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[Genetic sensorineural hearing loss in childhood].

Onur Turan1, Fazil Apaydin

  • 1Ege Universitesi Tip Fakültesi Kulak Burun Boğaz Hastaliklari Anabilim Dali, Izmir, Turkey.

Kulak Burun Bogaz Ihtisas Dergisi : KBB = Journal of Ear, Nose, and Throat
|July 19, 2002
PubMed
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Genetic factors significantly contribute to childhood sensorineural hearing loss, with consanguinity being a primary cause. Early detection of hearing impairments is crucial for successful auditory rehabilitation programs in children.

Area of Science:

  • Otolaryngology
  • Genetics
  • Pediatrics

Context:

  • Hearing loss is a significant disability in children.
  • Understanding the etiology and presentation of hearing loss is essential for effective management.
  • Previous studies have highlighted genetic factors but comprehensive data on presentation age and severity in specific populations are needed.

Purpose:

  • To determine the severity, age of presentation, and genetic causes of hearing loss in children.
  • To analyze the role of consanguinity and syndromic factors in childhood hearing impairment.
  • To evaluate the timeliness of diagnosis and its relation to potential rehabilitation outcomes.

Summary:

  • This study investigated 1645 children treated for hearing loss between 1993-1999, 212 children in 2000, and 443 students from a deaf school (1998-1999).

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  • Findings revealed that families became aware of hearing loss around age two, with presentation for treatment at age four. Moderate to severe hearing loss was prevalent (44% in one group, 28% in another).
  • Genetic analysis showed consanguinity in 27-33% of cases and identified syndromic (27 families) and nonsyndromic (86 families) etiologies, indicating a substantial role for genetic inheritance.

    • Impact:

    • Highlights the significant contribution of genetic factors, particularly consanguinity, to childhood sensorineural hearing loss.
    • Emphasizes the critical need for earlier detection of hearing loss to enhance the success of rehabilitation interventions.
    • Provides valuable data for public health strategies and genetic counseling concerning pediatric hearing impairment.