Pierre Robin syndrome: characteristics of hearing loss, effect of age on hearing level and possibilities in therapy planning

The Journal of Laryngology and Otology
J Handzić-CukD Gortan

Abstract

Hearing loss was studied in 22 patients with Pierre Robin syndrome (PRS) aged three to 12 years (median 5.0 years). The results were compared to those obtained in 62 patients with isolated cleft palate (ICP) aged one to 27 years (median 5.5 years). Hearing loss was more frequently found in PRS (73.3 per cent) than in ICP (58.1 per cent) patients (p = 0.02). PRS patients had more ears with moderate (21-40 dB) and severe (> 40 dB) hearing loss, disturbing their social contact, with no tendency to normalization with age (Spearman r = 0.065). In contrast to PRS, ICP patients showed a significant tendency to hearing level normalization with ageing (Spearman r = -0.453; p = 0.001). Planigraphs of temporal bones showed inadequately developed pneumatization of the mastoid bone in all PRS patients and in most ICP patients. No malformation of the inner or middle ear was found in either group. PRS patients have a significantly higher risk of conductive hearing loss than those with ICP. Use of tympanostomy (ventilation) tubes is therapy of choice in patients with Pierre Robin syndrome, and it should be introduced as early as possible, even at the same time as palatoplasty.

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Citations

Jun 16, 2000·Early Human Development·F RenaultJ J Baudon
Nov 2, 2013·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Traci FlynnLennart Magnusson
Jan 27, 2016·International Journal of Pediatric Otorhinolaryngology·H SundmanA Lohmander
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Sep 30, 2005·The Annals of Otology, Rhinology, and Laryngology·Philipp M GruenEdgar Bachor
Nov 15, 2019·The Journal of Craniofacial Surgery·Fatma Nihal Durmus KocaaslanÖzhan Çelebiler
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Jan 1, 2020·European Journal of Radiology·Felice D'ArcoGiacomo Talenti

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