Medical conditions of hearing loss in children - Cochlear™ India

There is a wide range of medical conditions which have hearing loss as a symptom or outcome.

Young girl in the park

On this page you’ll find a summary of some of the more common medical conditions that can lead to hearing loss in children. Please note, this is an overview and not an exhaustive list. Please consult your doctor or specialist for more detailed information on these or other conditions.

Atresia/microtia

Atresia is the absence of an external ear canal, and is often accompanied by microtia, where the external ear develops abnormally. The malformations of atresia/microtia can cause a conductive hearing loss. Atresia/microtia usually happens in isolation, but it can also occur as a symptom of syndromes such as Goldenhar and Treacher Collins. The reported incidence of atresia varies between one in every 10,000 and one in every 20,000 children1, 2 and is twice as common in boys as girls1. For 85% of children with atresia, the condition is unilateral2, or affecting only one side. However, when children have atresia as part of a syndrome, they have a 50% likelihood of being affected in both ears2.

Chronic Otitis Media

Otitis media (OM) is a middle ear infection which mostly affects children - it’s the most common childhood disease in the world. By the age of three, more than 83% of children will have experienced at least one episode of otitis media, while 46% of these children will have had three or more incidents. The stages of otitis media are diagnosed according to their severity: acute otitis media (AOM); otitis media with effusion (OME); chronic otitis media (COM); and chronic suppurative otitis media (CSOM). Children with recurrent episodes of AOM and those suffering from OME or CSOM have higher risks of developing conductive and sensorineural hearing loss. Periods of conductive hearing loss from otitis media may have a detrimental effect on speech development in children3 and are linked to educational problems, attention disorders and problems with social adaptation4.

Cleft lip and/or palate

Orofacial clefts occur when parts of the lip or palate (roof of the mouth) do not completely fuse together during pregnancy5. A cleft lip can vary in size, from a small notch in the edge of the lip to an opening that extends into the nose or the gums. The severity of a cleft palate can also vary, from a defect of the soft palate (at the back of the throat) to a complete cleft that reaches the hard palate (roof of the mouth). A child can be born with either a cleft lip or cleft palate or both. Both conditions can be successfully treated with surgery, especially if conducted within a child’s first 12-18 months5. Many children with cleft palate also have problems with their Eustachian tube, which can lead to fluid buildup in the middle ear and possibly hearing loss. For this reason, children with cleft palate should have their hearing regularly checked and may need to have their ears drained with small pressure equalisation tubes6.

Congenital deafness  

Congenital deafness is hearing loss that is present from birth. Around half of all cases of congenital deafness are thought to be caused by genetic factors. In other cases, hearing loss is transmitted through infections or other factors which affect a baby during pregnancy or at the time of birth. The viral disease rubella and the infection cytomegalovirus can both lead to congenital deafness if the mother contracts them during the early stages of pregnancy. Other infections can be transmitted from mother to baby during labour. A child’s hearing development may also be affected if the mother takes certain drugs during pregnancy, particularly some types of antibiotic7.

Down Syndrome

Down Syndrome (also known as Trisomy 21 or Trisomy G) is a chromosomal disorder caused by an extra 21st chromosome. People with Down Syndrome have common dysmorphic features such as epicanthal folds, macroglossia and transverse palmar crease, and suffer an increased risk of congenital heart defects, organic disorders and hearing loss. Chronic ear infections such as otitis media with effusion (OME) are the most common cause of hearing loss in children with Down Syndrome. Auditory system malformations and neural transmission problems can also cause hearing loss. Hearing impairment in Down Syndrome patients is typically conductive8, but sensorineural or mixed hearing loss may also occur9. The degree of hearing loss in children with Down Syndrome varies, but even mild cases can have serious consequences for their speech perception, language acquisition, academic achievement and social interaction10.

External Otitis (Otitis Externa)

External otitis is an inflammation of the external ear and/or ear canal. Its most commonly caused by an active bacterial or fungal infection, but may also be an offshoot of a skin condition, allergy or injury, with no microbial infection. External otitis is also referred to as ‘swimmer's ear, as it’s often a bacterial infection caused by excessive water exposure11. In most cases, the ear canal tissue swells and may become painful and/or tender to touch.

Meningitis

Meningitis is a viral or bacterial infection that causes inflammation and swelling of the membranes covering the brain and spinal cord. The most common symptoms of meningitis are headache and neck stiffness associated with fever, confusion or altered consciousness, vomiting, and an inability to tolerate light (photophobia) or loud noises (phonophobia). Hearing loss or deafness are amongst it possible serious complications. Meningitis may be caused by infection with viruses, bacteria or other microorganisms, and less commonly by certain drugs.

Oxotoxic drugs

Ototoxic medicines damage the ear and are a common cause of hearing loss, especially children who may need medicine on a regular basis. The first symptoms usually are ringing in the ears (tinnitus) and vertigo. Hearing loss caused by an ototoxic medicine tends to develop quickly but hearing may return to normal once the medicine is ceased. However some medicines can cause permanent damage to the inner ear.  Commonly used medicines that may cause hearing loss include: large doses of aspirin; nonsteroidal anti-inflammatory drugs; some antibiotics; loop diuretics used to treat high blood pressure and heart failure; and medicines used to treat cancer in children.

Sudden hearing loss

Sudden sensorineural hearing loss is a rapid loss of hearing (at least 30 decibels at three or more contiguous frequencies) that can happen all at once or over a period of up to three days12. It occurs across the entire age spectrum with equal prevalence in men and women, but most patients are between 50–60 years13. The hearing loss often starts as a sudden drop in hearing in one ear and sufferers may also experience tinnitus, aural fullness and/or vertigo. Anyone with symptoms should seek urgent medical care.  With more than 100 possible causes of this type of hearing loss, it’s rare for a specific cause to be precisely identified12. Some patients recover completely without medical intervention, often within the first three days. Others get better slowly over one to two weeks. A number of treatments, including steroids, may be used in the remaining cases where hearing loss doesn’t improve or worsens12.

Trauma

Acoustic trauma is injury to the inner ear’s hearing mechanisms from very loud noise. This may include an explosion near the ear, gunshots or loud music or machinery over a long period of time.   Symptoms include hearing loss, which is usually partial and involving high-pitched sounds, and ringing in the ear or tinnitus14. Hearing loss caused by acoustic trauma may worsen over time.

Treacher Collins Syndrome

Treacher Collins Syndrome (TCS), also known as Franceschetti syndrome or mandibulofacial dysostosis, is a genetic disorder resulting in craniofacial deformities. Treacher Collins is often accompanied by chronic respiratory insufficiency, sleeping apnea and a conductive hearing loss due to outer and middle ear malformations15. Middle ear malformation is present in most cases, while inner ear malformation with sensorineural hearing loss is unusual. The hearing loss is generally bilateral (occurring in both ears) with a conductive loss of about 50-70 decibels. Even in cases with normal and open external auditory canals, the ossicular chain (bones in the middle ear which help amplify sound) is often malformed16. Treacher Collins is rare, occurring approximately once in every 10,000 births1, 17.

 

Disclaimer

Please seek advice from your medical practitioner or health professional about treatments for hearing loss. They will be able to advise on a suitable solution for the hearing loss condition. All products should be used only as directed by your medical practitioner or health professional. 

Not all products are available in all countries. Please contact your local Cochlear representative.

Footnotes

  1. Kelley PE, Scholes MA. Microtia and congenital aural atresia.Otolaryngol Clin North Am. 2007;40:61-80.
  2. Mastroiacovo P, Corchia C, Botto LD, Lanni R, Zampino G, Fusco D. Epidemiology and genetics of microtia-anotia: a registry based study on over one million births. J Med Genet. 1995;32(6):453-7.
  3. Roberts K. A preliminary account of the effect of otitis media on 15-month-olds’ categorization and some implications for early language learning. J Speech Lang Hear Res. 1997;40(3):508-18.
  4. Yilmaz S, Karasalihoglu AR, Tas A, Yagiz R, Tas M. Otoacoustic emissions in young adults with a history of otitis media. J Laryngol Otol. 2006;120(2):103-7.
  5. Kids Health. [online] 2011. Available from URL: http://kidshealth.org/parent/medical/ears/cleft_lip_palate.html
  6. Pearman K. Ear Problems in Children with Cleft Palates, [online], CLAPA News issue 12, 2000. Available from URL: http://www.clapa.com/documents/earproblemsinchildren_withcleftpalates.pdf
  7. Doctissimo. Congential deafness [online] 2005. Available from URL: http://health.doctissimo.com/infants-health/infant-eye-ear-disorders/congenital-deafness.html
  8. Snik AF, Mylanus EA, Proops DW, Wolfaardt J, Hodgetts WA, Somers T, Niparko JK, Wazen JJ, Sterkers O, Cremers CW, Tjellström A. Consensus statements on the Baha system: Where do we stand at present? Ann Otol Rhinol Laryngol Suppl. 2005;195:2-12.
  9. Shott SR, Joseph A, Heithaus D. Hearing loss in children with Down syndrome. Int J Pediatr Otorhinolaryngol. 2001;61(3):199-205.
  10. Nicholson N, Christensen L, Dornhoffer J, Martin P, Smith-Olinde L. Verification of speech spectrum audibility for pediatric Baha Softband users with craniofacial anomalies. Cleft Palate Craniofac J. 2011;48(1):56-65.
  11. Medicine.net. 2011. [online] Available from URL: http://www.medicinenet.com/otitis_externa/article.htm
  12. National Institute on Deafness and other Communication Disorders, Sudden Deafness 2007.  [online] Available from URL: http://www.nidcd.nih.gov/health/hearing/pages/sudden.aspx
  13. Wynne M, Fritsch M, Diefendorf A, Sudden Hearing Loss, The Asha Leader. December 26 2001. [online]Available from URL: http://www.asha.org/Publications/leader/2001/011226/sudden_hearing_loss.htm
  14. Medline Plus 2011. [Internet] Available from URL: http://www.nlm.nih.gov/medlineplus/ency/article/001061.htm
  15. National institute on deafness and other communication disorders [online]. 2011. Available from URL: http://www.nidcd.nih.gov.
  16. Argenta LC, Iacobucci JJ. Treacher Collins syndrome: present concepts of the disorder and their surgical correction. World J Surg. 1989 ;13(4):401-9.
  17. Trainor PA, Dixon J, Dixon MJ. Treacher Collins syndrome: etiology, pathogenesis and prevention. Eur J Hum Genet. 2009;17(3):275-83.