Syndromic Sensorineural Hearing Loss Treatment & Management

  • Author: Stephanie A Moody Antonio, MD; Chief Editor: Arlen D Meyers, MD, MBA   more...
 
Updated: Apr 12, 2012
 

Medical Care

  • Medical therapy: Treat any middle ear disease, including otitis media, with the appropriate medical therapy.
  • Amplification
    • The goal of amplification is to use any residual hearing to at least orient patients to surrounding environments. Hearing amplification can generally be implemented successfully during the first 6 weeks of life.
    • Available hearing amplification devices include conventional analog hearing aids, digital hearing aids, bone conduction hearing aids, and bone-anchored hearing aids. Other middle and inner ear implantable devices are undergoing clinical trials.
  • Assistive listening devices and personal systems
    • Personal devices, such as FM trainers, aid in reducing the signal-to-noise ratio in various listening situations with significant background noise, eg, classrooms.
    • Telephone devices can include such items as volume controls and couplers for use with certain hearing aids, along with telecommunication devices for deaf persons who are unable to use standard telephones.
    • Closed captioning allows television use for individuals who are severely hearing impaired.
    • Signaling devices substitute visual signals for auditory signals. They can detect environmental sounds, such as doorbells, telephones, alarm clocks, fire alarms, or crying babies.
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Surgical Care

Surgical management of external and middle ear deformities can be recommended for bilateral hearing loss and some unilateral cases.

  • Cochlear implantation
    • Cochlear implants are electronic devices designed to convert mechanical sound energy into electric signals that can be delivered to the cochlear nerve.
    • Consider cochlear implantation for patients who do not significantly benefit from conventional hearing amplification.
    • To preoperatively ensure the presence of an intact cochlear nerve, consider MRI. CT scan of the temporal bones is routinely performed to identify cochlear abnormalities.
    • Children younger than 5 years who have restored auditory input via cochlear implantation achieve substantially better language skills. Cochlear implantation may be performed at age 1 year.
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Consultations

  • Geneticist
    • Geneticists may offer assistance in establishing the etiology of SNHL.
    • Geneticists also provide genetic counseling to address a family's questions about the etiology of the patient's hearing loss and the risk of recurrence for future children.
  • Audiologist
    • Audiologists assist in selecting the most appropriate hearing aid for the patient. Selection of the appropriate aid is critical and is usually the responsibility of the audiologist.
    • Systematic monitoring is necessary to ensure proper function of the device while monitoring speech and language development.
  • Speech and language pathologist
    • Patients' linguistic and communicative skills must be analyzed while understanding that language capability, and not the hearing level, is the final indication of a successful habilitative program.
    • Normally, language should first be presented to children who are hearing impaired through all available inputs, including auditory, visual, and tactile stimuli.
  • Ophthalmologist: Consider consultation to assess visual acuity and to evaluate any possible ocular components of syndromic hearing loss.
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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

B Viswanatha, MBBS, MS, DLO  Professor of Otolaryngology (ENT), Chief of ENT III Unit, Sri Venkateshwara ENT Institute, Victoria Hospital, Bangalore Medical College and Research Institute; PG and UG Examiner, Manipal University, India and Annamalai University, 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.

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 of Otolaryngology, Dentistry, and Engineering, 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; Medvoy Ownership interest Management position; Cerescan Imaging Consulting; Headwatersmb Consulting fee Consulting; Venturequest Royalty Consulting

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