Pediatric Hearing Loss

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Pediatric Hearing Loss

Pediatric Otolaryngology
Author Credentials 

Ericka King, MD
Assistant Professor
Department of Otolaryngology-Head and Neck Surgery
Oregon Health and Science University
Portland, OR
Kinger@ohsu.edu

Module Summary

Children with hearing loss can be at great risk of developmental delays. Types of hearing loss include conductive hearing loss, sensorineural hearing loss, or mixed hearing loss.  Regardless of type, early diagnosis and therapeutic intervention is critical to give ample opportunity to prohibit these delays from developing.  Hearing loss may be present at birth (congenital) or later in childhood (delayed onset), and may be genetic (syndromic vs nonsyndromic), infectious, ototoxic, traumatic or idiopathic in origin. Accurate diagnosis is essential, and early therapeutic intervention must be offered. In some cases, surgical options may exist to treat the hearing loss, but amplification trials with various types of devices remain an essential part of the treatment armamentarium.

Module Learning Objectives 
  1. Construct a differential diagnosis of pediatric conductive and sensorineural hearing loss.
  2. Describe the impact of hearing loss on speech and language development.
  3. Explain the limitations of universal newborn hearing screening.
  4. Appropriately order and interpret pediatric audiometric studies.
  5. Appropriately order and interpret imaging studies and other diagnostic testing in the evaluation of a child with hearing loss.
  6. Recommend appropriate therapeutic options for children with hearing loss.

Embryology

Learning Objectives 

Understand the embryologic development of the inner, middle and external ears.

References 
  1. Fuchs JC, Tucker AS. Development and integration of the ear. Curr Top Dev Biol. 2015;115:213-32.

Anatomy

Learning Objectives 
  1. Describe the structure of the inner, middle and external ears.
  2. Understand the auditory pathway from the cochlea to the auditory cortex.
References 
  1. Francis HW. Anatomy of the temporal bone, external ear, and middle ear. In: Flint PW, Haughey BH, Lund VJ, Niparko JK, Robbins KT, Thomas JR. Lesperance MM, editors. Cummings Otolaryngology, 6th ed. Philadelphia (PA): Saunders; 2015: 1977-86.
  2. Runge CL, Friedland DR. Anatomy of the auditory system. In: Flint PW, Haughey BH, Lund VJ, Niparko JK, Robbins KT, Thomas JR. Lesperance MM, editors. Cummings Otolaryngology, 6th ed. Philadelphia (PA): Saunders; 2015: 1987-93.

Pathogenesis

Learning Objectives 
  1. Describe the congenital anomalies of the inner ear; how does embryologic week of disruption correlate with severity?
  2. Understand the mechanism and presentation of hearing loss in children with congenital cytomegalovirus infection.
  3. Understand the relationship between enlarged vestibular aqueduct and progression of hearing loss.
  4. Develop an appreciation for the spectrum of defects seen in auditory neuropathy/dyssynchrony.
References 
  1. Huang BY, Zdanski C, Castillo M. Pediatric sensorineural hearing loss, part 1: practical aspects for neuroradiologists. Am J Neuroradiol. 2012;33(2):211-7.
  2. Foulon I, Naessens A, Foulon W, Casteels A, Gordts F. A 10-year prospective study of sensorineural hearing loss in children with congenital cytomegalovirus infection. J Pediatr. 2008;153(1):84-8.
  3. Duval M, Park AH. Congenital cytomegalovirus: what the otolaryngologist should know. Curr Opin Otolaryngol Head Neck Surg. 2014;22(6):495-500.
  4. Greinwald J,  DeAlarcon A, Cohen A, Uwiera T, Zhang K, Benton C, Halstead M, Meinzen-Derr J. Significance of Unilateral Enlarged Vestibular Aqueduct. Laryngoscope. 2013;123(6):1537-1546
  5. Nikolopoulos TP. Auditory dyssynchrony or auditory neuropathy: understanding the pathophysiology and exploring methods of treatment. Int J Pediatr Otolaryngol. 2014;7(2):171-3.

Basic Science

Learning Objectives 
  1. Understand the molecular basis of ototoxicity of common drugs.
  2. Describe what is known about how CMV induces hearing loss.
References 
  1. Duval M, Park AH. Congenital cytomegalovirus: what the otolaryngologist should know. Curr Opin Otolaryngol Head Neck Surg. 2014;22(6):495-500.
  2. Lanvers-Kaminsky C, Zehnhoff-Dinnesen AA, Parfitt R, Ciarimboli G. Drug-induced ototoxicity: Mechanisms, Pharmacogenetics, and protective strategies. Clin Pharmacol Ther. 2017 Apr;101(4):491-500.

Incidence

Learning Objectives 
  1. Generate a differential diagnosis of conductive hearing loss in children, in order of prevalence.
  2. Describe the contribution of genetics to the incidence of congenital and late-onset sensorineural hearing loss in children.
  3. Understand the acquired causes of congenital and late-onset sensorineural hearing loss; which are the most common?
References 
  1. Smith RJH, Bale JF, White KR. Sensorineural hearing loss in children. Lancet. 2005;365(9462):879-90.
  2. Kenna MA. Acquired hearing loss in children. Otolaryngol Clin N Am. 2015;48(6):933-53.
  3. Dougherty W, Kesser BW. Management of conductive hearing loss in children. Otolaryngol Clin N Am. 2015;48(6):955-74.
  4. Chang KW. Genetics of hearing loss – nonsyndromic. Otolaryngol Clin N Am. 2015;48(6):1063-72.
  5. Koffler T, Ushakov K, Avraham KB. Genetics of hearing loss – syndromic. Otolaryngol Clin N Am. 2015;48(6):1041-61.

Genetics

Learning Objectives 
  1. Summarize the most prevalent modes of inheritance for syndromic vs non-syndromic hearing loss. Draw a sample pedigree illustrating autosomal dominant, autosomal recessive, mitochondrial and X-linked inheritance.
  2. Describe the concepts of penetrance and expressivity, and give an example of each using hearing loss genes.
  3. Discuss the most common causes of non-syndromic hearing loss, including GJB2 and SLC26A4, including the hearing phenotypes associated with each.
  4. Describe next generation sequencing for the identification of hearing loss genes, how likely such testing is to identify a genetic mutation, and when to consider ordering it.
References 
  1. Alford RL, Darilek SA. Basic medical genetics for the otolaryngologist. Adv Otorhinolaryngol. 2011;70:10-7.
  2. Chang KW. Genetics of hearing loss – nonsyndromic. Otolaryngol Clin N Am. 2015;48(6):1063-72.
  3. Koffler T, Ushakov K, Avraham KB. Genetics of hearing loss – syndromic. Otolaryngol Clin N Am. 2015;48(6):1041-61.
  4. Alford RL et al. American College of Medical Genetics and Genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss. Genet Med. 2014;16(4):347-55.
  5. Sloan-Heggen CM et al. Comprehensive genetic testing in the clinical evaluation of 1119 patients with hearing loss. Hum Genet. 2016;135(4):441-50. 
  6. Greinwald J et al. Significance of unilateral enlarged vestibular aqueduct. Laryngoscope. 2013;123:1537-46.

Patient Evaluation

Learning Objectives 
  1. Take a comprehensive history and perform a complete physical examination for a child with hearing loss.
  2. Recognize physical exam findings that should elevate suspicion for syndromic hearing loss.
  3. Appropriately order audiometric, laboratory, genetic and imaging testing as needed to establish the etiology of hearing loss in a pediatric patient.
References 
  1. Cushing SL, Papsin BC. Taking the history and performing the physical examination in a child with hearing loss. Otolaryngol Clin N Am. 2015;48(6):903-12.
  2. Duval M, Park AH. Congenital cytomegalovirus: what the otolaryngologist should know. Curr Opin Otolaryngol Head Neck Surg. 2014;22(6):495-500.
  3. Alford RL et al. American College of Medical Genetics and Genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss. Genet Med. 2014;16(4):347-55.
  4. Liming BJ et al. International pediatric otolaryngology group consensus recommendations: hearing loss in the pediatric patient. Int J Pediatr Otorhinolaryngol. 2016;90:251-8.

Measurement of Functional Status

Learning Objectives 
  1. Understand the battery of audiometric testing available for children in different age groups.
  2. Discuss the effects of hearing loss on speech and global development.
  3. Describe the goals of universal newborn hearing screening programs, and contrast the pros and cons of different forms of newborn hearing screening.
  4. Discuss the need for surveillance audiometry in children with hearing loss.
References 
  1. Singleton AJ, Waltzman SB. Audiometric evaluation of children with hearing loss. Otolaryngol Clin N Am. 2015;48(6):891-901.
  2. Tonn CR, Grundfast KM. What an otolaryngologist should know about evaluation of a child referred for delay in speech development. JAMA Otolaryngol Head Neck Surg. 2014;140(3):259-65.
  3. Tharpe AM, Gustafson S. Management of children with mild, moderate, and moderately severe sensorineural hearing loss. Otolaryngol Clin N Am. 2015;48(6):983-994.

Imaging

Learning Objectives 
  1. Construct a paradigm for when to order imaging in the evaluation of a child with hearing loss, and which modality to order. Consider nature of hearing loss (conductive vs sensory vs neural), laterality and severity.
  2. Differentiate between the various cochleovestibular malformations on CT and MRI.
References 
  1. DeMarcantonio M, Choo DI. Radiographic evaluation of children with hearing loss. Otolaryngol Clin N Am. 2015;48(6):913-32.

Treatment

Learning Objectives 
  1. Discuss treatment options for children with congenital conductive hearing loss.
  2. Recognize the need for treatment and contrast rehabilitative vs surgical strategies for the child with single-sided deafness.
  3. Describe amplification options and when to consider cochlear implantation in children with sensorineural hearing loss and auditory neuropathy.
References 
  1. Dougherty W, Kesser BW. Management of conductive hearing loss in children. Otolaryngol Clin N Am. 2015;48(6):955-74.
  2. Tharpe AM, Gustafson S. Management of children with mild, moderate, and moderately severe sensorineural hearing loss. Otolaryngol Clin N Am. 2015;48(6):983-994.
  3. Iseli C, Buchman CA. Management of children with severe, severe-profound, and profound sensorineural hearing loss. Otolaryngol Clin N Am. 2015;48(6):995-1010.
  4. Lieu JEC. Management of children with unilateral hearing loss. Otolaryngol Clin N Am. 2015;48(6):1011-26.

Medical Therapies

Learning Objectives 
  1. Understand the role for medical therapy including antibiotics and intranasal steroids in children with chronic otitis media with effusion.
  2. Discuss the presentation, etiologies, and treatment for sudden sensorineural hearing loss in children.
  3. Summarize the current options and risks for antiviral therapy in children with CMV and hearing loss.
References 
  1. Rosenfeld RM et al. Clinical practice guideline:otitis media with effusion (update). Otolaryngol Head Neck Surg. 2016;154(IS):S1-41.
  2. Duval M, Park AH. Congenital cytomegalovirus: what the otolaryngologist should know. Curr Opin Otolaryngol Head Neck Surg. 2014;22(6):495-500.
  3. Kenna MA. Acquired hearing loss in children. Otolaryngol Clin N Am. 2015;48(6):933-53.

Surgical Therapies

Learning Objectives 
  1. Contrast the surgical options in the treatment of aural atresia, and the pros, cons and risks of each.
  2. Describe the surgical approach for cochlear implantation.
  3. Summarize the arguments for and against bilateral cochlear implantation in children.
  4. Discuss the speech and language outcomes associated with cochlear implantation in children with sensorineural hearing loss and auditory neuropathy spectrum disorder.
References 
  1. Dougherty W, Kesser BW. Management of conductive hearing loss in children. Otolaryngol Clin N Am. 2015;48(6):955-74.
  2. Nadaraja S, Gurgel RK, Kim J, Chang KW. Hearing outcomes of atresia surgery versus osseointegrated bone conduction device in patients with congenital aural atresia: a systematic review. Otol Neurotol. 2013;34(8):1394-9.
  3. Papsin BC, Gordon KA. Cochlear implants for children with severe-to-profound hearing loss. NEJM. 2007;357(23):2380-7.
  4. Harrison RV et al. Auditory neuropathy spectrum disorder (ANSD) and cochlear implantation. Int J Pediatr Otorhinolaryngol. 2015;79:1980-7.
  5. Papsin BC, Gordon KA. Bilateral cochlear implants should be the standard of children with bilateral sensorineural deafness. Curr Opin Otolaryngol Head Neck Surg. 2008;16:69-74.
  6. Ganek H, Robbins AM, Niparko JK. Language outcomes after cochlear implantation. Otolaryngol Clin N Am. 2012;45:173-85.

Rehabilitation

Learning Objectives 
  1. Correctly identify parts of a behind-the-ear hearing aid.
  2. Discuss the language outcomes expected in children wearing hearing aids.
References 
  1. Tharpe AM, Gustafson S. Management of children with mild, moderate, and moderately severe sensorineural hearing loss. Otolaryngol Clin N Am. 2015;48(6):983-994.
  2. Tomblin JB et al. Language outcomes in young children with mild to severe hearing loss. Ear Hear. 2015;36(S1):76S-91S.

Case Studies

  1. A 6-week-old girl presents with a failed newborn hearing screening by otacoustic emission testing. On diagnostic natural-sleep ABR, she is noted to have bilateral severe-to-profound sensorineural hearing loss.  Her family history is negative for early onset hearing loss, and her physical examination is otherwise unremarkable.  What is the most likely cause of her hearing loss? What other entities should be considered in the differential diagnosis? What further testing should be performed to delineate the nature of her loss? What other specialists should she see?
  2. A 14-year-old male presents with a history of right-sided progressive hearing loss, that was moderately-severe with a mixed conductive component in the low frequencies at his last test 6 months ago. He now presents with a moderate left-sided high frequency sensorineural hearing loss that started suddenly 1 week ago after playing tackle football. He has no family history of hearing loss, and no further diagnostic workup has been done.  What might you expect to see on CT temporal bone? What treatment would you consider at this time? Is there any additional evaluation you would recommend?
  3. A 6-year-old boy with single-sided deafness, autism, developmental delays and sensory issues presents for consideration of treatment. What options are available? What are the pros and cons for each?

Complications

Learning Objectives 
  1. Discuss the treatment of otitis media in children with cochlear implants.
  2. Summarize the complications seen in bone-anchored hearing aid surgery, abutment vs abutment-free magnetic systems.
  3. List the complications seen in aural atresia repair.
References 
  1. Rubin LG, Papsin B et al. Cochlear implants in children: surgical site infections and prevention and treatment of acute otitis media and meningitis. Pediatrics. 2010;126(2):381-91.
  2. Kraai T, Brown C, Neeff M, Fisher K. Complications of bone-anchored hearing aids in pediatric patients. Int J Pediatr Otorhinolaryngol. 2011;75:749-53.
  3. Baker S, Centric A, Chennupati SK. Innovation in abutment-free bone-anchored hearing devices in children: updated results and experience. Int J Pediatr Otorhinolaryngol. 2015;79(10):1667-72.
  4. Jovankovicova A et al. Surgery or implantable hearing devices in children with congenital aural atresia: 25 years of our experience. Int J Pediatr Otorhinolaryngol. 2015;79(7):975-9.

Review

Review Questions 
  1. What is the most common cause of pediatric hearing loss?
  2. What are the impacts of unilateral hearing loss on speech development and school performance?
  3. What is the correlation between an enlarged vestibular aqueduct and hearing loss, in terms of laterality, severity and progression?
  4. What is the likelihood of detecting a pathogenic mutation in a hearing loss gene in a child with unilateral sensorineural hearing loss?
References 
  1. Dougherty W, Kesser BW. Management of conductive hearing loss in children. Otolaryngol Clin N Am. 2015;48(6):955-74.
  2. Lieu JEC. Management of children with unilateral hearing loss. Otolaryngol Clin N Am. 2015;48(6):1011-26.
  3. Greinwald J et al. Significance of unilateral enlarged vestibular aqueduct. Laryngoscope. 2013;123:1537-46.
  4. Sloan-Heggen CM et al. Comprehensive genetic testing in the clinical evaluation of 1119 patients with hearing loss. Hum Genet. 2016;135(4):441-50. 
Resources 

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