eMedicine Specialties > Otolaryngology and Facial Plastic Surgery > Inner Ear

Inner Ear, Syndromic Sensorineural Hearing Loss: Follow-up

Author: Stephanie A Moody Antonio, MD, Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, Eastern Virginia Medical School
Coauthor(s): Barry Strasnick, MD, FACS, Chairman, Professor, Department of Otolaryngology - Head and Neck Surgery, Eastern Virginia Medical School
Contributor Information and Disclosures

Updated: Jun 25, 2009

Follow-up

Further Outpatient Care

  • Otologist
    • Patients should see an otologist on an annual and as-needed basis. Systematic otologic and audiologic follow-up leads to significant findings in up to 58% of children.
    • Frequent findings include problems with hearing aids, diseases of the external or middle ear, and progressive hearing losses.
  • Audiologist
    • Schedule audiologic reevaluation every 3 months during the first year and every 6 months thereafter.
    • Hearing aids should be calibrated periodically and new molds fitted when necessary.
    • Periodic audiometric testing is necessary to rule out fluctuation or progression of hearing loss.
  • Speech and language pathologist
    • Speech and language therapy is imperative to promote proper language and communication skills.
    • Follow-up must also reassess the accuracy of the initial diagnosis, and appropriate modifications to the habilitative plan must be implemented. Assessment of effectiveness of the educational program is critical to follow-up evaluations.

Deterrence/Prevention

Instruct patients to avoid ototoxic medications and loud noise exposure without hearing protection.

Prognosis

With proper amplification, speech and language therapy, and educational programs, a patient with SNHL can fully participate in the totality of adult life, including social activities and work.

Patient Education

  • Numerous educational methods are currently used for children with hearing impairment. These methods include auditory-oral training, cued speech, and total communication.
    • Auditory-oral training stresses acquisition of speech and language through enhancement of residual hearing. Lipreading skills, along with appropriate amplification, are heavily emphasized.
    • The cued speech approach is a visual-oral system that uses hand cues to supplement information received from lipreading. Hand cues alone are ambiguous, necessitating development of appropriate lipreading skills for language comprehension.
    • Manualism is a system of communication that stresses use of the manual alphabet (fingerspelling) and sign language for communication. American Sign Language (Ameslan) has been the language of the deaf population in the United States for more than a century. Ameslan does not follow English grammatical rules and has its own semantic system. Signed English uses syntax compatible with English grammar, giving people who are deaf knowledge of proper structure and usage of English.
    • The total communication method emphasizes manual, oral, and aural modes of communication. This method urges early use of residual hearing while accepting sign language as a normal means of communication. Speech and use of spontaneous expression are also encouraged.
  • Educators, individuals with hearing impairment, and parents still disagree on the most advantageous method of communication. The method selected has a profound influence on a child's ability to someday fully participate in the totality of adult life, including social activities and work. No single educational program is correct for all children with hearing impairment, but, rather, decisions should be individualized for each child.
  • For excellent patient education resources, visit eMedicine's Ear, Nose, and Throat Center. Also, see eMedicine's patient education article Hearing Loss.
 


More on Inner Ear, Syndromic Sensorineural Hearing Loss

Overview: Inner Ear, Syndromic Sensorineural Hearing Loss
Differential Diagnoses & Workup: Inner Ear, Syndromic Sensorineural Hearing Loss
Treatment & Medication: Inner Ear, Syndromic Sensorineural Hearing Loss
Follow-up: Inner Ear, Syndromic Sensorineural Hearing Loss
Multimedia: Inner Ear, Syndromic Sensorineural Hearing Loss
References
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References

  1. Information on Hearing Loss - Ten Syndromes Most Commonly Associated With Hearing Impairment. boystownhospital.org. Available at http://boystownhospital.org/Hearing/info/genetics/syndromes/ten.asp. Accessed April 14, 2009.

  2. Eisen MD, Ryugo DK. Hearing molecules: contributions from genetic deafness. Cell Mol Life Sci. Mar 2007;64(5):566-80. [Medline].

  3. Mehra S, Eavey RD, Keamy DG Jr. The epidemiology of hearing impairment in the United States: newborns, children, and adolescents. Otolaryngol Head Neck Surg. Apr 2009;140(4):461-72. [Medline].

  4. Morzaria S, Westerberg BD, Kozak FK. Systematic review of the etiology of bilateral sensorineural hearing loss in children. Int J Pediatr Otorhinolaryngol. Sep 2004;68(9):1193-8. [Medline].

  5. Saunders JE, Vaz S, Greinwald JH, Lai J, Morin L, Mojica K. Prevalence and etiology of hearing loss in rural Nicaraguan children. Laryngoscope. Mar 2007;117(3):387-98. [Medline].

  6. Usher Syndrome. Genetics Home Reference. Available at http://ghr.nlm.nih.gov/condition=ushersyndrome. Accessed April 14, 2009.

  7. Pendred Syndrome. Genetics Home Reference. Available at http://ghr.nlm.nih.gov/condition=pendredsyndrome. Accessed April 14, 2009.

  8. Jervell and Lange-Nielson Syndrome. Genetics Home Reference. Available at http://ghr.nlm.nih.gov/condition=jervellandlangenielsensyndrome. Accessed April 14, 2009.

  9. Cockayne Syndrome. Genetics Home Reference. Available at http://ghr.nlm.nih.gov/condition=cockaynesyndrome.

  10. Alstrom Disease. Genetics Home Reference. Available at http://ghr.nlm.nih.gov/condition=alstromsyndrome. Accessed April 14, 2009.

  11. Merchant SN, Burgess BJ, Adams JC, Kashtan CE, Gregory MC, Santi PA. Temporal bone histopathology in alport syndrome. Laryngoscope. Sep 2004;114(9):1609-18. [Medline].

  12. Norrie Disease. Genetics Home Reference. Available at http://ghr.nlm.nih.gov/condition=norriedisease. Accessed April 14, 2009.

  13. Alport Syndrome. Genetics Home Reference. Available at http://ghr.nlm.nih.gov/condition=alportsyndrome. Accessed April 14, 2009.

  14. Alstrom CH. Retinal degeneration combined with obesity, diabetes mellitus and neurogenous disease. Acta Psychiatrica Scandinavica. 1959;34:1-35.

  15. Belal A Jr. Usher's syndrome. (Retinitis pigmentosa and deafness). A temporal bone report. J Laryngol Otol. Feb 1975;89(2):175-81. [Medline].

  16. Brown OE, Meyerhoff WL, Ginsburg CM. Ear, nose and throat manifestations of systemic disease. In: Clinical Pediatric Otolaryngology. St. Louis, Mo: Mosby-Year Book; 1986.

  17. Chung CS, Robinson OW, Morton NE. A note on deaf mutism. Ann Hum Genet. 1959;23:357-366.

  18. Cockayne E. Dwarfism with retinal atrophy and deafness. Arch Dis Child. 1936;2:1.

  19. Coppage KB, Smith RJ. Branchio-oto-renal syndrome. J Am Acad Audiol. Jan 1995;6(1):103-10. [Medline].

  20. Fisch L. Deafness as part of a hereditary syndrome. J Laryngol Otol. 1959;73:355.

  21. Friedmann I, Fraser GR, Froggatt P. Pathology of the ear in the cardioauditory syndrome of Jervell and Lange-Nielsen (recessive deafness with electrocardiographic abnormalities). J Laryngol Otol. May 1966;80(5):451-70. [Medline].

  22. Gandolfi A, Horoupian D, Rapin I, DeTeresa R, Hyams V. Deafness in Cockayne's syndrome: morphological, morphometric, and quantitative study of the auditory pathway. Ann Neurol. Feb 1984;15(2):135-43. [Medline].

  23. Igarashi M, Takahashi M, Alford BR, Johnson PE. Inner ear morphology in Down's syndrome. Acta Otolaryngol. Jan-Feb 1977;83(1-2):175-81. [Medline].

  24. Illum P, Kiaer HW, Hvidberg-Hansen J, Sondergaard G. Fifteen cases of Pendred's syndrome. Congenital deafness and sporadic goiter. Arch Otolaryngol. Oct 1972;96(4):297-304. [Medline].

  25. Ishikiriyama S, Tonoki H, Shibuya Y, et al. Waardenburg syndrome type I in a child with de novo inversion (2)(q35q37.3). Am J Med Genet. Aug 1989;33(4):505-7. [Medline].

  26. Jacobson JT, Jacobson CA, Spahr RC. Automated and conventional ABR screening techniques in high-risk infants. J Am Acad Audiol. Oct 1990;1(4):187-95. [Medline].

  27. Jervell A, Lange-Nielsen J. Congenital deaf-mutism functional heart disease with prolongation of the Q-T interval and sudden death. Am Heart J. 1957;54:9-68.

  28. Kaplan J, Gerber S, Bonneau D, et al. A gene for Usher syndrome type I (USH1A) maps to chromosome 14q. Genomics. Dec 1992;14(4):979-87. [Medline].

  29. Kimberling WJ, Moller C. Clinical and molecular genetics of Usher syndrome. J Am Acad Audiol. Jan 1995;6(1):63-72. [Medline].

  30. Kimberling WJ, Weston MD, Moller C, et al. Localization of Usher syndrome type II to chromosome 1q. Genomics. Jun 1990;7(2):245-9. [Medline].

  31. Konigsmark B. Genetic and Metabolic Deafness. Philadelphia, Pa: WB Saunders Co; 1976.

  32. McLay K, Maran AG. Deafness and the Klippel-Feil syndrome. J Laryngol Otol. Feb 1969;83(2):175-84. [Medline].

  33. Miglets AW, Schuller D, Ruppert E, Lim DJ. Trisomy 18. A temporal bone report. Arch Otolaryngol. Jul 1975;101(7):433-7. [Medline].

  34. Nance WE, Sweeney A. Symposium on sensorineural hearing loss in children: early detection and intervention. Genetic factors in deafness of early life. Otolaryngol Clin North Am. Feb 1975;8(1):19-48. [Medline].

  35. National Newborn Screening Status ReportUpdated 03/11/09. Available at http://genes-r-us.uthscsa.edu/nbsdisorders.pdf.

  36. Nilsom LR. Nonendemic goiter and deafness. Acta Pediatrica Scandinavica. 1964;52:117-131.

  37. Ouyang SM, Yan D, Yuan HJ, et al. The genetic basis of non-syndromic hearing loss among Chinese. Journal Human Genetics. 2009;54(3):131-40.

  38. Reed WB. Congenital cutaneous diseases associated with central nervous system disorders. Postgrad Med. May 1967;41(5):527-36. [Medline].

  39. Refsum S. Heredopathia actica polyneuritis formis. Acta Psychiatrica Scandinavica. 1946;38:301-303.

  40. Rose SP, Conneally PM, Nance WE. Genetic analysis of childhood deafness. Childhood Deafness. 1977;19-35.

  41. Schachern PA. Mucopolysaccharidosis IH (Hurler syndrome) and human temporal bone pathology. Annals of ORL. 1984;93(1):65-69.

  42. Strome M. Down's syndrome: a modern otorhinolaryngological perspective. Laryngology. 1959;69:54-89.

  43. Usher CH. On the inheritance of retinitis pigmentosa, with notes of a case. London Ophthalmol Hosp Nep. 1914;19:130-136.

  44. Vrijens K, Van Laer L, Van Camp G. Human hereditary hearing impairment: mouse models can help to solve the puzzle. Hum Genet. Nov 2008;124(4):325-48. [Medline].

  45. Waardenburg PJ. A new syndrome combining developmental anomalies of the eyelids, eyebrows, and nose root with pigmentary defects of the iris and head hair and with congenital deafness. Am J Hum Gen. 1951;3:195-253.

  46. Wester DC, Atkin CL, Gregory MC. Alport syndrome: clinical update. J Am Acad Audiol. Jan 1995;6(1):73-9. [Medline].

  47. Wilson J. Deafness in developing countries. Approaches to a global program of prevention. Arch Otolaryngol. Jan 1985;111(1):2-9. [Medline].

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Further Reading

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Keywords

hearing loss, sensorineural hearing loss, syndromic sensorineural hearing loss, SNHL, inner ear, deaf, deafness, hearing impairment, hearing impaired, hard of hearing, auditory system

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Contributor Information and Disclosures

Author

Stephanie A Moody Antonio, MD, Assistant Professor, Department of Otolaryngology-Head and Neck Surgery, Eastern Virginia Medical School
Stephanie A Moody Antonio, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American Medical Association, American Neurotology Society, and Virginia Society of Otolaryngology-Head and Neck Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

Barry Strasnick, MD, FACS, Chairman, Professor, Department of Otolaryngology - Head and Neck Surgery, Eastern Virginia Medical School
Barry Strasnick, MD, FACS is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, American Auditory Society, American College of Surgeons, American Medical Association, American Tinnitus Association, Ear Foundation Alumni Society, Norfolk Academy of Medicine, North American Skull Base Society, Society of University Otolaryngologists-Head and Neck Surgeons, Vestibular Disorders Association, and Virginia Society of Otolaryngology-Head and Neck Surgery
Disclosure: Nothing to disclose.

Medical Editor

B Viswanatha, MBBS, MS, DLO, Professor of ENT, Sri Venkateshwara ENT Institute, Victoria Hospital, Bangalore Medical College and Research Institute, India
B Viswanatha, MBBS, MS, DLO is a member of the following medical societies: Association of Otolaryngologists of India, Indian Medical Association, and Indian Society of Otology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Gerard J Gianoli, MD, Clinical Associate Professor, Department of Otolaryngology-Head and Neck Surgery, Tulane University School of Medicine; Vice President, The Ear and Balance Institute; Chief Executive Officer, Ponchartrain Surgery Center
Gerard J Gianoli, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society, American Otological Society, Society of University Otolaryngologists-Head and Neck Surgeons, and Triological Society
Disclosure: Nothing to disclose.

CME Editor

Christopher L Slack, MD, Otolaryngology-Facial Plastic Surgery, Private Practice, Associated Coastal ENT; Medical Director, Treasure Coast Sleep Disorders
Christopher L Slack, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Arlen D Meyers, MD, MBA, Professor, Department of Otolaryngology-Head and Neck Surgery, University of Colorado School of Medicine
Arlen D Meyers, MD, MBA is a member of the following medical societies: American Academy of Facial Plastic and Reconstructive Surgery, American Academy of Otolaryngology-Head and Neck Surgery, and American Head and Neck Society
Disclosure: Covidien Corp Consulting fee Consulting; US Tobacco Corporation unstricted gift unknown; Axis Three Corporation Ownership interest Consulting; Omni Biosciences Ownership interest Consulting; Sentegra Ownership interest Board membership; Syndicom Ownership interest Consulting; Oxlo  Consulting; Medvoy Ownership interest Management position

 
 
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