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Tinnitus

MedGen UID:
52760
Concept ID:
C0040264
Finding
Synonyms: Ringing Buzzing Tinnitus; Ringing-Buzzing-Tinnitus
SNOMED CT: Tinnitus (60862001); Noise in ears (60862001); Observation of tinnitus (162349004); Noises in ear (162349004); Noises in head (162349004); Ringing in ear (162352007); Ringing in ears (60862001)
 
HPO: HP:0000360

Definition

Tinnitus is often described as a ringing in the ears. It also can sound like roaring, clicking, hissing, or buzzing. It may be soft or loud, high pitched or low pitched. You might hear it in either one or both ears. Millions of Americans have tinnitus. People with severe tinnitus may have trouble hearing, working or even sleeping. Causes of tinnitus include. -Hearing loss in older people. -Exposure to loud noises. -Ear and sinus infections. -Heart or blood vessel problems. -Meniere's disease. -Brain tumors. -Hormonal changes in women. -Thyroid problems. -Certain medicines. Treatment depends on the cause. Treatments may include hearing aids, sound-masking devices, medicines, and ways to learn how to cope with the noise. NIH: National Institute on Deafness and Other Communication Disorders .  [from MedlinePlus]

Conditions with this feature

Von Hippel-Lindau syndrome
MedGen UID:
42458
Concept ID:
C0019562
Disease or Syndrome
Von Hippel-Lindau (VHL) syndrome is characterized by hemangioblastomas of the brain, spinal cord, and retina; renal cysts and clear cell renal cell carcinoma; pheochromocytoma, pancreatic cysts, and neuroendocrine tumors; endolymphatic sac tumors; and epididymal and broad ligament cysts. Cerebellar hemangioblastomas may be associated with headache, vomiting, gait disturbances, or ataxia. Spinal hemangioblastomas and related syrinx usually present with pain. Sensory and motor loss may develop with cord compression. Retinal hemangioblastomas may be the initial manifestation of VHL syndrome and can cause vision loss. Renal cell carcinoma occurs in about 70% of individuals with VHL and is the leading cause of mortality. Pheochromocytomas can be asymptomatic but may cause sustained or episodic hypertension. Pancreatic lesions often remain asymptomatic and rarely cause endocrine or exocrine insufficiency. Endolymphatic sac tumors can cause hearing loss of varying severity, which can be a presenting symptom. Cystadenomas of the epididymis are relatively common. They rarely cause problems, unless bilateral, in which case they may result in infertility.
Ménière disease
MedGen UID:
7530
Concept ID:
C0025281
Disease or Syndrome
Meniere disease is a chronic illness characterized by intermittent episodes of vertigo lasting from minutes to hours, with fluctuating sensorineural hearing loss, tinnitus, and aural pressure (Sajjadi and Paparella, 2008).
Neurofibromatosis, type 2
MedGen UID:
18014
Concept ID:
C0027832
Neoplastic Process
Neurofibromatosis 2 (NF2) is characterized by bilateral vestibular schwannomas with associated symptoms of tinnitus, hearing loss, and balance dysfunction. The average age of onset is 18 to 24 years. Almost all affected individuals develop bilateral vestibular schwannomas by age 30 years. Affected individuals may also develop schwannomas of other cranial and peripheral nerves, meningiomas, ependymomas, and, very rarely, astrocytomas. Posterior subcapsular lens opacities that rarely progress to a visually significant cataract are the most common ocular findings and may be the first sign of NF2. Mononeuropathy that occurs in childhood is an increasingly recognized finding; it frequently presents as a persistent facial palsy, a squint (third nerve palsy), or hand/foot drop.
Polycythemia vera
MedGen UID:
45996
Concept ID:
C0032463
Neoplastic Process
Polycythemia vera, the most common form of primary polycythemia, is caused by somatic mutation in a single hematopoietic stem cell leading to clonal hematopoiesis. PV is a myeloproliferative disorder characterized predominantly by erythroid hyperplasia, but also by myeloid leukocytosis, thrombocytosis, and splenomegaly. Familial cases of PV are very rare and usually manifest in elderly patients (Cario, 2005). PV is distinct from the familial erythrocytoses (see, e.g., ECYT1, 133100), which are caused by inherited mutations resulting in hypersensitivity of erythroid progenitors to hormonal influences or increased levels of circulating hormones, namely erythropoietin (EPO; 133170) (Prchal, 2005).
Hereditary motor and sensory neuropathy with optic atrophy
MedGen UID:
140747
Concept ID:
C0393807
Disease or Syndrome
Hereditary motor and sensory neuropathy type VI is an autosomal dominant neurologic disorder characterized by peripheral neuropathy and optic atrophy (summary by Voo et al., 2003). Genetic Heterogeneity of Hereditary Motor and Sensory Neuropathy Type VI See also HMSN6B (616505), caused by mutation in the SLC25A46 gene (610826) on chromosome 5q22. For a general phenotypic description and a discussion of genetic heterogeneity of CMT, see CMT1B (118200).
Chiari malformation type I
MedGen UID:
196689
Concept ID:
C0750929
Disease or Syndrome
Chiari malformation type I (CM1) is the protrusion of the cerebellar tonsils through the foramen magnum, defined radiologically as tonsillar descent of 5 mm or more. CM1 is associated with syringomyelia (see 186700) in up to 80% of cases. Although many individuals with CM1 are asymptomatic, the malformation can cause headaches, ocular disturbances, otoneurologic disturbances, lower cranial nerve signs, cerebellar ataxia, or spasticity. Onset of symptoms is usually in the third decade of life (Speer et al., 2003). Since many cases of CM1 are asymptomatic, prevalence estimates may not be accurate. However, a retrospective investigation of brain MRIs reported the prevalence of CM1 to be 1 in 1,280 individuals (Meadows et al., 2000).
Deafness, autosomal dominant 33
MedGen UID:
473419
Concept ID:
C1414002
Gene or Genome
Spinal arachnoiditis
MedGen UID:
318191
Concept ID:
C1710146
Disease or Syndrome
A chronic adhesive arachnoiditis in the spinal arachnoid, with root and spinal cord symptoms similar to those caused by pressure from a tumor.
Episodic ataxia type 2
MedGen UID:
314039
Concept ID:
C1720416
Disease or Syndrome
Episodic ataxia type 2 (EA2) is characterized by paroxysmal attacks of ataxia, vertigo, and nausea typically lasting minutes to days in duration. Attacks can be associated with dysarthria, diplopia, tinnitus, dystonia, hemiplegia, and headache. About 50% of individuals with EA2 have migraine headaches. Onset is typically in childhood or early adolescence (age range 2-32 years). Frequency of attacks can range from once or twice a year to three or four times a week. Attacks can be triggered by stress, exertion, caffeine, alcohol, fever, heat, and phenytoin; they can be stopped or decreased in frequency and severity by administration of acetazolamide or 4-aminopyridine. Between attacks, individuals may initially be asymptomatic but commonly develop interictal findings that can include nystagmus, pursuit and saccade alterations, and ataxia.
Deafness, Y-linked 1
MedGen UID:
321613
Concept ID:
C1824927
Gene or Genome
Deafness, autosomal dominant 9
MedGen UID:
371327
Concept ID:
C1832425
Disease or Syndrome
Hereditary hearing loss and deafness may be conductive, sensorineural, or a combination of both; syndromic (associated with malformations of the external ear or other organs or with medical problems involving other organ systems) or nonsyndromic (no associated visible abnormalities of the external ear or any related medical problems); and prelingual (before language develops) or postlingual (after language develops).
Kanzaki disease
MedGen UID:
324539
Concept ID:
C1836522
Disease or Syndrome
Alpha-N-acetylgalactosaminidase (NAGA) deficiency is a very rare lysosomal storage disorder with atypical features. It is clinically heterogeneous with 3 main phenotypes: type I is an infantile-onset neuroaxonal dystrophy (609241); type II, also known as Kanzaki disease, is an adult-onset disorder characterized by angiokeratoma corporis diffusum and mild intellectual impairment; and type III is an intermediate disorder (see 609241) with mild to moderate neurologic manifestations (Desnick and Schindler, 2001).
Deafness, autosomal dominant 41
MedGen UID:
330834
Concept ID:
C1842371
Disease or Syndrome
Autosomal dominant deafness-41 (DFNA41) is characterized by onset of progressive sensorineural hearing loss usually in the second decade. The hearing loss is severe and ultimately affects all frequencies. Exposure to noise exacerbates the hearing loss, particularly at high frequencies (summary by Yan et al., 2013).
Paraganglioma and gastric stromal sarcoma
MedGen UID:
376098
Concept ID:
C1847319
Neoplastic Process
A rare, inherited disorder marked by tumors of the gastrointestinal tract and tumors that form in embryonic nervous tissue in the head, neck, and torso.
Deafness, autosomal dominant 36
MedGen UID:
376173
Concept ID:
C1847626
Disease or Syndrome
Hereditary hearing loss and deafness may be conductive, sensorineural, or a combination of both; syndromic (associated with malformations of the external ear or other organs or with medical problems involving other organ systems) or nonsyndromic (no associated visible abnormalities of the external ear or any related medical problems); and prelingual (before language develops) or postlingual (after language develops).
Episodic ataxia, type 3
MedGen UID:
376220
Concept ID:
C1847839
Disease or Syndrome
The hereditary ataxias are a group of genetic disorders characterized by slowly progressive incoordination of gait and often associated with poor coordination of hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. In this GeneReview the hereditary ataxias are categorized by mode of inheritance and gene (or chromosome locus) in which pathogenic variants occur.
Episodic ataxia, type 4
MedGen UID:
376222
Concept ID:
C1847843
Disease or Syndrome
The hereditary ataxias are a group of genetic disorders characterized by slowly progressive incoordination of gait and often associated with poor coordination of hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. In this GeneReview the hereditary ataxias are categorized by mode of inheritance and gene (or chromosome locus) in which pathogenic variants occur.
Deafness, autosomal dominant 39, with dentinogenesis imperfecta 1
MedGen UID:
340145
Concept ID:
C1854146
Disease or Syndrome
Deafness, autosomal dominant 16
MedGen UID:
349054
Concept ID:
C1858916
Disease or Syndrome
Vestibulocochlear dysfunction, progressive
MedGen UID:
395483
Concept ID:
C1860432
Disease or Syndrome
DFNA 2 Nonsyndromic Hearing Loss
MedGen UID:
436997
Concept ID:
C2677637
Disease or Syndrome
DFNA2 nonsyndromic hearing loss is characterized by symmetric, predominantly high-frequency sensorineural hearing loss (SNHL) that is progressive across all frequencies. At younger ages, hearing loss tends to be mild in the low frequencies and moderate in the high frequencies; in older persons, the hearing loss is moderate in the low frequencies and severe to profound in the high frequencies. Although the hearing impairment is often detected during routine hearing assessment of a school-age child, it is likely that hearing is impaired from birth, especially at high frequencies. Most affected persons initially require hearing aids to assist with sound amplification between ages ten and 40 years. By age 70 years, all persons with DFNA2 nonsyndromic hearing loss have severe-to-profound hearing impairment.
DEAFNESS, AUTOSOMAL DOMINANT 58
MedGen UID:
854817
Concept ID:
C3888210
Disease or Syndrome

Recent clinical studies

Etiology

Kehrle HM, Sampaio AL, Granjeiro RC, de Oliveira TS, Oliveira CA
Ann Otol Rhinol Laryngol 2016 Mar;125(3):185-94. Epub 2015 Sep 30 doi: 10.1177/0003489415606445. [Epub ahead of print] PMID: 26424781
Sedley W, Parikh J, Edden RA, Tait V, Blamire A, Griffiths TD
J Neurosci 2015 Nov 4;35(44):14822-8. doi: 10.1523/JNEUROSCI.2695-15.2015. PMID: 26538652Free PMC Article
Ramakers GG, van Zon A, Stegeman I, Grolman W
Laryngoscope 2015 Nov;125(11):2584-92. Epub 2015 Jul 7 doi: 10.1002/lary.25370. [Epub ahead of print] PMID: 26153087
Dewyer NA, Kiringoda R, Kram YA, Chang JL, Chang CY, Cheung SW
Otolaryngol Head Neck Surg 2015 Dec;153(6):1019-23. Epub 2015 Jun 26 doi: 10.1177/0194599815591532. [Epub ahead of print] PMID: 26115669
Houdayer E, Teggi R, Velikova S, Gonzalez-Rosa JJ, Bussi M, Comi G, Leocani L
Clin Neurophysiol 2015 Dec;126(12):2356-65. Epub 2015 Feb 16 doi: 10.1016/j.clinph.2015.01.027. [Epub ahead of print] PMID: 25753907

Diagnosis

Çakur B, Yaşa Y
J Oral Maxillofac Surg 2016 Jan;74(1):47-52. Epub 2015 Jul 31 doi: 10.1016/j.joms.2015.07.020. [Epub ahead of print] PMID: 26279489
Sedley W, Parikh J, Edden RA, Tait V, Blamire A, Griffiths TD
J Neurosci 2015 Nov 4;35(44):14822-8. doi: 10.1523/JNEUROSCI.2695-15.2015. PMID: 26538652Free PMC Article
Danesh AA, Lang D, Kaf W, Andreassen WD, Scott J, Eshraghi AA
Int J Pediatr Otorhinolaryngol 2015 Oct;79(10):1683-8. Epub 2015 Jul 26 doi: 10.1016/j.ijporl.2015.07.024. [Epub ahead of print] PMID: 26243502
Dewyer NA, Kiringoda R, Kram YA, Chang JL, Chang CY, Cheung SW
Otolaryngol Head Neck Surg 2015 Dec;153(6):1019-23. Epub 2015 Jun 26 doi: 10.1177/0194599815591532. [Epub ahead of print] PMID: 26115669
Houdayer E, Teggi R, Velikova S, Gonzalez-Rosa JJ, Bussi M, Comi G, Leocani L
Clin Neurophysiol 2015 Dec;126(12):2356-65. Epub 2015 Feb 16 doi: 10.1016/j.clinph.2015.01.027. [Epub ahead of print] PMID: 25753907

Therapy

Wu BP, Searchfield G, Exeter DJ, Lee A
N Z Med J 2015 Oct 16;128(1423):24-34. PMID: 26645752
Størmer CC, Laukli E, Høydal EH, Stenklev NC
Noise Health 2015 Nov-Dec;17(79):411-21. doi: 10.4103/1463-1741.169708. PMID: 26572701
Ozbay I, Kahraman C, Balikci HH, Kucur C, Kahraman NK, Ozkaya DP, Oghan F
J Laryngol Otol 2015 Jun;129(6):544-7. Epub 2015 May 4 doi: 10.1017/S0022215115000845. [Epub ahead of print] PMID: 25936355
Todt I, Rademacher G, Mutze S, Ramalingam R, Wolter S, Mittmann P, Wagner J, Ernst A
Acta Otolaryngol 2015 Aug;135(8):781-5. Epub 2015 Mar 26 doi: 10.3109/00016489.2015.1024332. [Epub ahead of print] PMID: 25812721
Argstatter H, Grapp M, Hutter E, Plinkert PK, Bolay HV
J Psychosom Res 2015 Mar;78(3):285-92. Epub 2014 Sep 3 doi: 10.1016/j.jpsychores.2014.08.012. [Epub ahead of print] PMID: 25224125

Prognosis

Folmer RL, Theodoroff SM, Casiana L, Shi Y, Griest S, Vachhani J
JAMA Otolaryngol Head Neck Surg 2015 Aug;141(8):716-22. doi: 10.1001/jamaoto.2015.1219. PMID: 26181507
Dewyer NA, Kiringoda R, Kram YA, Chang JL, Chang CY, Cheung SW
Otolaryngol Head Neck Surg 2015 Dec;153(6):1019-23. Epub 2015 Jun 26 doi: 10.1177/0194599815591532. [Epub ahead of print] PMID: 26115669
Ozbay I, Kahraman C, Balikci HH, Kucur C, Kahraman NK, Ozkaya DP, Oghan F
J Laryngol Otol 2015 Jun;129(6):544-7. Epub 2015 May 4 doi: 10.1017/S0022215115000845. [Epub ahead of print] PMID: 25936355
Roland LT, Lenze EJ, Hardin FM, Kallogjeri D, Nicklaus J, Wineland AM, Fendell G, Peelle JE, Piccirillo JF
Otolaryngol Head Neck Surg 2015 May;152(5):919-26. Epub 2015 Feb 24 doi: 10.1177/0194599815571556. [Epub ahead of print] PMID: 25715350Free PMC Article
Martines F, Sireci F, Cannizzaro E, Costanzo R, Martines E, Mucia M, Plescia F, Salvago P
Eur Arch Otorhinolaryngol 2015 Oct;272(10):2719-29. Epub 2014 Sep 5 doi: 10.1007/s00405-014-3275-0. [Epub ahead of print] PMID: 25190254

Clinical prediction guides

Størmer CC, Laukli E, Høydal EH, Stenklev NC
Noise Health 2015 Nov-Dec;17(79):411-21. doi: 10.4103/1463-1741.169708. PMID: 26572701
Danesh AA, Lang D, Kaf W, Andreassen WD, Scott J, Eshraghi AA
Int J Pediatr Otorhinolaryngol 2015 Oct;79(10):1683-8. Epub 2015 Jul 26 doi: 10.1016/j.ijporl.2015.07.024. [Epub ahead of print] PMID: 26243502
Ramakers GG, van Zon A, Stegeman I, Grolman W
Laryngoscope 2015 Nov;125(11):2584-92. Epub 2015 Jul 7 doi: 10.1002/lary.25370. [Epub ahead of print] PMID: 26153087
Dewyer NA, Kiringoda R, Kram YA, Chang JL, Chang CY, Cheung SW
Otolaryngol Head Neck Surg 2015 Dec;153(6):1019-23. Epub 2015 Jun 26 doi: 10.1177/0194599815591532. [Epub ahead of print] PMID: 26115669
Piotrowska A, Raj-Koziak D, Lorens A, Skarżyński H
Int J Pediatr Otorhinolaryngol 2015 Aug;79(8):1346-50. Epub 2015 Jun 11 doi: 10.1016/j.ijporl.2015.06.008. [Epub ahead of print] PMID: 26104480

Recent systematic reviews

Ramakers GG, van Zon A, Stegeman I, Grolman W
Laryngoscope 2015 Nov;125(11):2584-92. Epub 2015 Jul 7 doi: 10.1002/lary.25370. [Epub ahead of print] PMID: 26153087
Hall DA, Haider H, Kikidis D, Mielczarek M, Mazurek B, Szczepek AJ, Cederroth CR
Trends Hear 2015 Apr 24;19 doi: 10.1177/2331216515580272. PMID: 25910505Free PMC Article
Jastreboff PJ
HNO 2015 Apr;63(4):307-11. doi: 10.1007/s00106-014-2979-1. PMID: 25862626
Sereda M, Hoare DJ, Nicholson R, Smith S, Hall DA
Ear Hear 2015 Jul-Aug;36(4):417-29. doi: 10.1097/AUD.0000000000000140. PMID: 25587668Free PMC Article
Nemholt SS, Schmidt JH, Wedderkopp N, Baguley DM
BMJ Open 2015 Jan 5;5(1):e006649. doi: 10.1136/bmjopen-2014-006649. PMID: 25564147Free PMC Article

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