndrome, Willems De vries syndrome , Wittwer sydnrome, Wolf-Hirschorn syndrome, Wolfram syndrome, Woodhouse-Sakati syndrome, Wright dick syndrome, Xeroderma pigmentosum, X-linked diffuse leiomyomatosis-Alport syndrome, X-linked mental retardation-hypotonic facies syndrome, X-linked mixed hearing loss with stapes fixation, Yemenite deaf-blind hypopigmentation syndrome, Zellweger spectrum, Ziprkowski-Adam syndrome, Zunich neuroectodermal syndrome, Sickle cell anemia
Hematologic Polycythemia vera, Sickle cell anemia, Fanconi anaemia, Leukemia
Iatrogenic Mastoidectomy, Middle ear irradiation, Myringoplasty, Myringotomy, Nasopharynx irradiation, Radiotherapy, Skull irradiation
Infectious Disease Adenoiditis, Epstein-Barr virus , Herpes simplex, Herpes zoster, Influenza, Lassa fever, Lyme Disease, Mastoiditis, Measles, Meningitis, Meningococcal disease, Meningoencephalitis, Middle ear effusion, Middle ear infection, Mumps, Ramsay Hunt syndrome, Rickettsiae, Rocky mountain spotted fever, Rubella, Tertiary Syphilis, TORCH Syndrome, Typhus fever, Upper respiratory tract infection, Suppurative otitis media, Congenital cytomegalovirus infection, Congenital rubella infection , Congenital syphilis infection
Musculoskeletal / Ortho Acrocephalosyndactylia, Lobstein’s disease, Osteogenesis imperfecta, Osteopetrosis (malignant), Paget disease of bone, Temporal bone fracture
Neurologic Athabaskan brain stem dysgenesis, Basilar artery migraine, Benign paroxysmal positional vertigo, Cerebral palsy, Glomus jugulare tumor, Hydrocephalus, Intracranial space-occupying lesion, Kernicterus, Multiple sclerosis, Spinocerebellar ataxia, Strachan syndrome, Sudanophilic cerebral sclerosis, Superficial siderosis of the central nervous system, Kallmann syndrome, Neurofibromatosis type 2, Ramsay Hunt syndrome, Head injury
Nutritional / Metabolic Fabry’s Disease, Galactosamine-6-sulfatase deficiency, Galactose epimerase deficiency, Gangliosidosis GM1, type 1, I-cell disease, Morquio Disease, Mucolipidosis II , Mucosulfatidosis, Propionic acidemia, Succinyl-CoA synthetase deficiency, Thiamine-responsive megaloblastic anemia syndrome
Obstetric/Gynecologic Congenital cytomegalovirus infection, Congenital rubella infection , Congenital syphilis infection, Fetal methylmercury syndrome, Neonatal jaundice, Pregnancy, TORCH Syndrome
Oncologic Leukemia, Malignant external ear canal tumor, Metastases, Nasopharyngeal carcinoma, Paraganglioma, Acoustic neuroma, Nasopharyngeal cancer, Glomus jugulare tumor
Opthalmologic Strachan syndrome
Overdose / Toxicity Amikacin, Amphotericin B, Aspirin, Bumetanide, Capreomycin, Carboplatin, Cisplatin, Desferrioxamine, Etacrynic acid, Ethacrynic acid, Frusemide, Gentamicin, Kanamycin, Loop diuretics, Neomycin, Netilmicin, Nitrogen mustard, Quinine, Streptomycin, Vancomycin
Psychiatric No underlying causes
Pulmonary Branchiootic dysplasia
Renal / Electrolyte Berger’s Disease
Rheum / Immune / Allergy Allergic rhinitis, Macroglobulinemia, Polychondritis, Susac syndrome, Vogt-Koyanagi-Harada syndrome, Oculovestibuloauditory syndrome, Multiple sclerosis
Sexual No underlying causes
Trauma Barotitis Media, Barotrauma, Ear trauma , Head injury, Skull fracture, Temporal bone fracture, Tympanic membrane rupture
Urologic No underlying causes
Dental No underlying causes
Miscellaneous Aging, Cerumen impaction, Ear wax, Forein body in the ear, Noise-induced hearing loss, Physical straining (barotrauma), Rapid descent in air or water (barotrauma)

Causes in Alphabetical Order


References

Differentiating Hearing Impairment from other Diseases

Overview

References

Epidemiology and Demographics


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [[Mailto:charlesmichaelgibson@gmail.com|[1]]]; Associate Editor(s)-in-Chief:


Overview

10% of the population in the United States is affected by hearing loss. The prevalence of clinically significant hearing loss doubles with each passing decade of life. Approximately two-thirds of Americans aged 70 years or older have hearing loss.

Epidemiology and Demographics

  • 10% of the population in the United States is affected by hearing loss.
  • 1 in 1000 is deaf worldwide, So the global deaf population is approximately to be 0.1% of the total population.[1]
  • The figure is likely to be higher in developing countries than developed countries due to restricted access to health care, and, in some cultures, due to the high rate of intrafamilial marriages. The great majority of people with less than average hearing are elderly or developed hearing loss after leaving school.[2]
  • According to the U.S. National Center for Health statistics, approximately three-quarters of deaf and hard-of-hearing Americans experienced the onset of hearing loss after age 18.[2]
  • In the United States, sudden onset sensorineural hearing loss affects almost 5 to 27 per 1 hundred thousand people annually, with about 66,000 new cases per year.[3]
  • The prevalence of clinically significant hearing loss doubles with each passing decade of life.[4]
  • Approximately two-thirds of Americans aged 70 years or older have hearing loss, but only 15%–20% of U.S. older adults use hearing aids.[5]

References

  1. Harrington, Tom (2004-07-01). “Deaf Statistics: Other Countries”. Frequently Asked Questions: Deaf Statistics. Retrieved 2006-10-13. Check date values in: |date= (help)
  2. 2.0 2.1 Holt, Judith (1994). “DEMOGRAPHIC ASPECTS OF HEARING IMPAIRMENT: QUESTIONS AND ANSWERS”. DEMOGRAPHIC ASPECTS OF HEARING IMPAIRMENT. Retrieved 2006-10-13. line feed character in |title= at position 43 (help)
  3. Chandrasekhar SS, Tsai Do BS, Schwartz SR, Bontempo LJ, Faucett EA, Finestone SA; et al. (2019). “Clinical Practice Guideline: Sudden Hearing Loss (Update)”. Otolaryngol Head Neck Surg. 161 (1_suppl): S1–S45. doi:10.1177/0194599819859885. PMID 31369359.
  4. Nieman CL, Oh ES (2020). “Hearing Loss”. Ann Intern Med. 173 (11): ITC81–ITC96. doi:10.7326/AITC202012010. PMID 33253610 Check |pmid= value (help).
  5. Nieman CL, Oh ES (2020). “Hearing Loss”. Ann Intern Med. 173 (11): ITC81–ITC96. doi:10.7326/AITC202012010. PMID 33253610 Check |pmid= value (help).

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Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Overview

Hearing loss can occur in any age group. Any age people are susceptible for hearing impairment it depends on exposure to risk factors. Middle-aged adults can develop hearing loss due to genetic mutations, noise exposure, and ototoxic medications. Older adults also develop multifactorial age-related hearing deafness. Infections are the most common risk for hearing loss in young adults e-g labyrinthitis, meningitis, otitis media, otitis externa.[1]

Risk Factors


Risk Factors Associated with Hearing Loss[2]
Adult


Children



  • Family history of childhood hearing loss
  • Stay in the neonatal intensive care unit >5 d

References

  1. Nieman CL, Oh ES (2020). “Hearing Loss”. Ann Intern Med. 173 (11): ITC81–ITC96. doi:10.7326/AITC202012010. PMID 33253610 Check |pmid= value (help).
  2. Nieman CL, Oh ES (2020). “Hearing Loss”. Ann Intern Med. 173 (11): ITC81–ITC96. doi:10.7326/AITC202012010. PMID 33253610 Check |pmid= value (help).
Screening

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Overview

The different recommendations for screening for hearing loss, at what age, and also describes the various methods for screening including whisper, hand rub, audiometer, different questionnaire and using smartphones.

Screening

Who should be screened

There are different recommendations for who should be screened for deafness, at what different age and presence of risk factors:[1]

  • The general rule is; screening should be carried for at-risk adults, little benefit exists on universal screening of adults or suggested interval screening in any group without being in danger for the hearing disorder. [2]
  • There is insufficient evidence to screening among asymptomatic adults aged 50 years or older by U.S. Preventive Services Task Force [3]
  • Medicare mandates screening as a part of the annual wellness examination for adults older than 65 years.
  • Screen all adults once every decade until age 50 years and each 3 years afterward by the American Speech-Language-Hearing Association.

How to screen? [4]

  • Studies showed that screening with simple tests on a clinic visit, like the whisper test, audio-scope, an otoscope, and audiometer, or a self-assessment questionnaire, results in easy screening with successful therapeutic or compensatory treatment.
Finger rub Whisper test Handheld audiometer[5] Self-assessment questionnaire Mobile apps and smartphones[6]
Examiner gently rubs fingers together close to the patient’s ear.
Examiner stands at arm’s length (approximately 24 inches) behind the patient. The patient blocks 1 ear himself. Examiner whispers 5-6 letters/number combinations.
Examiner holds device in patient’s ear one at a time. The patient indicates an awareness of each tone. They are often used in practice but have limitations.


Single question: Do you believe you have hearing loss? (yes/no). Self-reported hearing difficulty changes by the degree of hearing loss ( mild/moderate, severe/profound) and sociodemographic characteristics and is typically difficult to select during a person with a light degree of deafness.

The advancement of science has led to the development of mobile technology-based screening options, such as the use of different mobile apps (for example, uHear, Mimi) and smartphone or tablet-based portable audiometers that can be connected to conduct screening for hearing impairment.
A positive result’s a failure to spot rub in ≥2 of 6 attempts. The positive result is failure to repeat ≥3 of the 6 combinations Positive result’s failure to spot either the 1000- or 2000-Hz frequency in both ears or the 1000- and 2000-Hz frequencies in 1 ear. There are multiple questionnaire assessment tests, for instance, Hearing Handicap Inventory for the Elderly Screening Version. It has a 10-item questionnaire with three options of “absent,” “present” and “sometimes,” They generally require special supra-aural headphones for monitoring.
  • Single question: Do you believe you have hearing loss? (yes/no). Self-reported hearing difficulty changes by the degree of hearing loss ( mild/moderate, severe/profound) and sociodemographic characteristics and is typically difficult to select during a person with a light degree of deafness [7][8] There are multiple questionnaire assessment tests for instance Hearing Handicap Inventory for the Elderly Screening Version. It has a 10-item questionnaire with three options of “absent,” “present” and “sometimes,”
  • The Weber and Rinne tests should not be used for general screening as they have little sensitivity for the detection of hearing loss. Elderly adults who know they have hearing impairment require audiometry for confirmation, while those who don’t know about their hearing should be screened with the whispered-voice test as described in the table. Elderly adults who perceive the whispered voice require no further testing, while those unable to perceive the voice require audiometry for screening.[9][10]
  • The advancement of science has led to the development of mobile technology-based screening options, such as the use of different mobile phone apps (for example, uHear, Mimi) and smartphone or tablet-based portable audiometers that can be connected to conduct screening for hearing impairment. They generally require supra-aural headphones for monitoring.[11]
  • After deafness is diagnosed, the Weber and Rinne test can be conducted to differentiate whether it’s a conductive, sensorineural, or mixed type hearing loss.
  • All patients with hearing loss should be offered a referral to an audiologist or an otolaryngologist. Worrisome symptoms accompanying hearing loss like headache, weight loss, bleeding, etc. need urgent referral to an otolaryngologist for further investigations.

Genetic tests

  • Genetic testing may be considered to be a significant tool for diagnosing hearing impairment in children. The genetic screening test is defined as the analysis of human DNA in order to detect heritable-related mutations in children as early as possible, to prevent cognitive impairment in kids.

References

  1. Nieman CL, Oh ES (2020). “Hearing Loss”. Ann Intern Med. 173 (11): ITC81–ITC96. doi:10.7326/AITC202012010. PMID 33253610 Check |pmid= value (help).
  2. “Integrated Care for Older People: Guidelines on Community-Level Interventions to Manage Declines in Intrinsic Capacity”. WHO Guidelines Approved by the Guidelines Review Committee. 2017. PMID 29608259.
  3. Moyer VA, U.S. Preventive Services Task Force (2012). “Screening for hearing loss in older adults: U.S. Preventive Services Task Force recommendation statement”. Ann Intern Med. 157 (9): 655–61. doi:10.7326/0003-4819-157-9-201211060-00526. PMID 22893115.
  4. Nieman CL, Oh ES (2020). “Hearing Loss”. Ann Intern Med. 173 (11): ITC81–ITC96. doi:10.7326/AITC202012010. PMID 33253610 Check |pmid= value (help).
  5. Strawbridge WJ, Wallhagen MI (2017). “Simple Tests Compare Well with a Hand-held Audiometer for Hearing Loss Screening in Primary Care”. J Am Geriatr Soc. 65 (10): 2282–2284. doi:10.1111/jgs.15044. PMC 5641245. PMID 28799200.
  6. Sandström J, Swanepoel de W, Carel Myburgh H, Laurent C (2016). “Smartphone threshold audiometry in underserved primary health-care contexts”. Int J Audiol. 55 (4): 232–8. doi:10.3109/14992027.2015.1124294. PMID 26795898.
  7. Yueh B, Shapiro N, MacLean CH, Shekelle PG (2003). “Screening and management of adult hearing loss in primary care: scientific review”. JAMA. 289 (15): 1976–85. doi:10.1001/jama.289.15.1976. PMID 12697801.
  8. Goman AM, Reed NS, Lin FR, Willink A (2020). “Variations in Prevalence and Number of Older Adults With Self-reported Hearing Trouble by Audiometric Hearing Loss and Sociodemographic Characteristics”. JAMA Otolaryngol Head Neck Surg. 146 (2): 201–203. doi:10.1001/jamaoto.2019.3584. PMC 6902177 Check |pmc= value (help). PMID 31750866.
  9. Bagai A, Thavendiranathan P, Detsky AS (2006). “Does this patient have hearing impairment?”. JAMA. 295 (4): 416–28. doi:10.1001/jama.295.4.416. PMID 16434632. Review in: Evid Based Nurs. 2006 Oct;9(4):120 Review in: Evid Based Med. 2006 Aug;11(4):116
  10. Goman AM, Reed NS, Lin FR, Willink A (2020). “Variations in Prevalence and Number of Older Adults With Self-reported Hearing Trouble by Audiometric Hearing Loss and Sociodemographic Characteristics”. JAMA Otolaryngol Head Neck Surg. 146 (2): 201–203. doi:10.1001/jamaoto.2019.3584. PMC 6902177 Check |pmc= value (help). PMID 31750866.
  11. Sandström J, Swanepoel de W, Carel Myburgh H, Laurent C (2016). “Smartphone threshold audiometry in underserved primary health-care contexts”. Int J Audiol. 55 (4): 232–8. doi:10.3109/14992027.2015.1124294. PMID 26795898.
Natural History, Complications and Prognosis

Overview

Different factors contribute to prognosis of hearing loss and psychological complications associated with deafness are a big concern for physicians.

Natural History, Complications and Prognosis

Natural History

65% of patients with sudden idiopathic sensorineural hearing loss recover completely within 14 days, independent of any type of medical or surgical treatment. There is a fundamental difference in the behavior of apical and basal cochlea losses recovery and hearing recovery is always better at low than at high frequencies.

Complications

Age-related hearing loss has been associated with[1][2]

Prognosis

The prognosis for hearing recovery for idiopathic SSNHL depends on a variety of things including the severity of hearing loss, the shape of the audiogram, age of onset, presence of vertigo, and presence or absence of other risk factors.[3][4]

Prognosis can be predicted according to the slope of the audiogram taken at the start of hearing difficulty (low-frequency losses do better than high-frequency losses), Inflammatory markers, erythrocyte sedimentation rates, hearing at 8 kHz, in some cases, it depends on speech discrimination scores and spatial disorientation symptoms.[5]

References

  1. Nieman CL, Oh ES (2020). “Hearing Loss”. Ann Intern Med. 173 (11): ITC81–ITC96. doi:10.7326/AITC202012010. PMID 33253610 Check |pmid= value (help).
  2. Lin FR, Yaffe K, Xia J, Xue QL, Harris TB, Purchase-Helzner E; et al. (2013). “Hearing loss and cognitive decline in older adults”. JAMA Intern Med. 173 (4): 293–9. doi:10.1001/jamainternmed.2013.1868. PMC 3869227. PMID 23337978. Review in: Evid Based Nurs. 2014 Apr;17(2):60-1
  3. Kuhn M, Heman-Ackah SE, Shaikh JA, Roehm PC (2011). “Sudden sensorineural hearing loss: a review of diagnosis, treatment, and prognosis”. Trends Amplif. 15 (3): 91–105. doi:10.1177/1084713811408349. PMC 4040829. PMID 21606048.
  4. Huafeng Y, Hongqin W, Wenna Z, Yuan L, Peng X (2019). “Clinical characteristics and prognosis of elderly patients with idiopathic sudden sensorineural hearing loss”. Acta Otolaryngol. 139 (10): 866–869. doi:10.1080/00016489.2019.1641218. PMID 31452421.
  5. Mattox DE, Simmons FB (1977). “Natural history of sudden sensorineural hearing loss”. Ann Otol Rhinol Laryngol. 86 (4 Pt 1): 463–80. doi:10.1177/000348947708600406. PMID 889223.
Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Social Impact | Future or Investigational Therapies

Case Studies

Case Studies

Case #1
Related Chapters
External Links

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