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Children who receive cochlear implants develop spoken language on a protracted timescale. The home environment facilitates speech-language development, yet it is relatively unknown how the environment differs between children with cochlear implants and typical hearing. We matched eighteen preschoolers with implants (31-65 months) to two groups of children with typical hearing: by chronological age and hearing age. Each child completed a long-form, naturalistic audio recording of their home environment (appx. 16 hours/child; >730 hours of observation) to measure adult speech input, child vocal productivity, and caregiver-child interaction. Results showed that children with cochlear implants and typical hearing were exposed to and engaged in similar amounts of spoken language with caregivers. However, the home environment did not reflect developmental stages as closely for children with implants, or predict their speech outcomes as strongly. Home-based speech-language interventions should focus on the unique input-outcome relationships for this group of children with hearing loss.
Sign languages, the languages used by and among deaf people, have long been misunderstood and undervalued. Chapter 13 shows what they really are: human languages. First, we have to rid ourselves of various misconceptions about sign languages. I then formulate the sign language argument for the Innateness Hypothesis, which is based on various parallelisms between signed and spoken languages that strongly suggest that, despite operating in completely different sensory channels, both are likely instantiations of the same mental language system. Both types of languages are processed in the same brain areas and show similar developmental patterns during acquisition and language breakdown. This supports the idea of a genetically anchored default language function for these brain areas. In support of this idea, sign language studies also provide us with examples in which grammatical structure emerges spontaneously when deaf children grow up without being exposed to a sign language. These so-called home sign systems can even give rise to new sign languages. This adds the argument from spontaneous emergence to our list of arguments that potentially support the Innateness Hypothesis.
Chapter 11 reviews an important third argument in support of the Innateness Hypothesis, which is based on another aspect of the maturational nature of language acquisition. Like the argument from stages, this argument directly relates to the process of language acquisition. The crucial point involves the claim, which we will refer to as the Critical Period Hypothesis (CPH), that language acquisition can only be successful if human beings are exposed to language input during a certain (early) period in their life span. The key claim is that language acquisition has to take place within a so-called critical (or sensitive) period, which, for human language, has been suggested to end around puberty. The evidence comes from cases of extreme deprivation, removal of brain tissue in operations or accidents, or from the fact that second language acquisition appears to be much harder after puberty than before it. We discuss various types of evidence that modify the beginning and end of the critical period and also ask why there would be such a period to begin with. We discuss in considerable detail a well-known case of language deprivation involving a girl named Genie.
This study aimed to use short-form visual analogue scale cochlear implantation questionnaires to evaluate subjective aspects at each out-patient visit. The correlation between subjective hearing tests using the short-form visual analogue scale and objective hearing outcomes was evaluated.
Method
This study was conducted in a single centre. Cochlear implant users (n = 199) evaluated their hearing on a scale of 0 to 100 for the right, left and both ears. The Japanese speech perception test (CI-2004) Japanese monosyllable speech perception test (67-S) and cochlear implantation threshold were used for the objective cochlear implantation evaluation.
Results
A significant correlation was found between the short-form visual analogue scale questionnaire and objective hearing outcome, for words (r = 0.64) and sentences (r = 0.62) in CI-2004 and 67-S (r = 0.56) tests. No significant correlation was found between the short-form visual analogue scale score and cochlear implantation threshold (r = −0.18).
Conclusion
Short-form visual analogue scale cochlear implantation questionnaires mean cochlear implant users spend less time answering subjective visual analogue scale questionnaires, and clinicians estimate a patient's cochlear implantation hearing and abnormality by chronological evaluation.
Cochlear Implants (CIs) enhance linguistic skills in deaf or hard of hearing children (D/HH). However, the benefits of CIs have not been sufficiently studied, especially with regard to communicative-pragmatics, i.e., the ability to communicate appropriately in a specific context using different expressive means, such as language and extralinguistic or paralinguistic cues. The study aimed to assess the development of communicative-pragmatic ability, through the Assessment Battery for Communication (ABaCo), in school-aged children with CIs, to compare their performance to a group of children with typical auditory development (TA), and to investigate if CI received under the age of 24 months promotes the typical development of such ability. Results show that children with CIs performed significantly worse than TA on the paralinguistic and contextual scales of the ABaCo. Finally, the age of first implantation had a significant role in the development of communicative-pragmatic ability.
This study aimed to determine anatomical landmarks for accurate and safe middle turn cochleostomy on cadaveric temporal bones.
Methods
In 17 cadaveric wet adult temporal bones, cortical mastoidectomy was performed, followed by extended posterior tympanotomy through which a middle turn opening was created anterior to the stapes footplate. Micro-measurements of various lengths were taken from the cochleostomy to normal middle-ear anatomical landmarks using a digital microscope.
Results
The mean length from the middle turn cochleostomy to the processus cochleariformis was 1.8 ± 0.3 mm and to the tympanic segment of the facial nerve was 2.2 ± 0.3 mm. The mean shortest length from the oval window to the osseous spiral lamina was 2.4 ± 0.3 mm and to the internal carotid artery was 5.0 ± 0.6 mm. The mean shortest length from the round window to the internal carotid artery was 4.3 ± 0.6 mm.
Conclusion
A middle turn cochleostomy can be safely drilled by using the measured lengths in difficult cases.
Cochlear implant is the standard treatment of choice for children and adults with severe to profound sensorineural hearing loss. The main objective of this study was to assess the knowledge, attitude and practices regarding cochlear implant among doctors other than otolaryngologists in a tertiary care academic institution.
Method
A 24-item knowledge, attitude and practices questionnaire was developed based on an extensive literature review and expert opinion and was administered to 100 non-otolaryngologists in a tertiary care academic institution to be completed in about 15 minutes. The data obtained was analysed to assess knowledge, attitude and practices regarding cochlear implant in this group.
Results
The results showed that awareness regarding the option of cochlear implants for elderly and unilateral deafness was deficient. Surgeons and doctors in higher specialties did better when it came to practice related to cochlear implant. The age and experience of doctors also improved knowledge and practice with regards to cochlear implant.
Conclusion
Improving awareness about cochlear implants and their benefits among non-otolaryngology colleagues can ensure that more people who could potentially benefit from cochlear implants will receive appropriate counselling and referral.
Age-related hearing loss (ARHL) is common and a known risk factor for social disengagement in later life. This study explored social functioning following a diagnosis of ARHL. Using a constructivist grounded theory approach we developed an interview schedule to advance a grounded theory from data collected from six older adults who used either hearing aids or cochlear implants. Interview questions concerned social functioning as well as focusing on their perspective of the impact of ARHL on cognitive functioning. We describe a grounded theory conceived as ‘Reconnecting to Others’. This theory posits that participants faced social challenges in relation to their ARHL, and resolved these challenges partly through the use of hearing aids and cochlear implantation. The theory also emphasises the importance of help from other hearing aid users for new users, and corroborates prior findings about strategies older adults with ARHL use to cope with their hearing impairment in various social situations. Once hearing aids and cochlear implants are used and adapted to with the help of peers, participants completed their journey by helping others who had received diagnoses of ARHL. Additionally, participants spoke of the pleasure of hearing again. Interestingly, no participant felt that their ARHL had impacted their cognitive functioning. Our theory provides a basis for explaining existing quantitative findings as well as creating new hypotheses for future testing.
Our study compares the intelligibility of French-speaking children with a cochlear implant (N = 13) and age-matched children with typical hearing (N = 13) in a narrative task. This contrasts with previous studies in which speech intelligibility of children with cochlear implants is most often tested using repetition or reading tasks. Languages other than English are seldom considered. Their productions were graded by naive and expert listeners. The results show that (1) children with CIs have lower intelligibility, (2) early implantation is a predictor of good intelligibility, and (3) late implantation after two years of age does not prevent the children from eventually reaching good intelligibility.
This paper presents our experience on delayed-onset haematoma formation after cochlear implantation, a topic which has not been well discussed in the literature.
Method:
Retrospective case review study.
Results:
Five children who had undergone cochlear implant surgery at 1.5 to 4 years of age (median, 2.5 years) were studied. The haematoma episodes occurred 2–12 years (median, 6 years) after cochlear implantation. Two patients had recurrent episodes. Two of the seven haematoma episodes were managed by needle aspiration alone, four by incision and drainage alone, and one by both needle aspiration and incision and drainage. Other than one patient with coagulopathy, there were no obvious predisposing factors, including trauma.
Conclusion:
The majority of delayed-onset haematomas occurred without obvious predisposing factors. Needle aspiration can differentiate a haematoma from an abscess or cerebrospinal fluid leakage, and it provides an effective immediate therapeutic solution. However, aseptic techniques are emphasised to minimise the chances of an uncomplicated haematoma converting into a septic one.
To compare stimulation parameters of peri-modiolar and anti-modiolar electrode arrays using two surgical approaches.
Methods:
Impedance, stimulation thresholds, comfortably loud current levels, electrically evoked compound action potential thresholds and electrically evoked stapedial reflex thresholds were compared between 2 arrays implanted in the same child at 5 time points: surgery, activation/day 1, week 1, and months 1 and 3. The peri-modiolar array was implanted via cochleostomy in all children (n = 64), while the anti-modiolar array was inserted via a cochleostomy in 43 children and via the round window in 21 children.
Results:
The anti-modiolar array had significantly lower impedance, but required higher current levels to elicit thresholds, comfort, electrically evoked compound action potential thresholds and electrically evoked stapedial reflex thresholds than the peri-modiolar array across all time points, particularly in basal electrodes (p < 0.05). The prevalence of open electrodes was similar in anti-modiolar (n = 5) and peri-modiolar (n = 3) arrays.
Conclusion:
Significant but clinically acceptable differences in stimulation parameters between peri-modiolar and anti-modiolar arrays persisted four months after surgery in children using bilateral cochlear implants. The surgical approach used to insert the anti-modiolar array had no overall effect on outcomes.
GJB2 gene mutations are highly prevalent in pre-lingual hearing loss patients from China. Pre-lingual deafness is a sensorineural disorder that can only be treated with cochlear implantation.
Method:
The prevalence of GJB2 gene mutations was examined in 330 randomly selected patients treated with cochlear implantation.
Results:
Overall, 276 patients (83.64 per cent) carried variations in the GJB2 gene. Seventeen different genotypes were identified, including 10 confirmed pathogenic mutations (c.235delC, c.299delAT, c.176del16, p.E47X, p.T123N, p.V167M, p.C218Y, p.T86R, p.V63L and p.R184Q), 3 polymorphisms (p.V27I, p.E114 G and p.I203 T) and 2 unidentified mutations (p.V37I and c.571 T > C).
Conclusion:
A total of 103 patients (31.2 per cent) carried 2 confirmed pathogenic mutations. The frequency of c.235delC was higher than that reported previously in the Jiangsu province. The two novel mutations identified, 69C > G and 501G > A, are likely to be polymorphisms.
In cochlear implantation, there are two crucial factors promoting hearing preservation: an atraumatic surgical approach and selection of an electrode that does not damage cochlear structures. This study aimed to evaluate hearing preservation in children implanted with the Nucleus Slim Straight (CI422) electrode.
Methods:
Nineteen children aged 6–18 years, with partial deafness, were implanted using the 6-step Skarzynski procedure. Electrode insertion depth was 20–25 mm. Hearing status was assessed with pure tone audiometry before surgery, and at 1, 5, 9, 12 and 24 months after surgery. Electrode placement was confirmed with computed tomography.
Results:
Mean hearing preservation in the study group at activation of the cochlear implant was 73 per cent (standard deviation = 37 per cent). After 24 months, it was 67 per cent (standard deviation = 45 per cent). On a categorical scale, hearing preservation was possible in 100 per cent of cases.
Conclusion:
Hearing preservation in children implanted with the Nucleus CI422 slim, straight electrode is possible even with 25 mm insertion depth, although the recommended insertion depth is 20 mm. A round window approach using a soft, straight electrode is most conducive to hearing preservation.
Auditory implantation into the inner ear is increasingly performed for a variety of indications. Infective complications are rare, but when they occur they can have devastating consequences.
Case reports:
This paper reports two cases where vestibular sequestration of the bony labyrinth developed following implantation into the middle ear.
Conclusion:
To the authors' knowledge, these are the first reported cases where vestibular sequestration has resulted from auditory implant surgery. This paper outlines the radiological changes characteristic of this pathology. It also describes the surgical and conservative treatment options for this condition, challenging the previously accepted belief that affected patients always require aggressive surgical intervention.
A dilemma occurs in the treatment of second-sided Ménière's disease in the only hearing ear, particularly in patients with severe symptoms such as ‘drop attacks’. This paper describes a patient treated with contralateral cochlear implantation prior to vestibular nerve section of the symptomatic ear.
Case report:
A 53-year-old man, with second-sided Ménière's disease and drop attacks in the only serviceable right ear, underwent successful left cochlear implantation 30 years after hearing loss, followed by right vestibular nerve section. The patient achieved control of Ménière's attacks and improved hearing. Although the patient experienced oscillopsia post-operatively, he was satisfied with his improved everyday functioning.
Conclusion:
Patients with severe second-sided Ménière's disease in the only hearing ear are a small but difficult treatment group. In those that are suitable for cochlear implantation in the non-serviceable ear, it is suggested that this be employed prior to surgical treatment of the Ménière's symptoms, even if the implanted ear has had no auditory stimulation for many years.
Cochlear implantation has been used to rehabilitate profoundly deafened adults for more than 25 years. However, surgical labyrinthectomy is often considered a contraindication to cochlear implantation, especially if there is a significant delay between the two procedures. As the role of cochlear implantation continues to expand, this idea requires reconsideration.
Case report:
A 59-year-old woman presented to our clinic after undergoing bilateral surgical labyrinthectomies for intractable Ménière's disease 21 years prior. Despite the significant time delay, she underwent cochlear implantation with a good audiological outcome and improved quality of life.
Conclusion:
Changes to the cochlea and vestibule following surgical labyrinthectomy include cochlear ossification and obliteration of the vestibule. These issues have been thought to limit the potential for cochlear implantation, especially when there is a significant delay between the two procedures. However, delayed cochlear implantation, even decades after labyrinthectomy, remains a viable treatment option which can benefit selected patients.
To assess the reliability of visually assessed thresholds of the electrically elicited stapedius reflex, recorded during cochlear implant surgery, compared with intra-operative tympanometric threshold assessment. Intra-operatively recorded electrically elicited stapedius reflex thresholds vary considerably, and differ from those measured post-operatively by means of impedance changes (i.e. using tympanometry). Thus, any confounding effect of different intra-operative techniques and visual assessment inaccuracies should be excluded.
Methods:
Both techniques (i.e. visual observation and tympanometry) were performed intra-operatively in six patients, and threshold values were compared.
Results:
Recorded electrically elicited stapedius reflex thresholds were very similar for both techniques. Visually assessed thresholds were slightly higher in some cases and lower in others, compared with tympanometric thresholds.
Discussion:
There was almost no difference between reflex thresholds measured with the two different techniques under the same intra-operative conditions. Therefore, we conclude that differences between intra- and post-operative thresholds are not due to the use of different measuring techniques. The main reason for such differences is probably the influence of intra-operative narcotics on reflex thresholds.
Vogt–Koyanagi–Harada disease is a chronic disorder involving the eye and the central nervous, auditory, vestibular and integumentary systems. This study aimed to determine the auditory and vestibular manifestations of this disease.
Methods:
Twenty-four patients diagnosed with Vogt–Koyanagi–Harada disease were assessed for auditory and vestibular dysfunction.
Results:
Uveitis presents in all cases. Sensory hearing loss was present in 50 per cent of cases, tinnitus in 42 per cent, vertigo in 17 per cent and headache in 17 per cent. Nine patients received systemic steroids. Six patients who were treated early regained their hearing, but three patients whose treatment was delayed did not. One patient with bilateral profound hearing loss underwent cochlear implantation, and achieved excellent post-implantation hearing.
Conclusion:
There is a high incidence of cochlear and vestibular end-organ involvement in patients with Vogt–Koyanagi–Harada disease. The adequacy and timing of treatment has a significant effect on the disease outcome. Vogt–Koyanagi–Harada disease appears to affect the inner ear end-organ. Patients who develop bilateral profound sensory hearing loss are suitable candidates for cochlear implantation.
We report a case of Sweet's disease associated with rapid, profound loss of hearing, against a background of progressive, bilateral, sensorineural hearing loss.
Results:
The clinical features were indistinguishable from those of immune-mediated inner ear disease. Establishment of a definitive diagnosis was a challenge due to the absence of a reliable diagnostic test. The patient was unresponsive to extensive immunosuppressive therapy and subsequently underwent cochlear implantation, with good hearing outcomes.
Conclusions:
Profound, bilateral, sensorineural hearing loss in the context of Sweet's disease may be related to the underlying immunological aetiology. Cochlear implantation can successfully restore hearing when immunotherapy fails.
This paper aims to report our experience with different multichannel cochlear implant devices, and to discuss the outcomes of our cochlear implant programme, together with the problems encountered.
Setting:
Cochlear implantation was undertaken in 117 patients (35 post-lingual and 82 pre-lingual cases; 70 males and 47 females) over a 12-year period. Three cochlear implant systems were used: Nucleus (22 and 24), Med-El and Advanced Bionics Clarion. An extended endaural incision was used in 78 cases and a minimally invasive approach in 39 cases.
Results:
Complications occurred in 16.2 per cent of patients. All patients showed a significant post-implantation improvement in their perception and discrimination of sound and speech. Better results were noted in pre-lingual patients under the age of six years. The cause of hearing loss was unknown in 81 per cent of patients.
Conclusion:
The outcomes of our cochlear implantation series are comparable to previous reports. The possibility of an abnormally rotated cochlea should be borne in mind when difficulty is encountered during cochleostomy.