Original Paper Audiology Neurotology

Audiol Neurotol 2015;20:237–242 DOI: 10.1159/000380940

Received: May 15, 2014 Accepted after revision: February 16, 2015 Published online: May 12, 2015

Congenital Middle Ear Anomalies: Anatomical and Functional Results of Surgery Stéphanie Quesnel Tarik Benchaa Sophie Bernard François Martine Paul Viala Thierry Van Den Abbeele Natacha Teissier  ENT Department, Robert Debré Hospital, AP-HP, Paris VII University Denis Diderot Sorbonne Paris Cité, Paris, France

Abstract The aim of this study was to describe the audiometric results following surgery in a consecutive series of pediatric patients with a congenital middle ear disorder. Retrospective chart review was performed for 29 consecutive children who underwent 33 middle ear surgeries for congenital ossicular chain anomaly between 1990 and 2012. Anomalies were classified into four groups according to the Teunissen and Cremers classification. Audiological parameters using four frequency averages (0.5, 1, 2 and 4 kHz) were assessed preand postoperatively. Clinical and audiometric follow-up times were, respectively, 49 ± 8 and 35 ± 5 months (mean ± SEM). Fifty-eight percent of all patients achieved an air-bone gap (ABG) ≤20 dB, 62.5% in class I, 50% in class II and 57.9% in class III. The improvement of the mean ABG was 13.6 dB, 19.2 dB for class I, 0.2 dB in class II and 15.4 dB in class III. Overall mean pure-tone averages improved 14.8 dB with 13.9 dB for class I; there was no improvement for class II and 20.2 dB for class III. The sensorineural hearing loss rate was 9%. This pediatric series showed that hearing results depend on type of anomaly. Class I and class III showed better hearing improvement than class II. © 2015 S. Karger AG, Basel

© 2015 S. Karger AG, Basel 1420–3030/15/0204–0237$39.50/0 E-Mail [email protected] www.karger.com/aud

Introduction

Congenital middle ear abnormality of the ear is a rare congenital malformation, leading to conductive hearing loss, in the range of 40–60 dB with normal tympanic membrane and no history of trauma or infection. The incidence is 1 in 15,000 births and this malformation is bilateral in 30–40% of cases. An ossicular chain malformation is sometimes associated with minor anomalies of the outer ear (incomplete aural atresia or defective folding) or some genetic syndromes. Classification systems categorizing middle ear anomaly and surgical findings have been developed to analyze the functional results of surgery [Teunissen and Cremers, 1993b; Charachon et al., 1994; Park and Choung, 2009]. Few studies have investigated this rare congenital anomaly. To our knowledge, this is the first study dealing with three classes of middle ear malformations in an exclusively pediatric population. The present study focuses on a cohort of 29 pediatric patients with congenital middle ear malformation who underwent functional surgery in our institution between 1990 and 2012, with a mean follow-up of 4 years. Audiometric outcomes were evaluated and comparisons were made between classes of ossicular chain abnormalities according to the Teunissen and Cremers classification. Dr. Stéphanie Quesnel ENT Department, Robert Debré Hospital 48 boulevard Sérurier FR–75019 Paris (France) E-Mail steph.quesnel @ gmail.com

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Key Words Aplasia · Conductive hearing loss · Middle ear · Ossicular replacement

This retrospective study analyzed the audiometric outcomes after functional surgery of patients with congenital middle ear abnormality. The surgeries were performed in our pediatric tertiary academic center between 1990 and 2012. The indications for functional surgery were a preoperative air-bone gap (ABG) of above 30 dB and an adequate cochlear reserve. The institutional database was reviewed and patients with complete individual case histories, including a preoperative and postoperative audiogram, and a clinical and audiometric follow-up greater than 2 months, were selected. Patients with contralateral single-sided deafness, complete aural atresia or inner ear malformations were excluded. According to these criteria, 29 patients (33 ears) were selected. Parameters, including patient age, gender, the incidence of unilateral or bilateral congenital middle ear anomalies, the presence or lack of incomplete aural atresia, associated genetic syndromes, pre- and postoperative audiometric testing, perioperative findings, and surgical technique used, were recorded for each patient. Ossicular chain anomalies identified during surgery were classified according to the Teunissen and Cremers classification system [Teunissen and Cremers, 1993b] and compared with information provided by temporal bone CT scans, when these data were available. Hearing results were assessed by comparing pre- and postoperative air conduction (AC) thresholds and pure-tone averages (PTAs) over four frequencies (0.5, 1, 2 and 4 kHz), closure of the ABG and improvement in AC for all patients, and for each and between ossicular abnormality classes. A postoperative ABG of 20 dB or less was considered to be a successful hearing result. The operations were performed by 3 surgeons: one performed 5 surgeries (15%), one performed 13 surgeries (40%) and one 15 surgeries (45%). As all were experienced surgeons and trained in the same way, we considered that our results were not affected by this fact. Paired (pre- and postoperative data) and independent Student’s t tests were performed. One-way ANOVA analyses comparing the classes of ossicular anomaly and the different techniques used were performed. Statistical significance was set at p < 0.05.

Results

Population Characteristics The study population consisted of 18 females and 11 males (20 female ears and 13 male ears). Congenital ossicular chain anomaly was predominant on the right side (22 vs. 11) and was bilateral in 4 patients (13.8%). The mean age of children at the time of surgery was 9.6 ± 3.5 years (mean ± SD; range from 2 to 14 years). With regard to external ear anomalies, incomplete aural atresia (a narrower ear canal than usual) was found in 33.3% of cases (n = 11/33 ears), enchondroma in 12.1% of patients (n = 4/33 ears) and preauricular pits in 6% of cases (n = 2/33 ears). In 20.7% of cases (n = 6/29), patients had an associated genetic syndrome: Treacher Collins’ syndrome in 3.4% of patients (n = 1/29), Apert’s syndrome in 3.4% of 238

Audiol Neurotol 2015;20:237–242 DOI: 10.1159/000380940

Table 1. Distribution of ossicular anomalies according to the Teunissen and Cremers classification

Class

Ears n

I: Stapes fixation only 8 II: Stapes fixation with other ossicular malformations 6 III: Ossicular malformation with mobile stapes 19 IV: Aplasia or dysplasia of the oval or round window 0 Total

% 24.2 18.2 57.6 0

33 100

cases (n  = 1/29), Goldenhar’s syndrome in 3.4% of patients (n  = 1/29), Beckwith-Wiedemann syndrome in 3.4% of cases (n = 1/29), and undetermined genetic syndrome in 6.9% of patients (n = 2/29). Categorization according to the Teunissen and Cremers classification system is summarized in table 1. The anomaly was bilateral in 14.3% of cases for class I, 20% for class II and 5% for class III patients. Preoperative CT scans of the temporal bone were performed in 82% of cases (n = 27/33) and demonstrated the ossicular anomaly features found during surgery in 81.5% of cases (n = 22/27). The clinical follow-up time was 49 ± 8 months (mean ± SEM) and the audiometric follow-up time was 35 ± 5 months (mean ± SEM). Functional Surgical Techniques Five types of functional surgical procedures, incus transposition, Teflon piston, partial ossicular chain reconstruction prosthesis, total ossicular chain reconstruction prosthesis or ossicle liberation, were used, depending on the type of ossicular chain anomaly present. Table 2 summarizes these procedures. Canaloplasty was performed in all cases of incomplete aural atresia (n  = 11/11) at the same time as ossicular chain surgery. In 2 cases (18.2%), a second canaloplasty was required because of a restenosis of the external auditory canal. At the time of data collection, there were no cases of persistent external auditory canal constriction. Complications Three cases (9%, 2 class I and 1 class II) had a sensorineural hearing loss greater than 10 dB (mean deterioration of bone conduction) after functional surgery. Among these, 1 case had a loss of 30 dB (class I). This patient had an inflammatory granuloma (a possible foreign body reQuesnel/Benchaa/Bernard/Martine/Viala/ Van Den Abbeele/Teissier

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Patients and Methods

Table 2. Type of functional surgery for all patients and according to ossicular anomaly

Incus transposition

Teflon piston

PORP

TORP

Ossicle liberation

Class I (n = 8) Class II (n = 6) Class III (n = 19)

0 (0) 1 (16.7)a 8 (42.1)

5 (62.5) 3 (50) 0 (0)

1 (12.5) 0 (0) 5 (26.3)

2 (25) 2 (33.3) 1 (5.3)

0 (0) 0 (0) 5 (26.3)

Total (n = 33)

9 (27.3)

8 (24.2)

6 (18.2)

5 (15.15)

5 (15.15)

Number and percent of ears (in parentheses). PORP  = Partial ossicular chain reconstruction prosthesis; TORP = total ossicular chain reconstruction prosthesis. a  In this case, stapes was removed and incus was placed between oval window and tympanic membrane.

Table 3. Mean overall AC thresholds

Total (n = 33)

500 Hz

1 kHz

2 kHz

4 kHz

PTA (0.5–4 kHz)

Preoperative, dB Postoperative, dB Improvement, dB

55±2.3 38.2±3.7 16.8**

52.4±2.6 34.5±3.7 17.9*

46.1±3 30.4±3.6 15.7*

42±2.7 33.2±4.1 8.8***

48.9±2.4 34.1±3.6 14.8**

Mean ± SEM. Student’s t test: * p < 0.0001; ** p < 0.001; *** p < 0.05.

Hearing Results The mean pre- and postoperative AC thresholds are shown in tables 3 and 4. The mean overall AC thresholds improved significantly for the four frequencies (0.5, 1, 2 and 4 kHz). The overall PTA improved from 48.9 ± 2.4 to 34.1 ± 3.6 dB (mean ± SEM, p < 0.001). In the class I ear group, an improvement was noticed at 2 kHz only (p  < 0.05). Two cases from this group (25%, n = 2/8) showed no improvement in hearing levels after surgery, while 1 case (12.5%, n  = 1/8) exhibited increased hearing levels at all Congenital Middle Ear Anomalies

frequencies (PTA: 51.25–70 dB). No improvement was observed in the class II ear group for either PTA or hearing across the four frequencies (n = 6). The class III ear group showed an improvement at all frequencies (n  = 19, p  < 0.0001). The pre- and postoperative ABGs along with ABG improvement are summarized in tables 5 and 6. The ABG improvement was 13.6 ± 3.4 dB (p < 0.001) in all cases, 19.2 ± 5.1 dB in the class I ear group (p < 0.05), 0.2 ± 10.8 dB in the class II ear group (not significant, p > 0.05) and 15.4 ± 4.2 dB in the class III ear group (p < 0.05). Twelve percent of cases (n = 4/33) closed the ABG to within 0–10 dB, 57.6% (n = 19/33) to within 0–20 dB, and 69.7% (n = 23/33) between 0 and 30 dB. Thirty percent of cases (n = 10/33) had a persistent ABG of more than 30 dB. Using an ABG inferior or equal to 20 dB as an indication of surgical success, surgery upon 62.5% of class I, 50% of class II and 57.9% of class III ears was considered to be successful. Influence of Class Groups on Hearing Results The mean preoperative PTAs and ABGs did not differ between the three classes of ossicular anomalies (Student’s t test, p > 0.05). The AC gain was statistically significant Audiol Neurotol 2015;20:237–242 DOI: 10.1159/000380940

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action to Teflon piston) in the tympanic cavity which led  to lysis of the round window and in the posterior labyrinth. Three cases (9%, 1 class I and 2 class II) had secondary prosthesis displacements. In 1 case, after 2 revision surgeries for prosthesis displacement, the prosthesis was removed (class II, total ossicular chain reconstruction prosthesis). In the other cases, no second surgical procedure was undertaken, and patients had hearing aids. One case (3%) had an external auditory canal cholesteatoma.

Table 4. Mean AC thresholds for each class ear group

500 Hz

1 kHz

2 kHz

4 kHz

PTA (0.5–4 kHz)

Class I (n = 8)

preoperative, dB postoperative, dB improvement, dB

60±4.3 43.7±8.4 16.3

58.8±5.2 44.3±9.2 14.5

54.4±5 38.7±7.8 15.7***

46.9±5.6 37.5±9.7 9.4

55±4.7 41.1±8.6 13.9

Class II (n = 6)

preoperative, dB postoperative, dB improvement, dB

46.6±6.5 45.8±13.9 0.8

40.3±8.3 40.8±12.9 –0.5

33.3±7.4 35.8±14.2 –2.5

30.1±7.6 36.7±14.3 –6.6

38.5±7 39.8±13.7 –1.3

Class III (n = 19)

preoperative, dB postoperative, dB improvement, dB

55.5±2.8 33.4±3.2 21.6*

52.6±2.9 28.4±3.1 24.2*

46.6±3.9 25.2±3 21.4*

43.4±3.2 30.3±4.2 13.1***

49.5±2.8 29.3±3.1 20.2*

Mean ± SEM. Student’s t test: * p < 0.0001; *** p < 0.05.

Table 5. Mean ABG for all ears and for each class

ABG (0.5–4 kHz)

Total (n = 33)

Class I (n = 8)

Class II (n = 6)

Class III (n = 19)

Preoperative, dB Postoperative, dB Improvement, dB

38±2.4 24.4±3.2 13.6±3.4**

44.3±4.3 25.2±7.2 19.2±5.1***

33.3±5.5 33.1±11.6 0.2±10.8

36.7±3.2 21.3±3.1 15.4±4.2***

Mean ± SEM. Student’s t test: * p < 0.0001; ** p < 0.001; *** p < 0.05.

Table 6. Success rates (%) according to postoperative value of ABG for all patients and for each class

ABG (0.5–4 kHz)

Total (n = 33)

Class I (n = 8)

Class II (n = 6)

Class III (n = 19)

≤10 dB ≤20 dB ≤30 dB >30 dB

12.1 (n = 4) 57.6 (n = 19) 69.7 (n = 23) 30.3 (n = 10)

12.5 (n = 1) 62.5 (n = 5) 62.5 (n = 5) 37.5 (n = 3)

0 (n = 0) 50 (n = 3) 66.7 (n = 4) 33.3 (n = 2)

15.8 (n = 3) 57.9 (n = 11) 73.7 (n = 14) 26.3 (n = 5)

Influence of Functional Surgical Techniques Used on Hearing Results The comparison between the five techniques employed (all patients combined) to improve hearing showed no 240

Audiol Neurotol 2015;20:237–242 DOI: 10.1159/000380940

significant difference for the AC gain and the ABG closure (one-way ANOVA, p = 0.63, F value = 0.65 and p = 0.71, F value = 0.52). Influence of Syndromes on Hearing Results No differences were observed pre- or postoperatively between patients with or without syndromes concerning the mean PTAs and ABGs (p > 0.05). Comparison of these patients in each class was not performed due to the low number of patients with syndromes in each class. Quesnel/Benchaa/Bernard/Martine/Viala/ Van Den Abbeele/Teissier

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between the three classes (one-way ANOVA, p < 0.05, F value = 3.49) but no differences were noticed in the ABG improvement (one-way ANOVA, p = 0.16, F value = 1.9). Class II ears showed poorer postoperative PTAs, ABGs and ABG improvement results when compared to other classes, but this was not found to be statistically significant.

Studies evaluating the surgical results of ossicular chain anomalies in the pediatric population are sparse. To our knowledge, there is one study in the literature dealing with isolated congenital stapes ankylosis in a pediatric population [Albert et al., 2006] and another dealing with ossicular malformations associated with a mobile footplate [Philippon et al., 2013]. Among the pediatric population in this current study, hearing impairment as a result of congenital ossicular chain anomalies was observed. These congenital anomalies are rare and their diagnosis is not easy to achieve in children. During childhood, chronic otitis leads to diagnostic difficulties. Moreover, when the malformation is unilateral the diagnosis is generally made later, as hearing testing of each ear separately is difficult to undertake before the age of 6. The stapes footplate fixation malformation (class I according to the Teunissen and Cremers classification) is thought to be the most common isolated middle ear anomaly and has been the subject of serial analyses more frequently than any other category of anomalies [Teunissen et al., 1990; Hashimoto et al., 2002; Raveh et al., 2002; Hung et al., 2003; Park et al., 2009]. Similar to the observations of Kojima et al. [Park and Choung, 2009], ossicular malformations associated with a mobile footplate were predominant (57.6%) in our study, while stapes fixation was found in 24.2% of cases and stapes fixation with other ossicular malformations in 18.2% of cases. Hearing results depended on the type of ossicular chain anomaly present. Surgery yielded better hearing results in cases of isolated stapes fixation (class I) and ossicular malformations associated with a mobile stapes (class III) than in stapes fixation with other ossicular malformations (class II) and aplasia or dysplasia of the oval or round windows (class IV) [Park and Choung, 2009; Park et al., 2009]. This is consistent with our results: class I and III ears showed 62.5 and 57.9% rates of success, respectively, and an ABG improvement of 19.2 and 15.4 dB, while class II ears showed a success rate of 50% and no ABG improvement (0.2 dB). According to the literature, class I anomalies can achieve 80% success, with the mean gain varying from 21 to 40 dB [Teunissen and Cremers, 1991; Albert et al., 2006; Park and Choung, 2009; Park et al., 2009; Thomeer et al., 2010]. Our success rates did not match those described, but it is noteworthy that in our study, this ossicular malformation was present in 8 ears, and Congenital Middle Ear Anomalies

among these, 2 had sensorineural impairment and 1 a prosthesis displacement leading to the need for hearing aids. Class II anomalies have previously been poorly investigated, and our study involved only 6 such ears. Despite that, our results are consistent with those described by other authors. Success rates varied from 30 to 70%, and the PTA varied from 18 to 23 dB [Teunissen and Cremers, 1991; Kisilevsky et al., 2009; Park et al., 2009; Thomeer et al., 2011]. These results showed that the surgical outcome of stapedial fixation may depend on the presence of superimposed ossicular chain malformations, since combined ossicular anomalies seem to adversely affect the hearing result. These poorer results may be due to an insufficient release of the malleus or the incus from fixation or due to a less stable positioning of the prosthesis in the case of a malleostapedotomy. In our study, surgery for ossicular malformation with mobile stapes (class III) yielded good results (tables 4–6): PTA improvement was 20.2 dB (p < 0.0001), ABG improvement was 15.4 dB (p < 0.05) and the surgery success rate was 57.9%. These results are similar to those found in other studies: PTA improvement varied from 16 to 30 dB and success rate varied from 56 to 70% [Teunissen and Cremers 1993a; Kisilevsky et al., 2009; Sakamoto et al., 2011; Thomeer et al., 2012; Philippon et al., 2013]. Two other studies showed particularly good success rates of 84.6 and 90% and also demonstrated that audiometric results are equivalent, regardless of the type of chain reconstruction surgery performed [Park and Choung, 2009; Park et al., 2009]. Our study focused on ossicular chain anomalies in a pediatric population which was poorly studied in the literature. Congenital ossicular anomalies are variable, however, they can be classified and this classification may help to predict the surgical outcomes. As in previous reports, our study showed that surgical outcome of stapedial fixation may depend on the presence of superimposed ossicular chain malformations, since combined ossicular anomalies appear to adversely affect  the hearing results. Furthermore, audiometric results are, in these particular cases, independent of the type of ossicular chain reconstruction method employed. Disclosure Statement The authors report no conflicts of interest, any sponsorship or funding arrangements relating to their study. The authors alone are responsible for the content and writing of the article.

Audiol Neurotol 2015;20:237–242 DOI: 10.1159/000380940

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Discussion

References

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Congenital middle ear anomalies: anatomical and functional results of surgery.

The aim of this study was to describe the audiometric results following surgery in a consecutive series of pediatric patients with a congenital middle...
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