Cochlear implantation in 13q deletion syndrome

Purpose

The advent of cochlear implantation has been followed by an explosion in surgical and technological advances with subsequent alterations in the treatment of sensorineural hearing loss management. Many individuals with rare genetic abnormalities who once may have not been considered candidates for cochlear implantation are now benefiting from cochlear implantation. One of these unusual syndromes is the 13q deletion syndrome. The clinical features of 13q deletion syndrome, a rare chromosomal abnormality, were originally described in the early 1960s, though the literature currently lacks defined phenotypic abnormalities. Patients with 13q deletion syndrome present with varying degrees of cognitive and growth delay, craniofacial dysmorphisms, and congenital malformations. The etiology of the sensorineural hearing loss has not been elucidated, and genes associated with other syndromes which include sensorineural hearing loss have been proposed as the causative agents. We describe the unique clinical and surgical considerations for 13q deletion syndrome and review the considerations when deliberating on cochlear implantation in syndromic hearing loss.

Materials and Methods

Case report.

Results

Successful cochlear implantation in a patient with 13q deletion syndrome.

Conclusion

Many patients with 13q deletion syndrome suffer from sensorineural hearing loss, and when appropriately selected may likely benefit from cochlear implantation. Many other syndromic patients following careful selection may also benefit from cochlear implantation and should not be excluded from consideration until appropriately evaluated.     

The cochlea magnesium content is negatively correlated with hearing loss induced by impulse noise

Objective

Magnesium is proved to attenuate acoustic trauma, and reactive oxygen species (ROS) formation is a critical role that involves hearing loss induced by impulse noise. We aimed to investigate the relationship between the cochlea magnesium content, ROS formation and hearing loss induced by impulse noise.

Methods

Ninety pigmented guinea pigs were exposed to impulse noise. Auditory thresholds were assessed by sound-evoked auditory brainstem response (ABR) 24 h before and 72 h after exposure to impulse noise. 4-Hydroxynonenal(HNE) used as a marker of ROS was determined immunohistochemically. The cochlea magnesium content was examined with the method of energy dispersive x-ray analysis, and the cochlea was also detected with scanning electron microscope. The relationship between the cochlea magnesium content, ROS formation and hearing loss was analyzed.

Results

There was loss of outer hair cell cilia accompanying with significant auditory threshold shift after impulse noise exposure. ROS was positive in the organ of Corti of all animals. The cochlea magnesium content was negatively correlated with ROS formation and hearing loss.

Conclusions

Inhibiting ROS formation is one of the mechanisms for magnesium to reduce acoustic trauma, and difference in cochlea magnesium contents is one of the factors that induce varying degrees of cochlear damage among each individual after acoustic trauma.     

Congenital and acquired cytomegalovirus infection and hearing evaluation in children

Objectives

Congenital cytomegalovirus (CMV) infection is one of the most common intrauterine diseases. In all, 1% of live births is affected by cytomegalovirus infection, while 90% neonates with perinatal infection do not show symptoms of disease. Symptomatic CMV is present in 5–10% of children. Typical clinical signs of CMV infection are microcephalia, mental retardation, progressive major amblyacousia, and neuromuscular infection. Hypoacusis is present in 30–60% of children with congenital symptomatic CMV – in most cases it is bilateral and applies to high frequency hearing loss. The purpose of this article is to emphasize the importance of hearing evaluation in children with congenital and acquired cytomegalovirus infection.

Patients and methods

A group of 70 children had serological and genetic screening for CMV DNA, using PCR method, in urine and blood. In this group, 52 children were diagnosed with congenital CMV and 18 children had acquired CMV. Audiological examinations including PTA, ABR, TEOAE and immittance audiometry were performed.

Results

Bilateral sensorineural hearing losses were found in 9 children, associated with mental and physical retardation, brain malformation and microcephalia, and unilateral losses in 3 children. In 40 cases, we did not observe hearing loss, although the level of bilirubin was high, and splenomegaly, hepatomegaly and paralysis of facial nerve were present. In the group of children with acquired CMV, we did not notice hearing loss.

Conclusions

This research proved that CMV infection often caused hearing loss. In spite of this, all children with congenital and acquired CMV should be monitored and assessed throughout their lifetime by an audiologist.     

Simultaneous translabyrinthine removal of acoustic neuroma and cochlear implantation

Objectives

To report of a 65-year-old woman with bilateral Meniere's disease was referred for cochlear implantation (CI) due to severe/profound sensorineural hearing loss.

Methods

During the assessment workup, a vestibular schwannoma in the right ear was found by MR imaging. She underwent a translabyrinthine removal of the acoustic neuroma (AN) with sparing of the cochlear nerve and concurrent ipsilateral CI with a Nucleus Freedom device (Cochlear Ltd., Lane Cove, New South Wales, Australia).

Results

Complete removal of the AN was achieved without complications. Neural Response Telemetry (NRT) measurements, which showed poor morphology at the intraoperative tests, rapidly improved after activation, similarly to electrically evoked auditory brainstem responses (E-ABR). The patient reached 100% speech perception performances within 2 months from implantation, in the monaural condition. She was relieved from vertigo spell up to 14 months after the operation.

Conclusion

Cochlear implantation at the time of acoustic neuroma removal with VIII nerve sparing can be a safe and effective hearing restoration procedure.     

Comparison of functional and morphologic characteristics of mice models of noise-induced hearing loss

Objectives

This study was conducted to compare morphologic and audiologic changes after noise exposure in two different strains of mice (CBA and C57) and to create morphologically proven models of noise-induced hearing loss.

Methods

Mice were exposed to white noise at 110-dB sound-pressure level for 60 minutes at the age of 1 month. Hearing thresholds and outer hair cell functions were evaluated by auditory brainstem response recordings and distortion product otoacoustic emission immediately and 22 days after noise exposure. Cochlear pathology was observed and compared by light and electron microscopic studies.

Results

Both mice strains showed hearing threshold shifts with decreased outer hair cell function immediately and 22 days after noise exposure. More severe auditory brainstem response threshold shifts were observed in C57 mice compared with CBA mice at click, 8-, 16-, and 32-kHz tone-burst stimuli. A cochlear morphologic study demonstrated predominant outer hair cell degeneration at all turns of the cochlea; degeneration was most severe at the basal turn in both mice strains. A scanning electron microscopic study revealed more severe ultrastructural damage of outer hair cells at each turn of the cochlea in C57 mice. The lateral wall of the cochlea was more severely degenerated in CBA mice.

Conclusion

Both mice strains showed consistent, permanent noise-induced hearing loss with different susceptibilities and site vulnerabilities. Further studies to investigate the mechanism of the different degree and cochlear site vulnerability to noise exposure between two mice strains are necessary.     

Compound heterozygosity for dominant and recessive GJB2 mutations in a Tunisian family and association with successful cochlear implant outcome

Objectives

Mutations of GJB2 encoding connexin 26 are the most common cause of hearing loss. They are responsible for up to 50% of ARNSHL. The pathogenic mutations in this gene are generally inherited recessively. Dominant mutations in GJB2 also cause hearing loss, either in isolated non-syndromic form or as part of a syndrome associated with various skin disorders.

Methods

We screened a Tunisian child affected by congenital, bilateral, profound, sensorineural hearing loss for mutations in GJB2 gene using PCR and direct sequencing.

Results

The proband was found to be compound heterozygous for recessive and dominant GJB2 mutations respectively p.V37I (c.109G > A) and p.R143Q (c.428G > A). Surprisingly the hearing mother is a carrier for this dominant GJB2 mutation. This proband underwent a cochlear implant at four years old. The evaluation using APCEI and IT-MAIS tests at six months post implantation indicates a successful cochlear implant outcome since the deaf child began to acquire language abilities and auditory sensation.

Conclusions

The p.R143Q mutation was described for the first time in Tunisia. We confirm the low penetrance of this mutation since the proband mother is a carrier despite her normal hearing. We show the effectiveness of cochlear implant to restore the communication abilities and auditory sensation for our patient.     

Autism and peripheral hearing loss: A systematic review

Objective

To systematically review the literature describing the relationship between autism spectrum disorder (ASD) and peripheral hearing loss including literature recommendations for audiological assessment and auditory habilitation in cases where peripheral hearing loss and ASD coexist.

Data sources

Published studies indexed in MEDLINE (1948–2011).

Review methods

The search strategy identified 595 potential studies. After a review of the titles, 115 abstracts were reviewed and 39 articles were retrieved and assessed independently by at least two authors for possible inclusion. 22 articles pertained to children with ASD and peripheral hearing loss, hearing assessment in children with ASD, audiological habilitation for children with ASD or hyper-responsiveness in children with ASD. 17 further studies were garnered from the reference section of the 22 papers.

Results

Controversy exists in the literature regarding prevalence of hearing impairment among individuals with ASD. In cases where ASD and hearing impairment co-exist, diagnosis of one condition often leads to a delay in diagnosing the other. Audiological assessment can be difficult in children with ASD and test–retest reliability of behavioural thresholds can be poor. In cases where hearing impairment exists and hearing aids or cochlear implantation are recommended, devices are often fit with special considerations for the child with ASD. Hyper-responsiveness to auditory stimuli may be displayed by individuals with ASD. Evidence or the suspicion of hyper-responsiveness may be taken into consideration when fitting amplification and planning behavioural intervention.

Conclusions

Prevalence rates of hearing impairment among individuals with ASD continue to be debated. At present there is no conclusive evidence that children with ASD are at increased risk of peripheral hearing loss. A complete audiological assessment is recommended in all cases where ASD is suspected so as not to delay the diagnosis of hearing impairment in the event that hearing loss and ASD co-exist. Objective assessment measures should be used to confirm behavioural testing in order to ensure reliability of audiological test results. Fitting of hearing aids or cochlear implantation are not contraindicated when hearing loss is present in children with ASD; however, success with these devices can be variable.     

Effect of age at cochlear implantation on auditory and speech development of children with auditory neuropathy spectrum disorder

Objective

To evaluate the auditory and speech abilities in children with auditory neuropathy spectrum disorder (ANSD) after cochlear implantation (CI) and determine the role of age at implantation.

Methods

Ten children participated in this retrospective case series study. All children had evidence of ANSD. All subjects had no cochlear nerve deficiency on magnetic resonance imaging and had used the cochlear implants for a period of 12–84 months. We divided our children into two groups: children who underwent implantation before 24 months of age and children who underwent implantation after 24 months of age. Their auditory and speech abilities were evaluated using the following: behavioral audiometry, the Categories of Auditory Performance (CAP), the Meaningful Auditory Integration Scale (MAIS), the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Standard-Chinese version of the Monosyllabic Lexical Neighborhood Test (LNT), the Multisyllabic Lexical Neighborhood Test (MLNT), the Speech Intelligibility Rating (SIR) and the Meaningful Use of Speech Scale (MUSS).

Results

All children showed progress in their auditory and language abilities. The 4-frequency average hearing level (HL) (500 Hz, 1000 Hz, 2000 Hz and 4000 Hz) of aided hearing thresholds ranged from 17.5 to 57.5 dB HL. All children developed time-related auditory perception and speech skills. Scores of children with ANSD who received cochlear implants before 24 months tended to be better than those of children who received cochlear implants after 24 months. Seven children completed the Mandarin Lexical Neighborhood Test. Approximately half of the children showed improved open-set speech recognition.

Conclusion

Cochlear implantation is helpful for children with ANSD and may be a good optional treatment for many ANSD children. In addition, children with ANSD fitted with cochlear implants before 24 months tended to acquire auditory and speech skills better than children fitted with cochlear implants after 24 months.     

Cochlear implantation in children with cochlear nerve deficiency

Objective

The aim of this study was to report on auditory performance after cochlear implantation in children with cochlear nerve deficiency.

Methods

A retrospective case review was performed. Five patients with pre-lingual profound sensorineural hearing loss implanted in an ear with cochlear nerve deficiency participated in the study. Postoperative auditory and speech performance was assessed using warble tone average threshold with cochlear implant, speech perception categories, and speech intelligibility ratings. All patients underwent high resolution computed tomography and magnetic resonance imaging.

Results

According to Govaerts classification, three children had a type IIb and two a type IIa cochlear nerve deficiency. Preoperatively, four patients were placed into speech perception category 1 and one into category 2. All patients had an improvement in hearing threshold with the cochlear implant. Despite this, at the last follow-up (range 18–81 months, average 45 months), only one girl benefited from cochlear implantation; she moved from speech perception category 2 to 6 and developed spoken language. Another child developed closed set speech perception and had connected speech that was unintelligible. The other 3 children showed little benefit from the cochlear implant and obtained only an improved access to environmental sounds and improved lipreading skills. None of these 4 children developed a spoken language, but they were all full-time users of their implants.

Conclusions

The outcomes of cochlear implantation in these five children with cochlear nerve deficiency are extremely variable, ranging from sporadic cases in which open set speech perception and acquisition of a spoken language are achieved, to most cases in which only an improved access to environmental sound develops. Regardless of these limited outcomes, all patients in our series use their device on a daily basis and derive benefits in everyday life. In our opinion, cochlear implantation can be a viable option in children with cochlear nerve deficiency, but careful counseling to the family on possible restricted benefit is needed.     

Analysis of electrical thresholds and maximum comfortable levels in cochlear implant patients

Objective

It is well known that a proper fitting of the cochlear implant processor is relevant to provide good quality in speech perception. The aim of this study is to extract statistical information to be applied for fitting the processor.

Methods

This study is based on the programming maps of 121 patients, aged from 18 months to 68 years at the moment of implantation. All subjects were implanted with the COMBI 40+ cochlear implant at San Cecilio University Hospital, Granada (Spain). The patients were classified into groups based on their age at implantation: younger than 5 years, between 5 and 16 years, and older than 16 years. The patients in each age-based group were divided into two subgroups, considering whether they had recent hearing experience or not. A special group including patients affected by severe damages in the cochlea was also defined.

Results

Relationships between the programming parameters and factors like the age at implantation, the hearing experience and the presence of severe cochlear damage were found. The THR levels for patients younger than 5 years were significantly lower than those for patients implanted between 5 and 16 years, and this group presented significantly lower THR levels than adults. The MCL levels were not significantly influenced by the age at implantation. A significant increment was observed for both, MCL and THR levels, when patients were affected by severe cochlear damage. A significant increment in the THR levels were observed for patients with no recent hearing experience, while no significant differences were found for MCL levels. This study also analyzes the distribution along the cochlea of the stimulation levels. In the case of patients not affected by severe cochlear damage, the most basal electrodes presented a significant increment in the stimulation levels with respect to the rest of electrodes.

Conclusion

This work provides information of great value for programming the speech processors, particularly when the subjective responses of the patients are not sufficient. The application in our ENT Service has reduced substantially the average time needed to obtain an acceptable fitting of the processor, especially in children. Our study also shows that electrical thresholds are a good indicator of the functionality of the auditory nerve. The analysis of this parameter highlights the importance of an early intervention as well as a deep insertion of the electrode carrier in order to obtain the maximum functionality from the cochlear implant.     

Cochlear implantation in patients with otosclerosis of the otic capsule

Objective

To evaluate outcomes of cochlear implantation of patients with otosclerosis of the otic capsule.

Acquisition of early auditory milestones with a cochlear implant

Objective

Speech acquisition after cochlear implant is a long process. Various studies have followed the auditory milestones in the early period after implantation. The aim of the present study was to track the development of hearing skills in the early period after cochlear implantation and evaluate which factors influence the process.

Methods

195 records of children implanted in the Hadassah Medical Center were examined retrospectively. Data on etiology, age at implantation and type of implant were collected. In addition, information on the rate of progress was measured: the first time that there was detection and identification of Ling sounds, the first time it was possible to obtain SDT (speech detection threshold), SRT (speech reception threshold) and an audiogram, and the first accurate repetition of VCV (vowel consonant vowel) sounds.

Results

Results show a consistent pattern of auditory milestone acquisition similar to that of normal development, from milestones that do not require decoding beginning with SDT, detection of Ling sounds followed by an audiogram which requires cooperation, to tasks that involve decoding starting with SRT and repetition of Ling sounds and finally VCV repetition.The children implanted before 24 months of age achieved the auditory milestones later than children implanted between 2 and 6 years, apparently since these tasks involve cognitive abilities which are not yet developed in the youngest children.Previous hearing experience improved the rate of acquisition of the auditory milestones and progress was faster in the second implanted ear compared to the first implanted ear.

Conclusion

More research is needed to address the relationship between acquisition of early auditory milestones and performance with the cochlear implant later on in life.     

Inner ear pathology of alpha-galactosidase A deficient mice,a model of Fabry disease

Objective

Fabry disease is characterized by genetic alpha-galactosidase A deficiency, resulting in accumulation of glycolipids (GL-3) and tissue damage. Hearing loss is also common and attributed to GL-3 accumulation in the inner ear. The only reported histological studies dealt with murine and human specimens. Accordingly, histopathological studies of the cochlea were performed on an alpha-galactosidase A deficient murine model of Fabry disease, using C57BL6/J mice as the controls.

Methods

The hearing ability was evaluated using the ABR threshold, while cochlear specimens were observed light microscopically and ultrathin temporal bone sections by TEM.

Results

HE staining showed no accumulation of GL-3 or abnormal cochlear morphology in the alpha-galactosidase A deficient mice, but toluidine blue staining and TEM revealed GL-3 accumulation in the stria vascularis and kidney. No GL-3 accumulation was detected in the C57BL6/J controls by either HE staining or TEM. The alpha-galactosidase A deficient mice and the controls showed no clear differences in the ABR threshold (hearing acuity), but for older animals the threshold was higher in the C57BL6/J controls.

Conclusion

In summary, although the alpha-galactosidase A deficient mice showed no clear hearing loss, GL-3 accumulation was demonstrated in the cochlea.     

Auditory dyssynchrony or auditory neuropathy: Understanding the pathophysiology and exploring methods of treatment

Objective

To review the literature on auditory dyssynchrony (AD) or neuropathy which is characterized by absent auditory brainstem responses, normal otoacoustic emissions, and word discrimination disproportional to the pure tone audiogram.

Results

Several papers attempt to estimate the prevalence of AD that seems to range between 0.23 and 2% in infants “at risk” for hearing impairment and it is considered to be responsible for approximately 8% of newly diagnosed cases of hearing loss in children per year. The prevalence gets even higher, reaching 10% in children with permanent hearing loss. It seems that AD is not a single disease but a spectrum of pathologies that affect the auditory pathway at the level of the inner hair cells, the synapses, or the auditory nerve. Many predisposing or contribution factors have been reported, including prematurity, hyperbilirubinemia, hypoxia, low-birth weight, immunological conditions, infectious diseases, etc. Before proposing any method of management, parents should be informed about the variation among patients and the changes that may appear in some children's audition over time. Sigh language, speech and language therapy, hearing-aids, and cochlear implantation have been used in the treatment of AD. In general, AD is rather refractory to conventional amplification and cochlear implantation has been found an effective method of management in young implanted children with severe/profound deafness and AD. However, the situation is more complicated and challenging in milder hearing losses.

Conclusion

The selected and proposed 30 related articles published in the International Journal of Pediatric Otorhinolaryngology aim to stimulate the related research in order to further explore the etiology, pathophysiology, and management of AD.     

An investigation of the first lexicon of Turkish hearing children and children with a cochlear implant

Introduction

Research on early cochlear implantation and first language milestones is limited. To compare language performance in cochlear implant (CI) users and hearing children, the establishment of normative data for both groups would be of benefit. To aid the data collection for Turkish hearing children and children with a CI diaries can be used.

Aims

This study aimed to document the first 100-word lexicon acquired by Turkish hearing children and children with a CI during the first 2 years of hearing experience, to determine the distribution of the first 100-word lexicon per word category, the rate of acquisition of words and the effects of age at implantation on language performance.

Methods

First word data was collected from 63 Turkish hearing children and 71 CI users implanted under 36 months of chronological age using a diary. The mean number of words recorded at each time interval was calculated. The time taken to achieve the first 100-word lexicon and the categories of the first words were documented. Performance under 18 months and over 24 months of age at first fitting was compared.

Results

By 19–21 months of hearing age both hearing and CI user's vocabularies were of similar size. CI users developed a lexicon earlier than hearing children, but once hearing children started to acquire words their acquisition rate was faster. The distribution of words acquired per category were similar. 83% of first words were shared by both groups. No significant difference in performance was found between: hearing versus: implanted children; or earlier (<18 months) versus later (≥24 months) implanted children.

Conclusions

The vocabulary of hearing children compared to CI users are similar in size and the category. Early access to auditory stimuli facilitates children with a hearing loss to develop vocabularies similar to hearing children in the short term.     

Barriers to early pediatric cochlear implantation

Objectives

Universal newborn hearing screening has significantly improved the ability to identify patients with congenital sensorineural hearing loss (SNHL), which results in earlier treatment and better hearing and development outcomes. It is recommended that patients born with SNHL who meet criteria receive cochlear implants (CIs) by a target age of 12 months, however many children are being implanted at an older age. This study aims to describe populations of pre-lingual patients with SNHL that are at risk for delayed implantation and to identify and analyze barriers that cause this delay.

Methods

Charts of patients receiving a CI between January 2008 and June 2012 at a tertiary care cochlear implant center were reviewed retrospectively. We looked at patient demographics, age at hearing loss diagnosis, age at implantation, and etiology of hearing loss. Barriers to implantation were identified through surveys completed by team members.

Results

Fifty-seven CI recipients were identified of which 42 were in patients with pre-lingual SNHL. SNHL etiology included: cochlear dysplasia (18%), GJB2/GJB6 (17%), acquired (10%) extreme prematurity (9%), and idiopathic (46%). The median age of SNHL diagnosis for pre-lingual patients was 15 months. Compared to private insurance, public insurance status was associated with SNHL diagnosis at a significantly later median age (20.0 vs. 4.0 months, p = 0.024), and with a significantly longer median interval from diagnosis to implantation (25.5 vs. 11.0 months, p = 0.029). While cochlear implant team members identified delayed insurance approval and medical comorbidities as reasons for delayed implantation, the most significant factor identified was parental, with delayed/missed appointments or reluctance for evaluations or surgery.

Conclusion

52% of patients with pre-lingual SNHL that met criteria for CI were implanted more than 12 months after diagnosis. Having public or no insurance was significantly associated with delayed implantation. Parental barriers were most common factors cited for delays in implantation. Overcoming these delays necessitates appropriate identification of at risk patients and creating a system to educate families and chaperone them through the process.     

Paediatric cochlear implantation in north-western Nigeria case report and review of our challenges

Objective

To report the commencement of cochlear implantation (CI) in children in Northern Nigeria and review the challenges encountered with a view to establishing CI programme.

Cases

Two children with post-meningitic sensorineural hearing loss had standard pre-implantation assessment and cochlear implantation surgery in Nigeria in conjunction with surgeons from another developing country. Post-implantation meningitis was encountered and managed in one of the patients. Post-CI rehabilitation started in a foreign country and continued in our centre after training of our speech therapist. Both patients had improvement in their hearing.

Conclusion

These showcase successful CI in patients from Sub-Saharan Africa, the challenges included need for development of surgical and post-CI rehabilitation personnel and affordability of the cost of the implant. The support of all the stakeholders needed for commencement of a CI programme was noted.     

A novel frameshift mutation (c.405delC) in the GJB2 gene associated with autosomal recessive hearing loss in two Tunisian families

Objectives

Mutations in GJB2 are found to be responsible for 50% of congenital autosomal recessive non-syndromic hearing loss, one of the most important mutations in this gene is the c.35delG, which is responsible for the majority of GJB2 related deafness in the Tunisian population. The aim of this study was to determine the molecular etiology of hearing loss in two Tunisian individuals.

Methods

We screened two Tunisian individuals affected by congenital, bilateral, profound, sensorineural hearing loss for mutations in GJB2 gene using PCR and direct sequencing.

Results

We identified a novel frameshift mutation in the GJB2 gene, the c.405delC resulting in a truncated protein (p.Tyr136Thrfs*32). It was found in compound heterozygosity with the c.35delG in two non-consanguineous unrelated families from Tunisia. One patient underwent a cochlear implant at 4 years. Initial evaluations post-implantation indicate a successful cochlear implant outcome since the patient began to acquire language abilities and auditory sensation.

Conclusions

With this novel GJB2 mutation, the mutational spectrum of this gene continues to broaden in our population. The occurrence of biallelic GJB2 mutations for the other deaf girl, despite the neonatal pain and hypotension due to complicated delivery, led us to confirm the importance of GJB2 screening for cochlear implant candidates regardless of the etiology of deafness in populations with a relatively high frequency of GJB2 mutation carriers.     

The development of auditory skills in infants with isolated Large Vestibular Aqueduct Syndrome after cochlear implantation

Objective

The purpose of this study was to investigate the auditory performance of infants with isolated Large Vestibular Aqueduct Syndrome (LVAS) after cochlear implantation, compare their performance with those of infants with a normal inner ear, and establish a database of auditory development.

Method

435 infants with congenital severe to profound hearing loss participated in this study. 62 infants in group A were diagnosed with isolated LVAS. 373 infants in group B had a normal inner ear. Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) was used to evaluate the development of auditory skills.

Results

The mean scores for auditory ability showed no significant difference between groups A and B. The mean scores for the three different auditory skills increased significantly over time. The differences were statistically significant in mean scores among the three different auditory skills for group B.

Conclusion

Auditory skills of infants with isolated LVAS developed rapidly after cochlear implantation, in a similar manner to those of infants with a normal inner ear. Cochlear implantation is an effective interventional approach and an established therapeutic option for infants with isolated LVAS.     

Epicutaneous patch test—A new diagnostic option to prevent the rejection of silicone-covered cochlear implants in children

Objectives

Cochlear implantation (CI) is a revolutionary method for hearing rehabilitation in patients with severe or profound sensorineural hearing loss. One of the surgical complications may be the necrosis of the skin flap above the receiver-stimulator coil, resulting in device extrusion. Our aim was to find the plausible causes of the silicone covered implant rejection.

Patients and methods

Authors present four cases of cochlear implant rejection, briefly describe their dermato-surgical solutions and analyse their innovative method – the epicutanoeus patch testing with silicone samples.

Results

They observed positive skin reaction in three of the four cases.

Conclusion

Authors analyse the applicability and results of their surgical solutions and the epicutaneous testing in connection with the prevention of skin flap necrosis and rejection of silicone-covered cochlear implants.