Evaluation of hearing loss

Summary

Hearing loss is one of the most common sensory impairments. An estimated 1.57 billion people globally had hearing loss in 2019.[1]

Hearing loss affects people of all ages and can be permanent if not treated correctly. Proper diagnosis rests on a thorough understanding of ear anatomy and physiology.

Anatomy

The human ear is divided into 3 parts: the external ear, the middle ear, and the inner ear.

Anatomy of the ear

Created by BMJ Knowledge Centre

The external ear consists of the auricle (or pinna), the ear canal, and the tympanic membrane (or ear drum). The tympanic membrane divides the external ear from the middle ear.
Like the external ear, in its normal state, the middle ear is filled with air. Connected to the malleus (or hammer) is the incus (anvil), which is then connected to the stapes (stirrup). On the medial wall of the middle ear is the promontory, which demarcates the inner ear and, specifically, the basal turn of the cochlea. The stapes sits in an area called the oval window. The round window is inferior to the oval window. These windows lead to the inner ear.
The inner ear is fluid-filled and contains the organs of hearing (the cochlea) and the organs of balance (the semicircular canals, utricle, and saccule). The cochleovestibular nerve connects the end organs to the auditory and vestibular pathways.Image

Physiology

Sound waves require a medium, such as air or water. The compression in a sound wave is channeled down the ear canal to the tympanic membrane. Vibrations of the tympanic membrane are then transmitted by the ossicular chain through the oval window into the cochlea. The vibrations of the cochlea cause a fluid wave, which stimulates hair cells within the cochlea. This generates an electrical impulse, which is transmitted along the cochlear nerve to the brain, where it is interpreted.

Anything that interferes with the movement of sound from the external ear to the middle ear to the inner ear, and then to the brain, can cause a hearing loss. The external ear and middle ear may appear normal on exam if the cause is in the inner ear or brain.

Classification of hearing loss

Generally, hearing loss is classified as conductive or sensorineural.

Conductive hearing loss: caused by pathology of the external ear, tympanic membrane or middle ear which disrupts sound transmission to the inner ear. Many causes can be treated successfully with surgery.
Sensorineural hearing loss: caused by abnormal function of the cochlea, auditory nerve, or higher auditory processing pathways.[2] Many sensorineural hearing losses are permanent because the human inner ear and hair cells have only limited ability to repair themselves.[3]

Library

Anatomy of the ear
Picture of a normal left ear
Foreign body in ear canal
Insect in ear canal with pressure equalization tube visible anteriorly
Squamous cell carcinoma of the ear canal
Ear canal with bony narrowing secondary to exostoses
Swollen ear canal, almost completely closed due to acute otitis externa
Otomycosis: fungal debris in ear canal
Erythematous bulging tympanic membrane due to acute otitis media
Right tympanic membrane perforation
Left ear with effusion behind intact tympanic membrane
Right attic cholesteatoma
Right ear with glomus tumor visible inferiorly behind intact tympanic membrane
Battle's sign
Hemotympanum

Citations

Key Articles

Chandrasekhar SS, Tsai Do BS, Schwartz SR, et al. Clinical Practice Guideline: Sudden Hearing Loss (Update). Otolaryngol Head Neck Surg. 2019 Aug;161(1_suppl):S1-S45.[Abstract][Full Text]
Schwartz SR, Magit AE, Rosenfeld RM, et al. Clinical practice guideline (update): earwax (cerumen impaction). Otolaryngol Head Neck Surg. 2017 Jan;156(1 Suppl):S1-29.[Abstract]
Tsai Do BS, Bush ML, Weinreich HM, et al. Clinical practice guideline: age-related hearing loss. Otolaryngol Head Neck Surg. 2024 May;170 Suppl 2:S1-54.[Abstract][Full Text]
American College of Radiology. ACR Appropriateness Criteria: hearing loss and/or vertigo. 2018 [internet publication].[Full Text]

Referenced Articles

1. GBD 2019 Hearing Loss Collaborators. Hearing loss prevalence and years lived with disability, 1990-2019: findings from the Global Burden of Disease Study 2019. Lancet. 2021 Mar 13;397(10278):996-1009.[Abstract][Full Text]
2. Chandrasekhar SS, Tsai Do BS, Schwartz SR, et al. Clinical Practice Guideline: Sudden Hearing Loss (Update). Otolaryngol Head Neck Surg. 2019 Aug;161(1_suppl):S1-S45.[Abstract][Full Text]
3. Nacher-Soler G, Garrido JM, Rodríguez-Serrano F. Hearing regeneration and regenerative medicine: present and future approaches. Arch Med Sci. 2019 Jul;15(4):957-967.[Abstract][Full Text]
4. Guest JF, Greener MJ, Robinson AC, et al. Impacted cerumen: composition, production, epidemiology and management. QJM. 2004 Aug;97(8):477-88.[Abstract][Full Text]
5. Roeser RJ, Ballachanda BB. Physiology, pathophysiology, and anthropology/epidemiology of human ear canal secretions. J Am Acad Audiol. 1997 Dec;8(6):391-400.[Abstract]
6. Crandell CC, Roeser RJ. Incidence of excessive/impacted cerumen in individuals with mental retardation: a longitudinal investigation. Am J Ment Retard. 1993 Mar;97(5):568-74.[Abstract]
7. Schwartz SR, Magit AE, Rosenfeld RM, et al. Clinical practice guideline (update): earwax (cerumen impaction). Otolaryngol Head Neck Surg. 2017 Jan;156(1 Suppl):S1-29.[Abstract]
8. Sharp JF, Wilson JA, Ross L, et al. Ear wax removal: a survey of current practice. BMJ. 1990 Dec 1;301(6763):1251-3.[Abstract][Full Text]
9. Chandler JR. Partial occlusion of the external auditory meatus: its effect upon air and bone conduction hearing acuity. Laryngoscope. 1964 Jan;74:22-54.[Abstract]
10. Aaron K, Cooper TE, Warner L, et al. Ear drops for the removal of ear wax. Cochrane Database Syst Rev. 2018 Jul 25;(7):CD012171.[Abstract][Full Text]
11. Goldman SA, Ankerstjerne JK, Welker KB, et al. Fatal meningitis and brain abscess resulting from foreign body-induced otomastoiditis. Otolaryngol Head Neck Surg. 1998 Jan;118(1):6-8.[Abstract]
12. Allanson BM, Low TH, Clark JR, et al. Squamous cell carcinoma of the external auditory canal and temporal bone: an update. Head Neck Pathol. 2018 Sep;12(3):407-18.[Abstract]
13. Sheehy JL. Diffuse exostoses and osteomata of the external auditory canal: a report of 100 operations. Otolaryngol Head Neck Surg. 1982 May-Jun;90(3 Pt 1):337-42.[Abstract]
14. Kemink JL, Graham MD. Osteomas and exostoses of the external auditory canal - medical and surgical management. J Otolaryngol. 1982 Apr;11(2):101-6.[Abstract]
15. Rosenfeld RM, Schwartz SR, Cannon CR, et al. Clinical practice guideline: acute otitis externa. Otolaryngol Head Neck Surg. 2014 Feb;150(1 suppl):S1-S24.[Abstract][Full Text]
16. Roland PS, Stroman DW. Microbiology of acute otitis externa. Laryngoscope. 2002 Jul;112(7 pt 1):1166-77.[Abstract][Full Text]
17. Rubin J, Yu VL. Malignant external otitis: insights into pathogenesis, clinical manifestations, diagnosis, and therapy. Am J Med. 1988 Sep;85(3):391-8.[Abstract]
18. Ress BD, Luntz M, Telischi FF, et al. Necrotizing external otitis in patients with AIDS. Laryngoscope. 1997 Apr;107(4):456-60.[Abstract]
19. Weinroth SE, Schessel D, Tuazon CU. Malignant otitis externa in AIDS patients: case report and review of the literature. Ear Nose Throat J. 1994 Oct;73(10):772-4, 777-8.[Abstract]
20. Rubin J, Yu VL, Kamerer DB, et al. Aural irrigation with water: a potential pathogenic mechanism for inducing malignant external otitis? Ann Otol Rhinol Laryngol. 1990 Feb;99(2 Pt 1):117-9.[Abstract]
21. Ford GR, Courteney-Harris RG. Another hazard of ear syringing: malignant external otitis. J Laryngol Otol. 1990 Sep;104(9):709-10.[Abstract]
22. Driscoll PV, Ramachandrula A, Drezner DA, et al. Characteristics of cerumen in diabetic patients: a key to understanding malignant external otitis. Otolaryngol Head Neck Surg. 1993 Oct;109(4):676-9.[Abstract]
23. Lieberthal AS, Carroll AE, Chonmaitree T, et al. The diagnosis and management of acute otitis media. Pediatrics. 2013 Mar;131(3):e964-99.[Abstract][Full Text]
24. Rosenfeld RM, Kay D. Natural history of untreated otitis media. Laryngoscope. 2003 Oct;113(10):1645-57.[Abstract]
25. Darrow DH, Dash N, Derkay CS. Otitis media: concepts and controversies. Curr Opin Otolaryngol Head Neck Surg. 2003 Dec;11(6):416-23.[Abstract]
26. Jackson C, Kaylie D, Coppit G, et al. Glomus jugulare tumors with intracranial extension. Neurosurg Focus. 2004 Aug 15;17(2):E7.[Abstract]
27. Woods C, Strasnick B, Jackson C. Surgery for glomus tumors: the Otology Group experience. Laryngoscope. 1993 Nov;103(11 Pt 2 Suppl 60):65-70.[Abstract]
28. Brodie HA, Thompson TC. Management of complications from 820 temporal bone fractures. Am J Otol. 1997 Mar;18(2):188-97.[Abstract]
29. Markou K, Goudakos J. An overview of the etiology of otosclerosis. Eur Arch Otorhinolaryngol. 2009 Jan;266(1):25-35.[Abstract]
30. Dobie RA. The burdens of age-related and occupational noise-induced hearing loss in the United States. Ear Hear. 2008 Aug;29(4):565-77.[Abstract]
31. Tsai Do BS, Bush ML, Weinreich HM, et al. Clinical practice guideline: age-related hearing loss. Otolaryngol Head Neck Surg. 2024 May;170 Suppl 2:S1-54.[Abstract][Full Text]
32. Hansen MP, Scott AM, McCullough A, et al. Adverse events in people taking macrolide antibiotics versus placebo for any indication. Cochrane Database Syst Rev. 2019 Jan 18;1:CD011825.[Abstract][Full Text]
33. Chawla N, Olshaker JS. Diagnosis and management of dizziness and vertigo. Med Clin N Am. 2006 Mar;90(2):291-304.[Abstract]
34. Basura GJ, Adams ME, Monfared A, et al. Clinical practice guideline: Ménière's disease. Otolaryngol Head Neck Surg. 2020 Apr;162(suppl 2):S1-55.[Abstract][Full Text]
35. Lemole, M., & Behbahani, M. (2013). Retrospective Study of Skull Base Fracture: A Study of Incidents, Complications, Management, and Outcome Overview from Trauma-One-Level Institute over 5 Years. Journal of Neurological Surgery Part B: Skull Base, 74(S 01), A239.
36. Ishman SL, Friedland DR. Temporal bone fractures: traditional classification and clinical relevance. Laryngoscope. 2004 Oct;114(10):1734-41.[Abstract]
37. Darrouzet V, Duclos JY, Liguoro D, et al. Management of facial paralysis resulting from temporal bone fractures: our experience in 115 cases. Otolaryngol Head Neck Surg. 2001 Jul;125(1):77-84.[Abstract]
38. Nageris BI, Popovtzer A. Acoustic neuroma in patients with completely resolved sudden hearing loss. Ann Otol Rhinol Laryngol. 2003 May;112(5):395-7.[Abstract]
39. Ogawa H, Suzutani T, Baba Y, et al. Etiology of severe sensorineural hearing loss in children: independent impact of congenital CMV infection and GJB2 mutations. J Infect Dis. 2007 Mar 15;195(6):782-8.[Abstract]
40. Grosse SD, Ross DS, Dollard SC. Congenital cytomegalovirus (CMV) infection as a cause of permanent bilateral hearing loss: a quantitative assessment. J Clin Virol. 2008 Feb;41(2):57-62.[Abstract]
41. Kadambari S, Williams EJ, Luck S, et al. Evidence based management guidelines for the detection and treatment of congenital CMV. Early Hum Dev. 2011 Nov;87(11):723-8.[Abstract]
42. Strasnick B, Jacobson JJ. Teratogenic hearing loss. J Am Acad Audiol. 1995 Jan;6(1):28-38.[Abstract]
43. Centers for Disease Control and Prevention. Congenital syphilis. 2021 [internet publication].[Full Text]
44. Walker GJ, Walker D, Molano Franco D, et al. Antibiotic treatment for newborns with congenital syphilis. Cochrane Database Syst Rev. 2019 Feb 15;2(2):CD012071.[Abstract][Full Text]
45. Van Camp G, Smith RJH. Hereditary Hearing Loss Homepage. 2021 [internet publication].[Full Text]
46. Mori T, Westerberg BD, Atashband S, et al. Natural history of hearing loss in children with enlarged vestibular aqueduct syndrome. J Otolaryngol Head Neck Surg. 2008 Feb;37(1):112-8.[Abstract]
47. Boskabadi H, Zakerihamidi M, Moradi A, et al. Risk factors for sensorineural hearing loss in neonatal hyperbilirubinemia. Iran J Otorhinolaryngol. 2018 Jul;30(99):195-202.[Abstract][Full Text]
48. Akinpelu OV, Waissbluth S, Daniel SJ. Auditory risk of hyperbilirubinemia in term newborns: a systematic review. Int J Pediatr Otorhinolaryngol. 2013 Jun;77(6):898-905.[Abstract][Full Text]
49. Edmond K, Clark A, Korczak VS, et al. Global and regional risk of disabling sequelae from bacterial meningitis: a systematic review and meta-analysis. Lancet Infect Dis. 2010 May;10(5):317-28.[Abstract]
50. Vlastarakos PV, Nikolopoulos TP, Tavoulari E, et al. Auditory neuropathy: endocochlear lesion or temporal processing impairment? Implications for diagnosis and management. Int J Pediatr Otorhinolaryngol. 2008 Aug;72(8):1135-50.[Abstract]
51. Kim MB, Zhang Y, Chang Y, et al. Diabetes mellitus and the incidence of hearing loss: a cohort study. Int J Epidemiol. 2017 Apr 1;46(2):717-726.[Abstract][Full Text]
52. Riera JL, Del R Maliandi M, Musuruana JL, et al. Sudden sensorineural hearing loss in systemic lupus erythematosus and antiphospholipid syndrome: a clinical review. Curr Rheumatol Rev. 2020;16(2):84-91.[Abstract]
53. Lobo D, López FG, García-Berrocal JR, et al. Diagnostic tests for immunomediated hearing loss: a systematic review. J Laryngol Otol. 2008 Jun;122(6):564-73.[Abstract]
54. Chawdhary G, Pankhania M, Douglas S, et al. Current management of necrotising otitis externa in the UK: survey of 221 UK otolaryngologists. Acta Otolaryngol. 2017 Aug;137(8):818-822.[Abstract][Full Text]
55. National Institute for Health and Care Excellence. Quality standard QS185. Hearing loss in adults. Jul 2019 [internet publication].[Full Text]
56. National Institute for Health and Care Excellence. Tinnitus: assessment and management. NICE guideline NG155. Mar 2020 [internet publication].[Full Text]
57. Yetiser S, Hidir Y, Gonul E. Facial nerve problems and hearing loss in patients with temporal bone fractures: demographic data. J Trauma. 2008 Dec;65(6):1314-20.[Abstract]
58. Talebnasab MH, Hougaard DD. Transient profound hearing loss and severe facial nerve palsy in schwannomas within the internal acoustic canal: a case report. Oncol Lett. 2023 Mar;25(3):126.[Abstract][Full Text]
59. Silveira RQ, Carvalho VT, Cavalcanti HN, et al. Multiple cranial nerve palsies in malignant external otitis: a rare presentation of a rare condition. IDCases. 2020;22:e00945.[Abstract][Full Text]
60. Danner CJ. Facial nerve paralysis. Otolaryngol Clin N Am. 2008 Jun;41(3):619-32, x.[Abstract]
61. Pretto Flores L, De Almeida CS, Casulari LA. Positive predictive values of selected clinical signs associated with skull base fractures. J Neurosurg Sci. 2000 Jun;44(2):77-82; discussion 82-3.[Abstract]
62. American College of Radiology. ACR Appropriateness Criteria: head trauma. 2020 [internet publication].[Full Text]
63. Johnson F, Semaan MT, Megerian CA. Temporal bone fracture: evaluation and management in the modern era. Otolaryngol Clin N Am. 2008 Jun;41(3):597-618, x.[Abstract]
64. Byun S, Lee JY, Kim BG, et al. Acute vertigo and sensorineural hearing loss from infarction of the vestibulocochlear nerve: a case report. Medicine (Baltimore). 2018 Oct;97(41):e12777.[Abstract][Full Text]
65. Lee H, Kim JS, Chung EJ, et al. Infarction in the territory of anterior inferior cerebellar artery: spectrum of audiovestibular loss. Stroke. 2009 Dec;40(12):3745-51.[Abstract][Full Text]
66. Lee H, Whitman GT, Lim JG, et al. Bilateral sudden deafness as a prodrome of anterior inferior cerebellar artery infarction. Arch Neurol. 2001 Aug;58(8):1287-9.[Abstract][Full Text]
67. Huang MH, Huang CC, Ryu SJ, et al. Sudden bilateral hearing impairment in vertebrobasilar occlusive disease. Stroke. 1993 Jan;24(1):132-7.[Abstract][Full Text]
68. Powers WJ, Rabinstein AA, Ackerson T, et al. 2018 guidelines for the early management of patients with acute ischemic stroke: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2018 Jan 24;49(3):e46-e110.[Abstract][Full Text]
69. Mattox R, Smith LW, Kettner NW. Recognition of spontaneous vertebral artery dissection preempting spinal manipulative therapy: a patient presenting with neck pain and headache for chiropractic care. J Chiropr Med. 2014 Jun;13(2):90-5.[Abstract][Full Text]
70. Lee VH, Brown RD Jr, Mandrekar JN, et al. Incidence and outcome of cervical artery dissection: a population-based study. Neurology. 2006 Nov 28;67(10):1809-12.[Abstract]
71. Arnold M, Bousser M. Clinical manifestations of vertebral artery dissection. Front Neurol Neurosci. 2005;20:77-86.[Abstract]
72. Gottesman RF, Sharma P, Robinson KA, et al. Clinical characteristics of symptomatic vertebral artery dissection: a systematic review. Neurologist. 2012 Sep;18(5):245-54.[Abstract]
73. Hanning U, Sporns PB, Schmiedel M, et al. CT versus MR Techniques in the Detection of Cervical Artery Dissection. J Neuroimaging. 2017 Nov;27(6):607-612.[Abstract][Full Text]
74. CADISS trial investigators., Markus HS, Hayter E, et al. Antiplatelet treatment compared with anticoagulation treatment for cervical artery dissection (CADISS): a randomised trial. Lancet Neurol. 2015 Apr;14(4):361-7.[Abstract][Full Text]
75. Peng J, Liu Z, Luo C, et al. Treatment of Cervical Artery Dissection: Antithrombotics, Thrombolysis, and Endovascular Therapy. Biomed Res Int. 2017;2017:3072098.[Abstract][Full Text]
76. Jensen J, Salottolo K, Frei D, et al. Comprehensive analysis of intra-arterial treatment for acute ischemic stroke due to cervical artery dissection. J Neurointerv Surg. 2017 Jul;9(7):654-658.[Abstract][Full Text]
77. Rosenfeld RM, Shin JJ, Schwartz SR, et al. Clinical practice guideline: otitis media with effusion (update). Otolaryngol Head Neck Surg. 2016 Feb;154(1 suppl):S1-41.[Abstract][Full Text]
78. National Institute for Health and Care Excellence. Otitis media with effusion in under 12s. Aug 2023 [internet publication].[Full Text]
79. Nelson HD, Bougatsos C, Nygren P, et al. Universal newborn hearing screening: systematic review to update the 2001 US Preventive Services Task Force Recommendation. Pediatrics. 2008 Jul;122(1):e266-76.[Abstract]
80. American Academy of Pediatrics. Early hearing detection and intervention (EHDI) [internet publication].[Full Text]
81. Atula S, Sinkkonen ST, Saat R, et al. Association of multiple sclerosis and sudden sensorineural hearing loss. Mult Scler J Exp Transl Clin. 2016 Jan-Dec;2:2055217316652155.[Abstract][Full Text]
82. Vikram KB, Naseeruddin K. Combined tuning fork tests in hearing loss: explorative clinical study of the patterns. J Otolarygol. 2004 Aug;33(4):227-34.[Abstract]
83. Ahmed OH, Gallant SC, Ruiz R, et al. Validity of the Hum test, a simple and reliable alternative to the Weber test. Ann Otol Rhinol Laryngol. 2018 Jun;127(6):402-5.[Abstract]
84. Olusanya BO, Davis AC, Hoffman HJ. Hearing loss grades and the International classification of functioning, disability and health. Bull World Health Organ. 2019 Oct 1;97(10):725-8.[Abstract][Full Text]
85. Berg AL, Prieve BA, Serpanos YC, et al. Hearing screening in a well-infant nursery: profile of automated ABR-fail/OAE-pass. Pediatrics. 2011 Feb;127(2):269-75.[Abstract]
86. Vlastarakos PV, Candiloros D, Papacharalampous G, et al. Diagnostic challenges and safety considerations in cochlear implantation under the age of 12 months. Int J Pediatr Otorhinolaryngol. 2010 Feb;74(2):127-32.[Abstract]
87. Wingard JC, Zhao HB. Cellular and deafness mechanisms underlying connexin mutation-induced hearing loss - a common hereditary deafness. Front Cell Neurosci. 2015;9:202.[Abstract][Full Text]
88. British Association of Audiovestibular Physicians. Guidelines for aetiological investigation into severe to profound bilateral permanent childhood hearing impairment. April 2015 [internet publication].[Full Text]
89. Shearer AE, Smith RJ. Massively parallel sequencing for genetic diagnosis of hearing loss: the new standard of care. Otolaryngol Head Neck Surg. 2015 Aug;153(2):175-82.[Abstract]
90. American College of Radiology. ACR Appropriateness Criteria: hearing loss and/or vertigo. 2018 [internet publication].[Full Text]
91. British Association of Audiovestibular Physicians; British Association of Paediatricians in Audiology. Guidelines for aetiological investigation of infants with congenital hearing loss identified through newborn hearing screening: best practice guidelines. May 2008 [internet publication].[Full Text]
92. Centers for Disease Control and Prevention. Laboratory testing. 2020 [internet publication].[Full Text]
93. British Association of Audiovestibular Physicians. Guidelines for aetiological investigation into unilateral permanent childhood hearing impairment. April 2015 [internet publication].[Full Text]
94. British Association of Audiovestibular Physicians; British Association of Paediatricians in Audiology. Aetiological investigation into bilateral mild to moderate permanent hearing loss in children. April 2009 [internet publication].[Full Text]
95. Bakker R, Aarts MC, van der Heijden GJ, et al. No evidence for the diagnostic value of Borrelia serology in patients with sudden hearing loss. Otolaryngol Head Neck Surg. 2012 Apr;146(4):539-43.[Abstract]
96. Li MM, Tayoun AA, DiStefano M, et al. Clinical evaluation and etiologic diagnosis of hearing loss: a clinical practice resource of the American College of Medical Genetics and Genomics (ACMG). Genet Med. 2022 Jul;24(7):1392-406.[Abstract][Full Text]
97. Centers for Disease Control and Prevention. Babies born with congenital cytomegalovirus (CMV). May 2022 [internet publication].[Abstract][Full Text]
98. Rawlinson WD, Boppana SB, Fowler KB, et al. Congenital cytomegalovirus infection in pregnancy and the neonate: consensus recommendations for prevention, diagnosis, and therapy. Lancet Infect Dis. 2017 Jun;17(6):e177-88.[Abstract]

Diseases