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Hearing levels in Pierre Robin syndrome

J Handzić1, M Bagatin, R Subotić

  • 1Ear, Nose, and Throat Department, Zagreb University of School of Medicine, Croatia.

The Cleft Palate-Craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association
|January 1, 1995
PubMed
Summary

Patients with Pierre Robin syndrome (PRS) frequently experience conductive hearing loss due to middle ear effusion. This hearing impairment is typically bilateral and significantly more common in PRS patients than in those without the syndrome.

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Area of Science:

  • Otolaryngology
  • Genetics
  • Pediatrics

Background:

  • Pierre Robin syndrome (PRS) is a complex congenital condition characterized by a specific set of facial abnormalities.
  • Hearing impairment is a potential comorbidity associated with craniofacial anomalies, necessitating thorough audiological evaluation.

Purpose of the Study:

  • To investigate the prevalence and characteristics of hearing loss in patients diagnosed with Pierre Robin syndrome (PRS).
  • To compare audiological findings in PRS patients with a control group of patients having cleft lip or cleft palate without PRS.

Main Methods:

  • Audiological assessments, including hearing tests, were conducted on 18 patients with Pierre Robin syndrome (PRS).
  • Results were compared to data from 243 patients with cleft lip, cleft palate, or both, without PRS.

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  • Analysis focused on identifying types of hearing loss, prevalence, and severity (mean hearing loss for air conduction in speech frequencies).

    • Main Results:

    • None of the PRS patients exhibited middle ear, inner ear malformations, or sensorineural hearing loss in speech frequencies.
    • Conductive hearing loss was prevalent in 83.33% of PRS patient ears, compared to 59.67% in the non-PRS group.
    • PRS patients showed a significantly higher mean hearing loss for air conduction in speech frequencies (24.5 dB) versus the non-PRS group (17.8 dB).

    Conclusions:

    • Hearing loss in Pierre Robin syndrome (PRS) is predominantly conductive, bilateral, and significantly more frequent than in patients without PRS.
    • Middle ear effusion was identified as the primary cause of hearing loss in PRS patients.
    • Effective treatment, including suction and ventilation tubes, successfully restored normal hearing levels in affected PRS patients.