Genetic Sensorineural Hearing Loss Follow-up

Updated: Jun 30, 2022
  • Author: Stephanie A Moody Antonio, MD; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Further Outpatient Care

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  • Otologist

    • Encourage follow-up annually and as needed.

    • Frequent findings include problems with the hearing aid, disease of the external or middle ear, and progressive hearing loss.

    • Follow-up must also reassess the accuracy of the initial diagnosis with appropriate modifications made to the habilitative plan.

  • Audiologist

    • After a device hearing amplification is in place, systematic monitoring is necessary to ensure proper function of the device while monitoring speech and language development.

    • Schedule audiologic reevaluation every 3 months during the first year and then every 6 months thereafter.

    • Calibrate hearing aids periodically and fit new molds 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.

    • A plan for systematic monitoring is required to ensure that a child with hearing impairment develops the necessary speech and communication skills to meet his or her daily communication needs.



The patient must avoid ototoxic medications and loud noise exposure in the absence of hearing protection.



Children with unilateral hearing loss have difficulty with sound localization and with hearing in settings with background noise that can make school life difficult. Among such children, the incidence of school-grade failure, distractibility, daydreaming, inability to follow directions, and behavioral problems increases.

Children with profound bilateral hearing loss have reductions in receptive and expressive language skills, rates of graduation from high school, reading levels, and math skills. Deaf individuals may have low rates of employment, few opportunities for financial gain, restricted socialization, language barriers that resulting in limited social groups and reduced quality of life.

Using the Peabody Picture Vocabulary Test, Fourth Edition (PPVT-4), a Danish study, by Mey et al, indicated that children with either Pendred syndrome or nonsyndromic enlarged vestibular aqueduct (NSEVA) have lower receptive language capabilities than do children with normal hearing, as well as children with congenital and hereditary nonsyndromic hearing impairment (mixed group). The investigators also found that children in the mixed hearing impairment group underwent cochlear implant surgery at an earlier median age than did the patients with Pendred syndrome/NSEVCA (age 11 mo vs age 43 mo, respectively). [35]



With proper amplification, speech and language therapy, and an educational program, a patient with SNHL may participate in mainstream society, obtain gainful employment, and be competent in adult life. Children with profound deafness that is rehabilitated with cochlear implants achieve language development on par with that of their peers.


Patient Education

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