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sensorineural hearing loss

Hearing impairment resulting from cranial nerve 8 injury. Etiology: 1) presbycusis due to aging (most common cause in elderly) - bilateral symmetrical high-frequency hearing loss [12] 2) noise-induced a) chronic exposure to high levels of sound b) generally at a frequency of about 4000 Hz c) loss of 6 dB hearing threshold after 4.5 hour outdoor music festival [11] - risk factors: nonuse of earplugs, alcohol, drugs, male d) irreversible, but preventable 3) pharmacologic agents a) aminoglycosides b) salicylates (including aspirin): reversible c) bleomycin d) chloroquine e) erythromycin f) ethacrynic acid g) furosemide h) nortriptyline i) quinidine & quinine 4) Meniere's disease: low-frequency hearing loss 5) acoustic neuroma (vestibular schwannoma) 6) congenital sensorineural hearing loss a) prenatal maternal infections 1] rubella 2] syphilis b) perinatal complications 1] erythroblastosis fetalis 2] anoxia 7) infections a) syphilis b) viral 8) autoimmune 9) perilymph fistula Pathology: - results from damage to the a) neural receptors of the inner ear (cochlea) b) nerve pathways to the brain (auditory nerve), or c) area of the brain that receives sound information (auditory cortex) Genetics: see familial deafness Clinical manifestations: 1) high-frequency sensorineural hearing loss 2) difficulty understanding conversations in a noisy environment 3) Rinne test: a) both air & bone conduction affected b) ratio of air/bone conduction remains normal at 2:1 c) both air & bone conduction are depressed to the same degree* [6] 4) tinnitus &/or vertigo common [5,6] 5) 90% of sudden onset sensorineural hearing loss is unilateral [5] * "Bone conduction is equal to air conduction" assumed equivalent per GRS11 [6] - statement made in reference to an audiogram that differs from the Rinne test in air/bone conduction [6] Special laboratory: - see hearing loss - audiometry for acute or subacute (< 6 months) hearing loss &/or screening [12] Radiology: - magnetic resonance imaging (MRI) of the posterior fossa & internal auditory canal with contrast to exclude acoustic neuroma & meningioma [5] Differential diagnosis: - hearing loss accompanied by ear pain & drainage is more likely to be conductive hearing loss - hearing loss associated with dizziness &/or vertigo or tinnitus is more likely to be sensorineural hearing loss [5] - vestibular dysfunction is common in the elderly [6] - vertigo (&/or vestibular dysfunction), asymmetric hearing loss & tinnitus suggests acoustic neuroma - fluctuating tinnitus, low-frequency hearing loss & episodic vertigo with horizontal nystagmus suggests Meniere's disease - high-frequecy sensorineural hearing loss, presbycusis - most common cause in elderly Management: 1) MRI & referral to otorhinolaryngology for sudden onset sensorineural hearing loss confirmed by audiometry* [5,8] a) surgical removal of acoustic neuroma b) if no clear cause of sudden hearing loss (< 4 days) is identified, a short course of systemic glucocorticoid is reasonable (efficacy controversial) [4,9] c) digital hearing aids vs cochlear implants 2) general a) most causes are not correctable b) avoid noisy environments - use of earplugs with exposure to loud noise [11] c) stop ototoxic medications d) for presbycusis [6] - digital hearing aids [6] - cochlear implant if not responsive to hearing aids [6] * >= 30 dB in <= 3 days

Related

cochlear implant familial deafness Meniere's disease; labyrinthine hydrops; endolymphatic hydrops ocular albinism with late-onset sensorineural deafness (OASD) Rinne test vestibular schwannoma (acoustic neuroma)

Specific

autoimmune sensorineural hearing loss autosomal dominant neurosensory deafness autosomal recessive neurosensory deafness central hearing loss hereditary nonsyndromic hearing loss noised-induced hearing loss non-syndromic sensorineural hearing loss presbycusis sudden sensorineural hearing loss

General

hearing loss (hearing impairment, hard of hearing, HOH)

References

  1. Clinical Anatomy Made Ridiculously Simple. Stephen Goldberg, MedMaster Inc, Miami, 1995
  2. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 42-44
  3. UCLA Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 12-15, 2001
  4. Schreiber BE et al Sudden sensorineural hearing loss The Lancet, Volume 375, Issue 9721, Pages 1203 - 1211, 3 April 2010 PMID: 20362815 doi:10.1016/S0140-6736(09)62071-7
  5. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 18. American College of Physicians, Philadelphia 2009, 2012, 2018,
  6. Geriatric Review Syllabus, 7th edition Parada JT et al (eds) American Geriatrics Society, 2010 - Geriatric Review Syllabus, 11th edition (GRS11) Harper GM, Lyons WL, Potter JF (eds) American Geriatrics Society, 2022
  7. McDonald R Acoustic neuroma: what the evidence says about evaluation and treatment. J Fam Pract. 2011 Jun;60(6):E1-4. PMID: 21647465
  8. Stachler RJ et al Clinical practice guideline: sudden hearing loss. Otolaryngol Head Neck Surg. 2012 Mar;146(3 Suppl):S1-35 PMID: 22383545 (corresponding NGC guideline withdrawn Nov 2017)
  9. Wei BP, Stathopoulos D, O'Leary S. Steroids for idiopathic sudden sensorineural hearing loss. Cochrane Database Syst Rev. 2013 Jul 2;7:CD003998. Review. PMID: 23818120
  10. Yamada M, Nishiwaki Y, Michikawa T, Takebayashi T. Impact of hearing difficulty on dependence in activities of daily living (ADL) and mortality: a 3-year cohort study of community-dwelling Japanese older adults. Arch Gerontol Geriatr. 2011 May-Jun;52(3):245-9. Epub 2010 May 23. PMID: 20546947
  11. Kraaijenga VJC, van Munster JJCM, van Zanten GA Association of Behavior With Noise-Induced Hearing Loss Among Attendees of an Outdoor Music Festival. A Secondary Analysis of a Randomized Clinical Trial. JAMA Otolaryngol Head Neck Surg. Published online April 19, 2018. PMID: 29710132 https://jamanetwork.com/journals/jamaotolaryngology/fullarticle/2677777
  12. NEJM Knowledge+ Otolaryngology - Dalrymple SN, Lewis SH, Philman S. Tinnitus: Diagnosis and Management. Am Fam Physician. 2021 Jun 1;103(11):663-671. PMID: 34060792 Free article. Review.
  13. Auditory Neuropathy http://www.nidcd.nih.gov/health/hearing/neuropathy.asp