Original Paper

Audiology
Audiol Neurotol 2015;20:237–242 Received: May 15, 2014
Neurotology Accepted after revision: February 16, 2015
DOI: 10.1159/000380940
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

Key Words Introduction

Aplasia · Conductive hearing loss · Middle ear · Ossicular
replacement 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
Abstract membrane and no history of trauma or infection. The
The aim of this study was to describe the audiometric results incidence is 1 in 15,000 births and this malformation is
following surgery in a consecutive series of pediatric pa- bilateral in 30–40% of cases. An ossicular chain malfor-
tients with a congenital middle ear disorder. Retrospective mation is sometimes associated with minor anomalies of
chart review was performed for 29 consecutive children who the outer ear (incomplete aural atresia or defective fold-
underwent 33 middle ear surgeries for congenital ossicular ing) or some genetic syndromes. Classification systems
chain anomaly between 1990 and 2012. Anomalies were categorizing middle ear anomaly and surgical findings
classified into four groups according to the Teunissen and have been developed to analyze the functional results of
Cremers classification. Audiological parameters using four surgery [Teunissen and Cremers, 1993b; Charachon et
frequency averages (0.5, 1, 2 and 4 kHz) were assessed pre- al., 1994; Park and Choung, 2009]. Few studies have in-
and postoperatively. Clinical and audiometric follow-up vestigated this rare congenital anomaly. To our knowl-
times were, respectively, 49 ± 8 and 35 ± 5 months (mean ± edge, this is the first study dealing with three classes of
SEM). Fifty-eight percent of all patients achieved an air-bone middle ear malformations in an exclusively pediatric
gap (ABG) ≤20 dB, 62.5% in class I, 50% in class II and 57.9% population.
in class III. The improvement of the mean ABG was 13.6 dB, The present study focuses on a cohort of 29 pediatric
19.2 dB for class I, 0.2 dB in class II and 15.4 dB in class III. patients with congenital middle ear malformation who
Overall mean pure-tone averages improved 14.8 dB with underwent functional surgery in our institution be-
13.9 dB for class I; there was no improvement for class II and tween 1990 and 2012, with a mean follow-up of 4 years.
20.2 dB for class III. The sensorineural hearing loss rate was Audiometric outcomes were evaluated and compari-
9%. This pediatric series showed that hearing results depend sons were made between classes of ossicular chain ab-
on type of anomaly. Class I and class III showed better hear- normalities according to the Teunissen and Cremers
ing improvement than class II. © 2015 S. Karger AG, Basel classification.
198.143.32.65 - 1/27/2016 6:12:53 PM

© 2015 S. Karger AG, Basel Dr. Stéphanie Quesnel

1420–3030/15/0204–0237$39.50/0 ENT Department, Robert Debré Hospital
McMaster University

48 boulevard Sérurier
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E-Mail steph.quesnel @ gmail.com
 Patients and Methods Table 1. Distribution of ossicular anomalies according to the
Teunissen and Cremers classification
This retrospective study analyzed the audiometric outcomes
after functional surgery of patients with congenital middle ear ab- Class Ears
normality. The surgeries were performed in our pediatric tertiary
academic center between 1990 and 2012. The indications for func- n %
tional surgery were a preoperative air-bone gap (ABG) of above
I: Stapes fixation only 8 24.2
30 dB and an adequate cochlear reserve. The institutional database
II: Stapes fixation with other ossicular malformations 6 18.2
was reviewed and patients with complete individual case histories,
III: Ossicular malformation with mobile stapes 19 57.6
including a preoperative and postoperative audiogram, and a clin-
IV: Aplasia or dysplasia of the oval or round window 0 0
ical and audiometric follow-up greater than 2 months, were se-
lected. Patients with contralateral single-sided deafness, complete Total 33 100
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 uni-
lateral or bilateral congenital middle ear anomalies, the presence
or lack of incomplete aural atresia, associated genetic syndromes,
pre- and postoperative audiometric testing, perioperative findings, cases (n  = 1/29), Goldenhar’s syndrome in 3.4% of pa-
and surgical technique used, were recorded for each patient. tients (n  = 1/29), Beckwith-Wiedemann syndrome in
Ossicular chain anomalies identified during surgery were classi- 3.4% of cases (n = 1/29), and undetermined genetic syn-
fied according to the Teunissen and Cremers classification system
[Teunissen and Cremers, 1993b] and compared with information
drome in 6.9% of patients (n = 2/29).
provided by temporal bone CT scans, when these data were available. Categorization according to the Teunissen and Cre-
Hearing results were assessed by comparing pre- and postop- mers classification system is summarized in table 1. The
erative air conduction (AC) thresholds and pure-tone averages anomaly was bilateral in 14.3% of cases for class I, 20% for
(PTAs) over four frequencies (0.5, 1, 2 and 4 kHz), closure of the class II and 5% for class III patients.
ABG and improvement in AC for all patients, and for each and
between ossicular abnormality classes. A postoperative ABG of
Preoperative CT scans of the temporal bone were per-
20 dB or less was considered to be a successful hearing result. formed in 82% of cases (n = 27/33) and demonstrated the
The operations were performed by 3 surgeons: one performed ossicular anomaly features found during surgery in 81.5%
5 surgeries (15%), one performed 13 surgeries (40%) and one 15 of cases (n = 22/27).
surgeries (45%). As all were experienced surgeons and trained in The clinical follow-up time was 49 ± 8 months (mean ±
the same way, we considered that our results were not affected by
this fact.
SEM) and the audiometric follow-up time was 35 ±
Paired (pre- and postoperative data) and independent Stu- 5 months (mean ± SEM).
dent’s t tests were performed. One-way ANOVA analyses compar-
ing the classes of ossicular anomaly and the different techniques Functional Surgical Techniques
used were performed. Statistical significance was set at p < 0.05. Five types of functional surgical procedures, incus
transposition, Teflon piston, partial ossicular chain re-
construction prosthesis, total ossicular chain reconstruc-
Results tion prosthesis or ossicle liberation, were used, depend-
ing on the type of ossicular chain anomaly present. Table 2
Population Characteristics summarizes these procedures.
The study population consisted of 18 females and 11 Canaloplasty was performed in all cases of incomplete
males (20 female ears and 13 male ears). Congenital os- aural atresia (n  = 11/11) at the same time as ossicular
sicular chain anomaly was predominant on the right side chain surgery. In 2 cases (18.2%), a second canaloplasty
(22 vs. 11) and was bilateral in 4 patients (13.8%). The was required because of a restenosis of the external audi-
mean age of children at the time of surgery was 9.6 ± 3.5 tory canal. At the time of data collection, there were no
years (mean ± SD; range from 2 to 14 years). With regard cases of persistent external auditory canal constriction.
to external ear anomalies, incomplete aural atresia (a nar-
rower ear canal than usual) was found in 33.3% of cases Complications
(n = 11/33 ears), enchondroma in 12.1% of patients (n = Three cases (9%, 2 class I and 1 class II) had a sensori-
4/33 ears) and preauricular pits in 6% of cases (n = 2/33 neural hearing loss greater than 10 dB (mean deteriora-
ears). In 20.7% of cases (n = 6/29), patients had an associ- tion of bone conduction) after functional surgery. Among
ated genetic syndrome: Treacher Collins’ syndrome in these, 1 case had a loss of 30 dB (class I). This patient had
3.4% of patients (n = 1/29), Apert’s syndrome in 3.4% of an inflammatory granuloma (a possible foreign body re-
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238 Audiol Neurotol 2015;20:237–242 Quesnel/Benchaa/Bernard/Martine/Viala/

DOI: 10.1159/000380940
McMaster University

Van Den Abbeele/Teissier
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 Table 2. Type of functional surgery for all patients and according to ossicular anomaly

Incus Teflon PORP TORP Ossicle

transposition piston liberation

Class I (n = 8) 0 (0) 5 (62.5) 1 (12.5) 2 (25) 0 (0)

Class II (n = 6) 1 (16.7)a 3 (50) 0 (0) 2 (33.3) 0 (0)
Class III (n = 19) 8 (42.1) 0 (0) 5 (26.3) 1 (5.3) 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 55±2.3 52.4±2.6 46.1±3 42±2.7 48.9±2.4

Postoperative, dB 38.2±3.7 34.5±3.7 30.4±3.6 33.2±4.1 34.1±3.6
Improvement, dB 16.8** 17.9* 15.7* 8.8*** 14.8**

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

action to Teflon piston) in the tympanic cavity which frequencies (PTA: 51.25–70 dB). No improvement was ob-
led  to lysis of the round window and in the posterior served in the class II ear group for either PTA or hearing
labyrinth. across the four frequencies (n = 6). The class III ear group
Three cases (9%, 1 class I and 2 class II) had second- showed an improvement at all frequencies (n  = 19, p  <
ary prosthesis displacements. In 1 case, after 2 revision 0.0001).
surgeries for prosthesis displacement, the prosthesis The pre- and postoperative ABGs along with ABG im-
was removed (class II, total ossicular chain reconstruc- provement are summarized in tables 5 and 6. The ABG
tion prosthesis). In the other cases, no second surgical improvement was 13.6 ± 3.4 dB (p < 0.001) in all cases,
procedure was undertaken, and patients had hearing 19.2 ± 5.1 dB in the class I ear group (p < 0.05), 0.2 ± 10.8
aids. dB in the class II ear group (not significant, p > 0.05) and
One case (3%) had an external auditory canal choles- 15.4 ± 4.2 dB in the class III ear group (p < 0.05). Twelve
teatoma. 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 =
Hearing Results 23/33) between 0 and 30 dB. Thirty percent of cases (n =
The mean pre- and postoperative AC thresholds are 10/33) had a persistent ABG of more than 30 dB. Using an
shown in tables 3 and 4. The mean overall AC thresholds ABG inferior or equal to 20 dB as an indication of surgical
improved significantly for the four frequencies (0.5, 1, 2 success, surgery upon 62.5% of class I, 50% of class II and
and 4 kHz). The overall PTA improved from 48.9 ± 2.4 to 57.9% of class III ears was considered to be successful.
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  < Influence of Class Groups on Hearing Results
0.05). Two cases from this group (25%, n = 2/8) showed no The mean preoperative PTAs and ABGs did not differ
improvement in hearing levels after surgery, while 1 case between the three classes of ossicular anomalies (Student’s
(12.5%, n  = 1/8) exhibited increased hearing levels at all t test, p > 0.05). The AC gain was statistically significant
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Congenital Middle Ear Anomalies Audiol Neurotol 2015;20:237–242 239

DOI: 10.1159/000380940
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 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 preoperative, dB 60±4.3 58.8±5.2 54.4±5 46.9±5.6 55±4.7

(n = 8) postoperative, dB 43.7±8.4 44.3±9.2 38.7±7.8 37.5±9.7 41.1±8.6
improvement, dB 16.3 14.5 15.7*** 9.4 13.9
Class II preoperative, dB 46.6±6.5 40.3±8.3 33.3±7.4 30.1±7.6 38.5±7
(n = 6) postoperative, dB 45.8±13.9 40.8±12.9 35.8±14.2 36.7±14.3 39.8±13.7
improvement, dB 0.8 –0.5 –2.5 –6.6 –1.3
Class III preoperative, dB 55.5±2.8 52.6±2.9 46.6±3.9 43.4±3.2 49.5±2.8
(n = 19) postoperative, dB 33.4±3.2 28.4±3.1 25.2±3 30.3±4.2 29.3±3.1
improvement, dB 21.6* 24.2* 21.4* 13.1*** 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 38±2.4 44.3±4.3 33.3±5.5 36.7±3.2

Postoperative, dB 24.4±3.2 25.2±7.2 33.1±11.6 21.3±3.1
Improvement, dB 13.6±3.4** 19.2±5.1*** 0.2±10.8 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 12.1 (n = 4) 12.5 (n = 1) 0 (n = 0) 15.8 (n = 3)

≤20 dB 57.6 (n = 19) 62.5 (n = 5) 50 (n = 3) 57.9 (n = 11)
≤30 dB 69.7 (n = 23) 62.5 (n = 5) 66.7 (n = 4) 73.7 (n = 14)
>30 dB 30.3 (n = 10) 37.5 (n = 3) 33.3 (n = 2) 26.3 (n = 5)

between the three classes (one-way ANOVA, p < 0.05, F significant difference for the AC gain and the ABG clo-
value = 3.49) but no differences were noticed in the ABG sure (one-way ANOVA, p = 0.63, F value = 0.65 and p =
improvement (one-way ANOVA, p = 0.16, F value = 1.9). 0.71, F value = 0.52).
Class II ears showed poorer postoperative PTAs, ABGs
and ABG improvement results when compared to other Influence of Syndromes on Hearing Results
classes, but this was not found to be statistically significant. No differences were observed pre- or postoperatively
between patients with or without syndromes concern-
Influence of Functional Surgical Techniques Used on ing the mean PTAs and ABGs (p > 0.05). Comparison
Hearing Results of these patients in each class was not performed
The comparison between the five techniques employed due to the low number of patients with syndromes in
(all patients combined) to improve hearing showed no each class.
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DOI: 10.1159/000380940
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 Discussion among these, 2 had sensorineural impairment and 1 a
prosthesis displacement leading to the need for hearing
Studies evaluating the surgical results of ossicular aids.
chain anomalies in the pediatric population are sparse. Class II anomalies have previously been poorly inves-
To our knowledge, there is one study in the literature tigated, and our study involved only 6 such ears. Despite
dealing with isolated congenital stapes ankylosis in a pe- that, our results are consistent with those described by
diatric population [Albert et al., 2006] and another deal- other authors. Success rates varied from 30 to 70%, and
ing with ossicular malformations associated with a mo- the PTA varied from 18 to 23 dB [Teunissen and Cremers,
bile footplate [Philippon et al., 2013]. Among the pedi- 1991; Kisilevsky et al., 2009; Park et al., 2009; Thomeer et
atric population in this current study, hearing al., 2011]. These results showed that the surgical outcome
impairment as a result of congenital ossicular chain of stapedial fixation may depend on the presence of su-
anomalies was observed. These congenital anomalies perimposed ossicular chain malformations, since com-
are rare and their diagnosis is not easy to achieve in chil- bined ossicular anomalies seem to adversely affect the
dren. During childhood, chronic otitis leads to diagnos- hearing result. These poorer results may be due to an in-
tic difficulties. Moreover, when the malformation is uni- sufficient release of the malleus or the incus from fixation
lateral the diagnosis is generally made later, as hearing or due to a less stable positioning of the prosthesis in the
testing of each ear separately is difficult to undertake case of a malleostapedotomy.
before the age of 6. In our study, surgery for ossicular malformation with
The stapes footplate fixation malformation (class I mobile stapes (class III) yielded good results (tables 4–6):
according to the Teunissen and Cremers classification) PTA improvement was 20.2 dB (p < 0.0001), ABG im-
is thought to be the most common isolated middle ear provement was 15.4 dB (p < 0.05) and the surgery success
anomaly and has been the subject of serial analyses more rate was 57.9%. These results are similar to those found
frequently than any other category of anomalies [Teunis- in other studies: PTA improvement varied from 16 to
sen et al., 1990; Hashimoto et al., 2002; Raveh et al., 2002; 30 dB and success rate varied from 56 to 70% [Teunissen
Hung et al., 2003; Park et al., 2009]. Similar to the obser- and Cremers 1993a; Kisilevsky et al., 2009; Sakamoto et
vations of Kojima et al. [Park and Choung, 2009], os- al., 2011; Thomeer et al., 2012; Philippon et al., 2013].
sicular malformations associated with a mobile foot- Two other studies showed particularly good success rates
plate were predominant (57.6%) in our study, while sta- of 84.6 and 90% and also demonstrated that audiometric
pes fixation was found in 24.2% of cases and stapes results are equivalent, regardless of the type of chain re-
fixation with other ossicular malformations in 18.2% of construction surgery performed [Park and Choung, 2009;
cases. Park et al., 2009].
Hearing results depended on the type of ossicular Our study focused on ossicular chain anomalies in a
chain anomaly present. Surgery yielded better hearing re- pediatric population which was poorly studied in the
sults in cases of isolated stapes fixation (class I) and os- literature. Congenital ossicular anomalies are vari-
sicular malformations associated with a mobile stapes able, however, they can be classified and this classifica-
(class III) than in stapes fixation with other ossicular mal- tion may help to predict the surgical outcomes. As in
formations (class II) and aplasia or dysplasia of the oval previous reports, our study showed that surgical out-
or round windows (class IV) [Park and Choung, 2009; come of stapedial fixation may depend on the presence
Park et al., 2009]. This is consistent with our results: class of superimposed ossicular chain malformations, since
I and III ears showed 62.5 and 57.9% rates of success, re- combined ossicular anomalies appear to adversely af-
spectively, and an ABG improvement of 19.2 and 15.4 dB, fect  the hearing results. Furthermore, audiometric re-
while class II ears showed a success rate of 50% and no sults are, in these particular cases, independent of the
ABG improvement (0.2 dB). type of ossicular chain reconstruction method em-
According to the literature, class I anomalies can ployed.
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; Disclosure Statement
Thomeer et al., 2010]. Our success rates did not match The authors report no conflicts of interest, any sponsorship or
those described, but it is noteworthy that in our study, funding arrangements relating to their study. The authors alone
this ossicular malformation was present in 8 ears, and are responsible for the content and writing of the article.
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242 Audiol Neurotol 2015;20:237–242 Quesnel/Benchaa/Bernard/Martine/Viala/

DOI: 10.1159/000380940
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