Syndromic Sensorineural Hearing Loss Treatment & Management

Updated: Jul 25, 2022
  • Author: Stephanie A Moody Antonio, MD; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Medical Care

See the list below:

  • 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.


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. [20, 21, 22]

To preoperatively ensure the presence of an intact cochlear nerve, consider MRI. CT scanning 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.

A study by Alzhrani et al found that the results of cochlear implantation in children with hearing loss due to a genetic syndrome (Waardenburg, Usher, or Dandy-Walker syndrome or albinism) approximated those in children with nonsyndromic hearing loss. The investigators reported that postimplantation auditory perception and speech intelligibility levels were similar in both study groups, as was the final pure-tone average. [23]

A literature review by Davies et al indicates that cochlear implants can benefit most patients with Usher syndrome. The studies reviewed offered agreement that at any age, cochlear implantation for Usher syndrome results in better outcomes with regard to pure-tone audiometry, speech production, and quality of life. However, with Usher syndrome type I, according to the investigators, patients are best served by bilateral cochlear implantation performed at the earliest feasible age. [24]



See the list below:

  • 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.