Hearing Impairment Treatment & Management
- Author: Rahul K Shah, MD, FACS, FAAP; Chief Editor: Glenn C Isaacson, MD more...
Conductive hearing loss
Manage conductive hearling loss (CHL) due to otitis media or its sequelae with a course of appropriate antibiotics. Patients with serous otitis media for longer than 3 months benefit from myringotomy and removal of the fluid in the middle ear. Ventilation tubes may ultimately be necessary. If the hearing loss continues, amplification with a hearing aid may be needed. Speech therapy is rarely necessary unless the loss is prolonged and cannot be corrected with amplification.
CHL that results from obstruction of the auditory canal because of cerumen or a foreign body should be treated by removing the obstruction.
Sensorineural hearing loss
Sensorineural hearing loss (SNHL) cannot be medically treated. In mild-to-moderate hearing loss, amplification with hearing aids is used to give the child as much auditory input as possible. Speech therapy may be beneficial. If the child requires special schooling, the school should determine how much speech training is routinely part of the school day. Preferential seating and use of FM systems should be discussed with the patient's family and teachers.
In older children and in adults with moderate-to-profound hearing loss, hearing aids may correct up to 40-60 dB. Beyond that, the limiting factor is the physical sound pressure exerted on the tympanic membrane, which becomes painful after a certain threshold. Young children with small ear canals may perceive pain at amplification volumes as low as 10-15 dB. Modern hearing aids can selectively amplify a specified range of frequencies more than others rather than all frequencies equally.
There are two main goals of amplification. The first is to provide language. After the hearing aid is fitted by using proper molds, the hearing aid is tested to see how well it matches the goals for loudness at various frequencies. With older children, speech recognition should be part of this testing. For young children, the goal is to optimize auditory input without causing pain. If the hearing aids are painful to use, children will avoid using them. If amplification is successful in providing improved spoken language comprehension without pain, its use in and out of school should be encouraged.
The second goal of amplification is to provide environmental cues. The use of hearing aids helps to connect young children to their environment, helps to maximize auditory language development if it helps them to hear any speech sounds, and uses auditory pathways to the brain, which may prevent the brain from “ignoring” them (as it does in cortically blinded laboratory animals). The ability to hear environmental sounds is important for safety and some general functioning. Important safety cues include car or truck horns, alarms, or even someone yelling “stop.” Functional cues might include class bells, oven timers, doorbells, or someone calling their name loudly.
Older children may choose not to use their hearing aids because they “don’t look cool.” Rather than making this a significant ongoing argument, parents should be reasonable. For example, if their child is succeeding in school, the hearing aids may not offer a substantial language benefit to their child’s functioning. Additionally, the child is likely safe in the classroom, so benefit might be gained by letting the child “win.” If the child prefers not to wear the hearing aids after school, parents should respect this decision if the child is participating in safe activities when he or she does not have increased risk of harm because of missed environmental cues.
There is no medical disadvantage for children choosing to not use hearing aids. In fact, many deaf adults use their hearing aids selectively or not at all because they find the extraneous noises and distortions they hear are more bothersome than helpful. They may decide to use their hearing aids only when they anticipate a particular benefit.
Conductive hearing loss
Some causes of CHL may be managed or aided surgically. Children with persistent chronic or recurrent otitis media with resultant effusions may benefit from the placement of myringotomy tubes to ventilate the middle-ear space to prevent negative pressure in this area. If otitis results in the destruction or fixation of the ossicles, surgery may improve ossicular function. Cholesteatoma is a surgical disease.
Bone-anchored hearing aids (BAHAs) may be useful in some patients. Examples are patients with microtia, those with anotia who are awaiting auricular reconstruction, and patients with persistent otorrhea who cannot use a hearing aid.
Sensorineural hearing loss
SNHL cannot be treated with surgical means other than cochlear implantation. Cochlear implantation may be an option in some children, but it should not be mistaken for a cure.
Consulting an otolaryngologist is imperative if the child has CHL. An otolaryngologist can provide advice pertaining to medical and potential surgical interventions. Consultation is also recommended if the child has profound SNHL and is a potential candidate for cochlear implants. The otolaryngologist is a crucial member of the multidisciplinary team needed to help patients with profound SNHL.
Specialists in early intervention
Early intervention programs are essential to help parents understand how to raise a deaf child or one hard of hearing. Such programs are also needed to begin discussing and implementing language and/or educational programs.
Consulting an audiologist is essential for evaluating patients for hearing aids and for fitting them.
Consultation with a geneticist is recommended if the cause of deafness may be syndromic or if the family history suggests a hereditary pattern.
Ophthalmologist, nephrologist, cardiologist, or other subspecialty consultants
Consulting these subspecialists is recommended if an identifiable syndrome implicates involvement of the visual, renal, cardiac, or any other organ system (eg, a deaf child with hematuria should see a nephrologist to check for Alport syndrome).
Similarly, if involvement of an organ system is anticipated because a child is diagnosed with a particular syndrome, a specialist may need to be involved (eg, a deaf child may benefit from aggressive ophthalmologic screening for the development of retinitis pigmentosa as in Usher syndrome, or simply because vision is so important to communication that mild losses in visual acuity should be managed promptly).
Occasionally, a specialist may be able to identify abnormalities that may lead to the diagnosis of a specific syndrome that would not be seen without specialized equipment (eg, ophthalmologist for a funduscopic examination or cardiologist for ECG and/or echocardiography).
Because almost any organ system is potentially related to hearing loss, there is no need to consult every subspecialist for each child with hearing loss. As noted earlier, even routine ECG testing for prolonged a QT interval or laboratory testing for the development of nephritis or changes in blood urea nitrogen and creatinine are not recommended universally.
Experts in managing hearing loss
Consult physicians with expertise in caring for patients with hearing loss, if available in your community. Some physicians have developed expertise in the field of deafness and may be available for consultation. They can offer information about associated medical conditions if present, as well as perspective about language and education, use of hearing aids and cochlear implants, and other equipment. The literature is filled with debates about the most appropriate educational venues for children who are deaf or hard of hearing. Many people involved in early intervention are affiliated with a particular program because it matches their personal biases.
Physicians with expertise in deafness may be more neutral. However, there is a clear bias in the literature towards spoken language as the outcome measure defining success, and relatively good literature about the success of an all-sign-language education (even the literature on reading ability can be questioned).
Follow-up of the interventions is as important in hearing impairment as in any other disability or medical condition. Physicians too frequently relegate the care of children who are deaf to audiologists and educators. Children who are deaf need ongoing referrals to an audiologist to monitor the progression of their hearing loss and to refit hearing aids to match changing losses and/or growth of their ears.
Pediatricians should monitor the child's linguistic and social development. They should ask about language and school performance. The child's placement in school may not be optimal for his or her abilities. For instance, a child who is not successfully learning lip-reading cannot learn math or science. Pediatricians should ask how the child is doing in school and in the family, how family members interact with the child, and how the parents discipline or instruct the child.
Children who cannot communicate with those around them may be frustrated and, therefore, act out or display withdrawal behaviors. These behaviors may be misinterpreted as being a behavioral or psychological problem rather than being reactions to the child's environment or situation.
Children who are deaf or hard of hearing are at particular risk for abuse. Physical abuse may be inflicted by parents who are frustrated because their child is not acting as they expect. Also, children who perpetrators perceive as being unable to report misconduct are at high risk for sexual abuse. Furthermore, most children with hearing impairment have no physical disabilities and may be attractive to a potential perpetrator. Deaf children may be socially isolated because of the communication barrier, they may be susceptible to individuals who give them special attention. Watch for physical signs of abuse or for behavioral manifestations of child abuse.
Morton NE. Genetic epidemiology of hearing impairment. Ann N Y Acad Sci. 1991. 630:16-31. [Medline].
Erenberg A, Lemons J, Sia C, Trunkel D, Ziring P. Newborn and infant hearing loss: detection and intervention.American Academy of Pediatrics. Task Force on Newborn and Infant Hearing, 1998- 1999. Pediatrics. 1999 Feb. 103(2):527-30. [Medline].
Johnson JL, White KR, Widen JE, et al. A multicenter evaluation of how many infants with permanent hearing loss pass a two-stage otoacoustic emissions/automated auditory brainstem response newborn hearing screening protocol. Pediatrics. 2005 Sep. 116(3):663-72. [Medline].
Katbamna B, Crumpton T, Patel DR. Hearing impairment in children. Pediatr Clin North Am. 2008 Oct. 55(5):1175-88, ix. [Medline].
Kenna MA. Neonatal hearing screening. Pediatr Clin North Am. 2003 Apr. 50(2):301-13. [Medline].
Kerschner JE. Neonatal hearing screening: to do or not to do. Pediatr Clin North Am. 2004 Jun. 51(3):725-36, x. [Medline].
Berg AL, Spitzer JB, Towers HM, Bartosiewicz C, Diamond BE. Newborn hearing screening in the NICU: profile of failed auditory brainstem response/passed otoacoustic emission. Pediatrics. 2005 Oct. 116(4):933-8. [Medline].
Cohn ES, Kelley PM, Fowler TW, et al. Clinical studies of families with hearing loss attributable to mutations in the connexin 26 gene (GJB2/DFNB1). Pediatrics. 1999 Mar. 103(3):546-50. [Medline].
Joint Committee on Infant Hearing, American Academy of Audiology, American Academy of Pediatrics, American Speech-Language-Hearing Association, and Directors of Speech and Hearing Programs in State Health and Welfare Agencies. Year 2000 position statement: principles and guidelines for early hearing detection and intervention programs. Pediatrics. 2000 Oct. 106(4):798-817. [Medline].
American Academy of Pediatrics, Joint Committee on Infant Hearing. Year 2007 position statement: Principles and guidelines for early hearing detection and intervention programs. Pediatrics. 2007 Oct. 120(4):898-921. [Medline].
US Preventive Services Task Force. Universal screening for hearing loss in newborns: US Preventive Services Task Force recommendation statement. Pediatrics. 2008 Jul. 122(1):143-8. [Medline].
Nelson HD, Bougatsos C, Nygren P. Universal newborn hearing screening: systematic review to update the 2001 US Preventive Services Task Force Recommendation. Pediatrics. 2008 Jul. 122(1):e266-76. [Medline].
Kennedy CR, McCann DC, Campbell MJ, et al. Language ability after early detection of permanent childhood hearing impairment. N Engl J Med. 2006 May 18. 354(20):2131-41. [Medline].
Alford RL, Arnos KS, Fox M, et al, ACMG Working Group on Update of Genetics Evaluation Guidelines for the Etiologic Diagnosis of Congenital Hearing Loss, Professional Practice and Guidelines Committee. 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. [Medline].
Roizen NJ. Etiology of hearing loss in children. Nongenetic causes. Pediatr Clin North Am. 1999 Feb. 46(1):49-64, x. [Medline].
McGee J, Walsh EJ. Cochlear Transduction and the Molecular Basis of Auditory Pathology. Cummings Otolaryngology: Head & Neck Surgery. 5th ed. St. Louis, Mo: Mosby; 2010. Chapter 146.
Hildebrand MS, Husein M, Smith RJH. Cochlear Genetic Sensorineural Hearing Loss. Cummings Otolaryngology: Head & Neck Surgery. 5th ed. St. Louis, Mo: Mosby; 2010. Chapter 147.
Marlin S, Garabédian EN, Roger G, et al. Connexin 26 gene mutations in congenitally deaf children: pitfalls for genetic counseling. Arch Otolaryngol Head Neck Surg. 2001 Aug. 127(8):927-33. [Medline].
Tashiro K, Konishi H, Sano E, Nabeshi H, Yamauchi E, Taniguchi H. Suppression of the ligand-mediated down-regulation of epidermal growth factor receptor by Ymer, a novel tyrosine-phosphorylated and ubiquitinated protein. J Biol Chem. 2006 Aug 25. 281(34):24612-22. [Medline].
Modamio-Hoybjor S, Mencia A, Goodyear R, et al. A mutation in CCDC50, a gene encoding an effector of epidermal growth factor-mediated cell signaling, causes progressive hearing loss. Am J Hum Genet. 2007 Jun. 80(6):1076-89. [Medline]. [Full Text].
Bondurand N, Pingault V, Goerich DE, et al. Interaction among SOX10, PAX3 and MITF, three genes altered in Waardenburg syndrome. Hum Mol Genet. 2000 Aug 12. 9(13):1907-17. [Medline].
Richardson SO. The child with "delayed speech". Contemp Pediatr. 1992. 9(9):55.
Shargorodsky J, Curhan SG, Curhan GC, Eavey R. Change in prevalence of hearing loss in US adolescents. JAMA. 2010 Aug 18. 304(7):772-8. [Medline].
Harrison M, Roush J, Wallace J. Trends in age of identification and intervention in infants with hearing loss. Ear Hear. 2003 Feb. 24(1):89-95. [Medline].
Smith RJ, Hone S. Genetic screening for deafness. Pediatr Clin North Am. 2003 Apr. 50(2):315-29. [Medline].
Genetic Evaluation of Congenital Hearing Loss Expert Panel. Genetics Evaluation Guidelines for the Etiologic Diagnosis of Congenital Hearing Loss. Genetic Evaluation of Congenital Hearing Loss Expert Panel. ACMG statement. Genet Med. 2002 May-Jun. 4(3):162-71. [Medline].
Choung YH, Moon SK, Park HJ. Functional study of GJB2 in hereditary hearing loss. Laryngoscope. 2002 Sep. 112(9):1667-71. [Medline].
Gasparini P, Rabionet R, Barbujani G, et al. High carrier frequency of the 35delG deafness mutation in European populations. Genetic Analysis Consortium of GJB2 35delG. Eur J Hum Genet. 2000 Jan. 8(1):19-23. [Medline].
Green GE, Scott DA, McDonald JM, Woodworth GG, Sheffield VC, Smith RJ. Carrier rates in the midwestern United States for GJB2 mutations causing inherited deafness. JAMA. 1999 Jun 16. 281(23):2211-6. [Medline].
Parry DA, Booth T, Roland PS. Advantages of magnetic resonance imaging over computed tomography in preoperative evaluation of pediatric cochlear implant candidates. Otol Neurotol. 2005 Sep. 26(5):976-82. [Medline].
[Guideline] Joint Committee on Infant Hearing. Year 2007 position statement: Principles and guidelines for early hearing detection and intervention programs. Pediatrics. 2007 Oct. 120(4):898-921. [Medline].
Joint Committee on Infant Hearing of the American Academy of Pediatrics, Muse C, Harrison J, Yoshinaga-Itano C, et al. Supplement to the JCIH 2007 position statement: principles and guidelines for early intervention after confirmation that a child is deaf or hard of hearing. Pediatrics. 2013 Apr. 131 (4):e1324-49. [Medline].
Berlin CI, Morlet T, Hood LJ. Auditory neuropathy/dyssynchrony: its diagnosis and management. Pediatr Clin North Am. 2003 Apr. 50(2):331-40, vii-viii. [Medline].
Brookhouser PE, Beauchaine KL, Osberger MJ. Management of the child with sensorineural hearing loss. Medical, surgical, hearing aids, cochlear implants. Pediatr Clin North Am. 1999 Feb. 46(1):121-41. [Medline].
American Academy of Pediatrics Joint Committee on Infant Hearing. Joint Committee on Infant Hearing 1994 Position Statement. Pediatrics. 1995 Jan. 95(1):152-6. [Medline].
Horn RM, Nozza RJ, Dolitsky JN. Audiological and medical considerations for children with cochlear implants. Am Ann Deaf. 1991 Apr. 136(2):82-6. [Medline].
|Organ or System||Syndrome||Inheritance Pattern||Hearing Loss||Obvious Physical Abnormalities|
|External ear||DiGeorge sequelae||Sporadic||CHL||Yes|
|Cardiac||Coloboma, heart disease, atresia choanae, retarded growth, and ear anomalies (CHARGE) syndrome||AD, AR, X linked, sporadic||SNHL, mixed||Yes|
|Jervell Lange-Nielson syndrome||AR||SNHL||No|
|Renal||Alport syndrome||AD, AR, X linked||SNHL||Yes or no|
|Branchio-oto-renal syndrome||AD||SNHL, CHL||Yes|
|Mental (retardation)||Noonan syndrome||Sporadic||SNHL||Yes|
|Cockayne syndrome, type I||AR||SNHL||Yes|
|Gustavson syndrome||X linked||SNHL||Yes|
|Lentigines, ECG, ocular, pulmonary, abnormal, retardation, and deafness (LEOPARD) syndrome||AD||SNHL||Yes|
|Black locks with albinism and deafness (BADS) syndrome||AR||SNHL||Yes|
|Endocrine and/or metabolic||Pendred syndrome||AR||SNHL||Yes or no|
|Wolfram syndrome||AR||SNHL||Yes or no|
|Kallmann syndrome||AD, AR, X linked||SNHL, mixed||Yes or no|
|Facial||Goldenhar syndrome||AD, AR||CHL, SNHL||Yes|
|Frontometaphyseal dysplasia||X linked||Mixed||Yes|
|Ophthalmologic||Usher syndrome||AR||SNHL||Yes or no|
|Harboyan syndrome||AR||SNHL||Yes or no|
|Jensen syndrome||X linked||SNHL||No|
|Orthopedic||Klippel-Feil sequelae||Sporadic||CHL, SNHL||Yes|
|Stickler syndrome||AD||CHL, SNHL, mixed||Yes|
|Craniometaphyseal dysplasia||AD, AR||CD||Yes|
|Oto-spondylo-megaepiphyseal dysplasia (OSMED) syndrome||AR||SNHL||Yes|