Single-Sided Deafness-Outcomes of Three Interventions for Profound Unilateral Sensorineural Hearing Loss: A Randomized Clinical Trial.

OBJECTIVE: A comparison of three interventions for profound unilateral sensorineural hearing loss. STUDY DESIGN: Prospective, crossover randomized clinical trial. PARTICIPANTS: Fifteen participants with profound unilateral sensorineural hearing loss. INTERVENTIONS: Three potential technical interventions were compared: Bone Conduction Device on softband, Contralateral Routing of Signal (CROS), and Remote Microphone . Each intervention was randomly trialed for a period of 3 weeks, separated by a 1 week washout period. OUTCOME MEASURES: Speech in noise recognition test performed under four conditions (lateral noise poorer ear, lateral noise better ear, speech poorer ear, speech better ear). Standardized questionnaires (Abbreviated Profile of Hearing Aid Benefit, Bern Benefit in Single Sided Deafness Questionnaire, and Speech, Spatial, and Other Qualities 12) were used to evaluate amplification benefit at baseline and following each intervention. RESULTS: The use of remote microphone provided the best results in the speech recognition in noise test. A benefit in some signal-to-noise ratios was presented of the CROS over bone conduction device on softband in the Speech Poor Ear condition. On questionnaires of benefit, participants did not rate a particular intervention as significantly better than any other. Following the study, CROS was the intervention preferred by the 8 of 15 participants (53%). The majority of participants (80%) chose to continue with an intervention rather than no treatment. CONCLUSION: The use of all interventions resulted in increased performance in speech recognition in noise and rated higher on subjective benefits in comparison with baseline. People with SSD are a heterogeneous population when considering perceived difficulties. Future research should focus on segmenting the population of SSD depending on factors such as etiology, high frequency loss in the better ear, and age of acquired loss for the poorer ear. This stratification may possibly increase the benefit for the patient in terms of more individual-based clinical routines.

Internationally Adopted Children With Unilateral Cleft Lip and Palate-Consonant Proficiency and Perceived Velopharyngeal Competence at the Age of 5.

OBJECTIVE: To compare consonant proficiency, consonant errors, and the perceived velopharyngeal (VP) competence in internationally adopted (IA) children with unilateral cleft lip and palate (UCLP) and nonadopted (NA) children with the same cleft-palate type at age 5. DESIGN: Case-control study based on phonetic transcriptions of standardized speech recordings of 5-year-olds at a tertiary hospital. PARTICIPANTS: Twenty-five IA children were compared to 20 NA children. All consecutive patients at a cleft lip and palate center participated. MAIN OUTCOME MEASURE(S): Consonant proficiency was measured using percentage consonants correct, percentage consonants correct-adjusted for age, percentage correct place, percentage correct manner, and consonant inventory. Cleft speech characteristics (CSCs), developmental speech characteristics (DSCs), and the perceived VP competence were also measured. RESULTS: The IA children had significantly lower values for all consonant proficiency variables (p < .05) and a smaller consonant inventory (p = .001) compared to the NA children. The IA children had a higher frequency of CSCs (IA = 84%, NA = 50%, p < .05) and DSCs (IA = 92%, NA = 65%, p = .057), and twice as many IA children as NA children had perceived VP incompetence (IA = 52%, NA = 25%, p = .17). CONCLUSIONS: Severe speech disorder was more common in IA children than in NA children at age 5. Most importantly, the speech disorders seem to be not only cleft-related. More detailed speech assessments with a broader focus are needed for IA children with UCLP. Longitudinal studies are recommended to further investigate the impact of speech difficulties in IA children's daily lives.

Surgical, speech, and hearing outcomes at five years of age in internationally adopted children and Swedish-born children with cleft lip and/or palate.

Internationally adopted children (IAC) with a cleft lip and/or palate (CL/P) tend to arrive with un-operated palates at an age at which their Swedish-born peers have completed their primary palate surgery. Our aim of the present study was to analyze surgical, speech and hearing outcomes of IAC at age 5 and compare with those of a matched group of Swedish-born children. Fifty children with CL/P born in 1994-2005 participated in the study. Twenty-five IAC were matched according to age, sex and cleft type with 25 Swedish-born children. Audio recordings were perceptually analyzed by two experienced, blinded speech-language pathologists. Hearing and speech statuses were evaluated on the same day for all children. Surgical timing and complications as in fistulas and requirement for secondary velopharyngeal (VP) surgery, speech evaluation results, and present hearing status were analyzed for all children of age 5 years. Results showed that primary palatal surgery was delayed by a mean of 21 months in IAC. IAC had a higher prevalence of velopharyngeal impairment that was statistically significant, a higher fistula rate, and experienced more secondary surgery than Swedish-born peers. Hearing loss due to middle ear disease was slightly more common among IAC, whereas the rate of treatment with tympanostomy tubes was similar between the two groups. In conclusion, IAC with CL/P represent a challenge for CL/P teams because of the heterogeneous nature of the patient group and difficulties associated with delayed treatment, and the results show the importance of close follow-up over time.

Speech production in 3-year-old internationally adopted children with unilateral cleft lip and palate.

BACKGROUND: In the last decade, a large number of children with cleft lip and palate have been adopted to Sweden. A majority of the children were born in China and they usually arrive in Sweden with an unoperated palate. There is currently a lack of knowledge regarding speech and articulation development in this group of children, who also have to deal with a late first language switch. AIMS: To study consonant proficiency in 3-year-old internationally adopted children with unilateral cleft lip and palate (UCLP) compared with peers with UCLP born in Sweden. Also to study the type and frequency of consonant errors and to perceptually compare velopharyngeal competence between the groups. METHODS & PROCEDURES: Thirty-two children born between 2006 and 2010 with UCLP participated in the study-14 adopted from China and 18 children born in Sweden. Both groups were treated by the same cleft palate team. Audio recordings at 3 years of age were perceptually analysed by blinded listeners. Consonant proficiency was measured via per cent consonants correct adjusted for age (PCC-A), per cent correct manners (PCM) and per cent correct places (PCP). The prevalence of audible nasal air leakage and velopharyngeal competence were judged and compared between groups. The type and frequencies of consonant errors related to place and manner of articulation were also analysed. OUTCOMES & RESULTS: The internationally adopted children had significantly fewer correct consonants compared with the Swedish-born children. This was true for PCC-A, PCP and PCM. This group also had significantly higher prevalence of glottal stops/fricatives and deleted target consonants more often. Also the internationally adopted children had a higher prevalence of incompetent velopharyngeal function. The only outcome variable with similar results in the groups was audible nasal air leakage. CONCLUSIONS & IMPLICATIONS: The present study indicated that there were significant differences regarding consonant proficiency and velopharyngeal competence between internationally adopted children with a UCLP and their Swedish-born peers with UCLP at the age of 3 years. Internationally adopted children with UCLP should be considered an at risk group for a higher prevalence of speech difficulties than non-adoptees. Thus, it is particularly important to follow this group of children over time. Longitudinal studies of speech and language development in internationally adopted children with UCLP are needed.

Severe to profound hearing impairment: quality of life, psychosocial consequences and audiological rehabilitation.

PURPOSE: To study the quality of life (QoL) and psychosocial consequences in terms of sick leave and audiological rehabilitation given to patients with severe to profound hearing impairment. METHOD: A retrospective study of data on 2319 patients with severe to profound hearing impairment in The Swedish Quality Register of Otorhinolaryngology, followed by a posted questionnaire including The Hospital Anxiety and Depression Scale (HADS). RESULTS: The results indicate greater levels of anxiety and depression among patients with severe or profound hearing impairment than in the general population, and annoying tinnitus and vertigo had strong negative effects on QoL. The proportion of sick leave differed between the studied dimensions in the study. The proportion of patients who received extended audiological rehabilitation was 38% in the present study. CONCLUSIONS: Treatment focused on anxiety, depression, tinnitus and vertigo must be given early in the rehabilitation process in patients with severe or profound hearing impairment. Because sick leave differs greatly within this group of patients, collaboration with the regional Social Insurance Agency is crucial part of the rehabilitation. The study also shows that presently, only a small proportion of patients in Sweden with severe to profound hearing impairment receive extended audiological rehabilitation. Implications for Rehabilitation Greater levels of anxiety and depression have been found among patients with severe or profound hearing impairment than in the general population, and annoying tinnitus and vertigo have strong negative effects on QoL in this group of patients. Only a small proportion of patients with severe to profound hearing impairment receive extended audiological rehabilitation today, including medical, technical and psychosocial efforts. Extended audiological rehabilitation focused on anxiety, depression, tinnitus and vertigo must be given, together with technical rehabilitation, early in the rehabilitation process in patients with severe or profound hearing impairment.

Speech and hearing in adults with 22q11.2 deletion syndrome.

The purpose of the study was to investigate the prevalence of velopharyngeal impairment, compensatory articulation, reduced intelligibility, and to rate the general impression of speech in adults with 22q11.2 deletion syndrome. The second purpose was to study the prevalence and type of hearing impairment in these adults. A referred, consecutive series of 24 adults with confirmed 22q11.2 deletion, 16 female and 8 males, with a mean age of 25 years (19-38 years) was included in the study. A blind assessment of speech by three experienced speech-language pathologists was performed. Sixteen (66%) patients had a mild to severe velopharyngeal impairment. The most prevalent symptoms of velopharygeal impairment were hypernasality and audible nasal airflow. The mean nasalance score was 33% (6-66%). Only two patients had disordered articulation; one of these had glottal articulation. A mean of 96% (88-100%) of single words were rated to be intelligible. To achieve these results half of the patients previously had velopharyngeal flap surgery. Forty-one percent (9/22) had mild-moderate hearing impairment; three had sensorineural type, four conductive and two had a mixed type. In conclusion the majority of the patients had no articulation errors and good intelligibility; while one-third still had moderate to severe problems with velopharyngeal impairment. Around 40% still had some hearing impairment, in most cases with a mild to moderate conductive component. Thus, a high prevalence of speech and hearing problems seems to be a part of the phenotype in adults with 22q11.2DS.

Cochlear implant combined with a linear frequency transposing hearing aid.

BACKGROUND: Adults with cochlear implants (CIs) are usually implanted unilaterally. To preserve binaural advantages, a noninvasive method involves maintaining the hearing aid (HA) on the contralateral ear; the choice of HA for this purpose is therefore crucial. In recent years, the use of frequency transposition has gained a renewed interest in clinical practice. This type of processing records information from the high-frequency region and conveys it to a low-frequency region where there is still some residual hearing. PURPOSE: To conduct an investigation and examine whether adults with unilateral CI derive benefits from a HA utilizing linear frequency transposition (LFT) on the contralateral ear. RESEARCH DESIGN: A two-period, single-blind, repeated-measures crossover design was conducted to examine the combination of LFT in conjunction with a CI. Speech recognition tests were performed in quiet and in noise with LFT either activated or deactivated. The Speech, Spatial and Qualities of Hearing Questionnaire (SSQ) was used to measure subjective benefit. STUDY SAMPLE: The participants were nine frequent bimodal users, five males and four females, with a moderate to profound high-frequency sensorineural hearing loss in the nonimplanted ear. INTERVENTION: The current study was conducted using the Widex Mind440 power (m4-19) behind-the-ear HA. The participants acted as their own control in a total of seven conditions: (1) bimodal with own HA, (2) CI only, (3) own HA alone, (4) bimodal new HA LFT-off, (5) new HA LFT-off, (6) bimodal new HA LFT-on, and (7) new HA LFT-on. DATA COLLECTION AND ANALYSIS: Monosyllabic words in quiet and the Swedish version of Hearing in Noise Test (HINT) were used as speech test materials. Stimuli were presented in sound field at a speech level of 65 dB sound pressure level (SPL) via a loudspeaker at a distance of 1 m from the participant in a sound-treated room. The SSQ was administered in each session evaluating the three bimodal conditions. SPSS software was used for statistical analyses. General linear model (GLM) analysis of variance for repeated measures was performed and followed with Bonferroni-adjusted post hoc pairwise comparisons. RESULTS: Participants performed better with CI only than with HA alone, and the bimodal conditions were superior to the CI alone. No significant differences (p > .05) were observed when comparing the LFT-on with LFT-off regardless of whether the use of CI was included in the different listening conditions in objective and subjective measurements. CONCLUSIONS: The results suggest an advantage for CI patients with a HA in the opposite ear, and that the LFT neither degraded nor enhanced speech performance in conjunction with a CI in quiet or in noise in comparison to when it was deactivated.

Audiological evaluation and self-assessed hearing problems in subjects with single-sided congenital external ear malformations and associated conductive hearing loss.

Previously, unilateral hearing impairment (UHI) has been considered of little consequence. However, a recent meta-analysis of children with UHI displayed educational and behavioural problems and possible delays of speech and language development. Further, patients with UHI consequently report hearing difficulties. Our study investigated hearing function, possible inner ear protection, and self-assessed hearing problems in 57 subjects aged between 3-80 years with single-sided congenital ear malformations and conductive UHI. Pure-tone thresholds and speech recognition (quiet, noise) were measured, and all patients completed a self-assessment questionnaire. Pure-tone thresholds corresponding to sensorineural function did not significantly differ between the normal (air conduction) and affected ear (bone conduction). However, speech recognition in both quiet and in noise was normal on the non-affected side but significantly worse on the malformed side. A moderate to high degree of self-assessed hearing problems were reported. In conclusion, hearing function in the affected ear was found to be subnormal in terms of supra threshold signal processing. Furthermore, a high degree of hearing difficulty was reported. Therefore, active treatment, surgery, or hearing amplification, might be considered.

BAHA in children and adolescents with unilateral or bilateral conductive hearing loss: a study of outcome.

OBJECTIVES: Bilateral BAHAs in adults with bilateral hearing loss (BHL) have proven to be superior to unilateral fitting, in both audiologically measurements and in overall patient satisfaction. There have been no similar studies in children. Furthermore, a recent meta-analysis of children with unilateral hearing loss (UHL) has shown numerous negative consequences. The objectives of the study were to investigate whether fitting of bilateral BAHAs in children with conductive BHL give additional hearing benefits, to investigate the effects of unilateral hearing aids in children with conductive UHL, and to identify different aspects of auditory problems in children with conductive UHL or BHL. STUDY DESIGN: This was a prospective study involving 22 children with either conductive UHL (unaided or with unilateral hearing aid) or conductive BHL (with unilateral or bilateral BAHAs) and 15 controls. METHODS: Baseline audiometry, tone thresholds in a sound field, speech recognition in noise and sound localization were tested without, and with unilateral and bilateral hearing aids. Two questionnaires, MAIS & MUSS and IOI-HA, were completed. RESULTS: Two problem areas were identified in the children with hearing impairment: in reactions to sounds and in intelligibility of speech. An additional BAHA in the children with BHL resulted in a tendency to have improved hearing in terms of better sound localization and speech recognition in noise. Fitting of unilateral hearing aids in the children with UHL gave some supplementary benefit in terms of better speech recognition in noise but no positive effect on ability to localize sound could be detected. Even so, all children fitted with hearing aids - either unilaterally or bilaterally - reported a positive outcome with their devices in the self-assessment questionnaire. CONCLUSIONS: Children with either UHL or BHL displayed several problems within the hearing domain. Fitting of bilateral BAHAs in children with BHL and of a single-sided hearing aid in children with UHL appears to have some supplementary audiological benefits and also renders high patient satisfaction. In order to investigate the possible supplementary effects of hearing aids, a 3-month trial of BAHA on Softband, either unilaterally or bilaterally, may be of value in children with conductive UHL or BHL, respectively.

Language skills in 5-8-year-old children with 22q11 deletion syndrome.

BACKGROUND: Language impairment and delayed language onset have been described, although not investigated in detail, in children with 22q11 deletion syndrome. AIMS: To investigate different areas of language: the ability to retell a narrative, phonology, syntax and receptive vocabulary in a group of 5-8-year-old children with 22q11 deletion syndrome regardless of whether or not they had a history of speech and language difficulties. Gender differences were also investigated. METHODS & PROCEDURES: Nineteen consecutively referred children with 22q11 deletion syndrome, ten girls and nine boys, between the ages of 5 and 8 years, participated in the study. The mean full-scale IQ of the group was 78. Six children had an autism spectrum disorder, attention deficit/hyperactivity disorder, or a combination of these. Three different language tests were used: (1) the Bus Story - a test of narrative speech and language; (2) an articulation test including all Swedish phonemes in different positions; and (3) the Peabody Picture Vocabulary Test - Revised (PPVT-R). OUTCOMES & RESULTS: All but two children had an information score in the retelling task of 1 SD below the population mean. A negative correlation between age and the information score implied that the older the children, the more severe the problems. One child had an average sentence length within the normal limits and five children had subordinate clauses within normal limits. A median of 4% of the utterances included grammatical errors. About 50% of the children had a complete consonant inventory. The phonological process analysis implied delayed rather than deviant development. The group had a moderately low score for receptive vocabulary. CONCLUSIONS: Language difficulties in all investigated areas of language were found. It is suggested that speech-language impairment is a common feature of 22q11 deletion syndrome. An implication of these results is that follow-ups of language skills are important not only for pre-school children, but also for school age children and adolescents with 22q11 deletion syndrome.

Prevalence of dizziness and vertigo in an urban elderly population.

The prevalence of balance symptoms (vertigo, dizziness, and dysequilibrium) was investigated in an epidemiological study of elderly people, the longitudinal and cross-sectional gerontological and geriatric population study from Göteborg, Sweden (H70). Three different age cohorts were studied, one at age 70, one at age 75 and one at ages 79, 82, 85, 88 and 90 years. Altogether 2011 participants answered the questionnaire at 3197 occasions. The overall prevalence of balance problems at age 70 was 36% (women) and 29% (men). Balance symptoms were more common among women than men, and increased with increasing age. At ages 88-90 years the corresponding values were 51-45%. The most common symptom was poor balance/general unsteadiness (11-41%). Rotatory symptoms occurred in 2-17%. Other types of symptoms were less common. Precipitating factors were rising from supine to sitting position in 17-40%. Balance symptoms in a side position were uncommon, but occurred more often when tilting the head backwards (up to 14%). Signs that possibly could indicate neurological involvement were uncommon. Falls in conjuncture to dizziness, vertigo and similar symptoms occurred in 7-15%, in about equal proportions indoors as outdoors.

A prospective cross-sectional study of speech in patients with the 22q11 deletion syndrome.

UNLABELLED: The purpose of this study was to investigate a consecutive series of 65 participants between 3 and 33 years of age (median age of 9 years and 4 months) with a confirmed 22q11.2 deletion, in order to ascertain the frequency and severity of articulation difficulties, velopharyngeal impairment (VPI), and the level of intelligibility. The majority had velopharyngeal impairment; over half of them to such a degree that surgery had been performed or was considered necessary. A high level of correct place and manner of consonants was only found in children with the 22q11 deletion syndrome from age 6. The most misarticulated consonants were stops and fricatives. Glottal articulation assessed in words and sentences was less frequent than expected according to earlier studies. A high prevalence of reduced intelligibility at different ages indicates an obvious communication limitation in younger children, and for some individuals even as teenagers and adults. EDUCATIONAL OBJECTIVES: As a result of this activity, the participant will have knowledge about the frequency and severity of: (1) articulation difficulties; (2) velopharyngeal impairment; and (3) the level of intelligibility in patients with a 22q11.2 deletion.