Hearing levels in Pierre Robin syndrome

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.
• 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.