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Tinnitus

MedGen UID:
52760
Concept ID:
C0040264
Disease or Syndrome
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 an auditory perception that can be described as the experience of sound, in the ear or in the head, in the absence of external acoustic stimulation. [from HPO]

Term Hierarchy

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.
Meniere 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. Because NF2 is considered an adult-onset disease, it may be underrecognized in children, in whom skin tumors and ocular findings (retinal hamartoma, thickened optic nerves, cortical wedge cataracts, third cranial nerve palsy) may be the first manifestations. Mononeuropathy that occurs in childhood is an increasingly recognized finding; it frequently presents as a persistent facial palsy or hand/foot drop.
Hereditary motor and sensory neuropathy with optic atrophy
MedGen UID:
140747
Concept ID:
C0393807
Disease or Syndrome
MFN2 hereditary motor and sensory neuropathy (MFN2-HMSN) is a classic axonal peripheral sensorimotor neuropathy, inherited in either an autosomal dominant (AD) manner (~90%) or an autosomal recessive (AR) manner (~10%). MFN2-HMSN is characterized by more severe involvement of the lower extremities than the upper extremities, distal upper-extremity involvement as the neuropathy progresses, more prominent motor deficits than sensory deficits, and normal (>42 m/s) or only slightly decreased nerve conduction velocities (NCVs). Postural tremor is common. Median onset is age 12 years in the AD form and age eight years in the AR form. The prevalence of optic atrophy is approximately 7% in the AD form and approximately 20% in the AR form.
Chiari type I malformation
MedGen UID:
196689
Concept ID:
C0750929
Congenital Abnormality
Arnold-Chiari type I malformation refers to a relatively mild degree of herniation of the posteroinferior region of the cerebellum (the cerebellar tonsils) into the cervical canal with little or no displacement of the fourth ventricle. It is characterized by one or both pointed (not rounded) cerebellar tonsils that project 5 mm below the foramen magnum, measured by a line drawn from the basion to the opisthion (McRae Line)
Episodic ataxia type 2
MedGen UID:
314039
Concept ID:
C1720416
Disease or Syndrome
Episodic ataxia is a genetically heterogeneous neurologic condition characterized by spells of incoordination and imbalance, often associated with progressive ataxia. Episodic ataxia type 2 is the most common form of EA (Jen et al., 2007). For a discussion of genetic heterogeneity of episodic ataxia, see EA1 (160120).
Autosomal dominant nonsyndromic hearing loss 9
MedGen UID:
371327
Concept ID:
C1832425
Disease or Syndrome
DFNA9 is an autosomal dominant adult-onset form of progressive sensorineural hearing loss associated with variable vestibular dysfunction (summary by Robertson et al., 2006).
Hyperostosis cranialis interna
MedGen UID:
327093
Concept ID:
C1840404
Disease or Syndrome
Hyperostosis cranialis interna is a bone disorder characterized by endosteal hyperostosis and osteosclerosis of the calvaria and the skull base. The progressive bone overgrowth causes entrapment and dysfunction of cranial nerves I, II, V, VII, and VIII (Waterval et al., 2010).
Autosomal dominant nonsyndromic hearing loss 43
MedGen UID:
330769
Concept ID:
C1842108
Disease or Syndrome
An autosomal dominant nonsyndromic deafness that has material basis in variation in the chromosome region 2p12.
Autosomal dominant nonsyndromic hearing loss 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).
Autosomal dominant nonsyndromic hearing loss 44
MedGen UID:
334525
Concept ID:
C1843895
Disease or Syndrome
Any autosomal dominant nonsyndromic deafness in which the cause of the disease is a mutation in the CCDC50 gene.
Deafness, X-linked 5
MedGen UID:
335096
Concept ID:
C1845095
Disease or Syndrome
X-linked deafness-5 is a neurologic disorder characterized by childhood onset of auditory neuropathy and later onset of distal sensory impairment affecting the peripheral nervous system (summary by Zong et al., 2015).
Autosomal dominant nonsyndromic hearing loss 36
MedGen UID:
376173
Concept ID:
C1847626
Disease or Syndrome
An autosomal dominant condition caused by mutations in the TMC1 gene, encoding transmembrane channel-like protein 1. It is characterized by bilateral progressive hearing loss.
Episodic ataxia type 3
MedGen UID:
376220
Concept ID:
C1847839
Disease or Syndrome
A very rare form of hereditary episodic ataxia with characteristics of vestibular ataxia, vertigo, tinnitus and interictal myokymia.
Episodic ataxia type 4
MedGen UID:
376222
Concept ID:
C1847843
Disease or Syndrome
A very rare form of hereditary episodic ataxia with characteristics of late-onset episodic ataxia, recurrent attacks of vertigo and diplopia.
Deafness, autosomal dominant 39, with dentinogenesis imperfecta 1
MedGen UID:
340145
Concept ID:
C1854146
Disease or Syndrome
Autosomal dominant nonsyndromic hearing loss 16
MedGen UID:
349054
Concept ID:
C1858916
Disease or Syndrome
An autosomal dominant nonsyndromic deafness that has material basis in variation in the chromosome region 2q23-q24.3.
Autosomal dominant nonsyndromic hearing loss 2A
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.
Autosomal recessive nonsyndromic hearing loss 77
MedGen UID:
412541
Concept ID:
C2746083
Disease or Syndrome
Any autosomal recessive nonsyndromic deafness in which the cause of the disease is a mutation in the LOXHD1 gene.
Vestibulocochlear dysfunction, progressive
MedGen UID:
419730
Concept ID:
C2931176
Disease or Syndrome
Autosomal dominant nonsyndromic hearing loss 64
MedGen UID:
481578
Concept ID:
C3279948
Disease or Syndrome
Any autosomal dominant nonsyndromic deafness in which the cause of the disease is a mutation in the DIABLO gene.
Partial lipodystrophy, congenital cataracts, and neurodegeneration syndrome
MedGen UID:
813897
Concept ID:
C3807567
Disease or Syndrome
Lipodystrophies are rare disorders characterized by loss of body fat from various regions and predisposition to metabolic complications of insulin resistance and lipid abnormalities. FPLD7 is an autosomal dominant disorder with a highly variable phenotype. Additional features, including early-onset cataracts and later onset of spasticity of the lower limbs, have been noted in some patients (summary by Garg et al., 2015). For a general phenotypic description and a discussion of genetic heterogeneity of familial partial lipodystrophy (FPLD), see 151660.
Autosomal dominant nonsyndromic hearing loss 33
MedGen UID:
854638
Concept ID:
C3887930
Disease or Syndrome
An autosomal dominant nonsyndromic deafness that has material basis in variation in the chromosome region 13q34.
Hearing loss, Y-linked 1
MedGen UID:
854748
Concept ID:
C3888076
Disease or Syndrome
Y-linked deafness-1 (DFNY1) is characterized by male-limited postlingual progressive sensorineural hearing loss of variable severity, with onset in the first to third decades of life (Wang et al., 2009). Genetic Heterogeneity of Y-Linked Deafness DFNY2 (400047) is caused by mutation in the TBL1Y gene (400033).
Autosomal dominant nonsyndromic hearing loss 50
MedGen UID:
854780
Concept ID:
C3888123
Disease or Syndrome
Autosomal dominant deafness-50 is a form of nonsyndromic hearing loss. Hearing impairment shows postlingual onset and is progressive (summary by Mencia et al., 2009).
Autosomal dominant nonsyndromic hearing loss 58
MedGen UID:
854817
Concept ID:
C3888210
Disease or Syndrome
Autosomal dominant deafness-58 (DFNA58) is characterized by postlingual sensorineural deafness, with tinnitus and vestibular dysfunction additionally present in some patients (summary by Lezirovitz et al., 2020).
Autosomal dominant nonsyndromic hearing loss 67
MedGen UID:
900413
Concept ID:
C4084712
Disease or Syndrome
DFNA67 is a form of nonsyndromic sensorineural hearing loss. Onset ranges from the first to the fourth year of life. Hearing loss initially affects high frequencies, with variable progression. There are no vestibular symptoms (Xing et al., 2015; Thoenes et al., 2015).
Spinocerebellar ataxia 44
MedGen UID:
1611168
Concept ID:
C4521563
Disease or Syndrome
Hearing loss, autosomal dominant 72
MedGen UID:
1614203
Concept ID:
C4539886
Disease or Syndrome
Hearing loss, autosomal dominant 77
MedGen UID:
1709284
Concept ID:
C5394499
Disease or Syndrome
Autosomal dominant deafness-77 (DFNA77) is characterized by progressive hearing loss affecting high frequencies beginning in the second to third decades of life and affecting all frequencies by the fourth or fifth decades (Li et al., 2019).
Hearing loss, autosomal dominant 82
MedGen UID:
1803416
Concept ID:
C5676948
Disease or Syndrome
Autosomal dominant deafness-82 (DFNA82) is characterized by onset of rapidly progressive bilateral sensorineural hearing loss usually early in the first decade, although later onset may rarely occur. Affected individuals often pass the newborn screening test before the onset of mild to profound hearing loss (Smits et al., 2019).
Hearing loss, autosomal dominant 86
MedGen UID:
1840976
Concept ID:
C5830340
Disease or Syndrome
Autosomal dominant deafness-86 (DFNA86) is characterized by late-onset progressive hearing loss through p53 (TP53; 191170)-mediated hair cell apoptosis (Zhang et al., 2020).

Professional guidelines

PubMed

Dalrymple SN, Lewis SH, Philman S
Am Fam Physician 2021 Jun 1;103(11):663-671. PMID: 34060792
Raoof N, Hoffmann J
Cephalalgia 2021 Apr;41(4):472-478. Epub 2021 Feb 25 doi: 10.1177/0333102421997093. PMID: 33631966Free PMC Article
Chandrasekhar SS, Tsai Do BS, Schwartz SR, Bontempo LJ, Faucett EA, Finestone SA, Hollingsworth DB, Kelley DM, Kmucha ST, Moonis G, Poling GL, Roberts JK, Stachler RJ, Zeitler DM, Corrigan MD, Nnacheta LC, Satterfield L
Otolaryngol Head Neck Surg 2019 Aug;161(1_suppl):S1-S45. doi: 10.1177/0194599819859885. PMID: 31369359

Curated

UK NICE Guideline NG155, Tinnitus: assessment and management, 2020

Recent clinical studies

Etiology

Jarach CM, Lugo A, Scala M, van den Brandt PA, Cederroth CR, Odone A, Garavello W, Schlee W, Langguth B, Gallus S
JAMA Neurol 2022 Sep 1;79(9):888-900. doi: 10.1001/jamaneurol.2022.2189. PMID: 35939312Free PMC Article
Narsinh KH, Hui F, Saloner D, Tu-Chan A, Sharon J, Rauschecker AM, Safoora F, Shah V, Meisel K, Amans MR
JAMA Otolaryngol Head Neck Surg 2022 May 1;148(5):476-483. doi: 10.1001/jamaoto.2021.4470. PMID: 35201283
Mazurek B, Hesse G, Dobel C, Kratzsch V, Lahmann C, Sattel H; Guideline group
Dtsch Arztebl Int 2022 Apr 1;119(13):219-225. doi: 10.3238/arztebl.m2022.0135. PMID: 35197187Free PMC Article
Dalrymple SN, Lewis SH, Philman S
Am Fam Physician 2021 Jun 1;103(11):663-671. PMID: 34060792
Baguley D, McFerran D, Hall D
Lancet 2013 Nov 9;382(9904):1600-7. Epub 2013 Jul 2 doi: 10.1016/S0140-6736(13)60142-7. PMID: 23827090

Diagnosis

Narsinh KH, Hui F, Saloner D, Tu-Chan A, Sharon J, Rauschecker AM, Safoora F, Shah V, Meisel K, Amans MR
JAMA Otolaryngol Head Neck Surg 2022 May 1;148(5):476-483. doi: 10.1001/jamaoto.2021.4470. PMID: 35201283
Mazurek B, Hesse G, Dobel C, Kratzsch V, Lahmann C, Sattel H; Guideline group
Dtsch Arztebl Int 2022 Apr 1;119(13):219-225. doi: 10.3238/arztebl.m2022.0135. PMID: 35197187Free PMC Article
Dalrymple SN, Lewis SH, Philman S
Am Fam Physician 2021 Jun 1;103(11):663-671. PMID: 34060792
Esmaili AA, Renton J
Aust J Gen Pract 2018 Apr;47(4):205-208. doi: 10.31128/AJGP-12-17-4420. PMID: 29621860
Baguley D, McFerran D, Hall D
Lancet 2013 Nov 9;382(9904):1600-7. Epub 2013 Jul 2 doi: 10.1016/S0140-6736(13)60142-7. PMID: 23827090

Therapy

Fuller T, Cima R, Langguth B, Mazurek B, Vlaeyen JW, Hoare DJ
Cochrane Database Syst Rev 2020 Jan 8;1(1):CD012614. doi: 10.1002/14651858.CD012614.pub2. PMID: 31912887Free PMC Article
Tang D, Li H, Chen L
Adv Exp Med Biol 2019;1130:109-128. doi: 10.1007/978-981-13-6123-4_7. PMID: 30915704
Wall M
Neurol Clin 2017 Feb;35(1):45-57. doi: 10.1016/j.ncl.2016.08.004. PMID: 27886895Free PMC Article
Lefaucheur JP, Antal A, Ayache SS, Benninger DH, Brunelin J, Cogiamanian F, Cotelli M, De Ridder D, Ferrucci R, Langguth B, Marangolo P, Mylius V, Nitsche MA, Padberg F, Palm U, Poulet E, Priori A, Rossi S, Schecklmann M, Vanneste S, Ziemann U, Garcia-Larrea L, Paulus W
Clin Neurophysiol 2017 Jan;128(1):56-92. Epub 2016 Oct 29 doi: 10.1016/j.clinph.2016.10.087. PMID: 27866120
Sierpina VS, Wollschlaeger B, Blumenthal M
Am Fam Physician 2003 Sep 1;68(5):923-6. PMID: 13678141

Prognosis

Jarach CM, Lugo A, Scala M, van den Brandt PA, Cederroth CR, Odone A, Garavello W, Schlee W, Langguth B, Gallus S
JAMA Neurol 2022 Sep 1;79(9):888-900. doi: 10.1001/jamaneurol.2022.2189. PMID: 35939312Free PMC Article
Fung C, Dinh PC, Fossa SD, Travis LB
J Natl Compr Canc Netw 2019 Dec;17(12):1557-1568. doi: 10.6004/jnccn.2019.7369. PMID: 31805527
Boyter E
JAAPA 2019 May;32(5):30-35. doi: 10.1097/01.JAA.0000554732.85914.91. PMID: 30969189
Gharaghani M, Seifi Z, Zarei Mahmoudabadi A
Mycopathologia 2015 Jun;179(5-6):415-24. Epub 2015 Jan 30 doi: 10.1007/s11046-015-9864-7. PMID: 25633436
James AL, Thorp MA
BMJ Clin Evid 2007 Mar 1;2007 PMID: 19454061Free PMC Article

Clinical prediction guides

Shin SH, Byun SW, Lee ZY, Park Y, Lee HY
J Int Adv Otol 2023 Nov;19(6):497-502X. doi: 10.5152/iao.2023.22901. PMID: 38088323Free PMC Article
Puri BK, Lee GS
P R Health Sci J 2021 Dec;40(4):188-191. PMID: 35077079
Beukes EW, Manchaiah V, Allen PM, Andersson G, Baguley DM
Prog Brain Res 2021;260:79-99. Epub 2020 Jul 24 doi: 10.1016/bs.pbr.2020.05.022. PMID: 33637233
Yakunina N, Nam EC
Otol Neurotol 2021 Feb 1;42(2):235-241. doi: 10.1097/MAO.0000000000002932. PMID: 33165158
Tinnitus Retraining Therapy Trial Research Group, Scherer RW, Formby C
JAMA Otolaryngol Head Neck Surg 2019 Jul 1;145(7):597-608. doi: 10.1001/jamaoto.2019.0821. PMID: 31120533Free PMC Article

Recent systematic reviews

Webster KE, Galbraith K, Harrington-Benton NA, Judd O, Kaski D, Maarsingh OR, MacKeith S, Ray J, Van Vugt VA, Burton MJ
Cochrane Database Syst Rev 2023 Feb 23;2(2):CD015171. doi: 10.1002/14651858.CD015171.pub2. PMID: 36827524Free PMC Article
Jarach CM, Lugo A, Scala M, van den Brandt PA, Cederroth CR, Odone A, Garavello W, Schlee W, Langguth B, Gallus S
JAMA Neurol 2022 Sep 1;79(9):888-900. doi: 10.1001/jamaneurol.2022.2189. PMID: 35939312Free PMC Article
Thai NLB, Mai NY, Vuong NL, Tin NM, Karam D, Refaey MA, Shahin KM, Soliman AL, Al Khudari R, Thuan TM, Sabbah GM, El-Qushayri AE, Karimzadeh S, Hirayama K, Huy NT
Am J Otolaryngol 2022 Mar-Apr;43(2):103337. Epub 2021 Dec 20 doi: 10.1016/j.amjoto.2021.103337. PMID: 34973662
Jafari Z, Kolb BE, Mohajerani MH
Can J Neurol Sci 2022 Mar;49(2):184-195. Epub 2021 Apr 12 doi: 10.1017/cjn.2021.63. PMID: 33843530Free PMC Article
Büttner-Teleagă A, Kim YT, Osel T, Richter K
Int J Environ Res Public Health 2021 Nov 7;18(21) doi: 10.3390/ijerph182111696. PMID: 34770209Free PMC Article

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    Curated

    • NICE, 2020
      UK NICE Guideline NG155, Tinnitus: assessment and management, 2020

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