Guideline sources The following summarized guidelines for the evaluation and management of chronic hearing loss are prepared by our editorial team based on guidelines from the American Cochlear Implant Alliance (ACI Alliance 2022), the American College of Medical Genetics (ACMG 2014), and the U.S. Preventive Services Task Force (USPSTF 2012). 1 2 3 4 5 5 6 Definition Chronic hearing loss is the loss of sound threshold sensitivity associated with difficulty in speech discrimination, causing social isolation and cognitive deficit. 4 Epidemiology Chronic hearing loss is caused by degenerative processes associated with aging (presbycusis), genetic mutations, noise exposure, therapeutic drugs (aminoglycosides, cisplatin), and chronic conditions. 5 Disease course Impaired hearing may lead to social isolation due to impaired communication, and is associated with increased risk of depression, falls, hospitalizations, frailty, and dementia. 5 Prognosis and risk of recurrence https://web.pathway.md/diseases/reclstZLJkap2jCLH 1/4 6/29/23, 3:53 AM Chronic hearing loss Pathway Mild hearing loss and moderate/severe hearing loss in the elderly are associated with a 39% and 21% increased risk of mortality, respectively. 6 Guidelines 1. Screening and diagnosis Indications for screening (older adults): insufficient evidence to assess the balance of benefits and harms of screening for hearing loss in asymptomatic adults 50 years of age. I 2. Diagnostic investigations Clinical history: Perform a comprehensive evaluation in all newborns and infants with confirmed hearing loss, including: patient-focused medical and birth histories three-generation pedigree and family medical history physical examination focusing on dysmorphic physical findings. E Show 2 more Genetic testing in patients with syndromic features: Perform the following for individuals with findings that suggest a syndromic genetic etiology for their hearing loss: provide pretest genetic counseling, and, with patient's informed consent, order genetic testing, if available, to confirm the diagnosis undertake appropriate studies to determine whether other organs are involved arrange appropriate near-term and long-term screening and management, including referrals to specialists, as indicated by the associated manifestations of the particular syndrome. Genetic testing in patients without dysmorphic features: use a tiered diagnostic approach for individuals lacking physical findings suggestive of a known syndrome and having medical and birth histories that do not suggest an environmental cause of hearing loss. 3. Therapeutic procedures Cochlear implants: obtain standard medical workup and monitoring in patients with sudden and/or rapid progression of single-sided deadness to determine if the hearing spontaneously improves or is recoverable with treatment. Offer cochlear implantation not earlier than 3-6 months after the sudden hearing loss to allow ample time for potential recovery of hearing. E Show 13 more 4. Patient education https://web.pathway.md/diseases/reclstZLJkap2jCLH 2/4 6/29/23, 3:53 AM Chronic hearing loss Pathway Indications for specialist referral: refer to a multidisciplinary care center, when available. E Clinical findings Patient demographics Symptoms Elderly age Asymmetric hearing loss Dizziness Past medical history Ear pain Feeling of fullness in the ear Autoimmune disorders Hearing loss Cholesteatoma Infection Congenital hearing loss Inner ear hair cell damage Genetic abnormality Tinnitus Inflammation Vertigo Medications M ni re's disease Social history Perforated eardrum SLE Exposure to loud noise Traumatic injury Head and neck exam Neurological exam Conductive hearing loss Sensorineural hearing loss References 1. Alford RL, Arnos KS, Fox M et al. American College of Medical Genetics and Genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss. Genet Med. 2014 Apr;16 4 347 55. Open 2. Moyer VA. Screening USPSTF for hearing loss in older adults: U.S. Preventive Services Task Force recommendation statement. Ann Intern Med. 2012 Nov 6;157 9 655 61. Open 3. Lisa R Park, Amanda M Griffin, Douglas P Sladen et al. American Cochlear Implant Alliance Task Force Guidelines for Clinical Assessment and Management of Cochlear Implantation in Children With Single- Sided Deafness. Ear Hear. 2022 Mar/Apr;43 2 255 267. Open 4. M. Charles Liberman, Leslie D. Liberman, and St phane F. Maison. Chronic Conductive Hearing Loss Leads to Cochlear Degeneration. PLoS One. 2015; 10 11 e0142341. Open 5. Lisa L. Cunningham and Debara L. Tucci. Hearing Loss in Adults. N Engl J Med. 2017 Dec 21; 377 25 2465 2473. Open 6. Kevin J Contrera, Josh Betz, Dane J Genther et al. Association of Hearing Impairment and Mortality in the National Health and Nutrition Examination Survey. JAMA Otolaryngol Head Neck Surg. 2015 Oct;141 10 944 6.2015 Oct;141 10 944 6. Open 7. Kevin J Contrera, Josh Betz, Dane J Genther et al. Association of Hearing Impairment and Mortality in the National Health and Nutrition Examination Survey. JAMA Otolaryngol Head Neck Surg. 2015 https://web.pathway.md/diseases/reclstZLJkap2jCLH 3/4 6/29/23, 3:53 AM Chronic hearing loss Pathway Oct;141 10 944 6. Open 8. Suzanne Cosh, Catherine Helmer, Cecile Delcourt et al. Depression in elderly patients with hearing loss: current perspectives. Clin Interv Aging. 2019 Aug 14;14 1471 1480. Open 9. Carrie L Nieman, Esther S Oh. Hearing Loss. Ann Intern Med. 2020 Dec 1;173 11 ITC81 ITC96. Open https://web.pathway.md/diseases/reclstZLJkap2jCLH 4/4