Sensorineural Hearing Loss

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Sensorineural Hearing Loss

Otology/Audiology
Author Credentials 

Jason Barnes, M.D.
200 First Street SW
Rochester, MN 55905
Phone: (507) 284-3856
Email: barnes.jason@mayo.edu 

Neil Patel, M.D.
200 First Street SW
Rochester, MN 55905
Phone: (507) 284-3856
Email: neilsubodh@gmail.com  

Matthew Carlson, M.D.
200 First Street SW
Rochester, MN 55905
Phone: (507) 284-3856
Email: carlson.matthew@mayo.edu  

Module Summary

Sensorineural hearing loss affects a broad range of people and has a diverse etiology. It can be caused by congenital defects, insults in the perinatal period, familial causes, noise exposure, and can be a natural part of aging, among many other causes. Additionally, it can occur suddenly in unexpecting, otherwise healthy patients. In all situations, a thorough history, head and neck evaluation with otoscopy and an audiogram are reasonable first steps to evaluate the cause, type, and magnitude of hearing loss. In turn, this information will inform management options and counseling. In many instances, conventional hearing aid amplification may be beneficial. In patients with bilateral, more severe sensorineural hearing loss, cochlear implantation may be a viable option. In other scenarios, particularly with asymmetric hearing loss, cerebellopontine angle tumors must be considered. This outline reviews and explores the myriad causes of sensorineural hearing loss and provides evaluation and treatment recommendations specific to the patient presentation and pathology.

Module Learning Objectives 
  1. Review the anatomy and embryology of the inner ear.
  2. Describe the physiology of hearing.
  3. Recognize the common congenital malformations of the inner ear.
  4. Cite the most common causes of acquired sensorineural hearing loss in children.
  5. Review the common causes of sensorineural hearing loss in adults.
  6. Explain the nuances of diagnosis and treatment of sudden sensorineural hearing loss.
  7. Identify the prevalence and pathophysiology of presbycusis.
  8. Describe the most common treatment for sensorineural hearing loss, including risks and benefits.

 

Embryology

Learning Objectives 
  1. Describe the process and timing of the development of the inner ear in utero.
References 
  1. “Development of the Ear.” Bailey's Head and Neck Surgery - Otolaryngology, by Byron J. Bailey et al., Wolters Kluwer, Lippincott Williams Et Wilkins, 2014.

Anatomy

Learning Objectives 
  1. Review the anatomy of the inner ear including the cochlea, scala tympani, scala vestibuli, organ of corti, outer hair cells, and inner hair cells.
References 

Alberti, P. (2001) The anatomy and physiology of the ear and hearing. Evaluation, prevention and control, 53-62, 2001. Retrieved April, 6 2019 from https://www.who.int/occupational_health/publications/noise2.pdf

Pathogenesis

Learning Objectives 
  1. Recognize the common etiologies of congenital hearing loss and their distributions. 
  2. Identify the pharmacologic agents most commonly associated with ototoxicity.
  3. List the common congenital malformations of the inner ear.
  4. Describe auditory neuropathy spectrum disorder.
  5. Understand the process of noise-induced hearing loss; compare and contrast noise-induced hearing loss to presbycusis.
  6. Report the possible etiologies and pathogenesis of sudden sensorineural hearing loss.
  7. Define the process of presbycusis.

 

References 
  1. Kenna MA. Acquired Hearing Loss in Children. Otolaryngol Clin North Am. 2015;48(6):933-53.
  2. Demarcantonio M, Choo DI. Radiographic Evaluation of Children with Hearing Loss. Otolaryngol Clin North Am. 2015;48(6):913-32.
  3. Harrison RV, Gordon KA, Papsin BC, Negandhi J, James AL. Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation. Int J Pediatr Otorhinolaryngol. 2015;79(12):1980-7.
  4. Stachler RJ, Chandrasekhar SS, Archer SM, et al. Clinical practice guideline: sudden hearing loss. Otolaryngol Head Neck Surg. 2012;146(3 Suppl): S1-35.
  5. Wattamwar K, Qian ZJ, Otter J, et al. Increases in the Rate of Age-Related Hearing Loss in the Older Old. JAMA Otolaryngol Head Neck Surg. 2017;143(1):41-45.

 

Basic Science

Learning Objectives 
  1. Describe the physiology of hearing including the components of the outer, middle and inner ear, in addition to the afferent central auditory pathway.
References 
  1. Alberti, P. (2001) The anatomy and physiology of the ear and hearing. Evaluation, prevention and control, 53-62, 2001. Retrieved April, 6 2019 from https://www.who.int/occupational_health/publications/noise2.pdf

Incidence

Learning Objectives 
  1. Describe the epidemiology congenital hearing loss.
  2. Explain the epidemiology of noise-induced hearing loss and recommendations for prevention. 
  3. Discuss the epidemiology of sudden hearing loss.   

 

References 
  1. Kenna MA. Acquired Hearing Loss in Children. Otolaryngol Clin North Am. 2015;48(6):933-53.
  2. World Health Organization. (2015) Hearing loss due to recreational exposure to loud sounds. Retrieved on April 6, 2015 from  https://apps.who.int/iris/bitstream/handle/10665/154589/9789241508513_eng.pdf;jsessionid=A7333DD45FB2C4A1F0A24074D7601959?sequence=1.
  3. Stachler RJ, Chandrasekhar SS, Archer SM, et al. Clinical practice guideline: sudden hearing loss. Otolaryngol Head Neck Surg. 2012;146(3 Suppl): S1-35.

 

Genetics

Learning Objectives 
  1. Describe the most common genetic variants associated with congenital sensorineural hearing loss.
References 
  1. Chang KW. Genetics of Hearing Loss--Nonsyndromic. Otolaryngol Clin North Am. 2015;48(6):1063-72.
  2. Koffler T, Ushakov K, Avraham KB. Genetics of Hearing Loss: Syndromic. Otolaryngol Clin North Am. 2015;48(6):1041-61.

 

Patient Evaluation

Learning Objectives 
  1. Describe patterns of sensorineural hearing loss and their associations. 
  2. Explain the process of newborn hearing screening. 
  3. Define the mechanism of Otoacoustic Emission.   
  4. Identify the characteristic findings of presbycusis. 
  5. Recall the appropriate evaluation of a patient with sudden hearing loss. 
  6. Cite several proposed definitions of asymmetric hearing loss.
  7. Describe audiologic features suggestive of retrocochlear pathology. 

 

References 
  1. Walker JJ, Cleveland LM, Davis JL, Seales JS. Audiometry screening and interpretation. Am Fam Physician. 2013;87(1):41-7.
  2. Year 2007 position statement: Principles and guidelines for early hearing detection and intervention programs. Pediatrics. 2007;120(4):898-921.
  3. Lonsbury-martin BL, Martin GK. Otoacoustic emissions. Curr Opin Otolaryngol Head Neck Surg. 2003;11(5):361-6.
  4. Nelson EG, Hinojosa R. Presbycusis: a human temporal bone study of individuals with downward sloping audiometric patterns of hearing loss and review of the literature. Laryngoscope. 2006;116(9 Pt 3 Suppl 112):1-12.
  5. Stachler RJ, Chandrasekhar SS, Archer SM, et al. Clinical practice guideline: sudden hearing loss. Otolaryngol Head Neck Surg. 2012;146(3 Suppl): S1-35.
  6. Saliba I, Bergeron M, Martineau G, Chagnon M. Rule 3,000: a more reliable precursor to perceive vestibular schwannoma on MRI in screened asymmetric sensorineural hearing loss. Eur Arch Otorhinolaryngol. 2011;268(2):207-12.
  7. Sweeney AD, Carlson ML, Shepard NT, et al. Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines on Otologic and Audiologic Screening for Patients With Vestibular Schwannomas. Neurosurgery. 2018;82(2): E29-E31.

 

Imaging

Learning Objectives 
  1. Describe the radiologic findings of congenital inner ear malformations. 
  2. Identify the possible radiologic findings of unilateral or asymmetric sensorineural hearing loss. 

 

References 
  1. Casselman JW, Offeciers EF, De foer B, Govaerts P, Kuhweide R, Somers T. CT and MR imaging of congential abnormalities of the inner ear and internal auditory canal. Eur J Radiol. 2001;40(2):94-104.
  2. Sennaroglu L, Saatci I. A new classification for cochleovestibular malformations. Laryngoscope. 2002;112(12):2230-41.
  3. Daniels RL, Swallow C, Shelton C, Davidson HC, Krejci CS, Harnsberger HR. Causes of unilateral sensorineural hearing loss screened by high-resolution fast spin echo magnetic resonance imaging: review of 1,070 consecutive cases. Am J Otol. 2000;21(2):173-80.

 

Treatment

Learning Objectives 
  1. Read the current labeling guidelines for cochlear implantation in children and adults and understand areas of expanding indications.
  2. Cite the recommended treatment for sudden sensorineural hearing loss. 

 

References 
  1. ASHA, Working group on Cochlear Implants. “Cochlear Implants Evaluation and Candidacy.” Cochlear Implants. Retrieved on April 6, 2019 from https://www.asha.org/policy/tr2004-00041/#sec1.5
  2. Stachler RJ, Chandrasekhar SS, Archer SM, et al. Clinical practice guideline: sudden hearing loss. Otolaryngol Head Neck Surg. 2012;146(3 Suppl): S1-35.

 

Surgical Therapies

Learning Objectives 
  1. Recognize the indications and risks of cochlear implantation.
References 
  1. ASHA, Working group on Cochlear Implants. “Cochlear Implant Surgery.” Cochlear Implants. Retrieved on April 6, 2019 from https://www.asha.org/policy/tr2004-00041/#sec1.5

 

Rehabilitation

Learning Objectives 
  1. Discuss the effects of hearing loss on quality of life.
  2. Describe the primary and secondary implications of untreated hearing loss.

 

References 
  1. Roland L, Fischer C, Tran K, Rachakonda T, Kallogjeri D, Lieu JE. Quality of Life in Children with Hearing Impairment: Systematic Review and Meta-analysis. Otolaryngol Head Neck Surg. 2016;155(2):208-19.
  2. Brodie A, Smith B, Ray J. The impact of rehabilitation on quality of life after hearing loss: a systematic review. Eur Arch Otorhinolaryngol. 2018;275(10):2435-2440.

 

Case Studies

  1. A 36-year-old female presents to the clinic with unilateral, right-sided hearing loss that suddenly developed two days prior. She describes the hearing loss as a “muffled” sound in the right ear that is constant. She also reports high-frequency ringing and a feeling of fullness in the same ear. An audiogram performed that same day demonstrates a normal pure-tone audiometry (PTA) and speech recognition thresholds (SRT) on the left. On the right, pure tone thresholds at 500 Hz, 1000 Hz, 2000 Hz and 4000 Hz are 55 dB, 60 dB, 60 dB and 55 dB, respectively and otoscopic examination reveals a normal appearing tympanic membrane and a well-aerated middle ear space. Explain your next step in evaluation, including the need for imaging. Describe accepted treatment regimens and the possible pathogenesis. 
  2. A 74-year-old man presents to the clinic with a 10-year history of progressively worsening hearing loss. He states that over several years, he has had more difficulty understanding what people are saying to him, particularly at restaurants and social gatherings. An audiogram is obtained, which demonstrates mild to moderate down-sloping sensorineural hearing loss that is symmetrical, in addition to 90% word recognition in each ear. Describe the benefits and drawbacks of conventional hearing aid amplification for this patient. Explain the likely pathogenesis of his hearing loss. Discuss the optimal management of his hearing loss.

Complications

Learning Objectives 
  1. Explain the possible drawbacks of noise amplification.
References 
  1. FDA. “Other Risks Associated with the Use of Cochlear Implants.” Benefits and Risks of Cochlear Implants. Retrieved on April 9, 2019 from https://www.fda.gov/medicaldevices/productsandmedicalprocedures/implantsandprosthetics/cochlearimplants/ucm062843.htm

Review

Review Questions 
  1. What is the process of the newborn hearing screen?
  2. What are the benefits to early noise amplification for children?
  3. What is the most common pathogenesis of presbycusis?
  4. What are the criteria for cochlear implantation in adults?
  5. What are the criteria for cochlear implantation in children?
  6. What is the definition of sudden sensorineural hearing loss and what is the recommended treatment?
  7. What are the clinical features of a patient with sensorineural hearing loss from a cerebellopontine angle tumor?

 

Resources 

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  1. Annual Meeting Webcast (AMW): 
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  3. Otolaryngology Patient Scenarios (OPS):