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Chapter 212 Evaluation of Hearing Loss

using personal music appliances are common-sense measures which help reduce risk and should be recommended. Other causes of preventable hearing loss include the use of aminoglycoside antibiotics and cisplatin , which are toxic to sensory hair cells. The amount of cumulative dose determines risk. Risk with aminoglycoside use averages 20%; exposure to a course of cisplatin chemotherapy is associated with a nearly 60% risk. To the extent use can be curtailed, the lesser the risk. Data suggesting a role for atherosclerotic risk factors in age-related hearing loss argue for efforts to address smoking, obesity, and poorly controlled diabetes (see Chapters 54, 102, and 235, respectively). Aggressive treatment of autoimmune disease may help limit its associated hearing loss. Treating folate deficiency and limiting the use of analgesics such as acetamino phen and NSAIDs can help lower risk of hearing loss. Referral As noted, sudden unexplained hearing loss demands urgent refer ral to an otolaryngologist for further evaluation, audiometry, and consideration of high-dose oral steroids. In most instances when the patient presents, the underlying cause remains unknown, and treatment decisions need to be made without the benefit of an etiologic diagnosis, recently aided by develop ment of evidence-based consensus guidelines (see Table 212-1). Systemic corticosteroids in such persons achieves significantly better outcomes (61% recovery vs. 32% for placebo), leading to their recommended use in idiopathic cases. Intratympanic ste roids appear equally effective and have the theoretical benefit of higher local drug concentrations, but costs are greater, and administration is more difficult. Referral is also indicated for further evaluation and treatment when a conductive hearing loss (such as otosclerosis or persistent serous otitis media) or a retrocochlear process (such as acoustic neuroma) is suspected or when simple symptomatic measures do not suffice. An asymmetric sensorineural hearing loss , especially when gradually progressive on serial audiometry, also warrants consideration for referral. The otolaryngologist needs to deter mine whether the patient is a candidate for medical or surgical therapy and whether a hearing aid is appropriate. Hearing loss resulting from traumatic rupture of the tympanic membrane also requires semiurgent referral especially when accompanied by vertigo, persistent bleeding, or profuse clear otorrhea. 1. Bainbridge KE, Hoffman HJ, Cowie CC. Diabetes and hearing impairment in the United States: radiometric evidence from the National Health and Nutrition Examination survey 1999 to 2004. Ann Intern Med 2008;149:1. ( Population study showing hearing impairment is common in diabetes. ) 2. Bi WL, Brewster R, Poe D, et al. Superior semicircular canal dehiscence syn drome. J Neurosurg 2017;127:1268. ( Description of this previously unappreciated treatable cause of hearing loss. ) 3. Cunningham LL, Tucci DL. Hearing loss in adults. N Engl J Med 2017; 377;2465. ( Excellent overall review for the generalist reader, with especially good sections on pathophysiology, genetics, and environmental risk factors. ) 4. Curhan SG, Eavey R, Shargordosky J, et al. Analgesic use and the risk of hearing loss in men. Am J Med 2010;123:231. ( Epidemiologic study; finds modest increase in risk, greatest for men under 50 years of age, those with prolonged use, and those taking acetaminophen or NSAIDs compared to aspirin. ) ANNOTATED BIBLIOGRAPHY Pathophysiology and Clinical Presentation

5. Durga J, Verhoef P, Anteunis LJC, et al. Effects of folic acid supplementation on hearing in older adults: a randomized, controlled trial. Ann Intern Med 2007;146:1. ( Supplementation slowed decline in hearing among persons who were folate deficient. ) 6. Mahboubi H, Lin HW, Bhattacharyya N. Prevalence, characteristics, and management patterns of hearing difficulty in the United States: the patient’s perspective. JAMA Otolaryngol Head Neck Surg 2018;44:65. ( A patient perspective of hearing loss and patterns of management changing; reasons discussed. ) 7. Morell RJ, Kim HJ, Hood LJ, et al. Mutations in the connexin 26 gene (GJB2) among Ashkenazi Jews with nonsyndromic recessive deafness. N Engl J Med 1998;339:1500. ( Mutations in the connexin gene were found in high frequency and were estimated to account for most nonsyndromic age-related hearing loss in this population. ) 8. Paterson DL, Robson JMB, Wagener MM, et al. Risk factors for toxicity in elderly patients given aminoglycosides once daily. J Gen Intern Med 1998;13:735. ( A prospective observational study in patients > 70 years of age; limit ing the duration of therapy to < 1 week can reduce the risk of ototoxicity. ) 9. Rauch SD. Idiopathic sudden sensorineural hearing loss. N Engl J Med 2008;359:833. ( Excellent review of this important condition. ) 10. Steel KP. A new era in the genetics of deafness. N Engl J Med 1998;339:1545. ( Though dated, a still excellent discussion of connexin. ) Diagnosis and Workup 11. Begai A, Thavendiranathan P, Detsky AS. Does this patient have hearing impairment? JAMA 2006;295:416. ( Excellent evidence-based review of basic clinical testing. ) 12. Bogardus ST Jr, Yueh B, Shekelle PG. Screening and management of adult hearing loss in primary care: clinical applications. JAMA 2003;289:1986. ( Useful case-based discussions; 42 references. ) 13. Koike KJ, Hurst MK, Wetmore SJ. Correlation between the American Acad emy of Otolaryngology–Head and Neck Surgery five-minute hearing test and standard audiologic data. Otolaryngol Head Neck Surg 1994;111:625. ( Evaluates the sensitivity and specificity of this hearing screen and proposes a modi fication of the cutoff score. ) 14. Lonsbury-Martin BL, Martin GK, McCoy MJ, et al. New approaches to the evaluation of the auditory system and a current analysis of otoacoustic emissions. Otolaryngol Head Neck Surg 1995;112:50. ( A review of otoacoustic emissions and their use in auditory evaluation. ) 15. Moyer VA; on behalf of the U.S. Preventive Services Task Force. Screening for hearing loss in older adults: U.S. Preventive Services Task Force Recom mendation. Ann Intern Med 2012;157:655. ( Finds evidence insufficient to make a recommendation for persons over the age of 50 years, but does endorse assessment in patients who report hearing loss. ) 16. Yueh B, Shapiro N, MacLean CH, et al. Screening and management of adult hearing loss in primary care. JAMA 2003;289:1976. ( A systematic review, find ing that there was no formal confirmation of benefit, but that there are effective screening and treatment modalities; 88 references. ) Management and Prevention 17. Cassel C, Penhoet E, Saunders R. Policy solutions for better hearing. JAMA 2016;315:553. ( Recommendations for improving prevention, access to care, and innovations in treatment. ) 18. Consensus Conference. Noise and hearing loss. JAMA 1990;263:3185. ( Recommends several steps to limiting noise-induced hearing loss. ) 19. Gates GA, Miyamoto RT. Cochlear implants. N Engl J Med 2003;349:421. ( A review of the technology. ) 20. Kitterick OT, Ferguson MA. Hearing aids and health-related quality of life in adults with hearing loss. JAMA 2018;319:2225. ( A summary of available evidence finding hearing aid use associated with better quality of life. ) 21. Larson VD, Williams DW, Henderson WG, et al. Efficacy of 3 commonly used hearing aid circuits: a crossover trial. JAMA 2000;284:1806. ( A very use ful comparison trial; all proved to be beneficial, with only minor differences. ) 22. Pacula JT, Yueh B. Hearing deficits in the older patient. JAMA 2012;307:1185. ( Very useful review focusing on management of geriatric patients with hearing loss; especially good section on hearing aids. ) 23. Rausch SD, Halpin CF, Antonelli PJ, et al. Oral vs intratympanic corticoste roid therapy for idiopathic sudden sensorineural hearing loss: a randomized trial. JAMA 2011;305:2071. ( Noninferiority trial finding intratympanic treatment not inferior. ) 24. Stachler RJ, Chandrasekhar SS, Archer SM, et al. Clinical practice guideline: sud den hearing loss. Otolaryngol Head Neck Surg 2012;146:S1. ( An excellent mul tidisciplinary evidence-based set of recommendations, with a focus on idiopathic disease. )

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Appendix 212-1 Audiometry (1)

Audiometry helps to classify a hearing loss as conductive or sensorineural and subclassify it according to the pattern detected. The basic audiogram consists of pure-tone air and bone con duction testing with evaluation of speech reception threshold

(SRT) and speech discrimination. The minimal intensity (in decibels) at which the patient perceives each tone is charted as the threshold for that frequency. The responses are recorded as indicated in Figure 212-3. The pure-tone air threshold curve