10.14639/0392-100X-N2271
Objective. To assess the hearing benefit with a unilateral bone conduction hearing aid in a cohort of children with unilateral aural atresia. Methods. Cross-sectional case series pilot study involving 7 children (median age: 10 years, range 6-11). All patients underwent pure-tone, speech, aided sound field and aided speech audiometry and Simplified Italian Matrix Test (SIMT) with and without bone conduction hearing aid (Baha 5® CochlearTM). Cognitive abilities were assessed in 5 patients. Results. The mean air conduction pure-tone average (PTA) of the atretic ear was 63.2 ± 6.9 dB, while the bone conduction PTA was 12.6 ± 4.7 dB. Speech discrimination score of the atretic ear was 88.6 ± 3.8 dB, while with the hearing aid it was 52.8 ± 1.9 dB. In the contralateral ear, there was no significant air-bone gap, and PTAs for air and bone conduction thresholds were within normal range (PTA ≤ 25 dB). The mean aided air conduction hearing threshold was 26.2 ± 7.97. Mean speech recognition threshold without the hearing aid was -5.1 ± 1.9 dB, and -6.0 ± 1.7 dB with the hearing aid tested with the SIMT. The mean score of the cognitive test was 46.8 ± 42.8.Conclusions. These preliminary findings should encourage clinicians in proposing a unilateral bone conduction hearing aid in children with unilateral atresia.
text/xml
1827-675X
2271
en
Patrizia Trevisi
http://creativecommons.org/licenses/by-nc-nd/4.0
ACTA Otorhinolaryngologica Italica
Simplified Italian Matrix Test
Benefits of bone conduction hearing aid in children with unilateral aural atresia
Text.Serial.Journal
https://www.actaitalica.it/
43
Otology
2023-05-16
2022-08-11
2023-05-19
2023-05-19
221-226
1
Patrizia Trevisi
2023/05/16
10.14639/0392-100X-N2271
221
Section of Otorhinolaryngology, Neurosciences Department, University of Padua, Padua, Italy
1827-675X
Simplified Italian Matrix Test
en
226
Benefits of bone conduction hearing aid in children with unilateral aural atresia
https://www.actaitalica.it/article/download/2271/992
43
ACTA OTORHINOLARYNGOLOGICA ITALICA 2023;43:221-226; doi: 10.14639/0392-100X-N2271




Otology


Benefits of bone conduction hearing aid
in children with unilateral aural atresia
Beneficio dell’apparecchio acustico a conduzione ossea nei bambini
con atresia auris unilaterale
Davide Brotto*, Flavia Sorrentino*, Diego Cazzador, Francesca Maritan, Silvia Montino, Anna Agostinelli,
Elisabetta Zanoletti, Alessandro Martini, Roberto Bovo, Patrizia Trevisi
Section of Otorhinolaryngology, Neurosciences Department, University of Padua, Padua, Italy
*D. Brotto and F. Sorrentino contributed equally to the study and should be considered first co-authors.



SUMMARY
Objective. To assess the hearing benefit with a unilateral bone conduction hearing aid in a
cohort of children with unilateral aural atresia.
Methods. Cross-sectional case series pilot study involving 7 children (median age: 10
years, range 6-11). All patients underwent pure-tone, speech, aided sound field and aided
speech audiometry and Simplified Italian Matrix Test (SIMT) with and without bone con-
duction hearing aid (Baha 5® CochlearTM). Cognitive abilities were assessed in 5 patients.
Results. The mean air conduction pure-tone average (PTA) of the atretic ear was 63.2 ± 6.9
dB, while the bone conduction PTA was 12.6 ± 4.7 dB. Speech discrimination score of the
atretic ear was 88.6 ± 3.8 dB, while with the hearing aid it was 52.8 ± 1.9 dB. In the con-
tralateral ear, there was no significant air-bone gap, and PTAs for air and bone conduction
thresholds were within normal range (PTA ≤ 25 dB). The mean aided air conduction hearing
threshold was 26.2 ± 7.97. Mean speech recognition threshold without the hearing aid was
-5.1 ± 1.9 dB, and -6.0 ± 1.7 dB with the hearing aid tested with the SIMT. The mean score
of the cognitive test was 46.8 ± 42.8.
Conclusions. These preliminary findings should encourage clinicians in proposing a unilat-              Received: August 11, 2022
eral bone conduction hearing aid in children with unilateral atresia.                                   Accepted: October 3, 2022
KEY WORDS: aural atresia, bone conduction hearing aids, unilateral hearing loss,
                                                                                                        Correspondence
conductive hearing loss, Simplified Italian Matrix Test
                                                                                                        Davide Brotto
                                                                                                        Section of Otorhinolaryngology, Neurosciences
RIASSUNTO                                                                                               Department, University of Padova, via Giustiniani
                                                                                                        2, 35128 Padua, Italy
Obiettivo. Verificare il beneficio uditivo con protesi monolaterale per via ossea in pazienti affetti
                                                                                                        Tel. +39 0498218776. Fax +39 0498211994
da atresia auricolare unilaterale, una rara causa di ipoacusia trasmissiva unilaterale congenita.       E-mail: davidebrotto@hotmail.it
Metodi. Uno studio pilota trasversale di casi ha coinvolto 7 bambini (età mediana di 10
anni, intervallo 6-11). I pazienti sono stati sottoposti ad audiometria tonale, vocale, tonale
protesica e vocale protesica e Matrix test Italiano semplificato (SIMT) con e senza appa-                 How to cite this article: Brotto D, Fla-
                                                                                                          via Sorrentino F, Cazzador D, et al. Benefits
recchio acustico a conduzione ossea (Baha 5® CochlearTM).
                                                                                                          of bone conduction hearing aid in children
Risultati. La soglia tonale media (PTA) della conduzione aerea dell’orecchio atresico era                 with unilateral aural atresia. Acta Otorhi-
di 63,2 ± 6,9 dB, mentre la PTA della conduzione ossea era di 12,6 ± 4,7 dB. Il punteggio                 nolaryngol Ital 2023;43:221-226. https://doi.
di discriminazione vocale (SDS) dell’orecchio atretico era di 88,6 ± 3,8 dB, con l’apparec-               org/10.14639/0392-100X-N2271
chio acustico l’SDS era di 52,8 ± 1,9 dB. Nell’orecchio controlaterale, non vi era alcun gap
aereo-osso significativo e le PTA per le soglie di conduzione aerea e ossea erano all’interno           © Società Italiana di Otorinolaringoiatria
dell’intervallo normale (PTA ≤ 25 dB). La soglia uditiva media per via aerea con l’appa-                e Chirurgia Cervico-Facciale

recchio acustico era di 26,2 ± 7,97 dB. La soglia media di riconoscimento vocale senza
                                                                                                                       OPEN ACCESS
l’apparecchio acustico era -5,1 ± 1,9 dB, mentre era -6,0 ± 1,7 dB con l’apparecchio acu-
stico testato con il SIMT. Il punteggio medio del test cognitivo era 46,8 ± 42,8.                       This is an open access article distributed in accordance with
Conclusioni. I risultati preliminari di questo studio incoraggiano i medici a proporre l’uso            the CC-BY-NC-ND (Creative Commons Attribution-Non-
                                                                                                        Commercial-NoDerivatives 4.0 International) license. The
unilaterale di un apparecchio acustico a conduzione ossea nell’atresia unilaterale nei bambini.         article can be used by giving appropriate credit and mentio-
                                                                                                        ning the license, but only for non-commercial purposes and
PAROLE CHIAVE: atresia auricolare, protesi acustiche a conduzione ossea, ipoacusia                      only in the original version. For further information: https://
unilaterale, ipoacusia trasmissiva, Matrix Test Italiano Semplificato                                   creativecommons.org/licenses/by-nc-nd/4.0/deed.en




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D. Brotto et al.




Introduction                                                      To evaluate the possible benefit of a bone-conduction hear-
                                                                  ing aid (BCHA) in background noise, we performed an
Unilateral aural atresia is a rare condition in which the ex-     audiological study on a group of paediatric patients with
ternal auditory canal is not developed thus causing a pure        unilateral aural atresia using the SIMT.
conductive hearing loss up to around 50 dB in air-bone
gap 1. Aural atresia is considered part of the oculo-auricu-
lo-vertebral spectrum (OAVS), a rare syndrome 2 in which          Materials and methods
patients can present a severe phenotype with aural, facial,       Patients
ocular and cervical spinal involvement called Goldenhar           Seven consecutive children (3 females and 4 males, mean
syndrome. The craniofacial structures derived from the first      age 9 ± 2 years, median 10, range 6-11) presenting with
and second pharyngeal arches may be altered on one (85%           congenital unilateral aural atresia underwent audiological
of cases) or both sides 3-5. The resulting typical facial asym-   evaluation with and without bone conduction hearing aid in
metry 6 might be accompanied by anomalies involving cra-          the affected side at a third referral University centre. Five
nial nerves 7, brain, internal carotid artery, inner ear 8 and    patients presented unilateral atresia on the right side. Five
salivary glands 9. These multiple anomalies can have dif-         patients underwent evaluation of cognitive abilities.
ferent clinical consequences, and in particular the hearing       No patient had previous experience of hearing aids accord-
loss caused by the aural atresia can have an impact on the        ing to the parents’ choice, despite being informed since the
communication and learning skills during the development.         first phases of the child’s life. All patients presented ap-
Although in bilateral aural atresia hearing rehabilitation        propriate development according to speech and language
with bone conduction hearing aids is nowadays manda-              evaluations.
tory with a specific bilateral fitting 10, in the latest years
the necessity to rehabilitate a unilateral deficit has also       Audiological assessment
emerged 11. Indeed, unilateral hearing loss impacts sound         The evaluation of hearing performance was carried out by
localisation and speech perception in background noise,           clinicians and technicians experienced in the management
thus being a non-negligible factor in the hearing and             of hearing loss and rare congenital diseases. Audiologi-
speech evolution of the child 12-14. Unfortunately, most of       cal assessment included evaluation of medical history and
the literature on this topic is based on small studies 15,16.     otoscopy, pure-tone, speech, aided sound field and aided
Nevertheless, recent studies show that patients affected by       speech audiometry and SIMT. All tests were conducted in
unilateral hearing loss may present poor academic 17 and          a 4.2 m x 2.5 m soundproof booth, using an Otometrics
speech performance 18, with non-negligible impact on the          MADSEN® Astera2 audiometer with a set of TDH39 head-
quality of life of the children 19. Patients with unilateral      phones and Indiana Line loudspeakers.
atresia can face ominous difficulties in noisy environments,      Pure tone audiometry (PTA) was performed for both air
with multiple speakers and high cognitive load, and pos-          and bone conduction and the PTA was measured for the
sible impact on scholastic performance causing long term          frequencies 0.5, 1, 2 and 4 kHz. Aided sound field audiom-
disabilities. This represents a major issue if concomitant        etry was performed with the bone conduction hearing aid
cognitive impairment is present. Nowadays, the currently          positioned on the mastoid of the affected ear by means of a
available bone conduction hearing aids are small, light and       headband. On the contralateral normal hearing ear, mask-
interconnected with smartphones, tablets, or remote micro-        ing narrow band noise was administered with headphones
phones, which are extremely useful for paediatric patients,       at 50 dB over the hearing threshold.
especially in scholastic environments in which background         Speech audiometry was performed in quiet, testing the
noise can impair listening abilities.                             atretic side with headphones, administering masking white
Up to now, some useful tests to prove hearing performance         noise in the contralateral normal ear. Aided speech audi-
in background noise are available, such as the Simplified         ometry was performed in free field conditions, with the
Italian Matrix Test (SIMT) that can give a quantitative           bone conduction hearing aid positioned on the mastoid of
evaluation of the hearing impairment in noisy conditions 20.      the affected ear using a headband. On the contralateral nor-
Hearing benefit with bone conduction hearing aids is re-          mal hearing ear, masking white noise was administered by
ported in conductive hearing loss due to multiple aetiolo-        means of headphones. Speech discrimination score (SDS)
gies such as chronic otitis, cholesteatoma and otosclero-         represents the sound level (in dB) at which the patient cor-
sis 21 in adult patients, but studies on the possible benefits    rectly identifies 100% of administered words.
in background noise in the paediatric population with uni-        The Italian Matrix Sentence Test (IMST) is a speech audiom-
lateral aural atresia are still lacking.                          etry in background noise with automatically adapted signal to



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noise ratio (SNR). It is structured into 20 randomised lists of              Two of the children were too tired for further activities and
five-word sentences, semantically unpredictable and adminis-                 results of the cognitive tests were not considered reliable.
tered after a training session to minimise the learning curve 22.            Patients were evaluated with the Raven’s Colored Progres-
The SIMT is specific for children aged 4 years or more and                   sive Matrices (CPM) test 1, the leading global non-verbal
uses 14 randomised lists of three-word sentences with the                    measure of mental ability in research, educational and clin-
same structure (number, adjective, noun). The background                     ical settings. The CPM test comprises 36 items (brightly
noise is presented at a fixed 65 dB sound pressure level                     coloured to attract and maintain children’s attention), di-
(SPL), while the speech material is actively adapted by the                  vided into three sets of 12 (set A, Ab and B), and ordered in
software. The result of the test is a score representing the                 terms of increasing difficulty. The sets have been designed
SNR in dB at which the patient can recognise 50% of the                      to distinguish between degrees of intellectual maturity by
speech material: the speech recognition threshold (SRT) 20.                  quantifying a child’s ability to form comparisons and to
A specific setup finalised to reproduce as faithfully a class-               reason by analogy 23.
room environment was chosen. Two loudspeakers were                           The Raven’s CPM results in a score that can be converted
placed respectively 1 meter in front and behind the sub-                     into a percentile, by mean of a specific conversion table
ject, the noise was presented at a fixed level of 65 dB at 0°                also considering the age of the subjects.
and 180° azimuth, the speech was presented at 0° azimuth                     Patients scoring less than 5 are considered to have men-
(Fig. 1) and the sentence level was automatically adapted                    tal abilities strongly below average, between 5 and 25 with
according to the preceding response.                                         results below average, results between 25 and 75 are con-
                                                                             sidered normal, scoring higher than 75 is considered above
                                                                             average and more than 95 are considered strongly above
Hearing aid
                                                                             average.
All patients were evaluated with and without the same bone
conduction hearing aid. The chosen hearing aid was the Ba-
                                                                             Statistical analysis
ha 5® CochlearTM set for a normal bone conduction thresh-
                                                                             Continuous variables were expressed as median and range.
old (PTA under 25 dB nHL). The positioning of the hearing
                                                                             The nonparametric Wilcoxon Signed Rank Sum test was
aid was performed by a physician experienced in audiology
                                                                             used to assess the modifications in hearing status before
using a headband. Children were invited to wear the device
                                                                             and after Baha 5® CochlearTM use. Spearman test was ap-
for at least an hour after which they underwent audiological                 plied to identify correlations between continuous variables.
testing. The correct position of the Baha 5® CochlearTM was                  The level of significance was α = 0.05. Statistical analysis
checked by a technician right before testing.                                was carried out with SPSS 24.0 software.
Assessment of cognitive abilities
Patients underwent evaluation of mental abilities performed                  Results
with a developmental psychologist experienced in paediat-                    Audiological and cognitive evaluation results for each pa-
ric development and hearing loss. The evaluation was per-                    tient are presented in Table I. The median air conduction
formed after the audiological evaluation to maximise chil-                   hearing threshold of the atretic ear, expressed as PTA was
dren’s attention capacities during the audiological testing.                 66.2 dB nHL (range 28.7-70.0 dB nHL), while the median




Figure 1. Schematic representation of the setting of audiological testing.



                                                                                                                                        223
D. Brotto et al.




bone conduction hearing threshold was 11.2 dB nHL (range                                      face congenital unilateral hearing loss with crucial impact
10.0, 23.3 dB nHL). The median aided air conduction hear-                                     in the global development of the child. Most probably, pa-
ing threshold was 25.0 dB nHL (range 18.7, 38.7 dB nHL).                                      tients with unilateral atresia might be “forced” to a unilateral
Median SDS of the atretic ear 88.6 ± 3.8 dB, with the hear-                                   hemispheric dominance if the atretic ear is not stimulated in
ing aid was 52.8 ± 1.9 dB nHL. In the contralateral ear, there                                the first years of life 25. The consequence can be a reduced
was no relevant air-bone gap, and PTAs for air conduction                                     benefit perception when a hearing aid is used in adulthood.
and bone conduction thresholds were within normal limits                                      In addition, the concomitant presence of poor cognitive
(PTA ≤ 25 dB). The median ABG significantly improved                                          performance or attention deficit and hearing loss might
with Baha 5® CochlearTM, from 51.2 dB nHL (range 40.0,                                        have tremendous impact on attention abilities and conse-
57.7 dB nHL) to 13.7 dB nHL (range 7.5-23.7 dB), (Wil-                                        quently on scholastic performance of affected children.
coxon Signed Rank test, p = 0.018). At SIMT, median SNR                                       Moreover, classrooms are known to be extremely noisy en-
without the hearing aid was -5.6 dB (range -7.0, -1.6 dB),                                    vironments in which acoustic conditions can be extremely
while it was -6.4 dB nHL (range -7.8, -2.3 dB nHL) with                                       challenging 26.
the hearing aid, which resulted in a statistically significant                                SIMT is a recently developed hearing test that is useful to
improvement (Wilcoxon Signed Rank test, p = 0.027). Five                                      evaluate hearing performance in background noise. The
of seven patients performed the cognitive test with a medi-                                   test is composed of words familiar to children and consists
an score of 37 (range 2-97). Cognitive status did not corre-                                  of an adapted version of the Italian Matrix Test, a test that
late with either Baha 5® CochlearTM -aided PTA or Baha 5®                                     is becoming more common in clinical and research set-
CochlearTM-aided ABG (Spearman correlation test, ρ = 0.2,                                     tings 20,22,27. In other countries, the simplified test has been
p = 0.741 and ρ = 0.1, p = 0.873, respectively).                                              shown to be feasible even in the elderly 28.
                                                                                              In the present study, all children were able to perform the
                                                                                              test since it is perceived as a game. Obviously, older chil-
Discussion
                                                                                              dren were more collaborative and completed the test rap-
The present study, performed in children with unilateral                                      idly. The difference in terms of cognitive abilities did not
atresia, highlights the benefits of a bone conduction hearing                                 seem to impair the evaluation nor did it correlate with the
aid (specifically the Baha 5® CochlearTM) in background                                       hearing results. Indeed, all patients showed better results
noise as measured by the SIMT. The benefit is noticeable                                      while using the hearing aid and children with poor cog-
in all patients, regardless of cognitive abilities.                                           nitive skills seem to have better hearing results. Since the
In recent studies, chronic conductive unilateral hearing loss                                 non-aided hearing performance (measured with the PTA)
has been shown to cause speech intelligibility deficits 24,                                   of the present cohort is in line with previous studies 1 and
poor academic performance 17, and overall, a reduced quality                                  most probably the benefits of a hearing aid can be expected
of life in the paediatric population 18. This can be exacer-                                  in larger populations of patients with unilateral atresia.
bated in patients with congenital conditions causing hear-                                    Despite the risk of a possible masking effect of the BCHA
ing impairment, such as aural atresia. These patients must                                    on the contralateral normal hearing ear, no patient showed a


Table I. Audiological and cognitive results of the patients.
 PT                      Age         Sex Side           AC-PTA                  BC-PTA AE            PTA          SDS          SDS             SRT          SRT         Cognitive
                                                           AE                      (dB)             BCHA           AE          BCHA            (dB)        BCHA          score
                                                          (dB)                                       (dB)         (dB)        AE (dB)                       (dB)          (%)
 1                             10          F        R          68.75               11.25            18.75         90            60             -5.8         -6.7            37
 2                              7          F        R          66.25               11.25             25           90            50             -6.9         -6.9            84
 3                              6          F        R            70                 23.3            38.75         90            60             -1.6         -2.3            NA
 4                             11          M        L            50                  10             18.75         90            30             -5.2         -6.1             2
 5                             10          M        L          58.75                 10             21.25         90            40              -7          -7.8            97
 6                              8          M        R          66.25               11.25            26.25         90            70             -3.8         -6.4            NA
 7                             11          M        R           62.5               11.25             35           80            60             -5.6         -5.9            14
 Mean values                  9.00         -        -          63.21               12.61            26.25        88.57         52.85          -5.13        -6.01           46.8
 Standard deviation           2.00                             6.95                4.74             7.87         3.77          13.8            1.89         1.75          42.08
PT: patient; F: female; M: male; R: right; L: left; AC: air conduction; PTA: pure-tone audiometry; AE: atretic ear; BC: bone conduction; SRT: speech recognition threshold; SDS: speech
discrimination score; BCHA: bone conduction hearing aid; NA: not available.




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                                                                                                                BCHA in unilateral atresia




decrease in auditory performance when tested with SIMT.          Conclusions
Consequently, the present study consolidates and reinforces
                                                                 The present study, conducted on a relatively small cohort
the idea that BCHA in unilateral conductive HL might im-
prove speech perception in noise thanks to the summation         of patients affected by unilateral aural atresia, shows that
effect, but the setting of this study prevented investigation    SIMT can be considered a feasible instrument to evaluate
of real binaural hearing. In this regard, the evaluation of      hearing performance in noisy context. Baha 5® CochlearTM
the possible masking effect of a BCHA on the contralateral       was shown to be a valuable device that can provide ben-
hearing should be considered beyond the objectives of the        efit in complex hearing conditions, and this benefit can be
present study, and this effect can deteriorate speech percep-    even more relevant for children with poor cognitive abili-
tion due to the reduction of binaural cues.                      ties. These findings suggest that early rehabilitation with
The main limitation of the study is the small sample size        a bone conduction hearing aid should be considered and
of the cohort considered. Unfortunately, the rarity of the       discussed with patients’ families as promising results have
pathology impairs the collection of large cohorts of patients    been documented. In conclusion, it is likely time to address
even more when considering the specific target of the study      the problem of good hearing in patients with unilateral atre-
(i.e. patients with unilateral atresia without experience of     sia from another side, the affected one.
bone conduction hearing aids). Moreover, the collaboration
of children is limited in time thus causing the loss of some     Conflict of interest statement
data. The objective for the future is to extend the cohort and   The authors declare no conflict of interest.
compare these results with cohorts of patients with longer
periods of training or that use BCHA from the first phases       Funding
of life.                                                         This research did not receive any specific grant from fund-
Consequently, the above-mentioned results should be              ing agencies in the public, commercial, or not-for-profit
considered preliminary, but they show that a benefit can         sectors.
be perceived by young unilateral atretic patients even im-
mediately after the very first use of the hearing aid. These     Author contributions
findings may be even better in patients with longer train-       DB, FS, PT: conceived the idea of the study; DB, FS, FM,
ing periods using the device on daily basis. In addition, the    SM, AA: developed the theory and performed the tests; DB,
modern bone conduction hearing aids allow extended con-          FS, DC, EZ, AM, RB, PT: verified the analytical methods
nectivity via Bluetooth®. This may be of particular interest     and supervised the findings of this work. All authors dis-
in the paediatric population since remote communication          cussed the results and contributed to the final manuscript.
systems can be connected to multiple devices and teachers
can use these features in scholastic environments. In addi-      Ethical consideration
tion, the hearing aid can be connected to smartphones and/       This study was approved by the Institutional Ethics Com-
or tablets, thus encouraging its use in ludic contexts. In any   mittee of the Azienda Ospedale Università Padova (proto-
case, instruments such as the Baha 5® CochlearTM are useful      col number 1077).
to increase the signal to noise ratio, suggesting their use in   The research was conducted ethically, with all study pro-
noisy environments. Moreover, this specific device can be        cedures being performed in accordance with the require-
worn with a headband, an arch, or implanted with a tem-          ments of the World Medical Association’s Declaration of
poral bone screw or magnetic contacts, allowing children         Helsinki.
to test the device and its benefits before surgical implanta-    All patients or parents of children gave written informed
tion. The daily experience with these patients is that many      consent for study participation and data publication.
children are enthusiastic to at least try a bone conduction
hearing aid. Nonetheless, it is common experience that aes-      References
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