Genetic Sensorineural Hearing Loss Treatment & Management

Updated: Jun 30, 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:

  • 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.


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.

A study by Alzhrani et al indicated that in children with hearing loss due to a genetic syndrome, cochlear implantation leads to similar results with regard to auditory perception and speech intelligibility as it does in children with nonsyndromic hearing loss. [33]



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. [34]

  • 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.



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