Genetic Sensorineural Hearing Loss Treatment & Management

  • Author: Stephanie A Moody Antonio, MD; Chief Editor: Arlen D Meyers, MD, MBA   more...
 
Updated: Jun 1, 2009
 

Medical Care

  • Treat any middle ear disease, including otitis media, with appropriate medical therapy.
  • Hearing amplification, whether with conventional or advanced technologic devices, is critical to the habilitation process. The goal of amplification is to take advantage of any residual hearing the patient may possess. At a minimum, the goal is to orient the patient to an acoustic event in his or her environment. Hearing amplification can usually be implemented with success by the age of 6 weeks.
  • Assistive listening devices and personal systems may be helpful.
    • Personal devices aid in reducing the signal-to-noise ratio in various listening situations, such as watching television, in classrooms, and in auditoriums.
    • Telephone devices include volume controls and couplers for use with certain hearing aids. For individuals unable to use standard telephone devices, telecommunication devices for the deaf are available.
    • Captioning allows individuals with severe hearing impaired to watch television.
    • Signaling devices, which substitute visual signals for auditory signals, are available to detect environmental household sounds such as the doorbell, ringing telephone, alarm from an alarm clock, fire alarm, or a baby's cry.
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Surgical Care

Surgical management of external and middle ear deformities may be recommended in bilateral cases.

  • Cochlear implantation
    • Consider cochlear implantation for patients who do not demonstrate significant benefit from conventional hearing amplification.
    • Cochlear implants are electronic devices designed to convert mechanical sound energy into electric signals that can be delivered to the cochlear nerve.
    • Perform a CT or MRI scan of the temporal bones prior to cochlear implantation to ensure the presence of an intact cochlea and cochlear nerve.
    • In children, substantially better performance is obtained when auditory input is restored with cochlear implantation in children younger than 2 years.
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Consultations

Participation of many members of the medical community is required to offer comprehensive service to the family of a person with hearing loss. Pediatricians, audiologists, speech-language pathologists, educational specialists, and otolaryngologists must contribute to these efforts.

  • Obtain an otolaryngology consult when hearing loss is suspected or diagnosed. The otolaryngologist identifies the hearing loss, assesses the cause, identifies risk factors, and obtains appropriate medical tests.
  • A geneticist may offer assistance in establishing the etiology of SNHL. The geneticist can also provide genetic counseling to address a family's questions about the etiology of the patient's hearing loss and the risk of hearing loss in future children. Arnos has evaluated the ethical and social implications of genetic testing.[17]
  • The audiologist is responsible for selection of the appropriate aid, which is a critical decision. Once hearing amplification is in place, systematic monitoring is necessary to ensure proper function of the device while monitoring speech and language development.
  • A speech and language pathologist can provide appropriate educational programs necessary to enrich social, emotional, and academic development. The patient's linguistic and communicative skills must be analyzed with the understanding that the final indication of the success of the habilitative program is the patient's language capability and not the level of hearing. As a general rule, initially present language to children who are hearing impaired using all available inputs, including auditory, visual, and tactile stimuli.
  • An ophthalmology evaluation is important to assess visual acuity and to evaluate any possible ocular components of syndromic hearing loss.
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Activity

Anecdotal reports associate increased risk of hearing loss with patients who have enlarged vestibular aqueducts and participate in contact sports.

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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.

Specialty Editor Board

Robert A Battista, MD, FACS  Assistant Professor of Otolaryngology, Northwestern University Medical School; Physician, Ear Institute of Chicago, LLC

Robert A Battista, MD, FACS is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery, American College of Surgeons, American Neurotology Society, and Illinois State Medical Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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: Vesticon, Inc. None Board membership

Christopher L Slack, MD  Private Practice in Otolaryngology and Facial Plastic Surgery, 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 Unrestricted 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; Cerescan Imaging Honoraria Consulting; GYRUS ACMI Honoraria Consulting

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Karen K Hoffmann, MD, to the development and writing of this article.

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