International Journal of Pediatric Otorhinolaryngology Extra
Volume 6, Issue 4 , Pages 178-181, December 2011

Goldenhar syndrome with moderate hearing loss: An FM system in a school environment

  • Mònica Carrión

      Affiliations

    • Education Resource Center for Hearing Impaired Children, Generalitat of Catalonia, Spain
    • ,
  • Genís Cardona

      Affiliations

    • University Vision Center Research Group, Technical University of Catalonia, Spain
    • Corresponding Author InformationCorresponding author at: Escola Universitària d’Òptica i Optometria de Terrassa, Violinista Vellsolà 37, 08222 Terrassa, Catalonia, Spain. Tel.: +34 93 739 8774.

Received 7 May 2010; received in revised form 19 July 2010; accepted 21 July 2010. published online 19 August 2010.

Article Outline

Abstract 

A young girl with Goldenhar syndrome and bilateral, unbalanced, fluctuating and progressive hearing loss successfully employed an FM system during classroom activities. Teacher and student questionnaires revealed overall satisfaction with the FM system, as well as a significant progress in the academic performance. Schoolchildren with mild hearing loss often reject the use of conventional hearing aids during school hours, when acoustic inadequacies and social stigma eclipse any perceived benefit. FM systems such as EduLink™ are a viable alternative in those situations and, in some instances, may predispose reluctant patients to accept a more conventional hearing solution.

Keywords: Classroom acoustics, Conductive hearing loss, FM systems, Goldenhar syndrome, Speech-perception

 

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1. Introduction 

Dr. Maurice Goldenhar [1] described several cases of oculoauricular dysplasia, with epibulbar dermoids, auricular appendages and periauricular fistulae, a constellation of abnormalities thereupon known as Goldenhar syndrome (GS). Upper lid coloboma and vertebral and facial anomalies were later included as part of the syndrome by Gorlin et al. [2], renaming it oculo-auriculo-vertebral dysplasia, a term that was further refined as facio-auriculo-vertebral sequence by Smith [3] to embrace both GS and hemifacial microsomia.

The prevalence of GS is difficult to estimate, as some cases with minor anomalies remain undetected and other cases with more extensive cranial malformations may be misdiagnosed [4]. It may nevertheless be safe to assume an incidence of about 1/5600, with a male:female ratio of 3:2 and a ratio of right versus left side affectation of 3:2 [5]. Some authors have postulated a minimal diagnostic criterion for GS, including the presence of at least two of the following: unilateral microtia, unilateral mandibular hypoplasia, uni- or bi-lateral epibulbar dermoids or vertebral malformation [6]. However, other manifestations are often combined with those of a classic GS and may involve cleft lip and/or palate, rib and extremities anomalies, congenital heart disease and mental retardation, amongst others [7], thus increasing the difficulty of a clear cut definition of the condition.

Improper development of the external, middle or inner ear may lead to different types of hearing impairment associated with GS, although conductive or mixed types tend to be more frequent and were encountered in 76% of the patients by Touliatou et al. [8]. Indeed, outer and middle ear abnormalities occur in over 90% of cases [9] and include microtia and atresia of the external ear canal, ossicular anomalies such as fusion between the malleus and incus, and otitis media with effusion [10]. Malformations of the inner ear usually involve dysplasia of the cochlea and the semicircular canals.

Interestingly, Tasse et al. [11] reported less severe forms of hearing loss in patients who inherited the disease in an autosomal dominant manner than those in whom the condition originated from a spontaneous mutation, the most common genetic origin of GS. Bilateral profound hearing deficit is nevertheless very rare in GS and most cases of unilateral impairment allow for a normal speech development and a good social interaction.

Surgical treatment of GS related anomalies usually consists of primary reconstruction of the affected areas, correction of ear deformities and extirpation of preauricular tags [12]. Given the highly heterogeneous nature of the condition, a case-by-case approach is necessary and interventions may be so diverse as to include cochlear implantation, anterior tympanotomy, ossiculoplasty, removal of obstructive tissue and others. GS patients without widespread external malformations may benefit from the use of standard hearing aids and extreme cases of bilateral microtia with atresia and conductive hearing loss are good candidates for the Bone Anchored Hearing Aid (BAHA) [13]. FM systems may prove useful for GS patients with mild or moderate hearing loss when challenged with inadequate acoustic environments such as classrooms, where lowered signal to noise ratios rend speech comprehension difficult.

The following report describes a case of young girl diagnosed with bilateral but asymmetric Goldenhar syndrome accompanied with moderate conductive right hearing loss and progressive left hearing deficit. Given the initial difficulty of a standard hearing aid solution, an FM system was recommended, and successfully employed, for classroom activities.

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2. Case report 

A 10-year-old Caucasian girl was diagnosed with GS when examined upon birth. She was born through cesarean section at the 39th week of gestation and scored 9/10 on the Apgar test. She presented facial asymmetry, bilateral auricular dysplasia, more pronounced in the right side, and micrognathia, which resulted in a certain difficulty to open the mouth and ingest aliments. Ophthalmological exploration revealed a slightly larger right palpebral fissure. No other anomalies were detected at that time and no significant familial antecedents were reported.

An initial audiometric evaluation through auditory evoked potentials, at 2 years of age, disclosed a conductive hearing loss in the right ear, with an auditory threshold at 70dB, whereas contralateral hearing was considered to be within the range of normality (see Fig. 1 and Table 1). These results were in agreement with initial pure tone audiometric examinations (see Fig. 2), in which bilateral normal sensorineural hearing was found to coexist with a mild hearing loss in the left ear and a moderately severe right impairment, caused by her significant external malformations. A recent longitudinal audiometric assessment, however, has exposed left hearing loss to be fluctuating in nature and progressive in time.

Table 1. Right and left auditory evoked potentials thresholds (in ms).
Right earLeft ear
IIIIVI–IIII–VIII–VIIIIVI–IIII–VIII–V
80dB 5.106.80 1.701.353.555.202.203.851.65
70dB 7.35
60dB
50dB 2.404.406.002.003.601.60
40dB
30dB 5.006.70 1.70
20dB 5.807.55 1.75

Tympanometry discovered bilateral type B, or flat, tympanograms, suggestive of fluid in the middle ear. Indeed, a possible malformation of the Eustachian tubes was hypothesized as the main cause of this recurrent problem, which failed to resolve through tympanocentesis. An ossiculoplasty, performed at 8 years of age, was not successful in significantly improving sound conduction on the left side.

The patient had developed lip reading skills from an early age as a means of helping her speech recognition. The increasingly complex school environment, however, in addition to her progressive left hearing deficit, proved too demanding for her abilities and resulted in a notable negative impact on her academic performance. The patient was initially reluctant to resort to conventional hearing aids for her daily activities. Unfortunately, her dysfunctional family and complex social background had an adverse effect on the psychological profile of the patient and also prevented her to receive the appropriate counsel regarding the importance of normal hearing and early conventional hearing aid use.

An FM system was recommended, to be employed during classroom hours in order to improve teacher–student communication. The EduLink™S (Phonak AG, Stäfa, Switzerland) (see Fig. 3), which does not require for any supplementary auditory prosthesis to be equipped, was used in the left ear, as right ear impairment was too severe to benefit from the amplification provided by this system (the most relevant characteristics of the EduLink™S FM system are summarized in Table 2). Hearing and academic performance improvements were subsequently assessed through the standard student and teacher questionnaires developed by the Education Resource Center for Hearing Impaired Children of the Generalitat of Catalonia, which are partially based on the SIFTER and LIFE rating instruments [14], [15]. Answers to these questionnaires revealed a satisfactory or very satisfactory appraisal of her understanding of the instructions and explanations given by her teachers, as well as a deeper level of involvement in general classroom discussions and activities, grading mathematics and foreign language subjects as the areas were her FM system was most useful and music education as the subject were its performance was most compromised. In addition, teachers acknowledged that, without implementing any significant change in their educational methodology, a significant improvement was noticed in their communication with the patient, in her ability to follow complex oral instructions and in her initiative to participate in group discussions and other situations were oral expression is fundamental. In general, the patient admitted a good satisfaction with her FM system.

Table 2. Most relevant technical specifications of the EduLink™S FM system.
Frequency range169.4–176.0MHz (HA band)
176.0–181.9MHz (HB band)
214.0–220.0MHz (NB band)
Frequency selectivity>50dB for a 150kHz separation
Signal to noise ratio45dB
Frequency output200–6,000Hz
Operating range20m
Supply312 battery (40–50h)
LegislationEN 300.422 and EN 300.445 (Europe)
FCC Part 15C (US)

It is of great interest to note that the experienced benefits of the FM system and her increasing hearing loss have recently persuaded the patient to employ conventional hearing aids for all her daily activities outside the classroom. In addition, and in order to facilitate her journey through the school curriculum, she has been receiving uninterrupted assistance, since she was 2 years old, from both a speech-language pathologist and a special educator.

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3. Discussion 

The present report describes the case of a young girl with GS associated with conductive bilateral but unbalanced hearing loss. From a very early age, the patient manifested attention, concentration and speech-discrimination difficulties, especially in the classroom, which resulted in a negative impact on her academic progress and social interaction abilities.

Although extensive regulation exists (ANSI S12-60-2002) [16], classroom acoustics are usually far from optimal. Finitzo-Hieber and Tillman [17] identified several factors with a harmful effect on speech-perception, both in children with and without hearing impairment, namely distance from speaker, room reverberation time and background noise. Indeed, for every time that distance from speaker doubles, a reduction of approximately 6dB occurs in the intensity of the signal, thus transforming speech intensity from an average of 70dBA at 1m to 58dBA at a distance of 4m. Room reverberation time should not exceed 0.6s for early reverberation signals to positively contribute to speech-perception while simultaneously avoiding the adverse influence of later reverberation waves. Finally, background noise, which is endemic in every school environment, often reaches 55dBA, well above the recommended maximum of 35dBA in unoccupied classrooms. The added contribution of these three factors serves to define the actual speed to noise ratio at every location in the classroom, relative to the speaker, a value that is further modulated by the frequency distribution of the signal (vowels, at lower frequencies, usually sound louder than consonants, at higher frequencies, albeit consonants play a dominant role in determining speech intelligibility) but that, in average, has been established at a minimum of 6dB for speech-perception in children with normal hearing. Besides, speech-discrimination has proved very sensitive to small changes in signal to noise ratio. Nillson et al. [18] reported a decrement of 10% in speech-discrimination for every 1dB of degradation in the signal to noise ratio in normal hearing conditions.

Children with hearing loss require a superior signal to noise ratio for speech-perception than normal hearing children, thus encouraging them, as in the present case, to develop adaptive mechanisms and strategies such as lip reading skills and selective sitting arrangements and body orientation in the classroom. However, the effectiveness of these and other tactics is prone to be greatly threatened in common classroom scenarios involving teacher mobility relative to the student or group discussions and activities.

Conventional hearing aids alone are not the best option in this type of challenging acoustic environments, and their benefits are probably absent when background noise reaches 60dBA. Besides, via peer pressure, schoolchildren of a certain age may negatively influence the acceptance of hearing aids and lead the wearer to perceive them as a social stigma. Although it is the responsibility of the professional to counsel parents on patience and persistence in working with the child to use the amplification in order to receive the benefits of that amplification, the particular family and social background characteristics of this case initially prevented this solution to be successfully implemented. The EduLink™S FM system offers an attractive, almost invisible, behind-the-ear design, additionally allowing the wearer to converse with classmates without removing the device.

FM systems have been documented to be effective among individuals who experience communication difficulties despite wearing conventional hearing aids [19], either as an alternative to those instruments or linked with them [20]. The close proximity of the microphone to the mouth of the speaker (15cm or less) minimizes the effect of distance, reverberation and background noise, while delivering the signal directly to the ears of the listener. FM systems in the classroom have been reported to improve signal to noise ratio by 20–30dB with reference to unaided conditions [21] and by 12–18dB when compared to conventional hearing aids [22]. Johnston et al. [23], in a group of children with auditory processing disorder, revealed that these benefits reverted in improvements in speech-perception, academic performance and psychosocial function which were even maintained after children ceased wearing their FM aids.

The present case report mirrors most of these findings, as evinced by the answers to the student and teacher questionnaires, obtained some time after the FM system was in use. It is of capital relevance to stress the fact that the experienced benefit of the FM system was a decisive factor to persuade the patient to employ conventional hearing aids for her daily activities. Furthermore, it may be worth mentioning that a significant part of our reported success stemmed from the high level of implication and cooperation of all intervening agents and from the task of the governmental Education Resource Center for Hearing Impaired Children in instructing the wearer and also the teachers in the most adequate use of the FM system, which resulted in a better understanding of the associated difficulties of hearing impaired schoolchildren and in the development of complementary strategies to ameliorate them.

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4. Conclusion 

The use of a FM system in a classroom acoustic environment has proved beneficial in the case of a young patient with conductive hearing impairment resulting from GS and as a viable alternative to conventional hearing aids. The improvement in signal to noise ratio which was attained with this system enhanced speech-perception and led to a significant progress in her academic performance, social interaction and quality of life in general. In addition, the perceived hearing benefit of the FM system has recently influenced the patient to employ conventional hearing aids.

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Conflict of interest statement 

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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References 

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PII: S1871-4048(10)00054-7

doi:10.1016/j.pedex.2010.07.004

International Journal of Pediatric Otorhinolaryngology Extra
Volume 6, Issue 4 , Pages 178-181, December 2011

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