© Borgis - New Medicine 3/2004, s. 87-88
Henryk Skarżyński1, Maciej Mrówka1, Paulina Młotkowska-Klimek1, Bożena Skarżyńska2
Effects of treatment of otosclerosis in children
1International Center of Hearing and Speech, Institute of Physiology and Pathology of Hearing,
Warsaw, Poland
Head: Prof. Henryk Skarżyński MD
2Department of Anatomy, Medical University, Warsaw, Poland
Head: Prof. Bogdan Ciszek MD, PhD
Summary
Otosclerosis in children is not a rare condition, however, many authors recommend only patient assessment in those cases and no surgical treatment before puberty. The aim of the present study was to analyze hearing results of stapedotomy in children with otosclerosis and to compare the results with those obtained in adults. The material at Otorhinolaryngosurgery Center, the International Center of Hearing and Speech, consisted of 37 children (38 ears) and adolescents under 16 years of age who were surgically operated for otosclerosis, and followed up for at least 6 months. Almost all the patients underwent stapedotomy with implantation of a teflon-piston prosthesis; stapes mobilization was performed in two cases. Pre- and postoperative hearing assessment included frequencies 500, 1000, 2000 and 4000 Hz. In 4 ears advanced obliterative otosclerotic lesions were found. Otosclerosis accompanied, the so-called, small middle ear congenital malformations (lack of the stapes suprastructure); one patient had a coexisting oto- and tympanosclerosis but without any tympanosclerotic foci in the oval window recess. In 6 ears, hearing impairment was of a mixed type, in other cases it was only conductive hearing loss. Mean preoperative air-bone gap reached 27.4 dB. Following surgery, a gap closure within 10 dB was achieved in 35 ears. An overclosure effect was obtained in 12 patients, with an average value of 3.2 dB. Mean hearing gain in all patients was 29.2 dB. The follow-up period (6-36 months) showed that the mean value of the reported air-bone gap was 3.8 dB, however, in 35 ears the hearing thresholds have been still better than those from before the surgical operation. Reoperations were performed in 4 patients in order to remove adhesions or to restore the connection between the prosthesis and footplate. Postoperative hearing results, not worse than in adults, seem to support the concept of employing surgical treatment in all children with recognized otosclerosis.
Introduction
Otosclerosis is usually diagnosed in patients within the age range between 20-40 years; paediatric patients in this group represent 15% (1). Studies conducted on temporal bone specimens have shown more frequent occurrence of otosclerosis than it is commonly believed. According to Guild (2), otosclerosis is found in 10% of the temporal bones; in children under 5 years of age, it represents only 0.6%; in patients between 30-50 years of age, the condition is gender-related (females - 18.5%, males - 9.7%). Only in 12% of patients otosclerotic tissue has been found to cover the oval window which results in fixation of the stapes.
There is a general trend to conduct long-term evaluation of children undergoing otosclerotic treatment. In most of them the operation is postponed until they grow up; stapedotomy is performed only in 6-7% of children (3; 1). Even though the disease does not last long, there are cases of advanced otosclerosis leading to a significant hearing loss (4; 5; 3, 1).
Objective
The objective of the present study was to assess the effects of the hearing results following stapedotomy in paediatric patients with otosclerosis, since in most centers in Poland children have to wait for the surgical treatment until puberty. The authors present their own experience based on the material of the Otorhino-laryngosurgery Centre, at the International Centre of Hearing and Speech, Warsaw.
Material and Method
The study material included 37 children and adolescents (38 ears) at the age of 16 years and under, with diagnosed and surgically treated otosclerosis who were followed up for 36 months. Almost all the patients underwent stapedotomy with implantation of teflon-piston prostheses; stapes mobilization was performed in two cases. Assessment of hearing pre- and post-operatively, and every 3 months, included frequencies of 500, 1000, 2000, and 4000 Hz.
Results
Children stapedotomies performed at the Centre accounted for 6.1% of the total number of stapedotomies performed. In 24 patients otosclerosis was accompanied by tinnitus which subsided after the operation.The patients´ mean age was14.6 years. In 4 ears (3 patients) obliterative advanced otosclerotic foci were found. In 2 cases otosclerosis was accompanied by small congenital malformations of the middle ear (lack of the stapes suprastructure). In one case otosclerosis was accompanied by tympanosclerosis, and one patient had congenital malformation syndrome.In 6 ears, a mixed type of hearing impairment was recognized; in the remaining ears, it was conductive. Mean preoperative air-bone gap reached 27.4 dB. The gap closure, achieved postoperatively, reached 10 dB in 35 of 38 operations. An overclosure effect was observed in 12 patients, and on average it was 3.2 dB. Mean hearing gain reached on average 29.2 dB. In a 3-year follow-up the mean air-bone gap was 3.8 dB, but in 35 ears hearing results have been still better than before the operation. Four patients underwent reoperations due to fixation of the prosthetic piston.
Discussion
Stapedotomy is thought to be an effective surgical technique in the treatment of otosclerosis. The success is usually achieved in 98% of patients operated. Complications due to appearing or deteriorating hearing occur in 0.6-3% (6). The only effective treatment of otosclerosis in children is by stapedotomy, and its early results do not differ significantly from those in adults; the long-term effects are usually better (4, 7, 8, 3, 9, 1, 10,11). Treatment of children with otosclerosis is even more difficult, since more often than in adult cases, the surgeon has to deal with congenital ear malformations, which leads to diagnostic problems and difficulties during operation (12; 10).
The present assessment shows that surgical treatment is an effective method in the treatment of paediatric otosclerosis, and short- and long-term results of stapedectomy do not differ much from those achieved in adult patients, whereas the long-term effects are even more promising.
Piśmiennictwo
1. Robinson M.: Juvenile otosclerosis: a 20-year study. Annals of Otology, Rhinology and Laryngology 1983; 92:561-565. 2. Guild S.R.: Histologic otosclerosis. Annals of Otology, Rhinology and Laryngology 1944; 53:246-256. 3. Namysłowski G, Ścierski W.: Ocena stanu słuchu u chorych po stapedotomii leczonych w II Klinice Laryngologii Śląskiej AM w Zabrzu. Otolaryngologia Polska 1999; suppl. 30: 469-471. 4. Cole J.M.: Surgery for otosclerosis in children. Laryngoscope 1982; 92:859-862. 5. Lippy W.H., Burkey J.M., Schuring A.G., Rizer F.M.: Short-and long-term results of stapedectomy in children. Laryngoscope 1998; 108, 569-572. 6. Murphy T.P., Wallis D.L.: Stapedectomy in the pediatric patient. Laryngoscope 1996; 106:1415-1418. 7. von Haacke N.P.: Juvenile stapedectomy. Clinical Otolaryngology 1985; 10, 9-13. 8. House J.W., Sheehy J.L., Antunez J.G.: Stapedectomy in children. Laryngoscope 1980; 90:1804-1809. 9. Namysłowski G., Ścierski W., Mrówka-Kata K., Bilińska-Pietraszek E.: Stapedotomia w leczeniu otosklerozy wieku dziecięcego. Otolaryngologia Polska 2001; 55:521-523. 10. Szymański M., Siwiec H., Golabek W., Morshed K.: Odległe wyniki stapedotomii u dzieci. Annales Universitatis 2001. 11. Millman B., Giddings N.A., Cole J.M.: Long-term follow-up of stapedectomy in children and adolescents. Otolaryngology Head and Neck Surgery 1996; 115:78-81. 12. Skarżyński H., Krzeska-Malinowska I., Miszka K., Niemczyk K., Szwedowicz P.: Stapedotomie wykonywane u dzieci. Otolaryngologia Polska 1999; suppl. 30:135:137.
