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Pediatric stapedectomy: Does cause of fixation affect outcomes?
International Journal of Pediatric Otorhinolaryngology 77 (2013) 1099–1102
Contents lists available at SciVerse ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Pediatric stapedectomy: Does cause of fixation affect outcomes? Ryan E. Neilan, Richard W. Zhang, Peter S. Roland, Brandon Isaacson, Kenneth H. Lee, J. Walter Kutz Jr.* University of Texas Southwestern Medical Center, Department of Otolaryngology, 5323 Harry Hines Boulevard, Dallas, TX 75390, United States
A R T I C L E I N F O
A B S T R A C T
Article history: Received 16 January 2013 Received in revised form 8 April 2013 Accepted 12 April 2013 Available online 17 May 2013
Objective: To compare outcomes of stapedectomy in patients with congenital stapes fixation versus juvenile otosclerosis. Methods: A retrospective chart review was performed from January 1, 1999 until January 1, 2011 to identify patients under 18 years old who underwent a stapedectomy. Age, gender, pre- and postoperative audiograms, intraoperative findings including etiology of stapes fixation, prosthesis type, and complications were recorded. Results: Twenty-two children were identified who had undergone a stapedectomy (two patients underwent sequential bilateral surgery) resulting in a total of 24 ears. The cause of fixation included juvenile otosclerosis (n = 7) and congenital stapes fixation (n = 17). The overall mean pre-operative airbone gap (ABG) was 34.7 dB (SD: 13.5) compared to a postoperative mean ABG of 9.0 (SD: 9.3) (p < 0.001). The mean postoperative ABG of 9.6 (SD: 10.5) in the congenital stapes fixation group was similar to the mean postoperative ABG of 7.2 dB (SD: 5.4) in children with juvenile otosclerosis (p = 0.6). Two patients developed delayed profound sensorineural hearing loss approximately two weeks after surgery. One patient with profound sensorineural hearing loss recovered to a profound mixed hearing loss with a speech discrimination score of 80%. Conclusions: Pediatric stapedectomy has comparable results to stapedectomy in adults regardless of the cause of stapes fixation; however, delayed sensorineural hearing loss may be higher in the pediatric population. ß 2013 Elsevier Ireland Ltd. All rights reserved.
Keywords: Stapedectomy Juvenile otosclerosis Congenital stapes fixation Pediatric conductive hearing loss
1. Introduction Conductive hearing loss (CHL) with a dry middle ear cleft is relatively uncommon in the pediatric population and is potentially correctable with surgical intervention. Aside from middle ear effusion, chronic otitis media, and aural atresia, the most common causes of pediatric CHL are congenital stapes fixation and juvenile otosclerosis. Less common causes of conductive hearing loss in children include stapes fixation from osteogenesis imperfecta, congenital cholesteatoma, and juvenile Paget’s disease. In previous studies, there have been differing reports whether patients with congenital stapes fixation have poorer hearing outcomes when compared to patients with juvenile otosclerosis [1–6]. The options for treating pediatric stapes fixation include observation, amplification with a hearing aid, bone anchored hearing aid, or a stapedectomy. Factors to consider when counseling families regarding treatment options include age, history of otitis media, eustachian tube function, family history of
* Corresponding author. Tel.: +1 214 648 3102; fax: +1 214 648 9122. E-mail addresses: [email protected], [email protected] (J. Walter Kutz Jr.). 0165-5876/$ – see front matter ß 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijporl.2013.04.009
hearing loss, associated congenital abnormalities, general medical condition, and laterality. Since otitis media is common in young children, a stapedectomy should be delayed until the child is no longer otitis prone. A CT should be performed before considering a stapedectomy in a child to evaluate for other associated ossicular or inner ear abnormalities. Significant bilateral CHL may bias the decision toward a stapedectomy rather than a hearing aid. We have reviewed our experience with primary stapedectomy in children under 18 years of age and compare hearing outcome between congenital stapes fixation and juvenile otosclerosis. 2. Methods Patients under 18 years of age who underwent a primary stapedectomy by the authors (P.S.R., B.I., J.W.K.) were identified through the clinic database using CPT codes 69660 and 69661. Patients with a history of chronic otitis media, aural atresia, or revision stapedectomy were excluded. Institutional review board approval was obtained before the onset of the study. A retrospective chart review of clinic notes and operative reports were used to record the following parameters: age at time of surgery, gender, laterality, radiographic findings, cause of stapes fixation, surgical technique, prosthesis type and length, facial
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nerve findings, associated ossicular findings, and pre- and postoperative pure tone audiometry. Postoperative audiograms were routinely obtained at 6–8 weeks postoperatively and annually thereafter. The pure tone average (PTA) for air and bone conduction was calculated by averaging thresholds at 500 Hz, 1000 Hz, 2000 Hz, and 3000 Hz. If 3000 Hz was not available, the mean of 2000 Hz and 4000 Hz was used. The mean of 1000 Hz, 2000 Hz, and 4000 Hz was used to calculate the overclosure of bone conduction. The last postoperative audiogram was used for analysis if the patient had multiple postoperative audiograms. Audiologic data were reported according to the published guidelines from the Committee on Hearing and Equilibrium for the evaluation of results of conductive hearing loss [7]. The cause of fixation was determined by examining the following: age, family history, duration of hearing loss, radiographic findings, and intraoperative findings. Univariate analysis consisting of the unpaired t-test was used to compare audiologic outcomes between patients with congenital stapes fixation and juvenile otosclerosis.
3. Results Twenty-two patients with complete pre- and postoperative audiologic data were identified. Two patients underwent sequential bilateral stapedectomy procedures resulting in 24 surgical procedures. Two patients developed delayed postoperative significant sensorineural hearing loss and were excluded from the data analysis as outliers and are discussed individually. Twelve patients were female and a right stapedectomy operation was performed in 13 patients. A partial or total stapedectomy using a bucket-handle prosthesis was performed in 21 ears. A laser stapedotomy with a Nitinol piston prosthesis was performed in three patients. All procedures were performed under general anesthesia. Seventeen ears had congenital stapes fixation as the cause of stapes fixation. Juvenile otosclerosis was the cause of fixation in seven ears. After excluding the two patients with postoperative sensorineural hearing loss, the mean age at the time of operation in patients with congenital stapes fixation was 9.5 years (range: 6.4– 13.6 years) compared to a mean age of 11.3 years (range: 6.7–18.0 years) in children with otosclerosis. This difference was not significant (p = 0.26). The mean follow up that included a comprehensive audiogram was 2.6 years and 1.3 years in the otosclerosis and congenital stapes fixation groups, respectively. A CT temporal bone was obtained on all patients considering surgical intervention to evaluate for inner ear or facial nerve abnormalities that may result in a contraindication for surgery. Most ossicular abnormalities were not appreciated on CT but were identified intraoperatively. Fig. 1 demonstrates a patient with abnormal ossicles. At the time of surgery, he was found to have a short lenticular process and absent stapes crura. Fig. 2 shows an
Fig. 2. Axial CT showing otosclerosis involving the fissula ante fenestrum.
example of juvenile otosclerosis with otosclerosis involving the fissula ante fenestrum. Table 1 compares pre- and postoperative air conduction pure tone average (PTA), bone conduction PTA, and change in air bone gap (ABG) for all children and also stratified by cause of fixation. The mean preoperative ABG for all cases was 34.7 dB (SD: 13.5). The mean preoperative ABG for congenital stapes fixation was 34.8 dB (SD: 15.0) compared to 34.4 dB (SD: 9.7) in ears with juvenile otosclerosis. The mean postoperative ABG for all cases was 9.0 dB (SD: 9.3). The mean postoperative ABG for congenital stapes fixation and juvenile otosclerosis was 9.6 (SD: 10.5) and 7.2 (SD: 5.4) respectively. The preoperative and postoperative outcomes were not statistically significantly different between patients with congenital stapes fixation versus those with juvenile otosclerosis. The mean overclosure of bone conduction was 4.0 dB (SD: 9.0) for all patients. Overclosure was higher in the otosclerosis group at 5.6 dB (SD: 9.5) when compared to overclosure of 3.4 dB (SD: 9.1) in the congenital fixation group. This difference did not reach significance (p = 0.41). Table 2 demonstrates postoperative ABG stratified by degree of closure. For all ears, 54.1% resulted in an ABG < 10 dB and 83.3% resulted in an ABG < 20 dB. For congenital stapes fixation, 52.9% resulted in an ABG < 10 dB and 82.3% resulted in an ABG < 20 dB. For juvenile otosclerosis, 57.0% resulted in an ABG < 10 dB and 85.6% resulted in an ABG < 20 dB. Seven of the seventeen (41.2%) ears with congenital stapes fixation had associated ossicular abnormalities. The most common associated abnormality was discontinuity of the incudostapedial joint, which was observed in four ears. The mean postoperative ABG was 6.7 dB (range: 0.6 dB to 10.0 dB) in this group. The other
Fig. 1. Axial CT showing ossicular abnormalities in a patient with congenital stapes fixation. Intraoperative findings demonstrated a short lenticular process and absent stapes crura.
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Table 1 Comparison of preoperative and postoperative hearing outcomes between patients undergoing a stapedectomy for congenital footplate fixation or juvenile otosclerosis.
Preoperative hearing thresholds
Air PTA Bone PTA ABG
Postoperative hearing thresholds
Air PTA Bone PTA ABG
Change in hearing thresholds
Change in ABG Overclosure
All ears (n = 22)
Congenital (n = 16)
Otosclerosis (n = 6)
Mean (SD) [range]
Mean (SD) [range]
Mean (SD) [range]
48.4 (12.9) [24.4–67.5] 13.8 (6.25) [0.0–59.4] 34.7 (13.5) [13.8–59.4] 18.8 (11.1) [6.9–48.8] 9.8 (6.8) [0.0–22.5] 9.0 (9.3) [ 3.8–33.1] 25.7 (14.3) [0.6–54.4] 4.0 (9.0) [ 13.5–16.7]
48.6 (14.0) [24.4–67.5] 13.8 (6.0) [3.1–26.3] 34.8 (15.0) [13.8–59.4] 20.1 (12.5) [6.9–48.8] 10.4 (7.34) [0.0–22.5] 9.6 (10.5) [ 3.8–33.1] 25.2 (16.3) [0.6–54.4] 3.4 (9.1) [ 13.5–16.7]
48.0 (10.4) [30.6–60.0] 13.6 (7.6) [0.0–22.5] 34.4 (9.7) [15.6–43.8] 15.4 (5.6) [8.1–23.8] 8.2 (5.3) [1.9–15.0] 7.2 (5.4) [0.0–13.8] 27.2 (7.7) [15.6–36.3] 5.6 (9.5) [ 11.7–16.7]
p-Value
0.93 0.96 0.96 0.40 0.51 0.60 0.77 0.41
All values are dB. PTA = Mean of 0.5, 1.0, 2.0, 3.0 kHz. Overclosure is measured by subtracting the mean postoperative bone conduction from the preoperative bone conduction using 1.0, 2.0, and 4.0 kHz. P-value compares outcomes in patients with congenital footplate fixation and juvenile otosclerosis using unpaired t-test.
abnormalities included an incus that was medially rotated, bony fixation from the scutum to the stapes capitulum, and partial lateral chain fixation. All patients with other ossicular abnormailities were treated with a partial or total stapedectomy and a bucket-handle prosthesis. Another patient required a drill-out procedure and had a bifid facial nerve coursing around the stapes. The final air-bone gap was 7.5 dB in this patient. None of the patients with juvenile otosclerosis had associated ossicular or facial nerve abnormailities. Two patients developed a significant decline in bone conduction thresholds postoperatively. The first child underwent a right stapedectomy using a bucket-handle prosthesis for congenital stapes fixation when he was 11 years old. His surgery was uneventful, but he presented to the emergency department ten days after surgery with vertigo and nausea. An audiogram was obtained that demonstrated no change in his bone conduction thresholds and partial closure of the preoperative ABG. He was seen one month later and the audiogram revealed profound sensorineural hearing loss. He returned to the operating room a week later and was found to have purulence in the middle ear space. The prosthesis was felt to be in good position but the handle was not over the incus. The handle of the prosthesis was placed over the incus and the perichondrium graft was left in place. He did not have dizziness postoperatively. He presented to the clinic 10 years later and the audiogram revealed a profound mixed hearing loss and a speech discrimination score of 80%. He did not want a revision stapedectomy or other treatment. The second patient with postoperative sensorineural hearing loss underwent a left stapedectomy using a bucket-handle prosthesis for juvenile otosclerosis at age 10 years. The patient experienced disequilibrium for several days after his uneventful surgery and was doing well at his initial follow up appointment two weeks later. Three weeks after surgery he developed acute Table 2 Comparison of postoperative air-bone gap between patients with congenital footplate fixation and juvenile otosclerosis. Final air-bone gap (dB)
All ears (n = 22) Congenital (n = 16) Otosclerosis (n = 6)
<10 dB (%)
10–20 dB (%)
>20 dB (%)
Sensorineural loss (%)
13 (54.1) 9 (52.9) 4 (57.0)
7 (29.2) 5 (29.4) 2 (28.6)
2 (8.3) 3 (17.6) 0 (0)
1 (4.2) 0 1 (14.3)
vertigo and subjective hearing loss. An audiogram was obtained and showed anacusis. He was placed on oral steroids, antibiotics, and antivirals. CT suggested the prosthesis may have been too long. A revision stapedectomy was performed where the prosthesis was removed and replaced with a shorter prosthesis. There was no evidence of infection. His disequilibrium resolved shortly after surgery, but his hearing did not recover. 4. Discussion Conductive hearing loss without a history of chronic otitis media is an uncommon cause of hearing loss and can be due to multiple causes including congenital stapes fixation, juvenile otosclerosis, tympanosclerosis, aural atresia, lateral ossicular chain fixation, or inner ear abnormalities such as semicircular canal dehiscence or an enlarged vestibular aqueduct. Congenital stapes fixation, juvenile otosclerosis, and some cases of tympanosclerosis can be corrected with a stapedectomy. However, significant sensorineural hearing loss or anacusis may occur in greater than 1% of cases. Therefore, a hearing aid should be discussed with the family as a risk-free option to significantly improve hearing. We discuss and encourage a hearing aid trial before considering a stapedectomy procedure; however, we do not require a hearing aid trial before surgery is considered. Some authors recommend waiting for the child to reach an age where he or she can make an informed decision before a stapedectomy is performed, especially in unilateral cases. Two of the children in this study developed significant sensorineural hearing loss during the postoperative period. Symptoms of labyrinthitis occurred 10–14 days after surgery in both patients despite having an uneventful initial postoperative evaluation. One patient had normal bone conduction audiometry eleven days after surgery and the remaining patient’s Weber tuning fork exam lateralized to the operated ear at the initial postoperative visit. The cause for delayed hearing loss is not fully understood. Acute labyrinthitis is a possibility and may be more common in children that are prone to acute otitis media. Therefore, a stapedectomy should not be performed if the child has a recent history of otitis media or active chronic otitis media. Other possible causes for delayed sensorineural hearing loss include perilymph fistula, middle ear granulation (i.e. reparative granuloma), aseptic labyrinthitis, or iatrogenic trauma. The two most common causes of conductive hearing loss in children without a history of otitis media are congenital stapes fixation and juvenile otosclerosis. In general, CHL in younger
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patients (less than 6 years old) is from congenital stapes fixation since juvenile otosclerosis usually presents in children older than 6 years [8]. The distinction is important since congenital stapes fixation can be associated with other anomalies including facial nerve abnormalities, other ossicular abnormalities, or inner ear abnormalities. One patient in the current study was found to have a bifid tympanic facial coursing superior and inferior to the stapes. This patient required a drill-out procedure for oval window atresia. His surgery was successful closing the ABG from 59.4 dB to 7.5 dB without facial nerve injury. The surgeon must be ready to address abnormalities of the incus and malleus such as lateral chain fixation or a shortened lenticular process. These abnormalities may require the surgeon to utilize various ossiculoplasty techniques beyond a standard stapedectomy. Previous studies have shown juvenile otosclerosis to have better hearing results than congenital stapes fixation [2,9]. In our series, the hearing outcomes measure by postoperative ABG was similar between congenital stapes fixation and otosclerosis. Preoperative workup should include audiometry with acoustic reflexes and a high resolution CT. A conductive hearing loss in the setting of intact reflexes suggests an inner ear third window such as enlarged vestibular aqueduct or semicircular canal dehiscence, and in such cases, a stapedectomy would be contraindicated. CT provides valuable information on the status of the ossicles, course of the facial nerve, and inner ear morphology. Inner ear abnormalities are generally a contraindication for a stapedectomy. In male children, CT is useful in diagnosing X-linked stapes gusher syndrome. This syndrome is characterized by bulbous internal auditory canals, a wide communication between the internal auditory canal and cochlea, and cochlear dysplasia. This condition is a contraindication to stapedectomy due to the high incidence of sensorineural hearing loss [10,11]. Limitations of this manuscript include the data collected in a retrospective manner and a relatively small sample size.
5. Conclusion Stapedectomy for CHL in children has a high rate of significant ABG closure with 83.3% of all operations resulting in closure of the air-bone gap to less than 20 dB. Although congenital stapes fixation has a higher incidence of associated ossicular abnormalities, the hearing outcomes after stapedectomy are similar to juvenile otosclerosis. Delayed sensorineural hearing loss may be more common in the pediatric age group. References [1] D.B. Welling, J.A. Merrell, M. Merz, E.E. Dodson, Predictive factors in pediatric stapedectomy, Laryngoscope 113 (2003) 1515–1518. [2] A. De la cruz, S. Angeli, W.H. Slattery, Stapedectomy in children, Otolaryngol. Head Neck Surg. 120 (1999) 487–492. [3] E. Lescanne, D. Bakhos, J.P. Metais, A. Robier, S. Moriniere, Otosclerosis in children and adolescents: a clinical and CT-scan survey with review of the literature, Int. J. Pediatr. Otorhinolaryngol. 72 (2008) 147–152. [4] W.H. Lippy, J.M. Burkey, A.G. Schuring, F.M. Rizer, Short- and long-term results of stapedectomy in children, Laryngoscope 108 (April (4 Pt 1)) (1998) 569–572. [5] M.L. Carlson, K.M. Van Abel, S. Pelosi, C.W. Beatty, D.S. Haynes, G.B. Wanna, et al., Outcomes comparing primary pediatric stapedectomy for congenital stapes footplate fixation and juvenile otosclerosis, Otol. Neurotol. (January) (2013), EPub ahead of print. [6] B.L. Massey, T.A. Hillman, C. Shelton, Stapedectomy in congenital stapes fixation: are hearing outcomes poorer? Otolaryngol. Head Neck Surg. 134 (May (5)) (2006) 816–818. [7] Committee on Hearing and Equilibrium, Committee on Hearing and Equilibrium guidelines for the evaluation of results of treatment of conductive hearing loss, Otolaryngol. Head Neck Surg. 113 (1995) 186–187. [8] E. Bachor, T. Just, C.G. Wright, H.W. Pau, C.S. Karmody, Fixation of the stapes footplate in children: a clinical and temporal bone histopathologic study, Otol. Neurotol. 26 (September (5)) (2005) 866–873. [9] F. Denoyelle, M. Daval, N. Leboulanger, A. Rousseau, G. Roger, N. Loundon, et al., Stapedectomy in children: causes and surgical results in 35 cases, Arch. Otolaryngol. Head Neck Surg. 136 (October (10)) (2010) 1005–1008. [10] P.D. Phelps, W. Reardon, M. Pembrey, S. Bellman, L. Luxom, X-linked deafness, stapes gushers, and a distinctive defect of the inner ear, Neuroradiology 33 (1991) 326–330. [11] W.E. Nance, R. Setleff, A. McLeod, A. Sweeney, C. Cooper, F. McConnell, X-linked mixed deafness with congenital fixation of the stapedial footplate and perilymphatic gusher, Birth Defects Orig. Artic. Ser. 07 (March (4)) (1971) 64–69.
Contents lists available at SciVerse ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Pediatric stapedectomy: Does cause of fixation affect outcomes? Ryan E. Neilan, Richard W. Zhang, Peter S. Roland, Brandon Isaacson, Kenneth H. Lee, J. Walter Kutz Jr.* University of Texas Southwestern Medical Center, Department of Otolaryngology, 5323 Harry Hines Boulevard, Dallas, TX 75390, United States
A R T I C L E I N F O
A B S T R A C T
Article history: Received 16 January 2013 Received in revised form 8 April 2013 Accepted 12 April 2013 Available online 17 May 2013
Objective: To compare outcomes of stapedectomy in patients with congenital stapes fixation versus juvenile otosclerosis. Methods: A retrospective chart review was performed from January 1, 1999 until January 1, 2011 to identify patients under 18 years old who underwent a stapedectomy. Age, gender, pre- and postoperative audiograms, intraoperative findings including etiology of stapes fixation, prosthesis type, and complications were recorded. Results: Twenty-two children were identified who had undergone a stapedectomy (two patients underwent sequential bilateral surgery) resulting in a total of 24 ears. The cause of fixation included juvenile otosclerosis (n = 7) and congenital stapes fixation (n = 17). The overall mean pre-operative airbone gap (ABG) was 34.7 dB (SD: 13.5) compared to a postoperative mean ABG of 9.0 (SD: 9.3) (p < 0.001). The mean postoperative ABG of 9.6 (SD: 10.5) in the congenital stapes fixation group was similar to the mean postoperative ABG of 7.2 dB (SD: 5.4) in children with juvenile otosclerosis (p = 0.6). Two patients developed delayed profound sensorineural hearing loss approximately two weeks after surgery. One patient with profound sensorineural hearing loss recovered to a profound mixed hearing loss with a speech discrimination score of 80%. Conclusions: Pediatric stapedectomy has comparable results to stapedectomy in adults regardless of the cause of stapes fixation; however, delayed sensorineural hearing loss may be higher in the pediatric population. ß 2013 Elsevier Ireland Ltd. All rights reserved.
Keywords: Stapedectomy Juvenile otosclerosis Congenital stapes fixation Pediatric conductive hearing loss
1. Introduction Conductive hearing loss (CHL) with a dry middle ear cleft is relatively uncommon in the pediatric population and is potentially correctable with surgical intervention. Aside from middle ear effusion, chronic otitis media, and aural atresia, the most common causes of pediatric CHL are congenital stapes fixation and juvenile otosclerosis. Less common causes of conductive hearing loss in children include stapes fixation from osteogenesis imperfecta, congenital cholesteatoma, and juvenile Paget’s disease. In previous studies, there have been differing reports whether patients with congenital stapes fixation have poorer hearing outcomes when compared to patients with juvenile otosclerosis [1–6]. The options for treating pediatric stapes fixation include observation, amplification with a hearing aid, bone anchored hearing aid, or a stapedectomy. Factors to consider when counseling families regarding treatment options include age, history of otitis media, eustachian tube function, family history of
* Corresponding author. Tel.: +1 214 648 3102; fax: +1 214 648 9122. E-mail addresses: [email protected], [email protected] (J. Walter Kutz Jr.). 0165-5876/$ – see front matter ß 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijporl.2013.04.009
hearing loss, associated congenital abnormalities, general medical condition, and laterality. Since otitis media is common in young children, a stapedectomy should be delayed until the child is no longer otitis prone. A CT should be performed before considering a stapedectomy in a child to evaluate for other associated ossicular or inner ear abnormalities. Significant bilateral CHL may bias the decision toward a stapedectomy rather than a hearing aid. We have reviewed our experience with primary stapedectomy in children under 18 years of age and compare hearing outcome between congenital stapes fixation and juvenile otosclerosis. 2. Methods Patients under 18 years of age who underwent a primary stapedectomy by the authors (P.S.R., B.I., J.W.K.) were identified through the clinic database using CPT codes 69660 and 69661. Patients with a history of chronic otitis media, aural atresia, or revision stapedectomy were excluded. Institutional review board approval was obtained before the onset of the study. A retrospective chart review of clinic notes and operative reports were used to record the following parameters: age at time of surgery, gender, laterality, radiographic findings, cause of stapes fixation, surgical technique, prosthesis type and length, facial
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nerve findings, associated ossicular findings, and pre- and postoperative pure tone audiometry. Postoperative audiograms were routinely obtained at 6–8 weeks postoperatively and annually thereafter. The pure tone average (PTA) for air and bone conduction was calculated by averaging thresholds at 500 Hz, 1000 Hz, 2000 Hz, and 3000 Hz. If 3000 Hz was not available, the mean of 2000 Hz and 4000 Hz was used. The mean of 1000 Hz, 2000 Hz, and 4000 Hz was used to calculate the overclosure of bone conduction. The last postoperative audiogram was used for analysis if the patient had multiple postoperative audiograms. Audiologic data were reported according to the published guidelines from the Committee on Hearing and Equilibrium for the evaluation of results of conductive hearing loss [7]. The cause of fixation was determined by examining the following: age, family history, duration of hearing loss, radiographic findings, and intraoperative findings. Univariate analysis consisting of the unpaired t-test was used to compare audiologic outcomes between patients with congenital stapes fixation and juvenile otosclerosis.
3. Results Twenty-two patients with complete pre- and postoperative audiologic data were identified. Two patients underwent sequential bilateral stapedectomy procedures resulting in 24 surgical procedures. Two patients developed delayed postoperative significant sensorineural hearing loss and were excluded from the data analysis as outliers and are discussed individually. Twelve patients were female and a right stapedectomy operation was performed in 13 patients. A partial or total stapedectomy using a bucket-handle prosthesis was performed in 21 ears. A laser stapedotomy with a Nitinol piston prosthesis was performed in three patients. All procedures were performed under general anesthesia. Seventeen ears had congenital stapes fixation as the cause of stapes fixation. Juvenile otosclerosis was the cause of fixation in seven ears. After excluding the two patients with postoperative sensorineural hearing loss, the mean age at the time of operation in patients with congenital stapes fixation was 9.5 years (range: 6.4– 13.6 years) compared to a mean age of 11.3 years (range: 6.7–18.0 years) in children with otosclerosis. This difference was not significant (p = 0.26). The mean follow up that included a comprehensive audiogram was 2.6 years and 1.3 years in the otosclerosis and congenital stapes fixation groups, respectively. A CT temporal bone was obtained on all patients considering surgical intervention to evaluate for inner ear or facial nerve abnormalities that may result in a contraindication for surgery. Most ossicular abnormalities were not appreciated on CT but were identified intraoperatively. Fig. 1 demonstrates a patient with abnormal ossicles. At the time of surgery, he was found to have a short lenticular process and absent stapes crura. Fig. 2 shows an
Fig. 2. Axial CT showing otosclerosis involving the fissula ante fenestrum.
example of juvenile otosclerosis with otosclerosis involving the fissula ante fenestrum. Table 1 compares pre- and postoperative air conduction pure tone average (PTA), bone conduction PTA, and change in air bone gap (ABG) for all children and also stratified by cause of fixation. The mean preoperative ABG for all cases was 34.7 dB (SD: 13.5). The mean preoperative ABG for congenital stapes fixation was 34.8 dB (SD: 15.0) compared to 34.4 dB (SD: 9.7) in ears with juvenile otosclerosis. The mean postoperative ABG for all cases was 9.0 dB (SD: 9.3). The mean postoperative ABG for congenital stapes fixation and juvenile otosclerosis was 9.6 (SD: 10.5) and 7.2 (SD: 5.4) respectively. The preoperative and postoperative outcomes were not statistically significantly different between patients with congenital stapes fixation versus those with juvenile otosclerosis. The mean overclosure of bone conduction was 4.0 dB (SD: 9.0) for all patients. Overclosure was higher in the otosclerosis group at 5.6 dB (SD: 9.5) when compared to overclosure of 3.4 dB (SD: 9.1) in the congenital fixation group. This difference did not reach significance (p = 0.41). Table 2 demonstrates postoperative ABG stratified by degree of closure. For all ears, 54.1% resulted in an ABG < 10 dB and 83.3% resulted in an ABG < 20 dB. For congenital stapes fixation, 52.9% resulted in an ABG < 10 dB and 82.3% resulted in an ABG < 20 dB. For juvenile otosclerosis, 57.0% resulted in an ABG < 10 dB and 85.6% resulted in an ABG < 20 dB. Seven of the seventeen (41.2%) ears with congenital stapes fixation had associated ossicular abnormalities. The most common associated abnormality was discontinuity of the incudostapedial joint, which was observed in four ears. The mean postoperative ABG was 6.7 dB (range: 0.6 dB to 10.0 dB) in this group. The other
Fig. 1. Axial CT showing ossicular abnormalities in a patient with congenital stapes fixation. Intraoperative findings demonstrated a short lenticular process and absent stapes crura.
R.E. Neilan et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 1099–1102
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Table 1 Comparison of preoperative and postoperative hearing outcomes between patients undergoing a stapedectomy for congenital footplate fixation or juvenile otosclerosis.
Preoperative hearing thresholds
Air PTA Bone PTA ABG
Postoperative hearing thresholds
Air PTA Bone PTA ABG
Change in hearing thresholds
Change in ABG Overclosure
All ears (n = 22)
Congenital (n = 16)
Otosclerosis (n = 6)
Mean (SD) [range]
Mean (SD) [range]
Mean (SD) [range]
48.4 (12.9) [24.4–67.5] 13.8 (6.25) [0.0–59.4] 34.7 (13.5) [13.8–59.4] 18.8 (11.1) [6.9–48.8] 9.8 (6.8) [0.0–22.5] 9.0 (9.3) [ 3.8–33.1] 25.7 (14.3) [0.6–54.4] 4.0 (9.0) [ 13.5–16.7]
48.6 (14.0) [24.4–67.5] 13.8 (6.0) [3.1–26.3] 34.8 (15.0) [13.8–59.4] 20.1 (12.5) [6.9–48.8] 10.4 (7.34) [0.0–22.5] 9.6 (10.5) [ 3.8–33.1] 25.2 (16.3) [0.6–54.4] 3.4 (9.1) [ 13.5–16.7]
48.0 (10.4) [30.6–60.0] 13.6 (7.6) [0.0–22.5] 34.4 (9.7) [15.6–43.8] 15.4 (5.6) [8.1–23.8] 8.2 (5.3) [1.9–15.0] 7.2 (5.4) [0.0–13.8] 27.2 (7.7) [15.6–36.3] 5.6 (9.5) [ 11.7–16.7]
p-Value
0.93 0.96 0.96 0.40 0.51 0.60 0.77 0.41
All values are dB. PTA = Mean of 0.5, 1.0, 2.0, 3.0 kHz. Overclosure is measured by subtracting the mean postoperative bone conduction from the preoperative bone conduction using 1.0, 2.0, and 4.0 kHz. P-value compares outcomes in patients with congenital footplate fixation and juvenile otosclerosis using unpaired t-test.
abnormalities included an incus that was medially rotated, bony fixation from the scutum to the stapes capitulum, and partial lateral chain fixation. All patients with other ossicular abnormailities were treated with a partial or total stapedectomy and a bucket-handle prosthesis. Another patient required a drill-out procedure and had a bifid facial nerve coursing around the stapes. The final air-bone gap was 7.5 dB in this patient. None of the patients with juvenile otosclerosis had associated ossicular or facial nerve abnormailities. Two patients developed a significant decline in bone conduction thresholds postoperatively. The first child underwent a right stapedectomy using a bucket-handle prosthesis for congenital stapes fixation when he was 11 years old. His surgery was uneventful, but he presented to the emergency department ten days after surgery with vertigo and nausea. An audiogram was obtained that demonstrated no change in his bone conduction thresholds and partial closure of the preoperative ABG. He was seen one month later and the audiogram revealed profound sensorineural hearing loss. He returned to the operating room a week later and was found to have purulence in the middle ear space. The prosthesis was felt to be in good position but the handle was not over the incus. The handle of the prosthesis was placed over the incus and the perichondrium graft was left in place. He did not have dizziness postoperatively. He presented to the clinic 10 years later and the audiogram revealed a profound mixed hearing loss and a speech discrimination score of 80%. He did not want a revision stapedectomy or other treatment. The second patient with postoperative sensorineural hearing loss underwent a left stapedectomy using a bucket-handle prosthesis for juvenile otosclerosis at age 10 years. The patient experienced disequilibrium for several days after his uneventful surgery and was doing well at his initial follow up appointment two weeks later. Three weeks after surgery he developed acute Table 2 Comparison of postoperative air-bone gap between patients with congenital footplate fixation and juvenile otosclerosis. Final air-bone gap (dB)
All ears (n = 22) Congenital (n = 16) Otosclerosis (n = 6)
<10 dB (%)
10–20 dB (%)
>20 dB (%)
Sensorineural loss (%)
13 (54.1) 9 (52.9) 4 (57.0)
7 (29.2) 5 (29.4) 2 (28.6)
2 (8.3) 3 (17.6) 0 (0)
1 (4.2) 0 1 (14.3)
vertigo and subjective hearing loss. An audiogram was obtained and showed anacusis. He was placed on oral steroids, antibiotics, and antivirals. CT suggested the prosthesis may have been too long. A revision stapedectomy was performed where the prosthesis was removed and replaced with a shorter prosthesis. There was no evidence of infection. His disequilibrium resolved shortly after surgery, but his hearing did not recover. 4. Discussion Conductive hearing loss without a history of chronic otitis media is an uncommon cause of hearing loss and can be due to multiple causes including congenital stapes fixation, juvenile otosclerosis, tympanosclerosis, aural atresia, lateral ossicular chain fixation, or inner ear abnormalities such as semicircular canal dehiscence or an enlarged vestibular aqueduct. Congenital stapes fixation, juvenile otosclerosis, and some cases of tympanosclerosis can be corrected with a stapedectomy. However, significant sensorineural hearing loss or anacusis may occur in greater than 1% of cases. Therefore, a hearing aid should be discussed with the family as a risk-free option to significantly improve hearing. We discuss and encourage a hearing aid trial before considering a stapedectomy procedure; however, we do not require a hearing aid trial before surgery is considered. Some authors recommend waiting for the child to reach an age where he or she can make an informed decision before a stapedectomy is performed, especially in unilateral cases. Two of the children in this study developed significant sensorineural hearing loss during the postoperative period. Symptoms of labyrinthitis occurred 10–14 days after surgery in both patients despite having an uneventful initial postoperative evaluation. One patient had normal bone conduction audiometry eleven days after surgery and the remaining patient’s Weber tuning fork exam lateralized to the operated ear at the initial postoperative visit. The cause for delayed hearing loss is not fully understood. Acute labyrinthitis is a possibility and may be more common in children that are prone to acute otitis media. Therefore, a stapedectomy should not be performed if the child has a recent history of otitis media or active chronic otitis media. Other possible causes for delayed sensorineural hearing loss include perilymph fistula, middle ear granulation (i.e. reparative granuloma), aseptic labyrinthitis, or iatrogenic trauma. The two most common causes of conductive hearing loss in children without a history of otitis media are congenital stapes fixation and juvenile otosclerosis. In general, CHL in younger
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patients (less than 6 years old) is from congenital stapes fixation since juvenile otosclerosis usually presents in children older than 6 years [8]. The distinction is important since congenital stapes fixation can be associated with other anomalies including facial nerve abnormalities, other ossicular abnormalities, or inner ear abnormalities. One patient in the current study was found to have a bifid tympanic facial coursing superior and inferior to the stapes. This patient required a drill-out procedure for oval window atresia. His surgery was successful closing the ABG from 59.4 dB to 7.5 dB without facial nerve injury. The surgeon must be ready to address abnormalities of the incus and malleus such as lateral chain fixation or a shortened lenticular process. These abnormalities may require the surgeon to utilize various ossiculoplasty techniques beyond a standard stapedectomy. Previous studies have shown juvenile otosclerosis to have better hearing results than congenital stapes fixation [2,9]. In our series, the hearing outcomes measure by postoperative ABG was similar between congenital stapes fixation and otosclerosis. Preoperative workup should include audiometry with acoustic reflexes and a high resolution CT. A conductive hearing loss in the setting of intact reflexes suggests an inner ear third window such as enlarged vestibular aqueduct or semicircular canal dehiscence, and in such cases, a stapedectomy would be contraindicated. CT provides valuable information on the status of the ossicles, course of the facial nerve, and inner ear morphology. Inner ear abnormalities are generally a contraindication for a stapedectomy. In male children, CT is useful in diagnosing X-linked stapes gusher syndrome. This syndrome is characterized by bulbous internal auditory canals, a wide communication between the internal auditory canal and cochlea, and cochlear dysplasia. This condition is a contraindication to stapedectomy due to the high incidence of sensorineural hearing loss [10,11]. Limitations of this manuscript include the data collected in a retrospective manner and a relatively small sample size.
5. Conclusion Stapedectomy for CHL in children has a high rate of significant ABG closure with 83.3% of all operations resulting in closure of the air-bone gap to less than 20 dB. Although congenital stapes fixation has a higher incidence of associated ossicular abnormalities, the hearing outcomes after stapedectomy are similar to juvenile otosclerosis. Delayed sensorineural hearing loss may be more common in the pediatric age group. References [1] D.B. Welling, J.A. Merrell, M. Merz, E.E. Dodson, Predictive factors in pediatric stapedectomy, Laryngoscope 113 (2003) 1515–1518. [2] A. De la cruz, S. Angeli, W.H. Slattery, Stapedectomy in children, Otolaryngol. Head Neck Surg. 120 (1999) 487–492. [3] E. Lescanne, D. Bakhos, J.P. Metais, A. Robier, S. Moriniere, Otosclerosis in children and adolescents: a clinical and CT-scan survey with review of the literature, Int. J. Pediatr. Otorhinolaryngol. 72 (2008) 147–152. [4] W.H. Lippy, J.M. Burkey, A.G. Schuring, F.M. Rizer, Short- and long-term results of stapedectomy in children, Laryngoscope 108 (April (4 Pt 1)) (1998) 569–572. [5] M.L. Carlson, K.M. Van Abel, S. Pelosi, C.W. Beatty, D.S. Haynes, G.B. Wanna, et al., Outcomes comparing primary pediatric stapedectomy for congenital stapes footplate fixation and juvenile otosclerosis, Otol. Neurotol. (January) (2013), EPub ahead of print. [6] B.L. Massey, T.A. Hillman, C. Shelton, Stapedectomy in congenital stapes fixation: are hearing outcomes poorer? Otolaryngol. Head Neck Surg. 134 (May (5)) (2006) 816–818. [7] Committee on Hearing and Equilibrium, Committee on Hearing and Equilibrium guidelines for the evaluation of results of treatment of conductive hearing loss, Otolaryngol. Head Neck Surg. 113 (1995) 186–187. [8] E. Bachor, T. Just, C.G. Wright, H.W. Pau, C.S. Karmody, Fixation of the stapes footplate in children: a clinical and temporal bone histopathologic study, Otol. Neurotol. 26 (September (5)) (2005) 866–873. [9] F. Denoyelle, M. Daval, N. Leboulanger, A. Rousseau, G. Roger, N. Loundon, et al., Stapedectomy in children: causes and surgical results in 35 cases, Arch. Otolaryngol. Head Neck Surg. 136 (October (10)) (2010) 1005–1008. [10] P.D. Phelps, W. Reardon, M. Pembrey, S. Bellman, L. Luxom, X-linked deafness, stapes gushers, and a distinctive defect of the inner ear, Neuroradiology 33 (1991) 326–330. [11] W.E. Nance, R. Setleff, A. McLeod, A. Sweeney, C. Cooper, F. McConnell, X-linked mixed deafness with congenital fixation of the stapedial footplate and perilymphatic gusher, Birth Defects Orig. Artic. Ser. 07 (March (4)) (1971) 64–69.