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

Published online by Cambridge University Press:  29 June 2007

Jadranka Handžić-Ćuk ,
Višeslav Ćuk ,
Ranko Rišavi ,
Vladimir Katić ,
Damir Katušić ,
Marijo Bagatin ,
Smiljana Štajner-Katušić  and
Damir Gortan
Jadranka Handžić-Ćuk*
Affiliation:
Department of Otolaryngology, Clinical Hospital ‘Sisters of Misericardiae’, Zagreb University Clinical Hospital Centre Šalata, Dubrava, Croatia
Višeslav Ćuk
Affiliation:
Department of Otolaryngology, Clinical Hospital ‘Sisters of Misericardiae’, Zagreb University Clinical Hospital Centre Šalata, Dubrava, Croatia
Ranko Rišavi
Affiliation:
Department of Otolaryngology, Clinical Hospital ‘Sisters of Misericardiae’, Zagreb University Clinical Hospital Centre Šalata, Dubrava, Croatia
Vladimir Katić
Affiliation:
Department of Otolaryngology, Clinical Hospital ‘Sisters of Misericardiae’, Zagreb University Clinical Hospital Centre Šalata, Dubrava, Croatia
Damir Katušić
Affiliation:
Department of Otolaryngology, University Clinical Hospital Centre Rebro, Dubrava, Croatia
Marijo Bagatin
Affiliation:
Department of Otolaryngology, Clinic for Maxillofacial Surgery, Dubrava, Croatia
Smiljana Štajner-Katušić
Affiliation:
Department of Otolaryngology, University Clinical Hospital, Dubrava, Croatia
Damir Gortan
Affiliation:
Department of Otolaryngology, Clinical Hospital ‘Sisters of Misericardiae’, Zagreb University Clinical Hospital Centre Šalata, Dubrava, Croatia
*
Address for correspondence: Dr Jadranka Handžić-Ćuk, Ear, Nose and Throat Department, (KBC-ORL Šalata), Zagreb University Clinical Hospital Centre, Šalata 4, 10000 Zagreb, Croatia. Fax: +385-1-424-001 or +385-1-443-469
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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.

Keywords

Pierre Robin syndromeHearing loss, conductiveMiddle ear ventilation
Type
Main Articles
Information
The Journal of Laryngology & Otology , Volume 110 , Issue 9 , September 1996 , pp. 830 - 835
Copyright
Copyright © JLO (1984) Limited 1996

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